Clinical Associate Professor, Cardiothoracic Surgery
BS, Massachusetts Institute of Technology, Biology and Humanities (1963)
MD, University of Pittsburgh (1967)
IntroductionQTc prolongation has been reported in adults following cardiopulmonary bypass; however, this phenomenon has not been studied in children with congenital cardiac disease. This study's aim was to formally assess QTc in children undergoing cardiac surgery.Pre-operative and post-operative electrocardiograms during hospital stays were prospectively analysed on 107 consecutive patients under 18 years of age undergoing cardiac surgery. QTc was measured manually in leads II, V4, and V5. Measurements of 440 and 480 milliseconds were used to categorise patients. Peri-procedural data included bypass and cross-clamp time, medications, and electrolyte measurements. Outcome data included arrhythmias, length of mechanical ventilation, and hospital stay. Patients with post-operative new bundle branch block or ventricularly paced rhythm were excluded.In all, 59 children were included, out of which 26 had new QTc over 440 milliseconds and 6 of 59 had new QTc over 480 milliseconds post-operatively. The mean increase in post-operative QTc was 25 milliseconds, p=0.0001. QTc over 480 was associated with longer cross-clamp time, p=0.003. Other risk factors were not associated with post-operative QTc prolongation. This phenomenon was transient with normalisation occurring in 67% of patients over 60 hours on average. One patient with post-operative QTc over 440 milliseconds developed ventricular tachycardia. There was no correlation between prolonged QTc and duration of mechanical ventilation, or hospital stay.ConclusionA significant number of children undergoing cardiac surgery showed transient QTc prolongation. The precise aetiology of QT prolongation was not discerned, though new QTc over 480 milliseconds was associated with longer cross-clamp time. In this cohort, transient QTc prolongation was not associated with adverse sequela.
View details for DOI 10.1017/S1047951111000175
View details for Web of Science ID 000293593800005
View details for PubMedID 21362209
The Eighth International Conference of the Pediatric Cardiac Intensive Care Society was held in Miami, Florida, December 8 to 11, 2010. The program included a session dedicated to the management of hypoplastic left heart syndrome (HLHS), with particular emphasis on the innovations that have led to contemporary schemes of management and the role of clinical trials in the evolution and acceptance of these strategies. An invited panel of experts reviewed the historical evolution of staged surgical reconstruction, the randomized clinical trials that have been undertaken thus far, and the extent to which these have, or have not, influenced individual and institutional approaches to management of HLHS.
View details for DOI 10.1177/2150135111406942
View details for PubMedID 23803996
IntroductionIn patients with varying degrees of left heart hypoplasia, it is often difficult to determine whether the left heart structures are adequate in size to support biventricular circulation. Historically, the decision to pursue a single ventricle or biventricular repair needed to be made early and was often irreversible. The hybrid procedure may be a better initial approach for patients with borderline left ventricles.We describe a series of four patients with various congenital cardiac malformations, all of whom had borderline left ventricles. Based on pre-operative echocardiograms, several scoring systems and left ventricle volumes were used to predict the optimal type of repair. A left ventricular volume of 20 millilitres per square metre was used as the minimum cut-off value for adequacy of biventricular repair.The left ventricular volumes for the patients were 17.1, 23.7, 25.4, and 25.8 millilitres per square metre. In none of the four patients were the calculations unanimous in the recommendation to pursue either type of repair. All patients underwent the hybrid procedure and then eventual single ventricle palliation (two patients) or biventricular repair (two patients). All survived with a mean follow-up of 18 plus or minus 3.9 months.The hybrid procedure may be the best option in patients with a borderline left ventricle. It can serve as a bridge to a more definitive repair when patients are older, larger, and for whom the decision between single ventricle and biventricular repair can be more easily made.
View details for DOI 10.1017/S1047951110001423
View details for Web of Science ID 000285981400005
View details for PubMedID 21070689
Epicardial pacing is the standard approach for permanent pacing in small children and patients with functionally univentricular physiology. The longevity of epicardial leads, however, is compromised by increased occurrences of exit block and lead fractures. We report our experience with a technique of placing a second ventricular lead, and attaching it to the atrial port of a dual chamber pacemaker to prevent the need for early re-operation in the event of failure of the primary epicardial lead. A retrospective review showed that, over the period from 2001 through 2007, epicardial ventricular pacemakers had been placed in 88 patients. In 6 of these, we had placed 2 ventricular leads, their median weight being 8.0 kilograms, with a range from 4.2 to 31.8 kilograms. Fracture of a lead occurred in 1 of the patients (17%) 8 months after placement, requiring reprogramming to pace from the atrial port. This possibility avoided the need for repeated emergent surgery. At a median follow-up of 1.5 years, with a range from 0.3 to 4.4 years, there have been no complications. During the same time period, overall failure of epicardial leads at our institution was 13%. Placement of a second ventricular epicardial pacing lead, attached to the atrial port of a dual chamber pacemaker, therefore, may provide a safe and effective means of ventricular pacing in the setting of epicardial lead failure, and may obviate the need for repeat, potentially urgent, pacemaker surgery.
View details for DOI 10.1017/S1047951109003710
View details for Web of Science ID 000266792000003
View details for PubMedID 19272204
There will be a continuing need to start new congenital heart surgery programs to serve communities experiencing significant population growth. However, small congenital heart programs frequently underperform their larger counterparts. This study summarizes the clinical outcome data for the first 42 months of a small, start-up congenital heart surgery program. Clinical outcomes were summarized from the start of the program in September 2003 through March 2007. Risk adjustment analysis was performed using the risk adjustment in congenital heart surgery (Rachs-1) risk adjustment model and Society of Thoracic Surgeons (STS) congenital database. Three hundred eighty-six operations have been performed during this time period, including 47 newborns, 96 infants, 217 children/adolescents, and 26 adults (greater than 18 years). There have been two operative mortalities (0.5%). Assigning these cases to the Rachs-1 categories, there were 64 level I, 188 level II, 90 level III, 12 level IV cases, with 32 "others." The predicted mortality for the 354 categorized cases calculates to be 17.4 (Rachs-1) and 10.2 (STS). The data demonstrate that a start-up program with a relatively modest surgical volume can achieve satisfactory clinical results. This model has relied upon careful case selection and direct, senior-level surgeon involvement through an affiliation with a university-based program. These results suggest that a small congenital heart surgery program can be successfully started if the circumstances are carefully controlled.
View details for DOI 10.1111/j.1540-8191.2008.00675.x
View details for Web of Science ID 000260499400007
View details for PubMedID 19016985
Our approach to the extracardiac conduit Fontan operation has evolved over time from full-pump, to partial-pump, to completely off-pump. This study is designed to report our overall experience with the extracardiac conduit Fontan operation and to evaluate the evolution in bypass technique on postoperative outcomes.From September 1992 to April 2005, 285 patients, median age 4.5 years (1.4-44 years), median weight 16 kg (9.4-94 kg), underwent a primary extracardiac conduit Fontan procedure. Early and late outcomes were analyzed for the entire cohort and for 2 patient groups depending on whether an oxygenator was used in the bypass circuit (166 patients; 58%) or not (119 patients; 42%).Early failure (including death and takedown) occurred in 7 patients (2.5%). Prevalence of new early postoperative sinus node dysfunction necessitating a permanent pacemaker was 0.4%, and that of new tachyarrhythmias necessitating discharge home on a regimen of antiarrhythmia medications was 2.5%. Ten-year actuarial freedom from Fontan failure, new sinus node dysfunction necessitating a permanent pacemaker, and reoperation for conduit thrombosis or stenosis was 90%, 96%, and 98%, respectively. Fenestration rate was lower (P = .001) in the no-oxygenator group (8%) than in the oxygenator group (25%). Patients in the no-oxygenator group had lower intraoperative Fontan pressure (12.0 +/- 2.3 vs 13.5 +/- 2.4 mm Hg, P < .001), common atrial pressure (4.6 +/- 1.8 vs 5.3 +/- 1.8 mm Hg, P = .003), and transpulmonary gradient (7.5 +/- 2.1 vs 8.3 +/- 2.2 mm Hg, P = .013) than did the oxygenator group.The extracardiac conduit Fontan operation coupled with minimal use of extracorporeal circulation is associated with favorable intraoperative hemodynamics, low fenestration rate, minimal risk of thrombosis or stenosis, and minimal early and late rhythm disturbance.
View details for DOI 10.1016/j.jtcvs.2006.05.066
View details for PubMedID 17059923
The use of a right ventricle to pulmonary artery (RV-PA) conduit in the Norwood procedure has been proposed to increase postoperative hemodynamic stability. A valve within the conduit should further decrease RV volume load. We report our clinical experience with this modification.From February 2002 through August 2005, we performed 88 consecutive Norwood procedures using RV-PA conduits. We used composite valved conduits made from cryopreserved homograft and polytetrafluoroethylene (PTFE) in 66 cases (54 pulmonary, 12 aortic homografts), other valved conduits in 14, and unvalved PTFE in 8 cases. Hospital survival was 88.6% overall and increased to 93.1% after the initial year. Early interventions were required in 18 patients (16 for cyanosis). Prestage II cardiac catheterization was performed at a mean age of 126 days. Mean Qp/Qs was 1, with mean aortic saturation 71%, mean O2 extraction 24%, and mean right ventricular end-diastolic pressure 9 mm Hg. Patient weight, use of an aortic homograft valve in the conduit, stage I palliation within the first year of our experience, and low O2 extraction and high transpulmonary gradient prestage II were risk factors for overall death. Early interventions were more frequent in aortic valve conduits compared with all other conduits.The valved RV-PA conduit was associated with low early mortality after the Norwood procedure. The majority of these patients had normal cardiac output and well-maintained RV function. There may be a higher risk for early conduit interventions and death when aortic valve homografts are used in the RV-PA conduit.
View details for PubMedID 16820644
A 2-day-old male with pulmonary atresia and an intact ventricular septum underwent central aortopulmonary shunt placement and right ventricular outflow tract reconstruction. Postoperatively, the patient had difficulty weaning from mechanical ventilation and was found to have a large thrombus occluding the entire right lower pulmonary artery. The patient underwent successful percutaneous catheter-based thrombectomy in the early postoperative period with the Possis AngioJet catheter. The patient had immediate improvement in gas exchange and was able to be weaned from the ventilator shortly thereafter. This represents the first published use of mechanical thrombectomy in a postoperative neonate.
View details for DOI 10.1002/ccd.20518
View details for PubMedID 16216022
View details for Web of Science ID 000232956403391
An isolated left common carotid artery (LCA) is an extremely rare condition with only four reported cases. In each case, the isolated carotid artery connects to the right or left pulmonary artery via the ductus arteriosus and the embryologic basis for the abnormalities is believed to reflect an error in the development of the branchial arches. We present a case of an isolated LCA connecting to the main pulmonary artery in association with a right aortic arch and an anomalous origin of the left subclavian artery from the descending aorta. The left ligamentus arteriosus was identified separately. This may represent a disturbance in the septation of the truncoaortic sac secondary to abnormal migration of neural crest cells rather than a pure developmental anomaly of the branchial arches.
View details for DOI 10.1007/s00246-004-0896-x
View details for Web of Science ID 000233663800041
View details for PubMedID 15565269
The two main theories regarding the pathogenesis of coarctation of the aorta are the Skodaic hypothesis of ductal tissue constricting the aorta at the level of the insertion of the ductus arteriosus and the flow theory of decreased ascending aortic blood flow in the fetus, which results in associated isthmic narrowing and a localized shelf. To document that ectopic ductal tissue constriction can cause coarctation of the aorta in the absence of a patent ductus arteriosus, we report three cases of infants presenting with critical coarctation who responded to prostaglandin E1 infusion without opening the ductus arteriosus.
View details for DOI 10.1007/s00246-003-0549-5
View details for Web of Science ID 000188455300010
View details for PubMedID 15043008
The extant nomenclature for mitral valve disease is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. Mitral valve disease has been subdivided into stenotic and regurgitant lesions. Lesions have been characterized further by etiology and by anatomic location: supravalvar, valvar, and subvalvar. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.
View details for PubMedID 10798424
The bidirectional Glenn procedure (BDG) is used in the staged surgical management of patients with a functional single ventricle. Controversy exists regarding whether accessory pulmonary blood flow (APBF) should be left at the time of BDG to augment systemic saturation or be eliminated to reduce volume load of the ventricle. The present study was a retrospective review of patients undergoing BDG that was conducted to assess the influence of APBF on survival rates.From 1986 through 1998, 149 patients have undergone BDG at our institution. Ninety-three patients had elimination of all sources of APBF, whereas 56 patients had either a shunt or a patent right ventricular outflow tract intentionally left in place to augment the pulmonary blood flow provided by the BDG. The operative mortality rate was 2.2% without APBF and 5.4% with APBF. The late mortality rate was 4.4% without APBF and 15.1% with APBF. Actuarial analysis demonstrates a divergence of the Kaplan-Meier curves in favor of patients in whom APBF was eliminated (P<0.02). One hundred seven patients have subsequently undergone completion of their Fontan operation, so the actuarial analysis includes the operative risk of this second operation.The results suggest that the elimination of APBF at the time of BDG may confer a long-term advantage for patients with a functional single ventricle.
View details for PubMedID 10567295
View details for PubMedID 10593280
The pulmonary autograft procedure for the treatment of aortic valve disease was developed and performed by Ross in 1967. The results he published in 1987 prompted increasing interest in the procedure. The International Registry of the Ross Procedure was established in 1993 to further examine longitudinal clinical outcomes.The results from the Ross registry document the continued and growing interest in the procedure with 2,523 patients currently enrolled, representing 122 centers and 166 surgeons worldwide.Mortality (1987 to present) reported in the registry is 2.5%. It should be noted that follow-up stands at 70%. The most important issues for the registry to track are the incidence of reoperation for autograft failure and the fate of the pulmonary homograft. Reoperation for all valve-related problems is low (5.4%), with an autograft explant rate of 1.9%. Overall registry data indicate that the right ventricular outflow tract revision rate is 2.8%, with this decreasing by half to 1.3% in the 1987 to present subgroup.Rigorous analysis of outcomes is difficult with registry follow-up currently at 70%; however, the general conclusions derived from the registry are supported by other individual series with excellent follow-up. Success of the registry depends on judicious efforts by all participating surgeons and coordinators in documenting long-term patient results and reporting them to the registry.
View details for Web of Science ID 000078397000030
View details for PubMedID 9930440
Children with functionally single ventricle in the setting of visceral heterotaxy (isomerism) may present a surgical challenge at the time of Fontan completion because of anomalies of systemic and pulmonary venous drainage. We have used an extracardiac conduit in this population to direct inferior caval venous blood to the pulmonary arteries. Over the past five years, nine children with heterotaxy and a functionally single ventricle underwent correction by placement of an extracardiac synthetic (Gore-Tex) conduit. All patients had previously undergone a bidirectional Glenn procedure. Age and weight at the time of insertion of the extracardiac conduit were 26 +/- 15 months, and 11 +/- 2 kilograms, respectively.Of the nine children, six had an uneventful recovery. One developed elevated venous pressures and required a 'fenestration procedure'. Two patients developed pleural effusions. Median length of stay in hospital was 10 days. All children are alive and well, with follow-up of 19 +/- 16 months. There have been no thromboembolic complications.The extracardiac conduit has worked well in our experience for the completion of the Fontan circulation in children with functionally single ventricle in the setting of visceral heterotaxy.
View details for Web of Science ID 000077254300013
View details for PubMedID 9855102
The modified Fontan procedure separates the systemic and pulmonary circulations in patients born with a functional single ventricle. Delayed recovery is frequently observed after this procedure. It was our hypothesis that complement activation or cytokine generation may contribute to the pathophysiology of this problem.We measured activated complement C3, thromboxane B2, interleukin-6, and tumor necrosis factor-alpha levels by immunoassay in 16 patients undergoing Fontan procedure. Patient plasma samples were obtained preoperatively, on initiation of cardiopulmonary bypass, after administration of protamine, and 1, 4, 8, and 24 hours postoperatively.There was no early or late mortality in this cohort of patients. Low cardiac output developed in 3 of 16 patients, and pleural effusions developed in 5. The median length of hospital stay was 9 days. Activated complement C3 levels increased from a baseline of 1,486 +/- 564 to 4,600 +/- 454 ng/mL after cardiopulmonary bypass and administration of protamine, and returned to baseline by 24 hours. The level of interleukin-6 increased from 42 +/- 32 to 176 +/- 22 pg/mL and at 24 hours remained elevated at 71 +/- 15 pg/mL. Neither thromboxane B2 nor tumor necrosis factor-alpha levels increased significantly.The data demonstrate threefold to four-fold increases in activated complement C3 and interleukin-6, indicating that both humoral and cellular systems are affected. It is our conclusion that complement and cytokine activation may contribute to the delayed recovery observed after Fontan procedure.
View details for Web of Science ID 000074274100050
View details for PubMedID 9647087
The purpose of this study was to assess the neurodevelopmental status of children after Fontan repair of functional single ventricle and to examine the relationship between cognitive function and selected patient characteristics.Neurodevelopmental tests including the Stanford-Binet Intelligence (IQ) scale and the Developmental Test of Visual Motor Integration (VMI) were administered to 32 children (26 months to 16 years of age) with complex single ventricle. The mean and distribution of IQ and VMI scores were compared with population norms. The relationship between test scores and patient characteristics was examined utilizing analysis of variance and correlational methods.The majority of children had intellectual function within the normal range (mean, 97.5 +/- 12.1). Below average VMI scores were found in 21.4% of children. There were no significant correlations between intellectual function or visual motor integration ability and preoperative oxygen saturation or age at Fontan. Children who had deep hypothermic circulatory arrest during a prior Norwood procedure tended to have a lower IQ score.Intellectual development in children with Fontan repair of complex heart defects is essentially within the normal range. Visual motor integration deficits may be more prevalent in these children. In our population, the duration and degree of preoperative hypoxemia had no apparent effect on cognitive function.
View details for Web of Science ID 000072843200011
View details for PubMedID 9521946
Pediatric cardiac care is costly and requires extensive resources. We studied the effect of clinical pathways on practice patterns and patient care outcomes in infants and children hospitalized for cardiac surgery.In consecutive patients admitted for selected cardiac surgical procedures before (n = 69) and after (n = 173) implementation of clinical pathways, outcomes including hospital length of stay, days in the ICU, time to extubation, ordering of blood studies, costs, and readmissions were compared. Data were analyzed for each of five cardiac surgical procedures: repair of an atrial septal defect, repair of a ventricular septal defect, division of a patent ductus arteriosus, repair of tetralogy of Fallot, and neonatal arterial switch operation to correct transposition of the great arteries.A significant reduction in length of hospital stay, including days in the ICU (decreased 1 to 2 days per admission), was achieved after the clinical pathway was implemented. Reductions in average duration of mechanical ventilation ranged from 28% for repair of a ventricular septal defect to 63% for repair of tetralogy of Fallot. The number of blood studies ordered decreased 20% to 30%. A significant reduction in hospital costs for each procedure, ranging from 16% to 29%, was also achieved with no adverse effects on patients' outcomes.Use of clinical pathways with children hospitalized for cardiac surgery can shorten length of stay in the hospital, reduce use of resources, and improve cost-effectiveness with beneficial outcomes for patients.
View details for PubMedID 9509223
Type B interrupted aortic arch with ventricular septal defect is a complex congenital heart defect that may have associated left ventricular outflow tract obstruction. Surgical management has evolved from a two-stage approach to the currently favored single-stage approach. The following data summarize our experience with the two-stage approach over a 15-year period.Between 1980 and 1995, 27 consecutive patients with type B interrupted aortic arch and ventricular septal defect underwent surgical management using the two-stage approach. There were 15 girls and 12 boys; 21 patients had the DiGeorge syndrome.Stage I was performed at a median age of 4 days. Twenty-six (96%) of 27 patients survived first-stage palliation. One patient survived stage I palliation but died before undergoing stage II. Twenty-five patients underwent second-stage repair at a median age of 6 weeks (range, 1 to 46 weeks). There were 2 early deaths and 1 late death. Actuarial analysis demonstrates 1- and 5-year survival rates of 85% and 81%, respectively. Twenty-two survivors have been followed up for an average of 8 +/- 2 years. Freedom from reoperation for arch graft enlargement has been 86% at 3 years and 55% at 5 years. Freedom from reoperation for left ventricular outflow tract obstruction has been 82% at both 3 and 5 years.The two-stage approach can achieve good mid- to long-term palliation of patients with type B interrupted aortic arch and ventricular septal defect. These results should provide a reference from which to gauge the long-term success of the single-stage approach.
View details for Web of Science ID 000071469400051
View details for PubMedID 9436572
Fluid retention is a common problem following transannular patch repair of tetralogy of Fallot. The present study was undertaken to evaluate whether humoral substances may contribute to this process. Patients undergoing tetralogy of Fallot repair using a transannular patch technique were compared to patients undergoing simple ventricular septal defect repair. Hormone levels were determined by radioimmunoassay. Fluid retention was defined as pleural effusions persisting beyond 5 days. The data demonstrate that patients undergoing tetralogy of Fallot repair had elevated levels of anti-diuretic hormone, renin, angiotensin II, and aldosterone as compared to a group with ventricular septal defect. The elevation in hormone levels in the tetralogy of Fallot group was principally accounted for by those who developed effusions postoperatively. These results suggest that activation of the renin angiotensin-aldosterone system may be one of the contributing factors to fluid retention following tetralogy of Fallot repair.
View details for Web of Science ID A1996VG27600022
View details for PubMedID 8880028
Pleural effusions after the modified Fontan procedure are unpredictable, increase morbidity, and prolong hospital stay. To assess the relation between preoperative characteristics and postoperative pleural drainage, we performed a retrospective study of 71 patients who underwent Fontan procedures. Analyses revealed no significant relation between duration of effusion and age at Fontan, preoperative oxygen saturation, pulmonary artery pressure, ventricular end-diastolic pressure, type of Fontan, or prior cavopulmonary anastomosis. Patients with significant aortopulmonary collateral vessels evidenced by angiographic opacification of the pulmonary arteries or veins had more prolonged pleural drainage. The duration of the pleural drainage was significantly less in patients who had aortopulmonary collateral occlusion.
View details for Web of Science ID A1996UP98200017
View details for PubMedID 8644596
The purpose of this study was to analyze the efficacy and safety of intravenous amiodarone in young patients with critical, drug-resistant arrhythmias.Intravenous amiodarone has been investigated in adults since the early 1980s. Experience with the drug in young patients is limited. A larger pediatric study group was necessary to provide responsible guidelines for the drug's use before its market release.Eight centers obtained institutional approval of a standardized protocol. Other centers were approved on a compassionate use basis after contacting the primary investigator (J.C.P).Forty patients were enrolled. Standard management in all failed. Many patients had early postoperative tachyarrhythmias (25 of 40), with early successful treatment in 21 (84%) of 25. Twelve patients had ventricular tachyarrhythmias: seven had successful therapy, and six died, none related to the drug. Eleven patients had atrial tachyarrhythmias: 10 of 11 had immediate success, but 3 later died. Fourteen patients had junctional ectopic tachycardia, which was treated with success (sinus rhythm or slowing, allowing pacing) in 13 of 14, with no deaths. Three other patients had supraventricular tachycardias, with success in two and no deaths. The average loading dose was 6.3 mg/kg body weight, and 50% of patients required a continuous infusion. Four patients had mild hypotension during the amiodarone bolus. One postoperative patient experienced bradycardia requiring temporary pacing. There were no proarrhythmic effects. Deaths (9 [23%] of 40) were not attributed to amiodarone.Intravenous amiodarone is safe and effective in most young patients with critical tachyarrhythmia. Intravenous amiodarone can be lifesaving, particularly for postoperative junctional ectopic tachycardia, when standard therapy is ineffective.
View details for Web of Science ID A1996UD65100041
View details for PubMedID 8609351
This article summarizes the case histories of six infants who had isolated secundum-type atrial septal defects (ASDs) and presented with failure to thrive in the first year of life. Patients underwent operative closure of their ASD with the expectation that this would improve their feeding and growth pattern. Five of the six children demonstrated little or no improvement in symptoms following operation. Each of these five patients have subsequently shown signs of developmental delay. Our experience with this group suggests that failure to thrive associated with a secundum ASD will frequently have a noncardiac basis.
View details for Web of Science ID A1996UR41900007
View details for PubMedID 8811405
An unusual case of an interatrial communication similar to a sinus venosus defect associated with an overriding pulmonary venous chamber is reported. There was no direct connection of the pulmonary venous system with either the superior vena cava or the low right atrium, as is usual with sinus venosus defects. This defect may represent the result of a sinus venosus defect associated with cor triatriatum.
View details for Web of Science ID A1996TL38400013
View details for PubMedID 8778706
Pulmonary artery distortion is a risk factor among candidates for the Fontan procedure. In 57 patients evaluated by catheterization after successful cavopulmonary anastomosis, 8 had proximal left pulmonary artery (LPA) stenosis, either discrete (4 patients) or long segment (4 patients). Median age was 27 months (range 19 to 60 months). Median weight was 11.4 kg (range 9.1 to 20.0). Mean diameter at LPA stenosis was 4.4 +/- 0.4. Proximal right pulmonary artery mean diameter was 10.4 +/- 1.0 mm. After angiographic and hemodynamic assessment, short 11F sheaths were placed in the right internal jugular (6 patients) or subclavian veins (2 patients). Pulmonary artery angioplasty and stent placement were performed. LPA stenoses were enlarged using 10 Palmaz stents dilated to 10 mm (7 patients) or to 12 mm (3 patients). Poststent angiograms showed that narrowest LPA dimensions were significantly enlarged to 9.9 mm +/- 1.0 mm, p < 001). There were no complications. Follow-up studies (catheterizations in 4 patients, echocardiograms in 8 patients) were performed 4 to 9 months after stent implantation. No restenosis was observed. Five patients had completion of their Fontan procedures; three patients are pending Fontan completion. This study demonstrates the efficacy and safety of the percutaneous use of Palmaz stents to correct pulmonary artery stenosis in young children after cavopulmonary anastomosis.
View details for Web of Science ID A1995TH12600014
View details for PubMedID 7484776
The modified Fontan procedure has become the treatment of choice for patients born with a univentricular heart. Although the operative mortality has steadily decreased in recent years, the hospital stay is still prolonged in many patients due to fluid retention and pleural effusions.We retrospectively analyzed subsets of patients undergoing the bidirectional cavopulmonary shunt (BDCPS) and modified Fontan procedure in an attempt to define factors influencing operative mortality and morbidity.Multivariate analysis of 64 patients undergoing BDCPS revealed that age 6 months or less, concomitant operation, mean pulmonary pressure of 15 mm Hg or less, and mean pulmonary artery ratio of 1.8:1 or less were not statistically significant indicators of risk. Abnormal pulmonary artery architecture was a significant predictor of early and late death (p < or = 0.01). Retrospective analysis of 71 patients undergoing the modified Fontan procedure revealed no significant relationship between duration of pleural effusions and age at operation, preoperative oxygen saturation, pulmonary artery pressure, ventricular end-diastolic pressure, or prior BDCPS: Patients with important aortopulmonary collateral vessels defined by angiography had prolonged pleural drainage. Selective use of the total extracardiac conduit and the fenestrated Fontan resulted in low overall mortality with no statistical differences between subsets of patients undergoing different modifications of the Fontan procedure.These data indicate that the operative risk for BDCPS or modified Fontan procedure is quite low if a procedure appropriate for the patient is selected. Abnormal pulmonary artery architecture is an important risk factor for death after the BDCPS: Aortopulmonary collateral vessels are associated with fluid retention and pleural effusions after a modified Fontan procedure. Important aortopulmonary collateral vessels should be occluded before or during the modified Fontan procedure.
View details for Web of Science ID A1995TW27300018
View details for PubMedID 8604933
The bidirectional Glenn (BDG) is frequently used in the staged surgical management of single ventricle patients. Controversy exists whether accessory pulmonary blood flow (APBF) sources should be left at the time of the BDG to augment systemic saturation or should be eliminated to reduce volume load of the ventricle. The present study was a retrospective review to assess the influence of APBF on outcome after the BDG.Ninety-two patients have undergone BDG at our institute during the interval from 1986 through 1994. At the time of BDG, 40 patients had either a systemic-to-pulmonary artery shunt or patent right ventricular outflow tract as an additional source of pulmonary blood flow. Fifty-two patients had elimination of APBF. There were three operative deaths (two with and one without APBF) and four procedures (two in each group) that failed and required subsequent revision. Thus, there were 85 patients who underwent successful operation. Effusions (defined as chest tube drainage exceeding 7 days' duration) occurred in 8 of 85 patients; this complication was seen in 7 of 36 patients (19%) with APBF and 1 of 49 patients (2%) without APBF (P < .05). There were 11 deaths, including 6 patients (17%) with APBF, 2 patients (4%) without APBF, and 3 of the patients (75%) who had a failed BDG.The data suggest that morbidity and mortality are lower in patients in whom APBF is eliminated at the time of the BDG.
View details for PubMedID 7586426
Compared the effects of two methods of preparing children for cardiac surgery. Forty-eight child-caregiver dyads were assigned to a treatment group, receiving information and coping skills training, or a control group, receiving information as routinely provided. Treatment group children were more cooperative and less upset in the hospital; better adjusted at home after discharge; higher functioning in school; and their functional health status improved faster. Treatment group caregivers expressed greater confidence in their ability to care for their children in hospital and at home after discharge. Results are discussed in terms of the cognitive appraisal model of stress coping using a multidimensional assessment of treatment effects.
View details for Web of Science ID A1995QY99500005
View details for PubMedID 7595819
Mortality is high for children with pulmonary atresia, intact ventricular septum, and important connections between the right ventricle and the coronary arteries because of myocardial ischemia: in systole, suprasystemic right ventricular pressure delivers deoxygenated blood to the coronary artery (or arteries) and in diastole, the right ventricle provides a lower resistance alternative to coronary perfusion of the myocardium. Tricuspid valve closure was performed in 10 such children. None had stenosis of native coronary arteries. A trial of tricuspid valve closure (by balloon) was performed in the cardiac catheterization laboratory in 5 of 10 patients. Seven of 10 children survived surgical closure of the tricuspid valve plus concurrent procedures; none had heart block. Two of the 3 nonsurvivors were probably in inoperable condition due to preoperative myocardial ischemia. Before operation, 4 patients had ischemic changes on electrocardiograms; these changes were abolished after operation. Three of 10 patients have had a Fontan operation with 2 survivors. We conclude that children with pulmonary atresia, intact ventricular septum, important connections between the right ventricle and the coronary arteries, and normal native coronary arteries should have surgical closure of the tricuspid valve within the first year of life and treated thereafter as patients with "tricuspid atresia."
View details for Web of Science ID A1995QP48700024
View details for PubMedID 7695421
Pleural effusions are a troublesome complication following bidirectional Glenn and Fontan procedures. It was our hypothesis that effusions may be related to alterations in hormones that regulate fluid homeostasis. We made serial determinations (by radioimmunoassay) of antidiuretic hormone, cortisol, aldosterone, angiotensin II, and renin in patients undergoing bidirectional Glenn (n = 16) and Fontan procedures (n = 24). There were six patients who developed effusions following surgery. These patients had a different endocrinological pattern characterized by persistent elevation in renin (28 +/- 9 vs 9 +/- 5 ng/mL per hour, p < 0.01) and angiotensin II (110 +/- 33 vs 33 +/- 14 ng/L, p < 0.01) on the fifth postoperative day as compared to patients who did not develop effusions. These data demonstrate that patients who develop effusions following bidirectional Glenn and Fontan procedures have activation of their renin-angiotensin system.
View details for Web of Science ID A1995QR19800003
View details for PubMedID 7772874
The Damus-Kaye-Stansel operation is useful in the management of complex congenital heart defects. We reviewed our experience with 23 patients who underwent a Damus-Kaye-Stansel procedure. The anastomotic technique was individualized depending on the anatomy. The aortic and pulmonary artery incisions were carried into the sinuses of Valsalva in 9 patients, the aorta was transected in 11 patients, and a patch was used to augment the anastomosis in 13 patients. Concurrent procedures included a Fontan operation (n = 9, mortality = 0), right ventricle-pulmonary artery conduit (n = 5, mortality = 0), bidirectional Glenn procedure (n = 6, mortality = 1), and central aortopulmonary shunt (n = 3, mortality = 2; emergency = 1). Survival is 87% with a median follow-up of 7 years (range, 2 months to 9.2 years). Four patients underwent late revision of the Damus-Kaye-Stansel connection. All survivors are asymptomatic. We conclude that the Damus-Kaye-Stansel connection provides excellent midterm results when the proximal anastomosis is adapted to the anatomy of the patient.
View details for Web of Science ID A1994PU98600010
View details for PubMedID 7979722
Thyroid hormone has numerous effects on cardiovascular function in the adult. The present study was undertaken to evaluate the effects of cardiopulmonary bypass and deep hypothermia on thyroid function in the neonate. Ten newborns were studied preoperatively and postoperatively. The total and free triiodothyronine, total and free thyroxine, thyroid-stimulating hormone, and thyroglobulin levels were measured by immunoassays. The data demonstrated a transient rise in the free thyroxine level associated with and followed by significant reductions in the free and total triiodothyronine, total thyroxine, thyroid-stimulating hormone, and thyroglobulin levels in the early postoperative period. By the fifth postoperative day, the free and total triiodothyronine and total thyroxine levels were returning toward the preoperative levels under the influence of an elevated thyroid-stimulating hormone level. These results suggest that the combination of cardiopulmonary bypass and deep hypothermia can result in a transient suppression of the pituitary-thyroid axis in the neonate.
View details for Web of Science ID A1994PL57200028
View details for PubMedID 7944754
Diminished cardiac function is a common manifestation following the modified Fontan procedure. Since thyroid hormone has important effects on cardiovascular function, the present study was undertaken to evaluate changes in thyroid hormone levels following this operation. A control group consisting of children undergoing open heart procedures other than a Fontan procedure was also evaluated. Serum total and free triiodothyronine (T3), total and free thyroxine (T4), thyroid stimulating hormone (TSH), and thyroglobulin were measured by immunoassays. The Fontan group demonstrated an initial increase in free T4, while free T3, total T3, total T4, TSH, and thyroglobulin were reduced. Over the subsequent days, free T4 decreased to below the preoperative value. By the fifth and eighth postoperative days, free T3, total T3, free T4, and total T4 remained reduced, while TSH and thyroglobulin began increasing toward the preoperative levels. The control group also demonstrated decreases in free T3 and TSH. However, these values had returned to baseline by the fifth postoperative day. The results indicate that children undergoing open heart surgery have suppression of the pituitary-thyroid axis, and that this is prolonged in patients undergoing Fontan procedure. The decreased levels of T3 following Fontan procedure may have adverse effects on the recovery of patients undergoing this operation.
View details for Web of Science ID A1994NN76200006
View details for PubMedID 8054727
Fluid retention is a frequent complication after the bidirectional Glenn and Fontan procedures. It was our hypothesis that this fluid retention may have a hormonal basis. To test this hypothesis, we made serial determinations of antidiuretic hormone, cortisol, aldosterone, angiotensin, and renin before and after operation in patients undergoing a bidirectional Glenn (n = 15) or Fontan (n = 18) procedure. Patients undergoing a bidirectional Glenn procedure had elevations in antidiuretic hormone, cortisol, and aldosterone immediately after operation. However, none of the hormonal elevations persisted by the fifth postoperative day. In contrast, patients undergoing a Fontan procedure had elevations in antidiuretic hormone, cortisol, aldosterone, renin, and angiotensin II in the immediate postoperative period. Five days postoperatively, the renin and angiotensin II levels remained elevated compared with the preoperative values. The data demonstrate that patients undergoing a bidirectional Glenn procedure and those undergoing a Fontan procedure have different patterns of hormonal response. These results suggest that hormonal factors may contribute to fluid retention after these operations.
View details for Web of Science ID A1994MR31800011
View details for PubMedID 8279919
Omental flaps have been used for the treatment of infected aortic prosthetic grafts in adults. We report the case of a 26-month-old male who developed a mycotic aneurysm 9 months following insertion of a ventricle to pulmonary artery conduit. The aneurysm was excised and the reconstructed outflow tract wrapped with omentum. The patient has not had recurrence of his aneurysm following this course of management.
View details for Web of Science ID A1994MU85600007
View details for PubMedID 8148544
View details for PubMedID 7509702
Since September 1979, 53 patients have required operation for systemic atrioventricular valve regurgitation at Children's Hospital and Health Center of San Diego. (Primary repairs of atrioventricular canal defects are excluded from this report.) Diagnoses include single ventricle, cardiomyopathy, congenital mitral insufficiency , Marfan's disease, rheumatic heart disease, and a history of prior repair of atrioventricular canal defect. Ages ranged from 4 months to 19 years; median age is 5 years. In 31 patients, the atrioventricular valve could be repaired. In 24 patients, the valve was replaced (including two patients previously repaired). There were four operative deaths, all in the valve replacement group: three following valve replacement, and one following emergency thrombectomy. Two early failures in the repair group required valve replacement. Techniques for repair included leaflet resection, commissural annuloplasty, ring annuloplasty, and chordal shortening. Follow-up reveals good-to-excellent status in 38 patients. There were seven late deaths: six following valve replacement (one death valve related). Current surgical technique permits repair of the systemic atrioventricular valve in many infants and children requiring operation for regurgitation. The long-term results of valve repair are good to excellent. Repair avoids the morbidity and mortality of valve replacement, e.g., anticoagulation, fixed orifice size, and catastrophic mechanical valve malfunction.
View details for Web of Science ID A1993MJ30200001
View details for PubMedID 8286865
Pulmonary atresia with intact ventricular septum has continued to have a high surgical mortality. This may relate to the nonuniformity of the anomaly. We have developed a management algorithm based on the right ventricular size and coronary anatomy. Patients with a well-developed ventricle and normal coronary arteries have undergone right ventricular outflow procedures. The adequacy of their right ventricles is subsequently evaluated; some patients are candidates for a four-chamber repair, whereas others are candidates for a Fontan procedure. Patients with severe hypoplasia of the right ventricle frequently have extensive ventriculo-coronary connections, and for these patients we have developed the technique of right ventricular obliteration. Over the past 7 years, we have treated 20 patients with pulmonary atresia with intact ventricular septum. Fourteen of the 20 patients underwent outflow tract procedures, with no operative mortality. There were two late deaths in this group. Six patients had hypoplasia of the ventricle with ventriculo-coronary connections and underwent right ventricular obliteration. There was one operative death and one late death in the group. Overall, 95% survived the neonatal period, and 80% were still alive at the time this article was written with an average follow-up of 32 months. We conclude that pulmonary atresia with intact ventricular septum can be successfully managed with the use of an algorithm based on ventricular size and coronary anatomy.
View details for Web of Science ID A1993MA73300022
View details for PubMedID 8412270
Thoracic aneurysms are relatively rare in infancy. We report our experience with two such patients, one with a false aneurysm of the ascending aorta and the other with a mycotic aneurysm of the main pulmonary artery. Neck cannulation prior to sternotomy, using cannulae designed for extracorporeal membrane oxygenation, allowed entry into the mediastinum under controlled circumstances and permitted the successful repair of the aneurysms.
View details for Web of Science ID A1993MC04500004
View details for PubMedID 8219534
The etiology of absent pulmonary valve syndrome is unknown. Several theories have been advanced to explain the associated findings of a rudimentary pulmonary valve, ventricular septal defect, aneurysmal pulmonary arteries, and absence of the ductus arteriosus. The patient presented here would contradict these currently held theories. A review of the literature follows the case report.
View details for Web of Science ID A1993KU10400007
View details for PubMedID 8461497
Common pulmonary vein atresia is a rare form of cyanotic congenital heart disease in which the pulmonary veins join to form a blind confluence that does not communicate with the heart or the major systemic veins. Twenty-one cases have been reported since the lesion was first described in 1962; only two patients with this lesion have survived. Over a 4-year period, common pulmonary vein atresia was diagnosed in five newborns referred to the San Diego Regional Extracorporeal Membrane Oxygenation Program. All five improved dramatically as a result of venoarterial bypass. Congenital heart disease was diagnosed at autopsy in the initial case and by cardiac ultrasound and/or catheterization in the others. Surgical repair was attempted in three neonates; all three required continued extracorporeal membrane oxygenation support postoperatively because of pulmonary hypertension and severe pulmonary parenchymal disease. One infant died of respiratory insufficiency at 3 months of age. The other two survived and were discharged from the hospital. The diagnostic and therapeutic dilemmas posed by this lesion and the life-saving potential for extracorporeal membrane oxygenation in this rapidly fatal cardiac anomaly are the bases of this report.
View details for Web of Science ID A1993KK57800021
View details for PubMedID 8424018
Mediastinitis following congenital heart surgery is relatively uncommon but is usually seen in the setting of postoperative low cardiac output. Conservative treatment utilizing debridement and irrigation is associated with significant morbidity and mortality. We report the successful application of the omental transfer technique in the treatment of mediastinitis in a 6 month old.
View details for Web of Science ID A1992JL05900011
View details for PubMedID 1392236
Since September 1985, 60 cryopreserved homograft valves (12 aortic and 48 pulmonary) have been implanted in the pulmonary position in 56 patients. There were 34 primary operations and 26 reoperations. The ages ranged from 15 days to 22 years; the follow-up ranged from 1 month to 5 1/2 years. The operative mortality was 3.3% (2/56). There were two late deaths (not valve related). Five patients underwent replacement of the homograft; in one patient the homograft was replaced with a heterograft valve. Pathological analysis of the explanted homografts revealed calcification of the wall with satisfactory leaflet function. The leaflets were relatively acellular. No evidence of inflammation or rejection was detected. Four explants were performed for nonvalve-related indications. There are 51 long-term survivors with a homograft in place. Forty-eight are in excellent condition; three are mildly symptomatic (not valve related). Benign pulmonary insufficiency murmurs are present in 29 patients. The presence of trivial or mild insufficiency was independent of the technique of implantation. In general, cryopreserved homograft valves function well in the right ventricular outflow tract. Longer term follow-up is necessary to confirm the superiority of the cryopreserved homograft when compared to the porcine heterograft in the right ventricular outflow tract.
View details for Web of Science ID A1991GX17200018
View details for PubMedID 1810558
The Damus-Kaye-Stansel operation is a useful technique for the treatment of complex cyanotic congenital heart disease when there is obstruction between the systemic ventricle and the aorta. Modifications of the technique include transection of the aorta and the pulmonary artery, anastomosis of the contiguous aortic and pulmonary walls, and connection of the distal aorta to the perimeter of the new bivalved proximal great artery. In addition, the bidirectional cavopulmonary shunt technique can be used with or without the Fontan procedure. Six patients underwent a Damus-Fontan operation, and all survived. Two patients underwent the Damus-cavopulmonary shunt (hemi-Fontan) procedure, and 1 survived. The postoperative status of the 7 survivors is good to excellent. Follow-up ranges from 2 months to 7 1/2 years.
View details for Web of Science ID A1991GG67900060
View details for PubMedID 1898172
The Epstein-Barr Virus (EBV) causes infectious mononucleosis, and has been strongly associated with certain human cancers. The virus is thought to exclusively bind to B lymphocytes and epithelial cells via receptors (CR2/CD21) that also interact with fragments of the third component of complement (C3). Recent evidence, however, has challenged this belief. We have used two-color immunofluorescence analysis using biotin-conjugated EBV and streptavidin-phycoerythrin along with fluorescein-conjugated anti-T cell antibodies and demonstrated that CD1-positive, CD3-dull (immature) human thymocytes express functional EBV receptors. In four replicate experiments, the binding of EBV to thymocytes ranged between 8 and 18%. This interaction is specific as evidenced by inhibition with nonconjugated virus, anti-CR2 antibodies, aggregated C3, and an antibody to the gp350 viral glycoprotein that the virus uses to bind to CR2. EBV can infect the thymocytes as evaluated by the presence of episomal EBV-DNA in thymocytes that had been incubated with the virus as short as 12 days or as long as 6 weeks. Episomal DNA analysis was performed by Southern blotting with a EBV-DNA probe that hybridizes to the first internal reiteration of the viral DNA. The presence of the EBV genome is also supported by the detection of EBV nuclear antigen 1 in infected thymocytes, assessed by Western blotting with EBV-immune sera. The EBV infection is specific as determined by blocking experiments using anti-CR2 and anti-gp350 antibodies. Finally, virus infection of thymocytes can act synergistically along with interleukin 2 and induce a lymphokine-dependent cellular proliferation. In view of previously reported cases of EBV-positive human T cell lymphomas, the possibility is raised that EBV may be involved in cancers of T lymphocytes that have not been previously appreciated.
View details for Web of Science ID A1991FE12800020
View details for PubMedID 1706754
The bidirectional cavopulmonary shunt improves systemic arterial oxygen saturation without increasing ventricular work or pulmonary vascular resistance. Since 1983, 17 patients have undergone a cavopulmonary shunt procedure (five primary operations, 12 secondary operations). Diagnoses were single ventricle complex (n = 4), hypoplastic right heart syndrome (n = 10), and hypoplastic left ventricle (n = 3). Age at primary operation ranged from 3 1/2 to 30 months (median 6 months). Weight ranged from 3.5 to 9.7 kg. Age at secondary operation ranged from 10 months to 14 years (median 15 months). Seven cavopulmonary shunt operations were performed without cardiopulmonary bypass (six via thoracotomy and one via sternotomy) and 10, with cardiopulmonary bypass. All patients in the bypass group had additional procedures: takedown of modified Blalock-Taussig shunt, seven patients; revision of right ventricular outflow tract, four patients; reconstruction of pulmonary arteries, four patients; tricuspid valvuloplasty, one patient; and Damus procedure, one patient. There was one (1/17) operative death (Damus procedure). One patient required early revision. Follow-up ranges from 1 to 53 months (median 23 months). Twelve of 16 had a good to excellent late result, with a rise in mean arterial oxygen saturation from 69% to 83%. Three patients died late (4 to 53 months) (pulmonary vascular disease, pulmonary arteriovenous malformations, and pneumonia, one patient each). There was one late failure (converted to Glenn shunt). The cavopulmonary shunt is an excellent palliative procedure when right atrium-pulmonary artery connection (modified Fontan) must be deferred because of age, weight, or anatomic considerations. Five patients have undergone right atrium-pulmonary artery connection later. In addition, at the time of the modified Fontan operation, the cavopulmonary shunt approach may optimize the anatomic connection (eight additional patients).
View details for Web of Science ID A1990DN23500004
View details for PubMedID 1694938
Of 581 aortic allografts implanted since 1967, 421 were analyzed for structural deterioration. This series is unique in that it includes patients from the early allograft experience. All allografts were cleanly procured, antibiotic sterilized, and either stored at 4 degrees C for up to 8 weeks or frozen to liquid nitrogen temperatures with cryopreservation to preserve the viable cusp fibroblasts. There were 25 frozen mounted aortic valves with a median time to valve failure of 12.1 years, which was not significantly different from the 12.5-year period for 114 fresh free-sewn aortic valves. The median time to valve failure was 6.6 years for 90 fresh-mounted aortic valves and 8.6 years for 192 fresh-mounted mitral valves (p = 0.05). The difference between all mounted and unmounted grafts was significant (p = 0.0001). In all groups, viable fibroblasts were present in specimens explanted up to 5 years after the operation. All specimens returned after more than 10 years were almost totally acellular. Evidence of increased collagen, suggesting that the fibroblasts survive implantation and then gradually die, was present in all specimens. This series suggests that durability of the unmounted viable allograft for aortic valve replacement is greater than for other types of tissue valves. Pre-mounted allografts for aortic or mitral valve replacement have a median survival of 8 years and are not more durable than other tissue valves.
View details for Web of Science ID A1989AE79300008
View details for PubMedID 2739425
Since 1979, 17 infants and children have undergone reoperation for systemic atrioventricular (AV) valve regurgitation 6 weeks to 7 years after repair of congenital heart defects. Prior operations were repair of incomplete or complete AV canal (14 patients), Mustard repair of complex transposition of the great arteries including ventricular septal defect closure (2 patients), or first-stage operation for hypoplastic left heart (1 patient). Age ranged from 6 months to 11 years. In 12 of the 17 patients (10, AV canal; 1, transposition; 1, hypoplastic left heart), valve reconstruction was possible. Operative techniques included a combination of septal cleft approximation, leaflet resection, commissural annuloplasty, or ring annuloplasty. There were no operative deaths, and there were no reoperations in the repair group. The condition of these patients has improved. Follow-up ranges from 1 month to 9 years (mean follow-up, 4.1 years). Five of the 17 patients (4, AV canal; 1, transposition) underwent valve replacement. There were no operative deaths. Follow-up ranges from 3 to 8 years. Three patients later underwent re-replacement of the prosthetic valve; there was 1 late death. The condition of all 4 survivors is improved. Substantial AV valve regurgitation can occur months or years after repair of congenital heart defects. A combination of reconstructive techniques may be useful in preserving native valve function and avoiding systemic AV valve replacement.
View details for Web of Science ID A1989U441000007
View details for PubMedID 2712624
In seven children with obstructed right ventricle-to-pulmonary artery conduits, balloon dilatation (BD) was performed 10-58 months after insertion of the conduit. Average valve gradient was reduced from 69 to 32 mmHg. Obstruction at the conduit-branch pulmonary artery connection became apparent after dilatation of the valve; these distal stenoses also were dilated. It is concluded that the useful lifetime of a right ventricle (RV) to pulmonary artery (PA) conduit may be extended by BD of an obstructed biological valve and/or BD of a stenotic conduit-pulmonary artery anastomosis.
View details for PubMedID 2980058
Sixteen patients with complex cyanotic congenital heart disease underwent an operation involving the use of the aorta and the main pulmonary artery as the egress for systemic arterial blood flow (the Damus-Kaye-Stansell operation, modified as discussed). The indication for this approach was either preoperative aortic outlet obstruction or likelihood that the repair would produce such obstruction. A modification of the original technique is introduced that involves transection of both great arteries, anastomosis of the contiguous aortic and pulmonic walls, and connection of the distal aorta to the perimeter of the new (bivalved) great artery. Eleven of the 16 patients survived operation: six of six who underwent a concurrent Fontan operation, four of six who underwent a concurrent right-ventricle-to-pulmonary-artery-conduit procedure, and one of four who underwent a concurrent systemic-pulmonary shunt procedure. Postoperative status in the 11 survivors is good: one patient has a gradient from the left ventricle to the aorta, 10 of 11 have little or no aortic regurgitation, and the remaining patients have a normal physiological status. We conclude that the Damus procedure is a useful addition to the surgical armamentarium for treatment of certain types of cyanotic congenital heart disease.
View details for PubMedID 3180404
Since September, 1985, 20 patients have undergone implantation of a homograft valve in the pulmonary position (16 pulmonary, 4 aortic). There were 11 primary operations and 9 reoperations. In 7 of 11 primary operations the homograft valve was utilized as a composite conduit with a short Dacron extension. In four of five reoperations for a failed porcine valved conduit, a composite homograft conduit was used. Four patients underwent implantation of a free homograft in a previously repaired right ventricular outflow tract (RVOT). Age ranged from 15 days to 22 years. There was one operative death (5%), a seven-week-old infant with truncus arteriosus. Long-term follow-up ranges from 1 to 30 months. Clinical performance has been satisfactory in 18 of 19 patients. One patient undergoing free implantation of a pulmonary valve in the RVOT required replacement at 18 months with a porcine valve. In this patient, pulmonary insufficiency was caused by distortion of the annulus secondary to dilatation and pulmonary hypertension. Nine of 18 survivors do not require medication. Eleven of 18 have trivial to mild pulmonary insufficiency murmurs without symptomatology. The homograft valve is extremely useful in reconstruction of the right heart, however, early insufficiency murmurs have been noted. Distortion of the valve annulus may contribute to the early onset of a benign insufficiency murmur. Residual distal obstruction or pulmonary hypertension may be a contraindication to the use of a free homograft in the orthotopic position.
View details for PubMedID 2980024
In three patients with complex cyanotic congenital heart disease, the conus was found to have no arterial outlet. The semilunar valves were at similar, caudad levels, and there was malposition of the great arteries. The aorta was related to the body--not the outflow portion--of the right ventricle in two patients with transposition and to the body of the left ventricle in one child with single ventricle. These cases confirm the independent embryological segregation of the conus and the truncus. Failure to recognize this condition can lead to erroneous relationship diagnoses and may adversely influence surgical decisions.
View details for PubMedID 3207481
Balloon dilatation (BD) of bioprosthetic valves was investigated in vivo and in vitro. Four children with stenotic bioprosthetic porcine valves in the pulmonary position underwent BD of the valve 10 to 24 months after its insertion. Average valve gradient was reduced from 47.5 to 27 mm Hg. Obstruction at the conduit-branch pulmonary artery connection became apparent after dilatation of the valve. These distal stenoses were also dilated. BD technique was tested in vitro with the use of nonstenotic valves in fresh conduits. No damage to the valve or to the conduit was found when oversized balloons were used in a standard fashion or intentionally inflated until rupture. It is concluded that conduit replacement may be deferred by balloon dilatation of obstructed biological valves and/or a stenotic conduit-pulmonary artery anastomosis.
View details for Web of Science ID A1987J078200013
View details for PubMedID 2954722
There is currently a renewed interest in the use of both fresh and commercially available frozen homograft valves for children and young adults. This has prompted us to review a series of 32 patients who received frozen homograft valves for aortic replacement between 1973 and 1975. The cryogenic technique evolved to include the use of selected antibiotics and equilibrated dimethyl sulfoxide solution to freeze homografts at a rate of 1 degrees C per minute to liquid nitrogen temperatures of -196 degrees C. Histologic sections of experimental frozen valves explanted 6 months postoperatively revealed the presence of viable donor cells, and tissue culture demonstrated the reproductive capacity of cusp fibroblasts. Of the 32 frozen viable homografts implanted in this series, 23 were inserted as free-sewn aortic replacements and nine were premounted on stents before implantation in the aortic position. There were two operative deaths, and three valves failed as a result of the technical problems of mounting in one patient and surgical insertion of a free graft in two patients. Of the 22 patients who remained at long-term risk, 13% with free-sewn grafts and 57% with premounted valves underwent reoperation for valve failure. After 10 years of follow-up, 15 (68%) of these patients, 12 (80%) with free-sewn and three (43%) with premounted valves, are alive with their original valve in place. Actuarial analysis shows that 58% of the 32 valves implanted are functional at the beginning of the eleventh year. There have been six late deaths resulting in an overall actuarial patient survival rate of 79% at 10 years and 69% after 13 years. These clinical results are believed to add support to our current application of the frozen homograft in selected patients.
View details for Web of Science ID A1987H627600003
View details for PubMedID 3573795
View details for PubMedID 2979921
In a patient with single ventricle, mitral atresia, common atrium, pulmonary stenosis, azygos continuation of the inferior vena cava and bilateral systemic artery--pulmonary artery anastomoses, the systemic veins were connected directly to the pulmonary arteries to achieve physiologic repair; the child was discharged five days after surgery and is asymptomatic three years postoperatively. This case illustrates the usefulness of azygos continuation of the inferior vena cava and emphasizes the need for low pulmonary artery pressure when a cavopulmonary anastomosis is done.
View details for Web of Science ID A1986D374500007
View details for PubMedID 3748840
Of 220 pediatric patients recovering from surgical repair of congenital heart disease, 147 (67%) met our criteria for early extubation and were extubated either in the operating room or within 6 h after admission to the surgical ICU. The duration of postoperative mechanical ventilation correlated with the duration of cardiopulmonary bypass during surgical repair. In patients undergoing repair of ventricular septal defect, preoperative measures of pulmonary vascular status (PPA/PAO and RP/RS) also correlated with the duration of mechanical ventilation.
View details for Web of Science ID A1985ARU6400010
View details for PubMedID 4028752
Two neonates with asplenia syndrome, subdiaphragmatic total anomalous pulmonary venous connection, and pulmonary stenosis underwent a palliative operation without cardiopulmonary bypass. With the use of a side-biting clamp, and anastomosis was created between the pulmonary venous confluence and the right atrium. (Since children with asplenia syndrome have common mixing lesions, pulmonary venous drainage to the right atrium is not physiologically detrimental.) The descending vertical vein was ligated. A systemic-pulmonary shunt was performed. Both infants were discharged from the hospital less than 1 week after the operation. Both infants subsequently died, at 4 months and at 16 months of age. At autopsy, pulmonary venous drainage was unobstructed, with a surgical orifice 86% to 90% of the aortic anulus. We conclude that, in infants with asplenia syndrome and obstructed total anomalous pulmonary venous drainage, relief of pulmonary venous obstruction can be accomplished without cardiopulmonary bypass.
View details for Web of Science ID A1984TM51600022
View details for PubMedID 6482494
A surgical approach is reported for a patient with pulmonary atresia, intact ventricular septum, and right ventricle to coronary artery communications through sinusoids. A shunt procedure was performed at two days of age; the right ventricular outflow tract was not opened. At subsequent catheterization, the tricuspid valve was temporarily closed with a balloon catheter and no change was seen in the ECG. At five months of age, the right ventricle was plicated and a patch was sewn over the tricuspid valve. One year after surgery, neither the right ventricular cavity nor the sinusoids could be demonstrated at angiocardiography; ECG changes of left ventricular ischemia have resolved, and the child is growing normally.
View details for Web of Science ID A1984ABC4300012
View details for PubMedID 6531265
From September, 1978, to January, 1983, 44 cyanotic infants and children underwent a systemic-pulmonary artery shunt for the treatment of reduced pulmonary blood flow. Age ranged from 18 hours to 4 years (mean age = 0.49 years). Weight ranged from 1.7 kg to 13.2 kg (mean weight = 4.9 kg). There were 12 classic Blalock-Taussig shunts, five central polytetrafluoroethylene shunts, six interposition modified Blalock-Taussig shunts, and 21 Great Ormond Street type of modified Blalock-Taussig shunts. No direct aorta-pulmonary artery anastomoses were performed. There was one postoperative death (1/44 = 2.3%) in a 1,700 gm neonate born with pulmonary atresia and intact ventricular septum (shunt patent). Four shunts required early revision: one thrombosed central shunt, a kinked patent interposition Blalock-Taussig shunt, a small but patent Blalock-Taussig shunt, and one excessive Great Ormond Street type of Blalock-Taussig shunt. Two late deaths were probably shunt-related: one Blalock-Taussig and one central. All four types of shunts provided good palliation, but the Great Ormond Street type of Blalock-Taussig shunt is our preferred shunt because of (1) low operative risk, (2) predictable patency (100% in our series), (3) lack of distortion of pulmonary arterial anatomy, and (4) technical ease of insertion as well as takedown.
View details for Web of Science ID A1984SZ65200009
View details for PubMedID 6204171
Eleven children are reported who had stenosis under a malposed aorta with gradients of 20 to 76 mm Hg between the right ventricle and aorta. The subaortic obstruction was caused by hypertrophy of the foreshortened infundibulum and malalignment of the infundibular septum relative to the remainder of the ventricular septum. Of these 11 patients, nine had a ventricular septal defect and seven had coarctation of the aorta. Rightward deviation of the infundibulum and aorta produced an unusually long left main coronary artery that was compressed by the stent of a bioprosthetic conduit valve in one patient. Serial cardiac catheterization studies in four patients showed progressive stenosis in each. Subaortic stenosis can develop in patients with malposition of the aorta and the frequency may be greater than 5% since milder forms are likely to occur. The obstruction can be progressive. The left coronary artery may be particularly vulnerable to compression after operative repair with an extracardiac conduit.
View details for Web of Science ID A1984TF81100003
View details for PubMedID 6540148
In the Congenital Heart Program at San Diego Children's Hospital, alterations in medical practice have reduced costs without impairing quality or access. Pediatric cardiac catheterization was done in 483 consecutive elective patients without overnight hospital stay. Hospital readmission was required in one patient for psoas tendinitis. Avoiding overnight hospital stay minimized attendant risks of hospital care, lessened psychosocial trauma and reduced the average hospital bill by $493 (29%). Hospital stay was also reduced for elective surgical correction of congenital heart disease on a case-by-case basis. Review of 151 consecutive cases (1978 through 1982) showed a decrease in both preoperative days in hospital and postoperative days in an intensive care unit. The duration of the postoperative stay was shortened from 6.8 days in 1978-1979 to 4.4 days in 1982 (P <.05). No increase in morbidity and no mortality resulted from the shortened perioperative hospital stay. Financial savings from this process averaged $991 per procedure.Diagnostic tests were reassessed and many precatheterization laboratory tests were eliminated. Without change in new patients seen or surgical volume, the use of cardiac catheterization decreased from 241 procedures in 1981 to 161 in 1982 and the number of operations without catheterization increased (11 to 22, 1981 to 1982). No increase in surgical morbidity or mortality was found comparing those diagnosed only by echocardiography with those who had preoperative cardiac catheterization. The decrease of 80 catheterizations in one year resulted in a savings of $188,800.True cost containment (reducing cost without reducing quality) can be accomplished in congenital heart programs. Similar cost containments in other disciplines may also be achieved.
View details for Web of Science ID A1984TB49900036
View details for PubMedID 6475038
Pulmonary vasoreactivity at sea level was studied in 22 children before and in 15 children after corrective cardiac surgery for congenital heart disease and pulmonary artery hypertension; 8 children were studied both before and after cardiac surgery. During cardiac catheterization in 28 children, pulmonary and systemic hemodynamics were determined in room air and during breathing of 15% oxygen, which corresponds to a maximal hypoxic level commonly encountered during airplane travel. Before surgery, 19 of 22 children tolerated 15% oxygen (O2), which caused the following hemodynamic changes from room air status: the ratio of pulmonary to systemic arterial pressure increased from 0.70 to 0.78 (p less than 0.05), the ratio of pulmonary to systemic flow decreased from 2.2 to 2.0 (p greater than 0.05) and the ratio of pulmonary to systemic vascular resistance increased from 0.33 to 0.40 (p less than 0.02). In two children, severe pulmonary vasoconstriction developed within 5 minutes of 15% oxygen administration, requiring immediate discontinuation of hypoxia; neither patient had lasting deleterious effects. There was no evidence of increased pulmonary vasoreactivity in children with Down's syndrome compared with genetically normal children. After corrective surgery in 15 children (including both of the hyperreactors), no significant pulmonary vascular response to 15% oxygen was found. It is concluded that, in a small number of children with unrepaired congenital heart disease and pulmonary artery hypertension, pulmonary vascular hyperreactivity can be induced by breathing 15% oxygen; this reaction is life-threatening but reversible with the administration of 100% oxygen.(ABSTRACT TRUNCATED AT 250 WORDS)
View details for Web of Science ID A1983RS14300019
View details for PubMedID 6226728
The acute and chronic consequences of subclavian artery transection were analyzed in the noninvasive vascular laboratory. Twenty-eight patients (aged 1 day to 4 2/12 years, median 2 months) underwent subclavian artery transection (23 Blalock-Taussig, four subclavian aortoplasty for coarctation or interrupted arch, and one division of aberrant left subclavian). Bilateral systolic brachial artery pressure (BAP) was measured by Doppler instrumentation to obtain a "BAP index": BAP1 = (operated side BAP/control side BAP). Velocity waveform tracings and bilateral forearm skin temperatures were also obtained during studies before and sequentially after operation (4 hours to 12 years). Five patients underwent exercise testing of the upper extremity. Nine patients were studied for manual preference and limb development. Before operation, mean BAP1 was 0.99. Immediately (4 to 48 hours) after operation, mean BAP1 was 0.39. Three weeks postoperatively, BAP1 was 0.62, and thereafter it remained at 0.70. All differences between preoperative, immediate postoperative and late postoperative BAP1 are significant (p less than 0.001). Exercise resulted in a significant (p less than 0.01) increase in BAP bilaterally. Forearm skin temperature was initially lower (p less than 0.01) on the operated side but approximated the control side by 1 week. Limb girth was less on the operated side (p less than 0.01), without evidence of altered manual preference. In conclusion, subclavian artery transection causes permanent reduction in BAP1. The affected limb appears to respond to increased metabolic demand by increasing limb blood flow.
View details for Web of Science ID A1983QZ53900003
View details for PubMedID 6865463
From 1975 to 1982, 31 infants were operated upon in the first year of life for aortic coarctation and congestive heart failure. Operations performed were resection and end-to-end anastomosis (RETE) in 14, subclavian flap aortoplasty (SFA) in six, patch aortoplasty (PA) in five, and other procedures in six. Thirty of the thirty-one (97%) survived the operation. To assess the effect of operation, 26 infants were studied noninvasively with Doppler arm-to-leg pressure measurements at rest and with stress. Preoperatively, the median arm-to-leg gradient at rest was 77 mm Hg. Serial postoperative Doppler studies demonstrated progressive changes in arm-to-leg pressure gradients: 69% had residual arm-to-leg gradients that spontaneously resolved, 13% had residual gradients that persisted, 13% had progressive increase in gradient, and one child had neither early nor late gradient. Stress testing often unmasked gradients not present in the resting state. No differences were noted among the three surgical groups: RETE, SFA, and PA. From our experience, we have made four conclusions with regard to repair of coarctation of the aorta in infants. First, surgical survival is expected. Second, the effect of the operation is dynamic, with four patterns defined: (1) complete relief of coarctation, (2) transient residual coarctation, (3) persistent residual coarctation, and (4) recurrent coarctation. Third, optimal surgical therapy seems to be an eclectic approach. Fourth, physiological evaluation of coarctation in infants can be obtained by Doppler techniques in conjunction with stress testing.
View details for Web of Science ID A1983QZ53900002
View details for PubMedID 6865469
Recent technologic advances make it possible to measure reliably serum or plasma ionized calcium with a high degree of accuracy. In this study, with the use of 16 mongrel dogs, the quantitation of the hemodynamic response to acute changes in ionized calcium was attempted. It was found that normal cardiac function required a normal level of ionized calcium. In intact animals, severe depression of myocardium is masked by pressor reflexes designed to maintain homeostasis. In their absence, myocardial depression is readily apparent. Animals that are hypercalcemic at the beginning of infusion respond in essentially the same manner. To a degree previously unsuspected, myocardial function depends upon a constant level of ionized calcium.
View details for PubMedID 15216269
View details for PubMedID 456020
We have encountered two cases of late calcification of the porcine heterograft. A patient in chronic renal failure died of sepsis and endocarditis fifteen months after replacement of the mitral and tricuspid valves. At postmortem examination, both heterograft valves exhibited severe calcification and thrombosis. A second patient with rheumatic heart disease and sickle cell disease underwent mitral valve replacement for severe regurgitation. Thirty months later, cardiac catheterization revealed prosthetic valve stenosis. The valve was replaced successfully, and the excised heterograft exhibited severe calcification with restriction of leaflet motion. Although calcification of the porcine heterograft is known to occur in patients with infection or disorders of calcium metabolism, dysfunction of the heterograft is rare in our experience.
View details for Web of Science ID A1979HE45300007
View details for PubMedID 454040
Three consecutive patients with Pseudomonas endocarditis were treated by early operation with no deaths. The indications for operation were severe failure, systemic embolization, and infection refractory to antibiotics. The organism is aggressive, characterized by early invasion of the myocardium. Wide débridement of the anulus is necessary to remove any vegetations or intramyocardial abscessed wall. Because multiple valve infection is common, it is important to evaluate all four cardiac valves at the time of operation. Removing a second rim of the mitral anulus for separate culture at the time of mitral valve replacement may demonstrate the degree to which the valve resection has removed all infection. Early operation is necessary for reinfection of the prosthetic valve; however, metastatic abscess should also be considered in the face of continued signs of infection postoperatively. Patients should receive a 6 week postoperative course of antibiotics which have been shown by in vitro testing to be serum bactericidal in at least a 1:8 dilution. The operative findings of invasion of the myocardium by the organism and the surgical success in this small series have resulted in our recommending earlier operation in patients with these indications.
View details for Web of Science ID A1979GR82600015
View details for PubMedID 423591
Clinical and angiographic or autopsy data, or both, on three children with a subdivided left atrium (cor triatriatum) and an associated endocardial cushion defect are reviewed. (One child had ostium primum defect, and two had complete atrioventricular [A-V] canal.) A fourth patient demonstrates the difficulties in differentiating subdivided left atrium from supravalve mitral stenosis in the presence of an endocardial cushion defect. The clinical findings are greatly influenced by the endocardial cushion defect. A pressure gradient between the pulmonary wedge and (left or right) ventricular end-diastolic pressures in patients with an endocardial cushion defect indicates pulmonary venous obstruction and should alert one to the possibility of these combined lesions. The exact diagnosis is made with injections of angiographic contrast medium into the proximal and distal left atrial chambers, to documented the respective relations of the pulmonary veins, left atrial appendage and A-V valves to these atrial chambers. All three patients with an endocardial cushion defect and a subdivided left atrium had an associated patent ductus arteriosus. The common association of subdivided left atrium with intracardiac, pulmonary venous and aortic anomalies is again demonstrated.
View details for Web of Science ID A1979HX54100018
View details for PubMedID 506937
One hundred consecutive patients undergoing aorta-coronary bypass grafting (ACBG) alone, without ventricular venting, were prospectively studied to determine the incidence and consequence of perioperative myocardial infarction (PMI) and the clinical variables that were predictive of PMI. Incidence was determined by serial electrocardiography (ECG) 100 patients; serum CK, GOT, and LDH (100 patients). CK isoenzymes (qualitative 100 patients, quantitated 50 patients); vectorcardiography (VCG) (78 patients); and 99mtechnetium pyrophosphate scintigraphy (TcPyp) (52 patients). The incidence of PMI by ECG was 9%; an additional 8% of cases was diagnosed by enzymes alone. The incidence of diagnostic change by VCG was 19% and by scintigraphy, 25%. Using at least one changed variable of the remaining three as the reference standard, the relative sensitivity and relative specificity of given variables in the diagnosis of PMI were as follows: ECG 67% and 100%, respectively; VCG 85% and 94%; scintigraphy 92% and 97%; and serum enzymes 86% and 96%. By univariate analysis, unstable angina was the only significant predictor of PMI. The operative mortality rate was 2% and the mortality rate at 12 months was 5%. There was a significantly greater mortality rate in patients with PMI diagnosed by ECG (p less than 0.01), in patients with unstable angina pectoris before operation (p less than 0.05), and in women (p less than 0.05).
View details for Web of Science ID A1979HG27100015
View details for PubMedID 313488
Mongrel dogs were subjected to hypothermic (28 degrees to 30 degrees C) cardiopulmonary bypass with hemodilution by 50%. In two groups of 8 dogs each, ventricular fibrillation was induced for 60 and 90 minutes, respectively, while the dogs were on bypass. A group of 6 dogs with the heart beating but nonworking served as control. Seven weeks after operation, hemodynamic measurements were made in the survivors (6 in each group) and the heart was fixed by perfusion with glutaraldehyde. Multiple transmural samples were taken from both ventricles. Light microscopy revealed solitary left ventricular scars (0.5 to 3 mm wide) in 2 hearts each from Groups 2 and 3. None of the hearts exhibited diffuse subendocardial fibrosis indicative of healed ischemic injury. All animals were hemodynamically normal. We conclude that in the nonhypertrophied heart, ventricular fibrillation up to 90 minutes with continuous bypass-sustained coronary perfusion (perfusion pressure at or above 70 mm Hg) offers protection from permanent myocardial injury.
View details for Web of Science ID A1979GM51000006
View details for PubMedID 453985
The expanded microporous polytetrafluoroethylene (PTFE) 4 mm vascular prosthesis has been used to create a central aortopulmonary shunt in 20 critically ill infants less than 3 weeks old. The infants ranged from 1 to 18 days old (5.25 days), and from 1.5 to 4.0 kg (2.9 kg). Conduit length ranged from 2 to 6 cm (4 cm). Sixteen patients had atresia of the tricuspid or pulmonary valve. There were 6 early deaths (30%), only 1 of which was shunt related. The mean preoperative arterial oxygen saturation was 62% (range, 33 to 80%), and mean postoperative saturation was 87% (range, 78 to 90%). There were 5 late deaths, 1 probably caused by shunt failure. Nine long-term survivors have done well. Follow-up ranges from 1 to 36 months (18 months). Factors influencing conduit function are length, technical considerations, and pulmonary vascular resistance. Late restudy in 5 of 9 survivors confirms patency and demonstrates bidirectional pulmonary blood flow. Since PTFE shunt flow capability is fixed, the infant may require repair or a second shunt within 24 months of the initial procedure.
View details for Web of Science ID A1979HY53000008
View details for PubMedID 518185
Although clinical and hemodynamic stability predicted outcome very well when left ventricular aneurysm was electively resected in 25 patients (95% survival), more discriminate criteria were essential for 20 patients undergoing urgent operation for severe myocardial decompensation (50% survival). Three methods of ventriculographic analysis primarily sensitive to the function of the non-aneurysmal left ventricle were evaluated. These methods separated patients undergoing urgent operation into a population with high operative risk (less than 18% survival) and a population with low operative risk (greater than 82% survival). These criteria also separated 15 patients undergoing operation within three months of myocardial infarction into a group with excellent prognosis (greater than 85% survival) and a group with poor prognosis (less than 15% survival). The high operative risk in patients undergoing urgent operation or operation within three months of myocardial infarction, when non-aneurysmal ventricular function is poor, may be too high; it should be undertaken only under unusual circumstances.
View details for Web of Science ID A1978GA34300024
View details for PubMedID 709773
During a six-year period, 46 severely symptomatic infants (average age, 5.1 months) underwent correction of ventricular septal defect (22 patients), total anomalous pulmonary venous connection (13 patients), and complete atrioventricular canal (11 patients), with the use of surface cooling to 20 degrees C. Cardiac repair was performed during circulatory arrest, and rewarming was performed with a pump oxygenator. Ten patients undergoing repair of ventricular septal defects were studied hemodynamically at 21 degrees C, before repair and at 37 degrees C after rewarming. Heart rate, left ventricular systolic pressure, maximum dp/dt, cardiac index, stroke work, and oxygen consumption were reduced substantially at 21 degrees C. Systemic vascualr resistance was increased at 21 degrees C. All changes were reversible with repair and rewarming. A protocol for hemodilution and crystalloid volume loading was devised to maintain urine output after early patients were noted to demonstrate renal dysfunction. With this protocol, survival rates were 89% for patients with ventricular septal defects, 67% for those with atrioventricular canal defects, and 85% for those with total anomalous pulmonary-venous connection.
View details for Web of Science ID A1978FF87700007
View details for PubMedID 678092
We reviewed current concepts and techniques of cardiac assistance, including orthotopic and heterotopic cardiac allografting. Whereas intraaortic balloon counterpulsation has had the greatest clinical use, other techniques are now becoming available, including the implantable abdominal left ventricular assistance device, and much effort is being expended on the development of artificial hearts and their power sources. Despite the present technical shortcomings, exciting advances have occurred in the clinical management of intractable acute or chronic ventricular failure and cardiogenic shock since the introduction of circulatory assistance in man. The continued development and implementation of these techniques will help reduce the still unacceptably high mortality rate in this group of patients.
View details for Web of Science ID A1978EU98300041
View details for PubMedID 348047
The thermodilution (TD) technique can be considered as the most useful method for cardiac output (CO) determinations in clinical routine. However, surgical procedures for pulmonary stenosis or atresia frequently induce pulmonary insufficiency (PI) of various degree; due to abnormal flow conditions, this may interfere with the validity of transvenous CO-determinations. This experimental study shows that the TD technique is accurate even in the presence of PI. This observation may be explained by the fact that PI results, on the one hand, in a higher distribution volume and, on the other hand, in an increased transit time of the indicator; both of these factors have opposite effects on CO calculations, thus possibly leading to a balance of either error.
View details for PubMedID 300189
A patient is reported with hypertrophic cardiomyopathy where midventricular obstruction was found in association with mitral stenosis. Partial relief of the intraventricular obstruction was obtained by extensive papillary and trabecular muscle resection in the mid-left ventricular area, in conjunction with mitral valve replacement with a porcine heterograft.
View details for Web of Science ID A1977EA30500006
View details for PubMedID 144020
A 24-year-old man presented with late stenosis of a cloth-covered Starr-Edwards valve (model 2320). Fibrous ingrowth occurred at the inflow orifice of the valve and all three struts were adherent to the aortic wall, creating a "tunnel" form of obstruction. The stenotic valve was replaced with a porcine heterograft, and the small aortic root was enlarged with a Dacron patch. This form of late prosthetic valve stenosis should have been prevented by enlargement of the aortic root at the time of valve insertion and by long-term anticoagulant therapy.
View details for PubMedID 830508
Twenty-eight dogs were subjected to 90 minutes of hypothermic (30 degrees C) cardiopulmonary bypass with moderate hemodilution. In 6 dogs the heart was vented and beating for 60 minutes. Eight dogs underwent ventricular fibrillation with coronary perfusion (VF + CP). In 14 dogs the aorta was cross-clamped for 60 minutes while the myocardium was protected by local cardiac hypothermia (ICA + LCH). Eighteen animals survived. Hemodynamic studies at seven weeks revealed no major differences among the three groups. At postmortem examination, no gross scarring was noted in any heart. Microscopical examination of 14 hearts was completely-normal. In the VF + CP group, 2 hearts had isolated microscopical scars. Similar linear subendocardial scars (less than or equal to 1.5 X 0.5 mm) were noted in 2 hearts subjected to ICA + LCH. Survival after 60 minutes of VF + CP or ICA + LCH did not result in long-term morphological injury to or functional impairment of the myocardium.
View details for Web of Science ID A1977DX43500004
View details for PubMedID 907400
The porcine heterograft has been shown to be a very satisfactory replacement for the diseased aortic valve. In certain cases, the advantage of a low thromboembolism rate without anticoagulation must be weighed against the suboptimal orifice size noted in the smallest heterografts. In using the largest replacement possible, the surgeon may encounter difficulty with closure of the aortotomy. Patch aortoplasty, as used in the 4 cases reported here, allows implantation of a larger heterograft. The technique is simple and adds little time to the operation. In selected cases, enlargement of the aortic root should result in better long-term hemodynamic performance.
View details for Web of Science ID A1976BX27300015
View details for PubMedID 132580
A 5-year-old child with asplenia, situs inversus, single ventricle, common atrium, severe subvalvular pulmonary stenosis, 1-transposition of the great arteries, and absent inferior vena cava presented with severe limitation (resting arterial saturation 74 per cent). At operation, the systemic venous atrium was partitioned with a Dacron baffle, so that hepatic venous and coronary sinus blood was enabled to drain with the pulmonary venous blood into the single ventricle and aorta. The superior portion of this atrium was anastomosed to the divided main pulmonary artery, so that most of the systemic venous blood was allowed to flow directly to the lungs. Intraoperative hemodynamic studies revealed a pulmonary artery pressure of 12/9 mm. Hg and a superior vena caval flow that was 88 per cent of the ascending aortic blood flow. Follow-up catheterization studies revealed an intact partition, no anastomotic gradient, superior vena cava pressure of 20 mm. Hg, arterial saturation of 84 per cent, and excellent flow of contrast from superior vena cava to atrium to pulmonary artery. Exercise tolerance was markedly improved, and chronic fluid retention was not observed. This operation offers a new alternative for long-term palliation of complex lesions amenable to exclusion of the right ventricle, such as single or common ventricle with unreconstructable anomalies of the atrioventricular valves.
View details for Web of Science ID A1976BK05500011
View details for PubMedID 55525
Surgery has become an accepted method of treatment for coronary artery disease and its complications. Revascularization results in significant improvement in symptoms for patients with angina pectoris. Occasionally, patients requiring surgery for angina pectoris will sustain reversible ischemic damage during operation; such patients can be successfully weaned from cardiopulmonary bypass with full recovery when intra-aortic balloon counterpulsation is used. Arrhythmias associated with ischemic damage to the myocardium also can be controlled when IABCP is used for physiologic assistance. Patients in cardiogenicshock of pulmonary edema after acute myocardial infarction have an ominous prognosis. When decompensation occurs, IABCP may be used to stabilize the patient and to allow study and corrective surgery if possible. The prognosis is better for patients with ventricular septal defect, although selected patients without a mechanical defect of the myocardium can be salvaged if the response to IABCP is favorable. Counterpulsation has also been shown to be useful in achieving pulsatile cardiopulmonary bypass and in assisting high-risk patients through operation. External pressure circulatory assist (EPCA) is less effective than IABCP in assisting the failing myocardium; however, the external device is noninvasive and may be a useful adjunct in situations where IABCP is not feasible.
View details for Web of Science ID A1976BE14100010
View details for PubMedID 1251307
A new arterial prosthesis made of polytetrafluoroethylene (OTFE) was evaluated in 10 infants with complex cyanotic congenital heart disease. All grafts used were 4 mm. in diameter and varied in length from 0.8 to 6 cm. The grafts were anastomosed to the main pulmonary artery or its bifurcation in 8 infants and to the right and left pulmonary arteries in one each. There were two early deaths, one of which was related to shunt failure. The advantages of a shunt to the main pulmonary artery are obvious, and the intraoperative procedure is facilitated with the prosthesis. Follow-up averages 9 months in the 8 survivors, and the patients have nearly doubled their preoperative weight. A shunt murmur is present in each case and the children have mild-to-moderate cyanosis at rest. Repeat aortograms in 2 patients, 8 and 10 months postoperatively, show a smooth graft without luminal narrowing. The aortic oxygen saturations were 73 per cent. The PTFE graft and/or anastomoses will not grow with the growth of the child and therefore may not accommodate growth by increased flow. This may prove to be a limiting factor in its long-term use in fants. We reserve the use of these grafts for infants with complex cyanotic defects undergoing emergency surgery or in older children in whom a conventional shunt is not possible or a previous shunt has failed.
View details for Web of Science ID A1976CE29400004
View details for PubMedID 957753
The coexistence of organic cardiac disease and chronic renal failure presents a therapeutic dilemma. Cardiac operations have been performed on patients who were undergoing hemodialysis or who had had successful renal transplantation. There are several aspects of management of these patients which differ from those of the routine cardiac surgical patient. Guidelines for management are outlined and discussed.
View details for Web of Science ID A1975V733500003
View details for PubMedID 1090271
Although the internal mammary artery bypass seems an ideal coronary bypass vessel, it has not usually been possible to use this vessel to bypass distal lesions in the right coronary circulation. In this experiment, the right internal mammary artery was utilized in retrograde fashion to evaluate this anatomically more suitable vessel as a bypass graft to the occluded right coronary artery in the dog. Reversal of acute myocardial ischemia was demonstrated by mapping epicardial ST-segment elevation and measuring surface pH. Preliminary long-term experiments have also demonstrated patency and perfusion of the distal right coronary artery by this method.
View details for Web of Science ID A1975AJ14000003
View details for PubMedID 239291
Eighty-eight operations for correction of intracardiac congenital heart defects were performed using local cardiac hypothermia for protection of the ischemic myocardium. Twenty-six patients underwent repair of tetralogy of Fallot, 23 had patch closure of ventricular septal defect, 24 had correction of various types of congenital aortic stenosis, and 15 were operated upon for other complex lesions. The overall operative mortality was 5.6%. Ischemia times ranged from 9 to 119 minutes (mean, 48 minutes). Ischemic arrest protected by local cardiac hypothermia provides an optimal operative field, permitting repair of uncomplicated intracardiac defects in a precise, unhurried manner. No hemodynamic abnormalities attributable to the technique were encountered.
View details for Web of Science ID A1975AU69700008
View details for PubMedID 1180598
Sternal closure after median sternotomy in the pediatric age group may be accomplished with non-resorbable sutures or stainless steel wire. The former has the disadvantages of chronic sinus formation and the latter may either break or cause pressure on the skin. The Pill-Wolvek Suture Lock Plate was used as an alternative method in 26 patients aged from seven days to six years. It has the advantage of greater strength and avoids the problem of prominent twisted wire ends. Healing has been excellent and there have been no complications attributed to the wires.
View details for PubMedID 15215921
View details for PubMedID 5506640
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