Unilateral branch pulmonary artery origin from a solitary arterial trunk with major aortopulmonary collaterals to the contralateral lung: anatomic and developmental considerations.
Seminars in thoracic and cardiovascular surgery
In both truncus arteriosus communis (TAC) and tetralogy of Fallot (TOF), there is a rare phenotype that includes a single branch pulmonary artery (PA) arising from a solitary great artery and major aortopulmonary collaterals (MAPCAs) supplying the contralateral lung. We describe the intracardiac and great vessel anatomy of infants with this phenotype, consider rationale for classifying patients as TOF vs. TAC, and describe surgical outcomes. Our institution's surgical database was reviewed for patients with a single branch PA from a solitary arterial trunk and contralateral MAPCAs from 2007 to 2019. Demographic, imaging, and surgical data were collected and described. All 11 patients underwent complete repair with a median right ventricular to aortic pressure ratio of 0.36 (range 0.26-0.50). At 0.1-9.1 years after repair (median 0.8 years) there was approximately balanced left-right lung perfusion (median 52% to the right lung, range 34-74%). The MAPCA lungs exemplified the full spectrum of PA and MAPCA anatomy, from absent intrapericardial PAs with all single supply MAPCAs to a normally arborizing PA with all dual supply MAPCAs and present pulmonary valve leaflet tissue. All patients had a systemic semilunar valve with 3 thin and similarly sized leaflets and fibrous continuity with the tricuspid valve, and all had coronary origins and outflow tract morphology more consistent with TOF. It is appropriate to classify all patients with a single anomalous PA from a solitary arterial trunk and MAPCAs to the contralateral lung as TOF rather than TAC Type A3. All variants were amenable to surgical repair.
View details for DOI 10.1053/j.semtcvs.2020.11.009
View details for PubMedID 33181302
Angiographic Anatomy of Major Aortopulmonary Collateral Arteries and Association With Early Surgical Outcomes in Tetralogy of Fallot.
Journal of the American Heart Association
Background Due in part to the heterogeneity of the pulmonary circulation in patients with tetralogy of Fallot and major aortopulmonary collateral arteries (MAPCAs), research on this condition has focused on relatively basic anatomic characteristics. We aimed to detail pulmonary artery (PA) and MAPCA anatomy in a large group of infants, assess relationships between anatomy and early surgical outcomes, and consider systems for classifying MAPCAs. Methods and Results All infants ( <1 year of age) undergoing first cardiac surgery for tetralogy of Fallot/MAPCAs from 2001 to 2019 at Stanford University were identified. Preoperative angiograms delineating supply to all 18 pulmonary segments were reviewed for details of each MAPCA and the arborization and size of central PAs. We studied 276 patients with 1068 MAPCAs and the following PA patterns: 152 (55%) incompletely arborizing PAs, 48 (17%) normally arborizing PAs, 45 (16%) absent PAs, and 31 (11%) unilateral MAPCAs. There was extensive anatomic variability, but no difference in early outcomes according to PA arborization or the predominance of PAs or MAPCAs. Patients with low total MAPCA and/or PA cross-sectional area were less likely to undergo complete repair. Conclusions MAPCA anatomy is highly variable and essentially unique for each patient. Though each pulmonary segment can be supplied by a MAPCA, central PA, or both, all anatomic combinations are similarly conducive to a good repair. Total cross-sectional area of central PA and MAPCA material is an important driver of outcome. We elucidate a number of novel associations between anatomic features, but the extreme variability of the pulmonary circulation makes a granular tetralogy of Fallot/MAPCA classification system unrealistic.
View details for DOI 10.1161/JAHA.120.017981
View details for PubMedID 33283588
Postoperative Recovery of Left Ventricular Function following Repair of Large Ventricular Septal Defects in Infants.
Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography
INTRODUCTION: Early postoperative left ventricular (LV) dysfunction is observed following repair of large ventricular septal defects (VSDs), but the frequency and rate of recovery of LV function are unknown. This study aims to characterize the incidence and rate of recovery of postoperative LV dysfunction following repair of large VSDs and to improve understanding of LV mechanics before and after VSD repair.METHODS: Infants who underwent surgical repair of an isolated large VSD were included. Pre- and postoperative echocardiographic images were reviewed, and LV function was assessed by both conventional echocardiography and speckle-tracking strain analyses. Postoperative LV dysfunction was defined as an LV ejection fraction (LVEF)<50% using the 5/6 area-length method. Echocardiograms were reviewed to assess LVEF and LV volume through 1-year follow-up.RESULTS: Of 104 infants evaluated (median age, 0.31 [0.22, 0.56] years), all had normal preoperative LVEF and 39 (38%) had postoperative LV dysfunction. Follow-up echocardiograms were available in 31 (80%) patients, all of whom had LVEF>50% within 9months of surgery. Lower preoperative apical four-chamber longitudinal strain (A4LS) and greater LV end-diastolic volume indexed to body surface area1.38 were independently associated with postoperative LV dysfunction. An absolute preoperative A4LS < 16.7% (area under the curve=0.87; 95% CI, 0.78-0.95; P<.001) was most strongly associated with postoperative dysfunction.CONCLUSIONS: Infants with LV dysfunction following repair of large VSDs recover function within 9months. Preoperative A4LS can be helpful to detect subclinical LV dysfunction in the setting of a large hemodynamically significant VSD and guide postoperative expectations for providers and families.
View details for DOI 10.1016/j.echo.2019.10.003
View details for PubMedID 31866322
Secondary repair of incompetent pulmonary valves after previous surgery or intervention: Patient selection and outcomes.
The Journal of thoracic and cardiovascular surgery
OBJECTIVES: Pulmonary valve (PV) regurgitation (PR) is common after intervention for a hypoplastic right ventricular outflow tract. Secondary PV repair is an alternative to replacement (PVR), but selection criteria are not established. We sought to elucidate preoperative variables associated with successful PV repair and to compare outcomes between repair and PVR.METHODS: Patients who underwent surgery for secondary PR from 2010 to 2017 by a single surgeon were studied. The PV annulus and leaflets were measured on the preoperative echocardiogram and magnetic resonance images, and the primary predictor variable was leaflet area indexed to ideal PV annulus area (iPLA) by magnetic resonance imaging. PV repair and PVR groups were compared using multivariable logistic regression, and with a conditional inference tree. Freedom from PV dysfunction and from reintervention were assessed with Kaplan-Meier survival analyses.RESULTS: Of 85 patients, 31 (36%) underwent PV repair. By multivariable analysis, longer PV total leaflet length (cm/m2) (beta=3.00, standard error [SE]=0.82, P<.001), larger PV z score (beta=1.34, SE=0.39, P=.001), and larger iPLA (beta=8.13, SE=2.62, P=.002) were associated with repair. iPLA of 0.90 or greater was 91% sensitive and 83% specific for achieving PV repair. At a median of 4.1years follow-up, there was greater freedom from significant PR in the PV repair group (log rank P=.008).CONCLUSIONS: Patients with an iPLA >0.9, and those with an iPLA between 0.7 and 0.9 with a PV annulus z score >0 should be considered for a native PV repair. At midterm follow-up, patients with a PV repair were not more likely to developPR or to require reintervention when compared with patients undergoing PVR.
View details for DOI 10.1016/j.jtcvs.2019.06.110
View details for PubMedID 31585750
- Transcatheter redirection of hepatic venous blood to treat unilateral pulmonary arteriovenous malformations in a Fontan circulation by short-term total exclusion of the unaffected lung CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS 2019; 93 (4): 660?63
Subcutaneous and Intravenous Treprostinil Pharmacokinetics in Children With Pulmonary Vascular Disease.
Journal of cardiovascular pharmacology
2019; 73 (6): 383?93
This study evaluated the pharmacokinetics of intravenous (IV) and subcutaneous (SC) treprostinil in pediatric patients with pulmonary vascular disease, and compared them with existing adult data from a similar cohort. Blood samples were collected from pediatric patients receiving steady-state IV or SC treprostinil and were assessed for plasma treprostinil concentration using liquid chromatography and tandem mass spectrometry. Forty participants, 15 receiving IV and 25 receiving SC treprostinil, were included in the analysis. Age ranged from 0.1 to 15.6 years. The median dose of treprostinil was 45.5 ng·kg·min with a range of 8-146 ng·kg·min. There was a linear relationship between treprostinil dose and plasma concentration with an R of 0.57. On average, there were higher blood concentrations per given dose of IV treprostinil compared with those per given dose of SC, but the difference was not significant. Compared with adult data, the slope of the pediatric data was similar, but the y-intercept was significantly lower. Additionally, the concentration per dose ratio was significantly higher in adults compared with children. Pediatric patients have significantly lower average blood concentrations of treprostinil per given dose compared with adults, and higher, but not significantly so, blood concentrations when treprostinil is administered IV as compared with SC administration.
View details for DOI 10.1097/FJC.0000000000000674
View details for PubMedID 31162247
Pulmonary hemorrhage in children with Alagille syndrome undergoing cardiac catheterization.
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions
To evaluate the incidence, severity, and outcomes of pulmonary hemorrhage in children with Alagille syndrome (AGS) undergoing cardiac catheterization, and to find variables associated with hemorrhage in this population.Children with AGS have a high incidence of bleeding complications during invasive procedures. It has been our impression that catheterization-associated pulmonary hemorrhage is more common in children with AGS, but there are no published data on this topic.This was a retrospective single institution study of children with AGS undergoing catheterization from 2010 to 2018. Pulmonary hemorrhage was defined as angiographic or fluoroscopic evidence of extravasated blood in the lung parenchyma, or blood suctioned from the endotracheal tube with documentation of pulmonary hemorrhage by the anesthesiologist or intensivist. Univariate comparisons were made between catheterizations that did and did not have pulmonary hemorrhage.Thirty children with AGS underwent 87 catheterizations, 32 (37%) with interventions on the branch pulmonary arteries (PA). There were 26 (30%) procedures with hemorrhage, the majority (65%) of which were self-limited or required less than 24?hr of mechanical ventilation. Moderate and severe hemorrhage occurred only in children with tetralogy of Fallot (TOF; 5 of 14, 36%). A higher right ventricle to aorta systolic pressure ratio (1.0 [0.85-1.1] vs. 0.88 [0.59-1.0], p = .029) and interventions on the branch PAs (14 of 26, 54% vs. 18 of 61, 30%, p = .032) were associated with hemorrhage.Pulmonary hemorrhage was common in children with AGS undergoing both intervention and diagnostic cardiac catheterization, and was associated with TOF, higher RV to aorta pressure ratio, and interventions on the branch PAs.
View details for DOI 10.1002/ccd.28508
View details for PubMedID 31584246
Texting preferences in a Paediatric residency
2017; 14 (6): 401?6
Text messaging is ubiquitous among residents, but remains an underused educational tool. Though feasibility has been demonstrated, evidence of its ability to improve standardised test scores and provide insight on resident texting preferences is lacking. The authors set out to evaluate: (1) satisfaction with a hybrid question-and-answer (Q&A) texting format; and (2) pre-/post-paediatric in-training exam (ITE) performance.A prospective study with paediatrics and internal medicine-paediatrics residents. Residents were divided into subgroups: adolescent medicine (AM) and developmental medicine (DM). Messages were derived from ITE questions and sent Monday-Friday with a 20 per cent variance in messages specific to the sub-group. Residents completed surveys gauging perceptions of the programme, and pre- and post-programme ITE scores were analysed.Forty-one residents enrolled and 32 (78%) completed a post-programme survey. Of those, 21 (66%) preferred a Q&A format with an immediate text response versus information-only texts. The percentage change in ITE scores between 2013 and 2014 was significant. Comparing subgroups, there was no significant difference between the percentage change in ITE scores. Neither group performed significantly better on either the adolescent or developmental sections of the ITE. Text messaging? remains an underused educational tool CONCLUSIONS: Overall, participants improved their ITE scores, but no improvement was seen in the targeted subgroups on the exam. Although Q&A texts are preferred by residents, further assessment is required to assess the effect on educational outcomes.
View details for DOI 10.1111/tct.12590
View details for Web of Science ID 000414616600005
View details for PubMedID 28078778
Coarctation Index Predicts Recurrent Aortic Arch Obstruction Following Surgical Repair of Coarctation of the Aorta in Infants.
2017; 38 (6): 1241?46
Recurrent aortic arch obstruction (RAAO) remains a major cause of morbidity following surgical neonatal repair of coarctation of the aorta (CoA). Elucidating predictors of RAAO can identify high-risk patients and guide postoperative management. The Coarctation index (CoA-I), defined as the ratio of the diameter of the narrowest aortic arch segment to the diameter of the descending aorta, has been used to help diagnose RAAO in neonates following the Norwood Procedure. We sought to assess the predictive value of the CoA-I on RAAO after CoA repair in infants with biventricular circulation. Clinical, surgical, and echocardiographic data of infants with biventricular circulation following neonatal CoA repair between 2010 and 2014 were evaluated. RAAO was defined using a composite quantitative outcome variable: a blood pressure gradient >20, a peak aortic arch velocity >3.5 m/s by echocardiogram, or a catheter-measured peak-to-peak gradient >20 within 2 years of surgery. Univariate and multivariate logistic regression analyses were used. Of the 68 subjects included in the analysis, 15 (22%) met criteria for RAAO. In the multivariate model, only CoA-I (OR 35.89, 95% CI 6.08-211.7, p < 0.0001) and use of patch material (OR 9.26, 95% CI 1.57-54.66, p = 0.014) were associated with increased risk of RAAO. The odds of developing RAAO was higher in patients with a CoA-I less than 0.7 (OR 33.8, 95% CI 5.7-199.5, p < 0.001). Postoperative CoA-I may be used to predict RAAO in patients with biventricular circulation after repair of CoA. Patients with a CoA-I less than 0.7 or patch aortoplasty warrant close follow-up.
View details for DOI 10.1007/s00246-017-1651-4
View details for PubMedID 28608147
Text4Peds: Feasibility of an Educational Text-Messaging Program for Pediatrics Residents.
Journal of graduate medical education
2014; 6 (4): 746-749
There is an ongoing effort to maximize educational material provided to residents who are in a time-constrained work environment. Mobile technology, principally smartphone applications and online modules, has shown educational promise.We developed a text-messaging program, Text4Peds, to assist residents with preparation for their pediatric board examinations. Goals were to assess (1) the feasibility of texting educational messages to residents, and (2) resident satisfaction and perceived usefulness of a texting program.We conducted a prospective study of pediatrics and combined internal medicine-pediatrics residents. Messages derived from the most missed pediatric in-training examination questions were sent daily to residents. After 3 months, residents completed surveys that gauged their perception on the educational value of the text messages and the effect on their pediatric board preparation. Feasibility of the system was assessed as a total percentage of messages successfully received by residents.Of 55 residents, 35 (64%) participated in the program. Of 2534 messages sent out to participants, 2437 (96.2%) were delivered successfully. Positive comments cited the texting of board facts as a quick, helpful, daily study tool. Residents liked that messages were sent at 2:00 pm, and most felt that 1 to 5 messages per week was appropriate. Drawbacks included character restrictions of messages, content limitations, and the lack of a question-answer format.An educational text message-based program was successfully implemented in our residency program. Messages were delivered with a high success rate, and residents found educational value in the messages.
View details for DOI 10.4300/JGME-D-13-00469.1
View details for PubMedID 26140130
View details for PubMedCentralID PMC4477575
- Text Messaging in Medical Education PEDIATRICS 2014; 133 (3): E491-E493