Daniel Murphy

Abstract

The management of congenital aortic stenosis in neonates and infants continues to be a surgical challenge. We have performed the modified Ross-Konno procedure for patients who have severe aortic insufficiency or significant residual stenosis after balloon aortic dilation. The midterm results of this procedure were evaluated in this subset of patients.Between 1994 and 2010, a total of 24 patients younger than 1 year of age underwent the modified Ross-Konno procedure. The diagnoses were aortic stenosis with or without subaortic stenosis (n = 16), Shone's complex (n = 7), and interrupted aortic arch with subaortic stenosis (n = 1). The aortic root was replaced with a pulmonary autograft, and the left ventricular outflow tract (LVOT) was enlarged with a right ventricular infundibular free wall muscular extension harvested with the autograft.Age at operation ranged from 1 to 236 days (median 28 days). The median follow-up period was 81 months (range 1-173 months). There was 1 early death and no late mortality. Overall the 1-, 2-, and 5-year survival rate was 95% ± 4.5%. Freedom from aortic stenosis was 94.7% ± 5.1% at 1, 2, and 5 years. Less than mild aortic insufficiency was 93.3% ± 6.4% at 2 years, and 74.7% ± 12.9% at 5 years. In total, 23 reoperations and reinterventions were performed; 14 were allograft conduit replacements. Two patients required aortic valve plasty. None required valve replacement. The reintervention-free rate was 64.6% ± 10.8% at 2 years and 36.9% ± 11.3% at 5 years.Pulmonary autografts demonstrated good durability with low mortality and morbidity. This study shows that the modified Ross-Konno procedure can be a practical choice in selective cases for complex LVOT stenosis in neonates and infants.

View details for DOI 10.1016/j.athoracsur.2012.03.007

View details for Web of Science ID 000305801600033

View details for PubMedID 22626750

Abstract

The aim of this study was to develop a simpler echocardiographic screening tool for aortic root dilation in pediatric patients with Marfan syndrome. Aortic root dilation represents the most common cause of morbidity and mortality in patients with Marfan syndrome. It is traditionally defined by nomograms, relating aortic root dimensions to height or body surface area. In this study, the descending aorta (DAo) was used as an internal reference, thereby eliminating the need for nomograms when screening for aortic root dilation.The diameters of the aortic root at the sinus of Valsalva and the DAo were measured in 35 patients with Marfan syndrome and 52 normal controls in the parasternal long-axis echocardiographic view. The root/DAo ratio was calculated.A root/DAo ratio > or = 2 provided optimal sensitivity for root dilation (100%), whereas a root/DAo ratio > or = 2.3 provided optimal specificity (100%).The root/DAo ratio provides a simple, rapid, and valuable screening test for aortic root dilation, independent of nomograms and body size.

View details for DOI 10.1016/j.echo.2009.06.002

View details for Web of Science ID 000270367900003

View details for PubMedID 19647408

Abstract

We report a 23-year-old male with history of double-inlet single ventricle with transposition of the great arteries who is s/p pulmonary artery banding, a Damus-Kaye-Stanzel anastomosis, and Fontan procedure during infancy and childhood who now presents with chest pain. A chest CTA at an outside hospital was thought concerning for the presence of a thoracic aortic aneurysm with dissection, prompting immediate transfer to our institution. However, repeat chest CTA at our institution revealed the predicted anastomoses based on his surgical procedures, which was misinterpreted as aortic aneurysm and dissection. An understanding of the physiology of his surgically repaired congenital heart disease is critical in interpreting his subsequent chest CTA and arriving at the appropriate clinical conclusion.

View details for DOI 10.1016/j.ijcard.2007.04.165

View details for Web of Science ID 000257950500043

View details for PubMedID 17689761

Abstract

Extensive evidence is available that cardiovascular structure and function, along with other biological properties that span the range of organism size and speciation, scale with body size. Although appreciation of such factors is commonplace in pediatrics, cardiovascular measurements in the adult population, with similarly wide variation in body size, are rarely corrected for body size. In this review, we describe the critical role of body size measurements in cardiovascular medicine. Using examples, we illustrate the confounding effects of body size. Current cardiovascular scaling practices are reviewed, as are limitations and alternative relationships between body and cardiovascular dimensions. The experimental evidence, theoretical basis, and clinical application of scaling of various functional parameters are presented. Appropriately scaled parameters aid diagnostic and therapeutic decision making in specific disease states such as hypertrophic cardiomyopathy and congestive heart failure. Large-scale studies in clinical populations are needed to define normative relationships for this purpose. Lack of appropriate consideration of body size in the evaluation of cardiovascular structure and function may adversely affect recognition and treatment of cardiovascular disease states in the adult patient.

View details for DOI 10.1161/CIRCULATIONAHA.107.736785

View details for Web of Science ID 000255394300014

View details for PubMedID 18443249

View details for DOI 10.1161/CIRCULATIONAHA.107.653584

View details for Web of Science ID 000254576500013

View details for PubMedID 18378625

View details for DOI 10.1161/CIRCULATIONAHA.107.653576

View details for Web of Science ID 000254065200011

View details for PubMedID 18347220

View details for PubMedID 16950481

Abstract

There is a large group of young adults who survived atrial baffle repair of transposition of the great arteries. Most survivors are asymptomatic, although nearly all have decreased exercise capacity. Loss of sinus rhythm and atrial arrhythmias are common and increase with age. There is concern about the ability of the right ventricle to function long term as a systemic pump, and recent publications have highlighted right ventricular dysfunction in this patient population. Sudden death and congestive heart failure are the main causes of death, and outcomes beyond 30 years are unknown. Pulmonary artery banding, late arterial switch, and cardiac transplantation are employed when intractable arrhythmias or right ventricular failure threaten survival or quality of life.

View details for PubMedID 15987628

Abstract

Specialized tertiary care facilities developed in response to the increasing numbers of adults with congenital heart disease (CHD). Because this patient population comprises a relatively new area of specialized cardiovascular interest, the first facilities necessarily evolved without preexisting guidelines or interaction.To characterize the major features of the six original and largest tertiary adults CHD facilities.Written questionnaire sent to six participating facilities in North America and Europe. Information was analyzed centrally.All but one facilities was established over 20 years ago, and each cares for over 1500 patients. Hospital admissions ranged from 100 to 660 patients/unit/year. Of the total number of registered patients, 52-81% had undergone one or more reparative surgeries. Reoperations constituted 25-80% of the 50-170 operations/unit/year. Overall mean surgical mortality was 1.9%/year. Inpatient and outpatient care was provided in adult (n = 4) or both adult and pediatric (n = 2) settings. All six facilities enjoyed close collaboration between adult and pediatric cardiologists, cardiac surgeons, nurse specialists and cardiac and non-cardiac consultants. Training and research were pivotal activities.Provision of comprehensive care by multidisciplinary teams including adult and pediatric cardiologists, cardiac surgeons, specialized nurses and other cardiac and non-cardiac consultants was the unifying feature for all six tertiary care facilities reported here. There were minor differences among them based on available resources, local expertise and national health care policies. There appears to be a significant shortfall in tertiary care provision for the adult with CHD that requires further planning and resource allocation. These data may be useful for new and evolving adult CHD services.

View details for DOI 10.1016/ijcard.2003.06.019

View details for Web of Science ID 000223117000014

View details for PubMedID 15262035

View details for DOI 10.1016/j.jacc.2004.06.019

View details for Web of Science ID 000222914200008

View details for PubMedID 15261687

Abstract

Adult patients with a history of transposition of the great arteries and atrial switch operation were studied with transthoracic echocardiography and cardiovascular magnetic resonance imaging. Measurements of ventricular dimensions and function by these 2 techniques in 18 consecutive patients were well correlated.

View details for DOI 10.1016/j.amjcard.2003.11.044

View details for Web of Science ID 000220075800033

View details for PubMedID 14996604

Abstract

Anomalous origin of the left main coronary artery from the right anterior coronary sinus has been associated with high incidence of sudden death in young adults. We describe a simplified approach to this rare congenital anomaly, which avoids the need for commissural post resuspension or relocation of the coronary button.

View details for Web of Science ID 000183968400059

View details for PubMedID 12842554