Bilateral atresia of the coronary artery ostia in patients with pulmonary atresia and intact ventricular septum is a rare condition with no reported survivors beyond early infancy. We report the first case of diagnosis by echocardiography followed by successful cardiac transplantation.
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http://dx.doi.org/10.1016/j.echo.2006.02.006 | DOI Listing |
Multimed Man Cardiothorac Surg
January 2025
• Department of Cardiac Sciences, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Jeddah, Saudi Arabia • King Abdullah International Medical Research Center, Ministry of National Guard Health Affairs, Jeddah, Saudi Arabia • College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Ministry of National Guard Health Affairs, Jeddah, Saudi Arabia.
Prostaglandin E1 is a potent vasodilator that prevents the ductus arteriosus from closing. Its use in neonates with cyanotic heart defects has revolutionized the management of children with cyanotic heart defects. Although the use of prostaglandin E1 is a temporary solution, the establishment of dependable pulmonary blood flow is of paramount importance.
View Article and Find Full Text PDFWorld J Pediatr Congenit Heart Surg
January 2025
Department of Pediatrics, Inova Health System, Falls Church, VA, USA.
Pulmonary atresia with ventricular septal defect (PA-VSD) is usually diagnosed by transthoracic or fetal echocardiography, with the prenatal diagnosis being feasible and accurate if fetal cardiology services are available. The limitations of transthoracic echocardiography (TTE) in the evaluation of PA-VSD include the complete evaluation of the pulmonary arteries and patent ductus arteriosus, quantitative evaluation of the right ventricle size and function, and delineation of associated cardiac anomalies such as coronary artery anomalies, anomalies of systemic or pulmonary venous return, and complex arch anomalies. Echocardiography also has limitations in evaluating hemodynamics such as flow volumes, shunts, and regurgitant fraction.
View Article and Find Full Text PDFWorld J Pediatr Congenit Heart Surg
January 2025
Division of Cardiac Surgery, University of Toronto, Toronto, ON, Canada.
The presentation of pulmonary vasculature in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) is highly variable-as is the number, size and position of the MAPCAs and their relationship with the native pulmonary artery system. The priority in the management of this disease should be attaining timely and complete unifocalization, as opposed to single-stage full repair in every case. The merit of early unifocalization is that it secures the pulmonary vascular bed by (a) avoiding loss of lung segments from progressive stenosis/atresia of MAPCA origins, (b) preventing lung injury from high pressure/flow in areas fed by large, unobstructed MAPCAs, and (c) restoring central continuity of the pulmonary vasculature.
View Article and Find Full Text PDFPediatr Cardiol
January 2025
Department of Pediatrics, Inova Children's Hospital, Fairfax, VA, USA.
Data on outcomes of extracorporeal membrane oxygenation (ECMO) are limited in patients with pulmonary atresia intact ventricular septum (PAIVS). The objective of this study was to describe the use of ECMO and the associated outcomes in patients with PAIVS. We retrospectively reviewed neonates with PAIVS who received ECMO between 2009 and 2019 in 19 US hospitals affiliated with the Collaborative Research for the Pediatric Cardiac Intensive Care Society (CoRe-PCICS).
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
December 2024
Children's Heart Institute, Children's Memorial Hermann Hospital, Houston, Texas.
A patient with known pulmonary atresia and intact ventricular septum and ductal stent presented with low cardiac output and arrythmia. Intraoperatively, the patient was found to have an anomalous left coronary artery arising from the pulmonary artery. After reimplantation of the left coronary artery to the aortic root and placement of a central shunt, the patient progressed well and was discharged home.
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