Complete Atresia of Coronary Ostia in Pulmonary Atresia and Intact Ventricular Septum S.E. Selamet, 1 D.T. Hsu, 1 H.M. Thaker, 2 W.M. Gersony 1 1 Department of Pediatrics, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA 2 Department of Pathology, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA Abstract. We describe the clinical course, echocardi- ography, angiography, and histopathology of a fe- male infant with pulmonary atresia and intact ventricular septum (PA/IVS) with complete coronary ostial atresia and right ventricle-dependent coronary circulation who survived for 7 weeks after palliative surgery. The patient expired from myocardial insuf- ficiency while waiting for a donor heart. Postmortem examination demonstrated atretic coronary ostia, ventricular sinusoids, and myocardial infarctions of various ages. This report suggests that neonates with PA/IVS who have this extreme form of coronary abnormality may potentially be managed medically and surgically until cardiac transplantation is avail- able. Keywords: Atresia of Coronary Ostia — Pulmonary Atresia — Ischemia — Myocardial Infarction Case Report Hypoplastic right heart, pulmonary atresia, and tricuspid atresia were diagnosed by fetal echocardiography in a 27-week-old fetus. The infant was born full term, delivered via cesarean section due to fetal bradycardia. APGARs were 7 at 1 minute and 8 at 5 minutes. Birth weight was 3940 g. Physical examination revealed a well-developed newborn fe- male, breathing comfortably on room air. On auscultation, the S 2 was single, and a 2/6 short systolic ejection murmur was audible at the left sternal border. There was no hepatomegaly. Extremities were warm and well perfused, and distal pulses were full. Capillary refill was less than 2 seconds. Oxygen saturation was 80% on room air. The electrocardio- gram showed normal sinus rhythm and left axis deviation with prominent left ventricular forces. Chest x-ray demonstrated clear lung fields and a mildly enlarged cardiac silhouette. The echocar- diogram revealed situs solitus with normally related great vessels, pulmonary atresia, tricuspid stenosis, and hypoplastic right ven- tricle with intact ventricular septum. A tortuous patent ductus ar- teriosus was demonstrated with good-sized branch pulmonary arteries. Myocardial sinusoids were demonstrated. Coronary ar- teries could not be well visualized. Qualitatively, left ventricular function was normal. Chromosome analysis showed 46XX. The patient was administered prostoglandin immediately after birth to maintain ductal patency. Cardiac catheterization was performed on the second day of life. Right ventricular pressure was suprasystemic (70/8 mmHg). Aortic pressure was 56/22 mmHg with a mean of 36 mmHg. Right ventricular angiography demon- strated pulmonary atresia, a severely hypoplastic right ventricle, and a small tricuspid valve. Multiple sinusoidal connections were seen in the right ventricular myocardium with filling of the right and left circumflex coronary arteries and no reflux into the aortic sinuses (Fig. 1). A retrograde ascending aortogram failed to dem- onstrate antegrade filling of the coronary arteries from either cor- onary ostia; therefore, selective coronary angiography could not be carried out. The patient was placed on the waiting list for cardiac transplantation. On day 14 of life, a 3.5-mm modified right Blalock–Taussig shunt operation was performed as a potential bridge to trans- plantation and prostoglandin was discontinued. On the first post- operative day, the patient displayed evidence of diffuse ST segment elevation on anterolateral leads associated with an increase in troponin from 1.9 to 226.3 units. On postoperative day 5, ven- tricular function was diminished and inotropic support was insti- tuted. In addition, echocardiographic evidence of left pulmonary artery stenosis was noted at the site of the ductus arteriosus, and prostaglandin was restarted to maintain left pulmonary artery blood flow. During the ensuing 3 weeks, the patient developed sepsis, narcotizing enterocolitis, and renal failure. Ventilatory and circulatory support was withdrawn on day 41. Postmortem examination of the heart demonstrated situs solitus with atrioventricular and ventriculoarterial concordance. The heart weighed 40.0 g. There was pulmonary atresia involving the right ventricular infundibulum, pulmonary valve, and proximal pulmonary trunk (Fig. 2). The ventricular septum was intact. The tricuspid valve was hypoplastic (9 mm) in circumference and ste- notic, and the right ventricle was hypoplastic with only an isolated inlet portion and a volume less than 1 ml. The coronary sinus was Correspondence to: S.E. Selamet, Division of Pediatric Cardiology, Department of Pediatrics, College of Physicians and Surgeons, Columbia University, Children’s Hospital of New York, Room 2, North 3959 Broadway, New York, NY 10032, USA; email: ss1807@columbia.edu Pediatr Cardiol 25:67–69, 2004 DOI: 10.1007/s00246-003-0517-0