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This report describes the use of the Edwards Sapien THV in a patient who had a short regurgitant/stenotic homograft with early bifurcation stenoses of the pulmonary arteries. A 48-mm AndraStent was positioned in the right pulmonary artery-homograft jailing the left pulmonary artery (LPA). To have an unobstructed access to the LPA, the stent strut leading to the LPA was broken using high-pressure balloon. A 23-mm Edwards Sapien THV was positioned in the stented homograft just proximal to the LPA origin with resolution of the stenosis and regurgitation., (Copyright © 2012 Wiley Periodicals, Inc.)

We describe a two-month-old infant with early congestive heart failure due to anomalous origin of the right coronary artery from the pulmonary artery. The diagnosis was made by two-dimensional and color flow Doppler echocardiography, confirmed by angiocardiography, and the case was successfully corrected at surgery. As opposed to the more frequent anomalous origin of the left coronary artery from the pulmonary trunk, this anomaly generally does not cause any typical clinical finding, often becoming an autoptic or surgical surprise after infancy or in adult age.

Objective: The goal of this study was to determine the patterns of the pulmonary circulation in patients with pulmonary atresia and asplenia., Background: The asplenic cardiac syndromes characteristically have complex cardiac anomalies including pulmonary stenosis or atresia. Definition of the pulmonary artery circulation and pulmonary venous connections is needed for consideration of surgical procedures., Methods: In 35 patients, the sources of pulmonary blood flow, anatomic features of pulmonary arteries and pulmonary venous connections were determined from angiograms or autopsy specimens., Results: The main pulmonary artery was absent or hypoplastic in 91% of patients; most had a ductus arteriosus. The right and left pulmonary arteries were confluent in 90% and usually of normal size (right 71%, left 63%). Total anomalous pulmonary venous connections were present in 38%., Conclusions: The anatomic features of the pulmonary arteries in pulmonary atresia associated with the asplenic cardiac syndrome are usually favorable for palliative surgical procedures. Total anomalous pulmonary venous connection may exist as a complicating factor.

The patterns of pulmonary circulation were assessed by angiography in a series of 24 patients with right atrial isomerism (asplenia syndrome) and pulmonary atresia. Mean age at catheterization was 21.5 days (range 1 day-11 months). The pulmonary arteries were confluent in 22 cases (91.7%). Eighteen patients (75%) had ductus-dependent pulmonary circulation in presence of confluent pulmonary arteries, similar to cases of pulmonary atresia combined with complex congenital heart disease. The ductus appeared in continuity with the aortic arch, shaping an acute angle with the descending aorta, as in cases of pulmonary atresia with ventricular septal defect. A bilateral ductus was present in 5 cases (20.8%), 3 with confluent and 2 with nonconfluent pulmonary arteries. One patient presented multiple systemic collaterals to the lungs. All the other patients had normal arborization of the pulmonary arteries. Seven patients presented an obstructed total anomalous pulmonary venous connection: 4 in inferior vena cava and 3 in superior vena cava. These anatomical observations provide useful information for the surgical palliation of these anomalies and, probably, warrant pre-operative angiographic evaluation in all the patients with right atrial isomerism.