Pulmonary atresia with intact ventricular septum, right-sided aortic arch, and an aorto-pulmonary collateral artery.
Described is a rare association in a patient with the heart in the left chest, namely pulmonary atresia with intact ventricular septum, fistulous coronary arterial connections, a right-sided aortic arch and an aorto-pulmonary collateral artery feeding one lung. The pulmonary arteries were non-confluent, with the right lung supplied by the right arterial duct originating from the under surface of the right-sided aortic arch, and the left lung supplied through the aorto-pulmonary collateral artery arising from the descending aorta. The surgical management is different in the setting of non-confluent pulmonary arteries.
Patel
C R
CR
Department of Pediatrics, Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA.
Spector
M L
ML
Zahka
K G
KG
D001013:Aorta, Thoracic / Q000002:abnormalities*; D003097:Collateral Circulation; D006330:Heart Defects, Congenital / Q000473:pathology*; D006346:Heart Septum; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D018633:Pulmonary Atresia / Q000473:pathology*