PEDIATRICS Vol. 32 No. 5 November 1963, pp. 841-854
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PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM

Brian Kiely M.D.1, Francisco Morales M.D., and David Rosenblum M.D.2

1 The Department of Radiology, State University of New York, Downstate Medical Center, Brooklyn, N.Y.
2 The Department of Pediatrics, New York University School of Medicine, New York, N.Y.

When the pulmonary valve is atretic and the ventricular septum intact, the right ventricle usually consists of a small chamber with a very thick wall capable of developing high pressure. This pathologic picture is associated with clinical findings similar to those in tricuspid atresia—cyanosis, decreased pulmonary flow, left ventricular preponderance on the electrocardiogram, and early death. The diagnosis may be confirmed by heart catheterization and selective angiocardiography with injection into the right ventricle, but the risk is great. Surgery has never been successful in the past; but because of the equally hopeless prognosis on medical treatment, attempts should continue to be made. Anastomosis of the superior vena cava to the right pulmonary artery appears to offer hope of success in the future.

Submitted on January 10, 1962
Accepted on April 30, 1963