Pulmonic stenosis, ventricular septal defect, and right ventricular pressure above systemic level.

IT IS USUALLY possible to distinguish pulmonic stenosis with an intact ventric-ular septum (designated as pulmonic stenosis) from pulmonic stenosis with a right-to-left shunt through a ventricular septal defect (te-tralogy of Fallot) by routine clinical, electro-cardiographic, and radiologic examination.1-3 If the diagnosis is still uncertain, it can as a rule be made by phonocardiography and car-diae catheterization,4 particularly if there is very severe pulmonic stenosis with an intact ventricular septum. Characteristically in severe pulmonic stenosis the right ventricular systolic pressure is much higher than in the systemic circulation and rises still higher after ectopic beats; the right ventricular pressure tracing is triangular; and right ventricular systole is prolonged so that on phonocardiog-raphy the second heart sound is widely split and the systolic murmur passes beyond aortic valve closure. Unfortunately these commonly accepted criteria may be unreliable. In this paper 10 patients are reported who, although meeting most or all of these criteria for the diagnosis of severe pulmonic stenosis (especially with systolic pressures much higher in the right ventricle than in the systemic arteries), nevertheless were proved later to have an associated ventricular septal defect with a right-to-left shunt. An attempt is made to indicate how even under these circumstances a correct preoperative diagnosis may be made. These 10 patients were admitted to the Chil-dren's Medical Center for investigation and operation , and all but 1 were seen after June 1957. Cardiac catheterizations as described previously from this laboratory5 were done in all, and phono-cardiogranms were taken with a Sanborn Twin-Beam photographic recorder. Results Clinieally all of these patients had onie or more signs suggestive of valvular pulmonic stenosis with an intact ventricular septunm. There were large "a" waves in the jugular venous pulse in 4; the systolic thrill in 7 anid the stenotic systolic murmur in 8 were maxi-inal at the second or third left intercostal space; the second heart sound was thought to be widely split in 4; and 2 with elinically severe pulmonic stenosis had no eyanosis. The electrocardiogram showed P pulmonale in 6 and muarked right ventricular hypertrophy in all 10 the R waves in lead V, were 20 to 30 mm. high in a and over 30 mm. high in 4. On x-ray, 8 had cardiae enlargement (marked in 4) and 4 had what was initerpreted as post-stenotic dilatation of the pulmonary artery. Any of these signls may be found in an otherwise classical tetralogy …