Studies of Congenital Heart Disease

Abstract

Congenital pulmonic stenosis is indicated by cardiac catheterization by the finding of a higher systolic pressure in the right ventricle than in the pulmonary artery. Eight cases of uncomplicated pulmonic stenosis are studied. The findings on history, physical examination, x-ray and fluoroscopy, and electrocardiogram have been analyzed and the variations in circulatory dynamics encountered in these individuals are described in detail.IT IS generally considered that congenital pulmonic stenosis unaccompanied by other cardiac defects is rare. Recently, Greene and coworkers1 have made a complete review of the literature and have collected 68 cases. Their article and the book of Brown2 contain excellent discussions of this disorder.Cardiac catheterization3 has made possible an accurate recognition of pulmonic stenosis by the finding of a higher systolic pressure in the right ventricle than in the pulmonary artery.4 Pollack, Taylor, Odel, and Burchell5 have recently published a report of 3 cases recognized by this technic, 4 more were reported by Greene and associates', and this paper deals with 8 more cases. METHODSEach patient remained in the hospital for at least four days. A history, physical examination, routine laboratory tests, electrocardiogram, x-ray examination, and fluoroscopy of the heart were carried out. Heart sound tracings were obtained in all cases. On the third day, venous catheterization was performed and the diagnostic routine described elsewhere4 was performed.The circulatory dynamics of 5 of the patients were studied at rest and also under conditions of split. The pulmonic second sound and mitral first sound were of normal intensity. In the third left intercostal space there was a Grade 4, rough, medium-pitched, systolic murmur, stopping just before the second sound. There was an accompanying thrill but no diastolic murmur. The systolic murmur was widely transmitted in diminished intensity over the precordium and faintly over the back. The lungs were clear, abdominal examination normal, radial and femoral pulses were strong, veins were not distended, and no edema could be discerned. TheHinton test 'was negative and urinalysis was normal.The hematocrit was 49 per cent, the hemoglobin 16.5 Gm., the white blood cell count and differential normal, and the routine blood chemistries were normal. X-ray and fluoroscopy revealed a heart of normal size. The pulmonary artery was enlarged and pulsating. The pulmonary vascular markings were within normal limits. The electrocardiogram showed incomplete right bundle branch block.Case 2. R. L. (P.B.B.H. #s6A875) was a 26 year old man in whom a heart murmur had been detected in childhood. He had never had symptoms of rheumatic fever, tuberculosis, or cardiac failure. He had always been fond of sports and was an enthusiastic skier. He had never noticed any decrease in exercise tolerance. Four months before admission, he contracted bacteriologically proven subacute bacterial endocarditis which was treated with penicillin in another city and pronounced cured. Becau...

Studies of the Pulmonary Circulation in Man at Rest. Normal Variations and the Interrelations Between Increased Pulmonary Blood Flow, Elevated Pulmonary Arterial Pressure, and High Pulmonary “Capillary” Pressures 1

“…As a matter of fact, neither occurs because of the high pulmonary arteriolar resistance. Pressures are maintained normal for the systemic circuit and high for the pulmonary circuit, blood-flows through the lung and through the periphery remain adequate for years, and the shunt from right-to-left and leftto-right remains minimal due to the essentially equal resistance offered by the pulmonary arterioles on the one hand and the systemic arterioles on the other, as Bing, Vandam, and Gray (16) first showed and we have confirmed (26). Bing and his associates did not measure pulmonary "capillary" pressure, but in calculating resistance assumed a pulmonary capillary or left auricular pressure of zero.…”

“…body PA-"PC" pressure gradient became definitely surface, pressure in the pulmonary artery began to wider. Since patients with an elevated pulmonary RELATION described elsewhere (26). The salient characteristics were that the systolic pressures in the brachial artery, pulmonary artery, and right ventricle were identical when recorded simultaneously.…”

“…The arteriolar resistances were enormous, being similar to or greater in magnitude than those in the normal systemic circuit. Comment It is not the purpose to discuss here the circulatory aspects and clinical course of Eisenmenger's complex, as this is treated in detail in another communication (26). From the point of view of pulmonary circulatory dynamics, it is of interest that there is a high resistance to blood flow through the pulmonary arterioles.…”

Experimental Arterial Stenosis: Post Stenotic Dilatation and Collateral Blood Flow

“…Arterial stenosis may be associated with an increase in the size of the post stenotic portion of the artery, as well as with an increase in the size-and possibly the number-of the collateral vessels (1,2,3,4,5,6,7). Although there is some quantitative information concerning the development of the collateral circulation, there is little such information concerning the degree and extent of the post stenotic dilatation (4,5,6,7).…”

“…Although there is some quantitative information concerning the development of the collateral circulation, there is little such information concerning the degree and extent of the post stenotic dilatation (4,5,6,7). Also, there is no general agreement as to whether these changes are primarily related to any existing tissue ischemia or to the change in pressure gradients in the involved vessels (2,4,8,9).…”