Allan L. Simon

Angiographic Anatomy of the Left Ventricle and Mitral Valve in Idiopathic Hypertrophic Subaortic Stenosis

The angiographic features of the left ventricle were examined in patients with idiopathic hypertrophic subaortic stenosis who had clinical and hemodynamic evidence of obstruction. Of 36 combined hemodynamic and angiographic studies considered to be technically satisfactory, 33 showed a characteristic combination of abnormalities. In the frontal projection in systole, a linear radiolucent area extended across the left ventricular outflow tract 2 to 2.5 cm below the aortic annulus, at a level corresponding to the site of intraventricular pressure change. In the left oblique and lateral projections, the mitral leaflets did not swing posteriorly in a normal fashion, but projected into the outflow tract during mid and late systole. The radiolucent line, seen in the frontal views, was considered to represent contact of the leading edge of the leaflet with the hypertrophied muscular interventricular septum. The jet of mitral regurgitation, when present, was seen immediately below the anterior mitral leaflet.Severe hypertrophy was also seen to involve the inferior portion of the muscular septum, causing displacement of the papillary muscles superiorly and to the left. This maldirection of the papillary muscles was postulated to cause abnormal traction on the chordae tendineae and to prevent normal movement of the mitral leaflets away from the septum during systole. The leaflets, held in the outflow tract, form the posterior component of the obstruction, the anterolateral component of which results from severe, asymmetric septal hypertrophy. It was proposed that this mechanism plays an important part in producing the intraventricular pressure gradient in many patients with idiopathic hypertrophic subaortic stenosis.

Juxtaductal aortic coarctation. Analysis of 84 patients studied hemodynamically, angiographically, and morphologically after age 1 year.

Although many studies of juxtaductal coarctation of the aorta have been reported, none has correlated clinical, hemodynamic, angiographic, anatomic, and operative findings. Of 84 patients (62 male and 22 female; age range, 1 to 49 years [mean 17]), all had murmurs; 76 had absent, diminished, or delayed femoral pulsations; 50 had cuff systolic blood pressures in the arm greater than 140 mm Hg, and 30 had diastolic pressures greater than 90 mm Hg. The average pressure gradients (mm Hg) by direct measurements above and below the coarctation in 35 patients were peak systolic, 45; mean, 17; and diastolic, 5. Rib notching, visible in chest roentgenograms in 43 patients, correlated directly with age and inversely with the diameter of the coarctation. Moderate or marked cardiomegaly by radiograph was present in only 1 of 48 patients with isolated coarctation and in 17 of 36 with associated cardiovascular malformations. Electrocardiograms were abnormal in more than two thirds of patients with associated anomalies, but were normal in more than three fourths of those with isolated coarctation. In 70 excised, serially sectioned coarctations the aortic lumens were completely occluded in 4 patients, up to 0.5 mm in internal diameter in 22 patients, from 0.6 to 2 mm in 26 patients, from 2.1 to 5 mm in 14, and greater than 5 mm in 4, and correlated directly with lumens measured angiographically. The most significant anatomic factor causing the coarctation was invagination of the media from the posterior aortic wall, but intimal proliferation (jet lesion) at and immediately distal to the invagination contributed to the narrowing. Three (each with associated anomalies) of 70 patients died early after coarctation repair. Systolic or diastolic blood pressures decreased early postoperatively in 58 (87%) of 67 surviving patients, and both pressures decreased in 42 (63%). Late postoperatively (mean follow-up, 4.7 years), the systolic blood pressure remained elevated in 25% of patients.

Congenital Fixed Subvalvular Aortic Stenosis: An Anatomical Classification and Correlations with Operative Results

Detailed assessments were carried out before and after operation in 33 consecutive patients with congenital fixed subvalvular aortic stenosis. Effective relief of obstruction to left ventricular outflow can be accomplished at minimal risk in patients with discrete subaortic stenosis, but a pressure gradient may persist after operation because of secondary hypertrophic obstruction which resolves with time. Tunnel subaortic stenosis is characterized by a diffusely narrowed left ventricular outflow tract. When an intraventricular pressure gradient was present in such patients, good but not complete relief of obstruction was achieved. In tunnel deformities with a gradient at the valve or annulus and in subaortic stenosis produced by mitral valve anomalies, operative intervention was ineffective.

Acquired right ventricular outflow obstruction in a child with neurofibromatosis

Abstract A case of severe acquired localized right ventricular obstruction in an 8 1 2 - year-old child with von Recklinghausens syndrome is presented. The similarity of this case to vascular involvement in previously reported cases is pointed out. It is suggested that vascular lesions in neurofibromatosis may have varied manifestations and that this syndrome should be thought of in the future as a generalized disease which may affect any part of the cardiovascular system. For the lesion here reported, excision of the fibromuscular band has resulted in disappearance of her pressure difference, murmur, and right heart failure.

Heart motion video-tracking (radarkymography) in diagnosis of congenital and acquired heart disease

Abstract Heart motion video-tracking (radarkymography) is a technic for recording the movements of cardiovascular structures. Over 100 patients with a variety of congenital and rheumatic heart lesions were studied. Characteristic graphic linear tracings were obtained in patients with mitral valve disease, obstruction to the aortic outflow tract, ventricular aneurysms and coarctation of the aorta. Radarkymography offers certain advantages over electrokymography, which to date has been the major technic for recording motion of the cardiac silhouette.

Clinical and Laboratory Findings in Patients with Nonobstructive Intraventricular Pressure Differences

Clinical and laboratory findings were examined in eight patients with nonobstructive intraventricular pressure differences resulting from catheter entrapment in obliterated apical portions of the left ventricular cavity. Cardiorespiratory symptoms were reported by six of the eight patients; three had angina pectoris, and two had experienced syncope. A soft systolic ejection murmur was noted at the cardiac apex or left sternal edge, or at both sites, in each patient at rest or following exercise; no patient had a bifid systolic arterial pulse contour. Paradoxical splitting of the second heart sound with respiration did not occur. In five patients the cardiac index was substantially elevated. In four, an intraventricular pressure difference was present in the basal state, while in the remaining four a pressure difference was elicited by the Valsalva maneuver and isoproterenol. In each patient the arterial pulse pressure consistently increased in the beat following a ventricular extrasystole, and angiographic evidence of left ventricular outflow obstruction was not observed. In all patients, obliteration of the apical portion of the left ventricular cavity during systole was demonstrated angiographically to result from an extreme degree of systolic emptying. Left ventricular hypertrophy was demonstrated angiographically in six patients, four of whom exhibited asymmetric hypertrophy, predominantly involving the interventricular septum. In two patients no abnormality of the left ventricular cavity contour was apparent. These clinical, hemodynamic, and angiographic findings allow differentiation of patients with nonobstructive pressure differences from those with hypertrophic subaortic stenosis.

The angiographic features of bicuspid and unicommissural aortic stenosis

Abstract Stenosis of a congenitally bicuspid aortic valve results in a characteristic angiographic appearance: In systole the valve leaflets are domed, and there is a central jet that corresponds to the orifice of the stenotic valve. In diastole the sinuses of Valsalva reflect the morphologic features of the 2 aortic cusps. In contrast, the anatomic derangement of unicommissural aortic stenosis is reflected in an angiogram which can be differentiated from that of bicuspid aortic stenosis. In this case, the stenotic orifice visualized by the jet in systole can be seen in contact with the posterior wall of the aorta; leaflet tissue and valve motion are seen only anteriorly. The diastolic appearance differs from that of the bicuspid valve by the presence of a deep sinus of Valsalva anteriorly and a very shallow posterior sinus, also resulting from the lack of leaflet tissue posteriorly. This differentiation is important since extensive valvulotomy on the unicommissural valve will result in significant aortic regurgitation postoperatively.

Diffuse Pulmonary Artery Calcifications in a Case of Eisenmenger's Syndrome

A 53-year-old diabetic Caucasian man with Eisenmengers syndrome and an atrial septal defect associated with extensive pulmonary artery calcification is presented. The significance of pulmonary artery calcification in congenital heart disease associated with shunts and the differential diagnosis of pulmonary artery calcification are discussed.

Correlation of Chest Roentgenograms and Hemodynamic Findings Following Surgical Repair of Ventricular Septal Defects

The diagnosis and hemodynamic evaluation of ventricular septal defect are made at cardiac catheterization. The present-day value of the plain chest roentgenogram, then, is in following the evolution of the defect, and it has proved especially useful in detecting Eisenmengers reaction and its associated pulmonary hypertension. Little emphasis, however, has been placed on the changes seen following surgical closure of the defect. This study was undertaken to determine the roentgen changes in the chest following successful closure of the ventricular septal defects and the rate at which such changes evolve. Materials and Methods The chest films and catheterization data of all patients operated upon for the repair of uncomplicated ventricular septal defects from January 1959 to December 1965 and in whom preoperative and postoperative cardiac catheterizations were performed were analyzed independently. Patients with residual postoperative shunts were included in the original analysis, and the person evaluating...

Correlation of Chest Roentgenograms and Hemodynamic Findings Following Surgical Closure of Secundum Atrial Septal Defects

Although the diagnosis of an atrial septal defect can be suspected on the plain roentgenograms of the chest (13), the critical features of such a defect—the degree of intracardiac shunting, the pulmonary artery pressure, pulmonary vascular resistance, and anatomic definition of the lesion—are determined by cardiac catheterization and angiocardiography (4, 7–10). The chief value of chest roentgenograms, therefore, is in following the progression of the lesion and the possible development of pulmonary hypertension (11). Roentgen observation of the changes in vascular structures following surgical closure may also give valuable information about the adequacy of the repair (10, 13, 14). The purpose of this study was to determine the roentgen changes occurring in the chest following complete closure of secundum atrial septal defects and the rate at which these changes occur. Materials And Methods The chest roentgenograms and catheterization data of all patients with surgical repair of secundum atrial septal de...