Richard D. Rowe

Discrete Subaortic Stenosis

Twenty-five patients with discrete subaortic stenosis were reviewed. Twenty were operated on for severe obstruction. Review of available data suggests there is a wide spectrum of the disease which may be divided into two main types designated I and II. Type I is a thin discrete membrane immediately under the aortic valve obstructing the outflow but not associated with narrowing of it. Type II is situated about 1 cm below the valve and consists of a fibrous ring, thicker than type I, and associated with muscular hypertrophy which narrows the outflow tract, encroaches on the anterior leaflet of the mitral valve, and may extend 1-2 cm downward. Both have a characteristic angiographic appearance. Results of surgery are good for type I but not satisfactory for type II. Aortic incompetence is likely to increase or develop following operation in both types.

Transient myocardial ischemia of the newborn infant: A form of severe cardiorespiratory distress in full-term infants

In three mature newborn infants a syndrome of cyanosis, congestive failure, and acuteleft ventricular failure has mimicked severe congenital heart disease. Weak arterial pulses, liver enlargement, and right heart overload in the electrocardigram suggested the diagnosis of hypoplasia of the left heart. It is postulated that the disorder is one of impaired coronary perfusion to portions of the right and left ventricular myocardium through increased work demands created by unusually brisk pulmonary vasoconstriction from hypoxia. The evidence for this tenet lies in the striking abnormality of left ventricular ejection at angiocardiography and the development of signs of myocardial ischemia on the electrocardiogram.

Pathogenesis of the supravalvular aortic stenosis syndrome

Experiences with 15 cases of the syndrome of supravalvular aortic stenosis are described. Clinical and epidemiologic relationships between the syndrome and infantile hypercalcemia suggest the existence of a common prenatal cause for both disorders.

Hemodynamic Data and Angiographic Findings after Mustard Repair for Complete Transposition of the Great Arteries

Eleven childrern with uncomplicated transposition of the great arteries repaired by the Mustard procedure were studied by atrial pacing, cardiac catheterization, and cineangiography. Nine had persistent postoperative ectopic cardiac rhythms. Atrial pacing in five suggested sinus node damage in four and A-V junctional conduction tissue damage in one.In seven children dye-dilution curves indicated small right-to-left shunts. In all seven superior vena cava cineangiograms showed leaks in the baffle at the superior atrial junction. In the other 10, sequential pressures in the superior vena cava, physiologic right atrium, and inferior vena cava showed no evidence of obstruction to systemic venous return. The three complications related to placement of the atrial baffle seemed to be due in large part to technical difficulties in suturing the baffle in the vulnerable sinus node region.There were no differences in the postoperative hemodynamics between the five patients repaired at a younger age because of severe cyanosis and the five larger children who had been repaired electively. Our results support the aim for early surgical correction in uncomplicated transposition of the great arteries.

Clinical Experience with the Use of Aortic Valve Homografts for Reconstruction of the Pulmonary Artery, Pulmonary Valve, and Outflow Portion of the Right Ventricle

Four patients with ventricular septal defect, infundibular right ventricular obstruction, and associated abnormalities of the pulmonary valve or main pulmonary artery have been treated by a combination of ventricular septal defect closure, infundibular myomectomy, and insertion of an aortic homograft into the pulmonary circulation. Pulmonary valvular competence has been restored in all four cases. A single fatality has resulted, and was associated with bacterial destruction of the homograft. The hemodynamics of pulmonary valvular insufficiency are discussed, and indications for homograft replacement of the pulmonary valve are outlined.

Ventricular septal defect in tetralogy of Fallot.

Abstract A study of 67 surgically unaltered specimens of tetralogy of Fallot at The Johns Hopkins Hospital revealed a group of 28 hearts in which the ventricular septal defect was surrounded entirely by muscular tissue. Since part of this muscle lay in the space between the tricuspid and aortic valve annuli, where it occupied a position taken by the parietal band in the normal heart, it is postulated that this muscle represents an inferior division of the embryonic infundibular septum (parietal band) in these specimens. The superior division of the parietal band forms the anterior margin of the ventricular septal defect and the superior border of the stenotic infundibulum. A similar but more rudimentary inferior portion of the infundibular septum may have been present in another 16 of the 67 specimens, as represented by a fibrous or fibromuscular band separating the aortic and tricuspid valve annuli.

Bundle of His Recordings in Congenital Complete Heart Block

Six children, aged between 3 and 19 years, with congenital complete heart block without associated cardiac disease were studied by His bundle electrography. All were symptomatic with resting ventricular rates over 40 beats/min, and electrocardiograms that showed a normal axis and QRS complex. His bundle to ventricular activation was within the normal range in all and the block was proximal to the bundle of His. Ventricular and atrial pacing did not result in retrograde or antegrade conduction. All the patients were thought to have a lesion proximal to the site where the bundle of His potential was recorded and, therefore, were unlikely to develop symptoms or require treatment.

Double-Lumen Flotation Catheter for Use in Complex Congenital Cardiac Anomalies

The use of the Swan-Ganz flow-directed right heart catheter should be a considerable aid in obtaining diagnostic information in complex congenital heart disease. It is safe and requires little technical expertise. Its use should decrease considerably the amount of radiation exposure to both patient and operator.

Hemodynamics of Aortic Valve Atresia

Hemodynamic data on 12 infants with aortic valve atresia, ranging from 1 to 3 mo of age, are presented. Peripheral arterial oxygen saturations ranged from 19 to 93%. The lowest saturations were found in patients with closed foramen ovales. Increasing age, increasing pulmonary vascular resistance, and a decrease in the pulmonary-to-systemic blood flow ratio all correlated with decreasing systemic arterial oxygen saturation. In seven of the nine infants under 1 mo of age, systemic arterial saturations ranged from 81 to 93%. Thus, we feel that the classification of this lesion as a form of cyanotic congenital heart disease is misleading and prefer to classify aortic valve atresia as an obligatory admixture lesion. Criteria for selection of patients for surgery are discussed and the minimum amount of hemodynamic data needed for such selection is outlined.

An analysis of errors in conventional cardiac catheterization data

Abstract The catheterization data obtained from 77 patients with isolated ventricular septal defect was examined for correlations between pressures and blood flow ratios. Although theoretically expected, and noted by other workers, 1 no significant correlation between left atrial mean pressure and pulmonary-to-systemic flow ratio or the ratio of pulmonary flow-to-predicted cardiac output could be found. An attempt was made to estimate the amount of error in the catheterization data. By comparing pulmonary-to-systemic blood flow ratios over time in 44 cases, a 57 per cent error was found. Comparing the percentage deviation from normal of systemic resistances in the same way for 32 patients, a 39 per cent error was noted. These figures are comparable to a 41 per cent error derived from typical catheterization data and to an average error of 57 per cent obtained by Mesel working with dogs with an artificially created VSD. We can only conclude that there is a need for a more accurate method of measuring blood flows or, until that exists, great care in obtaining and reporting conventional catheterization data.