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Clinical and cardiac catheterization data were collected from 39 infants with coarctation of the aorta and ventricular septal defect, 31 of whom were initially managed only by surgical repair of coarctation. Data were analyzed to determine mortality, morbidity, outcome and factors that might predict survival or the need for septal defect closure.Of the eight patients who did not require surgical treatment before 3 months of age, seven underwent coarctation repair alone at a mean age of 2.3 years. Of the 23 infants managed with coarctation repair alone, before age 3 months, 9 needed no additional surgical treatment and 6 required early and 8 required late repair of the ventricular septal defect. Seven infants underwent coarctation repair and simultaneous pulmonary artery bandnig and one eventually required debanding after spontaneous closure of the septal defect. The overall mortality rate in this series was 10.3% (mean follow-up time 5.7 years). Of 39 infants, 16 (41%) never required a second operation for ventricular septal defect closure.For patients who had only coarclation or coarctation repair with pulmonary artery banding at 1 cm/m2) on the basis of defect size at operative repair or echocardiographic or angiographic assessment. Defect size did not necessarily correlate with the need for operative repair. Stepwise multiple regression analysis revealed that increased right to left ventricular peak systolic pressure (p = 0.004) and decreased systemic venous oxygen content (p = 0.028) were significantly predictive of the eventual need for ventricular septal defect repair.Thus, most infants with coarctation of the aorta and ventricular septal defect do not require pulmonary artery banding or open heart closure of the ventricular septal defect at the time of coarctation repair. A significant number of infants will not require a second operation.

Infants with pulmonary atresia and intact ventricular septum (PA-IVS) usually require urgent surgical intervention. Thirty patients with this anomaly, seen at the Columbia-Presbyterian Medical Center between 1962 and 1978, had palliative operations, 26 within the first 3 days of life. Six underwent a closed pulmonary valvotomy alone, with no survivors; six had only a systemic–pulmonary artery shunt, with three early survivors. Because of this experience, 17 had a combined procedure of valvotomy and shunt, with 14 early survivors. One patient recently underwent a definitive right ventricular outflow patch procedure with cardiopulmonary bypass. Eight patients subsequently have had corrective open-heart procedures, with five patients surviving from 2 to 10 years. A unicusp aortic homograft was used for repair in five and a Hancock valved conduit in three. Four patients are presently awaiting operation. We conclude that the initial surgical management of these critically ill infants must not only increase pulmonary blood flow but in addition provide an opportunity for right ventricular growth. Thus we continue to advocate the combined procedure of a valvotomy plus a shunt to provide adequate palliation. Repeat catheterization should be performed within a year to confirm the adequacy of the valvotomy, since this is essential to maximal right ventricular enlargement and to allow for definitive correction at a later date.

A remarkable patient is described, a child who has survived until the age of 7 years with hypoplastic left heart syndrome (mitral and aortic atresia) without surgical intervention. The child has led an active, normal life and, aside from minimal cyanosis, has remained asymptomatic. The unique clinical course for this patient is the result of a number of favorable hemodynamic factors that have not been previously reported in an individual patient with hypoplastic left heart syndrome and intact ventricular septum: 1) widely patent ductus arteriosus, 2) adequate retrograde coronary flow, 3) unrestricted pulmonary venous return, and 4) absence of significant vascular obstructive disease. This documentation of longterm survival in a child without surgical treatment for mitral and aortic atresia suggests that successful early palliative treatment for infants with this syndrome could also result in a favorable prognosis.

A three and one-half month old infant with corrected transposition of the great vessels, pulmonary atresia, and intact ventricular septum is presented. Cardiac catheterization and angiocardiography disclosed a small blind right-sided pulmonary (anatomic left) ventricle with systolic pressure exceeding systemic levels. The chamber communicates with the coronary arterial system via transmyocardial sinusoids. To our knowledge, this is the first report of this syndrome complex diagnosed during life.

The clinical course of thirty-five patients, seventeen years of age and over, with ventricular septal defect observed during the past thirty years is reviewed. Thirty-one patients were studied by right heart catheterization since 1946; ten were recatheterized five to thirteen years after the initial investigation. The remaining four were examined at necropsy before cardiac catheterization technics were available. A wide spectrum of hemodynamic findings was noted at all ages in adult life. The two largest groups were those with normal pulmonary artery pressure (group i) and those with pulmonary artery pressure at approximately the systemic level (group iv). In the first group the patients were essentially asymptomatic and showed no change in pulmonary artery pressure with advancing age. In contrast, most of the patients with severe pulmonary hypertension were symptomatic in infancy or early childhood and probably always had elevated pulmonary artery pressure. They manifested progressive pulmonary vascular disease with the development of cyanosis and ultimately right-sided heart failure. Bacterial endocarditis was the cause of death in three of the four patients examined at necropsy before 1946. This complication was infrequent among the patients in recent years and was never a terminal event. In this series only five patients were forty years of age or over, the oldest was forty-nine years of age. The explanation for lack of patients beyond fifty years is not clear, but shortened survival time is suggested.

We have presented a brief review of some congenital anomalies of the pulmonary arterial vasculature, including aneurysms, coarctations and anomalous origins. Knowledge of these malformations is important to radiologists, since many of the surgically correctible lesions can be suspected from the plain chest roentgenograms and confirmed angiocardiographically.