Sylvia P. Griffiths

Spontaneous complete closure of a congenital coronary artery fistula

The first documentation is reported of spontaneous closure of a coronary artery to right ventricle fistula that was demonstrated initially in a 14 month old boy. Over a 4 year period after diagnosis, the characteristic continuous murmur gradually disappeared. When the patient was 5 1/2 years of age, selective coronary arteriography showed normal coronary vessels and circulation. Six other cases of coronary fistula observed during the past 10 years are also reviewed. This study supports the rationale for clinical follow-up rather than obligatory surgical intervention in asymptomatic patients with a small shunt who have no evidence of myocardial dysfunction.

Acute rheumatic fever in New York City (1969 to 1988): A comparative study of two decades

One hundred fifteen attacks of acute rheumatic fever were observed during the past two decades (1969 to 1988) at the Columbia-Presbyterian Medical Center; 104 were initial attacks and 11 were recurrences. The maximum number of cases (18) occurred during the first year of this period, 1969; thereafter the annual incidence decreased until a resurgence was apparent in 1985 to 1986, with 25 cases. There were no differences in the frequency of major manifestations nor the severity of carditis in the 1970s compared with the 1980s. Polyarthritis alone was present in 51 cases, carditis alone in 31, and combined carditis and polyarthritis in 28; chorea was diagnosed in 5. Congestive heart failure occurred in 17 attacks of carditis, including one death from fulminant disease in 1982. In contrast to recent reports, the majority of the affected population was urban, low income, and from crowded communities: more than half were Hispanic, predominantly from Dominican Republic families. The prevalence of acute rheumatic fever underscores the need for early detection and treatment of streptococcal pharyngitis. The observation that 9.5% of the attacks were recurrent reflects failure to comply with antimicrobial prophylaxis and reaffirms the need for more effective secondary prevention programs.

Aortic Origin of the Right Pulmonary Artery

The clinical course and pathologic findings in three cases of anomalous origin of the right pulmonary artery from the ascending aorta are described. The malformation is characterized by (1) absent right branch of the pulmonary artery, (2) a single large vessel arising from the ascending aorta, entering the hilus of the lung and constituting its sole arterial blood supply, and (3) normal size and structure of the right lung. A review of the literature indicates the rarity of this lesion as an isolated anomaly; patent ductus arteriosus is the most frequently described additional defect. Histologic examination of the right and left lungs reveals bilateral and symmetrical pulmonary vascular changes, despite the difference in their arterial supply. Some pathophysiologic observations based on the necropsy findings in these cases are discussed. Early diagnosis and surgical correction are imperative, since this malformation may lead to irreversible congestive heart failure and death in infancy. Surgical repair may be accomplished by transplanting the anomalous right pulmonary artery from the aorta to the main pulmonary trunk.

Muscular ventricular septal defects repaired with left ventriculotomy

Between 1974 and 1979 nine patients, aged 10 months to 4 years, underwent left ventriculotomy for closure of single or multiple defects in the muscular ventricular septum. The vertical incision paralleled the anterior descending branch of the left coronary artery near the apex of the left ventricle and ranged from 2.5 to 3.5 cm in length. Four patients also had a right ventriculotomy with closure of a high perimembranous ventricular defect in two. Serial electrocardiograms indicated changes of myocardial ischemia or necrosis. Left bundle branch block did not develop in any patient. Three patients died in the early postoperative period. The six surviving patients are living and well 2 to 7 years later. There is apparent complete closure of the ventricular defects, which was documented by cardiac catheterization in four cases. Two patients had cardiomegaly and left ventricular dysfunction as assessed with echocardiographic and angiographic study, whereas four displayed good cardiac function. In three of the latter patients, cardioplegia or deep hypothermia techniques were utilized intraoperatively. The observations indicate that left ventriculotomy of limited size is an acceptable approach to the difficult problem of repair of muscular ventricular defects but may involve some risk of compromise of the coronary circulation.

Congenital complete heart block: A classification and experience with 18 patients

Abstract The clinical course of 18 patients with congenital complete heart block, who ranged in age up to 32 years, has been reviewed. In 17, cardiovascular diagnostic studies or necropsy were performed. The frequency of ventricular inversion (congenitally corrected transposition of the great vessels) and associated cardiovascular malformations prompted a classification based on embryologic development of the heart. Ventricular inversion occurred in 8 patients, and normal bulboventricular loop development was present in 9. Cardiovascular malformations were noted in 13 of the 17 patients studied anatomically. Prognosis for well-being and long term survival is primarily related to (1) the presence or absence of cardiovascular defects and (2) the stability of the ventricular pacemaker. The complications of congestive heart failure or Stokes-Adams attacks occurred in 9 patients, 8 of whom had associated cardiovascular defects with a stable ventricular pacemaker. The only patient without a cardiovascular anomaly who experienced syncope had an unstable ventricular focus. Cardiovascular surgery is well tolerated in patients with congenital complete heart block. Artificial cardiac pacing is valuable in the supportive management of symptomatic infants with congestive heart failure or Stokes-Adams attacks. Its application in 2 infants, in whom a cardiac pacemaker was implanted, is described.

Scimitar vein draining to the left atrium and a historical review of the scimitar syndrome

Scimitar vein draining to the left atrium is the rarest of the pulmonary venolobar anomalies with less than a dozen reported cases. A case is reported of a patient whose plain film radiographic findings showed a typical scimitar vein but whose echocardiography showed normal pulmonary venous drainage. Cardiac catheterization confirmed drainage of the scimitar vein to the left atrium and systemic arterial supply which was embolized. The case described is used to review the history of “scimitar syndrome” with reflections on the significant contributions of Halasz and colleagues, who in 1956 helped define the anatomy, and Neill and colleagues, who in 1960 described the familial occurrence and clinical spectrum of the condition.

Implanted cardiac pacemakers in children: A report of their application in five patients☆

Abstract Five children, ranging in age from 7 months to 15 years, had cardiac pacemakers implanted between April 1963 and August 1965. A Chardack-Greatbatch unit was used in 4 patients with postsurgical complete heart block; a Cordis Atricor Junior unit was employed in 1 infant with congenital complete heart block. Pacemaker performance has been good, and each patient is clinically improved. Problems in pacemaker function have included: (1) increased myocardial stimulation threshold, (2) disturbance of the rate-control mechanism, (3) break in wire insulation and (4) generator failure. Pacing has been satisfactorily restored in each of these instances. Indications for pacemaker implantation in the pediatric age group depend upon the etiology of complete heart block. Permanent artificial pacing is recommended in all cases of postsurgical complete heart block. In congenital complete heart block, pacemaker implantation should be employed if symptoms of syncope or unremitting congestive heart failure develop.

Postoperative evaluation of mitral valve function in ostium primum defect with cleft mitral valve (partial form of atrioventricular canal).

Postoperative cardiovascular studies were carried out in eight patients with ostium primum defect and cleft mitral valve. Apical systolic murmurs were present in all patients, but in seven of them no mitral regurgitation was detected by pulmonary wedge pressure and left ventricular angiography. Surgical repair of the cleft anterior leaflet without resection of subjacent chordae tendineae, as performed in these patients, usually results in a competent mitral valve. Postoperative angiograms showed persistence of the characteristic preoperative deformity of the medial border of the left ventricular outflow tract especially in systole. As illustrated in one of these patients, subaortic obstruction occasionally complicates abnormalities of the atrioventricular valves.

Trends in cardiovascular surgery (1961 to 1977): Review of the new york city and state experience

Abstract Statistical data on surgery for congenital and acquired heart disease in New York City in the 17 years from 1961 through 1977 and in New York State in 1977 document trends in volume and types of operations. The ratio of open heart surgery for congenital versus acquired heart disease of 2:1 in 1961 was reversed to 1:6 by 1977 as the number of procedures for valvular and coronary heart disease increased. The case load for surgery for congenital heart disease using the open heart technique gradually increased until 1974 and then decreased. The number of closed heart operations was relatively constant. Observations suggest a finite number of open and closed heart operations for congenital cardiac malformations; the total of 1,381 cardiac operations in New York State in 1977 may continue to diminish if the decreasing birth rate persists. The number of operations for valvular heart disease has stabilized, but the number of operations for coronary artery disease has increased 7-fold since 1970. In 1977, coronary arterial bypass grafting in New York State accounted for 63 percent of operations for acquired heart disease (6,407) and the mortality rate was less than 3 percent.

Ventricular septal defect: Survival in adult life

Abstract 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.