Biomedical subjects
Publications and source records attributed to A S Nadas.
Twelve of 13 patients with Beckwith-Wiedemann syndrome were found to have cardiovascular abnormalities: congenital heart disease in 7 of the 12 and isolated cardiomegaly in the others. No specific type of cardiac abnormality predominated.
The course and prognosis of 208 patients with an ascending aorta to pulmonary artery anastomosis is reviewed. Mortality rate during, or within one month, of surgery was 24 per cent (50/208) and late mortality rate, prior to repair, was 10 per cent (21/208). An additional 5 per cent (10/208) died during subsequent intracardiac repair. Congestive heart failure developed in 25 per cent (53/208), pulmonary artery hypertension in 17 per cent (12/72), and pulmonary vascular obstruction in 6 per cent (4/72). An increase in orifice size of the stoma with time was documented in eight patients. Additional subsequent palliative surgery was required in 22 per cent (45/208). Mortality rate was directly related to age at operation and was highest in neonates less than one week of age. In infants with tetralogy of Fallot, a preliminary comparison of mortality rate between palliative surgery and primary repair clearly suggests that the latter is the preferred method of treatment.
Fifty-nine children with congenital asplenia were reviewed for episodes of severe infection. Seven children had isolated asplenia and 52 had asplenia associated with complex congenital heart disease (asplenia syndrome). A control group of eusplenic children with comparable cardiac lesions were assembled and used for comparative statistical analysis. There were 16 instances of documented sepsis among 59 children (27%). In those less than six months of age, the invading organism was usually gram-negative (Escherichia coli or Klebsiella). In children six months of age or older, the infecting organism was usually a pneumococcus or H. influenzae. When those with asplenia syndrome were compared to the control population, the former group had a significantly greater incidence of sepsis. Children with asplenia syndrome who survived the first month of life were at greater risk of dying from sepsis than from their heart disease. It is recommended that prophylactic antibiotics be administered to children with congenital absence of the spleen, commencing at three months of age, to be continued indefinitely.
Infusion of prostaglandin E1 into the main pulmonary artery of an infant with interruption of the aortic arch and a closing ductus arteriosus resulted in dilation of the ductus arteriosus and improved systemic perfusion. Treatment with prostaglandin E1 is recommended for infants with interruption of the aortic arch, critical coarctation of the aorta, and other lesions in which systemic perfusion is limited by a restrictive ductus arteriosus.
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The etiology of chronic congestive heart failure (CHF) after repair of tetralogy of Fallot was determined in 102 patients consecutively catheterized 1 to 12 years postoperatively. Chronic CHF was observed in 36/102 patients. The most prevalent abnormality leading to congestive failure (31/36) was a large residual ventricular septal defect alone or in combination with other lesions. All postoperative patients with pulmonary to systemic flow ratios greater than 2:1 (25/102) had congestive failure and evidence of biventricular dysfunction. Significant tricuspid regurgitation (N = 11) and persistent systemic to pulmonary artery shunts (N = 6) contributed to volume overload and congestive failure in the patients with large residual ventricular septal defect. Isolated severe residual right ventricular outflow tract obstruction was a common cause of chronic CHF. Pulmonary artery hypertension was present in 20/36 patients with CHF. The increased pulmonary pressure was not wholly due to an increased pulmonary flow since 7/20 patients had pulmonary vascular resistance greater than 3 mm Hg/L/min/m2. Our findings indicate that persistent or chronic congestive heart failure in postoperative tetralogy of Fallot patients requires bilateral cardiac catherterization since an identifiable and surgically correctable lesion is nearly always present.
The clinical course of 38 patients with congenital mitral stenosis (MS) is reviewed. Associated cardiac defects were present in 28 patients, including tetralogy of Fallot in five. In all but one of the eight patients with supravalvar mitral ring (SVR), there were concomitant abnormalities of the mitral valve. Delay in the diagnosis of MS was common. Serial cardiac catheterizations and pulmonary pathologic examination indicated that pulmonary vascular obstructive disease develops during childhood. Mitral valve surgery was performed in 19 of 38 patients: valvotomy alone in eight, excision of SVR in five (two ofwhom also had valvotomy) and mitral valve replacemtnt in seven. Additional non-mitral cardiac surgery was performed in 18 patients. Overall surgical mortality was 49%; mortality for surgery on the mitral valve was 26%. Only patients having mitral valve replacement or with isolated SVR which was then resected became asymptomatic and had normal hemodynamics on postoperative catheterization.
Thirty-nine ventilation and perfusion lung studies with xenon 313 were perfomed in 29 patients with D-transposition of the great arteries (DTGA). Fifteen patients had simple DTGA and 14 more complex anatomic features. Inequalitiies of ventilation (V) were present in 23% (9/39) and perfusion (Q) in 44% (17/39) of studies. Six patients with simple DTGA showed abnormal distribution of perfusion, all with increased flow to the right lung. Among the 14 patients with complex DTGA, eight (57%) had abnormal distribution of perfusion. In patients with pulmonary artery band, increased flow was directed toward the left lung, and in those with an aorticopulmonary anastomosis, increased flow was to the side of the shunt. Radionuclide angiograms were performed in 17 patients with DTGA, both before and after surgical repair. In all, an abnormal circulatory pattern was noted. Complete superior vena cava obstruction present in four patients and residual left to right shunt (Qp/qs greater than 1.7/liter) in three was confirmed at cardiac catheterization. Ventilation perfusion studies with xenon-313 and technetium-99m radionuclide angiogram were effective noninvasive techniques in assessing pulmonary function and hemodynamics in patients with DTGA.
The purpose of this study was to determine if previously documented perfomances deficits of patients with cyanotic congenital heart disease are peculiar to centrallly mediated visual tasks or are also manifested on tests of other centrally mediated sensory functions such as hearing. A simple auditory reaction time test was, therefore, performed on 239 patients with congenital heart disease, 43 of whom were cyanotic. Results indicated that (a) there is no significant relationship between level of arterial oxygen saturation and auditory RT. (b) Auditory RT for the group as a whole declines until the mid-teens and then rises, a pattern at variance with that of normal subjects. (c) The audiroty RT of females was significantly higher, i.e., slower, than that of males.
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A total of 161 infants and children, ranging in age from 1 day to 17 years at initial encounter (mean, 3.7 years), was seen over a 30 year period with primary myocardial disease (idiopathic myocarditis, nonobstructive cardiomyopathy, endocardial fibroelastosis, and an anatomically unknown category). These patients were observed from 1 hour to 23 years after initial encounter and cardiac disease has resolved in 27 per cent, resulted in death in 35 per cent, and continues in 38 per cent. The majority were first referred to us with congestive heart failure; all exhibited ST-T changes and cardiomegaly, 67 of 150 had left ventricular hypertrophy, 23 of 151 arrhythmias, and 55 of 153 pulmonary vascular congestion. Initial ventricular depolarization abnormalities were very frequent. Significant clinical predictors of fatal outcome included pulmonary vascular congestion, "northwest" axis deviation, and a cardiac index less than three L./min./M.2. Death occurred during the first year after initial encounter in 44 of 57 who died, and in all 13 with proved myocarditis. Primary myocardial disease is a serious disease of infancy and childhood, resulting in death or residual cardiac disease in three fourths of those affected.