Weidman, William H.

Two cases of severe neurologic symptoms after a Blalock-Taussig anastomosis are presented in detail. The histories of 116 patients with cyanotic congenital heart disease who had such an anastomotic operation at the Mayo Clinic were reviewed. There was a low incidence (less than 1%) of postoperative neurologic complications in patients without evidence of focal dysfunction of the central nervous system prior to operation. The review suggested an increased incidence of complications in those patients with a cerebrovascular abnormality before surgery. It is likely that these complications are related to alterations in the vertebrobasilar arterial system associated with this procedure.

The incidence of premature disability and death from complications of atherosclerosis in the adult American is so high that pediatricians must accept the responsibility of finding the threatened child and, insofar as possible, reducing the future risk. The Council of Rheumatic Fever and Congenital Heart Diseases of the American Heart Association formed a Committee which, during the past 2½ years, has met with a number of different experts in the field. These presentations have been supplemented by a selected review of the literature, and a workshop made up of experts in several related fields selected from the United States and a number of foreign countries to complement the above presentations.

Case-control studies suggest that increased erythrocyte sodium-lithium countertransport may predict increased susceptibility to the development of essential hypertension. To characterize interindividual variation in sodium-lithium countertransport and its relation to blood pressure levels in the general population, we studied 1,475 Caucasians between 5 and 89 years of age (711 males and 764 females) ascertained through 266 households with children in the schools of Rochester, Minnesota. Individuals who were taking antihypertensive agents or combinations of estrogen and progesterone were not included in the sample. A third-order polynomial regression on age accounted for only a small fraction of variability in sodium-lithium countertransport (2.8% in males, p less than 0.001; 2.1% in females, p less than 0.01), whereas a fourth-order regression on age accounted for a large proportion of variability in systolic blood pressure (45.7% in males, p less than 0.001; 52.5% in females, p less than 0.001) and diastolic blood pressure (39.8% in males, p less than 0.001; 33.0% in females, p less than 0.001). Mean sodium-lithium countertransport was higher in males than females at all ages; but the rank order of male and female means for systolic and diastolic blood pressure was age dependent. Positively skewed distributions for age-, height-, and weight-adjusted sodium-lithium countertransport in male and female cohorts between 5-19.9, 20-49.9, and 50-89.9 years of age were explained significantly better by postulating a mixture of two partially overlapping sodium-lithium countertransport distributions rather than a single normal distribution (p less than 0.01). Among men in the 20-49.9-year-old cohort, adjusted sodium-lithium countertransport values in the upper distribution were associated with higher systolic and diastolic blood pressure (mean +/- SD) than values in the lower distribution (for systolic blood pressure: 115 +/- 11 vs. 111 +/- 11 mm Hg, p less than 0.07; for diastolic blood pressure: 71.2 +/- 8.0 vs. 68.4 +/- 8.6 mm Hg, p less than 0.08). Among females in the 50-89.9-year-old cohort, adjusted sodium-lithium countertransport values in the upper distribution were associated with significantly greater diastolic blood pressure than values in the lower distribution (77 +/- 10 vs. 70 +/- 9 mm Hg, p less than 0.03).(ABSTRACT TRUNCATED AT 400 WORDS)

The results of complete repair of double-outlet right ventricle with pulmonic stenosis in 22 Mayo Clinic patients were analyzed. The overall mortality rate was 32%. Since 1960, the mortality rate has been reduced to 16%. Anomalies of the distribution of the right coronary artery, a second ventricular septal defect of the atrioventricular canal type, and significant residual pulmonic stenosis were associated with a higher mortality rate. The difficulties in the diagnosis of this uncommon condition are emphasized. Complete repair is best carried out after the patient is 4 years old. A systemic-to-pulmonary arterial shunt is the procedure of choice for those who are younger than this. In the occasional patient with anomalous distribution of the right coronary artery which precludes adequate relief of the pulmonic stenosis, use of an aortic homograft from the right ventricle to the pulmonary artery should be considered.

The results of complete repair of double-outlet right ventricle without pulmonic stenosis in 18 Mayo Clinic patients are analyzed; the over-all operative mortality rate was 22%. Pulmonary vascular obstructive disease and a second ventricular septal defect of the atrioventricular canal type were associated with a higher mortality rate. The difficulties in the diagnosis of this uncommon condition are emphasized. Patients with double-outlet right ventricle without pulmonic stenosis should have complete repair early in life before the onset of severe pulmonary vascular obstructive disease-the only specific contraindication to operation. Pulmonary artery banding may be preferable as a first-stage palliative procedure in infants less than six months old who do not respond to medical therapy.

The status of 58 patients, 3 to 57 years of age, with ventricular septal defect and a marked increase in pulmonary vascular resistance (pulmonary obstructive disease) was determined 5 years or longer after diagnostic catheterization. Forty-six (80%) were alive 5 years after that study. The probability of living 5 years was 95% for patients aged 10 through 19 years and 56% for those 20 years of age or older. The dissimilarity of actuarial curves between these two groups is consistent with the hypothesis that pulmonary vascular obstructive disease develops under age 20. Calculated pulmonary resistance increased with age of patient on admission to the study, suggesting its progressive nature. Hemoptysis was rare under age 20, and its presence over this age indicated a poor prognosis. The most common causes of death were "sudden" or "unknown." Most surviving patients were gainfully employed or attending school without severe symptoms.

Two cases in which large intramural ventricular cardiac fibromas were successfully removed from children are reported. In one the fibroma was principally septal, and in the other it lay in the free wall of the left ventricle. These are the second and third such operations reported. A review of these two cases plus 34 other reported cases of cardiac fibroma indicates that a primary mural tumor of the heart should be suspected in patients, particularly children, with unexplained cardiac failure, unexplained cardiac dysrhythmia, intracardiac calcifications, irregular shadows on roentgenograms, or unexplained newly developing cardiac symptoms and murmurs. Angiocardiography is the best way of confirming the diagnosis. In view of the potential hazard of these tumors, their surgical removal should be undertaken, and this experience indicates that even very large and extensive intramural ventricular tumors may be removed successfully.

Seventeen of 44 patients undergoing operation for complete correction of transposition of the great arteries survived operation and were dismissed to return home. Three late deaths followed. Mortality remains high among infants less than one year old and among patients of all ages who have severely elevated pulmonary vascular resistance. Nine of the last 13 patients, in whom repair was accomplished by the Mustard technique, have survived. Late results in all surviving patients have generally been excellent.

Records of 273 cases of ventricular septal defect from five participating institutions were studied. Insignificant hemodynamic disturbance (Roger's) was found in 11 per cent, moderate to large left-to-right shunts in 55 per cent, and severe pulmonary vascular disease ("Eisenmenger's") in 8 per cent. Because of insufficient data, 26 per cent of the cases were not classified hemodynamically. Thus, a spectrum of severity ranging from mild to severe was noted. There was evidence of congestive heart failure in infancy in 78 cases and a significant mortality occurred in this group, whether treatment was medical (mortality, 27 per cent) or surgical (mortality, 46 per cent). Six patients had hemodynamic evidence of severe progressive pulmonary vascular disease, three when they were less than 8 years old. Correlations of hemodynamic severity with clinical symptoms, roentgenographic findings, and electrocardiographic changes were reasonably good at the extreme ends of the severity spectrum but only fair in the middle range. Over-all mortality was high (28 per cent) for the 163 patients treated surgically, but mortality was also high (18 per cent) for the 110 patients treated medically. Ten of the 20 deaths in the medical group occurred in patients who had had congestive heart failure in infancy. Further study of the natural history of ventricular septal defect is clearly indicated for establishment of improved criteria for selection of cases for operation.

A total of 131 patients with the partial form of common atrioventricular canal were operated on between March, 1955 and January, 1964. The over-all hospital mortality rate was 3.8%; no deaths occurred in patients who had no or only mild incompetence of the mitral valve. Permanent heart block has not occurred in the last 93 cases. Results have been good in general, although there is evidence of residual mitral valve incompetence in many patients. In the few who had severe incompetence at the mitral valve before repair, the results have been considerably less satisfactory than in the group as a whole; therefore, further work is needed on this aspect of the problem.