The outcome of medical treatment of dilated cardiomyopathy in infants and children was reviewed to develop a predictive index for selection of patients likely to benefit from cardiac transplantation. The clinical findings, laboratory investigations, treatment and outcome of 20 patients (Group 1) less than 2 years of age at presentation and 12 patients (Group 2) greater than 2 years of age at onset were compared. Of 20 Group 1 patients, 5 (25%) died. Available autopsies (four patients) showed endocardial fibroelastosis. Of 15 survivors, 10 showed improvement in cardiac status and 5 remained unchanged. Ninety-three percent of survivors had dilated cardiomyopathy consistent with endocardial fibroelastosis by angiocardiography. All 12 Group 2 patients died. In addition to age at presentation and poor outcome, Group 2 differed from Group 1 in having a higher incidence of other family members with cardiomyopathy, more significant rhythm disturbances at presentation and a more rapid course to death. Risk factors of poor outcome in both groups included persistent cardiomegaly and the development of significant arrhythmias by Holter electrocardiographic monitoring. Cardiac transplantation is recommended for children with dilated cardiomyopathy presenting after age 2 years who survive 1 month. Those patients less than 2 years old at presentation whose condition has not improved after 1 year and who have persistent cardiomegaly or complex ventricular arrhythmias may also benefit from transplantation.

Six adolescents with persistent essential hypertension were examined to determine the effect of weight training on their blood pressure and hemodynamics. Five had first completed an endurance training program; one subject trained only by weight lifting. All subjects were reevaluated after 5 +/- 2 months of weight training, and 12 +/- 2 months after cessation of training. Endurance training resulted in an increase in VO2max and decreases in systolic and diastolic blood pressure. After weight training, VO2max had decreased to the level found prior to endurance training, and body weight was significantly increased. Systolic blood pressure after weight training was 17 +/- 4 mm Hg lower than when measured initially (P less than 0.01). Weight training maintained the reduction in diastolic pressure elicited by endurance exercise in those who initially had diastolic hypertension. Cessation of all forms of training resulted in no change in body weight, body fat, or VO2max from the values measured after weight training. Systolic pressure increased significantly with the cessation of training to a value not different from that measured initially. Diastolic pressure also increased after cessation of training, but was still below the initial value. The only significant hemodynamic change found was a reduction in systemic vascular resistance in response to weight training. Weight training in hypertensive adolescents appears to maintain the reductions in blood pressure achieved by endurance training, and may even elicit further reductions in blood pressure.

Twenty-five adolescents (aged 16 ± 1 years) whose blood pressure (BP) was persistently above the 95th percentile for their age and sex were studied before and after 6 ± 1 months of exercise training and again 9 ± 1 months after the cessation of training. Maximal oxygen consumption (V̇O22) increased significantly with training. There was no change in body weight or sum of skinfolds. Both systolic and diastolic BP decreased significantly with training; however, complete BP normalization was not achieved. When the subjects were retested 9 ± 1 months after cessation of training, systolic BP and V̇O22 max had returned to pretraining levels; however, diastolic BP was still below pretraining levels in the subjects who had diastolic hypertension initially. Except in subjects who initially had an elevated cardiac output, no consistent hemodynamic changes were found with training or cessation of training to account for the reductions in BP. The subjects whose resting cardiac outputs were high initially had significantly lower cardiac outputs after training as a result of decreases in both heart rate and stroke volume; however, vascular resistance remained unchanged. Sedentary control subjects with similar BP had no significant change in any of the variables measured over a similar period. These data indicate that moderate endurance exercise training can lower BP in otherwise healthy hypertensive adolescents as an initial therapeutic intervention.

Between 1975 and 1979, a group of 43 patients with d-transposition of the great arteries were diagnosed and underwent Rashkind balloon atrial septostomy at the time of initial catheterization. Thirty-six (88 percent) survived to the time of intraatrial baffle repair, and 31 (72 percent) are long-term survivors, 2 of them now awaiting repair. Palliative operations were performed in nine patients before definitive surgery; four of these patients are long-term survivors. Prostaglandin E1 infusion improved oxygenation and relieved acidosis in four patients. It is concluded that most patients with d-transposition of the great arteries will survive to elective intraatrial baffle repair between 6 and 12 months without surgical palliation in spite of significant hypoxemia. Prostaglandin E1 infusion may be lifesaving and provide sufficient palliation in patients with persistent hypoxemia and acidosis after balloon atrial septostomy.

One-hundred-fourteen hypertensive high school students were evaluated to determine whether a distinctive clinical profile could be identified; 71 normotensive students served as controls. Selected blood chemistry determinations, urinalysis, and chest roentgenograms were done to help rule out secondary causes of hypertension. Left ventricular function was assessed by echocardiography and systolic time intervals. The hemodynamic response to exercise was also evaluated. A significant number of the subjects 14 to 18 years of age with persistent systolic and/or diastolic pressure 1.65 SD above the mean for age and sex showed the following: obesity; elevated serum triglyceride concentration; basilar hypertrophy by electrocardiogram/vectorcardiogram; electromechanical systole and pre-ejection period shorter, and the ratio of the pre-ejection period over the left ventricular ejection time lower, than mean for age and sex as determined by systolic time intervals; volume indices depressed and cardiac contractile functions elevated as determined by echocardiography; higher blood pressure at start of exercise stress test and higher peak systolic and diastolic pressures during test, and a slower return of heart rate to base line level after the test. The above findings should be useful in following the course of a young individual with essential hypertension and may provide a means of evaluating therapeutic intervention.

Specific guidelines for surgical treatment of coarctation of the aorta are presented and the role of hypertension in the malformation is explored.

This is a report on the initial from an ongoing study of blood pressure in ninth to twelfth grade students in the St. Louis metropolitan area. The purpose was to establish standards, to determine the incidence of hypertension, and to examine the relationship of blood pressure to age, weight, height, sex, and race. Subjects with persistent hypertension were to be investigated and checked annually as long as they were in high school. Students in the ninth grade at the beginning of the project were to be screened each year for four years.

HD of 111 hypertensive (H) high school students and their response to exercise were determined by non-invasive techniques and compared with 67 normotensive (N). Left ventricular ejection time (LVET) and pre-ejection period (PEP) were determined from simultaneously recorded electrocardiogram (ECG), phonocardiogram and external carotid pulse tracings, cardiac index (CI), ejection fraction (EF), mean velocity of circumferential fiber shortening (Vcf) and percent shortening of internal diameter (SID) of the left ventricle were measured by echocardiography. Exercise was performed on a bicycle ergometer against 3 work loads ending with a 10 minute rest period. Heart rate (HR) and blood pressure (BP) were monitored during and after the exercise. ECG-VCG showed mild left ventricular hypertrophy in 40% of male H and 19% of female H. PEP was significantly shortened, PEP/LVET decreased in H when compared with N. CI was lower and EF, Vcf and SID higher in the H. The peak HR and BP during exercise in H were significantly higher and their return to baseline levels slower than N. The HD response to exercise was not significantly different between H and N. This study suggested that young H have an identifiable hemodynamic profile.

Evidence is presented from studies of the authors and of other investigators that primary hypertension is more common in children than was previously thought. Ninety-five percent of 131 asymptomatic children with incidental hypertension were considered to have primary hypertension after investigation for possible causes. The definition of hypertension was based on normal ranges of blood pressure for each age and sex. However, the definition of juvenile hypertension is still unsettled as is the question of treatment. Investigation of the effect of prolonged antihypertensive therapy on growth and development is needed. Primary hypertension in the young makes possible the study of the disease at its inception.