Efficacy and safety of lovastatin in adolescent males with heterozygous familial hypercholesterolemia: a randomized controlled trial.

Context: Heterozygous familial hypercholesterolemia (HeFH) is a common disorder associated with early coronary artery disease, especially in men. The age at which drug therapy should be started is still controversial, as is the use of 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors (statins).

Objective: To assess the lipid-lowering efficacy, biochemical safety, and effect on growth and sexual development of lovastatin in adolescent boys with HeFH.

Congenital heart disease associated with sporadic Kallmann syndrome.

A 17-year-old boy with Kallmann syndrome had complex congenital heart disease that included double-outlet right ventricle, d-mal-position of the great arteries, right aortic arch, and hypoplastic main pulmonary artery. He had neurosensory hearing loss and mental retardation. The 7 previously reported patients with Kallmann syndrome and cardiac abnormalities were short with height > or = 2 standard deviations below the mean for age (5/7), lacked a family history of Kallmann syndrome (6/6), and were mentally retarded (4/4).

Doppler echocardiographic assessment of filling and emptying parameters and ambulatory blood pressure measurements in normotensives and hypertensives.

To evaluate the relationship between office and ambulatory BP measurements and filling and emptying parameters, 15 hypertensive and 15 control subjects underwent both 24 hour ambulatory BP monitoring and Doppler echocardiography. No patient received antihypertensive medication for 3-4 weeks, had echocardiographic left ventricular hypertrophy (greater than or equal to 12 mm) or a diastolic BP greater than 100 mm Hg. The time from R wave on the electrocardiogram to onset of ejection was prolonged in hypertensives (P less than 0.

Family history of myocardial infarction and hemodynamic responses to exercise in young black boys.

The influence of family history of coronary artery disease on children's hemodynamic responses to exercise was examined with 25 black boys aged 7 to 10 years. Blood pressure, heart rate, cardiac output, stroke volume, and total peripheral resistance were evaluated during preexercise, peak exercise, and recovery stages. Children with a family history of CAD exhibited greater systolic blood pressure and total peripheral resistance during preexercise and peak exercise stages than did those without a family history of coronary artery disease.

Racial differences in hemodynamic responses to the cold face stimulus in children and adults.

The prevalence of essential hypertension is higher among blacks than whites. One reason for this difference may be that blacks are more reactive to stressors that produce vasoconstrictive responses. Two studies were conducted to test this hypothesis, one with 10- to 14-year-old males (20 whites, 20 blacks) and one with young adult males (10 whites, 10 blacks).

Facial dysmorphologic and skeletal cephalometric findings associated with conotruncal cardiac anomalies. off.

Anomalous conotruncal cardiac morphology and facial dysmorphology have been associated with neural crest-pharyngeal arch abnormalities. To assess these associations, 20 patients 3 to 18 years old with tetralogy of Fallot (TOF) or persistent truncus arteriosus (PTA) were evaluated by cardiologic, facial dysmorphic, and cephalometric criteria. The average number of facial abnormalities of neural crest derivation was two, while pharyngeal arch derivative abnormalities were observed with an average of five defects per subject.

Cardiac and peripheral vascular responses to adrenoceptor stimulation and blockade after cardiac transplantation.

A denervated heart coupled to a periphery previously exposed to high catecholamine levels provides a unique model to study adrenoceptor physiology. Six orthotopic transplant patients (1.3 +/- 0.

Exercise-induced differences in cardiac output, blood pressure, and systemic vascular resistance in a healthy biracial population of 10-year-old boys.

Previous studies have documented systolic blood pressure differences in response to dynamic exercise when blacks were compared with whites. This study was performed to validate these data and to determine if the cardiac output and systemic vascular resistance responses to exercise are different in a biracial population of 10-year-old boys. Nineteen blacks and 31 whites underwent maximum supine graded exercise tests with Doppler determination of cardiac output and measurement of systolic and diastolic blood pressure and systemic vascular resistance.

Left ventricular filling in sickle cell anemia.

M-mode echocardiography was performed on 11 normal black subjects and 38 patients with sickle cell anemia while they were at rest to evaluate their left ventricular (LV) systolic and diastolic function. The patients with sickle cell anemia were also evaluated by radionuclide exercise tests and, based on their ejection fraction (EF) response, were separated into 2 groups: a group with a normal EF response to exercise (73 +/- 9%, mean +/- standard deviation) and a group with an abnormal EF response to exercise (53 +/- 9%). Computer-assisted analysis of the M-mode echocardiograms identified abnormalities of diastolic function (impaired left ventricular filling) in patients with sickle cell anemia compared with the normal subjects.

Computer assisted echocardiographic assessment of left ventricular function before and after anatomical correction of transposition of the great arteries.

Left ventricular function before and after anatomical correction of transposition of the great arteries was assessed by computer assisted analysis of 78 echocardiographs from 27 patients obtained one year before to five years after operation. Sixteen patients had simple transposition, and 11 had complex transposition with additional large ventricular septal defect. Immediately after correction mean shortening fraction fell from 46(9)% to 33(8)%.

Assessment of coronary and aortic anastomoses after anatomic correction of transposition of the great arteries.

Anatomic correction of transposition of the great arteries always entails circumferential anastomoses of the aorta and coronary arteries. Long-term success of this procedure is predicted on adequate growth of these anastomotic sites. To assess the size of these arteries, we performed one or two cardiac catheterization on 25 children from 1 to 53 months (mean 18.

Left ventricular contractility and contractile reserve in humans after cardiac transplantation.

Limited data are available concerning left ventricular contractility and contractile reserve in the chronically denervated, transplanted human heart. This is primarily because of the inability of traditional tests of left ventricular performance to distinguish changes in contractility from alterations in ventricular loading conditions. In this study, load-independent end-systolic indexes of left ventricular contractility were measured by echocardiography and calibrated carotid pulse tracings in 10 patients who had undergone orthotopic cardiac transplant (age 48 +/- 4 years; interval from operation to study 1.

Electrocardiographic abnormalities in pediatric neuromuscular disease: a review.

Cardiac abnormalities, often heralded by electrocardiographic alterations, at times may become a serious problem in patients with neuromuscular disorders and occasionally lead to death. Electrocardiographic monitoring can identify patients whose conduction defects will benefit from the use of demand pacemakers.

Influence of two-stage anatomic correction on size and distensibility of the anatomic pulmonary/functional aortic root in patients with simple transposition of the great arteries.

To evaluate the results of the two-stage anatomic correction of simple transposition of the great arteries the size, distensibility, and histologic characteristics of the anatomic pulmonary root, which arises from the anatomic left ventricle and which we termed the functional aortic root after anatomic correction, were determined in seven patients before and twice after anatomic correction (mean 43 and 671 days) and the results were compared with those in normal control subjects. The diameter of the systolic sinus of the anatomic pulmonary root increased after banding on the average to 140% of normal, whereas the diameter of the diastolic sinus of the functional aortic root increased after anatomic correction on the average to 150% of normal. Diameters of both the systolic and diastolic sinuses of the functional aortic root remained 30% to 55% larger than normal after anatomic correction.

Assessment of left ventricular contractile state after anatomic correction of transposition of the great arteries.

When compared with intra-atrial baffle repairs for D-transposition of the great arteries (TGA), anatomic correction offers the theoretic advantage that it results in the use of the left ventricle (LV) as the systemic ventricle. Although the long-term success of this procedure depends on the function of the LV, little is known regarding LV postoperative contractile state. The LV end-systolic pressure-dimension and wall stress-shortening (% delta D) relationships, sensitive indexes of contractility, were studied during methoxamine-induced increases in afterload in eight patients with TGA and intact ventricular septa and in four patients with TGA and associated lesions.

Catheter evaluation of left ventricular shape and function 1 or more years after anatomic correction of transposition of the great arteries.

Twenty-eight children were reinvestigated by cardiac catheterization and angiography greater than 1 year after anatomic correction of transposition of the great arteries (TGA). Seventeen patients with simple TGA underwent banding of the pulmonary trunk plus or minus systemic to pulmonary artery shunt to prepare the left ventricle for anatomic correction. In addition to TGA, 10 of the remaining 11 patients had a large ventricular septal defect and 1 had an aorticopulmonary window.

Cardiac rhythm and conduction before and after anatomic correction of transposition of the great arteries.

To assess pre- and postoperative arrhythmias associated with anatomic correction of transposition of the great arteries, 34 patients had 145 standard electrocardiograms (ECGs) and 24 of these patients had 46 24-hour ECGs from 11 months before to 54 months after anatomic repair. Twenty-two patients underwent balloon atrial septostomy and 7 surgical atrial septectomy before the initial 24-hour ECG. Anatomic correction included repair of a large ventricular septal defect in 16 patients and an aortopulmonary window in 1 patient.

Fate of dynamic left ventricular outflow tract obstruction after anatomic correction of transposition of the great arteries.

Fourteen patients undergoing successful anatomic correction for transposition of the great arteries had subpulmonary gradients of 20 to 120 mm Hg (mean 40) across the left ventricular outflow tract before surgery. Ten patients had an intact ventricular septum, and four had an additional ventricular septal defect. In one patient obstruction was due to ballooning of the septal leaflet of the tricuspid valve through the ventricular septal defect.