We present data on presentation, treatment, and follow-up of 65 pediatric patients with primary hypertension treated over the past 12 years, including initial anthropometric data, pharmacologic treatment, time to control for both systolic and diastolic blood pressure (SBP/DBP), and maintenance of control over time. Data was normalized to standard deviation scores (SDS) for mathematical analysis, and antihypertensive medication dosages were converted to dosage equivalents for a single member of each antihypertensive class. We used multiple regression analysis and Kaplan- Meier survival curves to determine the time to control, medication, and dose effectiveness. Patients were seen for an average of seven visits over 25 months. Initial BPs averaged 134/71 mmHg (2.1/0.6 SDS). Patients were taller, heavier, and had higher body mass index than average for age and sex. By the fourth visit, SBP was <90th percentile in 79%. Ninety percent could be controlled, although 32 lost control at some point (at least 16 due to noncompliance). At the last visit, 46 were controlled, and 5/8 patients off medication remained normotensive. Only angiotensin-converting enzyme inhibitors and beta-blockers demonstrated significant association with BP control. This is the first study to document the time to control of BP, and it can serve as an initial standard for quality assessment.

Monosomy 21 is a rare chromosomal abnormality, with only nine cases reported in the literature. Affected infants display multiple dysmorphic features as well as skeletal, ocular, pulmonary, cardiac, renal, and genitourinary abnormalities. All monosomies are lethal except monosomy 21, but not all monosomy 21 fetuses survive to term. This report describes the echocardiographic findings and the congenital heart defects associated with the third case of molecularly confirmed full monosomy 21 in the literature. The cardiac defects included a mildly hypoplastic and hypertrophied left ventricle, a large ostium secundum atrial septal defect, a small anterior muscular ventricular septal defect, an interrupted inferior vena cava with azygos continuation, a parachute mitral valve, a bicuspid aortic valve, and a tortuous descending aorta. It also is the first description of a left pulmonary artery aneurysm and decreased left ventricular function as a component in the spectrum of defects found in full monosomy 21.

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We hypothesized that increased aortic forward pressure wave amplitude (Pf), which is determined by characteristic impedance (Zc) in the proximal aorta, is the primary hemodynamic determinant of obesity-associated higher left ventricular (LV) mass in adolescents. Aortic pulsatile hemodynamics were measured non-invasively in sixty healthy adolescents (age 14–19 yrs; 42% male; 50% black) by sequential recordings of pulse waveforms via tonometry, brachial BP (BP), and pulsed Doppler and diameter of the LV outflow tract using 2D echocardiography. Adolescents who were overweight/obese (n=23; age 16.0 ± 0.3 yrs; body mass index, BMI ≥85th percentile) had higher LV mass index (LVMI), brachial and carotid systolic BP and pulse pressure, normalized Zc and Pf compared with adolescents with healthy-weight (n=37; 16.7 ± 0.3 yrs; BMI <85th percentile, all P<0.01). In contrast, there was no difference in mean or diastolic BP, carotid-femoral pulse wave velocity, carotid augmentation index or aortic backward wave amplitude (all P>0.05). Stepwise multiple linear regression analysis that included age, sex, race, normalized Zc and brachial systolic BP revealed that BMI (B ± SE; 0.49 ± 0.20, P=0.02, R2=0.26), aortic Pf (0.22 ± 0.07; P<0.02, R2 change=0.11) and cardiac output (2.82 ± 1.02, P<0.01; R2 change=0.08) were significant correlates of LVMI (Total R2=0.44, P<0.01). These findings suggest that higher Pf is a major aortic hemodynamic determinant of increased LV mass in adolescents with elevated adiposity. Improper matching between aortic diameter and pulsatile flow during early systole potentially contributes to the early development of LV hypertrophy in childhood obesity.

Adult studies suggest a better functional outcome after aortic valve replacement with a pulmonary autograft compared with mechanical or homograft valves. Little is known about functional results after Ross surgery in growing children. This study reports formal exercise stress echocardiographic data from 26 pediatric Ross patients. A retrospective cohort study analyzed stress echocardiographic data of patients who underwent Ross surgery as a child (<17 years old). All patients were operated by a single surgeon and underwent a Bruce protocol stress echocardiogram on the treadmill. Twenty-six patients (4 girls) were 9.3 ± 5.0 years at surgery and 14.9 ± 3.5 years (range 6.6-19.7 years) at follow-up. Mean follow-up was 5.4 ± 3.7 years (median 4.2). All were asymptomatic. The exercise time was normal in 87% of cases at 12.8 ± 2.5 min. On stress echocardiography, the mean right-ventricular outflow tract (RVOT) gradient increased from 38 ± 22 mmHg at rest to 82 ± 33 mmHg after exercise, but this did not correlate with exercise times. Stress echocardiography is useful in evaluating patients after childhood Ross surgery for aortic valve disease. In this pediatric cohort, most patients achieved normal exercise capacity. The presence of mild or moderate RVOT obstruction had no significant impact on exercise capacity.

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We hypothesized that higher volume of physical activity (PA) in the past year would be associated with enhanced left ventricular (LV) diastolic function, exercise capacity and lower wave reflection in adolescents independent of time in sedentary activities (e.g., television viewing, etc.). Sixty healthy adolescents (age 16.4 ± 0.2 yrs) were studied. Subjects with higher (HPA, n=30, 25.7 ± 2.3 hrs/week) vs. lower (LPA, n=30, 6.9 ± 0.7 hrs/week) time spent performing PA in the past 12 months (Youth Adolescent Activity Questionnaire) demonstrated greater LV diastolic E/A (echo Pulsed doppler: 2.20 ± 0.07 vs. 1.90 ± 0.06, P<0.01), e′ (echo Tissue doppler: 0.19 ± 0.1 vs. 0.17 ± 0.1 m/s, P=0.03), VO2peak (40 ± 2 vs. 31 ± 2 ml/kg/min, p<0.01), exercise test duration (11.9 ± 0.5 vs.10.1 ± 0.4 min, P<0.01) and lower wave reflection (carotid aug index, −11.83 ± 2.6% vs. −4.77 ± 2.2%, P<0.05) compared with LPA. In the entire cohort, PA was associated with mitral E/A (r=0.31, P<0.02), e′ (r=0.30, P=0.05), carotid AI (r=−0.29, P<0.05), VO2peak (r=0.27, P<0.05) and exercise test duration (r=0.39, P<0.01). All relations except e′ remained significant after adjustment for time spent performing sedentary activities. Higher volume of PA in the past year is associated with enhanced LV diastolic function and exercise tolerance and lower wave reflection in healthy adolescents independent of time performing sedentary activities.