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This retrospective case-control study investigated cardiac dimensions and ventricular function in female adolescents with anorexia nervosa (AN) compared with controls.Echocardiographic measurements of left ventricular (LV) dimensions, LV mass index, left atrial size and cardiac index were made. Detailed measures of systolic and diastolic ventricular function were made including tissue Doppler imaging. Patients were stratified by body mass index ≤10th percentile (AN ≤ 10th) and10th percentile (AN 10th).Ninety-five AN patients and 58 controls were included. AN and AN ≤ 10th groups had reduced LV dimensions, LV mass index, left atrial size and cardiac index compared with controls. There were no differences between groups in measures of systolic function. Measures of diastolic tissue Doppler imaging were decreased in AN and AN ≤ 10th. No differences in echocardiographic measurements existed between controls and AN 10th.Female adolescents with AN have preserved systolic function and abnormalities of diastolic ventricular function. AN ≤ 10th may be a higher risk group.

Marfan syndrome (MFS) is an autosomal-dominant disorder of connective tissue caused by mutations in the fibrillin-1 (FBN1) gene. Mortality is often due to aortic dissection and rupture. We investigated the structural and functional properties of the heart and aorta in a [Fbn1C1039G/+] MFS mouse using high-resolution ultrasound (echo) and optical coherence tomography (OCT). Echo was performed on 6- and 12-month old wild type (WT) and MFS mice (n = 8). In vivo pulse wave velocity (PWV), aortic root diameter, ejection fraction, stroke volume, left ventricular (LV) wall thickness, LV mass and mitral valve early and atrial velocities (E/A) ratio were measured by high resolution echocardiography. OCT was performed on 12-month old WT and MFS fixed mouse hearts to measure ventricular volume and mass. The PWV was significantly increased in 6-mo MFS vs. WT (366.6 ± 19.9 vs. 205.2 ± 18.1 cm/s; p = 0.003) and 12-mo MFS vs. WT (459.5 ± 42.3 vs. 205.3 ± 30.3 cm/s; p< 0.0001). PWV increased with age in MFS mice only. We also found a significantly enlarged aortic root and decreased E/A ratio in MFS mice compared with WT for both age groups. The [Fbn1C1039G/+] mouse model of MFS replicates many of the anomalies of Marfan patients including significant aortic dilation, central aortic stiffness, LV systolic and diastolic dysfunction. This is the first demonstration of the direct measurement in vivo of pulse wave velocity non-invasively in the aortic arch of MFS mice, a robust measure of aortic stiffness and a critical clinical parameter for the assessment of pathology in the Marfan syndrome.

Heart transplantation is an increasingly acceptable therapeutic option for children with end-stage and complex congenital heart disease. With advances in surgery, immunosuppression, and follow-up care, functional outcomes need to be evaluated. We report the results of serial exercise testing performed using stress echocardiography in a cohort of pediatric HTP. HTP (n = 7) exercised on a semi-recumbent ergometer to volitional fatigue. Echocardiography-Doppler measurements, HR, and blood pressure were taken at rest and during staged exercise. Results were compared with healthy CON (n = 12). HTP did significantly less work during exercise (940 vs. 1218 J/kg, p0.03). Their SVI (33 vs. 49 mL/m(2), p0.003), CI (5.16 vs. 9.25 L/min/m(2), p0.0005), and HR (162 vs. 185 bpm, p0.02) were lower at peak exercise. HTP had a lower SF at peak exercise (48% vs. 52%, p0.03) and an abnormal relationship between the MVCFc and σPS. During follow-up, hemodynamics and left ventricular function remained relatively constant in HTP. HTP are able to exercise safely; however, their exercise tolerance is reduced, and hemodynamics and contractility are diminished. Over time, their hemodynamics and left ventricular function have remained relatively constant.

Skeletal manifestations are the hallmark of the osteogenesis imperfecta group of disorders. Extraskeletal involvement may, however, contribute significantly to morbidity. Structural cardiovascular anomalies reported in osteogenesis imperfecta include aortic root dilatation and aortic and mitral valve dysfunction. Herein is reported the first case of involvement of the right side of the heart in osteogenesis imperfecta.

We describe the morphologic variability in pulmonary atresia with intact ventricular septum (PAIVS) within a population-based study.An uncommon disease, PAIVS shows considerable morphologic heterogeneity. Clinical reports, based mostly on small samples of patients, may not reflect the true spectrum of pathology of this condition. We have studied the entire range of morphology in a prospective population-based study of patients over a five-year period (1991 to 1995). METHODS; As part of the United Kingdom and Ireland Collaborative Study of PAIVS, all 18 pediatric cardiac centers were visited by a single investigator. Morphologic features of each case were determined by direct review of the echocardiograms and angiocardiograms, from surgical and autopsy reports, and by review of pathology specimens where available.Among 183 live-born infants, atresia was valvar (membranous) in 74.7% and muscular in 25.3%. Muscular obliteration of the apical trabecular cavity, and in some cases its infundibulum, resulted in "bipartite" right ventricle (RV) in 33.6%, and a "unipartite" chamber in 7.7%. The remaining 58.7% had "tripartite" morphology. Coronary arterial abnormalities were identified in 45.8%, including arterial stenoses, interruptions and ectasia in 7.6%. Ebstein's malformation coexisted in 18 patients. Median tricuspid valvar size and RV inlet Z-scores were -5.2 and -5.1, respectively.This study provides unique data on the diverse pathology of PAIVS in an unselected population. This will help determine if published reports reflect the true spectrum of pathology of the condition.

Fifty patients with a mean age of 9.2 years (range, 1.2 to 17.5 years) had cardiac catheterization performed under standardized conditions plus a scalar ECG the previous day. Twenty different direct measurements and 25 derived measurements from the ECG were correlated with the resting peak systolic gradient across the aortic valve. Some of the best correlations were with the measured TAVF, TV6, QV6, and the sum of SV1 + RV6 with r values between .33 and .59. Another group of different patients with isolated aortic stenosis were studied with measurements of the important ECG segments. The r value of this "test" series was similar to that of the original group, so the groups were pooled. The best three-term regression equation involved TAVF, QV6, and the sum (SV1 + RV6), with r = .636. A scoring system was also devised to predict severity. If the TAVF is 0.1 mV or less or the TV6 is 0.3 mV or less or if there is no Q in V6, the gradient may be high. In our series, the ECG estimation of resting peak systolic gradient across the aortic valve in aortic stenosis was enhanced by the inclusion of TAVF and QV6 in the regression equation, as well as SV1 + RV6.