Living with Congenital Aortic Stenosis: Exercise Restriction, Patterns of Adherence, and Quality of Life.

Modern consensus panel guidelines recommend restriction from most organized sports for patients with moderate or severe aortic stenosis (AS). However, there is little published data on how frequently physicians deviate from guidelines, how well patients adhere to exercise restrictions, or the effect of restriction on patient-reported quality of life. In this study, we surveyed 93 subjects with AS and their cardiologists regarding participation in organized sports, physical activity, weightlifting, and exercise restriction. Subjects completed the pediatric quality of life inventory (PedsQL) and the pediatric cardiac quality of life inventory (PCQLI). We found that subjects with severe AS (n = 3) were commonly, but not universally, restricted from organized sports (n = 2, 66%). Subjects with moderate AS (n = 40) were rarely restricted from organized sports (n = 6, 17%). No physician-specific characteristics were associated with increased likelihood of recommending exercise restriction. Subjects were more likely to be restricted if they were older (16 years vs. 13 years, p 0.02) and had moderate versus mild AS (p 0.013). PCQLI scores for teens and young adults with AS (age 13-25) were lower than a comparison group of patients with mild congenital heart disease. For all age groups, the PedsQL social functioning score was lower for subjects with exercise restriction (p 0.052). In summary, cardiologists apply consensus guidelines leniently when restricting patients with moderate/severe AS from organized sports and weightlifting. Patients with AS routinely adhere to exercise restriction recommendations. Children and young adults with AS and exercise restriction have lower QOL scores in the social functioning domain.

Cardiovascular impact of the pediatric obesity epidemic: higher left ventricular mass is related to higher body mass index.

OBJECTIVE: To compare secular changes in body mass index (BMI) and left ventricular mass (LVM) in today's children versus children of a generation earlier. STUDY DESIGN: All healthy patients aged 2 to 19 years who underwent echocardiography at a single US academic medical center in 1986 to 1989 (prior era) and 2008 (current era) were included in this retrospective cross-sectional study. BMI, BMI z score, LVM indexed to height (LVMI), LVM z score, and relative wall thickness were calculated. Cardiac geometries were assigned based on LVM z score and relative wall thickness and classified as normal, concentric hypertrophy, eccentric hypertrophy, or concentric remodeling. Stepwise regression analysis was performed to identify determinants of LVMI. RESULTS: There were 350 subjects in the prior era and 350 age- and sex-matched subjects in the current era. Mean BMI and LVMI were both significantly higher in the current era than in the prior era (BMI, 19.9 ± 5.6 kg/m(2) vs 18.1 ± 3.8 kg/m(2), P = .0004; LVMI, 32.7 ± 7.8 g/m(2.7) vs 31.5 ± 8.1 g/m(2.7); P = .02). Determinants of LVMI in both eras were BMI z score, younger age, male sex, and African-American race. CONCLUSIONS: Today's children have higher BMI, LVMI, and predicted cardiovascular risk than their counterparts a generation earlier. Reversal of these trends is needed, and intervention is required.

Use of Amplatzer occlusion devices to occlude Fontan baffle leaks during fenestration closure procedures.

The authors describe the use of Amplatzer occlusion devices to successfully occlude Fontan baffle leaks in three patients. These additional right to left shunts were recognized at the time of elective transcatheter closure of surgically created fenestrations. A common site of baffle leak is described at the base of the right atrial appendage. This suture line serves to exclude the right atrial appendage from Fontan baffle flow and lies within the corrugated surface of the pectinate muscles, making it particularly vunerable to baffle leaks over time.