Pacemakers in patients with familial dysautonomia--a review of experience with 20 patients.

Familial dysautonomia (FD) is a genetic disease associated with a high incidence of sudden death. If fatal bradyarrhythmia is an etiological factor then the incidence of sudden death should decrease after pacemaker placement. Retrospective review of 596 registered FD patients revealed that 22 FD patients (3.

Transiently arterialized hemangiomas: relevant clinical and cardiac issues.

Although the majority of hemangiomas of infancy undergo an uncomplicated, predictable course of proliferation followed by involution, a subset of patients sustain a more fastidious course. These include hemangiomas that, at least during part of their life cycle, have a high flow (arterial) component. Hemangiomas with high flow are most frequently located in the liver.

Using health-related quality of life measures to predict cardiac function in survivors exposed to anthracyclines.

Purpose: As the number of pediatric cancer survivors increases, so does the number of survivors previously exposed to anthracyclines as part of their cancer therapy. Because screening is costly, some have suggested that health-related quality of life (HRQL) measures might be useful in focusing screening tests on those patients with cases most likely to display positive findings. This study reports on the predictive ability of HRQL measures to detect patients with abnormalities on serial cardiac testing.

Enalapril to prevent cardiac function decline in long-term survivors of pediatric cancer exposed to anthracyclines.

Purpose: To determine whether an angiotensin-converting enzyme (ACE) inhibitor, enalapril, prevents cardiac function deterioration (defined using maximal cardiac index [MCI] on exercise testing or increase in left ventricular end-systolic wall stress [LVESWS]) in long-term survivors of pediatric cancer.

Patients And Methods: This was a randomized, double-blind, controlled clinical trial comparing enalapril to placebo in 135 long-term survivors of pediatric cancer who had at least one cardiac abnormality identified at any time after anthracycline exposure.

Results: There was no difference in the rate of change in MCI per year between enalapril and placebo groups (0.

Paradoxically normal septal motion in Ebstein's anomaly.

Paradoxical septal motion has been reported as characteristic of Ebstein's anomaly. The patient reported here has the characteristic apical displacement of the tricuspid value, but septal motion is uncharacteristically normal. Because there is only mild tricuspid regurgitation, it is likely that the absence of right ventricular volume overload accounts for the normal septal motion in this patient.

Measuring health-related quality of life in children with heart disease.

This study tested an instrument for measuring health-related quality of life (HRQL) in children with heart disease. HRQL was measured using the New York University Children's Heart Health Survey in a sample of 0- to 20-year-old subjects with heart disease compared with a control group. Heart disease was associated with impairment on all subscales except psychological function.