Revisiting Wolff-Parkinson-White risk stratification: a malignant arrhythmia in a patient with intermittent pre-excitation.

It has been reported that the presence of intermittent pre-excitation indicates low risk of rapid conduction via the accessory pathway in atrial fibrillation. We report a case of a 10-year-old boy with a history of intermittent pre-excitation who presented with atrial fibrillation with very rapid conduction.

Exercise capacity improves after transcatheter closure of the Fontan fenestration in children.

OBJECTIVES: This study evaluated the aerobic capacity, exercise capacity, and arterial oxygen saturation (O(2)Sat) in children before and after transcatheter Fontan fenestration closure. DESIGN: Observational study comparing exercise parameters and hemodynamics before and after transcatheter fenestration closure in Fontan patients. OUTCOME MEASURES: Working capacity, exercise duration, oxygen consumption (VO(2)), and arterial O(2)Sat were evaluated during aerobic exercise. RESULTS: Twenty patients (mean age 11.4 years) underwent standardized exercise testing before and after fenestration closure. Twelve patients underwent cycle ergometry testing (mean age 14.8 years) (group 1), and eight younger patients (mean age 6.4 years) underwent Bruce treadmill testing (group 2). The same exercise protocol was used in each patient before and after fenestration closure (interval between tests: 118 +/- 142 days). Immediately following fenestration closure at cardiac catheterization, cardiac index decreased (3.0 to 2.1 L/minute/m(2)) and Fontan pressure increased (11 +/- 2 to 12 +/- 2 mm Hg) with an increased arterial saturation (92 to 96%) (P < .001). The total group demonstrated no significant change in pre- and postclosure maximal heart rates (164 +/- 21 and 169 +/- 19 bpm). Rest and exercise O(2)Sat increased (89 and 82 to 95 and 92%) (P < .0001). Exercise duration increased (7.7 +/- 1.9 to 9.2 +/- 2.4 minutes) (P < .0005). Maximal VO(2), indexed maximal VO(2), and total working capacity in kilopond-meters (kpm) increased (1.2 +/- 0.5, 27 +/- 7 and 2466 +/- 1012 to 1.3 +/- 0.4 L/minute, 31 +/- 9 mL/kg/minute and 2869 +/- 1051 kpm, respectively) (P < .005). CONCLUSION: In children with a univentricular heart after Fontan palliation, transcatheter fenestration closure improves exercise arterial O(2)Sat and aerobic capacity despite a restricted resting cardiac output documented by catheterization immediately after the closure procedure.

Chronic progressive cardiac dysfunction years after doxorubicin therapy for childhood acute lymphoblastic leukemia.

PURPOSE: Cross-sectional studies show that cardiac abnormalities are common in long-term survivors of doxorubicin-treated childhood malignancies. Longitudinal data, however, are rare. METHODS: Serial echocardiograms (N = 499) were obtained from 115 doxorubicin-treated long-term survivors of childhood acute lymphoblastic leukemia (median age at diagnosis, 4.8 years; median follow-up after completion of doxorubicin, 11.8 years). Results were expressed as z scores to indicate the number of standard deviations (SDs) above (+) or below (-) the normal predicted value. Median individual and cumulative doxorubicin doses were 30 mg/m2 per dose and 352 mg/m2, respectively. RESULTS: Left ventricular fractional shortening was significantly reduced after doxorubicin therapy, and the reduction was related to cumulative dose. z scores for fractional shortening transiently improved before falling to -2.76 more than 12 years after diagnosis. Reduced fractional shortening was related to impaired contractility and increasing afterload, consequences of a progressive reduction of ventricular mass, and wall thickness relative to body-surface area. Left ventricular contractility fell significantly over time and was depressed at last follow-up in patients receiving more than 300 mg/m2 of doxorubicin. Systolic and diastolic blood pressures were below normal more than 9 years after diagnosis. Even patients receiving lower cumulative doxorubicin doses experienced reduced mass and dimension. Fractional shortening and dimension at the end of therapy predicted these parameters 11.8 years later. CONCLUSION: Cardiac abnormalities were persistent and progressive after doxorubicin therapy. Inadequate ventricular mass with chronic afterload excess was associated with progressive contractile deficit and possibly reduced cardiac output and restrictive cardiomyopathy. The deficits were worst after highest cumulative doses of doxorubicin, but appeared even after low doses.

Effects of environmental exposures on the cardiovascular system: prenatal period through adolescence.

Exposures to drugs, chemical and biological agents, therapeutic radiation, and other factors before and after birth can lead to pediatric or adult cardiovascular anomalies. Furthermore, nutritional deficiencies in the perinatal period can cause cardiovascular anomalies. These anomalies may affect heart structure, the conduction system, the myocardium, blood pressure, or cholesterol metabolism. Developmental periods before and after birth are associated with different types of risks. The embryonic period is the critical window of vulnerability for congenital malformations. The fetal period seems to have lifelong effects on coronary heart disease and its precursors. During the weeks immediately after birth, susceptibility to myocardial damage seems to be high. Exposure to cancer chemotherapy or radiotherapy in childhood raises the risk of long-term progressive left ventricular dysfunction and other cardiovascular problems. In childhood and adolescence, use of recreational drugs such as cocaine and tobacco poses cardiovascular dangers as well. Where evidence about environmental exposures is limited, we have included models of disease and other exposures that are suggestive of the potential impact of environmental exposures.

Long-term enalapril therapy for left ventricular dysfunction in doxorubicin-treated survivors of childhood cancer.

PURPOSE: A common late effect of doxorubicin therapy for childhood cancer is reduced left-ventricular (LV) wall thickness resulting in elevated LV afterload and depressed LV function. Many children are given angiotensin-converting enzyme inhibitors, which have been studied primarily in adults. We document the long-term effects of angiotensin-converting enzyme inhibitors in doxorubicin-treated survivors of childhood cancer. PATIENTS AND METHODS: In this retrospective study, we reviewed records of 18 children who had regular echocardiographic examinations during enalapril therapy (mean age at cancer diagnosis, 8 years; mean time between completion of doxorubicin therapy and start of enalapril, 7 years; median follow-up since the start of enalapril, 10 years). RESULTS: Over the first 6 years of enalapril therapy, there was progressive improvement toward normal values in LV dimension, afterload, fractional shortening, and mass, but all these parameters deteriorated between 6 and 10 years. LV wall thickness deteriorated throughout the study period, as did LV contractility and systolic blood pressure. Diastolic blood pressure fell slightly. By 6 years on enalapril, all six patients who had had congestive heart failure at the start of enalapril therapy had either died or undergone cardiac transplantation, compared with three of the 12 asymptomatic patients. CONCLUSION: In doxorubicin-treated long-term survivors of childhood cancer, enalapril-induced improvement in LV structure and function is transient. The primary defect, which is LV wall thinning, continues to deteriorate, and thus the short-term improvement was mostly related to lowered diastolic blood pressure.