Prediction of postnatal clinical course in primary congenital dilated cardiomyopathy.

BACKGROUND: The aim of this study was to investigate the prediction of postnatal prognosis using fetal and perinatal data in patients with primary congenital dilated cardiomyopathy (PCDCM), and to estimate the incidence of this disease. METHODS: We examined correlations between fetal or perinatal data and postnatal clinical course in a multicenter retrospective study of eight patients with PCDCM. Incidence was calculated in a population-based study. RESULTS: All patients developed heart failure at a median of 8 days (range, 0-43 days), and six patients died or required extracorporeal artificial heart therapy at a median of 67 days (range, 0-92 days). The cardiothoracic area ratio from fetal echocardiography, the Apgar score, and the standard deviation of birth weight correlated significantly with the date at onset of heart failure. However, no data correlated with survival. Cumulative incidence of PCDCM was calculated as 1.21 per 100 000 total births (95% confidence interval, 0.37 to 2.06). CONCLUSIONS: Primary congenital dilated cardiomyopathy has a poor prognosis, but cardiothoracic area ratio from fetal echocardiography, body weight at birth, and Apgar score correlate with the timing of the onset of heart failure, and these indicators might therefore be useful for peri- and postnatal management.

[Corrected Transposition of the Great Arteries, Ventricular Septal Defect, Tricuspid Regurgitation and Pulmonary Hypertension Operated at Middle Age: Report of a Case].

A 45-year-old male with corrected transposition of great arteries and the ventricular septal defect (VSD) was considered to have no indication for the total repair because of severe pulmonary hypertension in his young age. He was suffered from heart failure with absence at the age of 44. Detail examinations revealed the severe tricuspid valve regurgitation with VSD. We reevaluated him for the operative indication by the aspect of pulmonary hypertension. His pulmonary vascular resistance decreased with the administration of 100% oxygen, and no pulmonary vascular obstructions were detected in the lung specimen. As a result, he underwent VSD patch closure and tricuspid valve replacement. His postoperative course was uneventful and he was discharged from our hospital at day 16 postoperatively. Our data suggested that reevaluation including lung biopsy should be important to determine operative indication for adult congenital heart disease.