MYOCARDIAL-DISEASE DIAGNOSED IN-UTERO - FEATURES AND OUTCOME IN 24 CASES

Objective: The aim of this study was to analyze the frequency, forms, and relative outcome of myocardial disease diagnosed in utero during u ltrasound screening for congenital heart disease (CHD). Methods: Twent y-four of 1440 fetuses at 18-36 weeks' gestation and at maternal or fe tal risk for CHD, examined by fetal echocardiography, were diagnosed t o have myocardial disease (1.7% of the population). Their data were an alyzed, retrospectively. Results: Nine of the 24 cases (37.5%) present ed with a pattern of dilated cardiomyopathy (DCMP), 4 had endocardial fibroelastosis (EFE), and 5 had myocarditis, due to cytomegalovirus in 3. One case had right ventricular DCMP-dysplasia. Of the 24 cases, 14 (58.3%) had features of hypertrophic CMP (HCMP); 9 had extracardiac a nomalies (ECA) (mostly renal and of the central nervous system); 3 wer e fetuses of diabetic mothers (FDM); and 2 had an idiopathic form, wit h a family history in 1. Heart failure was present in all cases with D CMP and in FDM. All the cases with DCMP died in utero or after a prema ture birth, despite antifailure maternal-fetal treatment. Out of 9 cas es with HCMP and ECA, 3 opted for termination of pregnancy, 1 died in utero, and 3 died after birth. Two of 9 survived, normalizing their ca rdiac pattern within 3-6 months, as well as the 3 FDM, who also surviv ed and normalized. Two cases with idiopathic HCMP are alive and stable at 12 and 14 months. Conclusions: Our data indicated a poor outcome f or fetuses with DCMP who present in utero with heart failure. There wa s a relatively high frequency of HCMP secondary to other conditions th at apparently determine the outcome.