Features and outcomes in utero and after birth of fetuses with myocardial disease

Abstract

Objectives. Ninety-one fetuses with dilated or hypertrophic cardiomyopathy (DCM, HCM) and myocarditis were studied. Results. Group 1 "DCM" included 19 fetuses: 13 with hydrops (FH) and 5 with associated extracardiac anomalies (ECAs) (15.8%). Group 2 "Myocarditis" included twelve fetuses, having 11 with FH. Group 3 "HCM" included sixty fetuses: 26 had associated ECAs, 17 had maternal diabetes, and 17 were "idiopathic"; however, in one case, a metabolic disorder was found postnatally, and 4 had familiarity for HCM. Outcomes. Ten cases opted for termination of pregnancy. Two cases with DCM and 1 with HCM were lost at follow-up. Out of the cases that continued pregnancy, with known follow-up, mortality was 68.75% in Group 1, 63.6% in Group 2, and 31.3% in Group 3 (the majority with severe ECAs). Surviving cases with DCM and myocarditis improved, 2 with HCM worsened, 6 remained stable, and 26 improved or normalized. Conclusions. Our data show more severe prognosis in DCM and myocarditis and forms with severe associated ECAs.

Figure 1

(a) Echocardiography of a fetus with endocardial fibroelastosis—a high echodensity of the left ventricular (LV) walls due to calcifications is evident. RV—right ventricle, A—aorta. (b) Fetus with myocarditis and fetal hydrops: both ventricles and atria are dilated and the arrows indicate the pericardial effusion. RV—right ventricle, LV—left ventricle.

Figure 2

Doppler findings in fetuses with DCM and fetal hydrops: (a) The tracing of the tricuspid valve (T) shows a reduced systolic A-wave, with respect to the diastolic E-wave; (b) holosystolic tricuspid regurgitation (ITr); (c) a reverse systolic flow of the inferior vena cava (small arrow); (d) a reverse systolic flow of ductus venosus (small arrow); (e) the fluctuation of the umbilical vein.

Figure 3

Flow chart summarizing the frequency and the outcomes in the different forms of myocardial disease.

Figure 4

(a) Fetus with hypertrophic cardiomyopathy in 2-D; (b) M-mode recording showing hypertrophy of the left ventricular walls, with a small cavity and mitral valve movement with diastolic apposition and anterior systolic motion. RV—right ventricle, LV—left ventricle, M—mitral valve.