Key result
Prenatal univentricular involvement is linked to higher survival in fetal primary cardiomyopathy.
Why the study?
Prenatal diagnosis and characterization of fetal cardiomyopathies, including arrhythmogenic right ventricular dysplasia, remain limited with unclear phenotype distribution and prognosis.
Cohort (n=21)
No
We report a case of arrhythmogenic right ventricular dysplasia (ARVD) diagnosed prenatally by echocardiography at 24 weeks gestation. The 4-chamber view showed a large outpouched area extending from below the tricuspid valve to the insertion of the moderator band; the affected wall appeared thin and akinetic, with absence of flow at color Doppler investigation and no evidence of cardiovascular failure. The size of the outpouched area was unchanged at subsequent controls (25 and 26 weeks gestation) when frequent extrasystoles occurred, probably of a ventricular origin. The pregnancy was terminated at 27 weeks. The histopathologic examination of the fetal heart showed the presence of clusters of adipocytes interspersed with myocardial fibers, consistent with the diagnosis of ARVD.
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Rustico et al. (2001) conducted a cohort in Fetal primary cardiomyopathy (n=21). Univentricular myocardial involvement vs. Biventricular myocardial involvement was evaluated on Survival at last follow-up (p=0.035). Among fetuses diagnosed with primary cardiomyopathy, survivors at last follow-up showed a significantly higher proportion of prenatal univentricular myocardial involvement compared to non-survivors (p=0.035).
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