Fetal hydrops due to right ventricular and tricuspid valve dysplasia. A case report.

We report a case of fetal hydrops at 32 weeks of gestation, diagnosed prenatally due to an unusual form of non-Ebstein dysplasia of the tricuspid valve and of the right ventricle, different from the classification used in the literature. Digoxin therapy, administered for two weeks in utero and then after the premature delivery, did not improve the massive right heart failure due to the tricuspid insufficiency, and the baby died at 4 days of life.

Variability of the Brachmann-de Lange syndrome.

Brachmann-de Lange syndrome (BDLS) is a relatively common multiple congenital anomaly/mental retardation syndrome, whose cause is unknown. The clinical variability of this condition is well-known. Recently some reports suggested the possible existence of a mild BDLS phenotype.