Acquired right heart outflow tract anomaly without systemic hypertension in recipient twins in twin-twin transfusion syndrome.
Significant hemodynamic changes are commonly observed in both fetuses in twin-twin transfusion syndrome. In the recipient twin there is cardiac dysfunction with hypertrophy of both ventricles and overall enlargement of the heart. We describe five cases of recipient twins in twin-twin transfusion syndrome with right ventricle hypertrophy, pulmonary stenosis and tricuspid regurgitation acquired in utero. These symptoms developed with no signs of systemic hypertension. Three of the five recipient twins survived and were developing normally at 3-30 months of age. Postnatal outcome is likely to have improved as a result of prenatal diagnosis of right ventricle outflow tract obstruction and timed delivery. These acquired anomalies of the right heart might be related to the particular hemodynamic conditions of the recipient twin. A global heart dilatation is logically expected, but this hypertrophy without dilatation is probably related to the shared plasma of fetuses with opposite hemodynamic conditions. This is what we consider as the hemodynamic-hormonal paradox. These acquired anomalies, though severe, are accessible to neonatal treatment if treated early.
Nizard
J
J
Fetal Medicine Unit, Paris-Ouest University, 78303 Poissy Cedex 03, France.
Bonnet
D
D
Fermont
L
L
Ville
Y
Y
D000328:Adult; D005260:Female; D005330:Fetofetal Transfusion; D006801:Humans; D017380:Hypertrophy, Right Ventricular / Q000209:etiology*; D011247:Pregnancy; D011666:Pulmonary Valve Stenosis / Q000209:etiology*; D014262:Tricuspid Valve Insufficiency / Q000209:etiology*; D014694:Ventricular Outflow Obstruction / Q000209:etiology*