Diagnostic ultrasound features and outcome of restrictive foramen ovale in fetuses with structurally normal hearts.

Intrauterine foramen ovale (FO) restriction in association with congenital heart disease (CHD) carries a poor prognosis. However, in the absence of CHD, the clinical importance of restrictive FO in the fetus is not well understood. We evaluated the antenatal prevalence, clinical presentation, diagnostic ultrasound features, and outcome of restrictive FO in fetuses without CHD. We reviewed the echocardiographic and clinical records of 23 fetuses diagnosed with a restrictive FO and structurally normal heart between 2001 and 2012. The atrial septum, dimensions of cardiac structures, left and right cardiac output and Doppler interrogation of cardiac flows were examined. The clinical outcomes of all fetuses with restrictive FO were analysed. Restrictive FO was identified in 23 of 1,682 (1.4%) fetuses with no CHD. Enlarged right heart structures (100%), hypermobile or redundant primum atrial septum (91%), increased right-to-left ventricular cardiac output ratio (91%), and posteriorly angulated ductus arteriosus (68%) were the most common echocardiographic findings associated with this rare phenomenon. Additional noncardiac systemic abnormalities were identified in 13 (56%) babies. Seven (30%) neonates developed persistent pulmonary hypertension, and 7 infants died. Antenatal restrictive FO is an underrecognised entity despite being a common cause of right heart dilatation in the fetus. In the absence of CHD, restrictive FO is well tolerated antenatally, but its frequent association with noncardiac abnormalities and pulmonary hypertension in the neonate are noteworthy.

Mitral valve repair in a patient with Ter Haar Syndrome.

Ter Haar Syndrome is one of the most deteriorating disorders defined in the medical era. Up to date only ten patients were reported. We present a living patient with Ter Haar Syndrome who has undergone successful mitral valve repair.