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2005-10-15-11 Answer to Case of the week #158 © Calì
Answer to Case of the week #158

January 6 - January 19, 2006

Giuseppe Calì, MD 1,Francesco Labate, MD2, Adriano Cipriani, MD3, Sergio Di Liberto, MD1

1. Department of Obstetrics and Gynecology, ARNAS Civico -  Palermo - Italy

2. Department of Obstetrics and Gynecology, AUSL 6 - Palermo - Italy

3. Unit of Pediatric Cardiology, ARNAS Civico - Palermo - Italy

This is a 22-year-old gravida 1, that was referred to our institution for a routine ultrasound scan. A scan performed at 30 weeks demonstrated a normal fetal biometry and morphology and the following is four-chamber view of the heart

The four-chamber view showed dilatation of the right atrium. No others signs of cardiac malformations (valvular heart disease or intracardiac shunt) were seen. The ventricles, interventricular septum, foramen oval, aorta and pulmonary artery were normal. The scan was repeated every 2 weeks demonstrating a normal growth with the persistence of right mega atrium but no sign of fetal heart arrhythmia (frequently observed in association with these cases).

At 36 weeks the patient underwent a cesarean section for premature rupture of the membranes. The neonate’s weight was 2,650 grams and he was in good clinical condition. An echocardiography was performed soon after birth and it confirmed the idiopathic right atrium dilatation, but no tricuspid regurgitation or other pathologies were found. After six months the right atrium is still dilated, however the baby enjoys good health with normal growth.



Idiopathic dilatation is a rare congenital heart malformation. When it is observed in utero, Ebstein"s anomaly is usually first suspected because of a frequent association with tricuspid regurgitation. The most commonly associations are:

  • arrhythmias,
  • cardiomegaly and
  • fetal hydrops in severe case.

Ebstein’s anomaly is one of the few congenital cardiac pathologies than can causes severe problems to the heart (hydrops, tachyarrhythmia, cardiomegaly) during intrauterine life. Perhaps the most important sign in differential diagnosis of Ebstein’s anomaly vs. idiopathic right mega atrium is the septal tricuspid leaflet which, in the latter condition, is in its normal position. Furthermore it is important to differentiate idiopathic right atrial dilatation from Ebstein"s anomaly because surgery (often not required for the first) is much more difficult in the second case.




  1. Idiopathic dilatation of the right atrium diagnosed in utero. Silva AM, Witsemburg M, Elzenza N, Stewart P. Rev Port Cardiol Feb 1992; 11(2):161-3
  2. Idiopathic dilatation of the right atrium simulating Ebstein"s anomaly. A propos of a case diagnosed in utero. Reinhardt-Owlya L, Sekarski N, Hurni M, Laurini R, Payot M. Arch Mal Coeur Vaiss May 1998; 91(5):645-9
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