Corresponding Author
Thomas Martens
Thom.martens@ugent.be
A 9-year-old asymptomatic boy underwent a screening check-up in order to be eligible for swimming, revealing a holosystolic murmur. Echocardiography demonstrated a sinus venosus defect with moderate dilation of the right cardiac chambers and a left to right shunt. A persistent left superior vena cava was also present draining to the coronary sinus, the right superior vena cava was draining to the left atrium(Figure 1). Surgery confirmed this diagnosis and subsequently a Warden procedure was performed re implanting the right sided superior vena cava to the right atrium. Transesophageal echo confirmed a good surgical result, the postoperative course was uneventful.
Drainage of the systemic venous system into the left atrium (LA) is a rare condition with occasionally a persistent left SVC draining into the LA (1). It is extremely rare for the right SVC to drain into the LA (2,3). The latter is often associated with other cardiac defects. Diagnosis is confirmed with echocardiography and computed tomography or magnetic resonance imaging.
Clinical presentation varies, depending on the presence of other congenital heart defects. In our case, the presence of bilateral SVCs with a bigger left SVC, together with the sinus venosus and PFO, probably was responsible for absence of clinical symptoms. However, cyanosis is possible, as was seen in a 2-year-old girl with a similar configuration (3), due to a prominent right to left shunt via the drainage of the right SVC to the LA considering that the sinus venosus defect was quite small.