DISCUSSION
There are many cases of LACV reported with left sided obstructive lesion. The largest study was done by Bernstein et al, who reported 25 patients of LACV with left sided obstructive lesions suc as mitral atresia, aortic stenosis, aortic coarctation and cor triatrium (4) In addition, LACV was reported as a concomitant anomaly in 3 patients with ventricular septal defect (VSD) ,atrial septal defect (ASD)-related shunts and pulmonary HT (5). Fujiwara et al reported a case of LACV in a boy being operated for tetralogy of Fallot. However, presence of LACV is very rare anomaly in the setting of normal heart morphology. Both of our cases did not have a concomitant cardiac pathology.
In addition to TTE and angiographic examinations, CT examination is also used in diagnosis. In the literature, the definitive diagnosis of venous return anomalies, which cannot be distinguished by TTE and angiographic examinations, has been clarified with CT scanning (6). In another case reported by Genç et al. LACV was detected incidentally during CT examination. (7). In each of our cases, CT was used to confirm the diagnosis.
Patients with LACV may present symptomatically. Symptoms may be dyspnea, tachypnea, pulmonary congestion findings, or systolic ejection murmur depending on the accompanying cardiac pathology and the amount of shunt. Rarely, it may be asymptomatic (7). In our cases LACV was detected incidentally and was asymptomatic.
LACV causes shunting between the pulmonary venous circulation and the systemic venous circulation, usually between the left atrium and the innominate vein, rarely between the pulmonary vein and the superior vena cava (SVC). In our first case, the direction of the shunt was from the innominate vein to the left atrium. In our second case, the flow direction was from the pulmonary vein to the innominate vein. In the literature, treatment modalities are selected depending on the amount and direction of the shunt flow. Transcatheter occlusion (8) and surgical correction treatments are applied (3),(5). In our first case, since the flow direction was from the innominate vein to the left atrium, surgical closure was preferred using the double ligation technique in terms of paradoxical embolism and infection risk. In our second case, it was decided to wait for treatment because the patient was young and asymptomatic..
In conclusion, , this report described a very rare case of LACV without any other cardiac abnormality. At the same time, the association of this rare anomaly in two siblings has not been reported in the literature. LACV may rarely be asymptomatic. If symptomatic, patients can also be treated noninvasively with device closure or surgical ligation.