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.