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[Left superior vena cava simulating abnormal pulmonary venous return].

P Morand, J P Binet, A Chantepie

Archives Des Maladies Du Coeur Et Des Vaisseaux

|October 1, 1980

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A rare congenital heart defect involving coarctation of the aorta and a left superior vena cava (SVC) draining into the left atrium was observed. This case presented a unique centrifugal circulation, successfully treated with surgical correction.

Area of Science:

Cardiovascular Surgery
Pediatric Cardiology
Congenital Heart Disease

Background:

Coarctation of the aorta is a congenital narrowing of the aorta.
A persistent left superior vena cava (SVC) draining into the left atrium is a rare anomaly.
These conditions can lead to complex circulatory patterns and cyanosis.

Observation:

A 7-year-old boy presented with coarctation of the aorta and a left SVC draining into the left atrium.
Unlike typical centripetal flow, this case exhibited centrifugal circulation from the left atrium to the left SVC.
A significant left-to-right shunt (4.2 L/min) was detected with a pulmonary to systemic flow ratio of 1.8.

Findings:

The unusual centrifugal circulation may be attributed to the proximity of left pulmonary veins to the left SVC and a functional mitral obstruction.

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No pulmonary hypertension was noted despite the shunt.

This specific circulatory pattern is exceedingly rare, with only two similar cases reported previously.

Implications:

Simultaneous surgical correction of aortic coarctation and ligation of the left SVC was performed via left thoracotomy without cardiopulmonary bypass.
This case highlights a rare variant of congenital heart disease and its successful surgical management.
Understanding such anomalies is crucial for accurate diagnosis and effective treatment strategies in pediatric cardiovascular surgery.