Partial anomalous pulmonary venous return (PAPVR) is uncommon in adults. The presence of concomitant lung sequestration, aortic coarctation and mitral valve prolapse (MVP) makes this case even rarer.
A 29-year-old male athlete underwent pre-employment physical examination. He had a grade 4/6 holosystolic murmur at the apex. 2D echocardiography revealed an anterior MVP with severe mitral regurgitation (MR) and a dilated left (L) ventricle with normal systolic function. CT aortogram showed a confluence of vessels on the L hilum draining into the dilated L pulmonary, hemiazygos and azygos veins, and superior vena cava (SVC) consistent with a PAPVR, an anomalous artery arising from the the descending thoracic aorta (T10) supplying the L lower lung (sequestered lung) and an aortic coarctation 3cm from the L subclavian artery. Oximetry run showed a step up at the superior SVC. Qp/Qs was 1.5. He was managed conservatively until 7 years later when he developed atrial fibrillation (AF) and moderate pulmonary hypertension. Mitral valve replacement and preemptive PAPVR repair was done without any complications.
The optimal time to do surgery in this complex case is not known and was done after the patient had AF which was likely secondary to severe MR.
Early recognition of PAPVR prior to the development of R-sided volume overload may improve clinical outcome. In the presence of other cardiac disorders which would warrant surgery, preemptive PAPVR repair can be done.
