Congenital atresia of unilateral pulmonary veins associated with a single ventricle: a rare case report and literature review

Circulation Journal : Official Journal of the Japanese Circulation Society
Hsing-Yuan LeeC C Laura Meng

Abstract

Congenital atresia or extreme hypoplasia of individual pulmonary veins is a rare condition that is usually asymptomatic if it only involves 1 or 2 segments of the lungs. It may be fatal if it occurs in combination with other complex cardiac defects. The patients often present with recurrent pulmonary infections, hemoptysis, or cyanosis in the latter case. A definitive diagnosis can be made by cardiac catheterization with selective pulmonary wedge angiography. The treatment of this condition is a challenge and is controversial, and the prognosis is usually poor. We describe the case of a male infant who suffered from shortness of breath and cyanosis since birth. On echocardiography and cardiac catheterization, he was found to have a complex congenital heart disease with a single ventricle (right ventricle morphology), mitral atresia, large atrial septal defect, and atresia of the right pulmonary veins; severe pulmonary hypertension was also observed. He underwent pulmonary artery banding at the age of 2.5 months, but he died because of a pulmonary infection at the age of 6 months. In this report, we present this rare case and review the previous literature.

References

Feb 15, 1985·The American Journal of Cardiology·H P GutgesellD G McNamara
Apr 1, 1983·Pediatric Cardiology·L B BeermanD R Fischer
Jan 1, 1982·Pediatric Cardiology·R M Bini, L M Bargeron
Jan 1, 1982·Cardiovascular and Interventional Radiology·J F JohnsonE V Barnes
Aug 23, 2001·AJR. American Journal of Roentgenology·L E HeynemanH P McAdams
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May 1, 1997·Cardiovascular Pathology : the Official Journal of the Society for Cardiovascular Pathology·K B ReaginW D Edwards Md

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Citations

Sep 8, 2012·European Journal of Cardio-thoracic Surgery : Official Journal of the European Association for Cardio-thoracic Surgery·Song-Wei YuePeng-Xu Ding
Mar 12, 2009·Circulation Journal : Official Journal of the Japanese Circulation Society·Kuang-Jen ChienKen-Pen Weng
Aug 4, 2015·Respiratory Medicine Case Reports·Cindy S DeMastes-CrabtreeRonald L Morton
Oct 23, 2018·Pediatrics International : Official Journal of the Japan Pediatric Society·Yuriko AbeMasahiko Sugitani

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