PAPVC with TS—Surgically managed with bilateral ectopic drainage ：a case report and literature review

Abstract

Abstract Background Partial anomalous pulmonary venous connection (PAPVC) is a congenital anomaly disease, which is more common on the right side and rarely involves the left .Turner syndrome(TS)is one of the most common chromosomal abnormalities in humans,and about half of people with TS have congenital or acquired cardiovascular disease.This is the only case of PAPVC with TS in our hospital in more than 70 years, and the disease is extremely rare internationally. We analyzed and studied these two unexpected related diseases from the aspects of clinical diagnosis and surgery, hoping to provide help for the research of TS disease in the cardiovascular field.Case presentation We report an unusual type of bilateral PAPVC, involving the right superior pulmonary veins(RSPV) draining into the superior vena cava(SVC)and the left superior pulmonary veins(LSPV) flowing into the left brachiocephalic vein(LBV) in a young child who was eventually diagnosed with TS.There is an intact intracardiac structure and no other obvious manifestations except for unexplained short stature.The child underwent successful surgery with cardiopulmonary bypass (CPB) support, behaving good recovery and was discharged on 8 days.Conclusions: Our findings identified a novel pattern of pulmonary venous variation in patients with TS and provide new insights in the large vascular neighborhood of the heart.Thanks to advances in image-assisted diagnosis and chromosomal karyotyping, this child was diagnosed at an early stage of the disease, avoiding the occurrence of poor prognosis. We should exclude the presence of PAPVC in TS patients, strengthen the understanding of the disease, develop individualized surgical treatment plan, so as to shun medical errors.