Anomalous systemic and pulmonary venous connections: neonata
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A 3-week-old, 2.6-kg boy was referred to our institution after postnatal diagnosis of a polymalformative syndrome associating complex congenital heart defect, left lung agenesis, gall bladder agenesis, imperforate anus, single umbilical artery, and intra-uterine growth retardation.

Echocardiography showed an unobstructed anomalous pulmonary venous return of the right pulmonary veins to an enlarged coronary sinus and persistent left superior vena cava without brachiocephalic vein, in the setting of levocardia with usual atrial arrangement. Cardiac computed tomography was performed to delineate left pulmonary veins; it demonstrated agenesis of the left pulmonary artery and veins, and hemiazygos return to left superior vena cava, associated with an uninterrupted inferior vena cava confirmed by subsequent transcatheter angiography.

After multidisciplinary team discussion, the anorectal malformation was addressed first. A hybrid cardiac management strategy was then applied, consisting in transcatheter occlusion of the hemiazygos vein with a 12-mm Amplatzer Vascular Plug-II implanted immediately after the origin of the left renal vein, followed by surgical unroofing of the coronary sinus, patent foramen ovale closure, and surgical re-implantation of the left superior vena cava on the right atrial appendage.

Family trio analysis using whole-genome sequencing failed to identify any rare, non-synonymous, pathogenic or likely pathogenic variant in genetic heart diseases associated genes. The 2-year follow-up has been uneventful. Although its aetiology remains unclear, authors believe clinicians should be aware of this unreported, unique congenital malformation of both systemic and pulmonary venous returns.

Source: https://academic.oup.com/ehjcr/article/5/9/ytab346/6363810
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