Loureiro, PGeorgiev, SEwert, PTanase, DEicken, AKammer, BDalla-Pozza, R2023-08-112023-08-112021Cardiovasc Diagn Ther . 2021 Apr;11(2):631-636.http://hdl.handle.net/10400.17/4638Cyanosis persisting after surgical repair of complex congenital heart disease (CHD) may be related to the underlying disease. However, extracardiac causes should be also considered. We report on a patient with heterotaxy syndrome and double outlet right ventricle, in whom postoperative cyanosis was associated with an Abernethy malformation type II causing a hepatopulmonary syndrome. Despite this complex anatomy, interventional closure of the portosystemic shunt was done with a Konar MF™-VSD Occluder. The patient recovered rapidly with relief of cyanosis within one month. This case highlights the importance of a careful diagnostic assessment in patients with complex CHD, who presents cyanoses after surgical repair. In addition, it shows the feasibility and safety of a percutaneous approach with complete closure of the vascular malformation in a patient with a complex anatomy.engHSM CAR PEDAbernethy MalformationCongenital Heart DiseaseCyanosisHepatopulmonary Syndromejournal article10.21037/cdt-20-380