P253 Patient after correction of Taussig-Bing anomaly with severe neoaortic regurgitation and after sudden cardiac arrest

Abstract

Abstract The Taussig –Bing anomaly is a rare congenital heart malformation that was first described in 1949 by Helen B. Taussig and Richard J. Bing. It is characterized by the presence of a large subpulmonic ventricular septal defect (VSD) and, usually, side-by-side great vessels that arise entirely from the right ventricle. Unlike TGA with VSD, this anomaly is characterized by the presence of a bilateral conus and the absence of aortomitral continuity. Increased pulmonary blood flow leads to early onset of pulmonary vascular disease, hence repair in the early infancy period is recommended. VSD closure combined with arterial switch is currently the preferred procedure. Lecompte introduced a new surgical technique that includes extensive resection of the conal septum and direct reimplantation of the pulmonary trunk on the superior margin of the right ventricular infundibulotomy. We present case of 18-year-old male patient with Taussig-Bing anomaly after pulmonary artery binding and closure of ductus arteriosus when one year old and after anatomical Jatene correction with modified Lacompte procedure at the age of two years. The patient was admitted to our Department in October 2018 after episode of sudden cardiac arrest in the mechanism of ventricular fibrillation. Echocardiographic examination revealed preserved systolic function of both ventricles (LVEF 62%, TAPSE 25 mm, RV S’ 13cm/s) without contractility disturbances. The enlarged and hypertrophic left ventricle consists of the primary cavity connecting by VSD (size 39 mm) with a fragment of the right ventricle separated from the rest of the right ventricle by a patch with Gore-Tex acting as a ventricular septum - without any leakage features (Figure 1A and 1B). We noticed also enlarged left atrium, increased diameter of neoaorty bulb (50 mm), severe neoaortic regurgitation (AV max: 1,85 m/s, ERO-70, VC-8mm, PHT 327 ms) (Figure 1C), moderate mitral regurgitation (type I according Carpentier) (Figure1D). On 8.01.2019 a subcutaneous ICD Boston Scientific Emblem with a subcutaneous defibrillation electrode was implanted. In performed spiroergometry decreased peak oxygen consumption (2,24 ml/min/kg), the study was stopped because of fatique, assessed expiratory exchange ratio, and episode of nsVT. After decision of Heart Team the patient is qualified for surgical treatment of valve defects. Abstract P253 Figure 1