Implantation of a Left Ventricular Assist Device in a Patient with a Congenitally Corrected Transposition of the Great Arteries and a Situs Inversus Totalis

Objectives: Congenitally corrected transposition of the great arteries (ccTGA) and situs inversus totalis (SIT) are rare congenital anomalies. This is a case report of a patient with both ccTGA and SIT who was supported with a left ventricular assist device (LVAD), resulting in a challenging clinical scenario.

Methods: A 30-year-old male patient presented with decompensated dilative cardiomyopathy (INTERMACS II). The patient had a history of interventional ASD closure several years ago. Other comorbidities include a right atrioventricular (AV) valve insufficiency II, a left AV valve insufficiency I and an intermittent AV block III, treated by a DDD pace maker with its electrodes placed inside the left atrium and ventricle. The function of the right ventricle (systemic ventricle) was severely impaired. The implantation of a HeartWare HVAD^® (HeartWare Inc., Framingham, MA) was intended as a bridge to heart transplantation. The inflow cannula was inserted into the diaphragmatic surface of the right ventricle (systemic ventricle) and the outflow cannula was connected to the ascending aorta, which was located to the right side of the pulmonary artery due to SIT. Resection of several trabeculae and parts of papillary muscles of the right AV valve was necessary to avoid future suction events.

Results: The surgery was uneventful and besides a respiratory decompensation on the fourth day after surgery requiring reintubation for three days there were no peculiarities. The patient was discharged home on postoperative day 33. He is doing well so far, living at home and waiting for heart transplantation. At the time of abstract submission, the patient has 165 days of LVAD support. The LVAD is running with 2900 rpm with a flow of 5.9 l/min.

Conclusion: To our knowledge, this is the first reported case in English literature of an LVAD implantation in a patient with the combination of both ccTGA and SIT. LVAD implantation is feasible, but utmost caution is inevitable to avoid future suction events.