Abstract

Case: A 62-year-old male with primary ciliary dyskinesia, bronchiectasis, dextrocardia, ccTGA, VSD, ASD, and severe ventricular dysfunction was listed for HLTx. Suitable organs became available from a 21-year-old male donor after brain death. The right sided aorta, persistent left superior vena cava (LSVC), RIJ, and LCFV were cannulated for CPB. The bloc was procured in the typical fashion along with the donor’s pericardium and intact innominate vein. During the recipient cardiectomy, the LSVC was transected proximally, and right atrial (RA) tissue preserved in continuity with the left inferior vena cava (LIVC). To achieve situs solitus positioning of the mediastinum, a pericardial sling was created by plicating the right pericardium to the diaphragmatic pericardium, and the left pericardium was augmented with excess donor pericardial tissue to create a neo-pericardium in the left chest. The recipient’s RA tissue was used to extend the LIVC by forming a cavoatrial conduit and anastomosed to the donor RA, which required a slightly counterclockwise rotation of the cardiac axis and facilitated positioning the left phrenic nerve posterior to the apex. The tracheal and aortic anastomoses were performed in the typical fashion. The donor innominate vein was of sufficient caliber and length to complete the anastomosis to the recipient LSVC and the donor RIJ was ligated. Discussion: HLTx remains the only definitive therapy for patients with combined end-stage cardiopulmonary disease. The leading indication for HLTx is congenital heart disease. Accordingly, centers performing HLTx must be experienced in complex vascular reconstructions and concomitant procedures to address a broad spectrum of anatomical variants. With thoughtful pre-operative planning, close communication with the procurement team, and surgical experience, these procedures may be performed without the use of synthetic materials that predispose to infectious and thromboembolic complications.

Full Text
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