Orthotopic heart transplant following implantation of a systemic ventricular assist device in a patient with congenitally corrected transposition of the great arteries and dextrocardia situs inversus totalis

Abstract

Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart disease that commonly results in congestive heart failure due to exposure of the anatomic right ventricle to systemic pressures. Additional anatomic abnormalities, such as situs inversus, in which the viscera are mirror images of normal anatomy, or dextrocardia in which the heart apex is located on the patient’s right, once were contraindications to transplant in the treatment of advanced heart failure. However, recent advancements now allow for the management of heart failure through mechanical circulatory support and subsequent transplant. This case presents a 52 year-old man with advanced heart failure secondary to CCTGA and dextrocardia situs inversalis totalis that was managed with a ventricular assist device (VAD) after maximal medical treatment, and subsequently received an orthotopic heart transplant. This case will also review CCTGA in relation to heart failure and the surgical challenges faced in terms of ventricular assist device implantation and orthotopic heart transplant.