Thoracic Organ Transplantation in Patients With Congenitally Corrected Transposition of the Great Arteries

Abstract

Purpose: To assess the outcome of adult patients with congenitally corrected transposition of the great arteries (ccTGA) undergoing heart or heart lung transplantation in the UK. Methods: Between 2001 and 2016, 10 patients underwent heart (n= 9) or heart-lung (n= 1) transplant for ccTGA (4 at Papworth, 6 at Newcastle). All patients had been managed medically and none had undergone prior corrective surgery for ccTGA or other cardiac defects. The mean age was 43.4±14.6 years (range 19-63), mean weight 67.6±13.8 kg and height 173±8.6 cm. The mean cardiac index was 1.9±0.6 L.min-1.m-2, mean wedge pressure 18.4±5.6 mmHg. Indication for heart-lung transplantation was raised pulmonary vascular resistance (8.8 Wood units). The aorta and pulmonary artery were kept long on the donor heart and direct anastomoses of the donor and recipient pulmonary arteries and aorta was possible in all cases without any need for prosthetic material. Results: Transplantation was successful in all 10 patients. The mean ischemic time was 157.9±43.6 min and CPB time was 239.9±72.9min. Three patients underwent transplantation within the last 5 years (all alive). Survival for the other 7 patients was 100% at 30 days, 1 and 5 years. Mean follow-up duration was 1,686.8 days (3-3291). There were 2 late deaths at 8? and 9 years post heart transplant. The mean ischemic time was 157.9±43.6(114-264) min and CPB time was 239.9±72.9(115-366) min. Conclusion: Heart or heart-lung transplant are suitable treatment options for severe systemic RV failure in cases of medically managed uncorrected ccTGA. Long vascular cuffs on the donor heart facilitate direct anastomoses with the recipient great vessels. Both early and late outcomes have been excellent. (Table presented).