Heart Transplantation in Dextrocardia-Heterotaxy Syndrome

Abstract

Purpose Heart transplantation in infants with dextrocardia-heterotaxy syndrome (HS) presents significant surgical challenges. There is limited data on surgical strategies to accomplish heart transplantation in these children. We report surgical strategy and outcomes of 5 patients with HS who underwent orthotopic heart transplant (HT). Methods Heart transplantation in children with HS between 2017-2020 in a tertiary care children s hospital were reviewed. Perioperative characteristics and post-operative outcomes of these 5 patients were obtained. All patients underwent pre-transplant volumetric analysis with chest computerized tomography scan to predict maximum donor size and orthotopic HT with the donor heart placed in a levoposition. Furthermore, recipient systemic veins were routed to the donor heart using extra-cardiac conduits and baffle techniques. Outcomes were compared with 10 size-matched HT patients who had no HS. Results For HS patients the median age at HT was 439 days (IQR:89-716), median weight was 8.4 kg (IQR: 4.1-10.8). For HS pts; waitlist time was 43 vs 38 days (p=0.68), median pulmonary artery pressure was 24 vs 20 mmHg (p=0.5), transpulmonary gradient was 13 vs 8 mmHg (p=0.68). There was no significant difference between groups relative to cardiopulmonary by-pass time (230 vs 163 min, p=0.13), graft ischemic time (196 vs 218 min, p=0.08), and mechanical ventilation time after HT (3.3 days vs 1.08 days, p=0.14). The length of hospital stay was longer for HS patients compared to the control group (99 vs 50 days, p=0.04). At almost one year of post-transplantation follow-up, no patient had rejection or required hospitalization for any infectious etiology in HS group. One patient required cardiac catheterization intervention of the extra-cardiac conduit between superior vena cava and right atrium. To date; all patients in HS are still alive except for one patient who died 8 months post-transplantation. Conclusion HT in infants is feasible with comparable one-year outcomes as other infants undergoing HT. Detailed preoperative planning using computed tomography volumetric analysis to define appropriate donor size and use of extra-cardiac systemic venous conduits facilitates successful HT in small infants with HS.