Long-Term Experience with Heart Transplantation in Patients with Congenital Heart Disease: Focus on Single-Ventricle Patients or the Fontan Circulation

Background: Heart transplantation (HTx) is a well-established therapy for end-stage heart failure of cardiomyopathy (CM) and congenital heart disease (CHD). Survival has improved, but is influenced by complexity of patient anatomy. We report our experience on patients who underwent HTx with focus on single ventricle (SV) morphology, use of VAD and waiting time.

Method: We reviewed retrospectively medical history and data bases of HTx patients (pediatric and adult patients with CHD) transplanted in our institution since 1988.

Results: Between 1988 and 2022, a total cohort of 201 patients [age 8.3 ± 7.4 years (0.01; 37.8), male:female ratio of 114:87; waiting time (WT) 180.1 ± 333.3 days (0; 2684)] underwent HTx due to CM (77.1%) or severe CHD (22.9%) – among them were 15 patients (7.46%) with SV physiology. VAD was used in 68 patients (33.8%; m:f ratio of 41:27) prior HTx [age at HTx 10.2 ± 6.9 years (0.05; 37.7), WT 273.7 ± 377.7 days (1; 2,597)]. 57 patients (83.8%) were diagnosed with CM, the remaining 11 patients had severe CHD. Mean survival of patients currently under medical follow-up was 15.2 ± 10.1 years (0.1; 32.3). 78 patients out of the total cohort (38.8%) died with a mean survival after HTx of 6.9 ± 6.8 years (0; 32.3). For sub-analysis, we divided the FU time into 3 decades: 1988–1999 vs. 2000–2010 vs. 2011–2022. Mortality rate: 52.3% vs. 47.6% vs. 15.5%; WT: 71.2 ± 118.8 (0; 901) vs. 161 ± 187.5 (4; 894) vs. 323.8 ± 492 (1; 2,684) days. Re-HTx was needed in 5 patients with a mean interval of 17.5 ± 5 years (11.1; 23.2) after primary HTx. A total of 15 (7.3%) patients of the overall cohort had SV morphology, m:f ratio of 14:1, age at HTx 7.4 ± 4.7 years (1.1; 16.7), WT 360.7 ± 685.6 days (1; 2,684). Indication for HTx was ventricular failure in 12 patients, “Fontan failure” with PLE in 3 patients, one of these patients with “Plastic Bronchitis” additionally. Five patients (66.7%) were bridged with SV-VAD. Four patients (26.7%) died with a mean survival after HTx of 3.4 ± 6 years (0; 12.4), with early and late mortality (CAV) in each 2 patients. In four patients, early mild-moderate rejection had been detected within the first year. Only one patient was diagnosed with PTLD (currently complete remission) as compared to seven patients in the total cohort.

Conclusion: HTx is an effective procedure in patients with CM and CHD incl. SV physiology. Over different decades use of VAD and WT seem continuously to increase, but early and late mortality are inversely influenced. VAD use and patients with SV physiology might increase complexity for the transplant team, but do not seem to influence early and late mortality at all.

Publication History

Article published online:
28 January 2023