Abstract
Introduction
Heart failure is the leading cause of death in adults with congenital heart disease (ACHD). Heart or heart and lung transplantation (H/HLTx) can be effective therapy for these patients, but unfavourable anatomy, end-organ damage, pulmonary vascular disease, HLA sensitization and lack of robust selection criteria currently limit its application.
Methods
Consecutive ACHD patients >16 years of age who were considered for H/HLTx at our tertiary centre between 2006 and 2018 constitute our study population. Baseline characteristics and outcome, including H/HLTx and death were obtained for all from designated databases, medical records and the UK Office for National Statistics.
Results
Of a total of more than 9,000 active ACHD patients, 130 (53.1% male, mean age 42.2, IQR 32.6–50.2 years) fulfilled inclusion criteria with a broad spectrum of underlying ACHD and considerable mortality across all anatomic subgroups [Table 1].
82% of patients had previous cardiac surgery (55.5% with ≥2 sternotomies or thoracotomies). Cardinal presenting features were poor functional capacity, low cardiac output, cyanosis and/or end-organ disease; “only” 47% had moderate or severe dysfunction of the systemic and 37% of the subpulmonary ventricle. Frequent comorbidities were atrial arrhythmia 68.7%, PH 30.4%, CKD 23.5%, liver dysfunction or cirrhosis 11.3%; 36.8% of patients had an ICD and 16.5% a CRT, whereas 79.8% were on diuretics, 70.5% MRA, 51.6% beta blockers, 32.6% ACEI, 18.9% ARB, 10.5% Digoxin and 29.5% on pulmonary vasodilators. HLA antibodies were positive in 47.3% (high sensitization >90% in 13.2%).
There was high overall mortality with 35.4% of patients dying over a median of 2.7 years (IQR 1.1–4.9). Only 13.6% from our cohort were transplanted, with a survival of 76.5% at a mean of 5.9 years from H/HLTx.
ACHD subgroup % of total % transplanted Mortality (%) Median follow-up (years) Univentricular heart 25.4 6.1 30.3 2.5 Systemic RV 21.5 7.4 35.7 2.6 Shunts with PAH / Eissenmenger 13.9 5.9 27.8 3 Tetralogy of Fallot with Pulmonary atresia 12.3 14.3 50 3.5 Left sided valvar disease, CoA, Marfan 10.8 14.3 50 0.6 CHD associated with cardiomyopathy 9.2 50 33.3 5.3 Other 6.9 22.2 22.2 2.2 RV: right ventricle; PAH: Pulmonary arterial hypertension; CoA: aortic coarctation.
Survival in end-stage heart failure
Conclusion
Of the small number of patients with heterogenous ACHD considered for H/HLTx in our contemporary practice, there was high overall mortality, whereas only a fraction of patients was actually transplanted. Better patient selection and timing are clearly warranted so that more ACHD patients benefit from this effective therapy.
Acknowledgement/Funding
None
Heart Transplantation in Congenital Heart Disease: In Whom to Consider and When?
