Due to impressive improvements in surgical repair options, even patients with complex congenital heart disease (CHD) may survive into adulthood and have a high risk of end-stage heart failure. Thus, the number of patients with CHD needing heart transplantation (HTx) has been increasing in the last decades. This paper summarizes the changing etiology of causes of death in heart failure in CHD. The main reasons, contraindications, and risks of heart transplantation in CHD are discussed and underlined with three case vignettes. Compared to HTx in acquired heart disease, HTx in CHD has an increased risk of perioperative death and rejection. However, outcome of HTx for complex CHD has improved over the past 20 years. Additionally, mechanical support options might decrease the waiting list mortality in the future. The number of patients needing heart-lung transplantation (especially for Eisenmenger’s syndrome) has decreased in the last years. Lung transplantation with intracardiac repair of a cardiac defect is another possibility especially for patients with interatrial shunts. Overall, HTx will remain an important treatment option for CHD in the near future. 1. Introduction Since the first successful heart transplantation in a human by Christiaan Barnard in 1967 [1], heart transplantation (HTx) has evolved from its experimental stage to an established treatment option for patients in end-stage heart failure [1–3]. In 1967, the allograft survived six hours. Only with the introduction of cyclosporine, further pediatric transplants followed in the 1980’s. While historically most patients undergoing orthotopic HTx suffered from end-stage ischemic or dilated cardiomyopathy, the proportion of patients with congenital heart disease has increased [4]. Congenital heart disease (CHD) is common affecting 0.4–1% of the population. With the advent of modern heart surgery, the majority of these patients, even those with complex lesions, now survive to adulthood [5, 6]. Thus, there is an increasing number of adult survivors with complex CHD [5]. Despite these incredible improvements in surgical repair options and thus outcome, many survivors of infant heart surgery for CHD are not cured and some remain at high risk of developing end-stage heart failure as young adults. Heart failure contributes importantly to the late morbidity and mortality in adult CHD [7–9]. In the end-stage of their disease, orthotopic HTx currently often remains the only viable treatment option. Given these changes in epidemiology of patients with CHD, over the next few decades we expect a rapidly
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