Heart-lung transplantation in Eisenmenger syndrome (two for the price of ?)

The current framework for care of pulmonary vascular disease in complex congenital heart disease (CHD) entails multidisciplinary medical management, congenital interventions to improve quality of life and reduce long-term complications, and eventually, consideration for heart-lung transplant (HLTx). Eisenmenger syndrome (ES), the development of severe pulmonary arterial hypertension (PAH) accompanied by right to left shunting in adults with CHD, has traditionally been associated with a poor prognosis. In this Heart ,1 paper the authors investigated the outcomes of (predominantly) HLTx in patients with ES. The overall survival rates were 84.1% and 69.7% at 1 year and 5 years, respectively. In a condition that was long considered incurable, they demonstrate that outcomes in the ES population with HLTx are comparable to contemporary outcomes for other diagnoses. The field of adult congenital heart disease (ACHD) lauds these efforts as they demonstrate the positive effects of medical advances on prolonged survival and the ability to longitudinally study rare disease outcomes with robust data. Importantly, this research suggests that with adequate planning and patient selection, the ACHD population is no longer relegated to medical management alone but can benefit from transplantation (figure 1). Figure 1 Conceptual diagram of the longitudinal care of patients with Eisenmenger syndrome. Congenital heart disease with shunt lesion that is diagnosed early can undergo early repair, and risk of pulmonary arterial hypertension is low. For those with congenital heart disease and shunt lesion who are diagnosed late, some will be candidates for medical optimisation and surgical repair. They may have persistent pulmonary artery hypertension despite repair and should be considered for pulmonary arterial hypertension medical therapy. For those who are diagnosed with Eisenmenger syndrome, surgical repair of shunt lesion is not possible. Thus, initiation and optimisation of pulmonary arterial hypertension medical therapy is crucial. In a select group of patients, medical therapy can …