Biventricular reverse remodelling achieved through combined surgical and optimal medical therapy in an adolescent female post-Rastelli procedure: a case report

Background: Advances in medical treatments have allowed many congenital heart disease patients to reach adulthood, resulting in an increase in adult congenital heart disease (ACHD) cases. Managing heart failure (HF) in ACHD patients is a significant concern, particularly due to limited understanding of optimal pharmacological therapies. Conduit dysfunction and right heart failure are common in the late phase after the Rastelli procedure, but right HF is not well understood, and standard medications for acquired HF are not particularly effective. Consequently, there is a knowledge gap regarding optimal treatment strategies. Case summary: We report the case of a 19-year-old woman with a history of Blalock-Taussig shunt, Rastelli procedure, and reoperation for conduit stenosis. She was diagnosed with chronic active Epstein-Barr virus infection and admitted due to exertional dyspnoea and orthopnoea. On admission, she exhibited signs of acute HF with severe left ventricular dysfunction and conduit stenosis. Initial management with diuretics was insufficient, requiring inotropic support. Right heart catheterization revealed conduit failure, necessitating surgical replacement. Postoperatively, optimized medical therapy, including beta-blockers, ivabradine, vericiguat, and SGLT-2 inhibitors, was administered. Over several months, her biventricular function improved significantly, and she underwent umbilical cord blood transplantation for EBV infection, with a favourable outcome. Discussion: This case highlights the importance of combining surgical and pharmacological strategies in managing complex ACHD with biventricular dysfunction. Identifying underlying causes, such as chronic myocarditis due to EBV infection, is crucial. Ivabradine allowed for beta-blocker increase, and with newer agents like vericiguat and SGLT-2 inhibitors, significantly improved cardiac function.