JSPCCS-AHA Joint Session（II-AHAJS）
先天性心疾患における新規心不全治療薬の夜明け/Dawn of new drugs for heart failure in patients with congenital heart disease
第一部「先天性心疾患における新規心不全治療薬の国内使用経験」
第二部「先天性心疾患における新規心不全治療薬の日米使用経験」

[II-AHAJS-09] Future perspective of anti-heart failure drugs in congenital heart disease

^○Hidekazu Ishida (Department of Pediatrics, Osaka University Graduate School of Medicine, Osaka, Japan)

With advances in surgical and medical treatment, many children with congenital heart disease (CHD) now survive into adulthood. However, they remain at risk of developing heart failure (HF). Several classes of drugs have been used to treat HF in CHD, including ACE inhibitors, beta-blockers, and diuretics. These drugs have been shown to improve symptoms, reduce hospitalizations, and improve survival in left ventricular HF with reduced ejection fraction (HFrEF). However, their efficacy and safety in the context of CHD especially with univentricular or systemic right ventricular failure patients are not established.In recent years, new drugs targeting different pathways involved in the pathophysiology of HF have been developed and tested in clinical trials. These include angiotensin receptor-neprilysin inhibitor (ARNI), mineralocorticoid receptor antagonist (MRA), SGLT2 inhibitor, sGC stimulator, and I_f channel inhibitor. All these new drugs have been shown to improve outcomes in patients with HFrEF and are recommended in current guidelines of Japan as well as US and Europe. More recently, clinical trials have shown the efficacy of SGLT2 inhibitors in patients with HF with preserved ejection fraction (HFpEF). In near future, clinical trials are strongly necessary to investigate the efficacy and safety of these new drugs in CHD, including repaired tetralogy of Fallot, congenitally corrected transposition of the great arteries, and Fontan patients.