Heart failure (HF) is the leading cause of morbidity and mortality in adults with congenital heart disease (1). The systemic right ventricle (sRV) is particularly susceptible to the development of HF (2). Several factors, such as tricuspid regurgitation and associated congenital defects, contribute to the development of HF in the sRV, but the most important determinants are the specific morphological and functional characteristics of the sRV (3). Although sacubitril/valsartan and SGLT1 inhibitors have shown favourable preliminary results (4,5), effective pharmacological therapies to prevent and treat HF in this specific patient population are still lacking. 

In the recently published multicentre retrospective study, Ansari Ramandi et al. addressed the problem of HF in patients with sRV in the setting of transposition of the great arteries after atrial switch operation (TGA-AS) and in patients with congenitally corrected transposition of the great arteries (CCTGA) (6). Adult patients with sRV from Dutch and Belgian centres were included. The authors aimed to determine risk factors for HF hospitalization, defined as admission to hospital for signs and symptoms of HF with initiation or increase of HF medication. Patients had no history of prior HF hospitalization and were followed for a median of 17.9 years. 

The authors showed that the incidence of HF hospitalization is high in this patient population. During follow-up, of 552 adults with sRV (70% TGA-AS, 30% CCTGA; median age 23.7 years at baseline, 64% male), 24.3% of patients required at least one hospitalization for HF. The median age at first hospitalization was 46.1 years, and the estimated incidence was 1.4 per 100 patients/year.

A 10-year prediction model for HF hospitalization was developed using different risk factors. A total of 54 patients were hospitalized for HF at 10 years. Several independent predictors for HF hospitalization at 10 years were identified, including age, NYHA functional class II, QRS duration >120 ms, atrial fibrillation on baseline ECG, moderate/severe sRV dysfunction (determined by visual assessment on echocardiography), use of diuretics, use of digoxin, and lower hemoglobin concentration. Five of clinically most important risk factors were used to formulate a 10-year risk score for HF hospitalization. The selected variables were age, functional class II, QRS duration >120 ms, atrial fibrillation on baseline ECG, and at least moderate sRV dysfunction. Each of the five variables was assigned a score, resulting in a maximum of 82.5 points. In the study population, the score ranged from 0 to 62 with a median of 12.5. Based on the score, the study population was divided into three groups: low HF risk (HF risk score 12.5), intermediate HF risk (HF risk score 12.5–23.5) and high HF risk (HF risk score >23.5). Patients in the intermediate- and high-risk groups had a significantly higher risk for HF hospitalization (HR 52.86 and 10.62, respectively) than patients in the low-risk group. 

An online tool for calculating the risk score is also available (https://hf-srv.shinyapps.io/risk-tool/). 

In conclusion, the study confirms that HF hospitalization in patients with sRV is common and increases with age. The management of HF in this complex patient population remains challenging. The proposed clinical stratification risk score may help to identify patients at higher risk for HF hospitalization and implement their management. The risk score uses clinical parameters that are easily obtained in each patient. As the tool is also available online, it is widely accessible and may be used in daily clinical practice, bearing in mind that the model has not been externally validated.