Heart failure in patients with a systemic right ventricle: A multicentre study with long-term follow-up

Aims: Adult patients with transposition of the great arteries (TGA) and an systemic right ventricle (sRV) are at risk for heart failure (HF). In this study, we investigated risk factors for HF hospitalization and developed a novel risk stratification tool to optimize risk prediction for clinical practice. Methods and results: In this international multicentre study, 522 patients with TGA and an sRV, without history of HF hospitalization, were followed retrospectively for a median of 17.9 years (interquartile range [IQR] 12.9–22.1). HF hospitalization was defined as a hospital admission for HF signs and symptoms with initiation or increase of HF medication. Predictors for HF hospitalization were established using a Cox regression analysis and were used to build a 10-year risk score. Of the 522 patients, 70% had an atrial switch operation and 30% had a congenitally corrected TGA. The median age at time of enrolment was 23.7 years (IQR 19.9–32.1) and 64% were male. During follow-up, 127 patients (24.3%) had at least one HF hospitalization. A risk stratification tool was built using the following independent predictors associated with a 10-year risk of HF hospitalization: age, New York Heart Association functional class ≥II, QRS duration >120 ms, atrial fibrillation, moderate/severe right ventricular dysfunction, with a C-statistic of 0.868 (95% confidence interval 0.823–0.913). Conclusion: During follow-up, 24.3% of sRV patients had at least one HF hospitalization. Five simple, clinically-accessible variables can be used as a risk score tool to identify patients at higher risk of HF hospitalization.