Heart failure and ventricular dysfunction in patients with single or systemic right ventricles

Background-Previous studies suggested a high incidence of congestive heart failure in patients with single and/or systemic right ventricles. The corresponding risk in an adult population is unknown. Methods and Results-A cohort of 188 consecutive adult patients with single or systemic right ventricles was prospectively assessed with gated radionuclide angiography (n=135) or 2D echocardiography (n=188) and followed up clinically. Clinical assessment showed 82.4% of the patients were in New York Heart Association class I or H, 13.3% were in class III, and 4.3% were in class IV. Heart failure occurred in 22.2% of patients with transposition of the great arteries and a Mustard procedure, 32.3% of patients with congenitally corrected transposition of the great arteries, and 40% of Fontan-palliated patients. Symptomatic patients had significantly lower anaerobic thresholds (10.3+/-2.8 versus 13.2+/-4.8 mL (.) kg(-1) (.) min(-1), P=0.006) and peak Vo(2) (15.2+/-4.8 versus 20.3+/-6.8 mL (.) ka(-1) (.) min(-1), P<0.00029). Systemic ventricular ejection fraction in symptomatic versus asymptomatic patients at rest was 34.8+/-15.7% versus 46.7+/-13.4% (P=0.00001). Mortality was 47.1% among symptomatic patients and 5% among asymptomatic patients at 15.7 years of postoperative follow-up. Seven of 12 patients with potentially correctable surgical lesions died or persisted in heart failure despite surgery. Best predictors for mortality were New York Heart Association class, systemic ejection fraction, and age at operation. Conclusions-Patients with single or systemic right ventricles have significant risk for heart failure accompanied by high mortality. This study suggests the importance of identifying this group of patients who are at risk for heart failure and considering strategies to preserve ventricular function.