Late Fontan failure in adult patients is predominantly associated with deteriorating ventricular function

Background: The Fontan operation is a palliative procedure and a substantial number of patients eventually experiences late Fontan circulation failure. Previous concepts of Fontan failure implicate increasing pulmonary vascular resistance (PVR) as a key contributor to late circulatory failure. However, data to support this assumption are sparse. We sought to characterize longitudinal hemodynamic and echocardiographic findings in adult failing Fontan patients. Methods: We performed a retrospective cohort study in adult Fontan patients, identifying patients with Fontan failure. Hemodynamic, echocardiographic and clinical data were recorded. Results: Of 173 adult patients (median follow-up after Fontan 20.2 years [IQR 15.7-24.3]), 48 (28%) showed signs of clinical Fontan failure. Thirty-seven patients (77.1%) exhibited ventricular dysfunction (systolic dysfunction defined by ejection fraction <= 45%, n = 22, or diastolic dysfunction defined by systemic ventricular end-diastolic pressure (SVEDP) >= 12 mmHg, n = 15). Elevated indexed PVR (>= 2.5 WU*m(2)) was only observed in 9 (18.8%) patients. Ejection fraction declined from 60% [IQR 55-65] to 47% [IQR 35-55] during follow-up (p < 0.001). Mean pulmonary artery pressure and SVEDP increased from 11 mmHg [IQR 9-15] to 15 mmHg [IQR 12-18] and from 7 mmHg [IQR 4-10] to 11 mmHg [IQR 8-15] (both p < 0.001), respectively, while indexed PVR did not change significantly (2.1 [IQR 1.1-2.4] vs. 1.7 [IQR 1.1-2.5] WU*m(2), p = 0.949). Fontan failure-associated mortality during follow-up was substantial (23/48; 48%). Conclusions: Systolic and diastolic ventricular dysfunction are frequent features in late Fontan failure in adults, while increases in PVR were rarely observed. The intricate interplay between hemodynamic compromises in Fontan failure deserves further research to optimize treatment strategies and outcome.

Automated summary

In late Fontan failure in adults, ventricular dysfunction is a common feature while increases in pulmonary vascular resistance are rarely observed. Over time, ejection fraction decreases, mean pulmonary artery pressure and systemic ventricular end-diastolic pressure increase, but indexed pulmonary vascular resistance does not significantly change.