Invasive and noninvasive hemodynamic assessment in adults with Fontan palliation

Background/objectives: Although echocardiographic-Doppler cardiac index (CI) assessment is widely used to guide heart failure management in patients with biventricular circulation, this application has not been studied in the Fontan population. The objective of this study was to: (1) determine the correlation between Doppler and cardiac catheterization CI calculation; (2) determine the association between Doppler CI and the occurrence of Fontan failure. Methods: Retrospective review of adult Fontan patients followed at Mayo Clinic Adult Congenital Heart Disease program, 1994–2015. Inclusion criteria were: systemic left ventricle and echocardiogram and cardiac catheterization performed within the same week. Fontan failure was defined as a composite of all-cause mortality, heart transplantation listing, and palliative care. Results: 59 patients (age 29 ± 6 years; men 32[54%]) underwent 97 studies. Of the 59, 41[69%] had atriopulmonary Fontan and 12 (20%) had cirrhosis. Compared to patients without cirrhosis, patients with cirrhosis had higher Doppler CI (3.6 ± 0.6 vs 2.8 ± 0.4 L/min ∗ m², p = 0.039); Fick CI (3.3 [2.5–3.7] vs 2.4 [1.6–3.1] L/min/m², p = 0.028); lower systemic vascular resistance (20 ± 3 vs 25 ± 4 WU ∗ m², p = 0.04). There was a positive correlation between Doppler and Fick CI (r = 0.52; p < 0.0001). Fontan failure occurred in 13 patients (22%) within 7.5 ± 2.1 years. In patients without cirrhosis, Fick CI and Doppler CI < 2.5 L/min/m² were associated with Fontan failure (odds ratio [OR] 1.58, p = 0.046) and (OR 1.43, p = 0.051) respectively. Conclusions: Doppler CI assessment in feasible in a selected group of Fontan patients and it is predictive of clinical outcomes. The application of this concept in systemic right ventricles deserves further research.