Exercise Capacity, NT-proBNP, and Exercise Hemodynamics in Adults Post-Fontan

Background: Cardiopulmonary exercise testing (CPET) and N-terminal pro–B-type natriuretic peptide (NT-proBNP) measurement are frequently performed in adults post-Fontan, but their correlations with exercise invasive hemodynamics are poorly understood. Moreover, whether exercise cardiac catheterization provides incremental prognostic information is unknown. Objectives: The authors sought to correlate resting and exercise Fontan pressures (FP) and pulmonary artery wedge pressure (PAWP) with peak oxygen consumption (VO₂) on CPET, NT-proBNP, and clinical outcomes. Methods: This was a retrospective cohort of 50 adults (age ≥18 years) post-Fontan undergoing supine exercise venous catheterization between 2018 and 2022. Results: Median age was 31.5 years (IQR: 23.7-36.5 years). Ventricular ejection fraction was 48.5% ± 13.0%. Exercise FP and PAWP were related to peak VO₂ and ln NT-proBNP levels. Patients with peak VO₂ <50% predicted had higher exercise FP (30.0 ± 6.8 mm Hg vs 19 mm Hg [IQR: 16-24 mm Hg]; P < 0.001) and PAWP (25.9 ± 6.3 mm Hg vs 15.1 ± 7.0 mm Hg; P <0.001) compared with those with more preserved exercise capacity. Exercise FP (30.0 ± 7.1 mm Hg vs 23.2 ± 7.2 mm Hg; P = 0.003) and PAWP (25.1 ± 6.7 mm Hg vs 18.8 ± 7.9 mm Hg; P = 0.006) were higher in those with NT-proBNP levels ≥300 pg/mL. During a follow-up of 0.9 years (IQR: 0.6-2.9 years), exercise FP and PAWP remained independently associated with a composite of death, cardiac transplantation, or hospitalization due to heart failure/refractory arrhythmias after adjusting for confounders. Conclusions: In adults post-Fontan, resting and exercise FP and PAWP were inversely related to exercise capacity on noninvasive CPET, and exercise hemodynamics were directly related to NT-proBNP levels. Exercise FP and PAWP were independently associated with clinical outcomes and might be more sensitive than resting values to predict clinical outcomes.