Hemodynamic Profiling of Patients with a Fontan Circulation Using Pulmonary Pressure/flow Relations During Dobutamine Stress and Pulmonary Vasodilation Testing.
American journal of physiology Heart and circulatory physiology（2025）
Abstract
The hemodynamics of Fontan pathophysiology and the effects of pulmonary vasodilator therapy are insufficiently understood. The aim was to evaluate hemodynamic responses to dobutamine induced stress and the effect of concomitant acute pulmonary vasodilation testing (APV) in patients with a Fontan circulation to identify hemodynamic phenotypes. Sixteen adult patients undergoing cardiac catheterization for clinical indication were included. Hemodynamic phenotyping was performed during baseline, dobutamine induced stress and concomitant APV (inhaled nitric-oxide with FIO2 1.0). Pulmonary vascular disease (PVD) was defined by pulmonary vascular resistance (PVR)>2Wood units or pulmonary artery pressure/pulmonary blood flow-slope (mPAP/Qp)>3mmHg/L/min. Patients were assigned to Group-A without PVD (N=8) or Group-B with PVD (N=8 mPAP/Qp>3 with N=3 PVR>2). For the total group; median cardiac output (Qs) was 5.2L/min and increased to 7.3L/min with dobutamine (p=0.005) without further change with APV (p=0.255). However, subgroup-analysis revealed that during dobutamine the increase in Qs occurred only in Group-A (+3.5L/min, p=0.012), and Qs decreased APV(-1.3L/min, p=0.0036). In contrast, in group-B Qs did not change with dobutamine (p=0.236), nor with APV (p=0.327). However, in contrast to group-A (p=0.889), in group-B Qp increased with APV(+1.3L/min, p=0.017) while the mPAP/Qp-slope improved significantly (6.2 to 1mmHg/L/min, p=0.017). Suggesting that APV improved Qp and oxygenation in patients with PVD, but had negative effects in those without PVD. This study shows that hemodynamic response to dobutamine induced stress and APV differs in patients with a Fontan circulation depending on the presence of pulmonary vascular disease. Hemodynamic phenotyping with sophisticated identification of pulmonary vascular disease potentially allows for patient-tailored treatment.
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