Does the pulmonary arterial capacitance index (PACi) correlate with disease severity and respond to PAH-specific therapy in children with CHD-associated PAH?
In children with CHD-associated PAH, the pulmonary arterial capacitance index is significantly reduced and inversely correlates with pulmonary vascular resistance and mean pulmonary artery pressure.
Objective: The pulmonary arterial capacitance index (PACi) has recently emerged as a dynamic marker of pulmonary vascular compliance. However, its clinical relevance in pediatric pulmonary arterial hypertension (PAH), and particularly its relationship with functional capacity and exercise tolerance in congenital heart disease (CHD), remains unclear. This study explored these associations and assessed changes following PAH-specific therapy. Materials and Methods: Thirty-five patients with CHD-associated PAH with a mean pulmonary artery pressure (mPAP)≥20 mmHg receiving PAH-specific therapy and 35 age-, sex-, and anthropometry-matched CHD controls without PAH were evaluated. Demographic characteristics, hemodynamic parameters, PACi, functional class, brain natriuretic peptide (BNP) levels, and six-minute walk test (6MWT) distances were compared. Pre- and post-treatment hemodynamic and clinical parameters were also analyzed in the PAH group. Results: Ventricular septal defect was the most common CHD in both groups. Children with PAH had significantly higher mPAP and pulmonary vascular resistance index (PVRi) and lower PACi than controls. PACi was inversely correlated with mPAP (r=-0.383, p=0.023) and PVRi (r=-0.812, p
Öztürk et al. (Sun,) studied this question.