Key result
Pulmonary arterial compliance assessed by IVUS was associated with a lower risk of advanced PAH (OR 0.56; 95% CI 0.37-0.85) and mortality (HR 0.60; 95% CI 0.41-0.87) during long-term follow-up.
Why the study?
Do pulmonary arterial stiffness indices assessed by IVUS predict advanced PAH and mortality in children with early pulmonary vascular disease?
Cohort (n=41)
Do pulmonary arterial stiffness indices assessed by IVUS predict advanced PAH and mortality in children with early pulmonary vascular disease?
Odds Ratio: 0.56 (95% CI 0.37–0.85)
In children with pulmonary vascular disease, pulmonary arterial stiffness indices assessed by IVUS are strong predictors of long-term progression to advanced PAH and mortality, even in those with favorable baseline hemodynamics.
Aims: Prognosis in children with pulmonary vascular disease (PVD) is closely linked to right ventricular (RV) failure due to increased RV-afterload. Pulmonary arterial (PA) stiffening is known to occur early in the course of PVD and constitutes a main component of RV-afterload. This study aimed to evaluate the clinical value of PA-stiffness in children with PVD by determining its association with advanced pulmonary arterial hypertension (PAH) and mortality at long-term follow-up. Methods and results: Forty-one children with various stages of arterial PVD, defined as mean PA-pressure ≥20 mmHg and/or pulmonary-to-systemic flow-ratio ≥1.2, and mean pulmonary capillary wedge pressure <15 mmHg, underwent cardiac catheterization with intravascular ultrasound (IVUS) imaging between 1994 and 1997 with follow-up until 2015. PA-stiffness indices evaluated were PA-area-compliance (PA-compliance) and PA-area-distensibility (PA-distensibility). During follow-up, advanced PAH was determined by echocardiography and cardiac catheterization. During a median follow-up of 19 years, in 31 (76%) patients PVD had reversed and 10 (24%) had advanced PAH. Six (15%) died due to PVD. In addition to conventional haemodynamics, PA-compliance and PA-distensibility were associated with advanced PAH at long-term follow-up (adjusted OR [95% CI] 0.56 [0.37-0.85] and 0.52 [0.31-0.86]), and mortality (adjusted HR [95% CI] 0.60 [0.41-0.87] and 0.67 [0.49-0.90]). Also in a subgroup of patients with favourable haemodynamics, baseline PA-compliance and PA-distensibility were lower in patients with advanced PAH at follow-up (P = 0.002 /P = 0.030). Conclusion: In children with PVD, PA-stiffness indices assessed by IVUS predict advanced PAH and mortality at long term follow-up. Especially in patients with favourable haemodynamics, assessment of intrinsic PA-stiffness may enhance the prognostication of disease progression and survival.
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Ploegstra et al. (2017) conducted a cohort in pulmonary vascular disease (n=41). Pulmonary arterial stiffness indices (PA-compliance and PA-distensibility) was evaluated on advanced pulmonary arterial hypertension (PAH) (OR 0.56, 95% CI 0.37-0.85). Pulmonary arterial compliance assessed by IVUS was associated with a lower risk of advanced PAH (OR 0.56; 95% CI 0.37-0.85) and mortality (HR 0.60; 95% CI 0.41-0.87) during long-term follow-up.
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