Pulmonary arterial hypertension‐associated genetic variants in combined post‐capillary and pre‐capillary pulmonary hypertension: a case report

Pulmonary hypertension is a common sequelae of left heart failure and may present as isolated postcapillary pulmonary hypertension (Ipc‐PH) or combined pre‐ and postcapillary pulmonary hypertension (Cpc‐PH). Clinical features associated with progression from Ipc‐PH to Cpc‐PH have not yet been described. We extracted clinical data from patients who underwent right heart catheterizations (RHC) on two separate occasions. Ipc‐PH was defined as mean pulmonary pressure >20 mmHg, pulmonary capillary wedge pressure >15 mmHg, and pulmonary vascular resistance (PVR) < 3 WU. Progression to Cpc‐PH required an increase in PVR to ≥3 WU. We performed a retrospective cohort study with repeated assessments comparing subjects that progressed to Cpc‐PH to subjects that remained with Ipc‐PH. Of 153 patients with Ipc‐PH at baseline who underwent a repeat RHC after a median of 0.7 years (IQR 0.2, 2.1), 33% (50/153) had developed Cpc‐PH. In univariate analysis comparing the two groups at baseline, body mass index (BMI) and right atrial pressure were lower, while the prevalence of moderate or worse mitral regurgitation (MR) was higher among those who progressed. In age‐ and sex‐adjusted multivariable analysis, only BMI (OR 0.94, 95% CI 0.90–0.99, p = 0.017, C = 0.655) and moderate or worse MR (OR 3.00, 95% CI 1.37–6.60, p = 0.006, C = 0.654) predicted progression, but with poor discriminatory power. This study suggests that clinical features alone cannot distinguish patients at risk for development of Cpc‐PH and support the need for molecular and genetic studies to identify biomarkers of progression.

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Clinical features do not identify risk of progression from isolated postcapillary pulmonary hypertension to combined pre‐ and postcapillary pulmonary hypertension

Babu

Annis

Garry

et al. 2023

Pulm. circ.

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Pulmonary arterial hypertension‐associated genetic variants in combined post‐capillary and pre‐capillary pulmonary hypertension: a case report

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Clinical features do not identify risk of progression from isolated postcapillary pulmonary hypertension to combined pre‐ and postcapillary pulmonary hypertension

Clinical features do not identify risk of progression from isolated postcapillary pulmonary hypertension to combined pre‐ and postcapillary pulmonary hypertension

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