Treatment of Idiopathic, Heritable, or Drug-Associated PAH in Patients with Cardiopulmonary Comorbidities

This protocol addresses first-line pharmacological management for patients with idiopathic (IPAH), heritable (HPAH), or drug-associated (DPAH) pulmonary arterial hypertension who also carry cardiopulmonary comorbidities that overlap with risk factors for HFpEF.

Clinical scenario: Patients with IPAH, HPAH, or DPAH who present with one or more cardiopulmonary comorbidities — including obesity, hypertension, diabetes, coronary heart disease, and/or a low diffusing capacity of the lungs for carbon monoxide (DLCO <45% of predicted value). These features, which are predominantly encountered in elderly patients, signal a phenotype that overlaps with HFpEF and requires a stratified treatment approach.

Treatment approach (partial overview)

In this setting, initial oral monotherapy is the preferred starting strategy — involving either a phosphodiesterase 5 inhibitor or an endothelin receptor antagonist. The complete regimen, sequencing, and clinical decision points are in the full protocol.

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References

DOI: 10.1093/eurheartj/ehac237

Treatment decisions in patients with IPAH/HPAH/DPAH or PAH-CTD should be stratified according to the presence or absence of cardiopulmonary comorbidities (Section 6.3.4.3) and according to disease severity assessed by risk stratification (Section 6.2.7).

Cardiopulmonary comorbidities are predominantly encountered in elderly patients and include risk factors for HFpEF such as obesity, diabetes, coronary heart disease, a history of hypertension, and/or a low DLCO.

In patients with IPAH/HPAH/DPAH and cardiopulmonary comorbidities, initial monotherapy with a PDE5i or an ERA should be considered.

Initial monotherapy is recommended for most of these patients, with PDE5is being the most widely used compounds according to registry data.

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