Treatment of PAH with Cardiopulmonary Comorbidities (HFpEF Risk Factors): Obesity, Hypertension, Diabetes, Coronary Heart Disease, or Low DLCO
Idiopathic, heritable, and drug-associated PAH (IPAH/HPAH/DPAH) in patients who also carry cardiopulmonary comorbidities — particularly risk factors associated with heart failure with preserved ejection fraction — represents a distinct clinical phenotype requiring a carefully individualised management strategy.
Clinical scenario
This protocol applies to patients with IPAH, heritable PAH, or drug-associated PAH who present with one or more of the following cardiopulmonary comorbidities, predominantly seen in elderly patients:
Obesity
Hypertension
Diabetes
Coronary heart disease
Low DLCO (<45% predicted)
Treatment approach
Treatment decisions in this population are stratified according to the presence of these cardiopulmonary comorbidities and disease severity. In patients who remain at elevated risk, additional PAH medication may be considered — but the selection and sequencing of therapy requires individual clinical judgement. The full structured regimen is available via the link below.
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 and according to disease severity assessed by risk stratification.
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.
In patients with IPAH/HPAH/DPAH with cardiopulmonary comorbidities who present at intermediate or high risk of death while receiving PDE5i or ERA monotherapy, additional PAH medication may be considered on an individual basis.
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