Treatment of Pulmonary Arterial Hypertension in Connective Tissue Disease — Non-Vasoreactive, Low or Intermediate Risk

This protocol applies to patients with non-vasoreactive pulmonary arterial hypertension who have a negative acute pulmonary vasoreactivity test. The population covered includes idiopathic PAH (IPAH), heritable PAH (HPAH), drug-associated PAH (DPAH), and PAH associated with connective tissue disease (PAH-CTD). Patients are at low or intermediate risk of death and are without cardiopulmonary comorbidities.

This protocol is specifically focused on non-vasoreactive patients with IPAH, HPAH, DPAH, and those with PAH-CTD. The absence of cardiopulmonary comorbidities — such as obesity, hypertension, diabetes mellitus, coronary heart disease, or signs of parenchymal lung disease — is a defining criterion of this patient group.

Treatment Approach

Management of this patient population may involve lung transplantation as a treatment pathway. The specific type of transplantation procedure depends on the patient's individual cardiac status. The complete evidence-based regimen — including full selection criteria, procedure details, and the structured clinical algorithm — is available via the link below.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1093/eurheartj/ehac237

This section describes drug treatment and is focused on non-vasoreactive patients with IPAH/HPAH/DPAH and on patients with PAH associated with connective tissue disease (PAH-CTD).

Cardiopulmonary comorbidities are conditions associated with an increased risk of left ventricular diastolic dysfunction, and include obesity, hypertension, diabetes mellitus, and coronary heart disease; pulmonary comorbidities may include signs of mild parenchymal lung disease and are often associated with a low DLCO (,45% of the predicted value).

Lung transplantation remains an important treatment option for patients with PAH refractory to optimized medical therapy.

Currently, most patients receive bilateral LTx, while combined heart–lung transplantation is reserved for patients who have additional non-correctable cardiac conditions.

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