Pulmonary arterial hypertension
ICD-10 I27.0 · ICD-11 BB01.0

Treatment of Non-Vasoreactive PAH — Connective Tissue Disease, Idiopathic, Heritable, or Drug-Associated, Without Cardiopulmonary Comorbidities, at Low or Intermediate Risk

This protocol addresses pulmonary arterial hypertension in patients who have tested negative on acute pulmonary vasoreactivity testing and carry a diagnosis of idiopathic, heritable, or drug-associated PAH, or PAH associated with connective tissue disease (PAH-CTD). It is designed for those who are free of cardiopulmonary comorbidities and are assessed at low or intermediate risk of death.

Clinical Scenario

Drug treatment targets non-vasoreactive patients with IPAH, HPAH, or DPAH, and those with PAH associated with connective tissue disease, who have a negative acute vasoreactivity result and no cardiopulmonary comorbidities at presentation.

Negative vasoreactivity test IPAH / HPAH / DPAH / PAH-CTD No cardiopulmonary comorbidities Low risk Intermediate risk
First-Line Approach (partial overview)

For patients at low or intermediate risk in this setting, initial oral combination therapy involving an endothelin receptor antagonist together with a phosphodiesterase-5 inhibitor is recommended. The specific agents, sequencing, monitoring intervals, and escalation criteria are set out in the full structured protocol.

Treatment Goals

The aim is to achieve and maintain a low-risk profile on optimised medical therapy. Risk status is assessed using WHO functional class, six-minute walk distance, and BNP/NT-proBNP levels. Patients who reach and sustain low-risk status continue on their established regimen.

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).

For patients presenting at low or intermediate risk, initial combination therapy with an ERA and a PDE5i is recommended.

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).

Achieving and maintaining a low-risk profile on optimized medical therapy is recommended as a treatment goal in patients with PAH. In patients who achieve a low-risk status with their initial PAH therapy, continuation of treatment is recommended.

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