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

Drug- or Toxin-Associated PAH: What to Do When Withdrawing the Causative Agent Is Not Enough

Clinical Scenario

This protocol addresses patients diagnosed with drug- or toxin-associated PAH — those with a relevant exposure to a drug or toxin associated with PAH, in whom all other causes of pulmonary hypertension have been excluded.

Why This Step Is Reached

Initial management of drug- or toxin-associated PAH centres on immediately discontinuing the presumed causative agent. In patients with mild disease and a low-risk profile, this step alone is observed over 3–4 months.

This protocol is indicated when that approach has not achieved partial or full reversal of PAH, or when pulmonary haemodynamics have not normalised on reassessment — or when the patient presents with more advanced disease at diagnosis.

Next Treatment Step — Partial Overview

PAH-specific therapy is initiated following the same principles applied to other forms of PAH, involving a combination of targeted drug classes. The complete structured regimen — including the full approach and sequencing — is available in the protocol below.

Instant Access to Structured Evidence-Based Regimens

References

It is recommended to make a diagnosis of drug- or toxin-associated PAH in patients who had relevant exposure and in whom other causes of PH have been excluded.

Treatment of DPAH follows the same basic principles as treating other forms of PAH.

Pulmonary arterial hypertension therapy should be initiated in patients who do not normalize their haemodynamics after withdrawing or in patients presenting with more advanced PAH at diagnosis.

DOI: 10.1093/eurheartj/ehac237

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