Treatment of Drug- or Toxin-Associated Pulmonary Arterial Hypertension
This protocol addresses patients diagnosed with pulmonary arterial hypertension in the setting of a relevant prior exposure to a drug or toxin known to be associated with PAH, after other causes of pulmonary hypertension have been systematically excluded.
Drug- or toxin-associated PAH is recognised when the patient has documented exposure to a known causative agent and alternative aetiologies of pulmonary hypertension have been excluded. Both conditions must be met to attribute PAH to the exposure.
The primary intervention involves discontinuing the suspected causative agent; what follows — including when PAH-specific therapy enters the picture — depends on haemodynamic response and risk profile. The complete structured algorithm is available in the full protocol.
The aim is partial or full reversal of PAH following removal of the causative agent, with normalisation of pulmonary haemodynamics reassessed over a defined observation window.
References
DOI: 10.1093/eurheartj/ehac237
- 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.
- In patients with suspected drug- or toxin-associated PAH, it is recommended to immediately discontinue the causative agent whenever possible.
- In patients with mild PH and a low-risk profile, discontinuing the trigger alone may be sufficient, and it is recommended that these patients be observed over 3–4 months before considering PAH therapy.
- Partial or full reversal of PAH has been reported after discontinuing the causative agent, at least for interferons and dasatinib.
- 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.