Treatment of Non-Vasoreactive Idiopathic, Heritable, or Drug-Associated PAH and PAH Associated with Connective Tissue Disease at Low or Intermediate Risk
This protocol covers management of pulmonary arterial hypertension in patients who test negative on acute pulmonary vasoreactivity testing — encompassing idiopathic, heritable, and drug-associated PAH, as well as PAH associated with connective tissue disease — presenting without cardiopulmonary comorbidities and at low or intermediate risk of death.
Clinical Scenario
- Non-vasoreactive idiopathic (IPAH), heritable (HPAH), or drug-associated (DPAH) PAH, or PAH associated with connective tissue disease (PAH-CTD)
- Negative acute pulmonary vasoreactivity test
- No cardiopulmonary comorbidities
- Low or intermediate risk of death
Approach — partial overview
Management in this population involves augmenting existing oral therapy with a parenteral vasoactive agent, with concurrent specialist referral for advanced evaluation; the complete structured regimen is available via the link below.
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).
- In patients with IPAH/HPAH/DPAH who present at intermediate–high or high risk of death while receiving ERA/PDE5i therapy, the addition of i.v./s.c. prostacyclin analogues and referral for LTx evaluation should be considered.
- In patients who are at intermediate–high or high risk while receiving oral therapies, the addition of i.v. epoprostenol or i.v./s.c. treprostinil and referral for LTx evaluation should be considered.
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