Treatment of PAH with Connective Tissue Disease — or Idiopathic, Heritable, or Drug-Associated PAH — When Vasoreactivity Is Negative and Risk Is High
This protocol addresses patients with pulmonary arterial hypertension who are confirmed non-vasoreactive on acute testing and are classified at high risk of death, without significant cardiopulmonary comorbidities.
The protocol applies across the following PAH subtypes, all presenting as non-vasoreactive:
- Idiopathic PAH (IPAH)
- Heritable PAH (HPAH)
- Drug-associated PAH (DPAH)
- PAH associated with connective tissue disease
A negative acute pulmonary vasoreactivity test is confirmed in all patients. No significant cardiopulmonary comorbidities accompany the PAH.
High risk of death
Lung transplantation is a central element of the treatment strategy in this high-risk, non-vasoreactive setting.
The complete protocol — including which transplantation approach applies and how patient selection is structured — is available in the full regimen below.
References
DOI: 10.1093/eurheartj/ehac237
In patients with IPAH/HPAH/DPAH who present at high risk of death, initial combination therapy with a PDE5i, an ERA, and i.v./s.c. prostacyclin analogues should be considered.
In patients presenting at high risk, initial triple-combination therapy including an i.v./s.c. prostacyclin analogue should be considered.
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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