Pulmonary hypertension (PH) arising in the context of underlying lung disease requires a structured clinical approach that addresses both the elevated pulmonary pressures and the driving pulmonary pathology. The evidence-based protocol for this scenario is defined and sequenced — the key elements are outlined below.

Clinical approach

The primary strategy focuses on optimising management of the underlying lung disease itself, along with targeted interventions for specific contributing physiological factors where indicated. Eligibility for lung transplantation evaluation is also part of the pathway for appropriate patients. There are important evidence-based constraints on the use of certain pulmonary-arterial-hypertension-specific medications in this group — the full criteria and sequencing are in the protocol.

References

DOI: 10.1093/eurheartj/ehac237

The therapeutic approach to group 3 PH starts with optimizing the treatment of the underlying lung disease, including supplementary oxygen and non-invasive ventilation, where indicated, as well as enrolment into pulmonary rehabilitation programmes.
In patients with lung disease and suspected PH, it is recommended to optimize treatment of the underlying lung disease and, where indicated, hypoxaemia, sleep-disordered breathing, and/or alveolar hypoventilation.
It is recommended to refer eligible patients with lung disease and PH for LTx evaluation.
The use of PAH medication is not recommended in patients with lung disease and non-severe PH.

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What is the treatment of pulmonary hypertension secondary to lung disease?

References