Pulmonary hypertension secondary to lung disease
ICD-10 I27.2 ICD-11 BB01.2

Treatment of Pulmonary Hypertension Secondary to Lung Disease: When to Refer and How to Approach

Patients with underlying lung disease can develop pulmonary hypertension as a secondary complication. When the severity of PH is suspected to be significant, or when the optimal treatment course is uncertain, the clinical situation requires careful specialist evaluation.

Clinical Situation

This protocol applies to patients with established lung disease in whom pulmonary hypertension is present and either its severity is suspected to be severe, or there is diagnostic or therapeutic uncertainty about how best to manage the PH component of their condition.

Treatment Approach

The recommended approach centres on referral to a specialist pulmonary hypertension centre, where an individualized treatment plan can be developed based on the patient's specific disease context — the full structured regimen is available via the protocol link below.
Complete treatment algorithm, specific interventions, and decision criteria are in the full protocol.
References
DOI: 10.1093/eurheartj/ehac237
  • In patients with lung disease and suspected severe PH, or where there is uncertainty regarding the treatment of PH, referral to a PH centre is recommended.
  • In patients with lung disease and severe PH, an individualized approach to treatment is recommended.
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