Pulmonary hypertension screening

Patients with a known BMPR2 mutation, scleroderma, and portal hypertension undergoing evaluation for liver transplantation should receive periodic screening for pulmonary hypertension (PH) through echocardiography.

• Studies have not shown an impact on outcomes with pulmonary hypertension screening;
• Despite that, there is an expert consensus that some groups of patients must be screened for pulmonary hypertension such as:
  • Patients with scleroderma spectrum disorders (especially the ones with corrected DLCO less than 80%);
  • Patients with mutations for a heritable form of PAH;
  • Patients with portal hypertension being considered for organ transplantation;
• These patients must be screened annually with echocardiography.^[1]

Shown below is a table summarizing the recommended screening in several medical conditions associated with elevated risk for PH.^[2][3]

Echocardiography findings that are suggestive of PH include:^[4]

1. Enlargement of the size of right atrium and right ventricle
2. Decrease in the function of the right ventricle
3. Displacement of the interventricular septum
4. Tricuspid regurgitation
5. Presence of pericardial effusion

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