Pulmonary hypertension screening
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assistant Editor(s)-in-Chief: José Eduardo Riceto Loyola Junior, M.D.[2]; Ralph Matar
Overview
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.
Screening
- 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]
Summary
Shown below is a table summarizing the recommended screening in several medical conditions associated with elevated risk for PH.[2][3]
Condition | Recommended screening |
Known BMPR2 mutation | Echocardiogram (yearly) |
BMPR2 mutation in a first degree relative | Genetic counseling BMPR2 genotyping |
Family history for PAH in 2 or more relatives | Genetic counseling BMPR2 genotyping |
Systemic sclerosis | Echocardiogram (yearly) |
Portal hypertension | Echocardiogram if orthotopic liver transplantation is in consideration |
Sickle cell disease | Echocardiogram (yearly) |
Previous use of fenfluramine | Echocardiogram in case of symptoms |
Congenital heart disease | Echocardiogram at the time of diagnosis |
Echocardiography findings
Echocardiography findings that are suggestive of PH include:[4]
- Enlargement of the size of right atrium and right ventricle
- Decrease in the function of the right ventricle
- Displacement of the interventricular septum
- Tricuspid regurgitation
- Presence of pericardial effusion
References
- ↑ Poch D, Mandel J (2021). "Pulmonary Hypertension". Ann Intern Med. 174 (4): ITC49–ITC64. doi:10.7326/AITC202104200. PMID 33844574 Check
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value (help). - ↑ ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension
- ↑ ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension
- ↑ ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension