Health Dictionary
Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Pulmonary hypertension screening
Introduction
Overview
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
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
References
- ↑ Poch D, Mandel J (2021). “Pulmonary Hypertension”. Ann Intern Med. 174 (4): ITC49–ITC64. doi:10.7326/AITC202104200. PMID 33844574 Check
|pmid=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
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