Pulmonary hypertension screening

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Pulmonary Hypertension Microchapters


Patient Information


Historical Perspective




Differentiating Pulmonary hypertension from other Diseases

Epidemiology & Demographics

Risk Factors


Natural History, Complications and Prognosis


Diagnostic Study of Choice

History & Symptoms

Physical Examination

Laboratory Findings


Chest X Ray



Echocardiography or Ultrasound

Right heart catheterization

Other Diagnostic Studies


Medical Therapy


Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

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Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assistant Editor(s)-in-Chief: Ralph Matar


Patients with a known BMPR2 mutation, scleroderma, and portal hypertension undergoing evaluation for liver transplantation should receive periodic screening for pulmonary hypertension (PH) through a thorough assessment of the presence of symptoms, physical examination, chest X ray, electrocardiography, and echocardiogram. Additional investigation with right heart catheterization should be performed if screening is suggestive of the presence of PH.


Shown below is a table summarizing the recommended screening in several medical conditionas associated with elevated risk for PH.[1][2]

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 that are suggestive of PH include:[3]

  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


  1. ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension
  2. ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension
  3. ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension

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