Pulmonary hypertension screening

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Pulmonary Hypertension Microchapters

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

Overview

Patients with a known BMPR2 mutation, scleroderma, and portal hypertension undergoing evaluation for liver transplantation should receive periodic screening through a thorough assessment of the presence of symptoms, physical examination, chestX ray, as well as elctrocardiography. Patients with HIV infection should not be screened for PH in the absence of any suggestive findings.[1]

Screening

Patients who are considered at high risk for the development of pulmonary hypertension and require periodic screening include:

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

These are the parameters that should be evaluated on Echocardiogram:

  1. Right atrial and right ventricular enlargement.
  2. Reduced right ventricular function.
  3. Displacement of the interventricular septum.
  4. Tricuspid regurgitation(TR).
  5. Tei index or myocardial performance index (measured by Doppler ultrasound).
  6. Pericardial effusion (presence also indicates a higher mortality rate).

The Tei index is an index of combined right ventricular systolic and diastolic function obtained by dividing the sum of both isovolumetric contraction and relaxation intervals by the ejection time.

References

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

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