Diagnosis of Pulmonary Hypertension

Publication Date: December 1, 2018
Last Updated: March 14, 2022

Diagnostic approach in patients with clinical suspicion for PH/pulmonary arterial hypertension

Established diagnostic tools

Electrocardiography

Since the US National Institutes of Health (NIH) registry report on primary PH in 1987, the ECG has been considered a reliable clue to the presence of PH.
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Blood tests and immunology

Blood tests are not useful for PH diagnosis, but distinguish some forms of PH and indicate end-organ compromise.
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Pulmonary function tests and arterial blood gases

Pulmonary function tests should include total lung capacity and diffusing capacity of the lung for carbon monoxide (DLCO).
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Cardiopulmonary exercise testing

Cardiopulmonary exercise testing (CPET) for diagnostic purposes can be done non-invasively or with haemodynamic testing.
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Transthoracic echocardiography

The transthoracic echocardiogram (TTE) remains the most important non-invasive screening tool and right heart catheterisation (RHC) remains mandatory to establish the diagnosis
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Ventilation/perfusion lung scanning

A normal V/Q scan remains the preferred diagnostic tool and rules out CTEPH.
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Chest computed tomography

Chest computed tomography (CT) demonstrating right ventricular dilation, right atrial dilation, enlarged main pulmonary artery (diameter ⩾29 mm) or a main pulmonary artery/ascending aorta diameter ratio ⩾1 is suggestive of PH. High-resolution non-contrast examination can identify parenchymal lung disease and discriminate between PH lung disease and PAH (group 3 versus group 1).
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Practice recommendations (including high-risk population screening recommendations)

Scleroderma (systemic sclerosis) and scleroderma spectrum

For patients with SSc and SSc spectrum with uncorrected DLCO 1.6 (assuming none-to-mild interstitial lung disease) and >2-fold upper limit of normal of NT-proBNP. If any of these screening tests are positive, these patients should be referred for RHC. For those with uncorrected DLCO ⩾80% of predicted, screening may be considered with TTE.
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HIV

Screen for PAH in HIV patients with symptoms or with more than one risk factor for HIV-PAH.
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Heritable

Genetic counselling of all idiopathic, anorexiant and familial PAH patients and first-generation asymptomatic family members of patients with known genetic mutations.
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Subsequent evaluations for PAH should be offered (e.g. CPET and TTE), in mutation-positive individuals.
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National databases for genotyping all PAH patients should be advocated by the WSPH. Biobanking of samples and/or genotyping should be mandated in future interventional studies in PAH patients and possibly in PH patients.
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Other heritable PH

Hereditary haemorrhagic telangiectasia

In symptomatic patients and those with heart failure or hepatic arteriovenous malformations, with HHT or family history of HHT, genetic testing and an echocardiogram should be undertaken. If TTE positive (tables 1 and 2) or suggestive of PH, RHC should be undertaken to distinguish the aetiology of PH.
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Portopulmonary hypertension

Echocardiographic screening is recommended in all patients with portal hypertension. If a tricuspid regurgitant jet of >3.4 m·s−1 or right atrial or right ventricular enlargement or dysfunction is found, then further evaluation with RHC and referral to PH expert centre is recommended.
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Congenital heart disease

Post-operative PAH screening in subgroup 4 should include clinical and echocardiographic and ECG screening during follow-up visits 3–6 months after correction and then throughout their planned long-term cardiological follow-up. Annual screening should be planned for corrected patients who presented with increased baseline pulmonary vascular resistance or with combinations of other predisposing factors.
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Recommendation Grading

Disclaimer

Overview

Title

Diagnosis of Pulmonary Hypertension

Authoring Organization

Publication Month/Year

December 1, 2018

Last Updated Month/Year

March 16, 2023

Document Type

Other

External Publication Status

Published

Country of Publication

European

Inclusion Criteria

Female, Male, Adolescent, Adult, Older adult

Health Care Settings

Ambulatory, Emergency care, Hospital, Medical transportation, Operating and recovery room

Intended Users

Social worker, respiratory therapist, physician, nurse, athletics coaching, nurse practitioner, physician assistant

Scope

Assessment and screening, Diagnosis, Prevention, Management, Treatment

Diseases/Conditions (MeSH)

D006976 - Hypertension, Pulmonary

Keywords

pulmonary hypertension, electrocardiogram (ECG), Transthoracic echocardiography, aterial hypertension, cardiopulmonary, Scleroderma, Scleroderma spectrum