What to look for if you suspect PAH

PAH often presents with vague or non-specific symptoms such as breathlessness, which may lead to early misdiagnosis.1 It’s important to pursue a definitive diagnosis of unexplained dyspnoea, especially if you have any suspicion that PAH may be the cause.

A thorough physical examination is a good starting point, as this may reveal signs of right ventricle (RV) dysfunction, such as:2
  • Left parasternal lift
  • Accentuated pulmonary component of second heart sound
  • Pansystolic murmur of tricuspid regurgitation
  • Diastolic murmur of pulmonary insufficiency
  • RV third heart sound
In more advanced disease, signs of right heart congestion can include:2
  • Increasing breathlessness
  • Elevated jugular venous pressure
  • Hepatomegaly
  • Ascites
  • Fluid retention
  • Cyanosis
  • Telangiectasia, digital ulceration and sclerodactyly are seen in scleroderma

Questions you can ask patients

The following questions will help you gain a better understanding of the extent of your patient’s breathlessness and how it may be progressing:

Associated diseases and risk factors of PAH can include:2

  • Family history: obtaining a history of unexplained heart disease may help to indicate possible heritable PAH, which is often misdiagnosed
  • Connective tissue disease e.g. systemic sclerosis
  • Congenital heart disease
  • Portal hypertension
  • HIV infection
  • Drug and toxin exposure: definite and possible associations

Click here for more information about the types of PAH.

References
  1. Strange G, et al. Pulm Circ 2013; 3(1):89–94.
  2. Galiè N, et al. Eur Heart J 2016; 37:67–119.
Footnotes
HIV=human immunodeficiency virus; PAH=pulmonary arterial hypertension; PH=pulmonary hypertension; RV=right ventricle; WHO-FC=World Health Organization functional class.

Classification of drugs and toxins associated with PAH

Definite Possible
Aminorex
Fenfluramine
Dexfenfluramine
Benfluorex
Methamphetamines
Dasatinib
Toxic rapeseed oil
Cocaine
Phenylpropanolamine
L-tryptophan
St John’s wort
Amphetamines
Interferon-α and -β
Alkylating agents
Bosutinib
Direct-acting antiviral agents against hepatitis C virus
Leflunomide
Indirubin (Chinese herb Qing-Dai)
Adapted from Simonneau G, et al. 2019.2

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This content is intended for Australian healthcare professionals. For more information on pulmonary hypertension, please contact your healthcare professional.
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World Health Organization functional class (WHO-FC) for patients with PH3,5

This system grades PH severity according to the patient’s functional status, by linking symptoms with activity limitations. WHO-FC remains a powerful predictor of outcomes in patients with PAH.

WHO - FC Description
I
Patients with PH in whom there is no limitation of usual physical activity; ordinary physical activity does not cause increased dyspnoea, fatigue, chest pain, or presyncope.
II
Patients with PH who have mild limitation of physical activity. There is no discomfort at rest, but normal physical activity causes increased dyspnoea, fatigue, chest pain, or presyncope.
III
Patients with PH who have a marked limitation of physical activity. There is no discomfort at rest, but less than ordinary activity causes increased dyspnoea, fatigue, chest pain, or presyncope.
IV
Patients with PH who are unable to perform any physical activity at rest and who may have signs of right ventricular failure. Dyspnoea and/or fatigue may be present at rest, and symptoms are increased by almost any physical activity.
Adapted from McGoon M, et al. 2004.5
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