Pulmonary hypertension history and symptoms

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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: Lisa Prior; José Eduardo Riceto Loyola Junior, M.D.[2]


The hallmark of pulmonary hypertension is progressive dyspnea. The most common symptoms of pulmonary hypertension include dyspnea, fatigue, chest pain and syncope or presyncope. Ortner syndrome may also be seen (characterized by hoarseness due to compression of the left laryngeal nerve caused by enlargement of the pulmonary artery).


The history should be focused on the areas including:[1]

  • The average time from the onset of the initial symptoms of PH to the diagnosis is approximately 2 years.[2] This can be attributed primarily due to the non-specificity of symptoms and the considerable overlap with symptoms of other pulmonary and cardiovascular diseases. Therefore, a detailed clinical history must be obtained.
  • Pulmonary arterial hypertension (PAH) does not typically present with orthopnea or paroxysmal nocturnal dyspnea, while pulmonary venous hypertension typically does.
  • Also, a history of exposure to cocaine, methamphetamine, alcohol leading to cirrhosis, and smoking leading to emphysema are considered significant.


Symptoms of pulmonary hypertension include:[1]

WHO Functional Classification

Clinically, a patient may be categorized based on the severity of symptoms into a particular class using the WHO modified functional classification system for pulmonary hypertension (modified from NYHA functional classification system for heart failure). The baseline WHO functional classification is used for the assessment of the severity of PH in order to tailor the choice of therapy. Shown below is a table summarizing the different functional classes.[5]

Class Description
  • No limitation of usual physical activity
  • No increased dyspnea, fatigue, chest pain, or presyncope upon ordinary physical activity
  • Mild limitation of physical activity
  • No discomfort at rest
  • Increased dyspnea, fatigue, chest pain, or presyncope upon normal physical activity
  • Marked limitation of physical activity
  • No discomfort at rest
  • Increased dyspnea, fatigue, chest pain, or presyncope upon less than ordinary activity
  • Inability to perform any physical activity at rest with/without signs of right ventricular failure
  • Dyspnea and/or fatigue may be present at rest
  • Increased dyspnea, fatigue, chest pain, or presyncope by almost any physical activity


  1. 1.0 1.1 1.2 Budev MM, Arroliga AC, Jennings CA (2003). "Diagnosis and evaluation of pulmonary hypertension". Cleve Clin J Med. 70 Suppl 1: S9–17. PMID 12716138. Unknown parameter |month= ignored (help)
  2. 2.0 2.1 Rich S, Dantzker DR, Ayres SM; et al. (1987). "Primary pulmonary hypertension. A national prospective study". Ann. Intern. Med. 107 (2): 216–23. PMID 3605900. Unknown parameter |month= ignored (help)
  3. 3.0 3.1 Carolyn H. Welsh; Michael E. Hanley (2003). Current diagnosis & treatment in pulmonary medicine. New York: Lange Medical Books / McGraw-Hill. ISBN 0-07-121971-4.
  4. Kawut SM, Silvestry FE, Ferrari VA; et al. (1999). "Extrinsic compression of the left main coronary artery by the pulmonary artery in patients with long-standing pulmonary hypertension". Am. J. Cardiol. 83 (6): 984–6, A10. PMID 10190427. Unknown parameter |month= ignored (help)
  5. Rich S, Rubin LJ, Abenhail L, et al. Executive summary from the World Symposium on Primary Pulmonary Hypertension 1998, Evian, France, September 6-10, 1998. Geneva: The World Health Organization.

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