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Pulmonary arterial hypertension

Pulmonary hypertension
Pulmonary hypertensive arteriopathy (4348170715).jpg
Micrograph showing pulmonary hypertensive arteriopathy with marked thickening of the tunica intima and tunica media.
Classification and external resources
Specialty Pulmonology, cardiology
ICD-10 I27.0, I27.2
ICD-9-CM 416.0, 416.8
DiseasesDB 10998
MedlinePlus 000112
eMedicine radio/583 med/1962 Secondary pulmonary hypertension Pediatric primary pulmonary hypertension Persistent newborn pulmonary hypertension
Patient UK Pulmonary hypertension
MeSH D006976
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Pulmonary hypertension (PH or PHTN) is an increase of blood pressure in the pulmonary artery, pulmonary vein, or pulmonary capillaries, together known as the lung vasculature, leading to shortness of breath, dizziness, fainting, leg swelling and other symptoms. Pulmonary hypertension can be a severe disease with a markedly decreased exercise tolerance. It was first identified by Ernst von Romberg in 1891. According to the most recent classification, it can be one of six different types.

The signs/symptoms of pulmonary hypertension include the following:

Less common signs/symptoms include non-productive cough and exercise-induced nausea and vomiting. Coughing up of blood may occur in some patients, particularly those with specific subtypes of pulmonary hypertension such as heritable pulmonary arterial hypertension, Eisenmenger syndrome and chronic thromboembolic pulmonary hypertension.Pulmonary venous hypertension typically presents with shortness of breath while lying flat or sleeping (orthopnea or paroxysmal nocturnal dyspnea), while pulmonary arterial hypertension (PAH) typically does not.

Other typical signs of pulmonary hypertension include an accentuated pulmonary component of the second heart sound, a right ventricular third heart sound, and parasternal heave indicating a hypertrophied right atrium. Signs of systemic congestion resulting from right-sided heart failure include jugular venous distension, ascites, and hepatojugular reflux. Evidence of tricuspid insufficiency and pulmonic regurgitation is also sought and, if present, is consistent with the presence of pulmonary hypertension.


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