Pulmonary hypertension

Pulmonary hypertension (PH or PHTN) is a condition of increased blood pressure in the arteries of the lungs.[7] Symptoms include shortness of breath, fainting, tiredness, chest pain, swelling of the legs, and a fast heartbeat.[7][2] The condition may make it difficult to exercise.[7] Onset is typically gradual.[8]

Pulmonary hypertension
Other namesPulmonary arterial hypertension,[1] Ayerza syndrome[2]
Pulmonary hypertension
SpecialtyPulmonology, cardiology
SymptomsChest pain, fatigue[3]
Usual onset20 to 60 years old[4]
DurationLong term[1]
CausesUnknown[1]
Risk factorsFamily history, pulmonary embolism, HIV/AIDS, sickle cell disease, cocaine use, COPD, sleep apnea, living at high altitudes[5][4]
Diagnostic methodFollowing ruling out other potential causes[1]
TreatmentSupportive care, various medications, lung transplantation[1][6]
MedicationEpoprostenol, treprostinil, iloprost, bosentan, ambrisentan, macitentan, sildenafil[1]
Frequency1,000 new cases a year (US)[2]

A patient is deemed to have pulmonary hypertension if the pulmonary mean arterial pressure is greater than 25mmHg at rest, or greater than 30mmHg during exercise.[9]

The cause is often unknown.[1] Risk factors include a family history, prior pulmonary embolism (blood clots in the lungs), HIV/AIDS, sickle cell disease, cocaine use, chronic obstructive pulmonary disease, sleep apnea, living at high altitudes, and problems with the mitral valve.[5][4] The underlying mechanism typically involves inflammation and subsequent remodeling of the arteries in the lungs.[5] Diagnosis involves first ruling out other potential causes.[1]

There is currently no cure for pulmonary hypertension, although research on a cure is ongoing.[7] Treatment depends on the type of disease.[6] A number of supportive measures such as oxygen therapy, diuretics, and medications to inhibit blood clotting may be used.[1] Medications specifically used to treat pulmonary hypertension include epoprostenol, treprostinil, iloprost, bosentan, ambrisentan, macitentan, and sildenafil.[1] Lung transplantation may be an option in severe cases.[6]

The frequency of occurrence is estimated at 1,000 new cases per year in the United States.[4][2] Females are more often affected than males.[2] Onset is typically between 20 and 60 years of age.[4] Pulmonary hypertension was identified by Ernst von Romberg in 1891.[10][1]


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