Pulmonary hypertension | |
---|---|
Other names | Ayerza syndrome[1] |
Pulmonary hypertension | |
Specialty | Pulmonology, cardiology |
Symptoms | Chest pain, fatigue[2] |
Usual onset | 20 to 60 years old[3] |
Duration | Long term[4] |
Causes | Unknown[4] |
Risk factors | Family history, pulmonary embolism, HIV/AIDS, sickle cell disease, cocaine use, COPD, sleep apnea, living at high altitudes[5][3] |
Diagnostic method | Following ruling out other potential causes[4] |
Treatment | Supportive care, various medications, lung transplantation[4][6] |
Medication | Epoprostenol, treprostinil, iloprost, bosentan, ambrisentan, macitentan, sildenafil[4] |
Frequency | 1,000 new cases a year (US)[1] |
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][1] The condition may make it difficult to exercise.[7] Onset is typically gradual.[8] According to the definition at the 6th World Symposium of Pulmonary Hypertension in 2018, a patient is deemed to have pulmonary hypertension if the pulmonary mean arterial pressure is greater than 20mmHg at rest, revised down from a purely arbitrary 25mmHg, and pulmonary vascular resistance (PVR) greater than 3 Wood units.
The cause is often unknown.[4] 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][3] The underlying mechanism typically involves inflammation and subsequent remodeling of the arteries in the lungs.[5] Diagnosis involves first ruling out other potential causes.[4]
As of 2022[update] there was no cure for pulmonary hypertension,[6] although research to find a cure is ongoing. 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.[4] Medications specifically used to treat pulmonary hypertension include epoprostenol, treprostinil, iloprost, bosentan, ambrisentan, macitentan, and sildenafil, tadalafil, selexipag, riociguat.[4] 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.[3][1] Females are more often affected than males.[1] Onset is typically between 20 and 60 years of age.[3] Pulmonary hypertension was identified by Ernst von Romberg in 1891.[9][4]
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