Childen's Hospital Boston 300 Longwood Avenue
Boston, MA 02115
(617) 355-6000
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Clinical Services (Pulmonary Hypertension Program):
Causes & Symptoms
Pulmonary hypertension can be divided into five categories, based on what can be determined about the cause.
Pulmonary arterial hypertension (PAH)
This type of pulmonary hypertension includes:
  • Idiopathic pulmonary hypertension -- When pulmonary hypertension occurs and there is no known cause, it is referred to as idiopathic pulmonary hypertension.

  • Familial pulmonary hypertension -- When pulmonary hypertension is inherited, it is referred to as familial pulmonary hypertension. It is estimated that at least 10 percent of pulmonary hypertension cases occur in families where at least one other person had it.

  • PAH related to other causes -- When pulmonary hypertension occurs as a result of exposure to toxins or the effects of other diseases, it is referred to as associated pulmonary hypertension. Causes include:

    • diseases such as scleroderma, sickle cell disease and lupus
    • heart diseases that cause shunting of extra blood through the lungs, such as patent ductus, ventricular septal defect and atrial septal defect
    • HIV infection
    • liver disease
    • diet drugs like fenfluramine and dexfenfluramine ("Fen-phen")

Pulmonary venous hypertension
This type of pulmonary hypertension is caused by diseases of the left side of the heart, including mitral valve disease and pulmonary vein stenosis.
Pulmonary hypertension associated with disorders of the respiratory system or hypoxemia
This type of pulmonary hypertension is caused by an inadequate amount of oxygen in the bloodstream as a result of either lung disease, exposure to high altitude or breathing disorders such as chronic lung disease, sleep apnea, emphysema and bronchitis.
Pulmonary hypertension caused by chronic thrombotic or embolic disease
This type of pulmonary hypertension is caused by blood clots in the pulmonary arteries, pulmonary embolism or sickle cell disease.
Pulmonary hypertension caused by disorders directly affecting the pulmonary blood vessels
This type of pulmonary hypertension is caused by inflammatory diseases such as schistosomiasis, sarcoidosis or pulmonary capillary disease.

Symptoms
Symptoms of pulmonary hypertension include:
  • shortness of breath with mild exertion
  • fatigue
  • chest pain or pressure
  • dizzy spells and fainting
  • fast heartbeat
  • ankle or leg swelling (edema)
  • bloating
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