Idiopathic Pulmonary Arterial Hypertension

(PPH; Unexplained Pulmonary Hypertension; Idiopathic Pulmonary Hypertension; Pulmonary Arterial Hypertension; Sporadic Primary Pulmonary Hypertension; Familial Primary Pulmonary Hypertension; Primary Pulmonary Hypertension)

Definition

Primary pulmonary hypertension (PPH) is a rare disease. It is high blood pressure in the blood vessels of the lungs.A person with PPH has extra muscle in the walls of these blood vessels. That extra muscle makes it more difficult for blood to flow through them. As a result, the right side of the heart has to work harder to push blood to the lungs. This additional strain can eventually lead to heart failure.PPH is a serious condition. It requires care from your doctor.
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Causes

The cause of PPH is unknown. Several factors may contribute to the development of the disease, including:
  • Autoimmune diseases
  • Exposure to certain drugs or chemicals
  • Genetic defects

Risk Factors

PPH is more common in women aged 30-40 years. Other factors that may increase your risk of PPH include:
  • Liver cirrhosis
  • Portal hypertension
  • HIV infection
  • Family history of PPH
  • Use of appetite suppressants (diet pills)
  • Cocaine use

Symptoms

Initial symptoms of PPH may be minor. They will get progressively worse. PPH may cause:
  • Shortness of breath (when you are active or at rest)
  • Abnormally rapid, deep breathing—hyperventilation
  • Fatigue
  • Progressive weakness
  • Fainting spells
  • Lightheadedness
  • Coughing up blood
  • Bluish tint to the lips and skin—cyanosis
  • Swelling of the legs and hands
  • Chest pain
  • Lack of appetite
  • Cold hands and feet
  • Low blood pressure

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