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Primary Pulmonary Hypertension (PPH)

What is primary pulmonary hypertension (PPH)?

To better understand PPH, it is helpful to break it down:
  • Primary: Main, central, or chief
  • Pulmonary: Related to, or affecting, the lungs
  • Hypertension: A common disorder in which blood pressure remains abnormally high (a reading of 140/90 mm Hg or greater)
Putting this all together, primary pulmonary hypertension is a progressive disease characterized by raised pulmonary vascular resistance (calculations of blood pressure, blood flow, and cardiac function). PPH is an unexplained (or “idiopathic”) disease. PPH occurs most commonly in young and middle-aged women, but it does occur in men, too. PPH runs in families in roughly 10% of cases, which suggests that there is a genetic predisposition present.

What are the symptoms of PPH?

The most common symptom of PPH is difficult or labored breathing, known as dyspnea. Other symptoms include:
  • Racing pulse or heart palpitations
  • Swelling in the ankles, legs, and eventually the abdomen
  • Chest pressure or pain
  • Dizziness or fainting spells
  • Fatigue

How is PPH diagnosed?

PPH is usually difficult to diagnose since all other possible diseases need to be investigated first to diagnose PPH through process of elimination. A PPH diagnosis can be made with a high degree of accuracy if care is taken to exclude all likely secondary causes. A good start to diagnosis is a thorough and detailed history and physical examination. There are a number of appropriate tests that will help your doctor uncover potential contributing factors, and these tests include:

What are the symptoms and/or secondary causes of PPH?

What are the available treatments for PPH?

There is no cure for PPH, nor is there a consistently accepted or successful therapeutic approach. However, treatment for this disease has improved dramatically over the past decade. Because PPH is a rare disease whose complexity poses tremendous challenges to the treating physician, it is recommended that patients be referred to a center with experience in management of this disease. The referring physician plays a major role in the day-to-day care of these patients, and an ongoing conversation between the treating physicians is crucial.

Where can I find more information on primary pulmonary hypertension (PPH)?

Primary Pulmonary Hypertension (PPH) Articles