Pulmonary Arterial Hypertension


Pulmonary Hypertension (PH) is a progressive lung disorder in which there is an abnormally high blood pressure in the arteries of the lungs. This, over time leads to an increased work on the right side of the heart and if untreated, can lead to right sided heart failure.

The early signs and symptoms of Pulmonary Hypertension are very non-specific and are usually not obvious until the condition has significantly progressed. The initial presenting symptoms are usually easy fatigability and shortness of breath with normal daily activities (such as climbing one flight of stairs). More advanced symptoms can include dizziness, fainting episodes, abdominal and chest pain and swelling of the legs.

There are several causes of Pulmonary Hypertension and the World Health Organization (WHO) categorizes the disease in one of 5 types:

  • WHO Group I:  Pulmonary Arterial Hypertension (PAH)
    • Idiopathic, Familial, Associated to Collagen Vascular Disease, Congenital Shunts, Portal Hypertension, HIV, Drugs, Toxins or other diseases.
  • WHO Group II:  Associated with Left Heart Disease
  • WHO Group III:  Associated with Lung Disease and/or Hypoxemia
  • WHO Group IV:  Associated to Chronic Thrombotic or Embolic Disease
  • WHO Group V:  Miscellaneous

Pulmonary Arterial Hypertension (PAH) refers specifically to a condition in which there is a gradual narrowing in the arteries in the lung that lead to increased resistance and decreased blood flow and over time lead to increased strain and failure of the right side of the heart.

The treatment of Pulmonary Hypertension (PH) varies depending on the etiology and suspected underlying mechanism of disease progression and thus requires an extensive evaluation. The evaluation for Pulmonary Hypertension includes several blood tests, breathing tests (Pulmonary Function Tests), Exercise tests, Lung Scans, Electrocardiograms, Echocardiograms, Sleep Study and Right Sided Heart Catheterization.

Once the diagnosis is established and the etiology is determined, then a treatment plan can be devised with your physician. There is no known cure for Pulmonary Arterial Hypertension.  The available therapies are intended to help improve your symptoms and slow the progression of the disease. There are select advanced pharmacological therapies available that will require a very close monitoring and interaction with your physician. Some of the medications that may be used in managing your Pulmonary Hypertension include: Phosphodiesterase-5 Inhibitors (sildenafil, tadalafil), Endothelin Receptor Blockers (bosentan, ambrisentan), Prostacyclins, Diuretics and Calcium Channel Blockers.  On occasion, patients are started on blood thinners to prevent the formation of blood clots. Ultimately, some patients will require lung transplantation.