What is Pulmonary Hypertension?

Pulmonary hypertension is a diffuse disorder of the pulmonary vasculature that is characterized by an elevated pulmonary artery pressure (a resting pulmonary artery pressure of 25 mm Hg or more). There is an increase in the pulmonary vascular resistance (PVR) in the pulmonary vessels.

The most recent classification of pulmonary hypertension includes five categories (Simonneau G, Robbins IM, Begetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54:S43):

  1. Pulmonary arterial hypertension (PAH)
    1. PAH is characterized by an increase in the pulmonary vascular resistance in the small pulmonary arterioles.
    2. It may be idiopathic, familial, related to drug toxicity, or associated with pulmonary veno-occlusive disease.
  2. Pulmonary hypertension owing to left heart disease
    1. In this category pulmonary hypertension is secondary to pulmonary venous congestion (e.g. left ventricular failure).
  3. Pulmonary hypertension owing to lung diseases and/or hypoxia
    1. In this category pulmonary hypertension is due to hypoxia as seen in a multitude of pulmonary diseases or bodily oxygenation problems (COPD).
  4. Chronic thromboembolic pulmonary hypertension (CTEPH)
    1. CTEPH arises from areas of previous pulmonary embolization wherein the walls of the vessels become thickened, thereby leading to narrowing and eventual obliteration of the vascular lumen.
  5. Pulmonary hypertension with unclear multifactorial mechanisms
    1. In this category pulmonary hypertension is due to a broad range of diseases (sarcoidosis, hematologic disorders, etc.)

In a patient with pulmonary hypertension it is imperative not only to establish the diagnosis, but also to establish the etiology for treatment is predicated on knowing both of these factors.

Read more about Pulmonary Hypertension Symptoms

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