Key result
Primary pulmonary hypertension is a severe disease with a mean survival of 2.8 years without treatment, which can be extended to over 10 years with appropriate medical therapy.
Primary pulmonary hypertension is a severe, progressive disease where appropriate medical therapy and lung transplantation can significantly extend survival from 2.8 years to over 10 years.
Emphasizes treatment urgency in PPH; leaves open need for prospective validation in contemporary cohorts.
Primary pulmonary hypertension (PPH) is a severe and progressive disease. It is characterized by persistent elevation of pulmonary artery pressure without any known cause. The hemodynamics demonstrates high pulmonary artery pressure and resistance to blood flow through the lung circulation. PPH is a diagnosis of exclusion. The diagnosis is often delayed because the initial symptoms may be mild and the exam findings subtle. The mean age of survival is 2.8 years without treatment. With treatment patients can survive for more than 10 years. Current lifetime treatment includes anticoagulation, oxygen, digoxin, calcium channel blockers, vasodilators, and diuretics. Promising results have been obtained with prostacyclin and endothelin receptor antagonists. Lung transplantation can be considered for patients with advances PPH and those not responding to medical therapy.
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Berkowitz et al. (2003) conducted a review in Primary pulmonary hypertension. Primary pulmonary hypertension is a severe disease with a mean survival of 2.8 years without treatment, which can be extended to over 10 years with appropriate medical therapy.
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