What is the prognosis of pulmonary arterial hypertension (PAH)?

Updated: Apr 25, 2018
  • Author: Kristin E Schwab, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Answer

The prognosis of patients with pulmonary arterial hypertension (PAH) is variable and depends on the etiology, severity, and treatment.

US registry data suggest a 5-year survival rate of 57% without treatment (from the time of diagnostic right-sided heart catheterization). [13] Risk score calculators for patients with newly diagnosed PAH are available and validated. [13, 14] In general, male sex, age older than 50 years, worse WHO functional status, and right ventricular dysfunction confer a worse prognosis. For example, patients with right-sided heart failure survive approximately 1 year without treatment.

Important to note is that longitudinal trends suggest that survival in patients with PAH has improved. Since the introduction of advanced pharmacotherapies, scleroderma-associated PAH, for example, has seen an improved prognosis. [15] Overall, although, etiology remains important for prognostication, patients with PAH secondary to connective-tissue disease, portal hypertension, and familial causes tend to have worse survival than patients with other etiologies of PAH.


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