Chest
Volume 126, Issue 1, Supplement, July 2004, Pages 78S-92S
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Prognosis of Pulmonary Arterial Hypertension*: ACCP Evidence-Based Clinical Practice Guidelines

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Although idiopathic pulmonary arterial hypertension is perceived as a progressive disease with a uniformly poor outcome, the natural history of disease is heterogeneous, with some patients dying within months of diagnosis and others living for decades. The course of the disease has also been altered by advances in medical therapies. The outcome of patients with other types of pulmonary arterial hypertension (PAH) has been less well characterized. Assessment of prognosis of such patients is important, as it influences both medical therapy and referral for transplantation. This chapter will provide evidence based recommendations to assess the prognosis of patients with PAH.

Section snippets

Materials and Methods

We conducted a computerized search of the MEDLINE bibliographic database from 1992 to October 2002. We searched using the term hypertension, pulmonary. The search was limited to articles concerning human subjects that were published in the English language and accompanied by an abstract. In addition, we searched the reference lists of included studies, practice guidelines, systematic reviews, and meta-analysis, and consulted with clinical experts to identify relevant studies missed by the

Expected Survival in PAH

The natural history of IPAH has been well described. The National Institutes of Health (NIH) Registry followed up 194 patients with IPAH enrolled at 32 clinical centers from 1981 to 1985.1 The estimated median survival was 2.8 years, with 1-year, 3-year, and 5-year survival rates of 68%, 48%, and 34%, respectively. Other series have studied the natural history of IPAH with similar results. Among a cohort of 61 IPAH patients followed up in Mexico, the mean survival was 25.9 ± 20.7 months (± SD]).

Demographics

Data regarding the prognostic implications of demographic variables such as age, gender, and time of onset of symptoms to diagnosis are inconsistent. The NIH Registry was the first large-scale evaluation of prognostic factors in IPAH. Age, time from onset of symptoms to diagnosis, and gender were not predictive of survival.1 In a retrospective, single center, uncontrolled case series4 of 61 patients with IPAH from India, younger age was associated with a worse prognosis. It should be noted that

Recommendations

As the majority of the evidence reviewed above is applicable to patients with IPAH, the following recommendations pertain to patients with IPAH. In most instances, data are insufficient to make recommendations for patients with PAH due to diagnosis other than IPAH. In patients with IPAH, the following parameters, as assessed at baseline, may be used to predict a worse prognosis:

  • 1.

    Advanced NYHA-FC. Quality of evidence: good; net benefit: substantial; strength of recommendation: A.

  • 2.

    Low 6MWT

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