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Previous PAH Registries
The REVEAL Registry is designed to build upon the information collected from previous PAH registries, and it will complement the data collected from a wide variety of current and future randomized clinical trials. In addition to the observational studies mentioned below, other PAH registries exist that seek to measure certain aspects of PAH and population subgroups within the broadly defined classification of patients diagnosed with WHO Group I PAH.
Historically, the incidence of PAH has been difficult to measure. Estimates suggest that more than 100,000 people in the United States suffer from PAH. As PAH research grows, we may find that PAH will be diagnosed more often as diagnostic and treatment options improve.
In 1981, the National Heart, Lung, and Blood Institute (NHLBI) initiated the National Primary Pulmonary Hypertension Patient Registry in order to better understand the disease. The registry enrolled 187 patients from 32 medical centers in the United States through 1985. Patients were followed prospectively for approximately 5 years. Analysis of the data revealed the following characteristics about patients in the study1, 2:
- A 1.7:1 female to male ratio
- The mean age at diagnosis was 36.4 years
- Females tended to present with more severe symptoms
- The mean time from onset of symptoms to diagnosis was 2.03 years
- Right ventricular hypertrophy was found in 87% of patients
- Right atrial pressure was elevated in 72% of patients
- The estimated median survival was 2.8 years
- Single-year survival rates of: 1 year, 68%; 3 years, 48%; and 5 years, 34%.
Subsequent to the NHLBI Registry, data has been published from two European PAH registries. The Pulmonary Hypertension Registry in France3 collected data from 674 adult patients with PAH from 17 university hospitals between 2002 and 2003. The Swiss Registry4 retrospectively collected data from 106 patients with severe primary pulmonary hypertension at four university centers.
References
1. Ann Intern Med, 1987; 107:216-23
2. Ann Intern Med, 1991; 115:343-49
3. Am J Respir Crit Care Med, 2006; 173:1023–30
4. Swiss Med Wkly, 2001; 131:346–50
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