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REVEAL Registry: Do Patients Diagnosed in the United States with Familial Pulmonary Arterial Hypertension Differ from Patients Diagnosed with Idiopathic Pulmonary Arterial Hypertension?

C. Gregory Elliott


Robyn Barst


Michelle Turner

David Badesch


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Conference: 2010 International PHA Conference and Scientific Sessions

Release Date: 06.24.2010

Presentation Type: Abstracts

Elliott CG1, Barst RJ2, Turner MP3, Badesch DB4.
1. Intermountain Medical Center & University of Utah, Murray, UT, USA
2. Columbia University College of Physicians & Surgeons, New York, NY, USA
3. ICON Clinical Research, Ellicott City, MD, USA
4. University of Colorado, Denver, CO, USA

BACKGROUND: Familial (F) and Idiopathic (I) are overlapping diagnostic subclasses of WHO Group 1 pulmonary arterial hypertension (PAH). The majority of FPAH patients have mutations in genes that code for TGFbeta cell surface receptors, and thus FPAH may present a unique phenotype. The purpose of the present study was to compare clinical characteristics of FPAH and IPAH patients enrolled in a large US-based registry, the Registry toEValuate Early And Long-term PAH Disease Management (REVEAL).

METHODS: REVEAL enrolled consecutively screened patients diagnosed with WHO Group 1 PAH (confirmed by right heart catheterization) at 54 academic and community centers distributed evenly across 4 US census regions. We compared demographic characteristics and survival of FPAH and IPAH patients. The 2003 (Venice) classification scheme for patients with PAH was used as REVEAL started before the 2009 Dana Point classification scheme was instituted.

RESULTS: FPAH (n=91) and IPAH (n=1439) patients differed with respect to mean age at diagnosis (38 and 47 years, respectively; P< 0.001) due, in part, to shorter times from first symptom to diagnostic catheterization (FPAH quartiles: 3.4, 6.4, 23.5 months; IPAH quartiles: 4.8, 12.4, 35.0; P=0.021). Although younger when diagnosed, FPAH patients had more severe hemodynamic abnormalities at diagnosis, including lower CI (1.9±0.7 and 2.3±0.9 L/min/m2, respectively; P=0.003) and higher mPAP (57±14 and 53±14 mmHg, respectively;P=0.003) and PVRI (29±13 and 23±12 Wood units•m2, respectively; P<0.0001). More FPAH patients were treated with intravenous epoprostenol (36% vs 22%, P=0.002). Two-year survival from enrollment was 81±4% in FPAH and 86±1% in IPAH patients (P=0.181). Two-year freedom from hospitalization was 53±6% in FPAH and 61±1% in IPAH patients (P=0.088).

CONCLUSIONS: In the United States, FPAH patients are diagnosed at an earlier age than IPAH patients; however, FPAH patients have more severe hemodynamic abnormalities at diagnosis. Furthermore, although FPAH is diagnosed at an earlier age, the more severe hemodynamic abnormalities at the time of FPAH diagnosis suggest the need for more active surveillance of family members at risk to develop PAH. However, two years from enrollment, similar proportions of FPAH and IPAH patients remained alive and free from hospitalization; whether this is due to more aggressive treatment of FPAH patients needs further evaluation including longer follow-up.