title: Four- and seven-year outcomes of patients with congenital heart disease-associated pulmonary arterial hypertension (from the REVEAL Registry).
abstract: Uncorrected congenital heart disease (CHD) frequently leads to pulmonary arterial hypertension (PAH), the most severe form of which is Eisenmenger syndrome (ES). We compared patients with idiopathic or heritable PAH (IPAH or HPAH; n = 1,626) against those with CHD-associated PAH (n = 353) who were enrolled in the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL Registry). Of patients with CHD-associated PAH, 151 had ES. Compared with the IPAH or HPAH cohort, the ES cohort had greater systemic blood flow (2 ± 1 vs 3 ± 2 L/min/m(2), p <0.001), lower mean right atrial pressure (10 ± 6 vs 7 ± 4 mm Hg, p <0.001), higher mean pulmonary artery pressure (53 ± 14 vs 65 ± 17 mm Hg, p <0.001), higher pulmonary vascular resistance index (22 ± 12 vs 32 ± 31 Wood units × m(2), p <0.001), and lower systemic arterial oxygen saturation at rest (92 ± 11% vs 84 ± 13%, p <0.001). At 4 years from enrollment and 7 years from diagnosis, survival rate was similar between IPAH or HPAH and CHD-associated PAH cohorts. For the overall CHD-associated PAH cohort, longer 6-minute walk distance, lower mean right atrial pressure, brain natriuretic peptide level <50 pg/ml, and the presence of acute vasoreactivity were predictors of survival at 4 years from enrollment; younger age and lower mean right atrial pressure were predictors of survival at 7 years from diagnosis. In conclusion, these observations support predicted physiologic differences (e.g., hemodynamics) between patients with IPAH or HPAH and patients with CHD-associated PAH, with or without a systemic-pulmonary shunt. These differences, however, did not translate into significantly improved 4- and 7-year survival rates in patients with ES versus IPAH or HPAH and CHD-associated PAH.
authors.affiliation: Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, NewYork.
authors.forename: Robyn J
authors.initials: RJ
authors.lastname: Barst
authors.affiliation: Department of Pediatrics, University of Colorado and Children's Hospital Colorado, Aurora, Colorado.
authors.forename: D Dunbar
authors.initials: DD
authors.lastname: Ivy
authors.affiliation: ICON Late Phase & Outcomes Research, San Francisco, California.
authors.forename: Aimee J
authors.initials: AJ
authors.lastname: Foreman
authors.affiliation: Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Michael D
authors.initials: MD
authors.lastname: McGoon
authors.affiliation: Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, NewYork. Electronic address: esb14@columbia.edu.
authors.forename: Erika B
authors.initials: EB
authors.lastname: Rosenzweig
mesh_terms: D000328:Adult; D006328:Cardiac Catheterization; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D006439:Hemodynamics / Q000502:physiology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D015994:Incidence; D008297:Male; D011379:Prognosis; D012042:Registries; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D014481:United States / Q000453:epidemiology; D055815:Young Adult