Congenital Heart DiseaseFour- and Seven-Year Outcomes of Patients With Congenital Heart Disease–Associated Pulmonary Arterial Hypertension (from the REVEAL Registry)
Section snippets
Methods
The design and baseline characteristics of the patients enrolled in the REVEAL Registry have been previously described.7, 8 REVEAL, a longitudinal registry involving 55 pulmonary hypertension centers in the United States (university affiliated and community hospitals), was designed to provide current information about the demographics, course, and management of newly and previously diagnosed patients with World Health Organization group 1 PAH. The data download for this analysis occurred on
Results
From March 30, 2006 to December 8, 2009, 3,515 patients with newly and previously diagnosed PAH were enrolled in REVEAL. The study cohort consisted of 1,979 patients (Figure 1), of whom 1,626 had IPAH (n = 1,530) or HPAH (n = 96), and 353 had CHD-associated PAH (hereafter referred to as CHD). Within the CHD group, most (n = 248) were unrepaired or partially repaired, including 151 patients classified as having ES, 91 patients classified as not having ES, and 6 patients for whom ES
Discussion
This analysis of REVEAL showed that despite hemodynamic differences between the IPAH or HPAH, repaired CHD, and ES cohorts, survival rate at 4 years from enrollment was similar across groups and survival rate at 7 years from diagnosis was similar between the IPAH or HPAH and ES cohorts. These findings appear to be incongruous with previous studies showing both slower PAH disease progression18 and significantly better survival rate5, 19 in patients with ES than in patients with IPAH or HPAH. It
Acknowledgment
Assistance in manuscript development was provided by Anna Lau, PhD, of Percolation Communications LLC. Ginny Lai of ICON Late Phase & Outcomes Research provided statistical programming support.
The authors are saddened to report the passing of Robyn J. Barst, MD, in April 2013. She was an esteemed physician, investigator, and colleague, and a distinguished leader in the field of pediatric pulmonary hypertension. Her contributions to the field are invaluable.
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2021, CJC OpenCitation Excerpt :However, as an increasing number of targeted drug therapies are being covered by national health insurance since 2020, new guidelines have been released, and PAH therapy has entered a new era in China. Previous reports have suggested that patients with postoperative PAH have a similar8,17 or even worse9 prognosis compared to patients with idiopathic PAH, based on smaller cohort studies.18,19 In the current study, our 2 cohorts shared significant similarities in clinical features, especially invasive hemodynamics, but their midterm prognosis differed significantly.
Institution where work was performed: 55 participating sites across the United States.
Funding and support for the REVEAL Registry was provided by Cotherix, Inc. and its affiliate Actelion Pharmaceuticals US, Inc. Funding for preparation of this manuscript was provided by Actelion Pharmaceuticals US, Inc., South San Francisco, California.
See page 154 for disclosure information.
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Deceased.