Usefulness of the Seattle Heart Failure Model to Identify Adults With Congenital Heart Disease at High Risk of Poor Outcome Ada Stefanescu, MDCMa, Eric A. Macklin, PhDc, Elaine Lin, MDd, David M. Dudzinski, MD, JDa, Jacob Johnson, MDb, Kevin F. Kennedy, MSc, Daniel Jacoby, MDe, Doreen DeFaria Yeh, MDa, Gregory D. Lewis, MDa, Robert W. Yeh, MD, MSca, Richard Liberthson, MDa, George Lui, MDf, and Ami B. Bhatt, MDa,* Our objective was to determine whether the Seattle Heart Failure Model (SHFM) differentiates patients with adult congenital heart disease (ACHD) at high versus low risk for cardiovascular outcomes and poor exercise capacity. The ACHD population is growing and presents increasingly for care in the community and at tertiary centers. Few strategies exist to identify the patients with ACHD at high risk for heart failure and mortality.We studied 153 adults with transposition of the great arteries, Ebstein anomaly, tetralogy of Fallot, double outlet right ventricle, and single ventricle from 2 ACHD centers. The primary outcome was cardiovascular death, with a secondary composite outcome of death, transplant, ventricular assist device, cardiovascular admission, and treatment for arrhythmia. We defined risk groups based on SHFM 5-year predicted survival: high (predicted survival 85%). Ten patients had the primary outcome of death, and 46 the combined end point. The hazard of death in the SHFM highversus the intermediate-risk group was 7.09 (95% confidence interval 1.5 to 33.4, p [ 0.01; no deaths in the low-risk group) and the hazard of the composite outcome between the highversus low-risk group was 6.64 (95% confidence interval 2.5 to 17.6, p [ 0.0001). KaplanMeier survival analysis showed greater probability of all-cause mortality (p [ 0.003) in the high-risk group. In conclusion, the SHFM can help identify subjects with ACHD at risk for adverse outcome and poor cardiopulmonary efficiency. This may add to the care of patients with ACHD in the community and streamline care at tertiary centers. Ó 2014 Elsevier Inc. All rights reserved. (Am J Cardiol 2014;113:865e870) Patients with adult congenital heart disease (ACHD) are at increased risk for mortality and morbidity, with a significant incidence of heart failure.1e5 These patients are seen increasingly in local practices6; however, there is no comprehensive validated model to help cardiologists identify high-risk patients. Cardiopulmonary exercise testing (CPET) is used as a surrogate marker for outcomes in ACHD7e16; it, however, requires expensive equipment and specific expertise and is therefore not widely available to the general cardiologists caring for the ACHD population in the community. The Seattle Heart Failure Model (SHFM) is a validated prediction model that estimates survival in adult patients with heart failure by using commonly obtained clinical and laboratory variables.17 To determine whether the SHFM could serve as a useful prognostic model in the ACHD population, we evaluated the extent to which the a Division of Cardiology, bDepartment of Medicine, and cBiostatistics Center, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts; dDivision of Cardiology, Montefiore Medical Center and Albert Einstein College of Medicine, Bronx, New York; eDivision of Cardiology, Yale University Medical Center, New Haven, Connecticut; and fDivision of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, California. Manuscript received August 30, 2013; revised manuscript received and accepted November 9, 2013. See page 869 for disclosure information. *Corresponding author: Tel: (617) 726-8510; fax: (617) 643-6844. E-mail address: [email protected] (A.B. Bhatt).

0002-9149/14/$ - see front matter Ó 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.amjcard.2013.11.043

SHFM differentiates high-risk from low-risk patients with ACHD and correlates with the CPET peak oxygen consumption (pVO2) in a population of patients with ACHD at 2 tertiary referral centers. Methods We retrospectively assessed the medical records of 153 patients with ACHD (aged >18 years) with complex congenital heart diagnoses which carry a high risk of heart failure,3e5 including transposition of the great arteries with a systemic right ventricle (TGA; congenitally corrected TGA and atrial switch), Ebstein anomaly, tetralogy of Fallot (TOF), double outlet right ventricle, and single ventricle (SV) physiology, from 2008 to 2012 at the Massachusetts General Hospital (Boston, Massachusetts) and Montefiore Medical Center (Bronx, New York), who had sufficient documentation and data for analysis of SHFM and outcomes. All patients with TOF had their primary surgical repair before inclusion in the study. A subset of 112 patients had been referred by their cardiologist for CPET as part of routine clinical follow-up protocols. This study was approved by both institutions’ local ethics committees. All patients were monitored at least annually. The primary outcome of the study was cardiovascular death; the secondary outcome was a composite of cardiovascular death, listing for transplant, placement of a ventricular assist device, cardiovascular hospitalizations (for arrhythmia or www.ajconline.org

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Usefulness of the Seattle Heart Failure Model to identify adults with congenital heart disease at high risk of poor outcome.

Our objective was to determine whether the Seattle Heart Failure Model (SHFM) differentiates patients with adult congenital heart disease (ACHD) at hi...
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