title: Predicting Outcomes Using the Heart Failure Survival Score in Adults with Moderate or Complex Congenital Heart Disease.
abstract: BACKGROUND
  Adults with congenital heart disease (CHD) face increased risk for morbidity and mortality with age, but few prognostic models exist.
  
  
  OBJECTIVE
  This study aims to assess whether the Heart Failure Survival Score (HFSS), which risk stratifies patients for heart transplantation, predicts outcomes in adults with moderate or complex CHD.
  
  
  METHODS
  This was a multicenter, retrospective study which identified 441 patients with moderate or complex CHD between 2005 and 2013, of whom 169 had all the HFSS parameters required to calculate the risk score. Because all study patients were deemed low risk by the HFSS, the score was dichotomized at the median (10.4). Outcomes included death, transplant or ventricular assist device (VAD), arrhythmia requiring treatment, nonelective cardiovascular (CV) hospitalizations, and the composite. Associations of mean HFSS and HFSS <10.4 with each outcome were assessed.
  
  
  RESULTS
  The cohort had mean ± standard deviation age of 33.6 ± 12.6 years, peak VO2 21.8 ± 7.5 mL/kg/min, HFSS of 10.45 ± 0.88, and median years follow-up of 2.7 (1.1, 5.2). There were five deaths (2.8%), no transplants or VADs, 25 arrhythmias (14.8%), 22 CV hospitalizations (13%), and 39 composites (23.1%). Lower mean HFSS was observed for patients who died (9.6 ± 0.83 vs. 10.5 ± 0.87, P = .02), arrhythmia requiring treatment (10.0 ± 0.70 vs. 10.5 ± 0.89, P = .005), CV hospitalizations (9.9 ± 0.73 vs. 10.5 ± 0.88, P = .002), and the composite (10.0 ± 0.70 vs. 10.6 ± 0.89, P < .001). The positive and negative predictive values of HFSS <10.4 for the composite were 34% and 88% respectively, with sensitivity and specificity 74% and 56%.
  
  
  CONCLUSIONS
  Although a low HFSS was significantly associated with outcomes, it did not adequately risk stratify adults with CHD, whose heterogeneous pathophysiology differs from that of the acquired heart failure population. Further studies are warranted to provide a more accurate prognosis.
authors.affiliation: Division of Cardiology, Department of Medicine, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Elaine Y
authors.initials: EY
authors.lastname: Lin
authors.affiliation: Department of Epidemiology & Population Health, Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Hillel W
authors.initials: HW
authors.lastname: Cohen
authors.affiliation: Division of Cardiology, Institute for Heart, Vascular, and Stroke Care, Massachusetts General Hospital, Harvard Medical School, Boston, Mass, USA.
authors.forename: Ami B
authors.initials: AB
authors.lastname: Bhatt
authors.affiliation: Division of Cardiology, Institute for Heart, Vascular, and Stroke Care, Massachusetts General Hospital, Harvard Medical School, Boston, Mass, USA.
authors.forename: Ada
authors.initials: A
authors.lastname: Stefanescu
authors.affiliation: Division of Cardiology, Institute for Heart, Vascular, and Stroke Care, Massachusetts General Hospital, Harvard Medical School, Boston, Mass, USA.
authors.forename: David
authors.initials: D
authors.lastname: Dudzinski
authors.affiliation: Division of Cardiology, Institute for Heart, Vascular, and Stroke Care, Massachusetts General Hospital, Harvard Medical School, Boston, Mass, USA.
authors.forename: Doreen
authors.initials: D
authors.lastname: DeFaria Yeh
authors.affiliation: Division of Cardiology, Institute for Heart, Vascular, and Stroke Care, Massachusetts General Hospital, Harvard Medical School, Boston, Mass, USA.
authors.forename: Jacob
authors.initials: J
authors.lastname: Johnson
authors.affiliation: Division of Cardiology, Department of Medicine, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY, USA.|Division of Cardiovascular Medicine, Department of Medicine, Stanford University School of Medicine, Stanford, Calif, USA.
authors.forename: George K
authors.initials: GK
authors.lastname: Lui
mesh_terms: D000328:Adult; D000367:Age Factors; D001900:Boston; D003661:Decision Support Techniques; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000175:diagnosis / Q000401:mortality / Q000601:surgery; D006333:Heart Failure / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality / Q000601:surgery; D016027:Heart Transplantation; D006760:Hospitalization; D006801:Humans; D008297:Male; D008875:Middle Aged; D009518:New York; D011237:Predictive Value of Tests; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D013997:Time Factors; D055815:Young Adult
keywords: ACHD; Model; Morbidity; Mortality; Prognosis; Risk