title: Right Heart Score for Predicting Outcome in Idiopathic, Familial, or Drug- and Toxin-Associated Pulmonary Arterial Hypertension.
abstract: OBJECTIVE
  This study sought to determine whether a simple score combining indexes of right ventricular (RV) function and right atrial (RA) size would offer good discrimination of outcome in patients with pulmonary arterial hypertension (PAH).
  
  
  BACKGROUND
  Identifying a simple score of outcome could simplify risk stratification of patients with PAH and potentially lead to improved tailored monitoring or therapy.
  
  
  METHODS
  We recruited patients from both Stanford University (derivation cohort) and VU University Medical Center (validation cohort). The composite endpoint for the study was death or lung transplantation. A Cox proportional hazard with bootstrap CI adjustment model was used to determine independent correlates of death or transplantation. A predictive score was developed using the beta coefficients of the multivariable models.
  
  
  RESULTS
  For the derivation cohort (n = 95), the majority of patients were female (79%), average age was 43 ± 11 years, mean pulmonary arterial pressure was 54 ± 14 mm Hg, and pulmonary vascular resistance index was 25 ± 12 Wood units × m(2). Over an average follow-up of 5 years, the composite endpoint occurred in 34 patients, including 26 deaths and 8 patients requiring lung transplant. On multivariable analysis, RV systolic dysfunction grade (hazard ratio [HR]: 3.4 per grade; 95% confidence interval [CI]: 2.0 to 7.8; p < 0.001), severe RA enlargement (HR: 3.0; 95% CI: 1.3 to 8.1; p = 0.009), and systemic blood pressure <110 mm Hg (HR: 3.3; 95% CI: 1.5 to 9.4; p < 0.001) were independently associated with outcome. A right heart (RH) score constructed on the basis of these 3 parameters compared favorably with the National Institutes of Health survival equation (0.88; 95% CI: 0.79 to 0.94 vs. 0.60; 95% CI: 0.49 to 0.71; p < 0.001) but was not statistically different than the REVEAL (Registry to Evaluate Early and Long-Term PAH Disease Management) score c-statistic of 0.80 (95% CI: 0.69 to 0.88) with p = 0.097. In the validation cohort (n = 87), the RH score remained the strongest independent correlate of outcome.
  
  
  CONCLUSIONS
  In patients with prevalent PAH, a simple RH score may offer good discrimination of long-term outcome.
authors.affiliation: Division of Cardiovascular Medicine, Stanford University and Stanford Cardiovascular Institute, Palo Alto, California. Electronic address: fhaddad@stanford.edu.
authors.forename: François
authors.initials: F
authors.lastname: Haddad
authors.affiliation: Division of Pulmonary Medicine, VU University Medical Center, Amsterdam, the Netherlands.
authors.forename: Onno A
authors.initials: OA
authors.lastname: Spruijt
authors.affiliation: Department of Anesthesia and Division of Critical Care, Montreal Heart Institute, Montreal University, Montreal, Quebec, Canada.
authors.forename: Andre Y
authors.initials: AY
authors.lastname: Denault
authors.affiliation: Cardiovascular and Thoracic Research Center, Marie-Lannelongue Surgical Center, Université Paris-Sud, Paris, France.
authors.forename: Olaf
authors.initials: O
authors.lastname: Mercier
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Stanford University and Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford, California.
authors.forename: Nathan
authors.initials: N
authors.lastname: Brunner
authors.affiliation: Division of Cardiovascular Medicine, Stanford University and Stanford Cardiovascular Institute, Palo Alto, California.
authors.forename: David
authors.initials: D
authors.lastname: Furman
authors.affiliation: Cardiovascular and Thoracic Research Center, Marie-Lannelongue Surgical Center, Université Paris-Sud, Paris, France.
authors.forename: Elie
authors.initials: E
authors.lastname: Fadel
authors.affiliation: Division of Pulmonary Medicine, VU University Medical Center, Amsterdam, the Netherlands.
authors.forename: Harm J
authors.initials: HJ
authors.lastname: Bogaard
authors.affiliation: Division of Cardiovascular Medicine, Stanford University and Stanford Cardiovascular Institute, Palo Alto, California.
authors.forename: Ingela
authors.initials: I
authors.lastname: Schnittger
authors.affiliation: Division of Cardiovascular Medicine, Stanford University and Stanford Cardiovascular Institute, Palo Alto, California.
authors.forename: Bojan
authors.initials: B
authors.lastname: Vrtovec
authors.affiliation: Division of Cardiovascular Medicine, Stanford University and Stanford Cardiovascular Institute, Palo Alto, California.
authors.forename: Joseph C
authors.initials: JC
authors.lastname: Wu
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Stanford University and Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford, California.
authors.forename: Vinicio
authors.initials: V
authors.lastname: de Jesus Perez
authors.affiliation: Division of Pulmonary Medicine, VU University Medical Center, Amsterdam, the Netherlands.
authors.forename: Anton
authors.initials: A
authors.lastname: Vonk-Noordegraaf
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Stanford University and Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford, California.
authors.forename: Roham T
authors.initials: RT
authors.lastname: Zamanian
mesh_terms: D000328:Adult; D001794:Blood Pressure / Q000502:physiology*; D004452:Echocardiography / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology*; D053208:Kaplan-Meier Estimate; D008297:Male; D011379:Prognosis; D012042:Registries; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D014481:United States / Q000453:epidemiology; D016278:Ventricular Function, Right / Q000502:physiology*
keywords: atrial function; heart failure; outcome; pulmonary hypertension; right heart