title: Elevated pre-transplant pulmonary vascular resistance is not associated with mortality in children without congenital heart disease: a multicenter study.
abstract: BACKGROUND
  Traditionally, an elevated pulmonary vascular resistance index (PVRI) has been a relative contraindication to pediatric orthotopic heart transplantation. This study examined the risk of elevated pre-transplant PVRI on early (30-day) and intermediate-term mortality in pediatric heart transplant recipients without congenital heart disease (CHD).
  
  
  METHODS
  A review of the prospective multicenter Pediatric Heart Transplant Study registry identified all patients without CHD in whom a pre-transplant PVRI was recorded. Of 35 participating centers, 29 reported heart transplants in children with a markedly elevated PVRI (> 5 WU × m(2), corresponding to the highest quartile). Multiphase parametric analysis was performed, adjusting for potential risk factors to assess the association of PVRI with early and intermediate-term mortality.
  
  
  RESULTS
  Between 1993 and 2011, 1,909 children without CHD underwent heart transplantation at a median age of 9.7 years (range, 1.6 months-18 years). Of those, 795 (42%) had a recorded or calculable pre-transplant PVRI, and PVRI > 5 WU × m(2) was present in 193 patients (24%). For all recipients, median pre-transplant PVRI was 3.15 WU × m(2) (range, 0.4-23 WU × m(2)); 2.8 WU × m(2) in infants < 1 year, 3.5 WU × m(2) in patients aged 1 to 10 years, and 3.0 WU × m(2) in patients aged > 10 years (p = 0.03). Multivariable hazard analysis controlling for graft ischemic time and pre-transplant ventilation showed no association of elevated PVRI with early mortality (relative risk, 1.2; p = 0.66), nor with intermediate mortality when controlled for year of transplant, age, race, and pre-sensitization (relative risk, 0.7; p = 0.27).
  
  
  CONCLUSIONS
  Elevation of PVRI did not affect post-transplant survival in this large, multicenter cohort of pediatric heart transplant recipients without CHD, suggesting that the barrier of elevated PVRI can be successfully overcome in this population.
authors.affiliation: Division of Pediatric Cardiology, Columbia University College of Physicians and Surgeons, New York, New York. Electronic address: mr2306@columbia.edu.
authors.forename: Marc E
authors.initials: ME
authors.lastname: Richmond
authors.affiliation: Seattle Children's Hospital, Seattle, Washington.
authors.forename: Yuk M
authors.initials: YM
authors.lastname: Law
authors.affiliation: Division of Cardiology, Children's Medical Center, University of Texas Southwestern Medical Center, Dallas, Texas.
authors.forename: Bibhuti B
authors.initials: BB
authors.lastname: Das
authors.affiliation: Division of Cardiology, Primary Children's Hospital, Salt Lake City, Utah.
authors.forename: Melanie D
authors.initials: MD
authors.lastname: Everitt
authors.affiliation: Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, Alabama.
authors.forename: Manisha
authors.initials: M
authors.lastname: Kukreja
authors.affiliation: Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, Alabama.
authors.forename: David C
authors.initials: DC
authors.lastname: Naftel
authors.affiliation: Seattle Children's Hospital, Seattle, Washington.
authors.forename: Mariska S
authors.initials: MS
authors.lastname: Kemna
authors.affiliation: Division of Pediatric Cardiology, Duke University School of Medicine, Durham, North Carolina.
authors.forename: Heather T
authors.initials: HT
authors.lastname: Henderson
authors.affiliation: Division of Pediatric Cardiology, Columbia University College of Physicians and Surgeons, New York, New York.
authors.forename: Kimberly
authors.initials: K
authors.lastname: Beddows
authors.affiliation: Division of Pediatric Cardiology, University of Florida, Gainsville, Florida.
authors.forename: F Jay
authors.initials: FJ
authors.lastname: Fricker
authors.affiliation: Children's Healthcare of Atlanta and Emory University School of Medicine, Atlanta, Georgia.
authors.forename: William T
authors.initials: WT
authors.lastname: Mahle
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000401:mortality* / Q000503:physiopathology; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D008297:Male; D057234:Preoperative Period; D011446:Prospective Studies; D011651:Pulmonary Artery / Q000503:physiopathology*; D018570:Risk Assessment / Q000379:methods*; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D014481:United States / Q000453:epidemiology; D014655:Vascular Resistance
keywords: cardiomyopathy; heart transplant outcome; mortality; pediatric; pulmonary vascular resistance index