title: Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum: a Congenital Heart Surgeons Society study.
abstract: BACKGROUND
  A bias favoring biventricular (BV) repair exists regarding choice of repair pathway for patients with pulmonary atresia with intact ventricular septum (PAIVS). We sought to determine the implications of moving borderline candidates down a BV route in terms of late functional health status (FHS) and exercise capacity (EC).
  
  
  METHODS
  Between 1987 and 1997, 448 neonates with PAIVS were enrolled in a multi-institutional study. Late EC and FHS were assessed following repair (mean 14 years) using standardized exercise testing and 3 validated FHS instruments. Relationships between FHS, EC, morphology, and 3 end states (ie, BV, univentricular [UV], or 1.5-ventricle repair [1.5V]) were evaluated.
  
  
  RESULTS
  One hundred two of 271 end state survivors participated (63 BV, 25 UV, and 14 1.5V). Participants had lower FHS scores in domains of physical functioning (P < .001) compared with age- and sex-matched normal controls, but scored significantly higher in nearly all psychosocial domains. EC was higher in 1.5V-repair patients (P = .02), whereas discrete FHS measures were higher in BV-repair patients. Peak oxygen consumption was low across all groups, and was positively correlated with larger initial tricuspid valve z-score (P < .001), with an enhanced effect within the BV-repair group.
  
  
  CONCLUSIONS
  Late patient-perceived physical FHS and measured EC are reduced, regardless of PAIVS repair pathway, with an important dichotomy whereby patients with PAIVS believe they are doing well despite important physical impediments. For those with smaller initial tricuspid valve z-score, achievement of survival with BV repair may be at a cost of late deficits in exercise capacity, emphasizing that better outcomes may be achieved for borderline patients with a 1.5V- or UV-repair strategy.
authors: Division of Pediatric Cardiac Surgery, Benioff Children's Hospital, University of California, San Francisco, San Francisco, Calif. Electronic address: tara.karamlou@ucsfmedctr.org.
authors: Tara
authors: T
authors: Karamlou
authors: Division of Cardiovascular Surgery, The Hospital For Sick Children and the University of Toronto, Toronto, Ontario, Canada.
authors: Jeffrey A
authors: JA
authors: Poynter
authors: Department of Cardiovascular Surgery, Children's Hospital of Michigan, Detroit, Mich.
authors: Henry L
authors: HL
authors: Walters
authors: Department of Cardiology, Children's Hospital, Boston, Mass.
authors: Jonathan
authors: J
authors: Rhodes
authors: Department of Cardiovascular Surgery, Children's Hospital of Michigan, Detroit, Mich.
authors: Igor
authors: I
authors: Bondarenko
authors: Division of Cardiology, Department of Pediatrics and Communicable Diseases, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, Mich.
authors: Sara K
authors: SK
authors: Pasquali
authors: Division of Cardiovascular Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pa.
authors: Stephanie M
authors: SM
authors: Fuller
authors: Department of Pediatric Cardiovascular Surgery, Primary Children's Hospital, Salt Lake City, Utah.
authors: Linda M
authors: LM
authors: Lambert
authors: Department of Quantitative Health Sciences, Research Institute, Cleveland Clinic, Cleveland, Ohio.
authors: Eugene H
authors: EH
authors: Blackstone
authors: Department of Pediatric and Congenital Heart Surgery, Cleveland Clinic, Cleveland, Ohio.
authors: Marshall L
authors: ML
authors: Jacobs
authors: Division of Cardiothoracic Surgery, Children's Hospital and Medical Center, Omaha, Neb.
authors: Kim
authors: K
authors: Duncan
authors: Division of Cardiovascular Surgery, The Hospital For Sick Children and the University of Toronto, Toronto, Ontario, Canada.
authors: Christopher A
authors: CA
authors: Caldarone
authors: Division of Cardiovascular Surgery, The Hospital For Sick Children and the University of Toronto, Toronto, Ontario, Canada.
authors: William G
authors: WG
authors: Williams
authors: Division of Cardiology, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors: Brian W
authors: BW
authors: McCrindle
mesh_terms: D002648:Child; D002675:Child, Preschool; D005080:Exercise Test; D005260:Female; D006304:Health Status; D006330:Heart Defects, Congenital / Q000503:physiopathology* / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D018633:Pulmonary Atresia / Q000503:physiopathology* / Q000601:surgery*; D013997:Time Factors
references: Am Heart J. 2006 Sep;152(3):427-33
references: 16923408
references: Med Care. 2001 Aug;39(8):800-12
references: 11468499
references: Pediatrics. 2010 Sep;126(3):498-508
references: 20805147
references: Ann Thorac Surg. 2004 Apr;77(4):1359-65; discussion 1365
references: 15063266
references: J Thorac Cardiovasc Surg. 2007 Dec;134(6):1429-36; discussion 1436-7
references: 18023658
references: Acta Paediatr. 2002;91(3):348-54
references: 12022311
references: Circulation. 2010 Jan 5;121(1):34-42
references: 20026781
references: J Am Coll Cardiol. 1990 May;15(6):1424-32
references: 2329245
references: J Adolesc Health. 2001 Aug;29(2):140-9
references: 11472873
references: Cardiol Young. 2001 Jul;11(4):431-8
references: 11558953
references: J Thorac Cardiovasc Surg. 2005 Oct;130(4):1071
references: 16214522
references: Circulation. 2003 Aug 19;108(7):857-62
references: 12900343
references: Eur J Cardiothorac Surg. 2003 Oct;24(4):516-20
references: 14500068
references: J Thorac Cardiovasc Surg. 2009 Nov;138(5):1160-6
references: 19837219
references: J Thorac Cardiovasc Surg. 1999 Dec;118(6):1046-55
references: 10595977
references: Circulation. 2006 Feb 28;113(8):1123-9
references: 16490823
references: Am J Cardiol. 2006 Jun 1;97(11):1638-43
references: 16728229
references: Eur J Echocardiogr. 2009 Jan;10(1):44-5
references: 18827032
references: J Thorac Cardiovasc Surg. 2004 Apr;127(4):1000-7; discussion 1007-8
references: 15052196
references: Ann Thorac Surg. 2006 Jun;81(6):2250-7; discussion 2258
references: 16731162
references: Cardiol Young. 2010 Dec;20(6):631-40
references: 20619060
references: J Thorac Cardiovasc Surg. 1993 Mar;105(3):406-23, 424-7; discussion 423-4
references: 8445920
references: J Thorac Cardiovasc Surg. 2004 Nov;128(5):710-7
references: 15514598
references: J Thorac Cardiovasc Surg. 2011 Jul;142(1):136-41
references: 21277589
references: J Am Coll Cardiol. 2008 Jul 8;52(2):99-107
references: 18598887
references: Pediatr Cardiol. 2006 Mar-Apr;27(2):191-8
references: 16261271
references: Cardiol Young. 2004 Jun;14(3):299-308
references: 15680024
references: J Thorac Cardiovasc Surg. 2012 Mar;143(3):569-75
references: 21908009
references: Heart. 2005 Apr;91(4):517-20
references: 15772218