Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum: A Congenital Heart Surgeons Society study

Tara Karamlou, Jeffrey A. Poynter, Henry L. Walters, Jonathan Rhodes, Igor Bondarenko, Sara K. Pasquali, Stephanie M. Fuller, Linda M. Lambert, Eugene H. Blackstone, Marshall L. Jacobs, Kim Duncan, Christopher A. Caldarone, William G. Williams, Brian W. McCrindle

Research output: Contribution to journalArticle

19 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)1018-1027.e3
JournalJournal of Thoracic and Cardiovascular Surgery
Volume145
Issue number4
DOIs
StatePublished - Apr 2013

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Exercise Test
Health Status
Exercise
Tricuspid Valve
Pulmonary Atresia with Intact Ventricular Septum
Surgeons
Oxygen Consumption
Survivors
Newborn Infant
Costs and Cost Analysis
Survival

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum : A Congenital Heart Surgeons Society study. / Karamlou, Tara; Poynter, Jeffrey A.; Walters, Henry L.; Rhodes, Jonathan; Bondarenko, Igor; Pasquali, Sara K.; Fuller, Stephanie M.; Lambert, Linda M.; Blackstone, Eugene H.; Jacobs, Marshall L.; Duncan, Kim; Caldarone, Christopher A.; Williams, William G.; McCrindle, Brian W.

In: Journal of Thoracic and Cardiovascular Surgery, Vol. 145, No. 4, 04.2013, p. 1018-1027.e3.

Research output: Contribution to journalArticle

Karamlou, T, Poynter, JA, Walters, HL, Rhodes, J, Bondarenko, I, Pasquali, SK, Fuller, SM, Lambert, LM, Blackstone, EH, Jacobs, ML, Duncan, K, Caldarone, CA, Williams, WG & McCrindle, BW 2013, 'Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum: A Congenital Heart Surgeons Society study', Journal of Thoracic and Cardiovascular Surgery, vol. 145, no. 4, pp. 1018-1027.e3. https://doi.org/10.1016/j.jtcvs.2012.11.092
Karamlou, Tara ; Poynter, Jeffrey A. ; Walters, Henry L. ; Rhodes, Jonathan ; Bondarenko, Igor ; Pasquali, Sara K. ; Fuller, Stephanie M. ; Lambert, Linda M. ; Blackstone, Eugene H. ; Jacobs, Marshall L. ; Duncan, Kim ; Caldarone, Christopher A. ; Williams, William G. ; McCrindle, Brian W. / Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum : A Congenital Heart Surgeons Society study. In: Journal of Thoracic and Cardiovascular Surgery. 2013 ; Vol. 145, No. 4. pp. 1018-1027.e3.
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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.",
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T1 - Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum

T2 - A Congenital Heart Surgeons Society study

AU - Karamlou, Tara

AU - Poynter, Jeffrey A.

AU - Walters, Henry L.

AU - Rhodes, Jonathan

AU - Bondarenko, Igor

AU - Pasquali, Sara K.

AU - Fuller, Stephanie M.

AU - Lambert, Linda M.

AU - Blackstone, Eugene H.

AU - Jacobs, Marshall L.

AU - Duncan, Kim

AU - Caldarone, Christopher A.

AU - Williams, William G.

AU - McCrindle, Brian W.

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N2 - 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.

AB - 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.

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