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 and Poynter, Jeffrey A. and Walters, Henry L. and Rhodes, Jonathan and Bondarenko, Igor and Pasquali, Sara K. and Fuller, Stephanie M. and Lambert, Linda M. and Blackstone, Eugene H. and Jacobs, Marshall L. and Duncan, Kim and Caldarone, Christopher A. and Williams, William G. and McCrindle, Brian W. (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. The Journal of Thoracic and Cardiovascular Surgery, V. 145 (N 4). pp. 1018-1027. ISSN 0022-5223

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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 ¼ .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. (J Thorac Cardiovasc Surg 2013;145:1018-27)

Item Type:	Article
Additional Information:	Read at the 92nd Annual Meeting of The American Association for Thoracic Surgery, San Francisco, California, April 28-May 2, 2012.
Subjects:	Cardiology
Divisions:	Departments > Department of Oncology and Medical Radiology
Depositing User:	Елена Шрамко
Date Deposited:	31 Mar 2021 15:24
Last Modified:	31 Mar 2021 15:24
URI:	http://repo.dma.dp.ua/id/eprint/6393

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