Morbidity in children and adolescents after surgical correction of truncus arteriosus communis.

Morbidity in children and adolescents after surgical correction of truncus arteriosus communis.
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DOI:
10.1016/j.ahj.2013.05.023
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发表时间:
2013-09
影响因子:
4.8
通讯作者:
Goldmuntz, Elizabeth
Goldmuntz, Elizabeth
中科院分区:
医学2区
文献类型:
--
作者:
O'Byrne, Michael L.;Mercer-Rosa, Laura;Zhao, Huaqing;Zhang, Xuemei;Yang, Wei;Cassedy, Amy;Fogel, Mark A.;Rychik, Jack;Tanel, Ronn E.;Marino, Bradley S.;Paridon, Stephen;Goldmuntz, Elizabeth

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Studies of outcome following operative correction of truncus arteriosus communis (TA) have focused on mortality and rates of re-intervention. We sought to investigate the clinical status of children and adolescents with surgically corrected TA. A cross-sectional study of subjects with TA was performed. Subjects underwent concurrent genetic testing, electrocardiogram, cardiac magnetic resonance imaging, cardiopulmonary exercise testing, and completed questionnaires assessing health status and health-related quality of life. Review of their medical history provided retrospective information on cardiac re-intervention and utilization of medical care. Twenty-five subjects with a median age of 11.8 (8.1-18.99) years were enrolled. The prevalence of 22q11.2 deletion was 32%. Incidence of hospitalization, cardiac re-intervention, and non-cardiac operations was highest in the first year of life. Combined catheter-based and operative re-intervention rates were 52% on the conduit and 56% on the pulmonary arteries. Right ventricular ejection fraction and end diastolic volume were normal. Moderate or greater truncal valve insufficiency was seen in 11% of subject, and truncal valve replacement occurred in 8% of subjects. Maximal oxygen consumption (p=0002), maximal work (p<0.0001), and forced vital capacity (p<0.0001) were all lower than normal for age and sex. Physical health status and health-related quality of life were both severely diminished. Patients with TA demonstrate significant co-morbid disease throughout childhood, significant burden of operative and catheter-based re-intervention, and deficits in exercise performance, functional status, and health-related quality of life.
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