Author + information
- Michael O'Byrne,
- Laura Mercer-Rosa,
- Huaqing Zhao,
- Xuemei Zhang,
- Ronn Tanel,
- Bradley Marino,
- Amy Cassedy,
- Mark Fogel,
- Jack Rychik,
- Wei Yang,
- Stephen Paridon and
- Elizabeth Goldmuntz
Studies of outcome following repair of interrupted aortic arch (IAA) have focused on mortality and rate of re-intervention. We sought to investigate the clinical status of subjects following IAA repair.
A cross-sectional assessment of subjects with IAA was performed, with genetic testing, electrocardiogram, cardiac magnetic resonance imaging, cardiopulmonary exercise testing, functional status and health-related quality of life (HRQOL) questionnaires (CHQ50 and PCQLI respectively). Review of their medical history provided retrospective information on the incidence of cardiac re-intervention and utilization of medical care.
Twenty-one subjects aged 11.6+/- 3.6 years with IAA were enrolled. The prevalence of 22q11.2 deletion was 67%. All underwent single stage operative repair. Re-intervention rates were 38% on the left ventricular outflow tract, 33% for the aortic arch, and 24% for both. Left ventricular ejection fraction was 71.7+/-5.8%. Percent predicted maximal oxygen consumption (68+/-18%) and forced vital capacity (71+/-15%), as well as maximal work (76+/-22), were diminished compared to published age and gender norms (p<0.0001). Functional status (p=0.0002 for transformed physical, p=0.0063 for transformed psychosocial) and HRQOL (total score) were diminished relative to historical controls with repaired mild congenital heart disease (p <0.0001) and those with Fontan operations (p=0.0179). Subjects demonstrated frequent utilization of both inpatient and outpatient resources including hospitalizations, non-cardiac operations, subspecialty visits, and medications.
Patients with IAA demonstrate persistent co-morbid disease, with frequent cardiac intervention, and deficits in exercise performance, functional status, and HRQOL.
Poster Sessions, Expo North
Sunday, March 10, 2013, 3:45 p.m.-4:30 p.m.
Session Title: Congenital Cardiology Solutions: Aortic Disease
Abstract Category: 13. Congenital Cardiology Solutions: Pediatric
Presentation Number: 1246-119
- 2013 American College of Cardiology Foundation