Left main coronary artery stent placement in a 7.0 kg infant with Williams Syndrome
Jeffrey G Weiner1, Joshua D Chew1, Thomas P Doyle1
1Division of Pediatric Cardiology, Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, TN, USA.
Journal of Structural Heart Disease
|July 2, 2019
Insights
A 9-month-old with Williams syndrome experienced severe left ventricular dysfunction post-surgery for aortic and pulmonary artery stenosis. Emergent stenting of the left coronary artery was required due to myocardial ischemia.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Clinical Case Reports
Background:
- Williams syndrome is associated with supravalvular aortic stenosis and pulmonary artery stenosis.
- Myocardial ischemia can be a severe complication in infants undergoing cardiovascular surgery.
Purpose of the Study:
- To report a rare case of emergent coronary artery stenting in an infant with Williams syndrome.
- To highlight the management of left ventricular dysfunction secondary to myocardial ischemia in this specific population.
Main Methods:
- Surgical patch augmentation for supravalvular aortic stenosis and pulmonary artery stenosis.
- Emergent drug-eluting left coronary artery stenting.
- Post-operative monitoring for left ventricular function and myocardial ischemia.
Main Results:
- The patient developed severe left ventricular dysfunction on post-operative day 1.
- Successful emergent stenting of the left coronary artery was performed.
- The case demonstrates a potential therapeutic option for myocardial ischemia in infants with Williams syndrome.
Conclusions:
- Infants with Williams syndrome undergoing complex cardiac surgery are at risk for myocardial ischemia.
- Early recognition and intervention, including coronary stenting, may be crucial for managing severe left ventricular dysfunction in this setting.
Abstract:
We report the case of a 9-month-old male with Williams syndrome who underwent patch augmentation of supravalvar aortic stenosis and pulmonary artery stenosis, and required emergent drug-eluting left coronary artery stenting on post-operative day 1 for severe left ventricular dysfunction related to myocardial ischemia.
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