Composite aortoplasty for recurrent coarctation after neonatal repair in Williams syndrome
Jeni L Marks1, Max B Mitchell, David N Campbell
1Department of Surgery, University of Colorado Health Sciences Center, Denver, Colorado, USA.
Insights
Williams syndrome often requires supravalvar aortic stenosis repair, but coarctation repair is more common in infants. This case highlights the need for detailed aortic evaluation before initial coarctation repair in neonates with Williams syndrome.
Area of Science:
- Cardiovascular Surgery
- Pediatric Cardiology
- Medical Genetics
Background:
- Williams syndrome is a genetic disorder associated with cardiovascular anomalies.
- Supravalvar aortic stenosis is the most common cardiac surgical indication in Williams syndrome.
- Aortic coarctation repair is frequently performed in neonates, but reoperation may be necessary.
Observation:
- A neonate with Williams syndrome underwent aortic coarctation repair.
- The patient required extensive reoperation within five months due to aortic arch and descending aorta complications.
- Surgical intervention involved a composite left subclavian artery flap and allograft patch aortoplasty under cardiopulmonary bypass and circulatory arrest.
Findings:
- This case demonstrates a complex surgical outcome following neonatal aortic coarctation repair in a patient with Williams syndrome.
- The need for reoperation underscores potential challenges in managing aortic arch abnormalities in this population.
Implications:
- Thorough pre-operative anatomical assessment of the aortic arch and descending aorta is crucial for neonates with Williams syndrome.
- Early and detailed imaging may prevent future complications and the need for reoperation.
- This case emphasizes the importance of tailored surgical strategies for complex congenital heart disease in genetic syndromes.
Abstract:
Supravalvar aortic stenosis is the most frequent operation required for Williams syndrome; however, coarctation repair is more common in patients requiring surgery in the first few months of life. We report on a child with Williams syndrome in whom extensive reoperation was required 5 months after neonatal aortic coarctation repair. A composite left subclavian artery flap and allograft patch aortoplasty of the aortic arch and descending aorta was performed through a left thoracotomy using cardiopulmonary bypass and circulatory arrest. Detailed anatomic evaluation of the aortic arch and descending aorta is recommended before initial coarctation repair in neonates with Williams syndrome.
