Modified Doty repair for severe supravalvular aortic stenosis in a pediatric patient with Williams Syndrome: A case report

Jesús García Pinzas; Cristhian Jeanpierre Cueva Álvarez; Martín Crosby Barabino

doi:10.47487/apcyccv.v7i4.646

Authors

Jesús García Pinzas Instituto Nacional de Salud del Niño de Breña. Lima, Peru. https://orcid.org/0009-0009-9090-4604
Cristhian Jeanpierre Cueva Álvarez Universidad de San Martín de Porres, Lima, Peru. https://orcid.org/0009-0004-0823-0692
Martín Crosby Barabino Universidad Continental, Lima, Peru. https://orcid.org/0009-0009-1123-2281

DOI:

https://doi.org/10.47487/apcyccv.v7i4.646

Keywords:

Heart Defect, Congenital, Aortic Stenosis, Supravalvular, Williams Syndrome

Abstract

A 2-year-old female patient with Williams syndrome, an imperforate anus, a right-sided cystic renal anomaly, and mild psychomotor delay presented with progressive severe supravalvular aortic stenosis, exertional dyspnea, and poor weight gain. Cardiac surgery was performed, using two expansion patches at the sinotubular junction and a third patch in the distal aorta. The systolic Doppler gradient was reduced from 87 to 20 mmHg, providing substantial early hemodynamic relief. The use of two patches for aortic enlargement has shown superiority over other techniques, providing better survival and longer intervals without reintervention. At follow-up, the patient remained asymptomatic and normotensive, with clinical and echocardiographic surveillance every 6 months.

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References

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