Comprehensive Repair Of Bilateral Outflow Tract Obstruction In Elastin Arteriopathy Syndrome
Alexander K. Reed, MD, Perry S. Choi, MD, John Lamberti, MD, Michael Ma, MD.
Stanford Medicine, Palo Alto, CA, USA.
Objective(s): To describe the approach to comprehensive single-stage repair of complex bilateral outflow tract obstruction in an elastin arteriopathy patient.
Methods: A 3.9 kilogram, 3-week-old patient with a non-Williams elastin arteriopathy syndrome was found to have biventricular hypertrophy and systolic failure in the setting of severe supravalvular aortic stenosis, bilateral hypoplastic pulmonary arteries, and coronary ostial disease. Preoperatively, cardiac catheterization revealed suprasystemic right ventricular and pulmonary arterial (PA) pressures. The patient underwent single-stage comprehensive repair involving modified Brom technique aortoplasty, bilateral pulmonary artery augmentation, and coronary ostia augmentation utilizing PA homograft patches. Homograft was used given the patient's young age and need for compliant tissue with future growth. This submission demonstrates these repair techniques.
Results: The patient tolerated the procedure well with immediate improvement in his left- and right-sided filling pressures. Serial postoperative echocardiograms revealed recovered biventricular systolic function without residual aortic stenosis or pulmonary valve hypoplasia.
Conclusions: Comprehensive, single-stage intervention provides a viable approach to repair complex, bilateral outflow tract obstruction in elastin arteriopathy syndrome.
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