Williams syndrome, and various elastin protein mediated arteriopathies, presents a
clinical challenge to pediatric cardiovascular specialists. In the severest phenotypes,
multilevel obstruction to the systemic and pulmonic arterial systems result in biventricular
dysfunction which can be imminently life-threatening. As a longstanding, quaternary
referral center for complex pulmonary arteriopathies and pediatric connective tissue
disease, Stanford Medicine Children's Health has developed a sizeable experience managing
these patients. This manuscript is a summary of our current strategies, with a focus
on our surgical techniques, peri-procedural considerations on timing and staging of
various interventions, and long-term results.
Keywords
Abbreviations:
Ao (aorta), AP (aortopulmonary), LV (left ventricle), PA (pulmonary artery), PAR (pulmonary artery reconstruction), PAS (pulmonary artery stenosis), RV (right ventricle), SVAS (supravalvar aortic stenosis), WS (Williams syndrome)To read this article in full you will need to make a payment
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References
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- Surgical repair of peripheral pulmonary artery stenosis: a 2-decade experience with 145 patients.J Thorac Cardiovasc Surg. 2022; (article in press)
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Article info
Publication history
Published online: February 02, 2023
Footnotes
Financial disclosure: The authors whose names are listed immediately above certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.
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