Williams Syndrome: Supravalvar Aortic, Aortic Arch, Coronary and Pulmonary Arteries: Is Comprehensive Repair Advisable and Achievable?

Michael Ma; Elisabeth Martin; Claudia Algaze; Ronnie Thomas Collins; Doff McElhinney; Richard Mainwaring; Frank Hanley

doi:10.1053/j.pcsu.2022.12.003

Williams Syndrome: Supravalvar Aortic, Aortic Arch, Coronary and Pulmonary Arteries: Is Comprehensive Repair Advisable and Achievable?

Michael Ma, Elisabeth Martin, Claudia Algaze, Ronnie Thomas Collins, Doff McElhinney, Richard Mainwaring, Frank Hanley

Pediatrics

Research output: Contribution to journal › Review article › peer-review

2 Scopus citations

Abstract

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.

Original language	English
Pages (from-to)	2-8
Number of pages	7
Journal	Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual
Volume	26
DOIs	https://doi.org/10.1053/j.pcsu.2022.12.003
State	Published - Jan 2023

Bibliographical note

Publisher Copyright:
© 2022 Elsevier Inc.

Funding

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.

Keywords

Coronary artery repair
Elastin arteriopathy
Pulmonary artery reconstruction
Pulmonary artery stenosis
Supravalvar aortic stenosis
Williams syndrome

ASJC Scopus subject areas

Surgery
Pediatrics, Perinatology, and Child Health
Cardiology and Cardiovascular Medicine

Access to Document

10.1053/j.pcsu.2022.12.003

Cite this

Ma, M., Martin, E., Algaze, C., Collins, R. T., McElhinney, D., Mainwaring, R., & Hanley, F. (2023). Williams Syndrome: Supravalvar Aortic, Aortic Arch, Coronary and Pulmonary Arteries: Is Comprehensive Repair Advisable and Achievable? Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual, 26, 2-8. https://doi.org/10.1053/j.pcsu.2022.12.003

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title = "Williams Syndrome: Supravalvar Aortic, Aortic Arch, Coronary and Pulmonary Arteries: Is Comprehensive Repair Advisable and Achievable?",

abstract = "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 = "Coronary artery repair, Elastin arteriopathy, Pulmonary artery reconstruction, Pulmonary artery stenosis, Supravalvar aortic stenosis, Williams syndrome",

author = "Michael Ma and Elisabeth Martin and Claudia Algaze and Collins, \{Ronnie Thomas\} and Doff McElhinney and Richard Mainwaring and Frank Hanley",

note = "Publisher Copyright: {\textcopyright} 2022 Elsevier Inc.",

year = "2023",

month = jan,

doi = "10.1053/j.pcsu.2022.12.003",

language = "English",

volume = "26",

pages = "2--8",

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Ma, M, Martin, E, Algaze, C, Collins, RT, McElhinney, D, Mainwaring, R & Hanley, F 2023, 'Williams Syndrome: Supravalvar Aortic, Aortic Arch, Coronary and Pulmonary Arteries: Is Comprehensive Repair Advisable and Achievable?', Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual, vol. 26, pp. 2-8. https://doi.org/10.1053/j.pcsu.2022.12.003

Williams Syndrome: Supravalvar Aortic, Aortic Arch, Coronary and Pulmonary Arteries: Is Comprehensive Repair Advisable and Achievable? / Ma, Michael; Martin, Elisabeth; Algaze, Claudia et al.
In: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual, Vol. 26, 01.2023, p. 2-8.

Research output: Contribution to journal › Review article › peer-review

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T1 - Williams Syndrome

T2 - Supravalvar Aortic, Aortic Arch, Coronary and Pulmonary Arteries: Is Comprehensive Repair Advisable and Achievable?

AU - Ma, Michael

AU - Martin, Elisabeth

AU - Algaze, Claudia

AU - Collins, Ronnie Thomas

AU - McElhinney, Doff

AU - Mainwaring, Richard

AU - Hanley, Frank

PY - 2023/1

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AB - 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.

KW - Coronary artery repair

KW - Elastin arteriopathy

KW - Pulmonary artery reconstruction

KW - Pulmonary artery stenosis

KW - Supravalvar aortic stenosis

KW - Williams syndrome

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Williams Syndrome: Supravalvar Aortic, Aortic Arch, Coronary and Pulmonary Arteries: Is Comprehensive Repair Advisable and Achievable?

Abstract

Bibliographical note

Funding

Keywords

ASJC Scopus subject areas

Access to Document

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