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Peri-procedural risk stratification and management of patients with Williams syndrome
R Thomas Collins Ii1,2, Margaret G Collins3, Michael L Schmitz2,4
1Department of Pediatrics, The University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.
Insights
Williams syndrome (WS) patients require careful peri-procedural management due to cardiovascular risks. This review offers safe anesthetic strategies and an expanded risk stratification system for WS patients undergoing procedures.
Area of Science:
- Cardiology
- Anesthesiology
- Genetics
Background:
- Williams syndrome (WS) is a genetic disorder affecting multiple systems, notably the cardiovascular system.
- Cardiovascular complications are the leading cause of death in WS patients.
- Peri-procedural sudden cardiovascular collapse is a known risk, particularly during anesthesia.
Purpose of the Study:
- To provide evidence-based guidelines for peri-procedural management of patients with Williams syndrome.
- To detail safe and effective anesthetic strategies considering hemodynamic impacts.
- To present an expanded risk stratification system for WS patients.
Main Methods:
- Literature review focusing on peri-procedural care and anesthesia in WS.
- Analysis of cardiovascular risks and anesthetic implications.
- Development of a risk stratification framework.
Main Results:
- Limited existing guidelines for peri-procedural WS management identified.
- Hemodynamic considerations are critical for anesthetic choices in WS.
- An expanded risk stratification system is proposed.
Conclusions:
- Safe peri-procedural care for WS patients necessitates tailored anesthetic strategies.
- The proposed risk stratification aids in optimizing patient safety.
- Further research into WS peri-procedural management is warranted.
Abstract:
Williams syndrome (WS) is a congenital, multisystem disorder affecting the cardiovascular, connective tissue, and central nervous systems in 1 in 10 000 live births. Cardiovascular involvement is the most common cause of morbidity and mortality in patients with WS, and noninvasive and invasive procedures are common. Sudden cardiovascular collapse in patients with WS is a well-known phenomenon, especially in the peri-procedural period. Detailed guidelines for peri-procedural management of patients with WS are limited. The goal of this review is to provide thoughtful, safe and effective management strategies for the peri-procedural care of patients with WS with careful consideration of hemodynamic impacts of anesthetic strategies. In addition, an expanded risk stratification system for anesthetic administration is provided.