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Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
Published on: August 8, 2022
Cardiomyopathy in childhood
Glyn D Williams1, Gregory B Hammer
1Department of Anesthesia, Stanford University School of Medicine, Stanford, California 94305, USA. jumbo@stanford.edu
Insights
Pediatric cardiomyopathy, a leading cause of heart failure, requires specialized anesthetic care. This review details disease types, outcomes, and perioperative management for high-risk children.
Area of Science:
- Pediatric Cardiology
- Anesthesiology
- Critical Care Medicine
Background:
- Cardiomyopathy is a significant cause of pediatric heart failure and a primary indication for heart transplantation.
- Limited literature exists to guide anesthesiologists managing high-risk pediatric patients with cardiomyopathy.
- Recent advancements have expanded knowledge of cardiomyopathy genotypes, phenotypes, and therapies.
Purpose of the Study:
- To review pediatric cardiomyopathy phenotypes and associated factors influencing clinical outcomes and perioperative complications.
- To provide guidance on anesthesia considerations for children with cardiomyopathy.
- To enhance understanding of disease management in this vulnerable population.
Main Methods:
- Literature review focusing on pediatric cardiomyopathy.
- Analysis of factors affecting clinical outcomes and perioperative complications.
- Synthesis of current knowledge on anesthesia management.
Main Results:
- Increased recognition of diverse cardiomyopathy genotypes and phenotypes in children.
- Identification of factors linked to adverse clinical outcomes and perioperative events.
- Emerging data from multicenter registries are improving disease understanding.
Conclusions:
- The complexity of pediatric cardiomyopathies necessitates a deep understanding of cardiac pathophysiology for perioperative care.
- Multisystem involvement, genetic disorders, and syndromic conditions are critical considerations.
- Thorough preoperative assessment and tailored intraoperative/postoperative plans are essential for optimal outcomes.
Purpose Of Review:
Cardiomyopathy is an important cause of heart failure and a major indication for heart transplantation in children. Unfortunately, there is a paucity of literature to guide the anesthesiologist who cares for these high-risk children. This review describes the cardiomyopathy phenotypes that occur in children and the factors that are associated with clinical outcomes and perioperative complications. Anesthesia considerations will be reviewed.
Recent Findings:
During the past decade, there has been a dramatic increase in knowledge related to cardiomyopathy. New genotypes and phenotypes are recognized and new therapies have been devised. Multicenter pediatric cardiomyopathy registries are obtaining data essential for enhanced understanding of the disease.
Summary:
The diverse spectrum and complexity of pediatric cardiomyopathies mandate a thorough appreciation of the cardiac pathophysiology pertinent to an individual child's perioperative management. Important issues include multisystem disease associated with syndromic or genetic disorders, appropriate preoperative patient assessment to adequately characterize patient risk and guide therapy, and intraoperative and postoperative care plans that target optimal outcomes.
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Cardiomyopathy VI: Nursing Management

