Cardiomyopathy in childhood.

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.