Cardiomyopathy is a disease of the myocardium associated with cardiac dysfunction that cannot be explained by abnormal loading conditions or congenital heart disease. Dilated cardiomyopathy (DCM) is a phenotypic class of cardiomyopathy that is defined by ventricular chamber dilation with dysfunction that is secondary to ineffective systolic shortening. In children without known structural heart abnormalities DCM is the most common cause of congestive heart failure. The outcome of patients presenting with DCM is variable, with some children who present with fulminant heart failure requiring mechanical circulatory support followed by transplantation while others recover normal function. This chapter details the perioperative considerations involved in care of a child with severe DCM.

The overall incidence of pediatric cardiomyopathy is estimated to be approximately 1–1.5 cases per 100,000 patients. The majority are due to dilated cardiomyopathy, which accounts for more than 50% of all cases. Hypertrophic cardiomyopathy is the second leading cause, with restrictive cardiomyopathy being the least common. While distinct classifications exist, in practice the variants occur in combination. However, there is generally a predominant phenotype diagnosed by echocardiography that allows for the formation of multicenter registries to track the epidemiologic, management, and outcomes related to pediatric cardiomyopathy. This chapter focuses on mixed hypertrophic and restrictive forms of cardiomyopathy and discusses the perioperative management of a patient with mixed cardiomyopathy.