INNOVATION ASSOCIATED WITH THE MANAGEMENT AND TREATMENT OF CHILDREN WITH HYPERTROPHIC CARDIOMYOPATHY – A REVIEW OF THE CURRENT LITERATURE

Innovative Strategies in Managing Chidren with Hypertrophic Hardiomyopathies

Abstract

Primary hypertrophic cardiomyopathy (HCM) is a common inherited abnormality in children and adults, and is known to be associated with genetic predisposition. Disease can be present in childhood, adolescence or adulthood. It is a frequent cause of disability and death in those of all ages. Indeed, it is the most common cause of sudden death in the young athlete. Typical symptoms include dyspnea, chest pain, palpitations, and syncope. The diagnosis is usually suspected on clinical examination and confirmed by imaging. Some patients are at increased risk of sudden cardiac death, heart failure, and atrial fibrillation. Patients with an increased risk of sudden cardiac death undergo cardioverter-defibrillator implantation; in patients with severe symptoms related to ventricular obstruction, septal reduction therapy (myectomy or alcohol septal ablation) is recommended. Life-long anticoagulation is indicated after the first episode of atrial fibrillation. In this review we concentrate on the so-called sarcomeric forms of HCM. The non-sarcomeric forms of the disease are both phenotypically and genetically different from the group to be discussed.