Cardiomyopathy in Cystic Fibrosis

Clinical Presentation and Pathophysiology

• Pulmonary vascular changes, such as those classified as Grade 3 Heath/Edwards, have been documented on autopsy in some CF patients with cardiac involvement 1
• For patients with significant hypertrophic cardiomyopathy, treatment options may include β-blocker medications or surgical procedures such as myomectomy to decrease outflow obstruction, similar to management approaches for other forms of HCM 1