Dilated cardiomyopathy in association with the ALCAPA syndrome

Abstract

Introduction: Dilated cardiomyopathy is a severe condition in which the heart muscle is weakened and no longer has the strength to pump blood throughout the body. The weakened heart is unable to pump more blood, therefore more blood remains to this level after each heartbeat. As larger amounts of blood remain in the lower chambers of the heart, they expand. Over time, the heart muscle loses its shape and becomes increasingly weaker. Objectives: Study of clinical evolution and ECG of patients diagnosed with associated dilated cardiomyopathy or ALCAPA Syndrome from a basic treatment with IEC, diuretics and B-blocking agents along with the surgical treatment. Materials and Methods: 10 children of which 9 diagnosed with dilated cardiomyopathy and 1 child who associates ALCAPA syndrome were evaluated by Ross score, NYHA and echocardiographic. Cardiac index measurement using ECG and CT after receiving basic treatment. Results: The 9 patients with dilated cardiomyopathy treated by the basic therapeutic method have evolved to improve cardiac indexes (Ross and NYHA) showing an increase in left ventricular ejection fraction gather up 20%. The patient with dilated cardiomyopathy and ALCAPA syndrome showed no improvement following therapy being directed to surgical treatment. Conclusions: Dilated cardiomyopathies in pediatric age have an 80% response rate to basic treatment with IEC, diuretics and B-blockers that lead to Ross and NYHA amelioration. Dilated cardiomyopathy associated with ALCAPA syndrome has one treatment option: heart transplant.