Evaluation of six cases with alcapa syndrome: A rare but treatable cause of dilated cardiomyopathy

Abstract

INTRODUCTION: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac anomaly that can be cured with surgical treatment. It accounts for 0.25 to 0.5% of all congenital heart diseases. ALCAPA is generally asymptomatic in the neonatal period. Dilated cardiomyopathy (DCM) and mitral valve insufficiency (MVI) are detected in patients with symptoms of heart failure. ALCAPA, which is a rare cause of DCM, early surgical treatment provides quite good prognosis in the first year of life. However, mortality is high in patients who do not receive surgical treatment.MATERIALS and METHODS: Six patients diagnosed with DCM at our clinic between January, 2005 and December, 2017 and detected with ALCAPA syndrome in their etiological assessment we re-evaluated retrospectivelyRESULTS: Of the 6 patients, 5 were female and 1 was male. The average age of diagnosis was 4,75 months. All patients had the signs and symptoms of heart failure at admission. All cases had the signs of ischemia in the chest leads, D1 and aVL in the electrocardiograms (ECGs). In allcases, echocardiography (ECHO) showed mitral insufficiency and the dilation of the left ventricle and the mean ejection fraction was 29,3% (18-39%), and the mean shortening fraction was 12,6% (8-19%) in the ECHOs. Diagnostic catheter angiography was performed for all patients and the diagnosis of ALCAPA was confirmed. All patients under went correction surgery with there implantation method. Four of the patients completely recovered after surgery. One patient died 5 days after the surgery. One patient is being followed up with medical treatment since there was no improvement in her cardiac functions after surgery.CONCLUSIONS: The ALCAPA syndrome should definitely be considered in the etiology of cases diagnosed with dilated cardiomyopathy and it should be kept in mind that ALCAPA is completely treatable disease with early diagnosis and treatment.