Ischemic cardiomyopathy due to late diagnosis of alcapa syndrome

ABSTRACT

INTRODUCTION: ALCAPA Syndrome (Anomalous Left Coronary from the Pulmonary Artery) is the anomalous origin of the left coronary artery from the pulmonary artery. Also known as BlandWhite-Garland Syndrome, it is a rare congenital heart defect, present in 0.25-0.5% of patients with congenital heart defects. It can occur in isolation or be associated with other birth defects. Usually the diagnosis is early, made in the prenatal period and the need for its correction is immediate. CASE REPORT A 63-year-old female patient sought emergency care with chest pain, dyspnea on moderate exertion, and palpitations. Myocardial scintigraphy was positive for ischemia, corresponding to the territory of the anterior descending artery. Cardiac catheterization was requested, in which an anomalous left coronary artery was observed in the pulmonary artery trunk. The transthoracic echocardiogram showed the following findings: akinesia of the apical and anteroapical septum, with hypokinesia of the anterolateral wall and of the mid-basal segments of the anterior wall, moderate left ventricular systolic dysfunction (ejection fraction of 37 %), pulmonary artery systolic pressure of 62 mmHg. It has magnetic resonance imaging of the heart, which shows the areas of infarction mentioned in the echocardiogram, without myocardial viability. DISCUSSION: ALCAPA syndrome is difficult to diagnose, and should be suspected in children with dilated cardiomyopathy. About 85% of cases manifest up to 2 months of age, but symptoms can be misinterpreted and the disease may be underdiagnosed. The disease requires surgical treatment to restore the flow of oxygenated blood to the myocardium. The procedure of choice consists of reimplanting the coronary artery in the aorta and it is estimated that the surgical mortality is less than 5-10%, with a good prognosis after reimplantation. The peculiarity of the case reported is due to the fact that the diagnosis was extremely late and with important irreversible hemodynamic REPERCUSSIONS the patient has an ischemic cardiomyopathy without myocardial viability, a condition usually related to chronic coronary artery disease or acute coronary syndromes, rarely related to ALCAPA, since due to the severity of the syndrome, its diagnosis is early. Currently, due to the lack of myocardial viability, surgical correction would not bring benefits or recovery of heart muscle, opting for clinical treatment for cardiac remodeling.