Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Detected by Echocardiography in an Asymptomatic Adult

Abstract

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital malformation. It is unusual for an ALCAPA patient to survive to adulthood. We present a case of an asymptomatic 54-year-old woman with this syndrome in which visualization of a markedly enlarged and tortuous right coronary artery and intercoronary collaterals by echocardiography raises suspicion for this disease and subsequently guides a step-by-step diagnosis. The patient lives well without surgery 3 years after diagnosis.Key words: anomalous origin of the left coronary artery from the pulmonary artery, congenital heart disease, echocardiography (Intern Med 52: 233-236, 2013)

Combined Non-Invasive Doppler Echocardiography and Coronary Computed Tomography Lead to Diagnosis of Anomalous Left Coronary Artery From the Pulmonary Artery (ALCAPA) Syndrome

“…Approximately 10% are diagnosed in adulthood due to exertional chest pain and dyspnea caused by inadequate myocardial perfusion. In extremely rare cases, ALCAPA without symptoms is accidentally detected on cardiac screening in adults, 9 and most have left-to-right shunting from RCA via LCA to main pulmonary artery through highly developed collateral vessels, as in the present case. Currently, the prognosis of ALCAPA is dramatically improved as a result of early diagnosis due to developments in Doppler echocardiography and improvement of surgical technique.…”

Anomalous origin of the left coronary artery from the pulmonary artery syndrome in an adult and his eight-year follow-up results

“…[1] In ALCAPA, the systemic arterial pressure (SAP) and PAP as well as the oxygen saturation (OS) levels are equal during the fetal period. [2] The pulmonary pressure falls and the ductus arteriosus closes in the neonatal period. The flow of the LCA reverses, and the perfusion pressure of this artery falls after closure of the ductus.…”

“…[5] Today, an increase in the number of diagnostic procedures helps us to detect ALCAPA in older patients; however, well developed collaterals, symptoms, and life-threatening arrhythmias occur less frequently in older patients. [5] Tian et al, [2] conducted a study that focused on the short-term follow-up of asymptomatic patients who refused surgical therapy and found that all were asymptomatic.…”

“…Coronary CT angiography is an important, noninvasive diagnostic tool used to delineate the abnormal origin, projection, and collaterals of coronary arteries. [2] In our case, we performed coronary CT in which we detected the abnormal origin of the LCA from the pulmonary artery and visualized the course of the LCA with its collaterals arising directly from the aorta.…”

ALCAPA – a need for guidelines for managing the adult type

“…There are still controversies about when to correct adult alCapa patients but the vast majority recommends surgical treatment in asymptomatic adults if only moderate chronic ischemia is present 1,2,7,8,9 . But still, especially in asymptomatic older patients, the risk-benefit ratio of surgery is unclear 9 .…”