A young patient with coronary artery anomaly, whose left anterior descending artery originated from the pulmonary artery, underwent cardiac arrest : case report - online article

Abstract

A rare congenital anomaly of the coronary arteries, in which the left coronary arterial system starts from the arteria pulmonalis, is known as Bland-White-Garland (BWG) syndrome. Isolated left anterior descending (LAD) or circumflex (Cx) arteries originating from the pulmonary artery are even more rare. These anomalies may cause myocardial ischaemia, myocardial infarction, arrhythmia and sudden death. Even if the patient is asymptomatic, he/she should undergo corrective surgery. Here we present the case of an 18-year-old male who survived sudden cardiac arrest during exercise. We identified intra-myocardial blood flow from transthoracic echocardiography, and performed coronary and computed tomographic (CT) angiography, which showed that all the coronary arteries were ectatic and curly and there were disseminated collaterals among the coronary arteries. We diagnosed 'anomalous left coronary artery from the pulmonary artery' (ALCAPA) syndrome, as additionally, the LAD originated from the pulmonary artery. We treated the patient with a left internal mammarian artery - left anterior descending artery (LIMA-LAD) graft.

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

“…Echocardiography can show the abnormal origin of the LCA from the pulmonary artery, the left coronary artery flow via Doppler imaging, the dilated RCA, severe left ventricular dysfunction, and mitral regurgitation. [1,5] Since our patient had poor echogenicity, only an indefinite turbulent flow in the pulmonary artery could be detected by color and continuous wave Doppler.…”

“…[3] Unless the latter type is diagnosed and treated, 90% of children die in their first year of life because of mitral regurgitation due to widespread myocardial ischemia and heart failure. [1,4] Adult patients can be completely asymptomatic or present with angina, dyspnea, syncope, myocardial infarction (MI), arrhythmias, or sudden cardiac death, [5] with sudden cardiac death secondary to malignant ventricular arrhythmias being the most common presentation. [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.…”

“…[1,4] Adult patients can be completely asymptomatic or present with angina, dyspnea, syncope, myocardial infarction (MI), arrhythmias, or sudden cardiac death, [5] with sudden cardiac death secondary to malignant ventricular arrhythmias being the most common presentation. [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.…”

“…[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.…”

Evaluation of patients with coronary artery anomalies

“…[18] It is valuable in visualizing abnormal coronary arteries, their origins, and projections. [19] Also, the sensitivity of angiography may be limited in the diagnosis of an anomalous coronary artery due to its invasive nature. [17] Therefore, CTA and/or magnetic resonance imaging is recommended for more definite spatial resolution before deciding surgery.…”