Congenital Absence of Left Circumflex Presenting After an Emotional Stressor

Abstract

SummaryBackgroundAbsence of the left circumflex artery (LCX) is an extremely rare congenital anomaly of the coronary circulation. While some coronary circulation anomalies are associated with significant complications, including sudden cardiac death and premature atherosclerosis, absence of the LCX is largely considered benign, though it has been associated with exertional chest pain, which may mimic acute coronary syndrome. Diagnosis is made when heart catheterization is performed in the work up for acute coronary syndrome or when computed tomography coronary angiography is performed during evaluation of coronary artery disease.Case ReportWe report a 55 year old female who presented with non-exertional chest pain in the setting of an emotional stressor. The initial work up was only significant for elevated troponins, and subsequent left heart catheterization revealed findings consistent with congenital absence of the LCX. No significant stenosis was appreciated, and no intervention was performed. Following catheterization, the patient’s troponins began to trend down, and her chest pain resolved.ConclusionsCongenitally absent LCX is a rare entity detected when work up is performed to rule out acute coronary syndrome in patients presenting with exertional chest pain. This is the first reported case of chest pain unrelated to physical activity reported in a patient with an absent LCX. There is no specific treatment for an absent LCX; however, proper identification of this anomaly and differentiation from complete occlusion of the LCX is important in making an accurate diagnosis of myocardial ischemia and for choosing the best intervention when ischemia is present.

Congenital anomaly of coronary artery: absence of left circumflex artery

“…It has been hypothesized that symptoms on exertion are possibly secondary to a 'steal' phenomenon. This phenomenon arises in situations of increased metabolic demands in the LCx territory leading to the diversion of blood flow to these regions resulting in ischemic changes in the LAD or RCA territories which can mimic an acute coronary event [7]. This can manifest as perfusion defects on stress thallium study [6].…”

Incidentally Detected Congenitally Absent Left Circumflex Artery by MDCT Coronary Angiogram

“…Congenital absence of left circumflex artery is an incidental benign finding on coronary angiography and most of these patients present with chest pain on exertion. [7][8][9][10][11] Varela et al [7] reported a case of congenitally absent left circumflex with block of right coronary artery causing inferolateral and posterior wall myocardial infarction. The various investigations available to diagnose this rare entity include transthoracic, transoesophageal echocardiography, cardiac magnetic resonance imaging, MDCT coronary angiography and invasive coronary angiography.…”

Congenital Absence of Left Circumflex Artery: A Case Report and Review of the Literature

“…The etiology of chest pain in these patients is not clearly understood. One of the explanations offered in literature is a steal phenomenon during exertion, where there may be transient ischemia in territories fed by the RCA and LAD due to the diversion of blood flow to anatomical regions usually fed by the left circumflex artery [ 3 ].…”

“…Due to its uncommon nature, diagnosis can be challenging, especially among patients who present with acute cardiac symptoms. It may initially be misdiagnosed as complete occlusion of the left circumflex artery [ 3 ]. A better understanding of the natural history and clinical implications of this condition may allow us to diagnose and manage this condition with an algorithmic approach.…”