"CIRCLE OF VIEUSSENS": A LIFE-SAVING CORONARY ARTERY ANOMALY IN A PATIENT WITH KAWASAKI DISEASE

Abstract

SESSION TITLE: Complications and Cardiovascular DiseaseSESSION TYPE: Case ReportsPRESENTED ON: 10/17/2022 03:15 pm - 04:15 pmINTRODUCTION: Kawasaki disease is a vasculitis of unknown etiology that involves medium-sized arteries throughout the body. It primarily affects young children but may have long-term morbidity up until adulthood. Kawasaki disease often causes cardiovascular abnormalities, most commonly coronary artery aneurysms [1]. The right and left coronary arteries, which arise from the ascending aorta, are the main supply line of the heart. Only 10% of the blood supply usually comes from collateral circulation. Raymond de Vieussens, in 1706 was the first person to study and report the intracoronary pathway between branches of the right coronary artery (RCA) and the left anterior descending artery (LAD), later named the circle of Vieussens. In patients with coronary artery stenosis, the Conus artery can serve as a principal source of collateral circulation. The Vieussens ring serves as a collateral connection between the conus branches of the RCA and LAD and is usually seen in patients with coronary-to-pulmonary fistulas. The Vieussens ring has specifically been described as connecting the conus artery and the proximal right ventricular branch of the LAD. We report a case of Kawasaki’s disease with severe coronary artery disease and the presence of a Vieussens arterial ring.CASE PRESENTATION: A 26-year-old Asian American female with a history of Kawasaki’s disease presented to the office with complaints of left shoulder discomfort. She also had a coronary artery aneurysm since birth. The patient denied shortness of breath, chest pain, palpitations, or dizziness. The Family history of cardiovascular disease was nonsignificant. She is hemodynamically stable, and her physical exam was normal. Serum biochemistry showed normal lipid and thyroid levels. EKG did not show any acute ischemic changes. Troponin level was normal. Transthoracic echocardiogram showed a preserved left ventricular ejection fraction of 55%. Coronary Computed Tomography Angiography (CCTA) showed LAD dissection along with high-grade stenosis in the proximal LAD at the junction between the first and second diagonal artery.Consequently, left heart catheterization was done to evaluate epicardial coronary artery stenosis, which revealed chronic total obstruction (CTO) of the proximal LAD just before the large diagonal branch took off. The left circumflex artery and the RCA had no angiographically significant disease; however, a right-to-left collateral arterial ring was seen supplying blood to the LAD in an anterograde fashion from the RCA through a 2.5 mm collateral consistent with a circle of Vieussens (Fig I). Left ventricular angiogram showed ejection fraction above 60% and normal aortic root caliber. No subclavian dissection was seen. The patient was continued on medical therapy with risk factor modification for Kawasaki Disease with a plan to re-evaluate for coronary ischemia in 4-6 weeks.DISCUSSION: Most patients with coronary artery disease and CTO have collateral circulation. The location and the extent of collaterals are variable, which determine ischemic symptoms and left ventricular contractility. The Conus artery is not visualized in 20% of coronary angiograms due to its independent origin or the injection of contrast distal to the origin [2]. 30- 50% of the population, the Conus artery originates in an independent ostium in the right sinus of Valsalva, anterior and superior to the ostium of the RCA which is an isolated Conus artery (ICA). The course of the Conus artery is usually short and rarely forms a channel with the LAD artery (left Conus artery), known as the circle of Vieussens [3].To our knowledge, this is the first reported case of Vieussens arterial ring as one of the coronary anomalies a patient with Kawasaki disease, which resulted in a protective, life-saving effect for the patient. Despite the CTO of the proximal LAD, our patient continued to get sufficient blood supply in anterograde fashion through the low-pressure system of the circle of Vieussens. Therefore, the patient remained asymptomatic with preserved LV function. The absence of collaterals or occlusion of the collaterals would have resulted in severe ischemia in the region of LAD.CONCLUSIONS: The circle of Vieussens is a rare collateral circulation between the RCA and the LAD, maintaining coronary circulation in dissection or severe stenotic lesions. It involves the proximal portions of the left or right coronary arteries, thereby helping to preserve normal heart functions in Kawasaki disease.Reference #1: Newburger JW, de Ferranti SD, Fulton DR. Cardiovascular sequelae of Kawasaki disease:management and prognosis. UpToDate website. Updated December 10, 2019. Accessed August 9, 2021.Reference #2: Levin DC, Beckmann CF, Garnic JD, Carey P, Bettmann MA. Frequency and clinical significance of failure to visualize the conus artery during coronary arteriography. Circulation. 1981;63:833-7.Reference #3: de Agustín JA, Marcos-Alberca P, Hernández-Antolín R, Vilacosta I, Pérez de Isla L, Rodríguez E, Macaya C, Zamorano J. Collateral circulation from the conus coronary artery to the anterior descending coronary artery: assessment using multislice coronary computed tomography. Rev Esp Cardiol. 2010 Mar;63(3):347-51.DISCLOSURES: No relevant relationships by Ibrar AnjumNo relevant relationships by Umer Zia SESSION TITLE: Complications and Cardiovascular Disease SESSION TYPE: Case Reports PRESENTED ON: 10/17/2022 03:15 pm - 04:15 pm INTRODUCTION: Kawasaki disease is a vasculitis of unknown etiology that involves medium-sized arteries throughout the body. It primarily affects young children but may have long-term morbidity up until adulthood. Kawasaki disease often causes cardiovascular abnormalities, most commonly coronary artery aneurysms [1]. The right and left coronary arteries, which arise from the ascending aorta, are the main supply line of the heart. Only 10% of the blood supply usually comes from collateral circulation. Raymond de Vieussens, in 1706 was the first person to study and report the intracoronary pathway between branches of the right coronary artery (RCA) and the left anterior descending artery (LAD), later named the circle of Vieussens. In patients with coronary artery stenosis, the Conus artery can serve as a principal source of collateral circulation. The Vieussens ring serves as a collateral connection between the conus branches of the RCA and LAD and is usually seen in patients with coronary-to-pulmonary fistulas. The Vieussens ring has specifically been described as connecting the conus artery and the proximal right ventricular branch of the LAD. We report a case of Kawasaki’s disease with severe coronary artery disease and the presence of a Vieussens arterial ring. CASE PRESENTATION: A 26-year-old Asian American female with a history of Kawasaki’s disease presented to the office with complaints of left shoulder discomfort. She also had a coronary artery aneurysm since birth. The patient denied shortness of breath, chest pain, palpitations, or dizziness. The Family history of cardiovascular disease was nonsignificant. She is hemodynamically stable, and her physical exam was normal. Serum biochemistry showed normal lipid and thyroid levels. EKG did not show any acute ischemic changes. Troponin level was normal. Transthoracic echocardiogram showed a preserved left ventricular ejection fraction of 55%. Coronary Computed Tomography Angiography (CCTA) showed LAD dissection along with high-grade stenosis in the proximal LAD at the junction between the first and second diagonal artery. Consequently, left heart catheterization was done to evaluate epicardial coronary artery stenosis, which revealed chronic total obstruction (CTO) of the proximal LAD just before the large diagonal branch took off. The left circumflex artery and the RCA had no angiographically significant disease; however, a right-to-left collateral arterial ring was seen supplying blood to the LAD in an anterograde fashion from the RCA through a 2.5 mm collateral consistent with a circle of Vieussens (Fig I). Left ventricular angiogram showed ejection fraction above 60% and normal aortic root caliber. No subclavian dissection was seen. The patient was continued on medical therapy with risk factor modification for Kawasaki Disease with a plan to re-evaluate for coronary ischemia in 4-6 weeks. DISCUSSION: Most patients with coronary artery disease and CTO have collateral circulation. The location and the extent of collaterals are variable, which determine ischemic symptoms and left ventricular contractility. The Conus artery is not visualized in 20% of coronary angiograms due to its independent origin or the injection of contrast distal to the origin [2]. 30- 50% of the population, the Conus artery originates in an independent ostium in the right sinus of Valsalva, anterior and superior to the ostium of the RCA which is an isolated Conus artery (ICA). The course of the Conus artery is usually short and rarely forms a channel with the LAD artery (left Conus artery), known as the circle of Vieussens [3]. To our knowledge, this is the first reported case of Vieussens arterial ring as one of the coronary anomalies a patient with Kawasaki disease, which resulted in a protective, life-saving effect for the patient. Despite the CTO of the proximal LAD, our patient continued to get sufficient blood supply in anterograde fashion through the low-pressure system of the circle of Vieussens. Therefore, the patient remained asymptomatic with preserved LV function. The absence of collaterals or occlusion of the collaterals would have resulted in severe ischemia in the region of LAD. CONCLUSIONS: The circle of Vieussens is a rare collateral circulation between the RCA and the LAD, maintaining coronary circulation in dissection or severe stenotic lesions. It involves the proximal portions of the left or right coronary arteries, thereby helping to preserve normal heart functions in Kawasaki disease. Reference #1: Newburger JW, de Ferranti SD, Fulton DR. Cardiovascular sequelae of Kawasaki disease:management and prognosis. UpToDate website. Updated December 10, 2019. Accessed August 9, 2021. Reference #2: Levin DC, Beckmann CF, Garnic JD, Carey P, Bettmann MA. Frequency and clinical significance of failure to visualize the conus artery during coronary arteriography. Circulation. 1981;63:833-7. Reference #3: de Agustín JA, Marcos-Alberca P, Hernández-Antolín R, Vilacosta I, Pérez de Isla L, Rodríguez E, Macaya C, Zamorano J. Collateral circulation from the conus coronary artery to the anterior descending coronary artery: assessment using multislice coronary computed tomography. Rev Esp Cardiol. 2010 Mar;63(3):347-51. DISCLOSURES: No relevant relationships by Ibrar Anjum No relevant relationships by Umer Zia