Anomalous origin of all three coronary arteries from right sinus of Valsalva.

Congenital anomalies of the coronary arteries, although uncommon, have the potential to cause serious myocardial damage, ischemic cardiomyopathy, and sudden cardiac death. We report a rare case of origin of all three coronaries from the right sinus of Valsalva.

Introduction

Congenital anomalies of the coronary arteries, although uncommon, could cause serious myocardial damage, ischemic cardiomyopathy, and sudden cardiac death. The anomalous origin of coronary artery has an incidence ranging from 0.03% to 5.64%. These anomalies vary with respect to number, location, orientation of the ostia, and origin of the coronary arteries. Some anomalies are merely anatomic variants without clinical relevance, while others may be life threatening. The ectopic origin of the left coronary artery from the right sinus of Valsalva is even rarer, occasionally associated to potentially serious sequelae. It has been reported that this type of anomaly is observed in approximately 0.08% of the population with an anomalous coronary artery.

Case report

A 58-year-old Caucasian man was admitted to our hospital complaining of worsening chest pain at rest, accompanied by diaphoresis. His risk factors for atherosclerosis were a history of smoking, diabetes type II and family history of coronary artery disease. He had also chronic obstructive pulmonary disease on oxygen treatment at home. In the emergency room of our hospital, his blood pressure and heart rate were normal. A 12-lead electrocardiogram showed nonspecific ST-T wave abnormalities. He had elevated cardiac Troponin (5.46 ng/L, normal <0.04 ng/L) and no other significant abnormalities were detected in his blood test. Echocardiography revealed preserved left ventricle systolic function with concentric left ventricle hypertrophy and no abnormalities of wall kinesis. Diagnosis of non-ST elevated myocardial infarction was made and emergent cardiac catheterization was performed because of the persistent chest pain. A 6 Fr Left Judkins and after 6 Fr Amplatz left 2 catheter were engaged unsuccessfully in the left sinus of Valsalva. Angiography showed therefore no vessels originating from the left coronary sinus of Valsalva. Right sinus was instead successfully cannulated and visualized with a 6 Fr Right Judkins catheter. At this time, we noticed that left anterior descending artery (LAD) and left circumflex artery (LCX) originated both from the right sinus of Valsalva (Fig. 1). No left main coronary artery (LMCA) was demonstrated. The right coronary artery (RCA) was dominant and had moderate stenosis on the first segment of the mid tract. The LAD showed a subtotal occlusive lesion in its mid portion. A Sion blue guidewire was passed through the LAD artery and the culprit lesion on the LAD was stented successfully with two Sirolimus DES in overlap (3.5 mm × 16 mm and 3.5 mm × 20 mm). Final angiography demonstrated TIMI 3 flow (Fig. 2). The patient's chest symptom fully resolved after the intervention. He followed an uneventful hospital course and consequently discharged in good clinical conditions on the fifth day of his hospitalization.

Fig. 1

(A) Left oblique anteriorcranial view shows that the entire coronary system arises from a single ostium in the right sinus of Valsalva. The mid tract of RCA has a moderate stenosis. (B) In this right oblique anterior view is more evident the course of LAD and of the LCX.

Fig. 2

(A) Right cranial oblique anterior view shows LAD subtotally occluded in its medium tract (white arrow). (B) Angiographic result of the LAD after percutaneous coronary intervention with stents deployment.

Discussion

Several classification systems for coronary artery abnormalities have been created. Yamanaka and Hobbs classified coronary variation based on origin and anatomical course relating to the ascending aorta and pulmonary trunk. The case here presented, namely LAD and LCX originated separately, belongs to the fifth type. In particular it is a combined type in whom the LAD passes anterior to the pulmonary artery, without an interarterial course; while the septal branch and the LCX are posterior to the aorta. This type of anomaly is very rare but potentially life-threating. We suspected the anomaly by visualization of an “avascular area” in the distribution of the left coronary artery disease and the absence of collaterals. However, sometimes the aberrant vessel cannot be found by coronary artery angiography and multidetector computed tomography may be helpful for diagnosis. Because of the unusual direction of the coronary artery and tortuous angle, catheterization of the single coronary ostium was difficult. It is also true that there can be serious complications of this procedure. The management of patients with an anomalous coronary artery is one of the most controversial aspects of this condition since no official guidelines have been published. It includes medical treatment or observation, coronary angioplasty with stent deployment, or surgical repair.4, 5 The therapy of the anomalously originating left coronary artery from the right sinus of Valsalva is mainly based on observations and experts opinion, since the literature consists of case reports. The lack of a widely accepted consensus is due to great heterogeneity of the pathophysiological characteristics and clinical manifestations. Surgical revascularization involves aortocoronary bypass, ostial reimplantation or the unroofing technique, which unbind the intramural segment of the coronary artery anomaly. The latter is realized by means of an incision in the wall shared by the coronary artery anomaly and the aorta. In this way a new larger orifice in the appropriate sinus could be created. There is a great controversy concerning surgical revascularization in these patients, especially in young people. Aortocoronary bypass grafting has been strongly criticized because of its “limited” patency and the inevitable competitive flow between the bypass and the coronary artery anomaly. However, in our case percutaneous angioplasty was chosen due to the persistent chest pain and to the positive markers of myocardial necrosis even without typical electrocardiogram or echo abnormalities. In conclusion, it is crucial to be always aware of anomaly origin of coronary arteries and try to definite their anatomical and clinical roles due to their severe life-threatening complications. In any cases, surgical or percutaneous revascularization may be a valid option for this kind of disease.

Conflicts of interest

The authors have none to declare.