Outcome of unroofing procedure for repair of anomalous aortic origin of left or right coronary artery

BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) from an incorrect sinus of Valsalva is a relatively rare congenital defect and is associated with sudden death. Several surgical techniques have been described to address this defect, but functional outcome has never been addressed. In this report we evaluate a consecutive series of patients treated with unroofing techniques using transthoracic echocardiography, graded exercise testing, and stress echocardiography to assess functional repair. METHODS: Nine consecutive patients (range 7 to 65 years) underwent surgical repair of AAOCA from 1995 to 2001. In all patients the intramural segment was at or below the level of the commissure. All patients underwent a modified unroofing procedure to move the coronary artery orifice to the appropriate sinus. In 2 patients, a new orifice was created without significant unroofing and disruption of the commissure. Patients were evaluated prospectively with exercise electrocardiography testing and by resting and stress echocardiography. RESULTS: Of the 9 patients, 8 presented with symptoms suggestive of ischemia (chest pain, dyspnea on exertion, or syncope). Six patients had anomalous left main coronary artery arising from the right sinus of Valsalva, and 3 patients had anomalous right coronary artery from the left sinus of Valsalva. Transthoracic echocardiography and graded exercise testing was performed in all 9 patients (mean 29 months, range 4 to 85 months) after repair. Of the 9 patients, 8 also underwent stress echocardiography. In 8 of 9 patients the newly created coronary artery ostium was visualized by either two-dimensional echocardiography or color flow Doppler. All patients were symptom free at the time of follow-up. Exercise stress echocardiography was negative in all patients. Of the 8 patients, 7 had normal left ventricular shortening. No patients had regional wall motion abnormalities suggestive of ischemia. All patients were intervention free except 1 patient who developed severe aortic insufficiency and underwent a subsequent Ross procedure 44 months after his initial procedure. CONCLUSIONS: Anomalous origin of a coronary artery from an incorrect sinus of Valsalva is known to be associated with increased risk of sudden death. Surgical correction can be carried out with minimal risk and good anatomic and functional results. Manipulation of the commissure can be avoided by creation of a neo-ostia without extensive unroofing of the intramural segment or manipulation of the intercoronary commissure. This may avoid aortic valve malfunction.     

Catastrophic myocardial ischemia resulting from a left coronary artery anomaly with an origin in the right sinus of Valsalva

A congenital left coronary artery anomaly originating from the right aortic sinus is a rare congenital defect associated with the risk of sudden death in young individuals. In most cases, the proximal portion of the anomalous left coronary artery exists between the ascending aorta and pulmonary trunk, and it has an intramural aortic course; this could critically impair the left coronary flow owing to compression of the anomalous left main trunk between the great vessels during exercise. Herein, we report a 14-year-old boy who experienced cardiac collapse due to an acute myocardial infarction after long-distance running. After resuscitation using percutaneous cardiopulmonary support, computed tomography and coronary angiography revealed an anomalous origin of the left main coronary artery in the right sinus of Valsalva and a proximal course between the aorta and pulmonary trunk. The patient was successfully treated using an unroofing procedure of the intramural left coronary artery.     

Unroofing aortic intramural left coronary artery arising from right pulmonary artery

We report an operative case of the anomalous origin of the left coronary artery from the right pulmonary artery with aortic intramural course. This particular anatomy was clearly identified with a preoperative angiogram. The restoration of the two-coronary system was accomplished by unroofing the intramural segment into the left sinus of Valsalva with encouraging midterm results. The details of this anatomical correction are described with a review of the literature.     

The "hinge-twist" technique for anomalous origin of the left coronary artery

A murmur was heard in an asymptomatic boy (age 4), and transthoracic echocardiography revealed anomalous origin of the left main coronary artery (LMCA) from the right sinus of Valsalva (age 6). Confirmed by catheterization and computed tomographic angiography (age 10), the LMCA followed a short interarterial course between the aorta and main pulmonary artery before supplying the anterior descending and circumflex coronary arteries. An intramural segment was not clearly seen. Results of stress testing were normal. Because sudden death was a concern, the patient underwent surgery at age 11. The "hinge-twist" technique was utilized in the absence of an intramural component or ostial stenosis in an effort to avoid the long-term complications of coronary reimplantation, ostial patching, or bypass grafting.     

Aortic intramural coronary artery in three hearts with transposition of the great arteries

Three hearts with transposition of the great arteries had an aberrant coronary artery coursing between the aortic and pulmonary roots. This coronary artery remained embedded in the aortic wall for some distance. In each case, on external inspection, the abnormal artery presented as a separate vessel at the site from which it usually originates in the left sinus of Valsalva. Internal inspection showed that in two cases the aberrant coronary orifice was in the posterior sinus, intimately related to the commissure between the left and posterior sinuses. In the third case the orifice was in an ectopic high position above this commissure. Diagnostic and surgical implications of this rare coronary arterial configuration are discussed.     

Surgical Repair of Anomalous Coronary Arteries Arising from the Opposite Sinus of Valsalva in Infants and Children

Abstract   Background: Unroofing of anomalous coronary artery originating from the opposite sinus of Valsalva has become the procedure of choice for this congenital lesion, with surgery performed in children as young as two years old. An increasing number of this anomaly is diagnosed in infancy with no clear indication whether surgical repair should be done in this age group. This paper reviews our experience with this anomaly, and focuses on its surgical management in infants. Methods: Between April 2002 and February 2007, eight patients underwent surgical repair of anomalous coronary artery arising from the opposite sinus of Valsalva and coursing between the aorta and pulmonary artery. Patients' age varied from two months to 28 years with a mean of 11.7 ± 11.1 years. Surgical Technique: Surgical repair involved unroofing the intramural segment of the anomalous coronary artery using cardiopulmonary bypass. Results: Two patients were younger than one year (Group A), and six patients were older than one year (group B). The mean intensive care unit stay was 2.5 ± 0.7 days for Group A and 2.8 ± 1.9 for Group B. The mean hospital stay was 4 ± 1.4 days for Group A and 4.3 ± 2.4 days for Group B. There was no mortality and no complications. The mean follow-up period is 14 ± 15.7 months with a range of one to 39 months. At the time of the last follow-up, all patients were asymptomatic in New York Heart Association class I and follow-up echocardiography on six of eight patients showed wide open coronary ostium. Conclusion: Unroofing the anomalous coronary artery arising from the opposite sinus of valsalva can be done in infants with minimal morbidity and mortality. Longer follow-up is needed to assess long-term results.     

Patch angioplasty and neo-ostium creation for intramural left coronary artery

Anomalous aortic origin of the coronary artery is a rare cardiac anomaly which induces myocardial ischemia and is associated with sudden death. We operated on a 25-year-old female with syncopal episodes who had an intramural left coronary artery. A neo-ostium was created in the left sinus but the initial neo-ostium seemed small because of the hypoplastic intramural segment of the left coronary artery. Therefore, saphenous vein patch angioplasty was added for ostial enlargement. The patient was symptom-free at one year follow-up and exercise stress test was negative for ischemia.     

Repair of anomalous origin of right coronary artery from the left sinus of Valsalva

Anomalous origin of the right coronary artery from the left sinus of Valsalva is a rare congenital anomaly but a relatively frequent cause of sudden death in the young. The medical records of 4 consecutive patients with this anomaly were retrospectively reviewed. The first 2 patients underwent coronary artery bypass and had early graft failure. The next 2 patients underwent coronary reimplantation and unroofing and have done well. This limited series suggests that bypass procedures used to treat anomalous origin of the right coronary artery from the left sinus of Valsalva are prone to early graft failure.     

Stentless aortic root bioprosthesis implantation and coronary artery bypass grafting in a patient with single right coronary artery.

Coronary arteries with anomalous origin from the aorta can be a risk factor during aortic root procedures. We report on the successful management of aortic root surgery in a 76-year-old man with a single coronary ostium. Preoperative computed tomography and angiography revealed an anomalous course of the left main coronary artery from the right sinus of Valsalva. A stentless aortic root bioprosthesis (Prima Plus) was implanted using a modified subcoronary technique. The origin of the left main coronary artery was approximately 2 mm beyond the ostium of the common trunk. Attention to the anatomic relationship of the anomalous coronary arteries to the aorta by clarifying the anatomy of coronary arteries in advance allowed us to safely perform aortic root surgery in a patient with an anomalous origin of the coronary arteries.     

Management of Anomalous Left Circumflex Artery Encircling the Aortic Annulus in a Patient Undergoing Multivalvular Surgery

Abstract   The abnormal origin of left circumflex artery from the right sinus of Valsalva with a retroaortic course is a well-known coronary anomaly usually without consequences. In patients undergoing aortic valve replacement, this finding becomes crucial because the left circumflex is at risk of injury during the procedure. The scenario is even more complex in patients undergoing multi-valve surgery. We report the diagnosis and successful operative strategy in a patient with anomalous left circumflex arising from the proximal right sinus of Valsalva undergoing double aortic and tricuspid valve surgery for active bacterial endocarditis.     

A case of surgical repair of aortic dissection associated with tear of the right coronary artery]

A 39-year-old female with dissecting aortic aneurysm of DeBakey type I, underwent replacement of the ascending aorta. We found that the right coronary artery originated just distally from the left sinus of Valsalva, run transversally in the aortic wall. Because the entry of dissection occurred at the right coronary artery, it was torn longitudinally. Aorto (graft)-rt. coronary bypass grafting was performed. She had no ischemic heart symptoms postoperatively. It is supposed that the dissecting aortic aneurysm was caused by the anomalous origin of the right coronary artery.     

Extended unroofing procedure for creation of a new ostium for anomalous left coronary artery.

A modified procedure to create an alternative ostium for the left coronary artery was successfully carried out in a patient having anomalous origin of the left coronary artery from the right coronary sinus of the aorta. The proximal portion of the artery had an intramural course. The newly constructed orifice was widely patent and functioning well 44 months later, without episodes of myocardial ischemia or aortic regurgitation.     

Anomalous origin of the right coronary artery from the left coronary sinus: case report and review of surgical treatments

An anomalous right coronary artery arising from the left sinus of Valsalva is a rare but potentially lethal abnormality. We present a case report and literature review of this anomaly as well as its surgical management in the face of unobstructed distal coronary arteries. Furthermore we report the use of intraoperative transesophageal stress echocardiography to evaluate adequacy of graft flow.     

Rare type of congenital aneurysm of the right sinus of Valsalva protruding superiorly into the pericardial space

We report a rare type of aneurysm of the sinus of Valsalva. The orifice of the aneurysm was in the right sinus of Valsalva and protruded superiorly, which is a direction not typical of right sinus of Valsalva aneurysms. Intraoperatively, it became clear that the aneurysm was located in the right sinus of Valsalva with extracardiac protrusion. Inspection through the aortotomy revealed that the orifice of the aneurysm opened between the orifice of the right coronary artery and the commissure between the right and left coronary cusps. The aneurysm was obliterated with a pledgeted suture from outside the orifice of the aneurysm. Early prophylactic surgical treatment of the aneurysm makes the operation simple and prevents subsequent development of complications, such as compression of the coronary artery, thrombosis, and/or spontaneous rupture.     

Two cases of anomalous origin of LAD from right coronary artery requiring coronary artery bypass

We experienced two cases with anomalous origin of the left anterior descending artery (LAD) from the proximal right coronary artery requiring coronary artery bypass grafting. A 66-yr old female with a long history of angina and a positive stress test had the anomalous artery coursing anterior to the right ventricular outflow tract. A 42-yr old male with worsening angina after an anteroseptal myocardial infarction had the anomalous artery running between the great vessels. Both patients underwent left internal mammary artery-to-LAD bypass on the beating heart with complete resolution of ischemic symptoms.Isolated coronary artery anomaly is an uncommon disease (0.6-1.2%) in patients undergoing cardiac catheterization. An anomalous origin of the LAD from the proximal right coronary artery (RCA) or the right sinus of Valsalva (RSV) is very rare, found in 1.2-6.1% of all coronary anomalies. This coronary anomaly has been considered potentially serious but functionally unimportant. We report two cases of anomalous LAD from the proximal RCA resulting in anterior wall ischemia which was effectively treated by coronary artery bypass surgery.     

A giant unruptured aneurysm of the sinus of Valsalva together with ectasia of the left coronary artery

A sinus of Valsalva aneurysm is defined as a dilatation of the aortic sinuses, between the aortic valve annulus and the sinotubular junction. They are rare and most frequently involve the right coronary sinus. We report a case of an unruptured giant sinus of Valsalva aneurysm in a patient associated with ectasia of the left main stem and left anterior descending coronary artery. The patient was successfully treated with aortic root replacement using a biologic conduit.     

Separate Origin of the Main Components of the Left Coronary Artery in Syrian Hamsters (Mesocricetus auratus)

This study describes a rare congenital coronary artery anomaly in the Syrian hamster; namely, the separate origin of the obtuse marginal and left circumflex arteries which are the main components of the left coronary artery. The hearts of nine affected animals were examined by means of a corrosion‐cast technique and histology. The hamsters belonged to a laboratory inbred family with a high incidence of coronary artery anomalies and bicuspid aortic valve. The aortic valve was tricuspid in three hamsters and bicuspid in the other six hamsters. In all cases, the right coronary artery was normal, whereas the left coronary artery main trunk was absent. The present anomalous coronary artery patterns could be classified into two main entities: (i) ectopic origin of the obtuse marginal artery from the right aortic sinus or from the right coronary artery, with the left circumflex artery arising from the left side of the aortic valve; and (ii) ectopic origin of both the obtuse marginal artery from the right aortic sinus or from the right coronary artery and left circumflex artery from the dorsal aortic sinus. In all cases, the obtuse marginal artery coursed to the right side of the heart through the ventral wall of the right ventricular outflow tract. When the left circumflex artery arose from the dorsal aortic sinus, it formed an acute angle with the aortic wall. This report seems to be the first to describe the separate origin of the main components of the left coronary artery in a non‐human mammalian species. In man, the congenital coronary artery and aortic valve defects reported herein may entail the risk of clinical complications. However, none of the affected hamsters showed signs of disease.     

Right coronary artery with high takeoff

Anomalous origin of the right coronary artery is an extremely rare anomaly. We describe the case of a patient whose right coronary artery was arising from the ascending aorta with high takeoff. We diagnosed the anomaly incidentally during the operation. After transverse aortotomy for aortic valve replacement, we recognized the transection of the right coronary artery. The right coronary artery ostium was located approximately 5 cm above the right sinus of Valsalva. It was showing a complete transmural course. We repaired the right coronary artery by bypassing it with a saphenous vein graft.     

Aneurysm of sinus of Valsalva

Aneurysm of sinus of Valsalva is a rare cardiac abnormality with congenital origin in most of the cases. If it is located in the right coronary sinus, it usually ruptures into a right heart chamber and frequently a ventricular septal defect (VSD) coexists with this condition. Early diagnosis and immediate surgical treatment can save the patient's life in most cases. All the 3 cases reported in this series had aneurysm of right sinus of Valsalva with associated VSD and mild degree of aortic regurgitation (AR). Two of the cases ruptured aneurysm into the right ventricle. Trans-esophageal echocardiography was used to confirm the diagnosis and all three showed good results with surgery.     

A rare case of an extracardiac unruptured aneurysm of the sinus of valsalva in a patient with single coronary artery

A 75-year-old man with gastric cancer underwent preoperative cardiac examination by echocardiography, and an unruptured extracardiac aneurysm was detected in the right sinus of Valsalva. Coronary angiography by multidetector computed tomography demonstrated a single left coronary artery. Patch closure of the orifice of the aneurysm of the right sinus of Valsalva was successfully performed for this extremely rare combination.