Hypoplastic left heart syndrome with anomalous origin of left coronary artery from the right pulmonary artery: successful surgical treatment in a neonate.

Anomalous origin of the left coronary artery from the right pulmonary artery in association with hypoplastic left heart syndrome is a rare congenital anomaly. We describe a successful simultaneous surgery for both anomalies during the first stage palliation in a neonate.     

Anomalous origin of the right coronary artery from the pulmonary artery.

Three patients with anomalous origin of the right coronary artery from the pulmonary artery are presented together with a review of the 14 patients with this anomaly previously reported. One of the current patients is the youngest to be diagnosed before operation and the youngest to undergo corrective operation. Although the lesion is usually asymptomatic, it may contribute to sudden death. The only suggestive physical finding is a continuous murmur with diastolic accentuation, which is present only in some patients. There are no diagnostic electrocardiographic or chest roentgenographic changes. Diagnosis is made best by selective left coronary arteriography showing retrograde filling of the right coronary artery from collateral vessels. Operative repair should consist either of direct reimplantation of the proximal right coronary artery into the aortic root or of saphenous vein bypass graft to the coronary artery with ligation of its origin. Operation is recommended when the lesion is recognized.     

A very rare case of single coronary artery anomaly, with left anterior descending and left circumflex arteries originating separately from proximal right coronary artery

Coronary artery anomalies are rare, with the reported prevalence of 0.2% to 1% in routine angiographic studies. Among them, presence of a single coronary artery is one of the rarest anomalies, comprising less than 3% of all coronary anomalies. In this article we report a case of single coronary artery anomaly with left anterior descending and left circumflex arteries arising separately from proximal right coronary artery, and left anterior descending artery reaching atrioventricular sulcus by passing between aortic and pulmonary artery trunci. The patient underwent off-pump coronary artery bypass grafting and is currently symptom-free.     

Cardiac arrest during exercise: anomalous left coronary artery from the pulmonary trunk

Anomalous origin of the left coronary artery from the pulmonary trunk is associated with high mortality in infancy but in rare cases the condition is diagnosed in adults. The present report describes three adult cases of this anomaly. Two of the patients (age 18 and 34 years) were resuscitated from cardiac arrest, which had occurred in relation to physical exercise. The third patient (20 years) had presented with angina pectoris and signs of ischaemia on exercise ECG. In all patients, coronary arteriography revealed a large right coronary artery with collateral filling of the left coronary artery, which originated from the pulmonary trunk. A successful surgical correction of the anomalous coronary artery was performed in two of the patients.     

Hypoplastic left heart syndrome with anomalous origin of the right coronary artery

Hypoplastic left heart syndrome in association with an anomalous origin of a coronary artery from the pulmonary artery is a very rare congenital malformation. In the few reported cases, the left coronary artery or the circumflex artery arises from the right pulmonary artery. We describe a newborn who presented with hypoplastic left heart syndrome, and at the time of operation had an anomalous origin of the right coronary artery from the right pulmonary artery that was detected. The patient underwent a successful modified Norwood procedure with direct reimplantation of the right coronary artery to the neo-aorta.     

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.     

Surgical treatment of anomalous coronary artery arising from the pulmonary artery

Anomalous coronary arteries arising from the pulmonary trunk is a rare but potentially fatal condition. We report the clinical presentation, surgical treatment and long-term follow-up of seven surgical cases of anomalous left coronary and one case of anomalous right coronary artery arising from the pulmonary artery. Age ranged from 7 months to 13 years (average: 5.09+/-3.7 years) and weight ranged from 7 to 50 kg (average: 19.9+/-8.8 kg). Follow-up was 100% complete: average 78 months (S.D.: 52.7 months). Direct reimplantation was the surgical technique in six cases, Takeuchi procedure in one case and subclavian artery interposition in one case. Concomitant mitral valve repair was undertaken in two cases. In two children the coronary artery anomaly was diagnosed and treated only after a first surgery for other congenital heart anomaly. Left ventricle ejection fraction was restored in those cases of pre-operative dysfunction. Mortality was not observed and all children are asymtomatic and free of reoperation.     

Anomalous origin of the pulmonary artery from the right coronary artery

A 5-year-old boy who had an anomalous origin of a stenotic pulmonary artery from the right coronary artery with ventricular septal defect and pulmonary atresia is reported. The diagnosis was made at operation. Successful repair was achieved for the complex anomaly by using a pulmonary artery homograft as a conduit and closing the ventricular septal defect.     

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presenting with ventricular fibrillation in an adult: a case report

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. The usual clinical course is severe left sided heart failure and mitral valve insufficiency presenting during the first months of life. However, in some cases collateral blood supply from the right coronary artery is sufficient and symptoms may be subtle or even absent. Arrhythmias or sudden cardiac death in adult life may be the first clinical presentation in patients with ALCAPA. We report a case, where a 39-year old woman presented with ventricular fibrillation during phycial exertion. Coronary angiography and CT-angiography revealed an anomalous origin of the left coronary artery, and an aortic reimplantation of the left coronary artery was performed followed by ICD implantation. A review of the literature on ALCAPA is presented along with CT images before and after surgery.     

Left superior pulmonary artery sling

A pulmonary artery sling is formed when the left pulmonary artery originates from the right pulmonary artery and encircles the distal trachea, coursing between the trachea and esophagus to reach the hilum of the left lung. Pulmonary artery slings are often associated with distal tracheal narrowing, due to either intrinsic stenosis or secondary compression by the anomaly itself. We report a very rare case in which the left superior pulmonary artery originated from the right pulmonary artery and then acted as a sling, the left inferior pulmonary artery originated from the pulmonary trunk.     

Surgical correction of congenital left coronary artery-main pulmonary artery fistula in association with anomalous right coronary artery.

The association of a left coronary artery-main pulmonary artery fistula and an anomalous right coronary artery originating from the main pulmonary artery is the subject of this report. This unique combination of congenital cardiac anomalies establishes a double coronary steal from the left coronary artery, which hemodynamically represents the sole source of myocardial perfusion. The left coronary artery-main pulmonary artery fistula was closed and the coexisting anomalous right coronary artery reimplanted into the anterior aspect of the ascencing aorta. A dual coronary supply was therefore established and thus eliminated the potential threat of total myocardial ischemia should the left coronary artery become critically compromised. Patency of both the left coronary artery and the transplanted right coronary artery was documented 1 year postoperatively by aortic root angiography.     

Communication between the right pulmonary artery and left atrium with aneurysm formation

Communication between the pulmonary artery and the left atrium is a rare congenital anomaly. A 7-year-old boy was referred to our hospital with a diagnosis of pulmonary arteriovenous fistula. Referral to a pediatric cardiologist had been delayed because he had only slight cyanosis and no heart murmur. Catheterization and computed tomography scans confirmed that the right pulmonary branches and pulmonary venous returns were normal and that an additional aneurysmal communication was located between the right pulmonary artery and the left atrium. Surgery proceeded uneventfully together with a cardiopulmonary bypass. The connection from the right pulmonary artery to the aneurysm was ligated, and the connection from the left atrium to the aneurysm was closed with a polytetrafluoroethylene patch. We describe this rare anomaly with cyanosis and no heart murmur.     

Right pulmonary artery agenesis and coronary-to-bronchial artery aneurysm

Isolated unilateral pulmonary artery agenesis is a rare congenital anomaly that may be complicated with hemoptysis, recurrent pulmonary infections or pulmonary hypertension. To our knowledge the occurrence of a coronary syndrome associated with a coronary-to-bronchial artery saccular aneurysmal collateralization has never been described before. A 44-year-old female presented a congenital right pulmonary artery agenesis associated with a hypotrophic and multicystic right lung complicated with recurrent bronchitis. This patient had a coronary syndrome for which the coronary artery imaging showed a coronary-to-bronchial artery collateralization with an aneurysm at this level. It gives rise to a coronary syndrome by coronary steal. Two bronchial collaterals arising from a diaphragmatic artery and the subclavian artery were also found on the computed tomography (CT)-scan. This last collateral also showed another saccular aneurysm. We first performed an embolization of those two aneurysms in order to decrease the risk of hemorrhage and coronary steal, before performing a right pneumonectomy. In this case, the surgery was indicated because of the pathological lung and the risk of postembolization ischaemia. The postoperative course was uneventful and the patient was doing well six months later.     

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.     

Anomalous left main coronary artery arising from the pulmonary artery in an adult: Treatment by direct reimplantation

We herein report the case of a 37-year-old woman in whom an anomalous origin of the left coronary artery from the pulmonary artery was surgically corrected. A magnetic resonance angiogram showed the left main coronary artery connecting to the right posterior portion of the pulmonary trunk, and exercise-stressed thallium-201 perfusion scintigrams demonstrated a large reversible anterior defect. She was successfully treated by direct aortic reimplantation of the abnormal left coronary artery. We were able to obtain a sufficient length of the left main trunk by excising the large cuff of pulmonary artery wall surrounding the ostium of the anomalous left coronary artery while transecting the pulmonary artery. Postoperative angiograms demonstrated a widely patent left coronary artery, a decrease in the size of the right coronary artery, and no collaterals, and exercise-stressed thallium-201 perfusion scintigrams demonstrated no remaining ischemic defect at all. Direct aortic reimplantation is an ideal operation but is still limited by the anatomical position of the left coronary artery. In this case, magnetic resonance angiography was an excellent method for deciding the optimum operative procedure for the anomalous left coronary artery. In addition, exercise thallium-201 scintigraphy was found to be useful in recognizing the revascularized effect of the left ventricle.     

Anomalous LAD and CX artery arising separately from the proximal right coronary artery--a case report of single coronary artery with coronary artery disease

Coronary artery anomaly has been reported at a rate of 0.6% to 1.3% in routine angiographic series. Moreover, single coronary artery is one of the rarest anomalies among coronary anomalies. Eventhough patients with coronary anomalies are usually asymptomatic, they may also be associated with myocardial ischemia, ventricular fibrillation, syncope, congestive heart failure, and sudden death. In this article, we report a case of single coronary artery anomaly with the left anterior descending (LAD) and left circumflex (LCx) coronary artery arising separately from the proximal right coronary artery. Since the presented case was associated with ischemic heart disease, coronary artery bypass grafting was carried out. He is currently well.     

Anomalous origin of the left coronary artery from the pulmonary artery: successful direct reimplantation in a 50-year-old man.

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital coronary artery anomaly that is often referred to as Bland White Garland syndrome. Most patients with this anomaly require surgical intervention early in life, and it is extremely rare that patients reach middle age without any symptoms. We present a 50-year-old man with this anomaly, who underwent direct reimplantation of the left main coronary trunk to the ascending aorta. His postoperative course was uneventful, and three and a half years after the operation, he is well and does not require medication. Several surgical procedures can be used to treat this anomaly, but we prefer to use direct reimplantation, whenever technically possible. To our knowledge, this patient is the oldest patient to have undergone a direct reimplantation without any angioplasty.     

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.     

Anomalous origin of the left coronary artery from the pulmonary artery. Surgical repair of an unusual form

The authors present a case of a 24-year-old woman with anomalous left coronary artery originating from the pulmonary artery. Surgical correction was performed by the direct transposition of the anomalous left coronary artery from the pulmonary artery to the ascending aorta, without interposed graft. Pre- and postoperative evaluations, including angiograms and cycloergometer stress test are presented. The advantages of this technique are discussed, emphasizing the creation of a two-coronary system without the use of prosthetic, arterial or venous grafts. In the present case the left coronary artery originated from the right posterior sinus of the pulmonary artery. The patient is doing well 23 months after operation.     

Anomalous origin of the left main coronary artery from the main pulmonary artery in an elderly patient

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome is an extremely rare diagnosis in elderly patients. We describe a 73-year-old female with ALCAPA who underwent successful repair of this coronary anomaly.