Pulmonary origin of left anterior descending coronary artery in tetralogy of Fallot

We report the case of a 4-year-old girl with an anomalous origin of the left anterior descending coronary artery from the pulmonary artery in association with tetralogy of Fallot. Creation of an intrapulmonary tunnel from a newly created aortopulmonary window to the coronary ostium in the pulmonary trunk and total repair of tetralogy of Fallot were successfully carried out. The advantage of intrapulmonary tunnel technique (Takeuchi method) for this very rare combination is stressed.     

One-stage correction of tetralogy of Fallot associated with anomalous origin of the left pulmonary artery from the aorta and aortopulmonary window

We report a rare case of tetralogy of Fallot associated with anomalous origin of the left pulmonary artery from the aorta and aortopulmonary window. Successful one-stage total correction was performed under cardiopulmonary bypass, including implantation of the left pulmonary artery, repair of aortopulmonary window, and correction of tetralogy of Fallot. The patient experienced an uneventful postoperative course and was asymptomatic at one-year follow-up.     

Translocation of Anomalous Right Coronary Artery to Aortic Side of the Aortopulmonary Window: A Different Approach for a Rare Combination

Abstract   A case of aortopulmonary window associated with anomalous origin of the right coronary artery from the pulmonary artery in a four-month-old boy is reported in this paper with a different method of repair. In this patient, surgical repair was done by transferring the right coronary artery from the pulmonary artery to the aorta at the aortopulmonary window side. The defect at the pulmonary artery was repaired by a pericardial patch, so no other secondary opening was used for the aorta in order to reimplant the coronary artery .     

Aortopulmonary window and anomalous coronary artery: an exceptional association

Aortopulmonary window is rarely associated with other cardiac anomalies, such as anomalous origin of one coronary artery. This exceptional association has been reported in 15 patients, with different surgical solutions. We describe 2 patients with aortopulmonary window with anomalous origin of the right coronary artery, one of which presented with tetralogy of Fallot-pulmonary atresia. A neonatal modified Blalock-Taussig shunt and ligature of the distal pulmonary artery at the level of pulmonary bifurcation was performed in the first patient, and the second patient underwent aortopulmonary window division with anomalous coronary artery reimplantation. Both patients had an uneventful postoperative course.     

Aortopulmonary window with anomalous origin of the right coronary artery

We report a case of an aortopulmonary window with a right coronary artery arising from the pulmonary trunk. This exceedingly rare anomaly with anomalous coronary artery presented without myocardial ischemia owing to the aortopulmonary window. The correct diagnosis was made by angiography and a successful surgical correction was performed.     

一期纠治主肺动脉窗或右肺动脉起源于升主动脉

目的总结主肺动脉窗或右肺动脉起源于升主动脉的外科纠治经验。方法17例病儿中单纯主肺动脉窗5例,右肺动脉起源于升主动脉4例,主肺动脉窗伴主动脉弓中断、法洛四联症、房室间隔缺损、二尖瓣反流和气管狭窄等8例。1例无手术指征,16例行解剖纠治,平均手术年龄1．7岁;平均体重8．5 kg。结果手术无死亡。2例延迟关胸;1例再进胸止血。随访无死亡,无主动脉瓣上狭窄及肺动脉狭窄。结论主肺动脉窗及右肺动脉起源于升主动脉易早期产生肺动脉高压,一经诊断需立即手术。手术方式首选与伴发畸形一期纠治。主肺动脉窗及右肺动脉起源于升主动脉尽管多有伴发畸形,但早期手术纠治可获得较佳中、长期疗效。     

Heart transplant for anomalous origin of left coronary artery from pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery is a congenital coronary artery malformation most commonly present in infancy. A variety of surgical procedures have been described to achieve physiological correction of the coronary flow abnormalities. These techniques are effective as long as there is potential for myocardial recovery. However the sequelae of chronic myocardial ischemia that characterize this entity often irreversibly damage the heart and preclude correction and palliation of the native anomaly. In this type of setting, heart transplantation is a realistic option. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) occasionally presents in adulthood. Anatomic repair with a two coronary artery system may not be optimal in patients presenting with ischemic cardiomyopathy. We report an adult patient with platelet factor 4 (PF4) antibodies who underwent orthotopic heart transplantation (OHT) for ALCAPA.     

Anomalous origin of the left coronary artery. A twenty-year review of surgical management.

Children with anomalous origin of the left coronary artery from the pulmonary artery are at risk for myocardial infarction and death. Surgical management of this condition in children has evolved significantly during the past 20 years. Between 1970 and 1990, a total of 20 of these patients underwent surgical intervention at two institutions. Age at operation ranged from 3 weeks to 11 years (mean, 26 months). Twelve patients had congestive heart failure, three were in cardiogenic shock, and two had cardiac murmurs. Operative techniques included ligation (n = 9), subclavian artery anastomosis (n = 5), aortic implantation (n = 3), internal mammary artery anastomosis (n = 1), intrapulmonary tunnel from aortopulmonary window to coronary artery (n = 1), and cardiac transplantation (n = 1). The three deaths in the series occurred at 3 weeks, at 2 months, and at 9 years after ligation. There have been no deaths after establishment of a two coronary artery system or after transplantation. Two of the five patients who had subclavian artery anastomosis to the anomalous coronary artery have severe anastomotic stenosis and collateralization. For patients with anomalous origin of the left coronary artery from the pulmonary artery, we recommend direct aortic implantation of the anomalous coronary artery at the time of diagnosis. Intrapulmonary tunnel from aortopulmonary window to coronary artery, or aorta-coronary bypass with internal mammary artery are recommended for children in whom aortic implantation is not anatomically feasible. Left coronary artery ligation is not indicated for these patients; those who have survived ligation should be considered for elective establishment of a two coronary artery system because of the risk of late death.     

Anomalous origin of the right coronary artery with aortopulmonary window: functional and surgical considerations.

A rare case of anomalous origin of the right coronary artery from the pulmonary artery associated with a large aortopulmonary window in a 4-month-old boy is reported. The right coronary artery is exposed to systemic pressure and carries fairly well-oxygenated blood to the myocardium. Angiographic diagnosis could be difficult because of the simultaneous filling of both great arteries which obscures the origin of the anomalous vessel. Closure of the aortopulmonary window alone could result in acute myocardial ischaemia. A plastic procedure for correction of this association of defects, which should allow normal growth, is described.     

A case of coronary artery-pulmonary artery fistula in tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (MAPCA)]

A case of four year old girl with tetralogy of Fallot, pulmonary atresia, major aortopulmonary collateral arteries and coronary artery to pulmonary artery fistula is reported. The patient was misdiagnosed as truncus arteriosus, pulmonary stenosis and major aortopulmonary collateral arteries (MAPCA) preoperatively. The ligation of MAPCAs was carried out through left thoracotomy as an initial procedure, because we believed that the area fed by MAPCAs had dual blood supply. However, the patient demonstrated pulmonary infarction post operatively. This complication forced us to postpone an intracardiac repair. At the time of intracardiac repair, after mentioned diagnosis was established and a Rastelli type operation and ligation of coronary-pulmonary artery fistula were carried out. A 18 mm Carpentier-Edwards valved conduit was used as an extracardiac conduit. In conclusion: 1) We experienced a rare association of coronary artery fistula in TOF with PA. 2) The MAPCAs functioning as a sole source of blood-supply to the lung should be precisely diagnosed preoperatively and uniforcalized before an intracardiac repair.     

Mirror image Berry syndrome: a case report of complex aortopulmonary malformation in viscero-atrial situs inversus

We report a very rare case of an infant with viscero-atrial situs inversus presenting mirror image Berry syndrome (aortopulmonary window, interrupted aortic arch, anomalous origin of the left pulmonary artery, and intact ventricular septum). Successful surgical treatment was accomplished 56 days following the initial rescue bilateral pulmonary artery banding.     

An alternative procedure for correction of anomalous origin of left coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital cardiac malformation that is commonly associated with mitral insufficiency. Direct implantation of the anomalous origin of the left coronary artery from the pulmonary artery into the aorta is ideal, but it may not be fit for some patients whose anomalous left coronary arteries arise remotely from the ascending aorta. To solve the same technical problem in a boy with anomalous origin of the left coronary artery from the pulmonary artery in combination with moderate mitral insufficiency, we successfully elongated the anomalous coronary artery by creating a tube-shape graft using part of the pulmonary arterial wall. Simultaneous mitral annuloplasty was performed after a dual-coronary repair.     

Bland-White-Garland syndrome in pregnancy: reoperation of ALCAPA with an internal thoracic radial artery "Y"-graft

Anomalous origin of the left coronary artery from the pulmonary artery is rare and more so in the adult. Reimplantation of the left main coronary into the aorta is successful in early life, but it may be more difficult in the adult who had a previous repair. We report a successful reoperation of anomalous origin of the left coronary artery from the pulmonary artery in an adult patient using a left internal thoracic and radial artery "Y"-graft. Composite arterial grafting has become an established and straight forward technique in coronary surgery, and it is appropriate and recommended for congenital lesions in adults and at any age if necessary.     

Anomalous coronary artery in a transplanted heart: a technical modification.

We report the case of a 47-year-old man who underwent orthotopic heart transplantation for valvular cardiomyopathy. At the time of cardiac catheterization we identified an anomalous origin of the donor left main coronary artery from the right coronary sinus of Valsalva. To decrease the risk of left main coronary artery compression during re-implantation of the transplanted heart, we performed a careful geometric arrangement of the aortopulmonary artery angle. This simple technical modification suggests that a heart with an anomalous left coronary artery may be safely used for organ donation.     

Evolution of the management of anomalous left coronary artery: a new surgical approach

Twelve patients with anomalous left coronary artery (LCA) from the pulmonary artery were treated surgically. Mean age was 13 months and mean weight, 7.7 kg. One of the following techniques was used in 8 patients: suture obliteration of the coronary orifice, ligation of the anomalous LCA and grafting of the internal mammary artery to the distal LCA, aortic reimplantation of the anomalous LCA, creation of an aortopulmonary window, and direct anastomosis to the right subclavian artery. In 4 patients, a new technique to establish a two-coronary artery system involved tube extension of the anomalous LCA with pulmonary artery wall and then anastomosis to the right subclavian artery using cardiopulmonary by-pass without aortic cross-clamping. There were two hospital deaths (17%) and one late death. The condition of the 9 long-term survivors was improved at late follow-up. Postoperative assessment showed smaller Q wave on the electrocardiogram, reduced cardiac size, and good left ventricular function. All patients in whom the repair was done using the new technique were free from postoperative cardiac complications, had shortened hospital stays, and were alive with a patent two-coronary artery system at late follow-up. We believe this approach is a safer method for establishing a two-coronary artery system in infants and small children with an anomalous LCA arising from the pulmonary artery.     

"Spiral-cuff" technique for repair of anomalous left coronary artery from the pulmonary artery

We describe a new technique for coronary transfer in the repair of anomalous origin of the left coronary artery from the pulmonary artery. The left coronary artery is elongated with the native pulmonary artery wall to form a spiral-shaped coronary cuff to construct an unstretched new left coronary artery system from the ascending aorta. A postoperative angiographic scan showed good flow of the left coronary artery without any kinking or narrowing. This technique is considered useful when an anomalous coronary artery arises a long way from the ascending aorta.     

Repair of interrupted aortic arch and associated malformations in infancy: indications for complete or partial repair

There is uncertainty regarding the best method of repair of interrupted aortic arch. The question is whether to perform primary definitive repair of this anomaly plus the associated defects versus arch repair only and palliation of the intracardiac defects, usually by pulmonary artery banding. Since 1976, 16 infants with interrupted aortic arch have been treated surgically. They were seen at 5.2 +/- 3 days of age and weighed 3.2 +/- 0.7 kg. The interruption occurred between the left carotid and left subclavian arteries (type B) in 9 and between the left subclavian artery and the descending aorta in 7 (type A). Isolated ventricular septal defect (VSD) was the only associated anomaly in 7 and aortopulmonary window, in 4. Two patients had truncus arteriosus type 1. Three had transposition of the great arteries: 1 with VSD and 2 with single ventricle. Prior to 1980, our policy was to palliate all patients. Between 1976 and 1980, 4 infants underwent left thoracotomy with arch repair plus pulmonary artery banding (3, VSD; 7, transposition of the great vessels and single ventricle) with only 1 (25%) survivor. Because of this high mortality, 8 patients with interrupted aortic arch and VSD or aortopulmonary window, seen since 1980, received complete repair with median sternotomy, end-to-end arch anastomosis, and closure of the VSD or aortopulmonary window utilizing profound hypothermia and circulatory arrest. All 8 survived.(ABSTRACT TRUNCATED AT 250 WORDS)     

Anatomical reconstruction of aorta and pulmonary trunk in patients with an aortopulmonary window

We report a modified technique for repair of aortopulmonary window in which a native pulmonary artery flap is created and the aortopulmonary window is completely divided. The pulmonary artery flap is used to reconstruct the ascending aorta, and the defect in the pulmonary trunk is closed with an autologous pericardial patch. This repair avoids the use of a fabric patch with its inherent disadvantages and allows anatomical reconstruction of both the ascending aorta and the pulmonary trunk with low risk of early or late distortion of the aortic and pulmonary root structures.     

Reconstruction of left main coronary artery with subclavian artery free graft in an infant.

We report the case of a 3-month-old infant with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) with absent left main coronary artery (LMCA). She underwent repair by reimplantation technique with the construction of a short LMCA using two opposite flaps. Two months later she was re-admitted in cardiac failure. Occlusion of the reconstructed LMCA was found by angiogram. At reoperation the right subclavian artery was used as a free interposition graft to reconstruct the LMCA. The post-operative course was uneventful. At 8 months she was asymptomatic and LMCA patency was demonstrated by angiogram.     

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

A previously unknown coronary artery anomaly is reported: origin of the left coronary artery from the right pulmonary artery. This unusual anomaly can probably be explained by the theory of dual embryonic development of the coronary arteries from angioblastic buds in the truncus arteriosus. It is thought that these buds anastomose with a capillary network that is developing on the surface of the ventricles. Complete repair of this lesion with restoration of a two-coronary artery system was accomplished in a 10-month-old baby. The coronary artery was detached from the pulmonary artery, with a button of pulmonary artery being retained around the coronary artery ostium. Direct systemic arterial flow to the left coronary artery was established by anastomosis of the left coronary to the right subclavian artery.