Cleft mitral valve in association with anomalous left coronary artery arising from pulmonary artery

An anomalous origin of the left coronary artery from the pulmonary artery is commonly associated with mitral valve insufficiency. Usually this is secondary to left ventricular dysfunction or papillary muscle ischemia. We describe the association of an anomalous left coronary artery from the pulmonary artery with structural abnormalities of the mitral valve (cleft mitral leaflet) in 2 children, both of whom were being investigated for primary mitral valve disease. Both underwent successful operations for coronary transfer and mitral valve repair.     

The need for follow-up after surgical correction of anomalous left coronary artery arising from the pulmonary artery

Four patients are described with an anomalous origin of the left coronary artery from the pulmonary artery. Three were treated by ligation of the abnormal left coronary artery at its anomalous origin; one of them died during surgery. One patient was treated with reimplantation of the left coronary artery into the aorta. Of the survivors 2 are in normal health, but 1 developed aortic valve incompetence after reimplantation of the left coronary artery into the aorta and 1 had persistent left ventricular wall motion abnormalities and developed mitral valve prolapse. The third survivor has impaired health with diffuse left ventricular wall motion abnormality. Careful follow-up after surgery for an anomalous left coronary artery arising from the pulmonary artery is necessary for secondary prevention of problems due to incomplete recovery of myocardium or problems as a consequence of surgical therapy.     

Anomalous left coronary artery from pulmonary artery with mitral stenosis

The usual presentation of anomalous left coronary artery from pulmonary artery is severe left-sided heart failure and mitral valve insufficiency presenting during the first months of life. The manifestations of left heart failure may be masked if pulmonary artery pressure remains high. We believe this is a rarest of rare case of anomalous left coronary artery from pulmonary artery with severe mitral stenosis and pulmonary hypertension in which pulmonary hypertension, along with good collateral circulation helped to preserve left ventricular function.     

Left coronary artery from the pulmonary artery: Physiological considerations of surgical correction

A 13-year-old boy with anomalous origin of the left coronary artery from the pulmonary artery has been studied before and after anastomosis of the artery to the aorta, using a saphenous vein graft. There was suggestive evidence of improved left ventricular function following operation. The presence of associated mitral regurgitation is suspected from the auscultatory findings.     

Anomalous origin of left coronary artery from pulmonary artery in association with intracardiac lesions. Report of two cases.

Two cases are presented to illustrate the diagnostic and therapeutic dilemma when an anomalous origin of the left coronary artery from the pulmonary artery occurs with a hemodynamically significant intracardiac lesion. Hemodynamic alterations resulting from the associated intracardiac lesion may obscure the clinical features of the coronary arterial abnormality, and the appearance of normal coronary arterial distribution with left ventricular or aortic root angiography may create a false impression of a normal origin of the coronary arterial supply. A high index of suspicion may be the only lead to the detection of an anomalous origin of the left coronary artery from the pulmonary artery in association with an intracardiac lesion. In addition to the intracardiac lesion known to be associated with coronary arterial abnormalities, the question of anomalous coronary supply should be raised by the finding of poor left ventricular function or mitral insufficiency after operation (unexplainable by other causes), by the development of poor myocardial function during cardiac catheterization or anesthesia, or by the significant enlargement of a coronary artery seen during left ventriculography or supravalvular aortography.     

"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.     

Anomalous origin of the left coronary artery from the pulmonary artery: new electrocardiographic, echocardiographic and surgical observations

The case of a two year old girl with anomalous origin of the left coronary artery from the pulmonary artery is described. She was never in heart failure but had cardiomegaly and anginal pain. The ECG showed a typical infarct pattern with left ventricular hypertrophy. An unusual finding was a prolonged QTc of 0.52. During cardiac catheterization and twice 24 hours later she developed ventricular fibrillation treated with electroshock and prevented later with propranolol. The QTc returned to normal after surgery. Echocardiography showed diastolic flutter and early systolic closure of the pulmonary valve. This disappeared after surgical correction. Transverse 2D echo of the aortic root showed a large right coronary artery which decreased in size after surgery. The left coronary artery was not seen on echocardiography. At cardiac catheterization the diagnosis of an anomalous origin of the left coronary artery from the pulmonary artery was established, with a large shunt to the pulmonary artery through the anomalous artery. Mild pulmonary hypertension and mild mitral regurgitation were present. At surgery, since direct implantation was technically impossible, the left coronary artery was successfully connected to the aorta via a 6 mm expanded Poly-Tetra-Fluoro-Ethylene (P.T.F.E.) graft.     

冠状动脉异常起源于肺动脉的外科治疗

目的总结冠状动脉异常起源于肺动脉的外科治疗经验及疗效随访分析。方法从1999年4月至2005年8月共对12例冠状动脉异常起源于肺动脉的患者进行了外科治疗,其中左冠状动脉起源异常8例,右冠状动脉起源异常4例。异常起源的冠状动脉直接吻合到主动脉9例,应用Takeuchi技术,即肺动脉内隧道重建冠状动脉3例。有7例左冠状动脉起源异常的患者,因中、重度二尖瓣关闭不全同期行二尖瓣成形;1例合并房间隔缺损的右冠状动脉起源异常的患者,同期在非体外循环下行房间隔缺损封堵术。结果无死亡及术后并发症,随访7~83个月,平均(32±24)个月,左冠状动脉起源异常的患者,左室舒张末径从术前的(45±7)mm减小至术后的(39±6)mm(P=0.011),左室短轴缩短率从术前的0.21±0.09增加至术后的0.35±0.06(P=0.006),7例二尖瓣成形的患者术后二尖瓣反流程度均减轻;所有患者活动能力正常,均无症状。结论外科治疗冠状动脉异常起源于肺动脉,需恢复2个冠状动脉系统供血;左冠状动脉起源异常的患者,术后左室功能明显改善,对中、重度二尖瓣关闭不全的患者,需同期行二尖瓣成形;外科治疗早、中期效果良好,远期结果需进一步随访。     

New technique in the transfer of an anomalously originated left coronary artery to the aorta.

A 21-month-old girl with an anomalous origin of the left coronary artery underwent a transfer of the left coronary artery to the aorta using a new technique of coronary prolongation. Because the anomalous left coronary artery arising from the left anterior aspect of the pulmonary trunk was too short to reach the aorta, a simple transfer of the left coronary artery to the aorta was deemed impossible. Therefore, a transfer was performed with the help of a coronary prolongation technique using the cuff of the pulmonary trunk and an aortic flap. The new route of the left coronary artery was established anterior to the pulmonary trunk. Postoperative angiography showed a patent left coronary artery without any narrowing or kinking, as well as an improved contractility of the left ventricle. Postoperative cardiac scintigraphy showed a decreased ischemic area. This technique is thus considered applicable when the orifice of the anomalous left coronary artery is too distant from the aorta for a direct anastomosis.     

Anomalous origin of the left coronary artery from the pulmonary artery A case using the autologous pulmonary arterial wall graft.

A 10-year-old girl with anomalous origin of the left coronary artery from the pulmonary artery underwent a two coronary system reconstruction with a new technique. Her left coronary artery was stenotic next to its origin and had a severe adhesion with the aorta. We made a bypass grafting between the aorta and the left coronary artery with a roll shaped transected main pulmonary arterial wall graft. The postoperative angiogram showed a good patency of a reconstructed left coronary artery. This technique is considered useful surgical option for bypass grafting to the left coronary artery when the left coronary artery has an obstructive lesion or a difficulty to dissect and mobilize.     

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.     

Successful mitral valve replacement and myocardial revascularization in an adult with anomalous origin of the left coronary artery from the pulmonary artery

A 35-year-old white woman with anomalous origin of the left coronary artery from the pulmonary artery had infective endocarditis and severe mitral regurgitation. She survived mitral valve replacement and occlusion of the anomalous left main stem, but subsequently deterioration of her condition necessitated urgent saphenous vein bypass grafts to the left anterior descending and circumflex coronary arteries. Pulmonary hypertension with raised right ventricular end-diastolic pressure probably compromised collateral flow from the right to the left coronary systems.     

Anomalous origin of the left coronary artery from the pulmonary artery. Surgical alternatives depending of the age of the patient

Four cases of anomalous origin of the left coronary artery from the pulmonary artery, three of which were operated upon in our Centre, are presented. An aortocoronary bypass with reversed autologous saphenous vein was carried out in both of the children; and in the adult, the anomalous ostium was closed from the interior of the pulmonary artery. After 84, 72, and 4 months of evolution, respectively, they were found to be asymptomatic, the mitral insufficiency had disappeared and the venous graft remained patent. In this paper, the clinical form of presentation, diagnostic possibilities and surgical treatment are analysed.     

Echocardiographic assessment of anomalous origin of the left coronary artery from the pulmonary artery

Transthoracic echocardiography of a 4-month-old boy with heart failure revealed poor left ventricular contractility with severe mitral regurgitation. Deformation analysis revealed significantly decreased circumferential strain in the anterior and anteroseptal segments. Furthermore, post-systolic shortening was evident in the anterior and lateral segments. The pulmonary arterial short-axis view showed that the left coronary artery originated from the pulmonary artery and color Doppler flow mapping showed retrograde flow in the left coronary artery. These findings were quite helpful for concluding the anomalous origin of the left coronary artery from the pulmonary artery.     

Surgical treatment for anomalous origin of left coronary artery from pulmonary artery.

Anomalous origin of the left coronary artery from the pulmonary artery carries a poor prognosis; most patients die early in life from myocardial infarction and congestive heart failure. During a 12-year period at the Texas Heart Institute, 13 patients with this congenital malformation ranging in age from 1 to 24 years underwent operation. The anomalous coronary artery was sutured or ligated, or both, close to the pulmonary artery in 3 patients, 2 before 1965. Aortocoronary bypass was performed in the remaining 10 patients, using an autogenous saphenous vein graft in 9 and a Dacron tube in 1. More recently a distal end-to-side anastomosis has been employed and is not the preferred method. If necessary, a pump oxygenator may be used to complete the procedure. The only death was that of the first patient in the series who underwent ligation of the left coronary artery and mitral annuloplasty for severe mitral insufficiency; Of the 12 patients available for long-term follow-up study, all but 1 were asymptomatic. Follow-up angiographic studies showed the graft to be patent in 7 patients; the longest period of graft patency was 8 years. Definitive operative therapy is preferable to simple ligation because it eliminates the left-to-right shunt from the right coronary artery to the pulmonary artery and establishes a double coronary artery system.     

Left coronary artery originating from the pulmonary artery correction and total myocardial blood flow measurements

In a boy aged 12 years correction of an anomalous coronary artery system was performed by suture of the left coronary artery origin at the pulmonary artery trunk and a vein by-pass to the proximal LAD. Peroperative blood flow gives values for myocardial perfusion before and after by-pass with relation to patency of the vein graft. Before correction pure coronary blood flow was 540 ml/min, shunt flow 540 ml/min; in total 1,080 ml/min through the right coronary artery. After correction the vein by-pass conducted 310 ml/min and the right coronary artery 180 ml/min. At follow-up 2 months later coronary angiography showed a normal right coronary artery without collateral connections to the left coronary artery system which was well perfused from the by-pass. Left heart hypertrophy was reduced from 568 ml/m2. A weak systolic murmur at the apex could be a slight unimportant mitral insufficiency.     

A long-term survivor of Bland-White-Garland syndrome with systemic collateral supply: A case report and review of the literature

Background  

land-White-Garland syndrome (anomalous origin of the left coronary artery from the pulmonary artery) is a rare disease which may result in myocardial infarction, congestive heart failure and sometimes death during the early infantile period. Case presentation: A succesfully treated case of a 45-year-old mother of 2 children with Bland-White-Garland syndrome and concomitant severe mitral regurgitation is presented. Subsequent therapy consisted of ligation of the anomalous origin of the left coronary artery, anastomosis of the left internal mammary artery to the left anterior descending branch and mitral valve replacement. Continuous blood flow from the left coronary artery ostium during extracorporeal circulation and aorta clamping suggested systemic collateral supply. Conclusions: Recognition and diagnosis of Bland-White-Garland syndrome is important due to its potentially life-threatening complications.     

Anomalous left coronary artery from pulmonary artery: autogenous arterial tube for aortic implantation

Many surgical procedures for the implantation of anomalous left coronary artery from pulmonary artery have been described. A dual coronary system offers most benefit and is most advantageous for the patient. Two autogenous flaps of the pulmonary trunk and ascending aorta, without mobilizing the coronary artery, were created for use in this procedure. This technique was successfully used in an 8 month-old patient with severe myocardial dysfunction and moderate mitral regurgitation. This procedure allows 2 coronary systems to be repaired for any anatomic change of the left coronary artery without the use of prosthetic material. There were no technical complications. During the postoperative course minimal inotropic support was used. The magnetic resonance study, 11 months postoperative, showed wood flow of the left coronary artery and mild mitral regurgitation. The operative technique was simple and its execution easy. The follow-up in the intermediate and late period showed the growth of this endothelized tube.     

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.     

Anomalous origin of left coronary artery from the right pulmonary artery in association with type III aortopulmonary window and interrupted aortic arch

Anomalous origin of the left coronary artery from the pulmonary artery, also known as Garland-Bland-White syndrome, usually occurs as an isolated condition. We report an infant with caudal regression sequence diagnosed with interrupted aortic arch type B and type III aortopulmonary window, who was found to have anomalous origin of the left coronary artery from the right pulmonary artery at surgical repair. Successful repair of the aortopulmonary window and interruption was performed with reimplantation of the left coronary artery into the ascending aorta. This report highlights the importance of closely assessing the coronary ostia in patients undergoing complex aortopulmonary window repair.