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.     

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.     

Repair of anomalous left main coronary artery arising from the pulmonary artery in infants: long-term impact on the mitral valve

Background. Infants presenting with anomalous left coronary artery off the pulmonary artery (ALCAPA) are generally in heart failure and often have significant mitral valve regurgitation (MR). Although establishing a dual coronary circulation is the procedure of choice, there remains controversy as to how the mitral valve is handled.

Methods. We reviewed our experience with this lesion at St. Louis Children’s Hospital. Over the past 15 years, 17 infants under 18 months of age have undergone repair, with all but one being treated with reimplantation of the left coronary artery into the aorta; the other underwent the Takeuchi procedure (intrapulmonary artery baffle) and was excluded from this evaluation. The average age and weight at operation were 0.5 ± 0.3 years and 6.1 ± 1.9 kg, respectively. All presented with varying degrees of heart failure and 9 patients also had either moderate or severe MR.

Results. There was one early and no late deaths after reimplantation of the left coronary artery. The left ventricular function postrepair improved from a preoperative shortening fraction of 0.19 ± 0.09 to 0.34 ± 0.08 (p < 0.01). Moderate or severe MR was present in 2 patients postoperatively, and both developed significant obstruction in the left coronary artery postoperatively as well. Both underwent mitral valve repair and revascularization of the left coronary artery.

Conclusions. Excellent results can be obtained in the treatment of this very high-risk group of patients. Mitral valve repair is not generally necessary at the time of the initial operation. However, should MR recur or persist late, it may herald the presence of a coexistent, significant coronary stenosis. Cardiac catheterization should be performed to assess the patency of the left coronary artery before performing mitral valve surgery.     

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.     

Anomalous coronary artery origin from the pulmonary artery: correlation between surgical timing and left ventricular function recovery

BACKGROUND: This study investigates the correlation between surgical timing and 15-year longitudinal left ventricular and mitral valve function, after repair of anomalous coronary artery origin from the pulmonary artery. METHODS: Between 1987 and 2002, 31 patients (median age, 7.1 months) underwent repair for anomalous origin of the left (n = 28), right (n = 2), or both (n = 1) coronary arteries from the pulmonary artery. Repair was accomplished by subclavian interposition in 5 patients, intrapulmonary tunnel in 12, and direct aortic reimplantation in 14. Primary mitral valve repair was never associated with coronary revascularization. Total follow-up was 186.4 patient-years (mean, 77.2 months). RESULTS: Fifteen-year actuarial survival was 92.9% +/- 4.9% for coronary transfer, 40.0% +/- 21.9% for subclavian interposition, and 89.9% +/- 7.5% for intrapulmonary tunnel (p = 0.019). Five patients required further intervention for supravalvular pulmonary stenosis (n = 3), baffle leak (n = 1), and mitral valve replacement (n = 1). Coronary transfer allowed best freedom from long-term reoperation (92.3% +/- 7.4%). Left ventricular shortening fraction increased from 17.3% +/- 6.3% before operation to 34.1% +/- 4.6% at last follow-up (p < 0.01). Regression analysis demonstrated a linear relationship between age at repair and shortening fraction recovery (r(2) = 0.573, p < 0.01). Patients younger than 6 months of age showed worse preoperative shortening fraction (15.9% +/- 5.2%) and best longitudinal shortening fraction recovery (36.4% +/- 5.1%; p < 0.001). Major improvement in mitral valve function was observed within 1 year from surgery in 90.4% of survivors. CONCLUSIONS: Repair of anomalous coronary artery origin from the pulmonary artery in younger symptomatic infants offers the best potential for recovery of left ventricular function, despite a worse initial presentation. Coronary transfer is associated with superior long-term survival and freedom from reoperation. Most patients with patent two-coronary repair will recover normal mitral valve function; therefore, simultaneous mitral valve surgery seems unwarranted.     

Origin of the left coronary artery from the right pulmonary artery: a report of successful surgery in a 3-month-old child

Successful reimplantation into the aorta of an anomalous left coronary artery arising from the right pulmonary artery is described in a 3-month-old child. Preoperative and postoperative angiograms are included, and serial echocardiography demonstrates the marked improvement in left ventricular function.     

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.     

Anomalous right coronary artery originating from the left main coronary artery

A 54-year-old man with no cardiac history presented with exertional angina. Cardiac catheterization revealed an anomalous right coronary artery originating from the left main coronary artery with external compression during its course between the aorta and the pulmonary artery. He was successfully managed with surgical reimplantation of the right coronary artery into the aorta.     

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.     

"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: repair by aortic reimplantation

From 1980 to 1990, 12 patients (mean age 2.5 years, range 5 months to 9 years) with anomalous origin of the left coronary artery from the pulmonary artery were treated surgically. Five infants were operated upon in the first year of life because of persistent symptoms of congestive heart failure. In all cases, a two-coronary system was constructed by direct aortic reimplantation of the anomalous vessel with no deaths early or late over a follow-up period of up to 10 years. The technique of reimplantation was facilitated by transection of the main pulmonary artery. One patient with severe mitral regurgitation underwent, in addition, a mitral annuloplasty. A pulmonary valvotomy was performed in another patient with associated pulmonary stenosis. Three patients are receiving medication. The reimplanted anomalous left coronary artery was patent in each reevaluated patient (10/12). Left ventricular function improved considerably in all cases. Patients with symptoms should undergo repair soon after diagnosis. Direct aortic reimplantation should be technically feasible in even the smallest infant. Operative mortality is related to preoperative conditions and severity of ischemic damage of the myocardium.     

Surgical correction of tetralogy of Fallot with anomalous origin of left pulmonary artery from the ascending aorta

A surgically corrected case of tetralogy of Fallot with anomalous origin of left pulmonary artery from the ascending aorta is presented. The patient was under-developed 3-year-old girl. She weighed only 1,000 grams at birth and had a systolic heart murmur and slight cyanosis. Echocardiography demonstrated the features of tetralogy of Fallot shortly after birth. Chest roentgenogram showed right ventricular enlargement, but vascular marking of lung was almost normal. Catheterization and cineangiography demonstrated the findings of tetralogy of Fallot. Final diagnosis of anomalous origin of left pulmonary artery from ascending aorta was confirmed by aortography. Surgical correction was accomplished using cardiopulmonary bypass with surface cooling. The left pulmonary artery was detached from the aorta and anastomosed to the main pulmonary artery. Pulmonary valve stenosis and severe infundibular hypertrophy were corrected, and ventricular septal defect was closed with a patch. Postoperative recovery was uneventful. On follow up after surgery, she appears to be in very good condition.     

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.     

左冠状动脉异常起源于肺动脉的外科治疗

１例患左冠状动脉异常起源于动脉的７岁女孩，行左冠状动脉的重建术获得成功。因异常起源的左冠状动脉源于肺脉的主干左后侧，右冠状主干较，不能直接植入升主动脉；病儿年龄小，大隐静脉和其他自体血管难以利用，故采用人工血管左冠状动脉旁路架桥术。术后病儿恢复顺利，研室功能正常，心血管造景证实人工血管桥和左冠状动脉 通畅、无狭窄或扭曲。作者认为，在左冠状动脉异常起源于肺动脉的病人，当左冠状动脉不能直接植入升主动脉     

Use of the Batista procedure to discontinue bypass following aortic reimplantation of an anomalous left coronary artery

A four-month-old infant with anomalous origin of the left coronary artery from the pulmonary artery developed severe left ventricular failure after aortic reimplantation, despite prolonged supportive cardiopulmonary bypass with high level inotropic support and several abortive attempts for discontinuation. The Batista procedure was performed and the patient was successfully weaned from bypass. Postoperative recovery was uneventful, and at one-year follow-up she remains asymptomatic.     

Unrecognized anomalous origin of the left coronary artery from the pulmonary artery as a cause of ventricular fibrillation after patent ductus arteriosus ligation in an infant

We present a case of an infant who developed ventricular fibrillation after patent ductus arteriosus (PDA) ligation. The infant had unrecognized anomalous origin of the left coronary artery from the pulmonary artery before PDA ligation. Acute reduction in systemic pulmonary artery pressures after PDA ligation resulted in an abrupt reduction in left main coronary artery blood flow. After prompt resuscitation, cardiac catheterization confirmed the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery. The infant subsequently underwent coronary artery translocation and recovered uneventfully.     

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.     

Four cases of the anomalous origin of the left coronary artery from the pulmonary artery

We report perioperative management of 4 patients with anomalous origin of the left coronary artery from the pulmonary artery. This report involves with 3 infant cases and an adult. Two infants underwent coronary reimplantation procedure and Takeuchi's method was performed on the other infant. In all infant cases, mitral valve plasty was performed to correct mitral regurgitation secondary to papillary muscle dysfunction. The adult patient underwent CABG with ligation of LCA. General anesthesia was performed with high doses of fentanyl in all cases. We employed a relatively high PaCO2 and low FIO2 in order to maintain a high pulmonary vascular resistance. It aims to decrease the incidence of left to right shunt. We used epinephrine to wean one infant and the adult from cardiopulmonary bypass. Perioperative course was uneventful with the use of catecholamines and high doses of vasodilators for left ventricular dysfunction and coronary perfusion under mechanical ventilation.     

Anomalous left coronary artery from the pulmonary artery in infants. Which operation? When?

The surgical management of anomalous left coronary artery from the pulmonary artery in infants and small children remains controversial, because the ideal surgical procedure and the optimal time for operation are yet to be determined. From 1977 to 1985, 22 patients less than 4 years of age (mean age 18.2 months) underwent direct aortic reimplantation of the anomalous left coronary artery. There were five operative deaths (23%, confidence limits 13%-36%). The determinant risk factor of early mortality was the severity of preoperative left ventricular dysfunction (p = 0.05), not age at operation (p = 0.64) or preoperative clinical status (p = 0.36). There were not late deaths (mean follow-up 38 months). All survivors but one were symptom free. The reimplanted anomalous left coronary artery was patent in each reevaluated case (9/17). Left ventricular function improved significantly in all survivors. Moderate to severe preoperative mitral incompetence lessened in all patients but one, without mitral valve repair. When technically feasible, direct aortic reimplantation of the anomalous left coronary artery is an attractive procedure because it offers a high rate of patency and avoids the potential drawbacks of procedures involving autogenous venous or arterial tissue. Optimal intraoperative myocardial preservation and institution of temporary left ventricular assistance at the end of the operation may decrease the operative risk. Left ventricular function nearly always recovers after successful revascularization, and resection of left ventricular myocardium is rarely indicated, if ever. Mitral incompetence almost always lessens, and the mitral valve should not be repaired at initial operation; however, residual mitral incompetence may necessitate reoperation in a few cases. In infants with moderate left ventricular damage (usually asymptomatic with medical therapy), surgical treatment should be delayed until 18 to 24 months of age so that it can be performed with a low operative risk. Infants with severely impaired left ventricular function and persistent congestive heart failure should probably undergo operation as soon as the diagnosis has been made.     

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。