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

Abnormal origin of the coronary artery in children with hypoplastic left heart syndrome is an extremely rare defect. We describe a newborn with concomitant hypoplastic left heart syndrome and abnormal origin of the left coronary artery arising from the right pulmonary artery. A Norwood procedure and direct reimplantation of the left coronary artery to the ascending aorta was performed.     

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.     

Coronary artery bypass graft for Bland-White-Garland syndrome in an old age; report of a case

We report a case of Bland-White-Garland syndrome with advanced age. The patient, a 67-year-old women, presented with a history of congestive heart failure. Coronary catheterization revealed an anomalous origin of the left coronary artery (LCA) from the trunk of the pulmonary artery and huge right coronary aneurysm. Myocardial single photon emission computed tomography (SPECT) showed previous myocardial infarction with reversible ischemia in left anterior descending (LAD) region. We performed LCA direct closure and coronary artery bypass graft. The patient recovered uneventfully without signs of ischemia. Although a bypass graft was patent, left ventricular function had not been improved immediately probably due to the coronary flow pattern changes.     

Infant orthotopic cardiac transplantation

Infant orthotopic cardiac transplantation has been recently applied to various forms of congenital heart disease with encouraging short-term results. Between June 1986 and September 1987 we evaluated 16 infants for orthotopic cardiac transplantation. Fourteen had hypoplastic left heart syndrome, one had endocardial fibroelastosis with aortic atresia, and one had anomalous pulmonary arterial origin of the left main coronary. Eight families accepted the treatment program and eight families refused (two because of associated anomalies and six on philosophical grounds). Of the eight patients who were candidates for orthotopic cardiac transplantation, one died 6 hours after diagnosis, one was allowed to die after 60 days because of acquired neurologic complications, and another had congenital cytomegalic virus infection. The remaining five patients (four with hypoplastic left heart syndrome, one with anomalous pulmonary arterial origin of the left main coronary) had orthotopic cardiac transplantation. The operation was performed with absorbable polydioxanone suture with deep hypothermia and circulatory arrest in four neonates for hypoplastic left heart syndrome (average time 47 minutes) and bicaval cannulation and continuous bypass in one 11-month-old infant for anomalous origin of the left main coronary. In-house retrieval was used in all. One neonate died of complications as a result of pretransplant donor heart dysfunction and size discrepancy, whereas the remaining three neonates and one infant survived and are home 23 months, 12 months, and 8 months (the patients with hypoplastic left heart syndrome) and 17 months (the patient with anomalous origin of the left main coronary) postoperatively. Triple-drug immunosuppression included cyclosporine, azathioprine, and prednisone. Rejection was diagnosed by clinical evaluation of child activity and monocyte cell cycle analysis from peripheral blood samples without myocardial biopsies. Routine echocardiograms, electrocardiograms, and chest x-ray films were not helpful. Six episodes of rejection were successfully treated in four patients. Twelve-month postoperative catherization in one patient (hypoplastic left heart syndrome) showed appropriate graft growth, no aortic or pulmonary anastomotic strictures, normal right and left ventricular function, and no coronary artery disease. We conclude that infant orthotopic cardiac transplantation is an acceptable procedure for severe forms of untreatable congenital heart disease. The excellent short-term results warrant continued application of orthotopic cardiac transplantation.     

Coronary artery bypass grafting with bilateral internal thoracic arteries for Bland-White-Garland syndrome in an adult

A 46-year-old man with anomalous origin of the left coronary artery from the pulmonary artery (Bland-White-Garland syndrome) is reported. We successfully performed coronary artery bypass grafting with the use of bilateral internal thoracic arteries and ligation of the anomalous left coronary artery. The patient was discharged from the hospital after an uneventful postoperative course and postoperative coronary angiography, which revealed patent internal thoracic arteries and no leakage of blood flow from the anomalous left coronary artery to the pulmonary artery. This surgical procedure is technically simple and useful for adult patients with Bland-White-Garland syndrome. To our knowledge, this is one of only a few reports on coronary artery bypass grafting with bilateral internal thoracic arteries as a treatment of Bland-White-Garland syndrome.     

White-Bland-Garland syndrome: the maximum natural development of the coronary anastomosis(II)

The anomalous origin of the left coronary artery from pulmonary artery (White-Bland-Garland syndrome) is a very rare coronary malformation, having despite the great mortality in the early childhood, an adult form, characterized by minor symptoms and long course. This paradoxical situation is totally dependent to the complete development of the coronary anastomosis, allowing the right coronary flow to perfuse the left myocardium. The theory of the "terminal arterial perfusion" of the heart, still persistent in some monographs is meeting in this natural situation its strongest opponent argumentation. The coronary anastomosis, clearly visible by coronarography are large enough (in late stages) to inverse the flow in left coronary artery, filling the pulmonary artery. Is the coronary-pulmonary shunt, responsible for the majority of symptoms, in the 5th or 6th decades. The adult form of the White-Bland-Garland syndrome is indicative for the maximum result we may hope to reach using the new revascularisation method for ischemic heart disease: new vessels formation and enhancing the coronary anastomosis by growth factors administration therapy.     

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.     

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 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.     

Anomalous left anterior descending coronary artery from pulmonary artery with ventricular septal defect

Anomalous origin of the left anterior descending coronary artery with associated congenital defects is very rare. An angiogram of a 47-year-old woman admitted for a ventricular septal defect closure revealed an anomalous left anterior descending coronary artery arising from the left posterior sinus of the pulmonary artery. During the surgical procedure, the origin of the left anterior descending coronary artery was closed with pledgetted polypropylene sutures through the pulmonary artery. The ventricular septal defect was closed with a patch through the right atrium, and the left anterior descending coronary artery was bypassed with the left internal mammary artery.     

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.     

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.     

Bland-White-Garland syndrome. Surgical treatment using a minimal-invasive approach

We report the surgical treatment of a Bland-White-Garland syndrome (BWG-syndrome) of adult type in a 61-year old female patient. Coronary catheterization revealed an anomalous origin of the left coronary artery from the trunk of the pulmonary artery. Based on excellent collateral perfusion of the artery from the right coronary artery, ligation near its origin from the pulmonary artery was attempted via a minimally-invasive approach. No saphenous vein bypass was implanted, no reimplantation of the anomalous vessel in the aorta was performed. The patient recovered uneventfully without signs of ischemia. Appearance of BWG-syndrome in adults is very rare, especially without symptoms of myocardial ischemia. The different modalities of the treatment of these syndromes in adult patients are often debated. In this case, closure without revascularisation appeared to be appropriate.     

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.     

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.     

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.     

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.     

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.     

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。     

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.