Analysis of the risk factors for extracorporeal membrane oxygenation use after surgical repair in patients with anomalous origin of the left coronary artery from the pulmonary artery combined with severe left ventricular dysfunction / 中国胸心血管外科临床杂志

@#Objective    To analyze the early outcomes of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) patients with severe left ventricular dysfunction after surgical repair, and to explore the predictors for extracorporeal membrane oxygenation (ECMO) support for these patients. Methods    The clinical data of ALCAPA patients with severe left ventricular dysfunction (left ventricular ejection fraction<40%) who underwent coronary artery reimplantation in the pediatric center of our hospital from 2013 to 2020 were retrospectively analyzed. The patients were divided into an ECMO group and a non-ECMO group. Clinical data of the two groups were compared and analyzed. Results    A total of 64 ALCAPA patients were included. There were 7 patients in the ECMO group, including 4 males and 3 females aged 6.58±1.84 months. There were 57 pateints in the non-ECMO group, including 30 males and 27 females aged 4.34±2.56 months. The mortality of the patients was 6.25% (4/64), including 2 patients in the ECMO group, and 2 in the non-ECMO group. The postoperative complications rate was significantly higher in the ECMO group than that in the non-ECMO group (P=0.041). There were statistical differences in the cardiopulmonary bypass time [254 (153, 417) min vs. 106 (51, 192) min, P=0.013], aortic cross-clamping (ACC) time (89.57±13.66 min vs. 61.58± 19.57 min, P=0.039), and preoperative left ventricular end-diastolic diameter/body surface area (132.32±14.71 mm/m2 vs. 108.00±29.64 mm/m2, P=0.040) between the two groups. Multivariate logistic regression analysis showed that ACC time was an independent risk factor for postoperative ECMO support (P=0.005). Receiver operating characteristic (ROC) curve analysis showed that the area under the ROC curve was 0.757, the sensitivity was 85.70%, specificity was 66.70%, with the cut-off value of 66 min. Conclusion    ACC time is an independent risk factor for postoperative ECMO support. Patients with an ACC time>66 min have a significantly higher risk for ECMO support after the surgery.

An Infant Case of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Successfully Treated with Modified Spiral Cuff Technique / 日本心臓血管外科学会雑誌

The patient was a six-month-old girl with an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). She had fever and visited a family physician at 5 months of age. Because of poor oxygenation, she was referred to our pediatric department and intubated soon after hospitalization. Echocardiography showed that the orifice of left coronary artery was just above pulmonary commissure, the left ventricular ejection fraction was 9%, and the level of mitral regurgitation was moderate. Right coronary angiography showed that the left coronary artery contrasted against the collateral arteries. The left coronary artery originated from the left side of the pulmonary trunk. After recovery of the general condition with medical therapy, the patient underwent coronary artery reconstruction by the modified spiral cuff technique on the 21st day of hospitalization. The temporary detachment of pulmonary valve and its commissure for making a margin around the left coronary artery enabled us to make the spiral cuff in almost the usual manner. She was moved to the intensive care unit with the support of extracorporeal membrane oxygenation (VA-ECMO) and was successfully weaned off the VA-ECMO 5 days after the surgery. The postoperative course was good, and she was discharged from our hospital 3 months after the surgery. The echocardiogram one year after the surgery showed a left ventricular ejection fraction of 30%, mild mitral regurgitation, and mild pulmonary regurgitation. Our experience indicates that the spiral cuff technique is a useful coronary reconstruction method for the treatment of ALCAPA, especially in cases presenting a considerable distance between the origin of the left coronary artery and the transplantation site. There are few reports regarding the surgical treatment of infantile ALCAPA showing reduced left ventricular function. Coronary artery reconstruction using the spiral cuff method and planned VA-ECMO are useful surgical procedures in such cases. Our experience also suggests that the establishment of a treatment strategy including mechanical support is essential to improve the results in severe ALCAPA cases.

Clinical characteristics of anomalous origin of the left coronary artery from the pulmonary artery in 91 children / 中华儿科杂志

Objective@#To explore the clinical manifestations, diagnosis, treatment and prognosis of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) .@*Methods@#A retrospective study identified 91 patients diagnosed with ALCAPA at Shanghai Children′s Medical Center from March 2010 to August 2017. According to the left ventricular ejection fraction (LVEF) at the time of consultation, patients were divided into the cardiac insufficiency group (n=54) and the normal cardiac function group (n=37). Clinical features (age of onset, clinical performance, etc) and auxiliary examinations (electrocardiogram, echocardiography, etc) between the two groups were compared using a t-test and a Chi-square test. Prognostic factors were analyzed by an ordered logistic regression and a Pearson correlation coefficient.@*Results@#(1) The age of diagnosis of patients in the cardiac insufficiency group who were usually misdiagnosed as cardiomyopathy was (10.0±2.6) months (20/54) , whereas the age of diagnosis of patients in the normal cardiac function group who were usually misdiagnosed as valvular diseases was (40.0±7.8) months (4/37). According to the pathophysiological mechanism, forty of the 54 (74%) patients in the cardiac insufficiency group were infantile type, and 78% patients (29/37) in the normal cardiac function group were adult type. (2) Preoperative electrocardiogram showed the deep Q wave in lead I occurred more frequently in the cardiac insufficiency group than in the normal cardiac function group (28/54 vs. 11/37, χ2=4.388, P=0.036). (3) Twenty patients died in the cardiac insufficiency group including 12 patients who died from postoperative cardiac pump failure and 8 children who did not undergo surgery due to poor prognosis and died from other reasons. There was no death in the normal cardiac function group. (4) Preoperative LVEF was the unique risk factor affecting prognosis (F=16.872, P=0.005). The preoperative LVEF was significantly lower than the postoperative LVEF ((37±11)% vs. (45±14)%, t=3.614, P=0.001) in the cardiac insufficiency group. During the follow-up period, 6 patients in the cardiac insufficiency group still presented with postoperative cardiac dysfunction, and the patients in the normal cardiac function group still had normal cardiac function.@*Conclusions@#Preoperative LVEF was the unique risk factor affecting prognosis of ALCAPA. Patients with infantile type ALCAPA and preoperative cardiac insufficiency should receive long-term follow-up treatment.

Clinical study on surgical repair of severe ALCAPA in infants and children / 中华胸心血管外科杂志

Objective To discuss the surgical treatment of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA) and the risk factors of postoperative recovery in infants and children.Methods A retrospective review of all patients who underwent surgical treatment of ALCAPA in Shanghai Children Medical Center(2003.1-2018.1) was conducted.Patients were divided into early surgery group (2003.1-2012.12) and late surgery group (2013.1-2018.1) according to the operation time,a preoperative left ventricular ejection fraction(LVEF) ＜0.35 was defined as severe group and LVEF≥0.35 as the control group in both surgery group.Meantime,and the clinical data among the groups were analyzed and compared.Results 102 patients were included in our study.According to the operation time and preoperative LVEF grouping:10 cases in the early severe group,early death in 4 cases(40％);28 cases in the early control group,and 3 cases died(10.7％)in hospital.Preoperative LVEF(0.29 ± 0.06 vs.0.53 ± 0.12),surgical age [(8.0 ± 7.9) months vs.(23.3 ± 27.7)months],and cardiopulmonary bypass time [(131.1 ± 39.6) min vs.(103.8 ± 29.8) min] were statistically different between the early two groups.The results of the late surgery had been improved:24 cases in the late severe group,4 cases died in hospital(16.7％);40 cases in the late control group,and early death in 2 cases (5％).In the late surgery groups,there was a statistically significant difference in preoperative LVEF(0.28 ±0.05 vs.0.59 ±0.12),left ventricular end-diastolic diameter(LVDD) Z-score(3.09 ±1.16 vs.2.11 ±0.95),and surgical age [(5.3 ±3.0) months vs.(24.8 ±30.5)months],clamping time [(67.1 ± 15.5) min vs.(82.7 ± 28.4) min].In the severe group,there was no significant difference in preoperative clinical data between early and late patients,and the early mortality decreased from 40％ in the early period to 16.7％ in the late period.In this study,13 cases(38.2％) of children with severe ALCAPA underwent mechanical circulation support(MCS).One patient died during MCS support and 2 died after weaning.Conclusion The early mortality severe ALCAPA remains high,which may be related to severe cardiac ischemia,left ventricular enlargement and age at surgical time.The modify of surgery technology and the use of MCS in the early clinical stage can improve the early survival rate.

Anomalous Origin of the Left Circumflex Coronary Artery from the Pulmonary Artery with Atrial Septal Defect / 日本心臓血管外科学会雑誌

<p>Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. This anomaly occurs in approximately one in 300,000 live births. Of the children diagnosed with this syndrome, 90% die within the first year of life because of myocardial ischemia and left ventricular failure. Survival into adulthood is rare and depends upon pre-existing or rapidly developing collateral vessels between the right and left coronary artery. This report concerns the surgical case of anomalous origin of the circumflex coronary artery from the left pulmonary artery complicated with atrial septal defect (ASD). A 34-year-old woman was admitted because of dyspnea on exertion. Echocardiography revealed normal cardiac function with secondary ASD. Coronary catheterization revealed an anomalous origin of the left circumflex coronary artery from the left pulmonary artery. Direct closure of the ASD and coronary artery bypass grafting for the circumflex coronary artery using an internal thoracic artery were performed, and the orifice of the circumflex coronary artery was ligated. The postoperative outcome was excellent. Most of the ALCAPA cases shows the main trunk of the left coronary artery arising from the pulmonary artery. This case demonstrates only the left circumflex coronary artery originating from the left pulmonary artery. Moreover ASD coexisted in this case. To the best of our knowledge, this is a very rare case of its type to be diagnosed and reported.</p>

Preoperative diagnosis and surgical treatment of anomalous origin of the left coronary artery from the pulmonary artery / 中国小儿急救医学

Objective To investigate the preoperative diagnosis,surgical treatment and results of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA).Methods We retrospectively analyzed the clinical data of 9 patients with ALCAPA who had undergone surgical treatment in Shanxi Children's Hospital from June 2010 to March 2014.Results There were 3 males and 6 females,aged from 4 months to 4 years old and 6 months,average age (1.14 ± 1.33)years,body weight from 5.6 to 18.0 kg,average weight(8.06 ± 3.99) kg.Echocardiography and spiral CT angiography were performed in all patients,one of which underwent cardiac catheter angiography.The cases of mitral regurgitation were mild in 2 cases,mild to moderate in 5 cases,and moderate to severe in 2 cases.Electrocardiogram showed abnormal Q wave,ST segment change and T wave inversion in lead Ⅰ,AVL and V4-6 in 9 cases.The X-ray sign was significant heart enlargement(C/T ratio 0.56-0.73,mean 0.61 ± 0.05).Echocardiography indicated ALCAPA,significant enlargement of left ventricle and decreased myocardial contractility(ejection fraction less than 50％).End diastolic diameter of left ventricle were more than 35 mm in 8 cases and aneurysm formation of left ventricular apex in 1 case.One case was confirmed ALCAPA by catheter angiography.Six cases had radioactive nuclide myocardial imaging,which showed different degree of myocardial infarction.All patients underwent reimplantation of left coronary artery into the aorta,mitral valvoplasty in 7 patients.One case died for low cardiac output syndrome.Others recovered and discharged.Postoperative complications included low cardiac output syndrome in 2 cases,pneumonia in 3 cases and supraventricular tachycardia in 1 case.During a followup of 3 months to 2 years,well development,good heart function,mitral valve regurgitation and ejection fraction improved in all case.Conclusion Echocardiography has important value in diagnosis of ALCAPA,which are confirmed by spiral CT angiography and catheter angiography.Preoperative nuclide examination for determinant of survival myocardium is helpful for surgery and prognosis.Reimplantation of left coronary artery into the aorta is the most ideal surgical procedure.

Preoperative evaluation of children with anomalous origin of left coronary artery from the pulmonary artery and the follow-up after surgery / 中华实用儿科临床杂志

Objective To retrospectively study 50 children with anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA) and find the simple and practical indexes that may predict myocardial viability and the possible risk factors that may affect the choice of operation and the recovery after surgery.Methods A retrospective study was performed in 50 children with ALCAPA [29 male,21 female,aged from 4 months to 18 years,average (4.49 ±4.30) years] between Apr.1999 and Mar.2013.The preoperative examination included electrocardiogram (ECG),echocardiography,chest X-ray,mutislice spiral computerized tomography (MSCT),angiocardiography et al.Follow-up data were obtained by reviewing the records made in out-patient and recent telephone call.According to myocardial 18F-FDG imaging,the extent of myocardial viability of 15 patients with ALCAPA was classified into 4 grades.0 normal myocardial; 1 viable myocardial ;2 partial viable myocardial;3 myocardial infarction.Meanwhile,the global clinical scores were calculated and divided into 10 levels according to the deviations of clinical manifestations which included abnormal Q waves,left ventricular ejection fraction (LVEF),left ventricular end-diastolic dimension (LVED),cardiothoracic ratio(CTR),intercoronary collaterals(ICC),mitral regurgitation(MR) and aneurysm.The relationship of the extent of myocardial viability and clinical features were studied.All patients with ALCAPA were classified into groups by the global clinical scores and the preoperative,perioperative and post operative clinical manifestations were compared among groups.Results The extent of myocardial viability was related well to the global clinical scores (r =0.936,P ＜0.001),LVEF(r =0.783,P ＜ 0.001),CTR (r =0.770,P ＜ 0.002),abnormal Q waves (r =0.667,P ＜ 0.01) and LVED (r =0.637,P ＜ 0.02),but was not related to age,MR and ICC (r =-0.206,-0.268,-0.342,all P ＞ 0.05).The results showed that grade 0-1 equaled scores 0-3,grade 2 equaled scores 3-5,grade 3 equaled scores ＞ 5.Chil-dren with less viable myocardium had more severe clinical symptoms.Among abnormal Q waves,LVEF ＜ 50％,CTR ＞0.65,ICC dysplasia,median to severe MR and the global clinical scores ＞ 3,the global clinical scores ＞ 3 and LVEF ＜50％ showed a good predict of myocardial infarction.The preoperative,perioperative and post operative clinical manifestations were compared between 2 groups divided by the global clinical scores (group A,scores ≤ 3 and group B,scores ＞3) in all patients.More infants and toddlers,abnormal Q waves,ICC dysplasia,RCA/AO ＜0.2,larger CTR and lower LVEF were found in group B than those in group A.The time of cross-clamp and CPB was not different between the 2 groups,but the postoperative ventilation time and postoperative ICU stay were longer in group B than in group A.Fortyseven patients underwent operation and there were no operative deaths.One infant with scores 9 died while waiting heart transplantation.Follow-ups from 1 to 168 months were conducted in 38 patients(80.9％,38/47 cases) and 1 patient with ligation of the left coronary artery (LCA) had a sudden death after 8 months of surgery.All patients had gotten smaller LVED after surgery.Nineteen patients had mild MR and 3 patients had mild supravalvular pulmonary stenosis in following-up.Of the 16 patients with LVEF ＜ 50％,14 had a recovery of LVEF,2 patients still had LVEF ＜ 50％.Conclusions In children with ALCAPA,the extent of myocardial viability evaluated by myocardial 18 F-FDG imaging is related closely to the preoperative clinical manifestations.The global clinical scores ＞ 3 and LVEF ＜ 50％ have a good predict of myocardial infarction.Even in young children with severely depressed left ventricular function,higher global clinical scores and more myocardial infarction,median and long-term follow-ups showed satisfactory recovery of cardiac function after successful restoration of a dual coronary arterial system.

Surgical Treatment of Bland-White-Garland Syndrome in an Adult Patient / 日本心臓血管外科学会雑誌

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly, known as Bland-White-Garland (BWG) 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 report the surgical treatment of a BWG syndrome in an adult patient. A 51-year-old man was admitted because of congestive heart failure and atrial fibrillation. Coronary catheterization revealed an anomalous origin of the left coronary artery from the pulmonary trunk and aneurysm of the right coronary artery. The patient was treated with a new technique using an internal tunnel created in the left side main pulmonary artery with a pericardial patch roll. A bypass with an artificial graft was made between this pericardial roll and the ascending aorta. Replacement of the aneurysm of the right coronary artery was also performed. The post operative course was uneventful. The postoperative CT showed the successful results of this operation.

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery : Report of an Adult Case

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), also termed Bland-White-Garland syndrome, is a rare congenital cardiac anomaly characterized by myocardial ischemia which becomes symptomatic during early infancy leading to infarction, left heart failure or even death. More than 90% of patients can be dead within first year of life if untreated, so adult type ALCAPA without surgical correction is quitely rare. We present a case of a 20-year-old man with ALPACA syndrome whose diagnosis took several years to be made because he was mistaken to have organic mitral regurgitation.

Diagnosis and Treatment of 6 Children with Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery / 实用儿科临床杂志

Objective To analyze diagnostic experience of children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) and assess its surgical results.Methods Six children with ALCAPA had been diagnosed in our hospital from Mar. 2006 to Oct.2007 aged from 6 months to 10 years old(median 43.5 months).The medical data included common clinical presentations,the findings of electrocardiogram(ECG),the echocardiogram,ECG-gated 64-slice computed tomography,the digital subtraction angiogram (DSA),surgical records and the outcome.Results Diaphoresis,fantod,breathlessness and difficult feeding were commonly presented in 5 cases ALCAPA and the 5 children presented with left ventricular failure in infancy.The predominant ECG findings were Q waves and ST segment changes in the anterolateral chest leads in 4 cases,ST changes in 1 case,and normal ECG in 1 case.Cross-sectional echocardiography showed dilated left ventricles with poor contractility in 4 cases,medium mitral regurgitation in 2 cases and severe mitral regurgitation in 2 cases,aneurysmal dilatation of the right coronary artery(RCA) in 2 cases.CT and DSA examinations showed characteristic changes in these children.The surgical reestablishment of a two-coronary system was performed in 5 cases,direct reimplantation in 2 cases and creation of an autologous extrapulmonary tunnel in 3 cases.Simultaneous mitral annuloplasty was performed in 2 cases.After operation,the symptoms of 5 cases were all improved and none died.Conclusions Understanding the clinical manifestations and lab findings of ALCAPA is helpful to making an early diagnosis of ALCAPA.Early surgical treatment can improve the patients′ outcome.