Anomalous origin of left pulmonary artery branch from the aorta with Fallot's tetralogy:one case report

We report the case of a ten-year-old boy who had often presented with respiratory distress since born. Chest X-ray showed an enlarged right ventricle. Echocardiography demonstrated characteristic features of Fallot＇s tetralogy and the left pulmonary artery could not be visualized. However, cardiac catheterization disclosed that the left pulmonary artery had an anomalous origin in the ascending aorta. The patient underwent total surgical correction of the defects. Nine days later, he was discharged. We present the results of a literature review of the incidence, physiopathological, clinical, diagnostic, and surgical characteristics of this rare disease.

Long-Term Outcomes of Systemic-to-Pulmonary Artery Shunt in Patients with Functional Single Ventricle and Heterotaxy Syndrome

Objectives: We sought to determine the long-term outcomes and mortality-associated factors after systemic-topulmonaryartery shunt (SPS) in patients with heterotaxy syndrome. Methods: We retrospectively analyzed allpatients with a functional single ventricle and heterotaxy syndrome who underwent SPS at our center from January2001 to April 2022. Results: This study involved 84 patients. Restrictive pulmonary blood flow requiringearly modulation was presented in 34 (40%) patients. Compared with patients without restrictive pulmonaryblood flow (N = 50 [60%]), the postnatal survival of these 34 patients was significantly lower at 10 years (log rank:p = 0.04), but the statistical significance disappeared at 20 years (log rank: p = 0.18). Among 31 patients whounderwent SPS, 11 (35%) had extracardiac total anomalous pulmonary venous connection (TAPVC). The survivalrate after SPS was 80% at 10 years. Cox regression analysis showed that extracardiac TAPVC (hazard ratio6.44, 95% confidence interval 1.23–33.7, p = 0.03) and pulmonary venous obstruction (PVO) at TAPVC repair(hazard ratio 11.2, 95% confidence interval 2.13–58.5, p = 0.004) were significantly associated with death. In25 patients who underwent bidirectional cavopulmonary shunt (BCPS), surgical interventions on the pulmonaryartery (PA) were performed after SPS in 7 of 9 patients with PA coarctation, 3 of 4 with non-confluent PAs, and4 of 12 with normal PAs. At SPS, primary central PA plasty was performed in three patients with PA coarctationand 2 with non-confluent PAs. There was no significant difference in the PA index before BCPS between patientswith and without primary central PA plasty (p = 0.49). Among 20 patients who underwent total cavopulmonaryconnection (TCPC), adverse events occurred in 7 (35%) patients, including death in 1 (5%), intervention for pulmonaryarteriovenous malformation (PAVM) in 3 (15%), and surgical intervention for PVO in 3 (15%). TheB-type natriuretic peptide concentration was significantly higher in patients with than without adverse events(p = 0.02). The adverse event-free survival rate after TCPC was 69% at 10 years. Conclusion: ExtracardiacTAPVC and PVO at TAPVC repair were significantly associated with death after SPS in patients who had heterotaxysyndrome with a single ventricle. Surgical interventions on the PA were frequently required after SPS inpatients with PA coarctation or non-confluent PAs. Although satisfactory survival was achievable after TCPC,late-onset PAVM and PVO remain concerns.

A Case of Pediatric Heart Failure Caused by Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Case Report and Literature Review

A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardial fi broelastosis and was treated withγ-globulin,prednisone,digoxin,and diuretics.Coronary computed tomographic angiography and coronary angiography were performed as there was no improvement after 2 months of treatment.Finally,the patient received a diagnosis of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA).ALCAPA is a rare congenital heart defect that can cause severe heart failure during infancy,and is easily misdiagnosed clinically.In this report,we show the process of misdiagnosis of the case and consult the relevant literature,hoping to improve the understanding and early diagnosis of ALCAPA.

Anomalous left coronary artery from pulmonary artery: Case series and brief review

Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly. In this study, we present all the ALCAPA patients which were admitted at our institution during April 2007-December 2010. Retrospective review of these patients regarding their clinical presentation and the use of diagnostic modalities will be presented in this series. There were total of five patients, three male and 2 female, with age range of 2 - 12 months. The most common symptoms at presentation were tachypnea (4/5) and poor feeding with irritability (3/5). Electrocardiogram was abnormal in 2/5 cases and chest X ray revealed cardiome-galy with pulmonary congestion in 4/5 patients. Echocardiogram showed mitral valve regurgitation in 5/5 cases (3 with moderate and 2 with mild to moderate), Left ventricular dilatation/dysfunction in 4/5 patients, echogenic left ventricular papillary muscles in 4/5 patients and prominent right coronary with strong suspecision of ALCAPA in 4/5 patients. Coronary angiography was performed in 4/5 cases to confirm the diagnosis. We conclude that by thorough clinical assessment along with ECG and CXR, the diagnosis of ALCAPA can be strongly suspected. Echocardiogram can almost always make the diagnosis of ALCAPA and coronary angiography can confirm the diagnosis in rare atypical cases.

Aortic implantation for anomalous origin of the left coronary artery from the pulmonary artery:A ten-year single center experience

Background At present, creation of a dual coronary system with direct aortic implantation is the preferred management for anomalous origin of left coronary artery from the pulmonary artery （ALCAPA）. However, controversy still exists over the initial management of mitral regurgitation （MR） at the time of ALCAPA repair. Methods Between March 2005 and March 2015, 52 consecutive patients （20 males and 32 females） underwent direct aortic implantation of ALCAPA. Median age at surgery was 10 months （range, 2 to 193）. Mean weight was 9.7_＋7.8 kg. In order to facilitate the analysis, description of MR was given a numeric grade： 0 = none, 1 = trivial, 2 = mild, 2.5 = mild-moderate, 3 = moderate, 3.5 = moderate-severe, and 4 = severe. Results The LV shortening fraction （LVFS） demonstrated significant improvement between presentation and discharge （32.1±10.1% vs. 28.6±9.6%, P = 0.023）, and there was also significant improvement in the mean MR grade between presentation and discharge （2.40＋1.16 vs. 2.91＋ 1.19, P = 0.001）. There were 4 hospital deaths （7.7%）. The median follow-up was 21 months （range 1.5 to 111）, 3 patients （5.8%） were lost to follow-up, 1 patient required reoperation for mitral valve replacement. Significant improvement was seen in LVFS between discharge and late follow-up （38.0±6.0% vs. 32.1±10.1%, P = 0.001）, however, the degree of MR did not dem- onstrate further change （2.29_＋1.20 vs. 2.40＋1.16, P = 0.541）. Conclusion Creation of a dual coronary system with direct aortic implantation of the left coronary artery results in complete recovery of left ventricular function. Concomitant mitral valve （MV） repair for ALCAPA patients with moderate-severe and severe MR is helpful to early MV function recovery.

Surgical Outcomes of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in Children： An Echocardiography Follow？up

Background：Anomalous origin of the left coronary artery from the pulmonary artery （ALCAPA） is a rare but potentially life-threatening congenital heart defect. A retrospective analysis was carried out to elucidate the surgical outcomes of ALCAPA in infants and children using follow-up echocardiography. Methods： From September 2008 to March 2017, 26 children diagnosed with ALCAPA underwent left coronary re-implantation. All surviving patients received echocardiography during follow-up. Results： The mortality rate after the operation was 11.5%. Before repair, twenty patients （76.9%） presented with left ventricular （LV） dysfunction. The mean Z-score of the preoperative LV end-diastolic diameter was 4.42 ± 2.09. Mitral regurgitation （MR） was present in all patients. Two patients （7.7%）, both with mitral valve prolapse, underwent mitral valve repair at the time of ALCAPA repair. Two children required postoperative extracorporeal membrane oxygenation. LV function normalized at a median time of 5.3 months （range：0.5–36.0 months）. The Z-score of the LV end-diastolic diameter decreased simultaneously. The degree of MR gradually decreased in all surviving patients.All patients had patency of the proximal left coronary artery confirmed by echocardiography at the most recent follow-up. Six patients （26.1%） showed supravalvar pulmonary stenosis and seven patients （30.4%） showed right pulmonary stenosis during follow-up. Conclusions： Coronary re-implantation was effective for rebuilding a dual coronary system in patients with ALCAPA and resulted in progressive improved LV function and reduced functional MR. Echocardiography was valuable for evaluating the outcomes. LV function, the degree of MR, and possible complications could be detected with follow-up echocardiography.

Occult anomalous origin of the left coronary artery from the pulmonary artery with atrial septal defect initially visualized by transthoracic echocardiography

Anomalous origin of left coronary artery from the pulmonary artery （ALCAPA） is a rare congenitalcardiac malformation. Here we report a case of ALCAPA with atrial septal defect （ASD） initially visualized by transthoracic echocardiography, subsequently confirmed by the coronary angiography.

Early variation of left ventricular dimension and mitral regurgitation after correction for anomalous origin of the left coronary artery from the pulmonary artery

Background Anomalous origin of the left coronary artery cardiac lesion resulting in myocardial ischemia even infarction, from the pulmonary artery is a rare congenital morphological impairment and dysfunction of left ventricle, together with mitral regurgitation. Here we will introduce our experience in the surgical repair of this kind of congenital lesion and the retrospective analysis about the improvement of left ventricular dimension and mitral regurgitation in early postoperative term. Method From May 1998 to July 2012, 38 consecutive patients with anomalous coronary artery from the pulmonary artery underwent surgical correction （33 received left coronary artery re-implantation, 4 left coronary artery ligation or primary closure, 1 Takeuchi procedure, and 10 simultaneous mitral valve plasty）. Left ventricular dimension, mitral regurgitation, and ejection fraction, were measured by color Doppler echocardiography preoperatively, and 1 month after discharge. Results Hospital survival was 94.7% （2 in-hospital deaths）. Ten paptients with more than moderate mitral regurgitation received simultaneous mitral plasty, one of whom was converted to mechanical prosthetic valve replacement. Mitral valve annuloplasty was applied in 9 cases of coronary re-implantation correction, 3 of whom also received additional mitral leaflet cleft repair. Meanwhile 8 patients underwent other different concomitant operations. Echocardiographic results for the survivals 1 month after discharge showed that left ventricular end-diastolic, endsystolic dimension decreased from 40.05 ± 5.56 mm and 28.94 ± 6.21 mm to 33.07 ± 6.82 mr, （P 〈 0.01） and 23.04 ±5.87 mm （P 〈 0.01） respectively. The average mitral regurgitation grade was also reduced from 2.36 ± 1.08 to 1.64 ± 93 （P 〈 0.05） in the group. All survival patients improved clinically and NYHA functional class decreased significantly from 2.37 ± 1.08 to 2.10 ±0.54 （P 〈 0.05）. Conclusions The surgical repair of anomalous origin of the left coronary artery from the pulmonary artery is safe and effective, and can get satisfactory dimensional and functional improvement of the left ventricle in early term. Although controversial, concomitant mitral valve plasty can be helpful for critical patients with severe mitral regurgitation.

Nursing cooperation in corrective surgery of anomalous origin of the left coronary artery from the pulmonary artery in adult

Background Anomalous Origin of Coronary Artery （AOCA）, a congenital heart disease with low incidence, can cause sudden cardiac death in the young. It＇ s surgical treatment is scarcely performed in clinical practice, thus the special nursing needed during the surgery for this rare disease is largely unrecognized. Method There were 7 adult patients who had suffered from AOCA received corrective operation in our hospital in the last 10 years. Preoperative preparation, nursing cooperation and emergency procedure were analyzed and summarized. Result The AOCA corrective surgery was all successfully completed on these 7 patients. The nursing key points were drawn from these patients. Conclusion Key points of nursing cooperation in AOCA correction surgery include： understanding the condition, mastering pathological anatomy preoperative, making strict application of gradient temperature control ,observing the change of the disease, and carrying out all kinds of emergency preparation during surgery. Meanwhile, careful check in all cases and literature data, participate in preoperative discussion, and skilled techniques are also important to the success of surgery.

右冠状动脉异位起源的急性心肌梗死合并心原性休克1例

右冠状动脉异位起源于左前降支是非常罕见的先天性异常,作为间隔支分支的右冠状动脉临床少有报道。本文报道1例此罕见病例,患者52岁女性,因“突发心前区疼痛4 h,晕厥1次”就诊,心电图提示V1~V4,V3R、V4R、V5R导联ST段抬高约0.1~0.3mV,血压63/40mmHg,考虑诊断为急性前壁、右心室ST段抬高型心肌梗死,心原性休克。予主动脉内球囊反搏(IABP)辅助下行急诊经皮冠状动脉介入治疗,处理罪犯病变时意外发现右冠状动脉作为一分支血管异位起源于第二间隔支开口处。

体外膜氧合成功救治左冠状动脉异常起源于肺动脉术后低心排血量综合征患儿1例

左冠状动脉异常起源于肺动脉(anomalous left coronary artery origin from pulmonary artery,ALCAPA)是一种发病率低、预后较差的先天性心脏畸形,常在婴儿期需要外科手术矫治。低心排血量综合征是指各种原因导致心排出量降低的一种病理异常表现,是心脏术后导致患者死亡的主要原因之一。体外膜氧合(extracorporeal membrane oxygenation,ECMO)是一种可以临时替代心肺功能的装置,在重症及急救领域应用广泛,但在ALCAPA术中及术后应用相关经验报道较少,本文分享一例ECMO成功救治ALCAPA术后低心排血量综合征的案例。

右冠状动脉异常起源于肺动脉的诊疗经验及术后随访研究

目的研究右冠状动脉异常起源于肺动脉(anomalous origin of right coronary artery from the pulmonary artery,ARCAPA)的临床特点,了解不同手术方式的预后情况。方法回顾性分析9例确诊为ARCAPA患儿(患者)的临床资料,包括临床表现、辅助检查、手术方式及随访情况。结果共计8例患儿(儿童),1例患者(成人);男7例,女2例;患儿中位月龄67.5个月,成人患者年龄69岁。患儿中4例因活动后胸闷气促就诊,3例因体检发现心脏杂音就诊,1例因脑梗死就诊完善心脏检查诊断,成人患者因气促胸闷行冠状动脉造影检查后诊断。心电图(ECG)检查示3例患儿无明显心肌缺血表现,1例部分下壁导联深Q波,4例(均>6岁)有不同程度右胸导联ST段抬高表现,成人患者提示完全性右束支传导阻滞(CRBBB)、右胸导联T波双向;经胸超声心动图(TTE)检查显示成人患者术前左心室舒张末期内径(LVEDD)增大明显伴左心室射血分数(LVEF)下降,房室瓣重度反流;有2例患儿术前左心室舒张末期内径增大,左心室射血分数均正常。所有患儿(患者)均完成手术治疗,成人患者行右冠状动脉(RCA)异位开口修补+冠状动脉旁路移植术,5例患儿行右冠状动脉直接移植至主动脉(AO)根部手术,1例患儿右冠状动脉延长后移植至主动脉根部,2例患儿行右冠状动脉结扎术。成人患者术后使用多巴胺5 d,米力农3 d强心治疗,3 d后转出重症监护室,术后复查经胸超声心动图示房室瓣反流好转,目前随访3年,左心室射血分数正常。患儿术后撤机时间为0.5~1 d,使用强心药多巴胺1~2 d,1~2 d后转出重症监护室。目前中位随访时间为36个月,所有术后患者均心功能正常,无需要再次手术患者,无死亡患者。结论ARCAPA患者早期无明显心功能不全症状,临床表现不典型,超声心动图可能漏诊或误诊,及时手术治疗预后效果好。

左冠状动脉异常起源于肺动脉致急性心肌梗死合并室性心动过速1例

左冠状动脉异常起源于肺动脉是一种死亡率极高的疾病,大约90%的患者在出生后的第1年死于心肌梗死和心力衰竭,很少有患者在不做手术的情况下活到成年。而超过50岁的成人左冠状动脉异常起源于肺动脉就更为少见,可能表现为无症状、心绞痛、劳力性呼吸困难、心肌梗死和心原性猝死。而以室性心动过速为首发表现的病例报道更罕见。本文报道1例独特的病例,患者为58岁女性,既往体健,因胸闷、心悸症状持续不缓解于当地医院行心电图检查发现室性心动过速,心肌损伤标志物阴性。5 h后于本院急诊行心电图检查发现胸前导联Q波,ST-T改变,肌钙蛋白I明显升高,后行冠状动脉造影及冠状动脉CT血管造影检查发现左冠状动脉异常起源于肺动脉。

肺动脉异常起源于升主动脉的病理分型及胚胎发生机制探讨

目的 肺动脉异常起源升主动脉的病理分型及胚胎发生机制探讨。方法 5例肺动脉异常起源于升主动脉 ,作心血管造影 (ACG)及磁共振 (MRI)检查。结果 5例病例中 3例为右肺动脉异常起源于升主动脉 ;2例为左肺动脉异常起源于升主动脉 ,4例属近端型 ,1例远端型。结论 肺动脉异常起源于升主动脉为少见的先天性心脏病 ,病理分型有二种 ,一种右肺动脉异常起源于升主动脉 ,占肺动脉异常起源升主动脉的绝大部分 ;另一种为左肺动脉异常起源升主动脉 ,此种类型少见 ,又可根据肺动脉起源离主动脉瓣及无名动脉的距离分近端型及远端型二种亚型。二种类型的胚胎发生学的解释不尽相同。肺动脉异常起源于升主动脉以前称为半永存动脉干 ,从胚胎角度讲与永存动脉干不同 ,故目前不提倡用半永存动脉干这个术语。

一侧肺动脉起源于主动脉及伴发畸形的一期纠治

目的探讨一侧肺动脉起源于主动脉及伴发畸形的纠治经验。方法18例一侧肺动脉起源于主动脉及伴发畸形的患儿,其中15例为右肺动脉起源于主动脉,3例为左肺动脉起源于主动脉。2例为单纯一侧肺动脉起源于主动脉,另16例分别伴有主动脉弓中断、主动脉缩窄、动脉导管未闭、主肺动脉窗、法洛四联症、室间隔缺损、房间隔缺损、二尖瓣返流、三尖瓣返流、气管狭窄等心内外畸形。其中无手术指征和外伤感染各2例;1例放弃治疗;13例手术纠治。结果13例手术患者无死亡病例,其中4例伴主动脉弓中断、主动脉缩窄患儿延迟关胸。术后3月~4年随访,除1例死于肺炎心衰外,余均存活,肺动脉压力下降,全组无主动脉瓣上狭窄及明显肺动脉分支狭窄。结论一侧肺动脉起源于主动脉患儿易早期发生肺动脉高压,一经确诊需立即手术。手术方式首选与伴发畸形一期纠治。患儿尽管可有各种心内外伴发畸形,但早期手术纠治可获得较佳中长期疗效。

单侧肺动脉异常起源于升主动脉的产前超声心动图诊断

目的探讨单侧肺动脉异常起源于升主动脉(AOPA)的产前超声心动图特征,提高对本病产前诊断的准确率。方法回顾性分析2012年6月1日至2018年10月30日经首都医科大学附属北京安贞医院(我院)产前诊断的6例AOPA胎儿的超声心动图特点,总结该病胎儿期的超声心动图特征。结果 5例胎儿为右肺动脉异常起源于升主动脉(AORPA),其中3例为孤立型AORPA,2例合并Berry综合征; 1例为左肺动脉异常起源于升主动脉(AOLPA),同时合并先天性肺动脉瓣缺如综合征。6例胎儿均为近端型AOPA,其中2例胎儿引产(1例AOLPA,1例合并Berry综合征)。出生的4例患儿中,1例Berry综合征患儿于出生后63 d因反复肺炎、心衰死亡; 2例行AORPA根治手术,术后均恢复良好; 1例刚出生,仍在随访中。近端型AOPA胎儿超声心动图特征:①多切面显示正常的肺动脉主干分叉处"八"字型结构消失,正常的肺动脉分支内径增宽;②在三血管气管切面(3VT)及左室长轴切面二维及彩色多普勒显像(CDFI)可显示升主动脉发出一粗大血管向肺内走行,该血管发出位置靠近主动脉瓣左后或右后侧壁的升主动脉;③CDFI显示异常肺动脉分支血流孤立,与主肺动脉缺乏确切连接;④频谱多普勒显示向肺内走行的血管为肺动脉血流频谱;⑤不合并其他心内畸形时,心内结构正常。结论超声心动图可对胎儿期的近端型AOPA做出准确的诊断,3VT及左室长轴切面对诊断近端型AOPA至关重要。

肺动脉异常起源于升主动脉的影像学诊断

目的 探讨肺动脉异常起源于升主动脉的影像学诊断价值。资料与方法 6例肺动脉异常起源于升主动脉 ,作心血管造影 (ACG)及MRI检查。结果 6例中 3例为右肺动脉异常起源于升主动脉 ,均为近端型 ;3例为左肺动脉异常起源于升主动脉 ,1例属近端型 ,2例远端型。结论 肺动脉异常起源于升主动脉为少见的先天性心脏病 ,心血管造影仍是诊断的主要手段 ,MRI是非创伤性检查中较为可靠、有潜力的诊断方法。

单侧肺动脉起源异常的超声心动图诊断及外科治疗

目的分析单侧肺动脉起源异常(AOPA)的超声心动图诊断及外科治疗经验。方法回顾性分析经手术证实的19例AOPA患者的超声心动图特点及外科手术治疗方法。结果 19例AOPA患者中,右肺动脉异常起源占68.42%(13/19);单纯肺动脉起源异常占31.58%(6/19),伴有其他心脏畸形者占68.42%(13/19)。3例患者接受动脉导管未闭结扎和异常起源肺动脉环缩术,余16例接受矫治术。超声心动图表现为主肺动脉分叉结构消失,缺如一侧的肺动脉由主动脉发出。超声诊断AOPA的准确率为94.73%(18/19)。术后超声心动图检测肺动脉吻合口及肺动脉分支无明显狭窄。结论超声心动图是诊断AOPA的有效方法,外科手术治疗效果满意。

肺动脉分支起源异常的超声心动图诊断

目的 对肺动脉分支起源异常超声心动图诊断方法及切面进行探讨。方法 对 6例肺动脉分支起源异常患者的超声心动图诊断及心导管检查结果进行对比分析。结果 超声心动图诊断与心导管检查结果基本一致。结论 超声心动图检查为确诊该病及伴随畸形的首选 ,准确 ,无创检查方法。

左冠状动脉起源于肺动脉6例患儿的诊断与治疗

目的分析左冠状动脉起源于肺动脉(ALCAPA)的诊断特点,评估其手术效果。方法本院2006年3月-2007年10月共收治ALCAPA患儿6例。男4例,女2例;年龄6个月～10岁,平均43.5个月。对这6例患儿的临床表现、心电图(ECG)特征、心脏超声、64排螺旋CT及心导管检查资料、手术治疗及转归进行分析。结果ALCAPA5例患儿有不同程度的多汗、烦躁、气促、拒食等临床表现,临床检查示心功能不全。ECG特异表现:4例患儿ECG存在前侧壁异常Q波及ST段改变,1例患儿存在ST段改变,1例患儿ECG大致正常。典型彩色超声心动图表现为:4例患儿出现左室扩大伴左室收缩功能减低;2例患儿存在二尖瓣轻-中量返流,2例患儿为大量二尖瓣返流;2例患儿表现为右冠状动脉扩张。增强CT及心导管检查有特征性改变。其中5例患儿行手术治疗:2例直接移植,3例行左冠状动脉重建术;2例重度二尖瓣返流患儿同时行二尖瓣成形术。1例无症状患儿未行手术治疗。术后患儿症状改善,无死亡病例。结论了解ALCAPA的病变特点及相关辅助检查,可提高ALCAPA的检出率,尽早手术治疗,改善预后。