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...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.展开更多
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 endocardi...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.展开更多
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 ...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.展开更多
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 t...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.展开更多
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 transth...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.展开更多
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, controv...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.展开更多
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 ...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.展开更多
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...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.展开更多
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...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。展开更多
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...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。展开更多
文摘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.
基金The project was funded by the Natural Science Foundation of Chongqing Science and Technology Commission(cstc2019jcyj-msxmX0866).
文摘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.
文摘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.
文摘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.
文摘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.
基金supported by Guangdong Province Medical Scientific Research Foundation(No.A2016116)
文摘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.
基金supported by The Twelfth National Five-Year Plan (Grant No. 2011BAI11B22)
文摘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.
基金supported by The National Five-Year Plan(No.2011BAI11B22)Guangdong province science and technology plan(No.2011A030400001)
文摘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.
文摘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。
文摘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。
