We report clinical, CT, invasive coronary angiography and intra-operative findings of a symptomatic elderly man with anomalous origin of left coronary artery from pulmonary artery (ALCAPA). ALCAPA is a rare syndrome w...We report clinical, CT, invasive coronary angiography and intra-operative findings of a symptomatic elderly man with anomalous origin of left coronary artery from pulmonary artery (ALCAPA). ALCAPA is a rare syndrome with anomalous origin of left main coronary artery (LMCA) from main pulmonary artery (MPA). Survival into adulthood is rare and our case is probably the oldest survivor who has undergone two coronary system repairs for this anomaly. The unique features of our case include: 1) Absence of “q” wave myocardial infarction on ECG;2) Absence of significant mitral regurgitation;3) Demonstration of stress induced perfusion defects in nuclear imaging which as per our knowledge has been done for the first time in the literature;4) A unique technique of closing anomalous LMCA origin by a pericardial patch, sutured on the internal aspect of MPA is the hallmark of this case.展开更多
Bland-White-Garland syndrome or ALCAPA is an abnormality of birth of the left coronary artery from the pulmonary artery. It is a diagnostic and therapeutic emergency because it is a curable cause of hypokinetic dilate...Bland-White-Garland syndrome or ALCAPA is an abnormality of birth of the left coronary artery from the pulmonary artery. It is a diagnostic and therapeutic emergency because it is a curable cause of hypokinetic dilated heart disease in infants. We report through this clinical case, the fourth case of infantile ALCAPA diagnosed in Senegal in a 7-month-old infant. The symptomatology began around the age of 2 months, with a grumpy state associated with more marked crying and moaning during feedings and bowel movements. The mother reported hospitalization for a severe lung infection when she was 6 months old. The examination noted an infant in poor general condition, retarded growth and weight, and a 3/6th holosystolic murmur at the apex. Troponinemia was positive at 43.90 ng/L. The electrocardiogram showed Q waves on the lower side, a sub endocardial lesion on the upper side and a ST segment elevation in aVR. Doppler echocardiography showed dilated cardiomyopathy with a mean alteration of systolic function of the left ventricle at 37%, a mean mitral insufficiency and a strong suspicion of a birth anomaly of the left coronary artery. The CT scan confirmed the diagnosis of ALCAPA. Surgicalreimplantation of the left coronary artery at the aortic level was performed at 10 months of life with a favourable outcome at D50 postoperative.展开更多
Background Anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA)is associated with myocardial ischemia.This study analyzed the predictive factors associated with prolonged mechanical ventilati...Background Anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA)is associated with myocardial ischemia.This study analyzed the predictive factors associated with prolonged mechanical ventilation(PMV)and prolonged duration of inotropic support(PDIS)following ALCAPA surgery with possible concomitant mitral valve surgery(MVS)in a relatively young population.展开更多
目的:分析超声心动图检查左冠状动脉异常起源于肺动脉(Anomalous origin of the left coronary artery from the pulmonary artery,ALCAPA)的超声表现。方法:回顾性分析6例ALCAPA患者的临床资料,采用超声心动图进行诊断,分析成人型和婴...目的:分析超声心动图检查左冠状动脉异常起源于肺动脉(Anomalous origin of the left coronary artery from the pulmonary artery,ALCAPA)的超声表现。方法:回顾性分析6例ALCAPA患者的临床资料,采用超声心动图进行诊断,分析成人型和婴儿型的超声表现。结果:6例患者中超声确诊5例(83.33%),均为成人型;提示诊断1例,为婴儿型。成人型的超声表现为左冠状动脉未开口于左冠窦,开口于肺动脉根部;室间隔和部分左室壁探及侧枝循环,室间隔和左室前壁运动减弱,伴有二尖瓣轻度或中度反流。婴儿型的超声表现为心内膜、二尖瓣腱索、乳头肌回声增强,室壁运动整体减弱,伴有二尖瓣中重度反流。结论:超声心动图可明确或提示性诊断ALCAPA,具有重要的临床意义。展开更多
Anomalous origin of the left coronary artery from the pulmonary artery （ALCAPA） is a rare congenital anomaly It demonstrated the combined effects of the absence of a normal coronary flow with a coronary steal and th...Anomalous origin of the left coronary artery from the pulmonary artery （ALCAPA） is a rare congenital anomaly It demonstrated the combined effects of the absence of a normal coronary flow with a coronary steal and the profound ischemia that can produce left ventricular dysfunction and mitral regurgitation. We here introduce the postoperative management of patients with repair of anomalous ori artery from the pulmonary artery, with an emphasis on its outcome. Methods Reco gin rds of the left coronary of 31 patients with anomalous origin of the left coronary artery from the pulmonary artery receiving surgery from 1998 to 2010 were reviewed retrospectively, 10 of which were treated with the mitral valve surgically at the same time. The age of patients was 4 months to 16 years （m kilograms）, all of which were diagno edian, sed of anomalous origin of the left coronary artery from the pulmonary by echocardiography and cardiac catheterization. After surgery, electrocardiogram, echocardiography, arterial blood pressure, transcutaneous oxygen saturation and central venous pressure were monitored. Common postoperative complications in our group were analysed. And preoperative and postoperative data including area of mitral regurgitation, left ventricular systolic diameter and left ventricular distolic diameter were obtained. Cardiopulmonary bypass time and mechanical ventilation time of postoperative patients with no pneumonia were compared with those with pneumonia. Binary logistic regression was applied for the analysis of the risk factors of postoperative pneumonia. Results Of 31 patients, 30 survived after surgery with early mortality of 3.23%. One patient died of severe low cardiac output syndrome. Mechanical ventilation time was 4 hours to 168 hours hours （mean, 39.68 ± 50.52 hours; median, 18 hours）. ICU stay was 16 hours to 425 hours （mean, 111.65 ± 127.03 hours; median, 44 hours）. In our group, common postoperative complications were myocardial ischemia（n = 12, 36.4%）, infection（n = 11, 33.3%） including pneumonia （n = 10, 30.3%）, postoperative tachyarrhythmia（n = 5, 15.2%）, low postoperative cardiac output（n = 2, 6.1%）, endocarditis（n = 1, 3.0%）. Compared with preop-erative data, postoperative data including area of mitral regurgitation, left ventricular systolic diameter and left ventricular distolic diameter decreased remarkably（P 〈 0.01）, with left ventricular ejection fraction significantly improved （P 〈 0.05）. Compared with mechanical ventilation time of patients with no postoperative pneumonia, time of those with pneumonia apparently prolonged（P 〈 0.01 ） while cardiopulmonary bypass time extended （P 〈 0.05）. Mechanical ventilation time was a risk factor of postoperative pneumonia （OR = 1.041, OR 95%CI = （1.010, 1.073）. Conclusions Proper therapy strategies according to the knowledge of the ALCAPA disease and the change of postoperative pathology and physiology play an important role in avoiding the postoperative complications and improving postoperative outcomes.展开更多
左冠状动脉异常起源于肺动脉(anomalous origin of the left coronary artery from the pulmonary artery,ALCAPA)是一种罕见又极具生命威胁的先天性冠状动脉畸形。发病率极低,每30万活婴中仅有1例,在先天性心脏病中所占比例约为0.24%至...左冠状动脉异常起源于肺动脉(anomalous origin of the left coronary artery from the pulmonary artery,ALCAPA)是一种罕见又极具生命威胁的先天性冠状动脉畸形。发病率极低,每30万活婴中仅有1例,在先天性心脏病中所占比例约为0.24%至0.46%。未行手术治疗的患儿在1岁内死亡率高达90%,若存在丰富的侧支循环,则可过渡至成人型,由于合并冠状动脉-肺动脉窃血所致的心肌缺血,大多数成年患者可在平均年龄35岁时发生猝死。近年来,随着心动超声图、心导管检查和增强CT等检查技术的提高,并给予早期的手术治疗,使ALCAPA的诊断率和预后得到了极大的改善。展开更多
Background The anomalous origin of the left coronary artery （LCA） from the pulmonary artery （ALCAPA） and congenital left main coronary artery atresia （CLMCA-A） are two kinds of very rare coronary heart diseases ...Background The anomalous origin of the left coronary artery （LCA） from the pulmonary artery （ALCAPA） and congenital left main coronary artery atresia （CLMCA-A） are two kinds of very rare coronary heart diseases which affect heart function profoundly.This study aimed to retrospectively illustrate the clinical features and therapy experience of ALCAPA and CLMCA-A patients.Methods From April 1984 to July 2012,in Beijing Anzhen Hospital,23 patients were diagnosed with ALCAPA and 4 patients with CLMCA-A.We summarized the clinical data of the 27 cases and retrospectively analyzed the clinical manifestation,diagnosis,and treatments of these two kinds of congenital coronary abnormalities.Results The 23 patients （13 males and 10 females,aged ranging from 2.5 months to 65 years） identified with ALCAPA were classified into infantile type （age of onset younger than 12 months,16 cases） and adult type （age of onset older than 12 months,7 cases）.Four patients were diagnosed with CLMCA-A （three males and one female,aged ranging from 3 months to 2 years）.The main clinical manifestations of infantile-type ALCAPA and CLMCA-A include repeated respiratory tract infection,heart failure,dyspnea,feeding intolerance,diaphoresis,and failure to thrive.And these two congenital coronary abnormalities might be misdiagnosed as endocardial fibroelastosis,dilated cardiomyopathy,and acute myocardial infarction.As for the adult-type ALCAPA,cardiac murmurs and discomfort of the precordial area are the most common presentations and might be misdiagnosed as coronary heart disease,myocarditis,or patent ductus arteriosus.In ECG examination：Infantile-type ALCAPA and CLMCA-A showed abnormal Q waves with T wave inversion in leads I,avL,and V4-V6,especially in lead avL.However,ECG of adult-type ALCAPA lacked distinct features.In chest radiography：pulmonary congestion and cardiomegaly were the most common findings in infantile-type ALCAPA and CLMCA-A,while pulmonary artery segment dilation was more common in adult type.In echocardiography,the common features of infantile-type ALCAPA and CLMCA-A included left ventricular enlargement,left ventricular systolic function normal or mildly reduced in CLMCA-A or significantly reduced in ALCAPA,and moderate to large mitral valve.It was performed in 9 of 23 cases of ALCAPA and showed the origin of the dilated right coronary artery （RCA） from the right sinus of the aortic root and absence of LCA origin in angiography.After opacification of RCA,reverse flow in the LCA and pulmonary artery was visualized through coronary artery collateral circulation.Angio was performed in three of the four cases of CLMCA-A and showed left main coronary artery was a blind end,with diameter of only 1.1-2.0 mm.Treatment and prognosis：21 patients with ALCAPA had cardiac surgery and 6 of them died postoperatively.Fifteen postoperative patients survived without overt symptoms within the follow-up period of 6-166 months （median 17 months）.As for treatment of CLMCA-A,four patients took digoxin and diuretics without undergoing cardiac surgery.Their clinical symptoms improved during the close follow-ups.Conclusions ALCAPA and CLMCA-A are two rare coronary artery abnormalities that affect cardiac function in infants and children.In younger patients with cardiomegaly and heart dysfunction these two congenital coronary diseases should be noticed.展开更多
文摘We report clinical, CT, invasive coronary angiography and intra-operative findings of a symptomatic elderly man with anomalous origin of left coronary artery from pulmonary artery (ALCAPA). ALCAPA is a rare syndrome with anomalous origin of left main coronary artery (LMCA) from main pulmonary artery (MPA). Survival into adulthood is rare and our case is probably the oldest survivor who has undergone two coronary system repairs for this anomaly. The unique features of our case include: 1) Absence of “q” wave myocardial infarction on ECG;2) Absence of significant mitral regurgitation;3) Demonstration of stress induced perfusion defects in nuclear imaging which as per our knowledge has been done for the first time in the literature;4) A unique technique of closing anomalous LMCA origin by a pericardial patch, sutured on the internal aspect of MPA is the hallmark of this case.
文摘Bland-White-Garland syndrome or ALCAPA is an abnormality of birth of the left coronary artery from the pulmonary artery. It is a diagnostic and therapeutic emergency because it is a curable cause of hypokinetic dilated heart disease in infants. We report through this clinical case, the fourth case of infantile ALCAPA diagnosed in Senegal in a 7-month-old infant. The symptomatology began around the age of 2 months, with a grumpy state associated with more marked crying and moaning during feedings and bowel movements. The mother reported hospitalization for a severe lung infection when she was 6 months old. The examination noted an infant in poor general condition, retarded growth and weight, and a 3/6th holosystolic murmur at the apex. Troponinemia was positive at 43.90 ng/L. The electrocardiogram showed Q waves on the lower side, a sub endocardial lesion on the upper side and a ST segment elevation in aVR. Doppler echocardiography showed dilated cardiomyopathy with a mean alteration of systolic function of the left ventricle at 37%, a mean mitral insufficiency and a strong suspicion of a birth anomaly of the left coronary artery. The CT scan confirmed the diagnosis of ALCAPA. Surgicalreimplantation of the left coronary artery at the aortic level was performed at 10 months of life with a favourable outcome at D50 postoperative.
文摘Background Anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA)is associated with myocardial ischemia.This study analyzed the predictive factors associated with prolonged mechanical ventilation(PMV)and prolonged duration of inotropic support(PDIS)following ALCAPA surgery with possible concomitant mitral valve surgery(MVS)in a relatively young population.
文摘目的:分析超声心动图检查左冠状动脉异常起源于肺动脉(Anomalous origin of the left coronary artery from the pulmonary artery,ALCAPA)的超声表现。方法:回顾性分析6例ALCAPA患者的临床资料,采用超声心动图进行诊断,分析成人型和婴儿型的超声表现。结果:6例患者中超声确诊5例(83.33%),均为成人型;提示诊断1例,为婴儿型。成人型的超声表现为左冠状动脉未开口于左冠窦,开口于肺动脉根部;室间隔和部分左室壁探及侧枝循环,室间隔和左室前壁运动减弱,伴有二尖瓣轻度或中度反流。婴儿型的超声表现为心内膜、二尖瓣腱索、乳头肌回声增强,室壁运动整体减弱,伴有二尖瓣中重度反流。结论:超声心动图可明确或提示性诊断ALCAPA,具有重要的临床意义。
基金supported by the National Science and Technology Infrastructure Program of the twelfth five-yearplan period(No:2011BAI11B22)
文摘Anomalous origin of the left coronary artery from the pulmonary artery （ALCAPA） is a rare congenital anomaly It demonstrated the combined effects of the absence of a normal coronary flow with a coronary steal and the profound ischemia that can produce left ventricular dysfunction and mitral regurgitation. We here introduce the postoperative management of patients with repair of anomalous ori artery from the pulmonary artery, with an emphasis on its outcome. Methods Reco gin rds of the left coronary of 31 patients with anomalous origin of the left coronary artery from the pulmonary artery receiving surgery from 1998 to 2010 were reviewed retrospectively, 10 of which were treated with the mitral valve surgically at the same time. The age of patients was 4 months to 16 years （m kilograms）, all of which were diagno edian, sed of anomalous origin of the left coronary artery from the pulmonary by echocardiography and cardiac catheterization. After surgery, electrocardiogram, echocardiography, arterial blood pressure, transcutaneous oxygen saturation and central venous pressure were monitored. Common postoperative complications in our group were analysed. And preoperative and postoperative data including area of mitral regurgitation, left ventricular systolic diameter and left ventricular distolic diameter were obtained. Cardiopulmonary bypass time and mechanical ventilation time of postoperative patients with no pneumonia were compared with those with pneumonia. Binary logistic regression was applied for the analysis of the risk factors of postoperative pneumonia. Results Of 31 patients, 30 survived after surgery with early mortality of 3.23%. One patient died of severe low cardiac output syndrome. Mechanical ventilation time was 4 hours to 168 hours hours （mean, 39.68 ± 50.52 hours; median, 18 hours）. ICU stay was 16 hours to 425 hours （mean, 111.65 ± 127.03 hours; median, 44 hours）. In our group, common postoperative complications were myocardial ischemia（n = 12, 36.4%）, infection（n = 11, 33.3%） including pneumonia （n = 10, 30.3%）, postoperative tachyarrhythmia（n = 5, 15.2%）, low postoperative cardiac output（n = 2, 6.1%）, endocarditis（n = 1, 3.0%）. Compared with preop-erative data, postoperative data including area of mitral regurgitation, left ventricular systolic diameter and left ventricular distolic diameter decreased remarkably（P 〈 0.01）, with left ventricular ejection fraction significantly improved （P 〈 0.05）. Compared with mechanical ventilation time of patients with no postoperative pneumonia, time of those with pneumonia apparently prolonged（P 〈 0.01 ） while cardiopulmonary bypass time extended （P 〈 0.05）. Mechanical ventilation time was a risk factor of postoperative pneumonia （OR = 1.041, OR 95%CI = （1.010, 1.073）. Conclusions Proper therapy strategies according to the knowledge of the ALCAPA disease and the change of postoperative pathology and physiology play an important role in avoiding the postoperative complications and improving postoperative outcomes.
文摘左冠状动脉异常起源于肺动脉(anomalous origin of the left coronary artery from the pulmonary artery,ALCAPA)是一种罕见又极具生命威胁的先天性冠状动脉畸形。发病率极低,每30万活婴中仅有1例,在先天性心脏病中所占比例约为0.24%至0.46%。未行手术治疗的患儿在1岁内死亡率高达90%,若存在丰富的侧支循环,则可过渡至成人型,由于合并冠状动脉-肺动脉窃血所致的心肌缺血,大多数成年患者可在平均年龄35岁时发生猝死。近年来,随着心动超声图、心导管检查和增强CT等检查技术的提高,并给予早期的手术治疗,使ALCAPA的诊断率和预后得到了极大的改善。
文摘Background The anomalous origin of the left coronary artery （LCA） from the pulmonary artery （ALCAPA） and congenital left main coronary artery atresia （CLMCA-A） are two kinds of very rare coronary heart diseases which affect heart function profoundly.This study aimed to retrospectively illustrate the clinical features and therapy experience of ALCAPA and CLMCA-A patients.Methods From April 1984 to July 2012,in Beijing Anzhen Hospital,23 patients were diagnosed with ALCAPA and 4 patients with CLMCA-A.We summarized the clinical data of the 27 cases and retrospectively analyzed the clinical manifestation,diagnosis,and treatments of these two kinds of congenital coronary abnormalities.Results The 23 patients （13 males and 10 females,aged ranging from 2.5 months to 65 years） identified with ALCAPA were classified into infantile type （age of onset younger than 12 months,16 cases） and adult type （age of onset older than 12 months,7 cases）.Four patients were diagnosed with CLMCA-A （three males and one female,aged ranging from 3 months to 2 years）.The main clinical manifestations of infantile-type ALCAPA and CLMCA-A include repeated respiratory tract infection,heart failure,dyspnea,feeding intolerance,diaphoresis,and failure to thrive.And these two congenital coronary abnormalities might be misdiagnosed as endocardial fibroelastosis,dilated cardiomyopathy,and acute myocardial infarction.As for the adult-type ALCAPA,cardiac murmurs and discomfort of the precordial area are the most common presentations and might be misdiagnosed as coronary heart disease,myocarditis,or patent ductus arteriosus.In ECG examination：Infantile-type ALCAPA and CLMCA-A showed abnormal Q waves with T wave inversion in leads I,avL,and V4-V6,especially in lead avL.However,ECG of adult-type ALCAPA lacked distinct features.In chest radiography：pulmonary congestion and cardiomegaly were the most common findings in infantile-type ALCAPA and CLMCA-A,while pulmonary artery segment dilation was more common in adult type.In echocardiography,the common features of infantile-type ALCAPA and CLMCA-A included left ventricular enlargement,left ventricular systolic function normal or mildly reduced in CLMCA-A or significantly reduced in ALCAPA,and moderate to large mitral valve.It was performed in 9 of 23 cases of ALCAPA and showed the origin of the dilated right coronary artery （RCA） from the right sinus of the aortic root and absence of LCA origin in angiography.After opacification of RCA,reverse flow in the LCA and pulmonary artery was visualized through coronary artery collateral circulation.Angio was performed in three of the four cases of CLMCA-A and showed left main coronary artery was a blind end,with diameter of only 1.1-2.0 mm.Treatment and prognosis：21 patients with ALCAPA had cardiac surgery and 6 of them died postoperatively.Fifteen postoperative patients survived without overt symptoms within the follow-up period of 6-166 months （median 17 months）.As for treatment of CLMCA-A,four patients took digoxin and diuretics without undergoing cardiac surgery.Their clinical symptoms improved during the close follow-ups.Conclusions ALCAPA and CLMCA-A are two rare coronary artery abnormalities that affect cardiac function in infants and children.In younger patients with cardiomegaly and heart dysfunction these two congenital coronary diseases should be noticed.
