INTERRUPTED aortic arch （IAA） is a rare congenital malformation that occurs in 5.8 per million live births. 1 IAA was thought to be incompatible with life once the ductus arteriosus closed. However, ifextensive coll...INTERRUPTED aortic arch （IAA） is a rare congenital malformation that occurs in 5.8 per million live births. 1 IAA was thought to be incompatible with life once the ductus arteriosus closed. However, ifextensive collateral circulation is p possible. We report treated successfully an unusual resent at case of with extra-anatomic under deep hypothermia drculatory arrest birth survival is IAA, who was aortic repair展开更多
BACKGROUND Aortic coarctation(CoA)is usually confused with interrupted aortic arch(IAA),especially adult type A interrupted aortic arch,due to their similar anatomical location.Although the main difference between the...BACKGROUND Aortic coarctation(CoA)is usually confused with interrupted aortic arch(IAA),especially adult type A interrupted aortic arch,due to their similar anatomical location.Although the main difference between them is whether arterial lumen exhibits continuity or not,the clinical manifestations are similar and connection exists between them.Adult type A IAA is considered as an extreme form of CoA,which is complete discontinuity of aortic function and lumen caused by degenerative arterial coarctation.This paper reports two cases(interrupted aortic arch and severe aortic coarctation)to analyze the difference and similarity between them.CASE SUMMARY The two cases of patients presented with hypertension for many years.Computed tomography angiography showed that the aortic arch and descending aorta were discontinuous or significantly narrowed with extensive collateral flow.The IAA patient refused surgical treatment and blood pressure could be controlled with drugs.While the CoA patient underwent stent implantation because of uncontrollable hypertension,the blood flow recovered smoothly and the blood pressures at both ends of the stenosis returned to normal after surgery.CONCLUSION Adult type A IAA and CoA have difference and similarity,and type A IAA is associated with CoA to a certain extent.The treatment method should be chosen based on the patient's clinical symptoms rather than the severity of the lesion.展开更多
BACKGROUND Interrupted aortic arch(IAA)is a rare congenital heart disease defined by an interruption of the lumen and anatomical continuity between the ascending and descending major arteries.It is usually found withi...BACKGROUND Interrupted aortic arch(IAA)is a rare congenital heart disease defined by an interruption of the lumen and anatomical continuity between the ascending and descending major arteries.It is usually found within a few hours or days of birth.Without surgery,the chances of survival are low.If IAA patients have an effective collateral circulation established,they can survive into adulthood.However,IAA in adults is extremely rare,with few reported cases.CASE SUMMARY A 27-year-old woman presented with a 6-year history of progressively worsening shortness of breath and chest tightness on exertion.She had cyanotic lips and clubbing of the fingers.A transthoracic echocardiogram revealed an enlarged heart and dilation of the main pulmonary artery.There was an abnormal 9 mm passage between the descending aorta and pulmonary artery.The ventricular septal outflow tract had a 14 mm defect.Doppler ultrasound suggested a patent ductus arteriosus and computed tomographic angiography showed the absence of the aortic arch.The diagnoses were ventricular septal defect,patent ductus arteriosus,and definite interruption of the aortic arch.Although surgical correction was recommended,the patient declined due to the surgical risks and was treated with medications to reduce pulmonary artery pressure and treat heart failure.Her condition has been stable for 12 mo of follow-up.CONCLUSION Although rare,IAA should be considered in adults with refractory hypertension or unexplained congestive heart failure.展开更多
The interrupted aortic arch (IAA) is rare congential heart defect and entails a very poor prognosis without surgical treatment. We describe a young adult patient with IAA associated with severe pulmonary hypertension ...The interrupted aortic arch (IAA) is rare congential heart defect and entails a very poor prognosis without surgical treatment. We describe a young adult patient with IAA associated with severe pulmonary hypertension and discuss the perioperative anesthetic management of single-stage surgical correction in this patient. Preoperative anesthetic preparation with better understanding of the malformation, intraoperative hemodynamic monitoring, and adequate alveolar ventilation in the perioperative period were all important factors contributing to a successful outcome.展开更多
Non-A non-B aortic dissection(AAD)is an infrequently documented condition,comprising of only a small proportion of all AADs.The unique anatomy of the aortic arch and the failure of the existing classifications to adeq...Non-A non-B aortic dissection(AAD)is an infrequently documented condition,comprising of only a small proportion of all AADs.The unique anatomy of the aortic arch and the failure of the existing classifications to adequately define individuals with non-A non-B AAD,have led to an ongoing controversy around the topic.It seems that the clinical progression of acute non-A non-B AAD diverges from the typical type A and B dissections,frequently leading to serious complications and thus mandating early intervention.Currently,the available treatment methods in the surgical armamentarium are conventional open,endovascular techniques and combined hybrid methods.The optimum approach is tailored in every individual case and may be determined by the dissection’s location,extent,the aortic diameter,the associated complications and the patient’s status.The management of non-A non-B dissections still remains challenging and a unanimous consensus defining the gold standard treatment has yet to be reached.In an attempt to provide further insight into this perplexing entity,we performed a minireview of the literature,aiming to elucidate the epidemiology,clinical course and the optimal treatment modality.展开更多
Background The coronary artery calcium （CAC） and aortic arch calcification （AoAC） are individually associated with cardiovascular disease and outcome. This study investigated the predictive value of AoAC combined ...Background The coronary artery calcium （CAC） and aortic arch calcification （AoAC） are individually associated with cardiovascular disease and outcome. This study investigated the predictive value of AoAC combined with CAC for cardiovascular diagnosis and outcome in patients with angina. Methods A total of 2018 stable angina patients who underwent chest X-ray and cardiac multi-detector computed tomography were followed up for four years to assess adverse events, which were categorized as cardiac death, stroke, myocardial infarction, or repeated revascularization. The extent of AoAC on chest X-ray was graded on a scale from 0 to 3. Results During the four years of fol- low-up, 620 patients were treated by coronary stenting and 153 （7%） adverse events occurred. A higher grade of AoAC was associated with a higher CAC score. Cox regression showed that the CAC score, but not AoAC, were associated with adverse events. In patients with CAC score 〈 400, AoAC showed an additive predictive value in detecting significant coronary artery disease （CAD）. A gradual increases in the risk of adverse events were noted if AoAC was present in patients with similar CAC score. Conclusions As AoAC is strongly correlated with the CAC score regardless of age or gender, careful evaluation of CAD would be required in patients with AoAC on conventional chest X-rays.展开更多
Objective Reviewing the experience in diagnosis and treatment of cases with aorta-pulmonary septal defect and aortic origin of right pulmonary artery complicated with interruption of aortic arch. Methods Clinical data...Objective Reviewing the experience in diagnosis and treatment of cases with aorta-pulmonary septal defect and aortic origin of right pulmonary artery complicated with interruption of aortic arch. Methods Clinical data from medical records for 5 patients (4 boys and 1 girl,age from 1 to 18 years on admission) who had been treated in Wuhan Asia Heart Hospital between 2005 and 2009展开更多
目的:主动脉弓离断(interruption of aortic arch,IAA)是一种罕见的先天性心脏疾病。本研究探讨IAA胎儿的超声心动图和病理学超微结构特征,并进一步分析其演变的病理过程。方法:回顾性分析产前诊断并经手术或尸体解剖确诊的IAA胎儿的产...目的:主动脉弓离断(interruption of aortic arch,IAA)是一种罕见的先天性心脏疾病。本研究探讨IAA胎儿的超声心动图和病理学超微结构特征,并进一步分析其演变的病理过程。方法:回顾性分析产前诊断并经手术或尸体解剖确诊的IAA胎儿的产前超声心动图、术后病理检查或尸体解剖结果等资料。采用产前超声心动图追踪观察IAA胎儿主动脉弓各节段内径及Z值,以及狭窄段主动脉弓的变化情况,结合尸体解剖及病理检查结果,探讨胎儿IAA可能的宫内演变过程及其发生的细胞学基础。结果:纳入34例IAA胎儿,分别有3、3、28例产前超声诊断为主动脉弓发育不良(aortic arch dysplasia,AAD)、主动脉缩窄(coarctation of aorta,CoA)、IAA。3例AAD和3例CoA胎儿均在产前超声检查1~2周后选择引产,并经尸体解剖证实为IAA。28例产前超声诊断为IAA的胎儿中6例为CoA进行性加重,在超声心动图追踪复查的过程中发现逐渐演变为A型IAA;其他22例首次产前超声即诊断为IAA。3例胎儿分娩后经手术矫正,27例选择终止妊娠,4例宫内死亡。胎儿IAA的超声心动图主要表现包括:心脏四腔心切面左心室明显小于右心室或差别不明显;三血管切面显示主动脉明显小于肺动脉;三血管-气管切面和主动脉弓切面显示主动脉与降主动脉不能相连;主动脉弓切面显示主动脉弓弯曲度变小、走行僵直,主动脉、动脉导管和降主动脉之间失去正常的“V”字形结构。彩色多普勒超声检查显示主动脉弓中断处无连续血流信号,动脉导管内可见血流反转。对7例IAA胎儿进行透射电镜检测,结果显示主动脉弓近离断处的弹力膜之间存在大量平滑肌细胞,数量较升主动脉近端明显增多且排列紊乱,弹力膜较升主动脉近端增厚、走行扭曲;离断处系带无明显正常的内皮细胞和管腔,只有少量断裂坏死内皮细胞的痕迹,大量粗短且排列紊乱的弹力膜和纵横交错的平滑肌细胞无规律分布。结论:产前超声心动图是诊断胎儿IAA的主要手段,通过术后追踪和尸体解剖发现胎儿IAA的并发症和疾病特征,有利于提高其诊断率;部分胎儿IAA可由AAD或CoA逐步演变而来,其发病机制可能与缺血缺氧和动脉导管收缩成分迁移有关。展开更多
Background:Asymptomatic coronary artery stenosis(ACAS)≥50%is common in patients with acute ischemic cerebrovascular disease(AICVD),which portends a poor cardiovascular and cerebrovascular prognosis.Identifying ACAS&g...Background:Asymptomatic coronary artery stenosis(ACAS)≥50%is common in patients with acute ischemic cerebrovascular disease(AICVD),which portends a poor cardiovascular and cerebrovascular prognosis.Identifying ACAS>50%early may optimize the clinical management and improve the outcomes of these high-risk AICVD patients.This study aimed to investigate whether aortic arch plaque(AAP),an early atherosclerotic manifestation of brain blood-supplying arteries,could be a predictor for ACAS>50%in AICVD.Methods:In this cross-sectional study,atherosclerosis of the coronary and brain blood-supplying arteries was simultaneously evaluated using one-step computed tomography angiography(CTA)in AICVD patients without coronary artery disease history.The patients were divided into ACAS≥50%and non-ACAS≥50%groups according to whether CTA showed stenosis≥50%in at least one coronary arterial segment.The AAP characteristics of CTA were depicted from aspects of thickness,extent,and complexity.Results:Among 118 analyzed patients with AICVD,29/118(24.6%)patients had ACAS≥50%,while AAPs were observed in 86/118(72.9%)patients.Increased AAP thickness per millimeter(adjusted odds ratio[OR]:1.56,95%confidence interval[CI]:1.18-2.05),severe-extent AAP(adjusted OR:13.66,95%CI:2.33-80.15),and presence of complex AAP(adjusted OR:7.27,95%CI:2.30-23.03)were associated with ACAS≥50%among patients with AICVD,independently of clinical demographics and cervicocephalic atherosclerotic stenosis.The combination of AAP thickness,extent,and complexity predicted ACAS≥50%with an area under the receiver-operating characteristic curve of 0.78(95%CI:0.70-0.85,P<0.001).All three AAP characteristics provided additional predictive power beyond cervical and intracranial atherosclerotic stenosis for ACAS≥50%in AICVD(all P<0.05).Conclusions:Thicker,severe-extent,and complex AAP were significant markers of the concomitant ACAS≥50%in AICVD,possibly superior to the indicative value of cervical and intracranial atherosclerotic stenosis.As an integral part of atherosclerosis of brain blood-supplying arteries,AAP should not be overlooked in predicting ACAS≥50%for patients with AICVD.展开更多
Background Interrupted aortic arch （IAA） is a rare congenital anomaly affecting 1.5％ of infants with congenital heart disease.Neonatal repair of IAA is required to avoid irreversible pulmonary vascular lesion.Howev...Background Interrupted aortic arch （IAA） is a rare congenital anomaly affecting 1.5％ of infants with congenital heart disease.Neonatal repair of IAA is required to avoid irreversible pulmonary vascular lesion.However,in China,patients with IAA associated with ventricular septal defect （VSD） and patent ductus arteriosus （PDA） over one year of age are common.So we investigated the outcome of surgical treatment of IAA with VSD and PDA in patients over one year of age.Methods From January 2009 to December 2012,19 patients with IAA have undergone complete single-stage repair.The patients＇ mean age was 4.4 years,ranging 1 to 15 years; and their mean weight was 12.8 kg,ranging 4.2 to 36.0 kg.Fifteen IAA were type A,four were type B.Preoperative cardiac catheterization data were available from all patients.Mean pulmonary arterial pressure （mPAP） and pulmonary vascular resistance （PVR） were measured.The measurements of postoperative pulmonary artery pressure were taken in the operating room at the end of the case.All patients underwent echocardiographic examinations before discharged from the hospital.In addition,cardiac catheterization and echocardiographic examinations were performed during follow-up.Selective brain perfusion through the innominate artery during aortic arch reconstruction was used in all patients.Mean follow-up was （1.6±0.8） years.Results There were two hospital deaths （2/19,11％）.One patient died of pulmonary hypertension crisis,and another died of postoperative low cardiac output.Five cases had other main postoperative complications but no postoperative neurologic complications.Seventeen survivors were followed up,and there were no late deaths or reoperation.Mean cross-clamp duration was （85±22） minutes and selective brain perfusion duration was （34±11） minutes.Two patients required delayed sternal closure at two days postoperatively.Intensive care unit and hospital stays were （9±8） days and （47±24） days,respectively.Pressure gradients across the anastomosis at most recent follow up were less than 22 mmHg.mPAP regressed significantly from preoperative （62.1±8.1） mmHg to postoperative （37.3±11.3） mmHg （P ＜0.001） and （24.2±6.0） mmHg at six months after discharged from the hospital （P ＜0.001）.The pulmonary vascular resistance also regressed significantly from preoperative （1 501.4±335.7） dyn·s·cm-5 to （485.0±215.1） dyn·s·cm-5 at six months after discharged from the hospital （P ＜0.001）.The majority of the seventeen patients （89％） were in New York Heart Association （NYHA） class Ⅰ,and 11％ remained in NYHA class Ⅱ.Conclusions Single-stage repair of patients with IAA,VSD and PDA over one year of age can have good surgical results and functional outcomes.Assessment and treatment of pulmonary artery pressure pre-operatively and postoperatively was crucial.mPAP and pulmonary vascular resistance may have regress significantly compared to preoperative values.展开更多
Treatment of aortic arch(AA)diseases is challenging for surgeons.Conventional open surgery remains the“gold standard”but is associated with significant morbidity and mortality despite improvements in techniques.In t...Treatment of aortic arch(AA)diseases is challenging for surgeons.Conventional open surgery remains the“gold standard”but is associated with significant morbidity and mortality despite improvements in techniques.In the last 2 decades,thoracic endovascular aortic repair(TEVAR)has become the first-line treatment for diseases of the descending aorta,and its indications have expanded gradually.Various strategies have been proposed to preserve supra-aortic branches in TEVAR:hybrid technique,chimney technique,fenestration technique(including custom-made fenestrated or“scalloped”stent grafts,in situ fenestration,and physician-modified fenestration),and branched stent grafts.Though acceptable outcomes of endovascular aortic repair have been documented,the evidence regarding its long-term safety and efficacy is lacking,and concerns remain about the risk of cerebrovascular events,retrograde type-A dissection,endoleaks,and branch occlusion.This review discusses the current status and progress of endovascular repair of the AA,and looks toward future trends.We believe that multidisciplinary collaboration of a“HENDO”team(professionals in hybrid repair,endovascular repair,open surgery,cardiovascular anesthesia,and genetics)is essential for future repair of the AA to provide the optimal treatment.展开更多
Background：Accurate assessment of intra-as well as extra-cardiac malformations and radiation dosage concerns are especially crucial to infants and children with interrupted aortic arch （IAA）.The purpose of this stu...Background：Accurate assessment of intra-as well as extra-cardiac malformations and radiation dosage concerns are especially crucial to infants and children with interrupted aortic arch （IAA）.The purpose of this study is to investigate the value of prospective electrocardiogram （ECG）-triggered dual-source computed tomography （DSCT） angiography with low-dosage techniques in the diagnosis of IAA.Methods：Thirteen patients with suspected IAA underwent prospective ECG-triggered DSCT scan and transthoracic echocardiography （TTE）.Surgery was performed on all the patients.A five-point scale was used to assess image quality.The diagnostic accuracy ofDSCT angiography and TTE was compared with the surgical findings as the reference standard.A nonparametric Chi-square test was used for comparative analysis.P＜0.05 was considered as a significant difference.The mean effective radiation dose （ED） was calculated.Results：Diagnostic DSCT images were obtained for all the patients.Thirteen IAA cases with 60 separate cardiovascular anomalies were confirmed by surgical findings.The diagnostic accuracy of TTE and DSCT for total cardiovascular malformations was 93.7％ and 97.9％ （P＞0.05）,and that for extra-cardiac vascular malformations was 92.3％ and 99.0％ （P ＜ 0.05）,respectively.The mean score of image quality was 3.77 ± 0.83.The mean ED was 0.30 ± 0.04 mSv （range from 0.23 mSv to 0.39 mSv）.Conclusions：In infants and children with IAA,prospective ECG-triggered DSCT with low radiation exposure and high diagnostic efficiency has higher accuracy compared to TTE in detection of extra-cardiac vascular anomalies.展开更多
Interrupted aortic arch （IAA） is a rare congenital cardiovascular disease with major intracardiac defects and always with multisystem non-cardiac malformations. It occurs in 1 ： 10,000 births, and about 1% of the p...Interrupted aortic arch （IAA） is a rare congenital cardiovascular disease with major intracardiac defects and always with multisystem non-cardiac malformations. It occurs in 1 ： 10,000 births, and about 1% of the patients with congenital heart defects. Therefore, it is important that we make a good diagnosis and accurate evaluation of their morphologic conditions before a heart operation. We presented a case of IAA diagnosed by ECGgated multi-slice computed tomography （MSCT） angiography.展开更多
Persistent fifth aortic arch (PFAA) is a rare congenital cardiovascular malformation that occurs when the pharyngeal fifth aortic arch does not degenerate during the embryonic period. The first case of PFAA was descri...Persistent fifth aortic arch (PFAA) is a rare congenital cardiovascular malformation that occurs when the pharyngeal fifth aortic arch does not degenerate during the embryonic period. The first case of PFAA was described in an autopsy specimen in 1969.[1] In 1973, the persistence of a left fifth aortic arch was first reported.[2] Since then, several case reports have described PFAA in different forms. PFAA stenosis associated with type A interruption of the aortic arch (type-A IAA) is most common in clinical practice and requires surgical intervention. From 2013 to 2018, six cases [Table 1 and Table 2] were diagnosed using echocardiography and computed tomography angiography (CTA). Five cases were successfully treated with surgery and followed-up. The purpose of this study was to summarize the clinical characteristics and diagnostic features of PFAA stenosis associated with type-A IAA to improve its diagnostic accuracy and allow for complete pre-operative preparation and proper treatment.展开更多
文摘INTERRUPTED aortic arch （IAA） is a rare congenital malformation that occurs in 5.8 per million live births. 1 IAA was thought to be incompatible with life once the ductus arteriosus closed. However, ifextensive collateral circulation is p possible. We report treated successfully an unusual resent at case of with extra-anatomic under deep hypothermia drculatory arrest birth survival is IAA, who was aortic repair
文摘BACKGROUND Aortic coarctation(CoA)is usually confused with interrupted aortic arch(IAA),especially adult type A interrupted aortic arch,due to their similar anatomical location.Although the main difference between them is whether arterial lumen exhibits continuity or not,the clinical manifestations are similar and connection exists between them.Adult type A IAA is considered as an extreme form of CoA,which is complete discontinuity of aortic function and lumen caused by degenerative arterial coarctation.This paper reports two cases(interrupted aortic arch and severe aortic coarctation)to analyze the difference and similarity between them.CASE SUMMARY The two cases of patients presented with hypertension for many years.Computed tomography angiography showed that the aortic arch and descending aorta were discontinuous or significantly narrowed with extensive collateral flow.The IAA patient refused surgical treatment and blood pressure could be controlled with drugs.While the CoA patient underwent stent implantation because of uncontrollable hypertension,the blood flow recovered smoothly and the blood pressures at both ends of the stenosis returned to normal after surgery.CONCLUSION Adult type A IAA and CoA have difference and similarity,and type A IAA is associated with CoA to a certain extent.The treatment method should be chosen based on the patient's clinical symptoms rather than the severity of the lesion.
文摘BACKGROUND Interrupted aortic arch(IAA)is a rare congenital heart disease defined by an interruption of the lumen and anatomical continuity between the ascending and descending major arteries.It is usually found within a few hours or days of birth.Without surgery,the chances of survival are low.If IAA patients have an effective collateral circulation established,they can survive into adulthood.However,IAA in adults is extremely rare,with few reported cases.CASE SUMMARY A 27-year-old woman presented with a 6-year history of progressively worsening shortness of breath and chest tightness on exertion.She had cyanotic lips and clubbing of the fingers.A transthoracic echocardiogram revealed an enlarged heart and dilation of the main pulmonary artery.There was an abnormal 9 mm passage between the descending aorta and pulmonary artery.The ventricular septal outflow tract had a 14 mm defect.Doppler ultrasound suggested a patent ductus arteriosus and computed tomographic angiography showed the absence of the aortic arch.The diagnoses were ventricular septal defect,patent ductus arteriosus,and definite interruption of the aortic arch.Although surgical correction was recommended,the patient declined due to the surgical risks and was treated with medications to reduce pulmonary artery pressure and treat heart failure.Her condition has been stable for 12 mo of follow-up.CONCLUSION Although rare,IAA should be considered in adults with refractory hypertension or unexplained congestive heart failure.
文摘The interrupted aortic arch (IAA) is rare congential heart defect and entails a very poor prognosis without surgical treatment. We describe a young adult patient with IAA associated with severe pulmonary hypertension and discuss the perioperative anesthetic management of single-stage surgical correction in this patient. Preoperative anesthetic preparation with better understanding of the malformation, intraoperative hemodynamic monitoring, and adequate alveolar ventilation in the perioperative period were all important factors contributing to a successful outcome.
文摘Non-A non-B aortic dissection(AAD)is an infrequently documented condition,comprising of only a small proportion of all AADs.The unique anatomy of the aortic arch and the failure of the existing classifications to adequately define individuals with non-A non-B AAD,have led to an ongoing controversy around the topic.It seems that the clinical progression of acute non-A non-B AAD diverges from the typical type A and B dissections,frequently leading to serious complications and thus mandating early intervention.Currently,the available treatment methods in the surgical armamentarium are conventional open,endovascular techniques and combined hybrid methods.The optimum approach is tailored in every individual case and may be determined by the dissection’s location,extent,the aortic diameter,the associated complications and the patient’s status.The management of non-A non-B dissections still remains challenging and a unanimous consensus defining the gold standard treatment has yet to be reached.In an attempt to provide further insight into this perplexing entity,we performed a minireview of the literature,aiming to elucidate the epidemiology,clinical course and the optimal treatment modality.
文摘Background The coronary artery calcium （CAC） and aortic arch calcification （AoAC） are individually associated with cardiovascular disease and outcome. This study investigated the predictive value of AoAC combined with CAC for cardiovascular diagnosis and outcome in patients with angina. Methods A total of 2018 stable angina patients who underwent chest X-ray and cardiac multi-detector computed tomography were followed up for four years to assess adverse events, which were categorized as cardiac death, stroke, myocardial infarction, or repeated revascularization. The extent of AoAC on chest X-ray was graded on a scale from 0 to 3. Results During the four years of fol- low-up, 620 patients were treated by coronary stenting and 153 （7%） adverse events occurred. A higher grade of AoAC was associated with a higher CAC score. Cox regression showed that the CAC score, but not AoAC, were associated with adverse events. In patients with CAC score 〈 400, AoAC showed an additive predictive value in detecting significant coronary artery disease （CAD）. A gradual increases in the risk of adverse events were noted if AoAC was present in patients with similar CAC score. Conclusions As AoAC is strongly correlated with the CAC score regardless of age or gender, careful evaluation of CAD would be required in patients with AoAC on conventional chest X-rays.
文摘Objective Reviewing the experience in diagnosis and treatment of cases with aorta-pulmonary septal defect and aortic origin of right pulmonary artery complicated with interruption of aortic arch. Methods Clinical data from medical records for 5 patients (4 boys and 1 girl,age from 1 to 18 years on admission) who had been treated in Wuhan Asia Heart Hospital between 2005 and 2009
文摘目的:主动脉弓离断(interruption of aortic arch,IAA)是一种罕见的先天性心脏疾病。本研究探讨IAA胎儿的超声心动图和病理学超微结构特征,并进一步分析其演变的病理过程。方法:回顾性分析产前诊断并经手术或尸体解剖确诊的IAA胎儿的产前超声心动图、术后病理检查或尸体解剖结果等资料。采用产前超声心动图追踪观察IAA胎儿主动脉弓各节段内径及Z值,以及狭窄段主动脉弓的变化情况,结合尸体解剖及病理检查结果,探讨胎儿IAA可能的宫内演变过程及其发生的细胞学基础。结果:纳入34例IAA胎儿,分别有3、3、28例产前超声诊断为主动脉弓发育不良(aortic arch dysplasia,AAD)、主动脉缩窄(coarctation of aorta,CoA)、IAA。3例AAD和3例CoA胎儿均在产前超声检查1~2周后选择引产,并经尸体解剖证实为IAA。28例产前超声诊断为IAA的胎儿中6例为CoA进行性加重,在超声心动图追踪复查的过程中发现逐渐演变为A型IAA;其他22例首次产前超声即诊断为IAA。3例胎儿分娩后经手术矫正,27例选择终止妊娠,4例宫内死亡。胎儿IAA的超声心动图主要表现包括:心脏四腔心切面左心室明显小于右心室或差别不明显;三血管切面显示主动脉明显小于肺动脉;三血管-气管切面和主动脉弓切面显示主动脉与降主动脉不能相连;主动脉弓切面显示主动脉弓弯曲度变小、走行僵直,主动脉、动脉导管和降主动脉之间失去正常的“V”字形结构。彩色多普勒超声检查显示主动脉弓中断处无连续血流信号,动脉导管内可见血流反转。对7例IAA胎儿进行透射电镜检测,结果显示主动脉弓近离断处的弹力膜之间存在大量平滑肌细胞,数量较升主动脉近端明显增多且排列紊乱,弹力膜较升主动脉近端增厚、走行扭曲;离断处系带无明显正常的内皮细胞和管腔,只有少量断裂坏死内皮细胞的痕迹,大量粗短且排列紊乱的弹力膜和纵横交错的平滑肌细胞无规律分布。结论:产前超声心动图是诊断胎儿IAA的主要手段,通过术后追踪和尸体解剖发现胎儿IAA的并发症和疾病特征,有利于提高其诊断率;部分胎儿IAA可由AAD或CoA逐步演变而来,其发病机制可能与缺血缺氧和动脉导管收缩成分迁移有关。
基金Beijing Municipal Administration of Hospitals Clinical Medicine Development of Special Funding Support(No.ZYLX201706)Beijing Municipal Natural Science Foundation(No.7172093).
文摘Background:Asymptomatic coronary artery stenosis(ACAS)≥50%is common in patients with acute ischemic cerebrovascular disease(AICVD),which portends a poor cardiovascular and cerebrovascular prognosis.Identifying ACAS>50%early may optimize the clinical management and improve the outcomes of these high-risk AICVD patients.This study aimed to investigate whether aortic arch plaque(AAP),an early atherosclerotic manifestation of brain blood-supplying arteries,could be a predictor for ACAS>50%in AICVD.Methods:In this cross-sectional study,atherosclerosis of the coronary and brain blood-supplying arteries was simultaneously evaluated using one-step computed tomography angiography(CTA)in AICVD patients without coronary artery disease history.The patients were divided into ACAS≥50%and non-ACAS≥50%groups according to whether CTA showed stenosis≥50%in at least one coronary arterial segment.The AAP characteristics of CTA were depicted from aspects of thickness,extent,and complexity.Results:Among 118 analyzed patients with AICVD,29/118(24.6%)patients had ACAS≥50%,while AAPs were observed in 86/118(72.9%)patients.Increased AAP thickness per millimeter(adjusted odds ratio[OR]:1.56,95%confidence interval[CI]:1.18-2.05),severe-extent AAP(adjusted OR:13.66,95%CI:2.33-80.15),and presence of complex AAP(adjusted OR:7.27,95%CI:2.30-23.03)were associated with ACAS≥50%among patients with AICVD,independently of clinical demographics and cervicocephalic atherosclerotic stenosis.The combination of AAP thickness,extent,and complexity predicted ACAS≥50%with an area under the receiver-operating characteristic curve of 0.78(95%CI:0.70-0.85,P<0.001).All three AAP characteristics provided additional predictive power beyond cervical and intracranial atherosclerotic stenosis for ACAS≥50%in AICVD(all P<0.05).Conclusions:Thicker,severe-extent,and complex AAP were significant markers of the concomitant ACAS≥50%in AICVD,possibly superior to the indicative value of cervical and intracranial atherosclerotic stenosis.As an integral part of atherosclerosis of brain blood-supplying arteries,AAP should not be overlooked in predicting ACAS≥50%for patients with AICVD.
基金This study was supported by Capital Citizens＇ Health Project Cultivation of Beijing Municipal Science and Technology Commissions （No. Z 11110007491100） and Beijing Municipal Natural Science Foundation （No. 7112046）.
文摘Background Interrupted aortic arch （IAA） is a rare congenital anomaly affecting 1.5％ of infants with congenital heart disease.Neonatal repair of IAA is required to avoid irreversible pulmonary vascular lesion.However,in China,patients with IAA associated with ventricular septal defect （VSD） and patent ductus arteriosus （PDA） over one year of age are common.So we investigated the outcome of surgical treatment of IAA with VSD and PDA in patients over one year of age.Methods From January 2009 to December 2012,19 patients with IAA have undergone complete single-stage repair.The patients＇ mean age was 4.4 years,ranging 1 to 15 years; and their mean weight was 12.8 kg,ranging 4.2 to 36.0 kg.Fifteen IAA were type A,four were type B.Preoperative cardiac catheterization data were available from all patients.Mean pulmonary arterial pressure （mPAP） and pulmonary vascular resistance （PVR） were measured.The measurements of postoperative pulmonary artery pressure were taken in the operating room at the end of the case.All patients underwent echocardiographic examinations before discharged from the hospital.In addition,cardiac catheterization and echocardiographic examinations were performed during follow-up.Selective brain perfusion through the innominate artery during aortic arch reconstruction was used in all patients.Mean follow-up was （1.6±0.8） years.Results There were two hospital deaths （2/19,11％）.One patient died of pulmonary hypertension crisis,and another died of postoperative low cardiac output.Five cases had other main postoperative complications but no postoperative neurologic complications.Seventeen survivors were followed up,and there were no late deaths or reoperation.Mean cross-clamp duration was （85±22） minutes and selective brain perfusion duration was （34±11） minutes.Two patients required delayed sternal closure at two days postoperatively.Intensive care unit and hospital stays were （9±8） days and （47±24） days,respectively.Pressure gradients across the anastomosis at most recent follow up were less than 22 mmHg.mPAP regressed significantly from preoperative （62.1±8.1） mmHg to postoperative （37.3±11.3） mmHg （P ＜0.001） and （24.2±6.0） mmHg at six months after discharged from the hospital （P ＜0.001）.The pulmonary vascular resistance also regressed significantly from preoperative （1 501.4±335.7） dyn·s·cm-5 to （485.0±215.1） dyn·s·cm-5 at six months after discharged from the hospital （P ＜0.001）.The majority of the seventeen patients （89％） were in New York Heart Association （NYHA） class Ⅰ,and 11％ remained in NYHA class Ⅱ.Conclusions Single-stage repair of patients with IAA,VSD and PDA over one year of age can have good surgical results and functional outcomes.Assessment and treatment of pulmonary artery pressure pre-operatively and postoperatively was crucial.mPAP and pulmonary vascular resistance may have regress significantly compared to preoperative values.
基金supported by the National Natural Science Foundation of China(81870345 and 81800400).
文摘Treatment of aortic arch(AA)diseases is challenging for surgeons.Conventional open surgery remains the“gold standard”but is associated with significant morbidity and mortality despite improvements in techniques.In the last 2 decades,thoracic endovascular aortic repair(TEVAR)has become the first-line treatment for diseases of the descending aorta,and its indications have expanded gradually.Various strategies have been proposed to preserve supra-aortic branches in TEVAR:hybrid technique,chimney technique,fenestration technique(including custom-made fenestrated or“scalloped”stent grafts,in situ fenestration,and physician-modified fenestration),and branched stent grafts.Though acceptable outcomes of endovascular aortic repair have been documented,the evidence regarding its long-term safety and efficacy is lacking,and concerns remain about the risk of cerebrovascular events,retrograde type-A dissection,endoleaks,and branch occlusion.This review discusses the current status and progress of endovascular repair of the AA,and looks toward future trends.We believe that multidisciplinary collaboration of a“HENDO”team(professionals in hybrid repair,endovascular repair,open surgery,cardiovascular anesthesia,and genetics)is essential for future repair of the AA to provide the optimal treatment.
文摘Background：Accurate assessment of intra-as well as extra-cardiac malformations and radiation dosage concerns are especially crucial to infants and children with interrupted aortic arch （IAA）.The purpose of this study is to investigate the value of prospective electrocardiogram （ECG）-triggered dual-source computed tomography （DSCT） angiography with low-dosage techniques in the diagnosis of IAA.Methods：Thirteen patients with suspected IAA underwent prospective ECG-triggered DSCT scan and transthoracic echocardiography （TTE）.Surgery was performed on all the patients.A five-point scale was used to assess image quality.The diagnostic accuracy ofDSCT angiography and TTE was compared with the surgical findings as the reference standard.A nonparametric Chi-square test was used for comparative analysis.P＜0.05 was considered as a significant difference.The mean effective radiation dose （ED） was calculated.Results：Diagnostic DSCT images were obtained for all the patients.Thirteen IAA cases with 60 separate cardiovascular anomalies were confirmed by surgical findings.The diagnostic accuracy of TTE and DSCT for total cardiovascular malformations was 93.7％ and 97.9％ （P＞0.05）,and that for extra-cardiac vascular malformations was 92.3％ and 99.0％ （P ＜ 0.05）,respectively.The mean score of image quality was 3.77 ± 0.83.The mean ED was 0.30 ± 0.04 mSv （range from 0.23 mSv to 0.39 mSv）.Conclusions：In infants and children with IAA,prospective ECG-triggered DSCT with low radiation exposure and high diagnostic efficiency has higher accuracy compared to TTE in detection of extra-cardiac vascular anomalies.
文摘Interrupted aortic arch （IAA） is a rare congenital cardiovascular disease with major intracardiac defects and always with multisystem non-cardiac malformations. It occurs in 1 ： 10,000 births, and about 1% of the patients with congenital heart defects. Therefore, it is important that we make a good diagnosis and accurate evaluation of their morphologic conditions before a heart operation. We presented a case of IAA diagnosed by ECGgated multi-slice computed tomography （MSCT） angiography.
文摘Persistent fifth aortic arch (PFAA) is a rare congenital cardiovascular malformation that occurs when the pharyngeal fifth aortic arch does not degenerate during the embryonic period. The first case of PFAA was described in an autopsy specimen in 1969.[1] In 1973, the persistence of a left fifth aortic arch was first reported.[2] Since then, several case reports have described PFAA in different forms. PFAA stenosis associated with type A interruption of the aortic arch (type-A IAA) is most common in clinical practice and requires surgical intervention. From 2013 to 2018, six cases [Table 1 and Table 2] were diagnosed using echocardiography and computed tomography angiography (CTA). Five cases were successfully treated with surgery and followed-up. The purpose of this study was to summarize the clinical characteristics and diagnostic features of PFAA stenosis associated with type-A IAA to improve its diagnostic accuracy and allow for complete pre-operative preparation and proper treatment.
