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Celastrol: A new potential therapeutic option in pulmonary hypertension associated with bronchopulmonary dysplasia 雷公藤红素:支气管肺发育不良伴肺动脉高压的一种新的潜在治疗选择
IF 18 Q4 Medicine Pub Date : 2023-09-01 DOI: 10.1016/j.acvdsp.2023.07.040
C.-M. Pilard , G. Cardouat , I. Gauthereau , P. Robillard , F. Sauvestre , F. Pelluard , S. Berenguer , L. Sentilhes , F. Coatleven , L. Renesme , E. Dumas-De-La-Roque , R. Marthan , P. Berger , V. Freund-Michel , C. Guibert

Introduction

Bronchopulmonary dysplasia is a common pathology of premature newborns caused by oxygen and mechanical ventilation, resulting in hypoalveolarization and impaired angiogenesis. It can be complicated by pulmonary hypertension (PH-BPD) with excess mortality. Celastrol has anti-inflammatory and antioxidant properties.

Objective

We addressed potential therapeutic interest of celastrol in PH-BPD.

Methods

Different concentrations of celastrol (0,1–0,5–1 mg/kg/d) were tested in a murine model of hyperoxic PH-BPD (14 days with 90% O2–Hx). We evaluated hemodynamic parameters with echocardiography, lung remodeling with histological methods and pulmonary arterial (PA) reactivity with myograph. Cytoplasmic calcium (Ca2+i) response to endothelin-1 (ET-1) was evaluated in human fetal PA smooth muscle cells (fPASMC) with spectrophotometry in normoxic (21% O2) and Hx conditions (48 h with 60% O2). Inflammation and oxidant stress were also addressed.

Results/Expected results

Celastrol reduces mortality at doses of 1 and 0,5 mg/kg/d compared to Hx control. At 1 mg/kg/d, it normalizes PA acceleration time and reduces vascular hyperreactivity to ET-1 inversely to phenylephrine and 5-HT, with no effect on the altered response to acetylcholine induced by Hx. At all doses, it decreases right heart hypertrophy and vascular remodeling but has no effect on media cells proliferation observed in Hx condition. It has also no impact on alveolarization and vascular density. Although celastrol (0.1 and 0.3 μM) decreases the increased Ca2+i response to ET-1 in Hx in fPASMC, expression of ET-1 receptors was unchanged. In fPASMC, celastrol 0.3 μM decreases the increased concentration of IL6 observed in Hx but has no effect on IL8 and MIF. In both models, Hx-induced oxidant stress remains mild with absence of protein carbonylation and lipid peroxidation and celastrol at 1 mg/kg/d increases heme oxygenase 1 expression.

Conclusion/Perspectives

Celastrol has a preventive dose-dependent effect on major hallmarks of PH induced by Hx and could be considered as a new potential therapeutic option for PH-BPD.

支气管肺发育不良是早产新生儿由氧气和机械通气引起的常见病理,导致肺泡发育降低和血管生成受损。它可并发肺动脉高压(PH-BPD),死亡率高。Celastrol具有抗炎和抗氧化的特性。目的探讨雷公藤红素在PH-BPD中的潜在治疗价值。方法采用高氧PH-BPD小鼠模型(90% O2-Hx 14 d),测定不同浓度的celastrol(0、1-0、5-1 mg/kg/d)。我们用超声心动图评估血流动力学参数,用组织学方法评估肺重塑,用肌图评估肺动脉反应性。用分光光度法测定了人胎儿PA平滑肌细胞(fPASMC)在常氧(21% O2)和高氧(60% O2)条件下(48 h)细胞质钙(Ca2+i)对内皮素-1 (ET-1)的反应。炎症和氧化应激也得到了解决。结果/预期结果与Hx对照相比,1和0.5 mg/kg/d剂量的雷公藤酚可降低死亡率。在1 mg/kg/d时,它使PA加速时间正常化,并降低血管对ET-1的高反应性,而与苯肾上腺素和5-HT相反,对Hx诱导的乙酰胆碱反应的改变没有影响。在所有剂量下,它都能减少右心肥厚和血管重构,但对Hx条件下观察到的介质细胞增殖没有影响。它对肺泡化和血管密度也没有影响。虽然雷公藤红素(0.1 μM和0.3 μM)降低了fPASMC中Hx中增加的ET-1对Ca2+i的响应,但ET-1受体的表达不变。在fPASMC中,celastrol 0.3 μM降低了Hx中IL6浓度的升高,但对IL8和MIF没有影响。在这两种模型中,hx诱导的氧化应激都是轻度的,没有蛋白质羰基化和脂质过氧化,1 mg/kg/d的celastrol增加了血红素加氧酶1的表达。结论/观点celastrol对Hx诱导的PH主要标志物具有剂量依赖性的预防作用,可被认为是PH- bpd的一种新的潜在治疗选择。
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引用次数: 0
Exploring the potential of artificial intelligence in pediatric echocardiography. Preliminary results from the first pediatric study using AI software developed for adults 探索人工智能在儿童超声心动图中的潜力。这是首个使用为成人开发的人工智能软件的儿科研究的初步结果
IF 18 Q4 Medicine Pub Date : 2023-09-01 DOI: 10.1016/j.acvdsp.2023.07.038
C. Vasile , X. Bouteiller , M. Avesani , C. Velly , C. Chan , J.B. Thambo , X. Iriart

Introduction

Transthoracic echocardiography is the first-line non-invasive investigation for assessing pediatric patients. Sometimes performing echocardiography in pediatric patients can be challenging for physicians and sonographers due to factors such as patient cooperation and time constraints.

Objective

Our primary purpose was to establish whether the Ligence Heart software validated for analyzing adult echocardiography could be transposed to pediatric patients.

Methods

The study was conducted at the University Hospital of Bordeaux between August and September 2022 and included 45 patients with normal or near-normal heart architecture who underwent a 2D TTE. The ultrasound scans were performed manually by two medical practitioners, a senior, a junior and the AI software provided by Ligence Heart Company. Twenty-three features were assessed through echocardiography for each patient. We compared the agreement between methods and observers.

Results/Expected results

The mean age of our patients at the time of evaluation was 8.2 years ± 5.7, and the main reason for referral to our service was the presence of a heart murmur. Bland-Altman analysis showed good agreement between AI and the senior physician for two parameters (aortic annulus and E wave) regardless of the age of the children included in the study. A good agreement between AI and physicians was also achieved for two other features (STJ and EF) but only for patients older than 9 years. For other features, a good agreement was found between physicians but not with the AI, or a poor agreement was established. In the first case, maybe proper training of the AI could improve the measurement, but in the latter case, for now, it seems unrealistic to expect to reach a satisfactory accuracy.

Conclusion/Perspectives

Based on this preliminary study on a small cohort group of pediatric patients, the AI soft originally developed for the adult population had provided promising results in evaluating aortic annulus, STJ, and E wave.

引言经胸超声心动图是评估儿科患者的一线非侵入性研究。有时,由于患者合作和时间限制等因素,在儿科患者中进行超声心动图检查对医生和超声学家来说可能是一项挑战。目的我们的主要目的是确定用于分析成人超声心动图的Ligence Heart软件是否可以用于儿科患者。方法该研究于2022年8月至9月在波尔多大学医院进行,包括45名心脏结构正常或接近正常的患者,他们接受了二维经胸超声心动图检查。超声波扫描由两名医生手动进行,一名大四,一名小三,人工智能软件由Ligence心脏公司提供。通过超声心动图对每位患者的23个特征进行评估。我们比较了方法和观察者之间的一致性。结果/预期结果在评估时,我们患者的平均年龄为8.2岁±5.7岁,转诊到我们服务的主要原因是存在心脏杂音。Bland-Altman分析显示,无论研究中儿童的年龄如何,AI和高级医生在两个参数(主动脉瓣环和E波)方面都有很好的一致性。AI和医生在其他两个特征(STJ和EF)方面也达成了良好的一致,但仅适用于9岁以上的患者。对于其他特征,医生之间达成了良好的协议,但与人工智能没有达成,或者达成了较差的协议。在第一种情况下,也许人工智能的适当训练可以改善测量,但在后一种情况下——就目前而言,期望达到令人满意的精度似乎是不现实的。结论/展望基于这项针对儿童患者小队列的初步研究,最初为成年人群开发的AI软件在评估主动脉瓣环、STJ和E波方面提供了有希望的结果。
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引用次数: 0
Éditorial 社论
IF 18 Q4 Medicine Pub Date : 2023-09-01 DOI: 10.1016/j.acvdsp.2023.08.001
Le Comité d’Organisation local, Caroline Ovaert, Virginie Fouilloux, Au nom de toute l’équipe médico-chirurgicale marseillaise
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引用次数: 0
Congenital disconnection of the pulmonary arteries 先天性肺动脉断裂
IF 18 Q4 Medicine Pub Date : 2023-09-01 DOI: 10.1016/j.acvdsp.2023.07.011
J. Karila Cohen, D. Bonnet

Introduction

Disconnected pulmonary artery is a rare anomaly that can be isolated or associated with complex intracardiac malformations. Early re-implantation of the disconnected pulmonary artery is recommended to allow growth and satisfactory pulmonary perfusion while preventing collateral arteries development.

Objective

To describe the characteristics of patients with disconnected pulmonary artery and, for those who had surgical re-implantation, to determine the incidence, delay and predictive factors of re-intervention for reconnected pulmonary artery stenosis.

Methods

We include patient with diagnosis of congenitally disconnected PA and surgical repair at our institution.

Results/Expected results

Retrospective observational study of 55 patients with a disconnected pulmonary artery. Fifty-one underwent surgical correction and were followed up at our institution between 2000 and 2022.

Disconnected pulmonary arteries were observed in isolation in 31% of the cases. The most frequent form was left pulmonary artery originating from the arterial duct (58%). The re-implantation was done at median age of 12 days. Anastomotic stenosis was observed during follow-up in 71% of the patients with 75% of them requiring re-intervention (55% of the population). The median delay to re-intervention was 3.2 years after re-implantation, and more than 25% of re-interventions on the re-implanted pulmonary artery occurred within the first postoperative year. We found more re-intervention if associated cardiac defect, without significant statistic difference. Weight at re-confluence, presence of ductal tissue at the origin of the pulmonary artery, and prior shunt placement on the disconnected pulmonary artery were not found to be risk factors for re-intervention (Figure 1).

Conclusion/Perspectives

After surgical re-implantation of pulmonary artery, more than 50% of patients required re-intervention for pulmonary artery stenosis. Technical improvements should be sought to reduce incidence of this complication.

引言肺动脉断开是一种罕见的异常,可以孤立或与复杂的心内畸形相关。建议尽早重新植入断开的肺动脉,以允许生长和令人满意的肺灌注,同时防止侧支动脉发育。目的描述肺动脉断开患者的特点,并针对那些进行手术再植入的患者,确定再介入治疗肺动脉狭窄的发生率、延迟和预测因素。方法我们包括诊断为先天性PA断开并在我们机构进行手术修复的患者。结果/预期结果55例肺动脉断开患者的回顾性观察研究。2001年至2022年间,我们机构对51例患者进行了手术矫正和随访。31%的患者孤立地观察到肺动脉断开。最常见的形式是源自动脉导管的左肺动脉(58%)。在中位年龄12天时进行再次植入。71%的患者在随访期间观察到吻合口狭窄,其中75%的患者需要再次干预(占总人数的55%)。再植入后再干预的中位延迟时间为3.2年,超过25%的再植入肺动脉再干预发生在术后第一年内。我们发现,如果相关心脏缺陷,则会有更多的再次干预,没有显著的统计学差异。再次汇合时的体重、肺动脉起点处导管组织的存在以及之前在断开的肺动脉上进行分流均未被发现是再次干预的风险因素(图1)。结论/透视在肺动脉手术再次植入后,超过50%的患者因肺动脉狭窄需要再次干预。应寻求技术改进以减少这种并发症的发生率。
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引用次数: 0
Surfao-cardio study: Trans-abdominal fetal ECG analysis in the second trimester of pregnancy in a cohort of healthy fetuses 体表-心脏研究:一组健康胎儿妊娠中期经腹胎儿心电图分析
IF 18 Q4 Medicine Pub Date : 2023-09-01 DOI: 10.1016/j.acvdsp.2023.07.017
M. Lachaud , R. Souriau , B. Rivet , J. Fontecave

Introduction

Although feasible with ultrasound, analysis of the fetal heart rhythm sometimes remains a challenge in the absence of access to the fetal electrocardiogram. In particular in case of suspected long QT, ultrasound does not allow the measurement of the ST segment.

Objective

Within the framework of the ANR SurFAO project, the Surfao-Cardio clinical study consisted in including 20 patients whose fetus did not present any cardiac morphological abnormality, between 22SA and 29SA. A trans-abdominal electrical recording was performed. The abdominal signals were formatted and processed to extract a fetal ECG. From the latter, measurements were made, automatically for RR intervals and manually for PR intervals.

These data were compared for each patient with ultrasound measurements in different modalities (M mode, Mitral-Aorta Profile, VCS-Aorta Doppler, Pulmonary Vein-Pulmonary Artery Doppler).

Results/Expected results

The fetal heart rate was analyzable in 12 of the 20 patients with a possible analysis of the QRS morphology for 12/12 patients and of the PR for 8/12 patients.

The correlations between the ultrasound data and the fetal ECG were respectively R = 0.79 (n = 12, P < 0.05) for heart rate and R = 0.81 (n = 8, P < 0.05) for PR intervals (Figure 1).

Conclusion/Perspectives

This preliminary study showed the feasibility of trans abdominal fetal ECG with good reliability of PR measurement. Some material limitations related to the recording of the trans-abdominal ECG were identified, giving hope for an improvement in the percentage of possible measurements and access to ST-segment measurement.

虽然超声是可行的,但在没有获得胎儿心电图的情况下,胎儿心律分析有时仍然是一个挑战。特别是在怀疑QT过长时,超声不允许测量ST段。目的在ANR SurFAO项目框架下,SurFAO - cardio临床研究包括20例胎儿在22SA至29SA之间未出现任何心脏形态异常的患者。经腹部电记录。腹部信号被格式化并处理以提取胎儿心电图。从后者进行测量,自动测量RR间隔,手动测量PR间隔。这些数据比较了每个患者在不同模式下的超声测量(M模式,二尖瓣-主动脉剖面图,vcs -主动脉多普勒,肺静脉-肺动脉多普勒)。结果/预期结果20例患者中12例可分析胎儿心率,12/12例可分析QRS形态学,8/12例可分析PR。超声资料与胎儿心电图的相关性R = 0.79 (n = 12, P <0.05), R = 0.81 (n = 8, P <结论/观点本初步研究表明经腹胎儿心电图测量PR的可行性和良好的可靠性。一些与经腹心电图记录有关的材料限制被确定,为可能测量的百分比和st段测量的获得提供了希望。
{"title":"Surfao-cardio study: Trans-abdominal fetal ECG analysis in the second trimester of pregnancy in a cohort of healthy fetuses","authors":"M. Lachaud ,&nbsp;R. Souriau ,&nbsp;B. Rivet ,&nbsp;J. Fontecave","doi":"10.1016/j.acvdsp.2023.07.017","DOIUrl":"10.1016/j.acvdsp.2023.07.017","url":null,"abstract":"<div><h3>Introduction</h3><p><span>Although feasible with ultrasound, analysis of the fetal heart rhythm sometimes remains a challenge in the absence of access to the </span>fetal electrocardiogram. In particular in case of suspected long QT, ultrasound does not allow the measurement of the ST segment.</p></div><div><h3>Objective</h3><p>Within the framework of the ANR SurFAO project, the Surfao-Cardio clinical study consisted in including 20 patients whose fetus did not present any cardiac morphological abnormality, between 22SA and 29SA. A trans-abdominal electrical recording was performed. The abdominal signals were formatted and processed to extract a fetal ECG. From the latter, measurements were made, automatically for RR intervals and manually for PR intervals.</p><p>These data were compared for each patient with ultrasound measurements in different modalities (M mode, Mitral-Aorta Profile, VCS-Aorta Doppler, Pulmonary Vein-Pulmonary Artery Doppler).</p></div><div><h3>Results/Expected results</h3><p>The fetal heart rate was analyzable in 12 of the 20 patients with a possible analysis of the QRS morphology for 12/12 patients and of the PR for 8/12 patients.</p><p>The correlations between the ultrasound data and the fetal ECG were respectively R<!--> <!-->=<!--> <!-->0.79 (<em>n</em> <!-->=<!--> <!-->12, <em>P</em> <!-->&lt;<!--> <!-->0.05) for heart rate and R<!--> <!-->=<!--> <!-->0.81 (<em>n</em> <!-->=<!--> <!-->8, <em>P</em> <!-->&lt;<!--> <!-->0.05) for PR intervals (<span>Figure 1</span>).</p></div><div><h3>Conclusion/Perspectives</h3><p>This preliminary study showed the feasibility of trans abdominal fetal ECG with good reliability of PR measurement. Some material limitations related to the recording of the trans-abdominal ECG were identified, giving hope for an improvement in the percentage of possible measurements and access to ST-segment measurement.</p></div>","PeriodicalId":8140,"journal":{"name":"Archives of Cardiovascular Diseases Supplements","volume":null,"pages":null},"PeriodicalIF":18.0,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45931529","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Cardiac magnetic resonance parameters to predict ventricular arrhythmias inducibility before pulmonary valve replacement in patient with tetralogy of Fallot 心脏磁共振参数预测法洛四联症患者肺动脉瓣置换术前室性心律失常的诱发性
IF 18 Q4 Medicine Pub Date : 2023-09-01 DOI: 10.1016/j.acvdsp.2023.07.006
MD Grégoire Albenque , PhD, MD Francis Bessière , PhD, MD Gilles Soulat , MD Arshid Azarine , MD Mohamed Bakloul , MD Emre Belli , PhD, MD Damien Bonnet , PhD, MD Loic Boussel , MD Eric Bruguière , PhD, MD Sarah Cohen , MD Hubert Delasnerie , MD Sylvie Di Filippo , MD Arnaud Dulac , MD Kevin Gardey , MD Roland Henaine , MD Laurence Iserin , PhD, MD Clément Karsenty , PhD, MD Magalie Ladouceur , MD Antoine Legendre , MD Sophie Malekzadeh-Milani , PhD, MD Victor Waldmann

Introduction

Ventricular arrhythmias and sudden death are recognized complications of tetralogy of Fallot (TOF). Electrophysiological study (EPS) is increasingly performed prior to pulmonary valve replacement (PVR) in these patients to evaluate and treat the arrhythmic substrate.

Objective

We aimed to assess the value of cardiac magnetic resonance (CMR) to refine pre-operative risk stratification in this population.

Methods

A prospective multicenter study was conducted to systematically assess EPS in patients with TOF referred for PVR from January 2020 to December 2021. Multiple CMR parameters were analyzed and correlated with EPS results.

Results/Expected results

Among a total of 120 patients enrolled, 108 (90.0%) patients who had a CMR prior to EPS were analyzed. A VA was inducible in 24 (22.2%) patients. Clinical characteristics of patients associated with EPS results are presented in Table 1. Concerning CMR variables, mean RV end-diastolic volume index was 157 ± 39 ml and was not statistically different in patients with positive vs. negative EPS (164 ± 49 vs. 155 ± 35, P = 0.395). However, a RV/LV end-diastolic volume index ratio > 2.4 was significantly associated with a higher likelihood of inducibility (OR 3.10, 95%CI 1.14–8.74, P = 0.040). Mean RV end-systolic volume index tended to be upper in patient with positive EPS (101 ± 35 vs. 86 ± 26, P = 0.072). The RV ejection fraction measured on CMR also tended to be slightly lower in patients with positive EPS (40 ± 7 vs. 43 ± 9, P = 0.069) unlike the LV ejection fraction (P = 0.600). Mean pulmonary annulus diameter were also significantly associated with positive EPS (31 ± 8 vs. 26 ± 7 mm, OR 1.10 [1.02–1.18] per 1 mm increment, P = 0.012). A pulmonary annulus diameter > 28 mm was associated with an OR of 2.9 [1.10–8.11] (P = 0.047). In multivariable analysis, RV/LV end-diastolic volume index ratio > 2.4 (OR 4.6 [1.2–22.5], P = 0.036) and history of atrial arrhythmia (OR 10.9 [2.6–60.8], P = 0.002) remained independent predictors of VA inducibility (Figure 1).

Conclusion/Perspectives

In patients with TOF prior to PVR, RV/LV end-diastolic volume index ratio and pulmonary annulus diameter are associated with ventricular arrhythmia inducibility during EPS. CMR parameters, in combination with other clinical factors, may be us

室性心律失常和猝死是法洛四联症(TOF)公认的并发症。越来越多的患者在肺瓣膜置换术(PVR)前进行电生理研究(EPS),以评估和治疗心律失常的基底。目的评估心脏磁共振(CMR)技术在该人群术前风险分层中的应用价值。方法从2020年1月至2021年12月,进行一项前瞻性多中心研究,系统评估TOF患者的EPS。分析了多个CMR参数与EPS结果的相关性。结果/预期结果在总共120例入组患者中,分析了108例(90.0%)EPS前有CMR的患者。24例(22.2%)患者可诱导VA。EPS结果相关患者的临床特征见表1。在CMR变量方面,EPS阳性与阴性患者的平均左室舒张末期容积指数为157±39 ml,差异无统计学意义(164±49 vs 155±35,P = 0.395)。然而,右室/左室舒张末期容积指数比>2.4与较高的诱导可能性显著相关(OR 3.10, 95%CI 1.14-8.74, P = 0.040)。EPS阳性患者右心室收缩末期平均容积指数较高(101±35∶86±26,P = 0.072)。与左室射血分数(P = 0.600)不同,EPS阳性患者CMR测量的右室射血分数也略低(40±7比43±9,P = 0.069)。平均肺环直径也与EPS阳性显著相关(31±8 vs. 26±7 mm, OR为1.10 [1.02-1.18]/ 1 mm, P = 0.012)。A肺环直径;28 mm与OR为2.9[1.10-8.11]相关(P = 0.047)。在多变量分析中,左室/左室舒张末期容积指数比>2.4 (OR 4.6 [1.2-22.5], P = 0.036)和心房心律失常史(OR 10.9 [2.6-60.8], P = 0.002)仍然是室性心律失常诱发性的独立预测因子(图1)。结论/观点在PVR前TOF患者中,左室/左室舒张末期容积指数比和肺环直径与EPS期间室性心律失常的诱发性相关。CMR参数,结合其他临床因素,可能有助于改善术前风险分层。
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引用次数: 0
Adult interrupted aortic arch revealed by resistant arterial hypertension 成人主动脉弓中断表现为顽固性高血压
IF 18 Q4 Medicine Pub Date : 2023-09-01 DOI: 10.1016/j.acvdsp.2023.07.048
A. Boutaled , M. Tabat , Y. Mekouar , G. Bennani , A. Drighil , G. Lembarki

Introduction

Interrupted aortic arch (IAA) is a rare congenital heart condition where there is a complete discontinuation between the ascending and descending aorta. The association with a patent ductus arteriosus or developed arterial collateral allows survival until adulthood in extremely rare cases. Transthoracic echocardiography is helpful for the diagnosis, but the exact interruption site and collateral vessels are more accurately highlighted by computed tomography angiography (CTA) and magnetic resonance imaging (MRI).

Results/Expected results

A 50 year-old-patient with a history of hypertension was admitted for severe high blood pressure. The main functional symptom that prompted his consultation was a grade II NYHA dyspnea evolving for few weeks. Physical examination found a blood pressure of 190/110 mmHg at the right arm, 120/70 mmHg at the left arm, and 110/70 mmHg at lower limbs. Cardiac and pulmonary auscultation were normal. The electrocardiogram showed left ventricular hypertrophy. Transthoracic echocardiography revealed severe left ventricular hypertrophy with good left ventricular function and dilatation of the ascending aorta measured at 47 mm. The suprasternal notch view suggested a type B IAA. CTA confirmed the diagnosis, exhibiting an IAA located between the left carotid and left subclavian artery with an arterial collateral network emerging from descending branch of the transverse cervical artery (Figure 1).

Conclusion/Perspectives

Adult forms of IAA are a rare and complex manifestation of a congenital heart condition. They present significant challenges due to the severity of symptoms and high operative risk involved in treatment. An accurate and timely diagnosis is essential and can be achieved through a combination of echocardiography and advanced imaging techniques such as CTA or MRI angiography. A multidisciplinary approach involving a team of specialists is crucial for optimizing the management of these patients.

主动脉弓中断(IAA)是一种罕见的先天性心脏病,在升主动脉和降主动脉之间存在完全中断。合并动脉导管未闭或动脉侧枝发育的病例极少存活至成年。经胸超声心动图有助于诊断,但计算机断层血管造影(CTA)和磁共振成像(MRI)更准确地显示确切的中断部位和侧支血管。结果/预期结果患者50岁,有高血压病史,因严重高血压入院。促使他就诊的主要功能症状是持续数周的II级NYHA呼吸困难。体检发现右臂血压190/110 mmHg,左臂血压120/70 mmHg,下肢血压110/70 mmHg。心肺听诊正常。心电图显示左室肥厚。经胸超声心动图显示左心室严重肥厚,左心室功能良好,升主动脉扩张47mm。胸骨上切迹显示为B型IAA。CTA证实了诊断,显示IAA位于左颈动脉和左锁骨下动脉之间,颈横动脉降支出现动脉侧支网络(图1)。结论/观点成人形式的IAA是先天性心脏病罕见且复杂的表现。由于症状的严重性和治疗的高手术风险,它们提出了重大的挑战。准确和及时的诊断是必不可少的,可以通过超声心动图和先进的成像技术如CTA或MRI血管造影相结合来实现。涉及专家团队的多学科方法对于优化这些患者的管理至关重要。
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引用次数: 0
Risk of infective endocarditis after hybrid Melody mitral valve replacement in infants: The French experience 婴儿混合型Melody二尖瓣置换术后感染性心内膜炎的风险:法国经验
IF 18 Q4 Medicine Pub Date : 2023-09-01 DOI: 10.1016/j.acvdsp.2023.07.021
P. Padovani , V. Fouilloux , J.B. Thambo , M. Ly , P. Aldebert , O. Baron , F. Roubertie , N. Benbrik , C. Ovaert , Z. Jalal , A.E. Baruteau

Introduction

Surgical management of mitral valve (MV) disease is challenging in infants. We aimed at reporting the French experience with Melody mitral valve replacement (MVR).

Methods

A retrospective cohort study reporting the French experience with Melody MV replacement (MVR).

Results

Seven symptomatic infants (complete atrioventricular septal defect [n = 4, down syndrome: n = 3], hammock MV [n = 3]) underwent Melody MVR (age: 3 months [28 days–8 months], weight: 4.3 kg [3.2–6.4 kg]) because of severe MV regurgitation (6) or mixed MV disease (1) and 14 mm (11–16 mm) MV annulus. In 2 patients whose MV was felt irreparable, Melody MVR was performed straightaway. The others underwent 2 (1–3) previous attempts of MV repair; 3 were on ECMO. Melody MVR led to competent valve and low gradient (3 mmHg, [1–4 mmHg]). One patient died 3 days post-implant from ECMO-related stroke. Of the 6 discharged home patients, 3 (50%) were readmitted for a definite diagnosis (1) or high suspicion (2) of infective endocarditis, of which 2 died. Over the follow-up, one underwent balloon expansions of the valve at 9- and 16-months post-implant, and mechanical MVR at 2 years; another is currently planned for transcatheter Melody valve dilation.

Conclusion

Melody MVR may be considered in selected infants with small MV annulus as an alternative to mechanical MVR. Our experience highlights a high-risk of late IE that deserves further consideration.

婴儿二尖瓣(MV)疾病的外科治疗具有挑战性。我们的目的是报道法国梅洛迪二尖瓣置换术(MVR)的经验。方法回顾性队列研究,报道法国梅洛迪置换术(MVR)的经验。结果7例有症状的婴儿(完全性房室间隔缺损[n = 4,唐氏综合征[n = 3],吊床MV [n = 3])因重度中压返流(6)或混合性中压病变(1)及14 mm (11 ~ 16 mm)的中压环而行Melody MVR(年龄:3个月[28天~ 8个月],体重:4.3 kg [3.2 ~ 6.4 kg])。2例患者感觉MV无法修复,立即行Melody MVR。其余患者接受2(1-3)次中压修复尝试;3例进行体外膜肺氧合。旋律MVR导致阀值正常且梯度低(3 mmHg, [1-4 mmHg])。1例患者在植入后3天死于ecmo相关中风。出院的6例患者中,3例(50%)因确诊(1)或高度怀疑(2)感染性心内膜炎再次入院,其中2例死亡。在随访中,1例患者在植入后9个月和16个月行瓣膜球囊扩张术,2年后行机械MVR;另一个目前计划用于经导管梅洛迪瓣膜扩张。结论对于有选择的中压环小的婴儿,可以考虑采用旋律MVR作为机械MVR的替代方案。我们的经验突出了晚期IE的高风险,值得进一步考虑。
{"title":"Risk of infective endocarditis after hybrid Melody mitral valve replacement in infants: The French experience","authors":"P. Padovani ,&nbsp;V. Fouilloux ,&nbsp;J.B. Thambo ,&nbsp;M. Ly ,&nbsp;P. Aldebert ,&nbsp;O. Baron ,&nbsp;F. Roubertie ,&nbsp;N. Benbrik ,&nbsp;C. Ovaert ,&nbsp;Z. Jalal ,&nbsp;A.E. Baruteau","doi":"10.1016/j.acvdsp.2023.07.021","DOIUrl":"10.1016/j.acvdsp.2023.07.021","url":null,"abstract":"<div><h3>Introduction</h3><p>Surgical management of mitral valve (MV) disease is challenging in infants. We aimed at reporting the French experience with Melody mitral valve replacement (MVR).</p></div><div><h3>Methods</h3><p><span>A retrospective cohort study reporting the French experience with Melody </span>MV replacement (MVR).</p></div><div><h3>Results</h3><p><span>Seven symptomatic infants (complete atrioventricular septal defect [</span><em>n</em> <!-->=<!--> <!-->4, down syndrome: <em>n</em> <!-->=<!--> <!-->3], hammock MV [<em>n</em> <!-->=<!--> <!-->3]) underwent Melody MVR (age: 3 months [28 days–8 months], weight: 4.3<!--> <!-->kg [3.2–6.4<!--> <span>kg]) because of severe MV regurgitation (6) or mixed MV disease (1) and 14</span> <!-->mm (11–16<!--> <span>mm) MV annulus. In 2 patients whose MV was felt irreparable, Melody MVR was performed straightaway. The others underwent 2 (1–3) previous attempts of MV repair; 3 were on ECMO. Melody MVR led to competent valve and low gradient (3</span> <!-->mmHg, [1–4<!--> <!-->mmHg]). One patient died 3 days post-implant from ECMO-related stroke. Of the 6 discharged home patients, 3 (50%) were readmitted for a definite diagnosis (1) or high suspicion (2) of infective endocarditis, of which 2 died. Over the follow-up, one underwent balloon expansions of the valve at 9- and 16-months post-implant, and mechanical MVR at 2 years; another is currently planned for transcatheter Melody valve dilation.</p></div><div><h3>Conclusion</h3><p>Melody MVR may be considered in selected infants with small MV annulus as an alternative to mechanical MVR. Our experience highlights a high-risk of late IE that deserves further consideration.</p></div>","PeriodicalId":8140,"journal":{"name":"Archives of Cardiovascular Diseases Supplements","volume":null,"pages":null},"PeriodicalIF":18.0,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46441178","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Index 指数
IF 18 Q4 Medicine Pub Date : 2023-09-01 DOI: 10.1016/S1878-6480(23)00285-9
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引用次数: 0
BeGraft aortic stents: A European multi-centre experience reporting acute safety and efficacy outcomes for the treatment of vessel stenosis in congenital heart diseases BeGraft aortic stent:一项欧洲多中心经验报告了先天性心脏病血管狭窄治疗的急性安全性和有效性
IF 18 Q4 Medicine Pub Date : 2023-09-01 DOI: 10.1016/j.acvdsp.2023.07.024
G. Butera , M. Remonato , M. Pilati , M. Jones , M. Carminati , S. Hascoet , L. Giugno , P. Betrian , A.E. Baruteau , E. Pascall , H. Lucron , G. Milani

Introduction

Stent implantation for the treatment of vessel stenosis in congenital heart diseases has become the preferred method of treatment. Availability of new covered stents may decrease complications and may have an important role in the management of patients with complex anatomy.

Objective

The aim of this study was to evaluate the feasibility and safety of the pre-mounted cobalt-chromium stent-graft covered ePTFE Aortic BeGraft in a broad spectrum of congenital vascular lesions.

Methods

A retrospective study of 112 implanted Be Graft Aortic stents between 2016 and 2022 in six different European centers was performed.

Results/Expected results

One hundred four patients with mean age 13 years (range 1–70 years; 56.5 kg, range 11–115 kg) underwent BeGraft stent implantation. Seventy three patients had aortic coarctation, seven BeGraft stents were implanted in patients with Fontan circulation for either restoration of Fontan conduit patency in three patients and fenestration closure in four). Fourteen BeGraft aortic stents were implanted in 12 patients with dysfunctional RVOT. Eleven patients with complex CHD had miscellaneous indications for stent implantation: superior vena cava stenosis in 3 patients, rehabilitation of percutaneous Potts shunt in two patients, exclusion of a porto-caval fistula in one patient, relief of pulmonary arteries stenosis in 3 patients and percutaneous PA debanding in two patients with ccTGA. All the stents were implanted successfully. Median stent diameter was 16 mm (range 7–24 mm). Major complications were the following: one aortic dissection, 2 stent balloon rupture, 1 patient experienced a cerebral embolization without neurological consequences and 1 patient had a femoral artery occlusion requiring vascular surgery.

Conclusion/Perspectives

BeGraft stent can be used safely and effectively in a wide spectrum of congenital heart diseases. Whether these good results will be stable in the long term follow up still needs to be investigated given its recent introduction into clinical practice.

导读支架植入术治疗先天性心脏病血管狭窄已成为首选的治疗方法。新的覆盖支架的可用性可能会减少并发症,并可能在复杂解剖结构患者的管理中发挥重要作用。目的评估预安装钴铬支架覆盖ePTFE主动脉瓣在广谱先天性血管病变中的可行性和安全性。方法回顾性分析2016年至2022年在欧洲6个不同中心植入的112例Be瓣主动脉支架。结果/预期结果104例患者,平均年龄13岁(范围1-70岁;56.5公斤,范围11-115公斤)接受了BeGraft支架植入术。73例患者主动脉缩窄,其中3例为恢复Fontan导管通畅,4例为关闭开窗,7例为Fontan循环患者植入BeGraft支架。在12例RVOT功能不全患者中植入14个BeGraft主动脉支架。11例复杂冠心病患者支架植入术指征繁杂:3例上腔静脉狭窄,2例经皮Potts分流康复,1例门静脉瘘排除,3例肺动脉狭窄缓解,2例ccTGA经皮PA去带。所有支架均成功植入。支架中位直径为16mm(范围7 - 24mm)。主要并发症如下:1例主动脉夹层,2例支架球囊破裂,1例无神经系统后果的脑栓塞,1例需要血管手术的股动脉闭塞。结论/观点begraft支架可以安全有效地用于广泛的先天性心脏病。这些良好的结果是否会在长期随访中稳定仍需要调查,因为它最近被引入临床实践。
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引用次数: 0
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Archives of Cardiovascular Diseases Supplements
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