Congenital heart defect

先天性心脏病
  • 文章类型: Journal Article
    房间隔缺损(ASDs)导致右心室容积负荷。ASD闭合并不总是缓解症状或提高运动能力,这可能是由左心室(LV)血流动力学受损解释的。这项研究使用来自心脏磁共振(CMR)成像和肱动脉血压的无创LV压力-容积(PV)环路评估了儿童ASD闭合的效果。与对照组相比。
    23名ASD儿童接受了CMR,其中17例在ASD关闭后7(6-9)个月进行了重新检查。包括12个对照。根据时间分辨的LV体积和肱动脉血压,从PV回路中得出血液动力学变量。ASD关闭后,左心室容量增加[76(70-86)与63(57-70)mL/m2,P=0.0001];它仍然小于对照组[76(70-86)vs.82(78-89)mL/m2,P=0.048]。与对照组相比,患有ASD的儿童有更高的收缩力[2.6(2.1-3.3)与1.7(1.5-2.2)mmHg/mL,P=0.0076]和动脉弹性[2.1(1.4-3.1)vs.1.4(1.2-2.0)mmHg/mL,P=0.034]。ASD关闭后,两者的收缩性[2.0(1.4-2.5)mmHg/mL,P=0.0001]和动脉弹性[1.4(1.3-2.0)mmHg/mL,P=0.0002]减少。
    尽管左心房分流导致低左心室充盈和右心室增大,LV仍然有效,没有证据表明儿童LV血流动力学受损.因此,在年轻时闭合ASD,同时心室是顺应性的,对于LV功能是有益的。LV体积,然而,ASD关闭后保持较小,这可能会影响长期心血管风险和运动表现。
    UNASSIGNED: Atrial septal defects (ASDs) lead to volume-loaded right ventricles (RVs). ASD closure does not always alleviate symptoms or improve exercise capacity, which is possibly explained by impaired left ventricular (LV) haemodynamics. This study evaluated the effect of ASD closure in children using non-invasive LV pressure-volume (PV) loops derived from cardiac magnetic resonance (CMR) imaging and brachial blood pressure, compared with controls.
    UNASSIGNED: Twenty-three children with ASD underwent CMR, and 17 of them were re-examined 7 (6-9) months after ASD closure. Twelve controls were included. Haemodynamic variables were derived from PV loops by time-resolved LV volumes and brachial blood pressure. After ASD closure, LV volume increased [76 (70-86) vs. 63 (57-70) mL/m2, P = 0.0001]; however, it was still smaller than in controls [76 (70-86) vs. 82 (78-89) mL/m2, P = 0.048]. Compared with controls, children with ASD had higher contractility [2.6 (2.1-3.3) vs. 1.7 (1.5-2.2) mmHg/mL, P = 0.0076] and arterial elastance [2.1 (1.4-3.1) vs. 1.4 (1.2-2.0) mmHg/mL, P = 0.034]. After ASD closure, both contractility [2.0 (1.4-2.5) mmHg/mL, P = 0.0001] and arterial elastance [1.4 (1.3-2.0) mmHg/mL, P = 0.0002] decreased.
    UNASSIGNED: Despite the left-to-right atrial shunt that leads to low LV filling and RV enlargement, the LV remains efficient and there is no evidence of impaired LV haemodynamics in children. Closure of ASD at young age while the ventricle is compliant is thus beneficial for LV function. LV volumes, however, remain small after ASD closure, which may impact long-term cardiovascular risk and exercise performance.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    背景:经皮肺动脉瓣植入术(PPVI)是右心室流出道功能失调的患者手术的公认替代治疗方法。患者的选择对于避免尝试治疗的严重并发症至关重要。如破裂或解剖,尤其是钙化的流出道。我们描述了一个意外的钙化同种移植瓣膜和主肺动脉破裂的病例,通过紧急植入自我膨胀的金星P-瓣膜成功治疗(金星医疗技术,杭州,中国)无需使用覆膜支架进行预支架置入。
    方法:一个13岁的男孩之前做过两次法洛四联症手术,一个是完全修复,另一个是用于肺返流的同种移植瓣膜导管。他表现为呼吸困难和严重的右室流出道梗阻(RVOTO),并有钙化的流出道和主肺动脉。在导管实验室,非顺应性球囊扩张导致导管破裂。患者保持血流动力学稳定,破裂用自膨式VenusP-Valve治疗,不需要覆膜支架联合球囊扩张瓣膜或进一步的外科手术.
    结论:使用充气球囊进行术前评估对于检查组织顺应性和确定PPVI的适用性是必要的。然而,这种情况伴随着导管破裂的风险。这种并发症的危险因素是钙化和同种移植的使用。这些破裂主要通过预防性或治疗性覆膜支架控制,需要手术的比例很低。然而,有严重的破裂导致血胸和死亡。在现有文献中,没有类似的导管破裂报告病例,自膨式肺动脉瓣支架已经管理好了。心脏周围的纤维化和胶原组织,在开放手术后形成的,有助于控制这些病例的出血。
    应更仔细地检查患者对PPVI手术的适用性,特别是患者的同种移植和钙化在他们的管道。此外,导管破裂可能是可控的自膨式人工肺动脉瓣,特别是在以前手术的病人中,这一假设的适用性值得在未来的研究中研究。
    BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) is a recognized alternative treatment to surgery for patients with dysfunctional right ventricular outflow tracts. Patient selection is essential to avoid serious complications from attempted treatment, such as rupture or dissection, especially of the calcified outflow tracts. We describe a case with an unexpected rupture of a calcified homograft valve and main pulmonary artery, which was treated successfully by emergency implantation of a self-expanding Venus P-Valve (Venus MedTech, Hangzhou, China) without the need for pre-stenting with a covered stent.
    METHODS: A 13-year-old boy had two previous operations of tetralogy of Fallot, one a total repair and the other a homograft valved conduit for pulmonary regurgitation. He presented with dyspnea and severe right ventricular outflow tract obstruction (RVOTO) and had a calcified outflow tract and main pulmonary artery. In the catheter laboratory, a non-compliant balloon dilation resulted in a contained rupture of the conduit. The patient remained hemodynamically stable, and the rupture was treated with a self-expandable Venus P-Valve without the need for a covered stent combined with a balloon-expandable valve or a further surgical procedure.
    CONCLUSIONS: Preprocedural evaluation with an inflating balloon is necessary to examine tissue compliance and determine suitability for PPVI. However, this condition is accompanied by a risk of conduit rupture. Risk factors of this complication are calcification and homograft use. These ruptures are mostly controlled with a prophylactic or therapeutic covered stent, with a low rate of requiring surgery. However, there are severe ruptures which lead to hemothorax and death. In the available literature, there was no similar reported case of conduit rupture, which a self-expandable Pulmonary valve stent has managed. It seems that fibrosis and collagen tissue around the heart, formed after open surgeries, can contribute to the control of bleeding in these cases.
    UNASSIGNED: The suitability of patients for the PPVI procedure should be examined more carefully, specifically patients with homograft and calcification in their conduit. Furthermore, conduit rupture might be manageable with self-expandable artificial pulmonary valves, specifically in previously operated patients, and the applicability of this hypothesis is worth examining in future research.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    右肺动脉异常起源于升主动脉是一种罕见的先天性心脏畸形,可导致婴儿早期死亡。这些患者有早期发展为显著肺动脉高压的风险。生命早期的手术管理势在必行。
    起源于升主动脉的异常肺动脉(通常称为半心)是一种罕见的先天性心脏缺陷,需要在新生儿期立即处理。我们报告了一例罕见的起源于升主动脉的右肺动脉异常变异,并伴有肺动脉闭锁和法洛四联症。上述组合使得这种心脏缺陷的外科治疗要求极高。
    UNASSIGNED: Anomalous origin of right pulmonary artery from the ascending aorta is a rare congenital heart malformation that results in early infant mortality. These patients are at risk for the early development of significant pulmonary hypertension. The surgical management during the early period of life is imperative.
    UNASSIGNED: Anomalous pulmonary artery originating from the ascending aorta (often called hemitruncus) is a rare congenital cardiac defect requiring immediate management in the neonatal period. We report a case of a rare variant of anomalous right pulmonary artery originating from the ascending aorta in combination with pulmonary atresia and tetralogy of Fallot. The above-mentioned combination makes the surgical management of such cardiac defect exceedingly demanding.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    冠状动脉阻塞是经导管主动脉瓣置换术(TAVR)的一种罕见但破坏性的并发症,可能需要修改小叶。一名38岁的男子出现在我们中心,患有四瓣主动脉瓣并伴有严重的主动脉瓣反流。在TAVR之前,用生物假体或天然主动脉扇贝有意裂伤进行双小叶修饰,以防止医源性冠状动脉阻塞(BASILICA)技术,从4创建6小叶。患者对手术耐受良好,症状改善。随访经胸超声心动图显示生物主动脉瓣功能正常。该病例通过全面的术前分析和术中成像指导证明了该程序的可行性。
    Coronary artery obstruction is an uncommon yet devastating complication of transcatheter aortic valve replacement (TAVR) and may necessitate leaflet modification. A 38-year-old man presented to our center with quadricuspid aortic valve with severe aortic regurgitation. Double leaflet modification was performed with the Bioprosthetic or native Aortic Scallop Intentional Laceration to prevent Iatrogenic Coronary Artery obstruction (BASILICA) technique prior to TAVR, creating 6 leaflets from 4. The patient tolerated the procedure well with symptomatic improvement. Follow-up transthoracic echocardiogram showed normal bioprosthetic aortic valve function. This case demonstrates feasibility of this procedure with comprehensive preprocedural analysis and intraprocedural imaging guidance.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    暂无摘要。
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    暂无摘要。
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    部分肺静脉回流异常的双重连接是一种非常罕见的先天性异常,其中至少有一个肺静脉,但不是全部,引流到左心房和全身静脉循环,随后从左到右分流。
    Double connection of partial anomalous pulmonary venous return is a very rare congenital anomaly where at least one pulmonary vein, but not all, drains into the left atrium and systemic venous circulation with subsequent left to right shunt.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    多达一半的先天性心脏病(CHD)成人经历心理困扰,包括焦虑。
    本文旨在:1)评估疾病感知在解释冠心病成年人的社会人口统计学和医学变量以外的焦虑症状中的贡献;2)研究应对方式的潜在中介作用。
    在2019年6月至2021年4月期间在蒙特利尔心脏研究所招募CHD成年患者进行这项横断面研究。参与者对自我报告问卷(医院焦虑和抑郁量表,简短的疾病感知问卷,和简短的COPE)。医学特征(冠心病复杂性,NYHA功能类,和心脏设备)是从医疗记录中收集的。我们进行了分层多元线性回归和中介分析。
    在223名参与者中(平均年龄46±14岁,59%的妇女),15%有临床显著的焦虑症状。医学和社会人口统计学变量解释了焦虑症状变化的15%。增加疾病感知解释了焦虑变化的另外18%。该R2变化是显著的(F[1,188]=49.06,P<0.0001)。与医学和社会人口统计学变量的总和相比,疾病感知解释了更多的方差(18%)。对疾病更具威胁性的感知与更大的焦虑症状相关(β=0.45,P<0.0001)。此外,疾病感知与应对有关,这与减少焦虑症状有关。应对方式占疾病感知对焦虑总影响的20%。
    疾病感知和应对与成人冠心病患者的焦虑有关。未来的措施应该评估是否针对这些潜在的可改变的因素有效地预防或减轻成人冠心病患者的焦虑症状。
    UNASSIGNED: Up to one-half of adults with congenital heart disease (CHD) experience psychological distress, including anxiety.
    UNASSIGNED: This paper sought to: 1) assess the contribution of illness perception in explaining anxiety symptoms beyond sociodemographic and medical variables in adults with CHD; and 2) investigate the potential mediating effect of coping style.
    UNASSIGNED: CHD adult patients were recruited at Montreal Heart Institute between June 2019 and April 2021 for this cross-sectional study. Participants responded to self-reported questionnaires (Hospital Anxiety and Depression Scale, Brief Illness Perception Questionnaire, and Brief COPE). Medical characteristics (CHD complexity, NYHA functional class, and cardiac devices) were collected from medical records. We conducted hierarchical multiple linear regression and mediation analyses.
    UNASSIGNED: Of the 223 participants (mean age 46 ± 14 years, 59% women), 15% had clinically significant anxiety symptoms. Medical and sociodemographic variables explained 15% of the variation in anxiety symptoms. Adding illness perception explained an additional 18% of the variation in anxiety. This R2 change was significant (F[1,188] = 49.06, P < 0.0001). Illness perception explained more variance (18%) than medical and sociodemographic variables combined. A more threatening perception of illness was associated with greater anxiety symptoms (β = 0.45, P < 0.0001). Furthermore, illness perception was associated with coping, which was linked to reduced anxiety symptoms. Coping response style accounted for 20% of the total effect of illness perception on anxiety.
    UNASSIGNED: Illness perception and coping are associated with anxiety in adults with CHD. Future initiatives should assess whether targeting these potentially modifiable factors effectively prevents or mitigates anxious symptoms in adults with CHD.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    关于天然或复发性非严重主动脉缩窄(CoA)的成人解剖和生理参数与运动能力的相互作用的数据有限。
    这项研究的目的是使用运动负荷超声心动图和心脏磁共振成像来确定这些患者运动能力的解剖和生理相关性。
    我们在2015年至2020年期间对我们机构的54名非重度CoA(修复或未修复)成年人进行了单中心回顾性分析。使用超声心动图获得静息缩窄梯度。使用运动负荷超声心动图评估运动梯度和功能能力。使用磁共振成像获得主动脉解剖结构。
    缩窄与隔膜比与运动分钟数(r=0.56,P<0.01)和代谢当量(r=0.49,P<0.01)相关。在控制使用β受体阻滞剂后,这些关系仍然显着,瓣膜疾病,和缩窄修复的类型。运动时间与平均静息梯度相关(r=-0.39,P<0.05)。缩窄与隔膜比与峰值和平均静息梯度相关(r=-0.34,P<0.05;r=-0.48,P<0.01)。缩窄与隔膜比≤0.7的患者的代谢当量较少(11.1±1.9vs12.8±2.2,P<0.05),运动分钟数(10.3±2.0vs12.6±2.7,P<0.05)。
    在非严重的天然或复发性CoA患者中,通过解剖大小和梯度,运动能力降低与缩窄严重程度相关。缩窄与隔膜比≤0.7的患者可能代表非严重CoA患者的一部分,其临床症状仅由运动压力测试引起。运动压力测试和横断面成像可能有助于识别那些可以考虑进行早期缩窄干预的人。
    UNASSIGNED: There is limited data regarding the interplay of anatomic and physiologic parameters with exercise capacity in adults with native or recurrent nonsevere coarctation of the aorta (CoA).
    UNASSIGNED: The objective of this study was to use exercise stress echocardiography and cardiac magnetic resonance imaging to identify anatomic and physiologic correlates of exercise capacity in these patients.
    UNASSIGNED: We conducted a single-center retrospective analysis of 54 adults with nonsevere CoA (repaired or unrepaired) followed at our institution between 2015 and 2020. Resting coarctation gradients were obtained using echocardiography. Exercise gradients and functional capacity were assessed using exercise stress echocardiography. Aorta anatomy was obtained using magnetic resonance imaging.
    UNASSIGNED: Coarctation-to-diaphragm ratio correlated with minutes of exercise (r = 0.56, P < 0.01) and metabolic equivalents (r = 0.49, P < 0.01). These relationships remained significant after controlling for use of beta-blockers, valvular disease, and type of coarctation repair. Minutes of exercise correlated with mean resting gradients (r = -0.39, P < 0.05). Coarctation-to-diaphragm ratio correlated with peak and mean resting gradients (r = -0.34, P < 0.05; r = -0.48, P < 0.01). Patients with coarctation-to-diaphragm ratio ≤0.7 achieved fewer metabolic equivalents (11.1 ± 1.9 vs 12.8 ± 2.2, P < 0.05) and minutes of exercise (10.3 ± 2.0 vs 12.6 ± 2.7, P < 0.05).
    UNASSIGNED: In patients with nonsevere native or recurrent CoA, reduced exercise capacity is correlated with coarctation severity by anatomic size and gradients. Those with a coarctation-to-diaphragm ratio ≤0.7 may represent a subset of patients with nonsevere CoA whose clinical symptoms are only elicited with exercise stress testing. Exercise stress testing and cross-sectional imaging may help identify those who could be considered for earlier coarctation intervention.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    来自肺动脉的异常右冠状动脉(ARCAPA)是一种罕见的先天性异常,没有更常见的异常左冠状动脉的典型表现。我们介绍了一名患有法洛四联症的婴儿,在术前超声心动图上有非典型发现。心脏计算机断层扫描(CT)扫描显示ARCAPA。术中证实并成功修复。在存在其他先天性心脏缺陷的情况下,密切关注超声心动图的冠状动脉和低阈值的附加成像可以成功诊断ARCAPA。
    An anomalous right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital anomaly that does not have the typical presentation of the more common anomalous left coronary artery. We present an infant with tetralogy of Fallot with atypical findings on the preoperative echocardiogram. A cardiac computerized tomographic (CT) scan showed ARCAPA. This was confirmed intraoperatively and repaired successfully. Close attention to coronaries on echocardiography and a low threshold for additional imaging can successfully diagnose ARCAPA in the presence of additional congenital heart defects.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

公众号