Adult congenital heart disease

成人先天性心脏病
  • 文章类型: Journal Article
    背景:关于肺动脉高压(PH)患者的麻醉方法选择尚无共识。我们报告了2例严重PH患者在开腹手术中安全进行神经轴麻醉而无需全身麻醉的情况。
    方法:案例1:一名59岁女性患有房间隔缺损和巨大的腹部肿瘤,平均肺动脉压(PAP)为39mmHg,肺血管阻力(PVR)为3.5木质单位。案例2:一名23岁的女性,患有遗传性肺动脉高压(平均PAP,65mmHg;PVR,16.45木材单位)。两名患者均在神经轴麻醉下进行了开腹手术,无循环塌陷,术中给予血管收缩剂。
    结论:尽管麻醉护理必须根据PH的病理和严重程度进行个性化,对于接受腹部手术的重度PH患者,神经轴麻醉可能是一种选择。
    BACKGROUND: There is no consensus regarding the choice of anesthetic method for patients with pulmonary hypertension (PH). We report two cases in which neuraxial anesthesia was safely performed without general anesthesia during open abdominal surgery in patients with severe PH.
    METHODS: Case 1: A 59-year-old woman had an atrial septal defect and a huge abdominal tumor with a mean pulmonary arterial pressure (PAP) of 39 mmHg and pulmonary vascular resistance (PVR) of 3.5 Wood units. Case 2: A 23-year-old woman who had hereditary pulmonary artery hypertension (mean PAP, 65 mmHg; PVR, 16.45 Wood units). Both patients underwent open abdominal surgery under neuraxial anesthesia without circulatory collapse with intraoperative administration of vasoconstrictors.
    CONCLUSIONS: Although anesthetic care must be personalized depending on the pathology and severity of PH, neuraxial anesthesia may be an option for patients with severe PH undergoing abdominal surgery.
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  • 文章类型: Journal Article
    目的:提供有关预防的最新概述,诊断,成人先天性心脏病患者感染性心内膜炎的治疗。
    结果:对感染性心内膜炎患者的护理在专业团队领域正在发生变化,诊断,和预防。心内膜炎团队应参与ACHD患者的护理。2023年Duke感染性心内膜炎标准和2023年欧洲心脏病学会指南更新了诊断标准,包括新的主要标准,例如CT和正电子发射计算机断层扫描与18F-脱氧葡萄糖(FDG)扫描。免疫学,PCR,和基于核酸的测试现在是分离感染性生物的可接受手段。由于耐药性和副作用,不再推荐克林霉素用于抗生素预防。对于成年和怀孕的ACHD患者中的特定先天性心脏病,必须特别考虑抗生素的预防和管理。感染性心内膜炎(IE),一个潜在的破坏性临床实体,是对患有先天性心脏病(ACHD)的成年人的健康的可怕威胁。IE需要一种系统的预防方法,在多学科IE团队的参与下进行早期诊断和管理。IE的诊断和管理发生了变化,这反映在更新的诊断标准中。及时的血培养和影像学检查仍然是诊断的主要手段,然而,血液培养的时间,微生物检测,和类型的诊断成像,如正电子发射计算机断层扫描与18F-氟脱氧葡萄糖(FDG)扫描是新的。二叶主动脉瓣,室间隔缺损,经导管肺动脉瓣置换术,和法洛四联症是ACHD人群中IE风险较高的诊断。以下文章将重点介绍预防策略,除了ACHD患者IE的新型诊断和治疗方法。
    OBJECTIVE: Present an updated overview of the prevention, diagnosis, and management of infective endocarditis in adult patients with congenital heart disease.
    RESULTS: Care for patients with infective endocarditis is changing in the areas of specialized teams, diagnostics, and prevention. Endocarditis teams should be involved in the care of ACHD patients. The 2023 Duke Criteria for Infective Endocarditis and the 2023 European Society of Cardiology Guidelines have updated the criteria for diagnosis including new major criteria such as CT and positron emission computed tomography with 18F-fluorodeoxyglucose (FDG) scans. Immunological, PCR, and nucleic acid-based tests are now acceptable means to isolate infective organisms. Clindamycin is no longer recommended for antibiotic prophylaxis due to resistance and side effect profile. Special considerations for antibiotic prophylaxis and management must be made for specific congenital heart diseases in adulthood and pregnant ACHD patients. Infective endocarditis (IE), a potentially devastating clinical entity, is a feared threat to the health of adults with congenital heart disease (ACHD). IE needs a systematic approach for its prevention, early diagnosis and management with a multidisciplinary IE team\'s involvement. There have been changes in the diagnostics and management of IE, which is reflected in updated diagnostic criteria. Timely blood cultures and imaging continue to be the mainstay of diagnosis, however the timing of blood cultures, microbiological testing, and types of diagnostic imaging such as the positron emission computed tomography with 18F-fluorodeoxyglucose (FDG) scan are new. Bicuspid aortic valves, ventricular septal defects, transcatheter pulmonary valve replacements, and tetralogy of Fallot are diagnoses at higher risk for IE in the ACHD population. The following article will focus on the preventive strategies, in addition to novel diagnostic and therapeutic approaches of IE in ACHD patients.
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  • 文章类型: Journal Article
    成人先天性心脏病(ACHD)人口正在增长,风险预测对于预测不良结局和在患者生命周期中咨询患者非常重要。
    本研究旨在描述血液生物标志物在ACHD中的长期预后价值。
    在这项前瞻性观察性队列研究中,602名中度或复杂ACHD患者纳入研究(中位年龄32.5岁[IQR:24.7-41.2],42%为女性,90%纽约心脏协会I)。N末端脑钠肽前体(NT-proBNP),高敏肌钙蛋白T,生长分化因子15,高敏C反应蛋白,抑制致瘤性-2和半乳糖凝集素-3,以及全血细胞计数,肾功能,LDL,和HDL进行测量。应用Cox模型将选定的生物标志物与全因死亡率的主要终点和死亡率或心力衰竭的次要终点相关联。根据相关预后因素调整后的标准化HR,包括年龄,性别,诊断的复杂性,被报道。
    在424名(70.4%)患者中存在异常的生物标志物水平。在平均10.1年的随访中,41例(6.8%)患者死亡,81例(13.5%)发生心力衰竭。观察到主要和次要终点与红细胞分布宽度之间的关联,NT-proBNP,和生长分化因子15。在多生物标志物模型中,只有NT-proBNP与死亡率相关(HR:2.74;95%CI:2.01-3.74).NT-proBNP显著改善了临床预测模型的C统计量(0.85-0.92)。仅基于NT-proBNP,可以识别低风险患者。NT-proBNP<76ng/L的患者10年无心力衰竭生存率为98.5%。
    血液生物标志物在ACHD中具有预后价值。NT-proBNP改善了风险预测,能够识别低危患者。它的常规使用应在ACHD中实施。
    UNASSIGNED: The adult congenital heart disease (ACHD) population is growing and risk prediction is important to predict adverse outcome and consult patients during their lifecourse.
    UNASSIGNED: This study aims to describe the long-term prognostic value of blood biomarkers in ACHD.
    UNASSIGNED: In this prospective observational cohort study, 602 patients with moderate or complex ACHD were included (median age 32.5 years [IQR: 24.7-41.2], 42% female, 90% New York Heart Association I). N-terminal pro-brain natriuretic peptide (NT-proBNP), high-sensitive-troponin T, growth differentiation factor 15, high-sensitive-C-reactive protein, suppression of tumorigenicity-2 and galectin-3, as well as full blood count, renal function, LDL, and HDL were measured. Cox models were applied to relate the selected biomarkers with the primary end point of all-cause mortality and secondary end point of mortality or heart failure. Standardized HRs adjusted for relevant prognostic factors, including age, sex, and complexity of diagnosis, were reported.
    UNASSIGNED: Abnormal biomarker levels were present in 424 (70.4%) patients. During a median follow-up of 10.1 years, 41 (6.8%) patients died and 81 (13.5%) developed heart failure. Associations were observed between the primary and secondary end point and red cell distribution width, NT-proBNP, and growth differentiation factor 15. In a multibiomarker model, only NT-proBNP remained associated with mortality (HR: 2.74; 95% CI: 2.01-3.74). NT-proBNP significantly improved the C-statistic of the clinical prediction model (0.85-0.92). Based on NT-proBNP alone, low-risk patients could be identified. Patients with NT-proBNP <76 ng/L showed a 10-year heart failure-free survival of 98.5%.
    UNASSIGNED: Blood biomarkers have prognostic value in ACHD. NT-proBNP improves risk prediction and is able to identify low-risk patients. Its routine use should be implemented in ACHD.
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  • 文章类型: Case Reports
    房间隔缺损(ASDs),包括很大一部分先天性心脏异常,包括一个罕见且更具诊断挑战性的子集,称为窦性静脉ASDs(SVASDs)。自闭症在女性中更普遍,随着手术和经导管介入治疗的进展,40岁以下患者的预后总体上是有利的.然而,40岁以上成人未确诊的自闭症患者,尤其是女性,经常导致严重的并发症,包括肺动脉高压,心房颤动,艾森曼格综合征,死亡率超过50%。我们详细的案例研究集中在一个肥胖的42岁西班牙裔移民女性慢性呼吸衰竭错误归因于肺动脉高压,导致未诊断的SVASD并发症进展。使用对比增强的经食管超声心动图(TEE)进行的进一步调查阐明了她初次就诊四年后的正确诊断。本报告探讨了导致患者延迟诊断和发展肺动脉高压导致艾森曼格综合征的晚期心脏并发症的潜在因素,这使她无法进行手术干预。此外,本报告开创了对新诊断为SVASD的成人病例报告的首次全面审查,揭示并发症的性别差异。
    Atrial septal defects (ASDs), comprising a significant portion of congenital cardiac anomalies, encompass a rarer and more diagnostically challenging subset known as sinus venosus ASDs (SVASDs). ASDs are more prevalent in females, and the prognosis for patients under 40 years of age is generally favorable with advancements in surgical and transcatheter interventions. However, undiagnosed ASDs in adults above 40 years old, especially females, often lead to severe complications, including pulmonary hypertension, atrial fibrillation, Eisenmenger syndrome, and a mortality rate exceeding 50%. Our detailed case study focuses on an obese 42-year-old Hispanic migrant female with chronic respiratory failure misattributed to pulmonary hypertension, resulting in the progression of complications from undiagnosed SVASD. Further investigation using contrast-enhanced transesophageal echocardiography (TEE) elucidated the correct diagnosis four years after her initial presentation. This report explores the potential factors contributing to the patient\'s delayed diagnosis and development of advanced cardiac complications of pulmonary hypertension leading to Eisenmenger syndrome that precluded her from procedural intervention. Furthermore, this report pioneers the first thorough review of case reports in adults newly diagnosed with SVASD, revealing sex-based differences in complications.
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  • 文章类型: Journal Article
    关于这些特征知之甚少,治疗模式,和成人先天性心脏病(ACHD)患者入住心脏重症监护病房(CICU)的结局。
    作者试图更好地定义当代流行病学,治疗模式,以及CICU中ACHD入院的结果。
    重症监护心脏病学试验网络是北美CICU的多中心网络。参与中心在2017年至2022年的2个月年度快照中提供了连续入院的前瞻性数据。我们分析了ACHD患者与没有ACHD患者的入院特征和结果。多变量逻辑回归用于评估ACHD与非ACHD入院的死亡率。
    在42个站点的23,299CICU招生中,有441例(1.9%)ACHD入院。分流性病变最为常见(46.1%),其次是右侧病变(29.5%)和复杂病变(28.7%)。ACHD入院比非ACHD入院年轻(中位年龄46岁对67岁)。ACHD入院更常见于心力衰竭(21.3%vs15.7%,P<0.001),一般医疗问题(15.6%对6.0%,P<0.001),和房性心律失常(8.6%vs4.9%,P<0.001)。ACHD入院的序贯器官衰竭评估评分中位数较高(5.0vs3.0,P<0.001)。ACHD入院的总住院时间更长(8.2天vs5.9天,P<0.01),尽管住院死亡率没有差异(12.7%vs13.6%;年龄和性别校正OR:1.19[95%CI:0.89-1.59],P=0.239)。
    本研究说明了ACHDCICU准入的独特方面。进一步调查管理特定ACHD相关CICU入院的最佳方法,如心源性休克和急性呼吸衰竭,是有保证的。
    UNASSIGNED: Little is known regarding the characteristics, treatment patterns, and outcomes in patients with adult congenital heart disease (ACHD) admitted to cardiac intensive care units (CICUs).
    UNASSIGNED: The authors sought to better define the contemporary epidemiology, treatment patterns, and outcomes of ACHD admissions in the CICU.
    UNASSIGNED: The Critical Care Cardiology Trials Network is a multicenter network of CICUs in North America. Participating centers contributed prospective data from consecutive admissions during 2-month annual snapshots from 2017 to 2022. We analyzed characteristics and outcomes of admissions with ACHD compared with those without ACHD. Multivariable logistic regression was used to assess mortality in ACHD vs non-ACHD admissions.
    UNASSIGNED: Of 23,299 CICU admissions across 42 sites, there were 441 (1.9%) ACHD admissions. Shunt lesions were most common (46.1%), followed by right-sided lesions (29.5%) and complex lesions (28.7%). ACHD admissions were younger (median age 46 vs 67 years) than non-ACHD admissions. ACHD admissions were more commonly for heart failure (21.3% vs 15.7%, P < 0.001), general medical problems (15.6% vs 6.0%, P < 0.001), and atrial arrhythmias (8.6% vs 4.9%, P < 0.001). ACHD admissions had a higher median presenting Sequential Organ Failure Assessment score (5.0 vs 3.0, P < 0.001). Total hospital stay was longer for ACHD admissions (8.2 vs 5.9 days, P < 0.01), though in-hospital mortality was not different (12.7% vs 13.6%; age- and sex-adjusted OR: 1.19 [95% CI: 0.89-1.59], P = 0.239).
    UNASSIGNED: This study illustrates the unique aspects of the ACHD CICU admission. Further investigation into the best approach to manage specific ACHD-related CICU admissions, such as cardiogenic shock and acute respiratory failure, is warranted.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    由于外科技术的进步,先天性心脏病患者现在可以很好地生活到成年期,改善医疗管理,以及新型治疗剂的开发。随着先天性心脏病患者年龄的增长,许多需要基于导管的介入治疗残余缺陷,最初修复或缓解的后遗症,或获得性心脏病。在过去的30年中,先天性心脏病患者的经导管介入治疗的类型和数量呈指数级增长。随着医疗技术和设备设计的改进,包括使用设计用于治疗获得性瓣膜狭窄或反流的装置,以前因各种情况需要进行心脏直视手术的患者现在可以接受基于经皮心脏导管的手术.这些程序中的许多程序都很复杂,并且发生在由多学科团队提供最佳服务的复杂患者中。这篇综述旨在强调一些目前可用的经导管介入治疗成人先天性心脏病的方法。每次干预的临床结果,以及任何特殊考虑因素,以便读者可以更好地理解手术和成人先天性心脏病患者。
    Patients with congenital heart disease now live well into adulthood because of advances in surgical techniques, improvements in medical management, and the development of novel therapeutic agents. As patients grow older into adults with congenital heart disease, many require catheter-based interventions for the treatment of residual defects, sequelae of their initial repair or palliation, or acquired heart disease. The past 3 decades have witnessed an exponential growth in both the type and number of transcatheter interventions in patients with congenital heart disease. With improvements in medical technology and device design, including the use of devices designed for the treatment of acquired valve stenosis or regurgitation, patients who previously would have required open-heart surgery for various conditions can now undergo percutaneous cardiac catheter-based procedures. Many of these procedures are complex and occur in complex patients who are best served by a multidisciplinary team. This review aims to highlight some of the currently available transcatheter interventional procedures for adults with congenital heart disease, the clinical outcomes of each intervention, and any special considerations so that the reader may better understand both the procedure and patients with adult congenital heart disease.
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  • 文章类型: Journal Article
    接受经皮PFO封堵术的卵圆孔未闭(PFO)患者与接受药物治疗的PFO患者相比,发生房颤(AF)的风险更大。如果接受治疗,封堵后房颤似乎具有良好的耐受性,但可能会增加中风的风险。据报道,封堵后房颤发生在3.7%至7.4%的患者中;然而,跨设备的发病率仍然不确定。本研究旨在评估封堵后房颤的频率,房扑,和6个PFO封堵器中的心律失常。
    2001年至2021年期间,有44例患者接受了经皮PFO封堵术,并进行了适当的随访。该程序使用雅培AmplatzerPFO进行,AmplatzerASD,AmplatzerCribriform,NMT心脏密封,GoreHelex,或Gore心形装置.房颤发生率,房扑,在关闭后6个月内通过心电图评估心律失常。多变量逻辑回归评估了封堵后房颤或房扑的潜在预测因素。
    封堵后房颤或房扑在30例(6.7%)6个月内发生,其发病率在不同设备之间有显著差异。与其他装置相比,Gore心脏型封堵后房颤或房扑事件的发生频率最高(16.8%)。Gore心形装置,更大的设备尺寸,男性和男性与封堵后房颤或房扑的风险增加相关。
    在Gore心脏造形装置中更可能发生封堵后房颤或房扑,在男性中,以及使用较大装置进行PFO闭合的患者。虽然它对于完全关闭更有效,研究表明,Gore心形装置是封堵后房颤或房扑的独立预测因子.
    UNASSIGNED: Patients with a patent foramen ovale (PFO) who undergo percutaneous PFO closure are at a greater risk of developing atrial fibrillation (AF) compared with patients whose PFOs are managed medically. Postclosure AF appears to be well tolerated if treated but may increase the risk for stroke. Postclosure AF is reported to occur in 3.7% to 7.4% of patients; however, incidence across devices remains uncertain. This study aims to evaluate the frequency of postclosure AF, atrial flutter, and arrhythmias in 6 PFO closure devices.
    UNASSIGNED: Four hundred forty-five patients underwent percutaneous PFO closure with appropriate follow-up between 2001 and 2021. The procedure was performed using Abbott Amplatzer PFO, Amplatzer ASD, Amplatzer Cribriform, NMT CardioSEAL, Gore Helex, or Gore Cardioform devices. Incidence of AF, atrial flutter, and arrhythmias were assessed by electrocardiogram within 6 months from closure. Multivariate logistic regression evaluated potential predictors of postclosure AF or atrial flutter.
    UNASSIGNED: Postclosure AF or atrial flutter occurred in 30 patients (6.7%) within 6 months, and its incidence was significantly different across devices. Gore Cardioform had the greatest frequency of postclosure AF or atrial flutter events (16.8%) compared with other devices. The Gore Cardioform device, larger device sizes, and male sex were associated with greater risk of postclosure AF or atrial flutter.
    UNASSIGNED: Postclosure AF or atrial flutter was more likely to occur in the Gore Cardioform device, in males, and in patients who underwent PFO closure with larger devices. Although it is more effective for complete closure, the Gore Cardioform device was shown to be an independent predictor of postclosure AF or atrial flutter.
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