BACKGROUND & AIMS: Endoscopic transthoracic sympathicotomy (ETS) is a recently developed technique to divide sympathetic nerves. ETS has been shown to improve symptoms and reduce ischemia in patients with severe angina pectoris. Low heart rate variability (HRV) in patients with ischemic heart disease carries an adverse prognosis. HRV reflects autonomic response of the heart and a shift in the sympathovagal balance towards parasympathetic dominance could be a marker of improved prognosis. HRV might also be used as an indicator of surgical success in sympathetic heart denervation. Heart rate was recorded in 57 patients before and after ETS. Registration was recorded during controlled respiration in the supine position and at tilt test over 10 minutes and spectral analysis was performed. Twenty-four hour Holter recordings were analyzed in the time domain. During the controlled setting, the high-frequency (HF) component (0.15 to 0.40 Hz) increased significantly whereas the low-frequency (LF) component (0.04 to 0.15 Hz) did not change significantly. The LF/HF ratio at tilt test was reduced from 1.3 to 0.8 (p <0.01). The time-domain analysis showed a significant increase of the mean RR interval (923 to 1,006 ms, p <0.001) and indexes reflecting parasympathetic tone also increased significantly (the root-mean square of difference measured from 24.3 to 29.5 ms, p <0.001 and the proportion of adjacent RR intervals >50% measured from 5.5% to 8.2%, p <0.01), whereas measurements reflecting global HRV did not change. In addition to relief of symptoms and reduced ischemia in severe angina pectoris, ETS caused a shift of sympathovagal balance toward parasympathetic tone. This might explain the anti-ischemic effect and have prognostic implications.

背景与目标： 内窥镜经胸交感神经切开术（ETS）是一种新近开发的用于分割交感神经的技术。 ETS已显示可改善重度心绞痛患者的症状并减少缺血。缺血性心脏病患者的低心率变异性（HRV）会带来不利的预后。 HRV反映了心脏的自主神经反应，交感神经平衡向副交感性优势的转变可能是预后改善的标志。 HRV也可以用作交感神经去神经术手术成功的指标。 ETS前后有57例患者记录了心率。在仰卧位的受控呼吸过程中和10分钟的倾斜测试中记录配准，并进行光谱分析。在时域中分析了二十四小时的动态心电记录。在受控设置期间，高频（HF）分量（0.15至0.40 Hz）显着增加，而低频（LF）分量（0.04至0.15 Hz）没有显着变化。倾斜测试的LF / HF比从1.3降低到0.8（p <0.01）。时域分析显示平均RR间隔显着增加（923至1,006 ms，p <0.001），反映副交感神经张力的指标也显着增加（差异的均方根从24.3上升至29.5 ms，p <0.001而相邻RR间隔> 50％的比例从5.5％降至8.2％，p <0.01），而反映总体HRV的测量值没有变化。除了缓解症状和减轻严重心绞痛的局部缺血外，ETS还引起交感神经平衡向副交感神经的转变。这可能解释了抗缺血作用并具有预后意义。

BACKGROUND & AIMS: -2

背景与目标： -2

BACKGROUND & AIMS: :Left ventricular (LV) dysfunction and/or heart failure (HF) are frequent complications of hypertension and myocardial infarction (MI), placing affected patients at increased risk of significant morbidity and premature death. Given that the renin-angiotensin-aldosterone system (RAAS) is activated and of pathophysiological importance in such patients, a strong therapeutic rationale exists to target the main effector mechanism (that is, angiotensin II [Ang II]) in order to lessen the associated morbidity and mortality burden. Angiotensin-converting enzyme (ACE) inhibitors have been shown to reduce mortality and LV dysfunction and to slow disease progression in patients with HF, including high-risk, post-MI patients. However, ACE inhibitors (ACE-Is) may not provide optimal long-term RAAS blockade (a finding that is associated with a worse prognosis) and many patients are unable to tolerate such therapy (because of troublesome dry cough, for example). In contrast, Ang II receptor blockers (ARBs) may block the RAAS more completely than ACE-Is and appear to be better tolerated. Several large-scale trials gave evaluated the efficacy of ARBs in patients with LV dysfunction and/or HF (including high-risk, post-MI patients), and have confirmed their utility as an efficacious and well-tolerated alternative to ACE-Is in this setting.

背景与目标： ：左心室（LV）功能障碍和/或心力衰竭（HF）是高血压和心肌梗塞（MI）的常见并发症，使受影响的患者罹患显着发病率和过早死亡的风险增加。鉴于肾素-血管紧张素-醛固酮系统（RAAS）已被激活并且在此类患者中具有重要的病理生理意义，因此存在针对主要效应机制（即血管紧张素II [Ang II]）的强大治疗原理，以减轻相关的疾病。发病率和死亡率负担。血管紧张素转换酶（ACE）抑制剂已被证明可以降低HF患者（包括高危MI后患者）的死亡率和LV功能障碍，并减缓疾病进展。但是，ACEI（ACE-Is）可能无法提供最佳的长期RAAS阻断作用（这一发现与更坏的预后相关），并且许多患者无法忍受此类治疗（例如，由于干咳引起的麻烦）。相比之下，Ang II受体阻滞剂（ARBs）可能比ACE-Is更完全地阻断RAAS，并且似乎具有更好的耐受性。几项大规模试验评估了ARB在LV功能不全和/或HF患者（包括高风险，MI后患者）中的疗效，并证实了其作为ACE-Is的有效且耐受性良好的替代品的用途。此设置。

BACKGROUND & AIMS: -2

背景与目标： -2

BACKGROUND & AIMS: :In this issue of Immunity, Bezbradica et al., (2006) uncover an unsuspected role for the cytokine GM-CSF in the thymic development of invariant NKT cells, a role that licenses these cells to secrete effector cytokines upon activation in the periphery.

背景与目标： Bezbradica等人（2006）在此期《免疫》杂志中揭示了细胞因子GM-CSF在恒定NKT细胞的胸腺发育中的作用，这一作用并未受到人们的怀疑，该作用使这些细胞在外围激活后可以分泌效应细胞因子。

BACKGROUND & AIMS: BACKGROUND/AIMS:Apoptosis has been implicated in the pathogenesis of fulminant hepatic failure (FHF) potentially involving caspases. Thus far, apoptosis in FHF has mainly been studied in animal models while human data are sparse. METHODS:Caspases-3, -8 and -9 activities and Fas expression were analyzed in correlation to TdT-mediated dUTP nick end labelling (TUNEL) positive apoptotic cells in livers of patients with FHF (n=26), chronic liver disease (CLD) (n=60) and normal controls (NC) (n=10). RESULTS:Numbers of TUNEL-positive cells were higher in FHF than in CLD and NC (P<0.001) correlating to the intrahepatic activities of caspase-3. The highest caspase-3 activities were found in fulminant hepatitis B, significantly surpassing those in FHF of any other etiology. In fulminant hepatitis B, caspase-9 activity was also higher than in controls, while caspase-8 activation was not higher than in NC. Unlike caspase-3, caspases -8 and -9 activities were not correlated to the numbers of TUNEL positive cells. Fas expression was also the highest in FHF but did not differ between hepatitis B virus-FHF and other FHF. CONCLUSIONS:Our data indicate differential activation of intrahepatic caspases in FHF depending on the underlying etiology. Massive activation of caspases in fulminant hepatitis B confirms a pivotal role of apoptotic pathways in the pathogenesis of human fulminant hepatitis B.

背景与目标： 背景/目的：细胞凋亡与可能涉及胱天蛋白酶的暴发性肝衰竭（FHF）的发病机制有关。迄今为止，主要是在动物模型中研究了FHF中的细胞凋亡，而人类数据却很少。
方法：分析FHF（n = 26），慢性肝病（CLD）患者肝脏中Taspase-3，-8和-9的活性以及Fas表达与TdT介导的dUTP缺口末端标记（TUNEL）阳性凋亡细胞的相关性。 ）（n = 60）和正常对照（NC）（n = 10）。
结果：与caspase-3的肝内活性相关，FHF中TUNEL阳性细胞数高于CLD和NC（P <0.001）。在暴发性乙型肝炎中发现最高的caspase-3活性，大大超过了其他任何病因的FHF中的caspase-3活性。在暴发性乙型肝炎中，caspase-9的活性也高于对照组，而caspase-8的激活并不高于NC。与胱天蛋白酶3不同，胱天蛋白酶-8和-9的活性与TUNEL阳性细胞的数量无关。 Fas表达在FHF中也最高，但在乙型肝炎病毒FHF和其他FHF之间没有差异。
结论：我们的数据表明FHF肝内胱天蛋白酶有不同的激活作用，这取决于潜在的病因。暴发性乙型肝炎中胱天蛋白酶的大量激活证实了凋亡途径在人类暴发性乙型肝炎发病机理中的关键作用。

BACKGROUND & AIMS: :Subclinical hypothyroidism usually is asymptomatic, but it can be associated with various adverse cardiologic outcomes. With the objective of gaining insight into the role of thyroid-stimulating hormone (TSH) in congenital heart abnormalities, this study measured serum TSH concentrations in different subtypes of grown-up congenital heart disease (GUCHD) patients. Serum TSH (reference range, 0.34-5.6 mIU/L), creatinine, cholesterol, C-reactive protein (CRP), N-terminal proB-type natriuretic peptide (NT-pro-BNP), and 24-h proteinuria were measured in 249 GUCHD patients. Of 24 GUCHD patients (9.6 %) with a TSH level higher than 5.6 mUI/L, nine were cyanotic (37.5 %) and seven (29.1 %) had Down syndrome. The GUCHD patients with serum TSH exceeding 5.6 mIU/L had a significantly higher level of serum NT-pro-BNP (195.1 [0.28; 5,280.3] vs 57.6 [0.00; 929.8]; p = 0.001) and CRP (0.30 [0.06; 1.87] vs 0.16 [0.00; 1.40]; p = 0.011] than those with a TSH level of 5.6 mIU/L or lower. No significant differences were found in serum creatinine, lipids, or 24-h proteinuria between the two groups. The T4 concentrations in the GUCHD patients with TSH exceeding 5.6 mIU/L were within the normal range (0.89 ± 0.23 ng/dL). In the multivariate analysis, cyanosis (odds ratio [OR], 6,399; 95 % confidence interval [CI] 2,296-17,830; p < 0.001), Down syndrome (OR, 6,208; 95 % CI, 1,963-19,636; p = 0.002), and NT-pro-BNP concentrations (OR, 1,001; 95 % CI, 1,000-1,002; p < 0.026) proved to be risk factors for TSH levels higher than 5.6 mIU/L. Because subclinical hypothyroidism entails a cardiovascular risk, the authors postulate that TSH screening should be included in the routine follow-up evaluation of GUCHD patients with cyanosis or Down syndrome.

背景与目标： 亚临床甲状腺功能减退症通常无症状，但可能与各种不良心脏预后相关。为了深入了解甲状腺刺激激素（TSH）在先天性心脏病中的作用，本研究测量了不同类型的成年先天性心脏病（GUCHD）患者的血清TSH浓度。测量血清TSH（参考范围0.34-5.6 mIU / L），肌酐，胆固醇，C反应蛋白（CRP），N端proB型利钠肽（NT-pro-BNP）和24小时蛋白尿。 249位GUCHD患者。 TSH水平高于5.6 mUI / L的24名GUCHD患者（9.6％）中，有9名发（37.5％）和7名（29.1％）患有唐氏综合症。血清TSH超过5.6 mIU / L的GUCHD患者的血清NT-pro-BNP显着更高（195.1 [0.28; 5,280.3] vs 57.6 [0.00; 929.8]; p = 0.001）和CRP（0.30 [0.06; 1.87） [] vs. 0.16 [0.00; 1.40]； p = 0.011]，TSH水平为5.6 mIU / L或更低；两组之间的血清肌酐，脂质或24小时蛋白尿无显着差异。 TSH超过5.6 mIU / L的GUCHD患者的血药浓度在正常范围内（0.89±0.23 ng / dL）。在多变量分析中，紫（（比值[OR]为6,399； 95％置信区间[CI]为2,296- 17,830; p <0.001），唐氏综合症（OR，6,208; 95％CI，1,963-19,636; p = 0.002）和NT-pro-BNP浓度（OR，1,001; 95％CI，1,000-1,002; p <0.026） ）被证明是TSH水平高于5.6 mIU / L的危险因素。由于亚临床甲状腺功能减退症会引起心血管疾病风险，因此作者推测TSH筛查应包括在GUCHD的常规随访评估中紫osis或唐氏综合症患者。

BACKGROUND & AIMS: INTRODUCTION:Cornelia de Lange syndrome (CdLS) is produced by mutations in genes that encode regulatory or structural proteins of the cohesin complex. Congenital heart disease (CHD) is not a major criterion of the disease, but it affects many individuals. The objective of this study was to study the incidence and type of CHD in patients with CdLS. MATERIAL AND METHOD:Cardiological findings were evaluated in 149 patients with CdLS and their possible relationship with clinical and genetic variables. RESULTS:A percentage of 34.9 had CHD (septal defects 50%, pulmonary stenosis 27%, aortic coarctation 9.6%). The presence of CHD was related with neonatal hospitalisation (P=.04), hearing loss (P=.002), mortality (P=.09) and lower hyperactivity (P=.02), it being more frequent in HDAC8+ patients (60%), followed by NIPBL+ (33%) and SMC1A+ (28.5%). While septal defects predominate in NIPBL+, pulmonary stenosis is more common in HDAC8+. CONCLUSIONS:Patients with CdLS have a high incidence of CHD, which varies according to the affected gene, the most frequent findings being septal defects and pulmonary stenosis. Perform a cardiologic study in all these patients is suggested.

背景与目标： 简介：Cornelia de Lange综合征（CdLS）是由编码粘着蛋白复合物的调节蛋白或结构蛋白的基​​因突变产生的。先天性心脏病（CHD）不是该疾病的主要标准，但会影响许多人。这项研究的目的是研究CdLS患者冠心病的发生率和类型。
材料与方法：对149例CdLS患者的心脏病学发现及其与临床和遗传变量的可能关系进行了评估。
结果：34.9％的人患有冠心病（房间隔缺损50％，肺动脉狭窄27％，主动脉缩窄9.6％）。冠心病的存在与新生儿住院（P = .04），听力损失（P = .002），死亡率（P = .09）和多动症（P = .02）有关，在HDAC8患者中更常见（ 60％），其次是NIPBL（33％）和SMC1A（28.5％）。尽管间隔缺损在NIPBL中占优势，但肺动脉狭窄在HDAC8中更常见。
结论：CdLS患者的CHD发病率很高，根据患病基因的不同而不同，最常见的发现是房间隔缺损和肺动脉狭窄。建议对所有这些患者进行心脏检查。

BACKGROUND & AIMS: :The cardioprotective drugs used for treatment against ischemia/reperfusion (MI/R) injury have been well evaluated and are considered inadequate. The Chinese herbal medicine formula, Xinji pill (XJP) has been used traditionally for the prevention and treatment of ischemic heart diseases for decades. In the present study, the cardioprotective effects of XJP against MI/R injury were assessed in vivo and its possible mechanism was examined. Male Sprague‑Dawley rats were selected for establishing an MI/R model, which was induced by ischemia for 30 min followed by 24 h reperfusion. Drugs and saline were administered intragastrically from day 14 prior to MI/R. Blood samples were collected for biochemical detection. The rats were then sacrificed and cardiac muscle tissues were harvested. The mRNA expression levels of antioxidant genes were measured by reverse transcription‑quantitative polymerase chain reaction and the protein levels were measured by western blotting. Pretreatment with XJP for 14 days protected the heart against I/R‑induced myocardial function disorder, protected against heart injury, as demonstrated by normalized serum levels of lactate dehydrogenase and creatine kinase, and suppressed oxidative stress. XJP markedly upregulated the expression of antioxidant genes, including superoxide dismutase, catalase, glutathione reductase and glutathione peroxidase, and promoted the protein expression of heme oxygenase‑1 and NFE2‑related factor 2 (Nrf2) in the heart tissues. Furthermore, Akt kinase was confirmed to be upstream of Nrf2 in the XJP treatment. LY294002, a specific inhibitor of Akt, significantly eliminated the cardioprotective effects of XJP. In conclusion, these results demonstrated that XJP exhibited notable cardioprotective properties, in which the Akt/Nrf2 signaling pathway may be involved.

背景与目标： ：用于抵抗缺血/再灌注（MI / R）损伤的心脏保护药物已得到很好的评估，被认为是不足的。几十年来，传统的中草药配方辛集丸（XJP）被用于预防和治疗缺血性心脏病。在本研究中，体内评估了XJP对MI / R损伤的心脏保护作用，并研究了其可能的机制。选择雄性Sprague-Dawley大鼠建立MI / R模型，该模型由局部缺血30分钟然后再灌注24 h诱导。从MI / R之前第14天开始，在胃内施用药物和盐水。收集血样用于生化检测。然后处死大鼠并收获心肌组织。通过逆转录定量聚合酶链反应测量抗氧化剂基因的mRNA表达水平，并通过蛋白质印迹法测量蛋白质水平。 XJP预处理14天可以保护心脏免受I / R诱导的心肌功能障碍的侵害，防止心脏受伤，如血清乳酸脱氢酶和肌酸激酶水平的正常化以及抑制氧化应激所证明的。 XJP明显上调了抗氧化基因的表达，包括超氧化物歧化酶，过氧化氢酶，谷胱甘肽还原酶和谷胱甘肽过氧化物酶，并促进了心脏组织中血红素加氧酶-1和NFE2相关因子2（Nrf2）的蛋白表达。此外，在XJP处理中，证实Akt激酶位于Nrf2的上游。 LY294002是Akt的特异性抑制剂，可显着消除XJP的心脏保护作用。总之，这些结果表明，XJP具有明显的心脏保护特性，其中可能涉及Akt / Nrf2信号传导途径。

BACKGROUND & AIMS: OBJECTIVE:To investigate: (1) the course of coronary heart disease risk factors (lipid profiles and body mass index (BMI)) in the first five years after discharge from inpatient spinal cord injury (SCI) rehabilitation and (2) the association between lifestyle (physical activity, self-care related to fitness, smoking, alcohol, body mass and low-fat diet) and coronary heart disease risk factors during that period. DESIGN:Prospective cohort study. PARTICIPANTS/METHODS:Individuals with SCI (N=130). Total cholesterol (TC), high-density lipoprotein (HDL), low-density lipoprotein (LDL), triglycerides (TG) and BMI were determined at discharge from inpatient rehabilitation and 1 and 5 years after discharge. Using multilevel regression models, the effects of lifestyle (drinking alcohol, smoking, active lifestyle and self-care) on the lipid profiles and BMI were determined. RESULTS:After correction for lesion and personal characteristics, no changes in lipid profiles in the five years after discharge were seen, whereas the BMI increased significantly with 1.8 kg m(-2). A high percentage was at risk of cardiovascular disease due to high BMI (63-75%) or HDL (66-95%). The individuals who indicated to maintain their fitness level as good as possible and the individuals with a low BMI showed better lipid profiles. Individuals with a more active lifestyle showed higher HDL levels. Individuals who avoid smoking showed a 1.5 kg m(-2) higher BMI. CONCLUSION:Lipid profiles seem to stabilize in the years after discharge from inpatient SCI rehabilitation, whereas the BMI increased. Lifestyle factors associated with a favorable lipid profile and BMI could be identified.

背景与目标： 目的：调查：（1）住院脊髓损伤（SCI）康复出院后的最初五年中，冠心病危险因素（血脂和体重指数（BMI））的病程，以及（2）在此期间的生活方式（体育锻炼，与健身，吸烟，饮酒，体重和低脂饮食有关的自我保健）和冠心病的危险因素。
设计：前瞻性队列研究。
参与者/方法：具有SCI的个人（N = 130）。在住院康复出院时以及出院后1和5年测定总胆固醇（TC），高密度脂蛋白（HDL），低密度脂蛋白（LDL），甘油三酸酯（TG）和BMI。使用多级回归模型，确定了生活方式（饮酒，吸烟，积极的生活方式和自我保健）对血脂和BMI的影响。
结果：校正病灶和个人特征后，出院后五年内血脂没有变化，而BMI显着增加，为1.8 kg m（-2）。高百分比的BMI（63-75％）或HDL（66-95％）导致罹患心血管疾病的风险较高。表示要尽可能保持健康水平的个体和BMI较低的个体表现出更好的血脂状况。生活方式更加活跃的人表现出较高的HDL水平。避免吸烟的人的BMI升高1.5 kg m（-2）。
结论：住院SCI康复出院后的几年中，脂质分布似乎稳定，而BMI升高。可以确定与良好的脂质状况和BMI相关的生活方式因素。

BACKGROUND & AIMS: :Myocardial extracellular volume fraction (ECV) reflecting diffuse myocardial fibrosis can be measured with T1 mapping cardiovascular magnetic resonance (CMR) before and after the application of a gadolinium-based extracellular contrast agent. The equilibrium between blood and myocardium contrast concentration required for ECV measurements can be obtained with a primed contrast infusion (equilibrium contrast-CMR). We hypothesized that equilibrium can also be achieved with a single contrast bolus to accurately measure diffuse myocardial fibrosis in patients with congenital heart disease (CHD). Healthy controls (n = 17; median age 24.0 years) and patients with CHD (n = 19; 25.0 years) were prospectively enrolled. Using modified Look-Locker inversion recovery T1 mapping before, 15 min after bolus injection, and during constant infusion of gadolinium-DOTA, T1 values were obtained for blood pool and myocardium of the left ventricle (LV), the interventricular septum (IVS), and the right ventricle (RV) in a single midventricular plane in short axis or in transverse orientation. ECV of LV, IVS and RV by bolus-only and bolus-infusion correlated significantly in CHD patients (r = 0.94, 0.95, and 0.74; p < 0.01, respectively) and healthy controls (r = 0.96, 0.89, and 0.64; p < 0.05, respectively). Bland-Altman plots revealed no significant bias between the techniques for any of the analyzed regions. ECV of LV and RV myocardium measured by bolus-only T1 mapping agrees well with bolus-infusion measurements in patients with CHD. The use of a bolus-only approach facilitates the integration of ECV measurements into existing CMR imaging protocols, allowing for assessment of diffuse myocardial fibrosis in CHD in clinical routine.

背景与目标： ：应用基于g的细胞外造影剂前后，可通过T1映射心血管磁共振（CMR）来测量反映弥漫性心肌纤维化的心肌细胞外体积分数（ECV）。 ECV测量所需的血液和心肌造影剂浓度之间的平衡可通过灌注造影剂（平衡造影剂CMR）获得。我们假设通过单次对比推注也可以达到平衡，以准确测量先天性心脏病（CHD）患者的弥漫性心肌纤维化。前瞻性纳入健康对照组（n = 17）；中位年龄24.0岁； CHD患者（n = 19； 25.0岁）。使用改良的Look-Locker反转恢复T1映射，在推注大剂量，之后15分钟以及在持续输注--DOTA期间，获得了左心室（LV），心室间隔（IVS）的血池和心肌的T1值，右心室（RV）在短心轴或横向方向的单个心室中平面内。仅推注和推注输注的LV，IVS和RV的ECV在CHD患者（分别为r = 0.94、0.95和0.74； p <0.01）和健康对照者（r = 0.96、0.89和0.64； p中显着相关）分别<0.05）。布兰德-奥特曼（Bland-Altman）图显示，任何被分析区域的技术之间均无明显偏差。通过仅推注T1测绘测得的LV和RV心肌的ECV与CHD患者的推注量测量非常吻合。仅推注方法的使用有助于将ECV测量值集成到现有的CMR成像协议中，从而可以在临床常规中评估CHD中弥漫性心肌纤维化。

BACKGROUND & AIMS: :Anxiety and depression for 399 survivors of a 1,102-member heart disease cohort were assessed 8 to 9 years post-hospitalization. Approximately one-third reported symptoms of emotional distress and one-fourth were on anti-anxiety drugs. Predictors of anxiety and depression were analyzed through logistic regression. Subjects age 65 and over were less likely than younger patients to report anxiety or depression and also reported less heart-associated disability, the strongest predictor of distress for both age groups. Other significant predictors included a previous history of distress, low income, female sex, and beta blocker use.

背景与目标： ：在住院后8到9年评估了1,102名心脏病患者队列中399名幸存者的焦虑和抑郁情绪。大约三分之一的人报告说有情绪困扰症状，而四分之一则是在服用抗焦虑药。焦虑和抑郁的预测因素通过逻辑回归分析。 65岁及65岁以上的受试者比年轻患者报告焦虑或抑郁的可能性更低，而且与心脏相关的残疾报告的可能性也较小，这是两个年龄组的最强烈的痛苦预测指标。其他重要的预测指标包括以前的困扰，低收入，女性和使用β-受体阻滞剂的病史。

BACKGROUND & AIMS: :We performed a retrospective cohort analysis of pregnancies among women with moderate to complex congenital heart disease or pulmonary hypertension over a 12-year period, resulting in a cohort of 107 cases in 65 women. Neuraxial analgesia or anaesthesia was provided in 84%, 89% and 95% of spontaneous vaginal, operative vaginal and caesarean deliveries, respectively. The caesarean delivery rate was 43% compared to our institution average of 27% over the same period (p = 0.02), and 38% had operative vaginal deliveries compared to a 10.5% institution rate (p < 0.01). Invasive monitoring was used in 28% of all deliveries. There were one maternal and two neonatal deaths. This study provides detailed anaesthetic and peripartum management of women with congenital heart disease, a patient population in whom evidence-based practice and data are largely lacking. We observed a predominance of neuraxial anaesthetic techniques, increased caesarean and operative delivery rates, and favourable maternal and neonatal outcomes. Multicentre studies and registries to compare anaesthetic and obstetric management strategies further and delineate risk factors for adverse outcomes are required.

背景与目标： ：我们对12年来中度至复杂性先天性心脏病或肺动脉高压妇女的妊娠进行了回顾性队列研究，得出65例妇女的107例队列。自发性阴道，手术阴道和剖腹产分别有84％，89％和95％提供神经镇痛或麻醉作用。剖腹产率为43％，而同期我们机构的平均水平为27％（p = 0.02），而手术阴道分娩的比例为38％，而机构率为10.5％（p <0.01）。在所有分娩中有28％使用了侵入式监测。有1例孕产妇死亡和2例新生儿死亡。这项研究为患有先天性心脏病的妇女提供了详细的麻醉和围产期治疗方法，该病患者中大量缺乏循证医学实践和数据。我们观察到神经麻醉技术占优势，剖腹产和手术分娩率增加，孕产妇和新生儿预后良好。需要进行多中心研究和登记，以进一步比较麻醉和产科管理策略，并描述不良后果的危险因素。