肺血管阻力与体外循环后肺动脉高压的关系

目的观察先天性心脏病术前肺血管阻力(Rp)、肺动脉/体循环压(Pp/Ps)、肺循环/体循环血流(Qp/Qs)比值与术中肺/体循环压力比值(Rp/Ps)间的关系,评估Rp对术中肺动脉高压危象的预测作用,及其对术中应用一氧化氮(NO)的指征作用.方法本组患儿共46例,术前心导管检查证实均为先天性心脏病伴肺动脉高压,并拟行心脏直视手术.术前用药及麻醉方法基本相同.术前Rp、Pp/Ps、Qp/Qs值在心导管检查时由Fick公式计算.体外循环(CPB)结束前分别在肺动脉及升主动脉直接测定肺动脉压(Pp)和体循环压(Ps),计算出术中Pp/Ps,并将其与术前Rp、Pp/Ps、Qp/Qs值分别进行相关分析.结果术前Rp值与CPB脱机时的Pp/Ps比值呈正相关(r2=0.86,P<0.05 ,n=46),而术前Pp/Ps比值和Qp/Qs比值则与CPB脱机时的Pp/Ps比值无明显关系.术前Rp<7.0Wood unit m2的病例术中均未发生肺动脉高压危象.发生肺动脉高压危象的4例中,术前Rp值平均为(12±2.6)Wool unit m2(7.4～15).结论术前Rp可有效的预测CPB后肺动脉高压危象的发生,并可作为术中使用NO吸入治疗的指征.     

循环miR-19a作为先天性心脏病相关肺动脉高压的标志物

目的 miRNA是一类约高度保守的小分子非编码RNA,具有调控细胞分化、增殖、凋亡的功能,与心血管疾病密切相关。因肺动脉高压是血管增殖性疾病,与血管再生相关的miR-19a可能与肺动脉高压密切相关,循环miRNA可作为先天性心脏病相关肺动脉高压的无创诊断标志物。方法收集室间隔缺损合并重度肺动脉高压患者血浆14例(病例组),室间隔缺损不伴肺动脉高压患者血浆14例(对照组)。分别从血浆中提取总RNA并进行质量检测,进行逆转录反应,获得相应miRNA的cDNA,以U6snRNA为内参进行标定,进行实时荧光定量PCR反应,绘制标准曲线,根据标准曲线和各样本的定量PCR结果计算miR-19a的表达量并绘制ROC曲线。结果两组样本的总RNA均有较好的质量,可以满足miRNA芯片检测和实时定量PCR的实验要求。病例组血浆中miR-19a拷贝数明显高于对照组。ROC曲线下面积为0.865(95%可信区间,CI=0.727-1.002,P0.001),循环miR-19a诊断先天性心脏病相关肺动脉高压具有较高的敏感性和特异性。结论先天性心脏病相关肺动脉高压患者血浆中miR-19a显著增高,循环miR-19a可作为先天性心脏病相关肺动脉高压新的无创性诊断标志物。     

靶向药物联合缺损修复治疗成人先天性心脏病相关肺动脉高压的预后研究

目的:分析靶向药物联合缺损修复(Treat-Repair策略)治疗成人先天性心脏病合并肺动脉高压患者的有效及安全性.方法:回顾分析2010年12月至2019年9月,于我中心接受缺损修复的54例,平均年龄(31.07±10.09)岁的成人左向右分流先天性心脏病合并肺动脉高压[平均肺动脉压力(mPAP)≥25 mmHg,1...     

中度低温停循环结合选择性脑灌注在婴儿主动脉弓部手术中的应用

目的 总结中度低温停循环(moderate hypothermia circulatory arrest,MHCA)结合选择性顺行脑灌注(sective antegrade cerebral perfusion,SACP)技术在婴儿主动脉弓重建手术中的应用经验.方法 回顾性分析上海市儿童医院心胸外科于2012年1月至2018年12月间完成的主动脉弓病变合并心内畸形矫正的患儿50例.依据中心温度将患儿分为深低温停循环(deep hypothermia circulatory arrest,DHCA)组及MHCA组,每组25例.所有患儿均在体外循环(cardiopulmonary bypass,CPB)下行一期手术治疗.主动脉弓重建过程中采用低温停循环技术,通过无名动脉SACP(25~40 ml·kg^-1·min^-1)的CPB管理方法.心肌保护采用康斯特器官保护液(HTK液).记录两组患者的一般资料及术中、术后指标.结果 两组均无与CPB相关的神经系统并发症.两组患儿年龄、体重、病种、术前肝肾功能及术前左心室射血分数值比较差异均无统计学意义(P>0.05).CPB时间MHCA组较DHCA组明显缩短[(120.00±22.60)min比(137.40±22.88)min,P=0.019];术后24 h胸腔引流量及正性肌力药物评分MHCA组较DHCA组明显减少,分别为[(49.84±20.66)ml比(78.20±52.31)ml,P=0.03;(9.72±2.47)分比(12.24±3.07)分,P=0.004].结论 在婴儿主动脉弓重建手术中,采用DHCA或MHCA结合SACP的技术均能均减少术后神经系统并发症发生,不增加术后其他并发症.MHCA可减少CPB时间、术后胸腔引流量及术后血管活性药物的使用量.     

先天性心脏病合并中重度肺动脉高压患者的术后护理效果

目的分析先天性心脏病合并中重度肺动脉高压患者的术后护理效果。方法选取2013年7月~2016年2月我院收治的先天性心脏病合并中重度肺动脉高压手术患者72例作为研究对象,按照1:1比例分为对照组和试验组,各36例。对照组术后采用常规护理,试验组术后采用综合护理,观察并比较两组护理效果。结果试验组同对照组术后并发症发生率比较,差异有统计学意义(P0.05)。结论先天性心脏病合并中重度肺动脉高压患者术后实施综合护理,可有效预防并发症,提高效果,具有积极的临床使用和推广价值。     

小儿心脏手术期间深低温停循环对脑血流及氧合的影响

作者回顾分析以下方面:麻醉剂和手术方法;脑血流测定;脑氧合测定;脑血流研究;脑氧合研究;展望未来。 深低温停循环(DHCA)是治疗小儿复杂先天性心脏病的必要条件之一。DHCA与外科医生、心血管医生及麻醉医生均有关系。DHCA已较成熟,神经损伤的发生率很低。研究证实,DHCA后即刻脑组织不会发     

深低温停循环后急性肾损伤与CHOP蛋白相关

目的探讨深低温停循环引起的急性肾损伤是否与内质网应激的核心蛋白CHOP相关。方法 16只SD大鼠随机分为3组:深低温停循环组(DHCA组,n=6)、体外循环组(CPB组,n=6)和对照组(SHAM组,n=4)。分别于术前及术后收集大鼠股动脉血样检测血肌酐水平。术后处死大鼠取肾组织,于光镜及电镜下进行形态学观察,使用Western blot法检测肾组织内质网相关蛋白CHOP的表达量,且使用Real Time PCR法检测CHOP蛋白m RNA表达。结果术前三组大鼠之间基线血肌酐值无差异,处死时DHCA组的血肌酐显著高于CPB组及SHAM组。形态学观察显示,SHAM组大鼠肾小管结构基本正常;线粒体及核糖体形态及结构均正常,CPB组肾小管结构损伤较DHCA组较轻,线粒体及核糖体均有轻度损伤;DHCA组肾小管结构损伤较重,且线粒体及核糖体损伤较重。内质网应激相关蛋白CHOP及其m RNA的表达在DHCA组显著高于CPB组及SHAM组。结论深低温停循环可导致明确的急性肾损伤,该损伤可能与内质网应激的核心蛋白CHOP相关。     

高原地区先天性心脏病并发重度肺动脉高压的围术期管理

目的:探讨降低高原地区先天性心脏病并发肺动脉高压患者围术期并发症和手术死亡率的策略,评估吸入一氧化氮（NO)在先天性心脏病并发重度肺动脉高压患儿围术期的治疗效果。方法: 回顾性分析2009年6月~2012年12月我院收治的31例高原地区先天性心脏病并发重度肺动脉高压患者围术期管理经验,分析其术前、术中和术后等治疗因素对患者预后的影响,以及术后患者吸入NO 前后的心率、平均动脉压、中心静脉压、动态肺顺应性及氧合指数的变化。结果: 1例患者死亡,其余30 例患者在经过严格围术期管理及吸入NO 治疗后,心率、血压无明显改变,平均超声肺动脉压力、动态肺顺应性、氧合指数比治疗前明显改善,有统计学意义。结论: 严密的围术期管理及NO吸入治疗对治疗高原地区先天性心脏病并发重度肺动脉高压患者具有较好的临床效果。     

小儿肺动脉高压的诊断与治疗

肺动脉压力超过正常称为肺动脉高压(Pul-monary Hypertension),即当肺动脉收缩压超过4.00kPa(30mmHg)或肺动脉平均压超过2.67kPa(20mmHg)时,则表示有肺动脉高压存在(新生儿2周内,正常肺动脉压力可稍高)。在小儿时期,肺动脉高压常见于以下疾病:先天性心脏病、新生儿持续肺动脉高压(持续性胎儿循环)、缺氧性疾患(支气管哮喘、婴幼儿肺炎、高原性肺动脉高压及支气管肺发育不良等)及原发性肺动脉高压等。     

抑肽酶在深低温停循环手术中的应用现状

医用抑肽酶是从牛肺中提取的一种非特异性丝氨酸蛋白酶抑制剂，属于多肽类，由16种氨基酸，58个残基组成。现已公认，在常规体外循环(CPB)手术中应用抑肽酶能显著减少术中、术后出血及输血需要量。但在深低温停循环(DHCA)手术中，抑肽酶是否仍具有以上作用，观点并不统一。现就抑肽酶在DHCA手术中的应用情况及研究进展综述如下。     

Pulmonary arterial hypertension in congenital heart disease

Pulmonary arterial hypertension (PAH) is a recognized complication of congenital systemic to pulmonary arterial cardiac shunts. The prognosis of PAH in this situation is better than primary or other secondary forms of PAH. Our knowledge of the pathophysiology of PAH complicating congenital heart disease has evolved over the past decade. Despite differences in etiology and pathobiology, therapies that have proven successful for primary PAH may benefit this group of patients.     

成人先天性心脏病肺动脉高压的新认识

成人先天性心脏病患者表现为一个正在扩大的并需要终身接受3级护理的群体。一些成人先心病的患者最后发展为不同程度的肺动脉高压,影响患者的生存质量,发病率和病死率。然而近期对肺动脉高压病理生理学理解的提高和靶向治疗的出现使人们在成人先天性心脏病肺动脉高压患者病因学、临床表现、预后和治疗策略方面有了新的认识。     

Audit of the aetiology and prevalence of pulmonary hypertension in a tertiary hospital setting

Appropriate diagnosis and initiation of disease‐specific treatment is an important therapeutic goal in idiopathic pulmonary arterial hypertension. We evaluated the prevalence and aetiology of moderate‐to‐severe pulmonary hypertension in a cohort of patients referred for inpatient echocardiography, with significant pulmonary hypertension documented in 4.6%. Pulmonary hypertension complicating left heart disease was the most common aetiology, with idiopathic pulmonary arterial hypertension less frequent.     

Congenital heart disease associated pulmonary arterial hypertension

Pulmonary arterial hypertension associated with congenital heart disease, although common (15%-30%) in all-comers with congenital heart disease, is variable in terms of clinical manifestations, severity of associated pulmonary arterial hypertension, and response to therapy and outcomes (depending on lesion anatomy, pulmonary circulation flows and pressures, and presence and timings of surgeries). Evaluation includes imaging and catheterization. Surgical or another anatomic correction may be desirable after rigorous preinterventional assessment. Patients who are not surgical candidates or who fail to improve early or late after surgery may have the potential to respond to idiopathic pulmonary arterial hypertension therapies. Lung or heart/lung transplantation remains an option for selected recalcitrant patients.     

Retrograde cerebral perfusion as an adjunct to prolonged hypothermic circulatory arrest.

STUDY OBJECTIVE: This study was designed to evaluate the use of retrograde cerebral perfusion (RCP) combined with deep hypothermic circulatory arrest (DHCA) in the treatment of complex congenital and adult cardiac disease. DESIGN: Retrospective chart review of 52 cardiac surgery patients (34 male and 18 female; age range, 3 weeks to 89 years old; mean age, 60 years old) who received RCP in conjunction with DHCA from July 1991 through August 1998. RESULTS: Surgical procedures consisted of the following: (1) repair of ascending aortic aneurysms (n = 16); (2) repair of type A aortic dissection (n = 16); (3) repair of arch aneurysms (n = 10); (4) renal cell carcinoma with tumor extension to the inferior vena cava (IVC) and right atrium (n = 5); (6) coronary artery bypass grafting and concomitant aortic valve replacement with calcified aorta (n = 2); (7) Norwood procedure and take down of a Pott's shunt (n = 2); and (8) massive air embolism treatment (n = 1). Mean RCP time was 39 min (range, 3 to 88 min). Thirteen patients had RCP times > 60 min. Mean core temperature (rectal or bladder) was 19 degrees C (range, 15 degrees to 28 degrees C). There were six early deaths, four of which were related to persistent low-output cardiac failure, and two resulted from perioperative stroke. All remaining patients recovered fully without neurologic deficits. CONCLUSION: RCP is a reliable and technically appealing tool that does the following: (1) it improves DHCA safety and is applicable in a variety of clinical settings with relative ease; (2) it potentially provides oxygen and nutritional support to the brain during DHCA; (3) it helps remove air and other debris from the cerebral vessels; and (4) it is useful in dealing with congenital heart disease and tumor extension into the IVC.     

Pulmonary arterial hypertension in congenital cardiac disease--the need for refinement of the Evian-Venice classification

Pulmonary hypertension associated with congenital systemic-to-pulmonary shunts has been classified, in the Evian-Venice classification, as Pulmonary Arterial Hypertension, which includes a heterogeneous group of conditions. Emerging options for treatment of patients with pulmonary arterial hypertension are related to those with the idiopathic form of the disease, but may also improve quality of life and survival in patients with pulmonary arterial hypertension associated with congenital cardiac disease. Despite the evident similarities in pulmonary vascular disease, it is important also to recognise the differences between patients in whom pulmonary arterial hypertension is the consequence of systemic-to-pulmonary shunts as opposed to those with other conditions. Patients with pulmonary hypertension associated with congenital cardiac disease themselves constitute a heterogeneous population, in which generalisation may be hazardous. Specific considerations need to be given to the type of cardiac diagnosis, the prognosis and evolution of pulmonary vascular disease, and the circulatory physiology before embarking on new strategies for medical treatment in the individual patient. In this review, we highlight the features that require specific attention in these patients. In addition, we discuss briefly the data currently available on the effectiveness of the new anti-proliferative drugs in patients with the Eisenmenger syndrome.     

中度低温停循环脑保护研究进展

主动脉弓部手术是治疗StandfordA型主动脉夹层的主要方法,该手术中脑保护的最佳方式临床上上存在争议。深低温停循环一直是进行主动脉弓部手术的基础,随着研究的深入,深低温的弊端逐渐被认识,中度低温停循环开始应用于主动脉弓部手术。本文主要对此进行综述。     

先天性心脏病相关性肺动脉高压患者血miR-18a、miR-27b、miR-130a、miR-204水平研究

目的 探索先天性心性脏病相关性肺动脉高压（PAH）患者血清中miR-18a、miR-27b、miR-130a和miR-204的表达水平,以及与PAH的相关性.方法 收集昆明医科大学附属延安医院心血管内科2012年4～12月被确诊为先天性心脏病的患者78例,其中肺动脉压正常组37例,合并轻、中度肺动脉高压组25例,重度肺动脉高压组16例;另设正常对照组20例.应用实时荧光定量PCR （Taqman探针法）检测miR-18a、miR-27b、miR-130a、miR-204在血液中的表达水平,分析先天性心脏病相关性肺动脉高压与miR-NA表达水平之间的关系.结果 先天性心脏病相关肺动脉高压患者血miR-18a、miR-27b、miR-130a表达水平显著上调,miR-204表达水平显著下调.随着肺动脉压力的不断增高,血miR-18a、miR-27b、miR-130a表达量明显升高,miR-204表达量显著下调.直线相关分析显示,先天性心脏病继发性肺动脉高压患者血miR-18a、miR-27b、miR-130a表达水平与肺动脉压力存在高度正相关关系（r=0.927,r=0.927,r=0.933,P＜0.01）,miR-204表达水平与肺动脉压力存在高度负相关关系（r=-0.773,P＜0.01）.结论 血miR-18a、miR-27b、miR-130a、miR-204水平可作为判断先天性心脏病合并肺动脉高压较好的临床血清学指标,预判价值较高.     

Pulmonary alveolar capillary dysplasia in infants: A rare and deadly missed diagnosis

The pulmonary alveolocapillary dysplasia (ACD) with pulmonary vein misalignment (PVM) is a rare condition characterized by a congenital anomaly of the development of the pulmonary parenchyma. We present a case of an 8-month-old infant who died quickly from acute respiratory failure complicating an unknown ACD. We also describe its epidemiological characteristics in infants and we discuss the diagnosis's difficulties. In this case, a pulmonary arterial hypertension was decompensated by an infection. A medico-legal autopsy was performed. As for the Histological examination, it showed the features of ACD/PVM.     

Abnormal endothelial factor VIII associated with pulmonary hypertension and congenital heart defects

In patients with pulmonary hypertension associated with congenital heart defects, ultrastructural abnormalities are observed in endothelial cells, which suggest heightened metabolic function. If endothelial production of the von Willebrand factor (vWF) is increased, this may be associated with abnormal interactions with platelets leading to worsening of the pulmonary hypertension. We therefore evaluated vWF in 30 patients with pulmonary hypertension (25 with congenital heart defects) and in 30 individuals with normal pulmonary arterial pressure (12 with congenital heart defects). We measured the antigenic (vWF: Ag) and biologic (VWF: rist) activity of vWF in plasma and assessed endothelial vWF: Ag directly by an immunoperoxidase stain applied to lung biopsy tissue. Because of considerable variance and small size, the group of five patients with pulmonary hypertension and without congenital heart defects were excluded from statistical analyses. Patients with pulmonary hypertension and congenital heart defects had significant higher vWF: Ag levels than individuals with normal pulmonary arterial pressure without congenital heart defects (p less than .05), whereas values in those with normal pressure and congenital heart defects were intermediate. In lung biopsy tissue available from 29 patients in this study and from 11 others we previously reported, immunostain of pulmonary arterial endothelium for vWF was intense (suggesting increased production) in 29 of 32 with pulmonary hypertension and congenital heart defects and in only one of eight with normal pulmonary arterial pressure and congenital heart defects (p less than .01). Only three patients with congenital heart defects and pulmonary hypertension and increased vWF: Ag, however, had increased vWF: rist. Compatible with this discrepancy was a loss of vWF high-molecular weight forms as determined by both crossed immunoelectrophoresis and multimeric analysis. Our results suggest that increased vWF in most patients with congenital heart defects and pulmonary hypertension is associated with increased production of a biologically deficient molecule lacking high-molecular weight forms.