小儿左向右分流先天性心脏病中TXA2变化的探讨

为探讨TXA2在先天性心脏病(先心病)肺动脉高压形成中的作用,我们采用放免法测定了36例实验对象血浆TXB2的浓度,结果表明与对照组相比,血浆TXB2在左向右分流先心病不伴肺动脉高压组[(125.04±23.55)pg/mL与(63.72±15.51)pg/mL]、肺动脉高压组[(126.42±31.28)pg/mL与(63.72±15.51)pg/mL]均明显升高,但左向右分流先心病不伴肺动脉高压组和肺动脉高压组之间[(125.04±23.55)pg/mL与(126.42±31.28)pg/mL]、中度和重度肺动脉高压组之间[(126.79±21.55)pg/mL与(126.05±41.09)pg/mL]无显著差异.表明血小板活化释放的TXA2并不直接介导先心病肺动脉高压的形成.     

婴幼儿左向右大分流先天性心脏病的外科治疗

目的 :评估婴幼儿左向右大分流先心病外科手术治疗的效果。方法 :回顾分析 1999- 0 1～ 2 0 0 3- 0 1手术治疗婴幼儿左向右大分流先心病 12 8例。均一期手术 ,病种为室间隔缺损、房间隔缺损、动脉导管未闭、单心房等。结果 :术前的营养支持 ,术中的精细操作 ,术后的正性肌力药物及强效肺血管扩张剂 PGE1 的合理使用 ,持续静注速尿 ,维持水电解质平衡是手术成功的关键因素。随访 1个月～ 3年 ,全组病例均获得了较好效果。结论 :左向右大分流先心病伴有肺炎、心衰及并发肺动脉高压 ,经内科治疗无效时应及时手术治疗。     

Quantitative evaluation of right ventricle function by transthoracic echocardiography in childhood congenital heart disease patients with pulmonary hypertension

Introduction and Objective: The present study aims to quantitatively evaluate the right ventricle (RV) function by means of transthoracic echocardiography in normal children and childhood congenital heart disease patients with pulmonary hypertension. Patients and Methods: This study was conducted in a cohort including 40 healthy children and 30 pediatric patients with pulmonary hypertension who were diagnosed under close surveillance at the study center between October 2009 and November 2010. Results: Statistically significant differences were found between the patient and control groups for the right ventricle myocardial performance index (RVMPI), the left ventricle myocardial performance index (LVMPI), the tricuspid valve systolic flow velocity (Ts), the ratio of systolic pulmonary artery pressure to the right ventricle outflow tract systolic flow velocity time integral (sPAP/RVOT VTI), and the ratio of systolic pulmonary artery pressure to right ventricle outflow tract systolic flow velocity time integral × heart rate (sPAP/[RVOT VTI×HR]). When the children were divided into three groups based on their pulmonary vascular resistance significant differences emerged that predicted an increasing severity of RV dysfunction. Significant differences were also observed for the RVMPI, the LVMPI, and the Ts as well as for echocardiographic pulmonary flow (Qp) and systemic flow (Qs). Discussion: The present study demonstrates that echocardiographic parameters can be used for the quantitative detection of RV dysfunction in childhood congenital heart disease patients with high pulmonary artery pressure (systolic, diastolic, and mean) or pulmonary vascular resistance.     

Pulmonary blood flow profiles with reduced right ventricular function in lambs

Summary The determinants of right ventricular (RV) performance with damaged RV free wall, such as occurs with RV infarction, are still unclear. Using 20-MHz Doppler ultrasound equipment, we investigated the changes in pulmonary blood flow velocity profiles before and after ligation of the right coronary artery. RV dp/dt, stroke volume, RV stroke work, aortic pressure and cardiac output decreased and central venous pressure rose after the ligation. The RV stroke work — enddiastolic pressure relationship indicated impaired RV function following ligation. We observed shortened acceleration time (65.0 ± 15.1 vs 54.4 ± 6.2ms,P < 0.05) and reduced maximum velocity of forward flow (59.0 ± 5.9 vs 52.5 ± 7.6cm/s,P < 0.05) after the ligation. Acceleration was interrupted earlier after ligation than before ligation. These alterations in flow are thought to be a consequence of the altered movement of the RV free wall and ventricular septum induced by RV infarction.     

心脏磁共振成像对左向右分流型先天性心脏病合并肺动脉高压的诊断价值及右心室功能评估

目的 探讨心脏磁共振成像(CMR)对左向右分流型先天性心脏病(CHD)合并肺动脉高压(PAH)的诊断效能及对患者右心室功能的评价作用.方法 选取2012年9月至2020年7月就诊于徐州医科大学附属医院确诊为CHD的42例患者,根据右心导管检查的平均肺动脉压力(mPAP)是否≥25 mmHg将患者分为CHD+PAH组(2...     

Pulmonary intravascular ultrasound in infants and children with congenital heart disease

A three-layered appearance of the pulmonary arterial wall has only been described by intravascular ultrasound in adults or autopsy studies of patients with pulmonary hypertension. Thus, pulmonary intravascular ultrasound was performed in 11 patients during heart catheterization to test the hypothesis that distinct layers of peripheral pulmonary arteries can be imaged in infants and children with congenital heart disease. A 3.5 Fr 30 MHz ultrasound catheter was used to image proximal pulmonary arteries with an internal diameter of 3 to 6 mm and distal pulmonary arteries with an internal diameter of 1.5 to 2 mm. Three layers were identified in the proximal arteries of 10 patients but could not be identified in the distal arteries of any patient. There was a significant linear correlation between the indexed dimension of the medial echolucent vascular wall layer and pulmonary vascular resistance. We conclude that intravascular ultrasound can identify vascular changes consistent with medial hypertrophy in the branch pulmonary arteries of young patients with corresponding degrees of pulmonary hypertension. Cathet. Cardiovasc. Diagn. 41:395–398, 1997. © 1997 Wiley-Liss, Inc.     

Lung biopsy findings in previously inoperable patients with severe pulmonary hypertension associated with congenital heart disease

Background

Congenital heart disease with near-systemic pulmonary arterial pressures, previously thought to have irreversible pulmonary vascular disease (PVD), has been successfully corrected at our institution recently. Whether the PVD is reversible remains unknown. This study aimed to examine the nature of the pulmonary arterial vessels in these selective patients.

Methods

All patients with congenital heart disease and severe pulmonary hypertension (PH) were selected using Diagnostic-treatment to undergo radical repair (n = 49). Lung biopsy specimens were obtained during operation. The nature of PVD was determined by Heath-Edwards classification system. All specimens were quantitatively analyzed by calculating percentage media wall area, percentage media wall thickness and arteriole density.

Results

Transcutaneous oxygen saturation of all selected patients increased significantly after Diagnostic-treatment (P < 0.001). There were no operative deaths. Mean pulmonary artery pressure and pulmonary vascular resistance regressed significantly postoperatively (P < 0.001). The incidence of postoperative PH was 59.2% (29/49). Of 49 selected patients with severe PH, 38 (77.6%) showed grade I change, 5 (10.2%) showed grade II change, 4 (8.2%) showed grade III change and only 2 (4%) showed grade IV change with plexiform lesion. The percentage media wall area, percentage media wall thickness and arteriole density were significantly increased in patients associated with PH than in normal subjects (P < 0.001). Follow-up data showed the reversal of PVD in these 2 patients with plexiform lesions.

Conclusions

The PVD in these selective patients with congenital heart disease and severe PH using a Diagnostic-treatment-and-Repair strategy is generally reversible and these patients are operable in current era.     

内皮素、降钙素基因相关肽与先天性心脏病肺动脉压、肺血流量关系的研究

目的 探讨先天性心脏病(CHD)肺动脉高压(PH)的发病机理。方法 采用非平衡放射免疫分析法测定52例肺高压(同伴Qp增加)和30例肺循环血流量(Qp)增加的先心病患儿上腔静脉(SVC)、右心房(RA)、右心室(RV)、肺动脉(PA)、肺静脉(PV)部位血浆ET-1浓度和SVC部位CGRP浓度,18例健康儿童作为对照。结果 血浆ET-1浓度随着PH程度的加重和Qp的增加而升高,ET-1与肺动脉收缩压(SPAP)、全肺阻力(PVP)和Qp呈正相关(r=0.93,0.79,0.65;P<0.01),CGRP则相反(r=-0.90,-0.82,-0.55;P<0.01)。SVC、RA、RV部位间ET-1浓度无显著差异(P>0.05),而RV、PA、PV间ET-1浓度都有显著差异(P<0.01或<0.05)。ET-1与CGRP呈负相关(r=-0.93,P<0.01)。结论 先心病PH和Qp增加皆可导致血浆ET-1浓度升高,PH时更著,升高之来源为肺循环。ET-1促进了先心病PH的形成和发展。Qp增加是PH形成的启动因子。CGRP的浓度下降和CGRP/ET-1的失衡可能是PH发病的另一因素。     

Transcatheter interventions for multiple lesions in adults with congenital heart disease

Recent advances in diagnosis, surgery and interventional management have significantly changed the quality of life of patients with congenital heart disease. Historically, congenital heart disease patients with multiple cardiac lesions have been referred for surgery; however, with the advent of newer technologies and expertise, transcatheter treatment has evolved as an alternative option. A series of patients who underwent interventional procedures for multiple congenital heart disease lesions with excellent procedural and medium-term outcomes is reported.     

选择性肺动脉造影评价先天性心脏病肺动脉高压的初步分析

通过选择性肺动脉造影检查，了解肺血管病变程度。对４３例先天性左向右分流型心脏病病人行选择性肺动脉造影检查，通过分析肺循环时间、肺动脉终末单支血管计数、肺动脉分级、毛细血管像等指标，并与右心导管（４３例）和肺病理（７例）结果进行比较分析。结果显示：随着肺血管病变加重，肺循环时间延长；肺动脉终末单支血管计数、肺动脉分级减少；肺毛细血管象分布不均。结论：选择性肺动脉造影检查可以比较准确地判定肺血管病变程度。     

先心病合并重度肺动脉高压双向分流的外科对策

目的:探讨先天性心脏病伴重度肺动脉高压双向分流的外科对策及疗效。方法:回顾分析32例患者的临床资料,其中,室间隔缺损(VSD)22例,VSD合并房间隔缺损(ASD)5例,动脉导管未闭5例。术前均有不同程度劳力后紫绀,心脏B超提示双向分流,术中测量平均肺动脉压/平均体循环压>0.85,平均0.90±0.15,采用心脏停跳手术6例,不停跳手术26例。房间隔均采用自体心包片做成单向活瓣,以减轻术后早期右心负荷。结果:早期死亡3例(9.4％,3/32)。术后早期均有明显低氧血症,呼吸机支持,平均35.26±17.59小时,无气管切开病例。随访2～86(平均23.6±17.2)月,晚期死亡2例(6.3％,2/32),顽固右心衰3例(9.4％,3/32),其余24例(75％,24/32)恢复良好。结论:先心病伴重度肺动脉高压部分双向分流的病例,采用浅低温心跳不停心内直视手术有利于心肺功能保护,术中作房间隔单向活瓣有利于其早期恢复。     

吸入依洛前列环素对先天性心脏病继发肺动脉高压患者的急性血管扩张试验

目的探讨吸入依洛前列环素（iloprost）对先天性心脏病（CHD）继发肺动脉高压（PAH）患者的肺动脉压力（PAP）、肺血管阻力（PVR）、心指数（CI）的影响。方法给予34例CHD继发PAH患者吸入iloprost和生理盐水,分别测量吸入iloprost和生理盐水后肺动脉收缩压（SPAP）、肺动脉舒张压（DPAP）、肺动脉平均压（MPAP）、PVR,CI、体循环平均压（MAP）和心率（HR）,比较两组指标的差异。结果吸入iloprost后PAH患者的SPAP、DPAP、MPAP和PVR下降,CI增加,MAP、HR无明显变化。结论吸入iloprost可安全有效地降低PAH患者的PAP和PVR,增加CI。     

成人先天性心脏病合并肺动脉高压患者的心脏磁共振评估

目的 探讨心脏磁共振（cardiac magnetic resonance,CMR）评价先天性心脏病合并肺动脉高压患者心室功能的临床价值.方法 对26例先天性心脏病合并肺动脉高压的患者行CMR检查,分别测量并计算右心室与左心室的短轴缩短率、舒张末期直径、舒张末期容积、收缩末期容积、每搏排血量、射血分数等心功能参数及主动脉、肺动脉直径,同时评价室间隔运动、心肌延时强化.采用配对样本t检验比较左、右心室功能参数,采用两个独立样本t检验比较室间隔运动正常组和异常组的右心功能情况,采用卡方检验比较室间隔运动异常与心肌延时强化的关联.结果 右心室舒张末期直径、舒张末期容积、收缩末期容积均显著高于左心室,差异有统计学意义（P＜0.05）;右心室短轴缩短率、射血分数均显著低于左心室,差异有统计学意义（P＜0.05）.26例患者中,14例室间隔运动异常,14例出现心肌延时强化.室间隔运动异常组心肌延时强化出现比例明显高于室间隔运动正常组,差异有统计学意义（P＜0.05）.同时,室间隔运动异常组的右心室舒张末期直径、舒张末期容积均显著高于正常组,差异有统计学意义（P＜0.05）;右心室短轴缩短率显著低于正常组,差异有统计学意义（P＜0.05）;射血分数低于正常组,但差异无统计学意义（P=0.08）.结论 合并肺动脉高压的成人先天性心脏病患者右心功能较左心功能差,室间隔运动异常患者的右心功能更差,室间隔运动异常患者出现心肌延时强化比例高.CMR能够提供先天性心脏病合并肺动脉高压患者的左、右心室功能及相关结构信息,对治疗和预后有重要价值.     

左向右分流先天性心脏病患儿血浆内皮素-1水平变化及意义

目的 :探讨影响先天性心脏病 ( CHD)患儿血浆内皮素 - 1( ET- 1)水平的因素及临床意义。方法 :选择69例左向右分流的 CHD患儿 ,其中伴肺动脉高压 ( PH) 5 0例 ,轻度 PH17例 ,中度 PH16例 ,重度 PH17例 ;不伴PH 19例。分别采股静脉血 ,伴有中、重度 PH 3 3例患儿随机给予前列腺素 E1 ( PGE1 ,16例 ) 2 0 ng/ ( kg· min)静脉滴注或卡托普利 ( 17例 ) 1mg/ ( kg· d)口服治疗 15 d后再采血 ,用放射免疫法测定样品中 ET- 1含量。结果 :1肺血流量增加 ,血浆 ET- 1水平亦增加 ,达正常对照 2倍多 ( P <0 .0 0 1) ;2 CHD并 PH者血浆 ET- 1水平较无PH者明显增高 ( P<0 .0 0 1) ,ET- 1含量随肺动脉压力的升高而增加 ;3 PGE1 可显著降低 PH患儿的平均肺动脉压 ( P <0 .0 5 )及血浆 ET- 1水平 ( P <0 .0 5 ) ;4卡托普利可使患儿的平均肺动脉压及血浆 ET- 1水平下降 ,但与治疗前比较差异无显著性意义 ( P >0 .0 5 )。结论 :PGE1 和卡托普利可能延缓 PH的发展。     

Efficacy of exercise training in pulmonary arterial hypertension associated with congenital heart disease

Background

The objective of this prospective study was to assess the efficacy of exercise training as add-on to medical therapy in patients with congenital heart disease associated pulmonary arterial hypertension (CHD–APAH).

Methods

Patients with invasively confirmed CHD–APAH received in-hospital exercise training for 3 weeks and continued at home. Efficacy parameters were evaluated at baseline, after 3 and 15 weeks. Medical treatment remained unchanged. Worsening events and survival rate were assessed in a follow-up period of 21 ± 14 months.

Results

Twenty consecutive CHD–APAH patients (16 female, 4 male, mean pulmonary arterial pressure 60 ± 23 mm Hg) were included. Patients significantly improved the mean distance walked in 6 min compared to baseline by 63 ± 47 m after 3 weeks (p < 0.001) and by 67 ± 59 m after 15 weeks (p = 0.001). Quality of life-score (p = 0.05), peak oxygen consumption (p = 0.002) and maximal workload (p = 0.003) improved significantly by exercise training after 15 weeks. The 1- and 2‐year survival rates were 100%, the transplantation-free survival rate was 100% after 1 year and 93% after 2 years.

Conclusion

Exercise training as add-on to medical therapy may be effective in patients with CHD–APAH and improved work capacity, quality of life and further prognostic relevant parameters. It was associated with an excellent long-term survival. Further randomized controlled studies are needed to confirm these results.     

有搏动性双向Glenn术治疗小婴儿复杂先心病的临床分析

目的 回顾性总结3岁以内复杂先心病患儿行有搏动性双向Glenn术的治疗效果,探讨双向Glenn分流术的手术时机.方法 收集2008年1月至2013年7月间3岁内接受双向Glenn术的77例患儿资料,根据年龄分为A组(小婴儿组,＜6个月,23例)和B组(婴幼儿组,6-36个月,54例),比较两组患儿围手术期及术后随访资料.结果 A、B两组患儿的手术时间[(148.20±21.21)min、(154.87±52.37)min]、呼吸机辅助时间[(30.63±23.86)h、(24.61±18.83)h]、ICU滞留时间[(6.35±4.16)d、(4.90±2.57)d]、术后住院时间[(12.11±3.68)d、(10.03±4.82)d]、血制品使用量[(401.21±276.79)ml、(435.76±226.95)ml]及术后引流量[(15.68±7.32)ml/kg、(17.78±8.43)ml/kg]比较差异均无统计学意义(P＞0.05).术后两组患儿肺动脉均较术前明显发育(P＜0.05),两组间发育程度比较差异无统计学意义(P＞0.05).18例患儿(A组6例,B组12例)后期接受了TCPC治疗.结论 对于无法行一期根治的复杂发绀型先心病患儿,即使是＜6个月的小婴儿,有搏动性双向Glenn术都是安全、有效的治疗方法.     

先天性心脏病继发性肺动脉高压患者血浆中miRNA的研究

目的 应用微小RNA （microRNA,miRNA）芯片技术研究先天性心脏病合并重度肺动脉高压（pulmonary arterial hypertension,PAH）患者和不合并PAH患者血浆中miRNA表达谱的差异,并初步预测差异表达的miRNA调控的靶基因.方法 收集室间隔缺损（ventricular septal defect,VSD）合并重度PAH（PAH组）和不合并PAH患者（对照组）的血浆.分别提取总RNA,然后采用miRNA芯片进行miRNA表达谱差异分析,并对结果进行实时定量聚合酶链反应（polymerase chain reaction,PCR）验证.运用Targetscan、Pictar、Miranda软件预测可能调控的靶基因.结果 miRNA芯片结果提示：与对照组相比,左向右分流先天性心脏病继发重度PAH患者血浆中表达上调的miRNA有50个,表达下调的miRNA有36个.实时定量PCR验证miR-98与芯片结果一致,表达上调.靶基因预测软件显示内皮素（ET-1）为hsa-miR-98的重要靶基因.结论 miRNA在先天性心脏病继发重度PAH患者血浆中存在差异性表达,miRNA可能与PAH的发生、发展密切相关,血浆miR-98有可能成为先天性心脏病合并重度PAH的新的分子生物学标志物.