儿童先天性心脏病磁共振成像

先天性心脏病(先心病)相当常见,在每1000个新生儿中就有7人左右.儿童先心病通常首选超声心动图枪查,如明确诊断,即行治疗,如不能明确诊断,则做心脏磁共振或多层螺旋CT.CT的主要问题是有大量射线.磁共振对手术前后的儿童先心病人可成为解剖和功能的一站式检查手段.本文介绍磁共振对儿童先心病人的扫描技术和临床应用.自旋同波...     

Contrast-enhanced magnetic resonance angiography of the great arteries in patients with congenital heart disease: an accurate tool for planning catheter-guided interventions

Background: Catheter-guided interventions are increasingly used for relief of lesions in patients with congenital heart disease. Exact anatomical imaging with measurement of the vascular structures is crucial in the planning of such interventions. This can be provided non-invasively and without radiation by contrast-enhanced MR angiography (CE-MRA). Aim: To evaluate the accuracy of the measurements of the vessels obtained by CE-MRA in comparison to those obtained by conventional X-ray angiography (XRA). Methods: Measurements of the diameters of aorta and pulmonary arteries were performed retrospectively and blinded on the CE-MRA and XRA images, in comparable locations. The limits of agreement between the two methods were calculated. Results: Twenty-one CE-MRA and XRA were performed in 20 children with congenital heart disease, median age 4 years (1 day–13 years), weight 18 kg (3.2–74 kg). The time interval between CE-MRA and XRA was 2.6 ± 2.3 months. A total of 98 measurements, 38 of the aorta and 60 of the pulmonary arteries were performed on the images obtained by each technique. The correlation between CE-MRA and XRA measurements was excellent, r=0.97, p < 0.0001. The mean difference between the two techniques was 0.018 ± 1.1 mm; the limits of agreement –2.14 and + 2.18 mm. Similar agreement was found for measures of the aorta (r=0.97, mean difference 0.20 ± 1.08 mm) and of the pulmonary arteries (r=0.97, mean difference 0.048 ± 0.89 mm). Conclusions: CE-MRA provides accurate quantitative anatomical information, which highly agrees with XRA data, and can therefore be used for planning catheter-guided procedures.     

Guidelines and protocols for cardiovascular magnetic resonance in children and adults with congenital heart disease: SCMR expert consensus group on congenital heart disease

Cardiovascular magnetic resonance (CMR) has taken on an increasingly important role in the diagnostic evaluation and pre-procedural planning for patients with congenital heart disease. This article provides guidelines for the performance of CMR in children and adults with congenital heart disease. The first portion addresses preparation for the examination and safety issues, the second describes the primary techniques used in an examination, and the third provides disease-specific protocols. Variations in practice are highlighted and expert consensus recommendations are provided. Indications and appropriate use criteria for CMR examination are not specifically addressed.     

4D flow在儿童先天性心脏病诊断中的应用

先天性心脏病是最常见的先天性畸形,MRI在评价其术后心脏功能中具有重要作用。新技术四维血流分析（4D flow）可同时基于3个互相垂直的维度编码获得相位流速编码电影、多参数分析血流向量和峰值流速,并能可视化分析心内及心外结构的血流。本文对4D flow原理及其在儿童先天性心脏病中的应用进展进行综述。     

先天性心脏病患儿术后希氏束性心动过速治疗效果

目的探讨先天性心脏病患儿术后希氏束性心动过速的治疗效果。方法希氏束性心动过速患儿14例均及时给予抗心律失常治疗。结果14例经胺碘酮、普罗帕酮＋普萘洛尔、低温疗法等治疗后,12例恢复良好,2例死亡。结论小儿先天性心脏病术后尽早明确希氏束性心动过速诊断并给予有效治疗,可取得良好治疗效果。     

Intensive care outcome of adult patients operated on for congenital heart disease

Objective To describe the ICU outcome and the most frequent complications observed in adult patients operated on for a congenital heart disease. Design and setting Retrospective analysis of prospectively collected data and chart review in an adult cardiovascular ICU of a university hospital. Patients 156 patients older than 15 years with congenital heart disease undergoing cardiac surgery between June 2001 and June 2005. Results According to the initial cardiac malformation, patients were divided in four groups with different operative risk based on the Euroscore: those diagnosed bicuspid aortic valve (n = 73) had a score of 5, those with tetralogy of Fallot (n = 33) 5.5, those with simple cardiac defect (n = 26) 3, and those with complex malformations (n = 24) 6. Only two patients (one with tetralogy of Fallot and one with complex malformations) died during the hospitalization (1.2%). Conclusion Euroscore clearly overestimates the risk of surgery in this population of adults with congenital heart disease. Mortality and morbidity were low in those diagnosed bicuspid aortic valve, tetralogy of Fallot, or simple cardiac defect, justifying early surgery for incipient complications. Patients with complex congenital defect require prolonged ICU stay, sometimes with mechanical cardiac support, but their overall good outcome justifies these efforts.     

经食管超声心动图在儿童复杂性先天性心脏病手术治疗中的应用价值

目的探讨经食管超声心动图(TEE)对儿童复杂性先天性心脏病(CHD)手术治疗的价值。方法复杂性CHD患者95例,年龄0.3～15.6岁(平均4.3岁),均在体外循环前后进行TEE检查。结果术前TEE首要诊断与手术诊断符合率100%,其中仅7例(7.4%)TEE次要诊断与手术诊断不符。TEE对经胸超声心动图诊断做出补充或修正9例(9.5%),其中6例(6.3%)由于TEE的发现而作出手术方案改变或相应处理。术后TEE成功显示率95.8%(91/95),在91例显示成功患者中,达到预期效果且无残余问题63例(69.2%),发现残余问题28例(30.8%),其中2例(2.2%)由于TEE的发现立即予以处理。结论术前TEE可准确诊断儿童复杂性CHD,有助于制定手术方案,术后TEE可及时发现残余问题,有助于提高手术成功率。     

Accelerated free breathing ECG triggered contrast enhanced pulmonary vein magnetic resonance angiography using compressed sensing

Background

To investigate the feasibility of accelerated electrocardiogram (ECG)-triggered contrast enhanced pulmonary vein magnetic resonance angiography (CE-PV MRA) with isotropic spatial resolution using compressed sensing (CS).

Methods

Nineteen patients (59 ± 13 y, 11 M) referred for MR were scanned using the proposed accelerated free breathing ECG-triggered 3D CE-PV MRA sequence (FOV = 340 × 340 × 110 mm³, spatial resolution = 1.5 × 1.5 × 1.5 mm³, acquisition window = 140 ms at mid diastole and CS acceleration factor = 5) and a conventional first-pass breath-hold non ECG-triggered 3D CE-PV MRA sequence. CS data were reconstructed offline using low-dimensional-structure self-learning and thresholding reconstruction (LOST) CS reconstruction. Quantitative analysis of PV sharpness and subjective qualitative analysis of overall image quality were performed using a 4-point scale (1: poor; 4: excellent).

Results

Quantitative PV sharpness was increased using the proposed approach (0.73 ± 0.09 vs. 0.51 ± 0.07 for the conventional CE-PV MRA protocol, p < 0.001). There were no significant differences in the subjective image quality scores between the techniques (3.32 ± 0.94 vs. 3.53 ± 0.77 using the proposed technique).

Conclusions

CS-accelerated free-breathing ECG-triggered CE-PV MRA allows evaluation of PV anatomy with improved sharpness compared to conventional non-ECG gated first-pass CE-PV MRA. This technique may be a valuable alternative for patients in which the first pass CE-PV MRA fails due to inaccurate first pass timing or inability of the patient to perform a 20–25 seconds breath-hold.     

十字交叉心合并先天性心脏病的超声诊断体会

目的探讨十字交叉心合并先天性心脏病的超声诊断体会。方法回顾分析3例十字交叉心合并先天性心脏病患者的二维超声和彩色多普勒检查情况,并与手术结果对比分析。结果 3例十字交叉心患者,心房均为正位,心室左袢,房室序接一致。1例成人心室大动脉连接关系一致,为动脉导管未闭和卵圆孔未闭;1例胎儿和1例儿童为大血管转位伴室间隔缺损。结论超声心动图能准确诊断十字交叉心及合并的心血管畸形,有重要的临床应用价值。     

超声心动图不同切面筛查胎儿先天性心脏病的敏感性研究

目的探讨超声心动图各常用切面观在筛查胎儿先天性心脏病中的敏感性,寻找一种操作更简便,诊断准确性更高的最佳切面。方法应用四腔心切面观（4cv）、四腔心切面加主动脉根部短轴切面观（4CV＋AR～SAV）、四腔心切面加左右室流出道切面观（4CV＋VOTV）、四腔心切面加三血管平面观（4CV＋3VV）、四腔心切面加三血管-气管平面观（4CV＋3VVT）筛查2600例孕22～38周的胎儿,比较各切面诊断先天性心脏病的敏感性。结果超声检查胎儿先天性心脏病40例,经引产和产后证实39例。4CV、4CV＋（AR—SAV）、4CV＋VOTV、4CV＋3VV和4CV＋3VVT筛查胎儿先天性心脏病的敏感性分别为49％、62％、75％、90％和90％。结论4CV＋VOTV、4CV＋3VVT筛查胎儿先天性心脏疾病的敏感性高,4CV＋VOTV＋3VVT可作为产前筛查胎儿先天性心脏病的常规检查切面。     

体外循环心内直视下先天性心脏病手术疗效分析

目的 探讨心内直视下先天性心脏病手术体外循环管理经验.方法 回顾性总结2005年1月至2009年1月80例4岁以下体外循环心内直视下先天性心脏病手术患儿的临床资料.结果 80例患儿手术经过基本顺利,未发生与体外循环相关的严重并发症,主动脉开放后自动复跳75例,电击复跳5例,无死亡病例.结论 体外循环心内直视下先心病矫治手术技术安全、可行,随着各种诊断技术的提高,以往被视为无法生存的许多复杂重症先心病患儿,可得到有效治疗.     

婴幼儿先天性心脏病伴肺动脉高压的X线表现

目的 探讨婴幼儿先天性心脏病伴肺动脉高压的X线表现,提高诊断正确率减少误诊。方法 65例先心伴肺动脉高压,都经过胸片、心电图、超声心动图、心导管及心血管造影检查,63例作了手术治疗。结果 VSD45例,VSD PDA16例,DPA 2例,65例均表现为胸廓前后径与胸廓横径比率及心胸比率扩大,而升主动脉宽径相对变窄,两肺呈弥漫型肺气肿。结论 两肺弥漫型肺气肿,右上肺血管扩张并相互重叠呈片状阴影,左侧下肺叶出现不张,左心房扩大是婴幼儿先天性心脏病伴肺动脉高压的主要X线表现。     

Cardiac magnetic resonance imaging in valvular heart disease

Cardiac magnetic resonance imaging (CMR) has rapidly gained acceptance as an accurate, reproducible and non‐invasive imaging method for assessment of a wide range of cardiovascular diseases. However, CMR has not been used widely for diagnostic purposes in valvular heart disease (VHD). Unlike echocardiography it has no body habitus‐related limitations and can thus be used to complement echocardiography. It is an especially good alternative for clinical follow‐up in patients with VHD, as it allows accurate measurement of valvular dysfunction and related ventricular burden. Additionally, CMR is an ideal method for evaluating complex congenital heart disease and determining the significance of its components. It can also be used to study the physiological course of valvular dysfunction and response to therapeutic interventions. In this review, we present a basic introduction to CMR methodology, including its advantages and potential problems, and the physiology and quantification in VHD. We also discuss clinical applications of CMR in VHD. Furthermore, we describe how a CMR study statement should be structured in order to increase clinical use of this valuable methodology in cardiology.     

Use of a 1.0 Tesla open scanner for evaluation of pediatric and congenital heart disease: a retrospective cohort study

Background

Open cardiovascular magnetic resonance (CMR) scanners offer the potential for imaging patients with claustrophobia or large body size, but at a lower 1.0 Tesla magnetic field. This study aimed to evaluate the efficacy of open CMR for evaluation of pediatric and congenital heart disease.

Methods

This retrospective, cross-sectional study included all patients ≤18 years old or with congenital heart disease who underwent CMR on an open 1.0 Tesla scanner at two centers from 2012–2014. Indications for CMR and clinical questions were extracted from the medical record. Studies were qualitatively graded for image quality and diagnostic utility. In a subset of 25 patients, signal-to-noise (SNR) and contrast-to-noise (CNR) ratios were compared to size- and diagnosis-matched patients with CMR on a 1.5 Tesla scanner.

Results

A total of 65 patients (median 17.3 years old, 60% male) were included. Congenital heart disease was present in 32 (50%), with tetralogy of Fallot and bicuspid aortic valve the most common diagnoses. Open CMR was used due to scheduling/equipment issues in 51 (80%), claustrophobia in 7 (11%), and patient size in 3 (5%); 4 patients with claustrophobia had failed CMR on a different scanner, but completed the study on open CMR without sedation. All patients had good or excellent image quality on black blood, phase contrast, magnetic resonance angiography, and late gadolinium enhancement imaging. There was below average image quality in 3/63 (5%) patients with cine images, and 4/15 (27%) patients with coronary artery imaging. SNR and CNR were decreased in cine and magnetic resonance angiography images compared to 1.5 Tesla. The clinical question was answered adequately in all but 2 patients; 1 patient with a Fontan had artifact from an embolization coil limiting RV volume analysis, and in 1 patient the right coronary artery origin was not well seen.

Conclusions

Open 1.0 Tesla scanners can effectively evaluate pediatric and congenital heart disease, including patients with claustrophobia and larger body size. Despite minor artifacts and differences in SNR and CNR, the majority of clinical questions can be answered adequately, with some limitations with coronary artery imaging. Further evaluation is necessary to optimize protocols and image quality.     

婴幼儿先心病术后呼吸机支持治疗

目的 利用呼吸机对婴幼儿先心病患儿进行术后支持治疗以提高外科治疗效果。方法 用呼吸机不同的机械通气方式对366例3岁以下婴幼儿先心病患儿进行术后支持治疗，促进其心肺功能的恢复。结果 本组366例3岁以下婴幼儿先心病患儿呼吸机支持时间最长32d，最强2.5h，平均14.2h；机械通气过程中并发呼吸道感染16例（4.4%)；再插管8例（2.2%)；术后近期死亡率12%（3.3%)。结论 婴幼儿先心病患儿术后呼吸的支持与管理有其自身的特点，抓住这些特点就可以使手术的死亡率与并发症的发生率进一步下降，从而获得满意的疗效。     

Tachyarrhythmias and catheter ablation in adult congenital heart disease

Advances in surgical technique have had an immense impact on longevity and quality of life in patients with congenital heart disease. However, an inevitable consequence of these surgical successes is the creation of a unique patient population whose anatomy, surgical history and haemodynamics result in the development of a challenging and complex arrhythmia substrate. Furthermore, this patient group remains susceptible to the arrhythmias seen in the general adult population. It is through a thorough appreciation of the cardiac structural defect, the surgical corrective approach, and haemodynamic impact that the most effective arrhythmia care can be delivered. Catheter ablation techniques offer a highly effective management option but require a meticulous attention to the real-time integration of anatomical and electrophysiological information to identify and eliminate the culprit arrhythmia substrate. This review describes the current approach to the interventional management of patients with tachyarrhythmias in the context of congenital heart disease.     

先天性心脏病并发感染性心内膜炎75例临床分析

目的探讨先天性心脏病并发感染性心内膜炎(IE)的临床特点．为其诊断与治疗提供参考。方法回顾性分析75例住院患中各类先天性心脏病IE的发生率、临床特点、血培养、心内膜受累和治疗情况。结果①先天性心脏病IE中主动脉瓣受累的发生率最高(11／11)；②手术证实累及多个瓣膜及病变多样；③尿检查改变、肝肿大和脾肿大的发生率及血培养阳性率下降，可能与抗生素广泛应用及开展侵袭性检查有关。本组血培养阳性率35．7％(20／50)．致病菌以草绿色链球菌为常见(14／25)；④手术治疗45例。治愈44例，死亡1例．其余内科治愈13例，因动脉栓塞或心功能恶化而自行出院14例，死亡3例。结论适当的内科治疗与积极的外科治疗相结合是治疗IE的重要手段，对提高存活率、降低病死率有重要意义。     

肺血少型复杂紫绀型先天性心脏病手术的护理

目的 :探讨肺血少型复杂紫绀型先天性心脏病手术的护理措施。方法 :做好术前准备 ,呼吸机参数调节适宜 ,尽早拔管 ,采用“V”型体位 ,合适的中心静脉压 ,以维持循环稳定及预防并发症。结果 :全组 37例死亡 3例 ,余 34例并发乳糜胸、肺不张各 1例 ,胸腔积液 7例 ,均治愈出院。随访1～ 38个月 ,术后患儿活动耐力明显提高 ,平均动脉血氧饱和度由 (73± 7) %提高至 (92± 5 ) %。结论 :做好术前护理 ,术后取正确体位 ,早期拔管 ,强化呼吸道护理 ,维持循环稳定 ,及早抗凝 ,是肺血少型复杂紫绀型先天性心脏病手术后康复的正确措施。     

3.0TMR自动呼吸导航对比增强全心冠状动脉磁共振血管成像的临床应用

目的 评价3.0T MR自动呼吸导航全心冠状动脉磁共振血管成像(CMRA)诊断冠状动脉狭窄的临床应用价值.方法 对50例临床疑诊或确诊冠心病或心肌病的患者行自动呼吸导航对比增强CMRA,其中33例经冠状动脉造影(CAG)证实.评价CMRA图像质量,并与CAG相对照,评价CMRA诊断冠状动脉狭窄的效能.结果 1例CMRA图像质量较差,为1级;余49例CMRA图像质量均满足诊断要求,为2~4级.33例经CAG证实的患者共286段血管中,CRMA可显示238段(238/286,83.22%),未显示48段(48/286,16.78%).以患者数、血管支和血管段为单位,CMRA诊断冠状动脉狭窄的敏感度分别为80.95%(17/21)、79.31%(23/29)、84.62%(33/39),特异度分别为75.00%(9/12)、91.09%(92/101)、75.00%(33/44),阳性预测值(PPV)分别为85.00%(17/20)、71.88%(23/32)、75.00%(33/44),阴性预测值(NPV)分别为69.23%(9/13)、93.88%(92/98)、96.91%(188/194),准确率分别为78.79%(26/33)、88.46%(115/130)、92.86%(221/238).结论 3.0T MR自动呼吸导航对比增强全心CMRA有助于诊断冠状动脉狭窄.