小儿心肌致密化不全的研究

发布时间：2018-05-12 21:16

本文选题：心肌致密化不全 + 先天性心脏病　；参考：《山东大学》2014年博士论文

【摘要】：背景心肌致密化不全(Noncompaction of the ventricular myocardium, NVM)是一种罕见的特殊类型的先天性心室肌发育不全性心肌病,目前病因尚不明确,病变主要累及心脏左心室或(和)右心室,主要特征为左心室或(和)右心室腔内存在大量粗大突起的肌小梁及深陷隐窝,可合并其他心脏畸形(congenital heart disease, CHD),并导致一系列相关的临床症状,其症状主要为心功能不全、心律失常和各系统栓塞,但其症状的首发年龄差别很大,而且临床表现各异,预后差别也较大。到目前为止,国内外仅有少量文献报道,且各方面报道结果差异较大。随着人们对本病认识的加深和成像技术的不断提高,近年来越来越多的心肌致密化不全被报道,文献主要以个案报道为主,少数病例报道病例数在10例-50例之间,50例以上的病例报道也不过2-3篇。再加上心肌致密化不全分型复杂也较为模糊,又使得较大样本报道的有效性大大降低,报道从临床症状、心电图异常率、栓塞发生率到死亡率等等差别比较大,譬如死亡率从2%到60%不等,这对于本病的认识造成非常的不利影响。心肌致密化不全合并先天性心脏发生率可达78%,越来越多的心肌致密化不全合并先天性心脏病患者被发现,其手术治疗和介入治疗方法的疗效观察尚不多见,主要是个案报道。尽管人们对心肌致密化不全的研究付出了很大的努力,但是目前病因尚不明确,发病机制也不是很清楚,治疗方法多是对症治疗和心脏移植。解决以上问题,将会使人们对本病的认识向前大大推进。研究目的由于心肌致密化不全尚存在以上问题,本研究的研究目的就是通过研究大型心脏中心的病历资料并对其进行相关分析,解决以上问题。1、明确心肌致密化不全的临床特点及各分型的特点,包括发病率、症状、体征、心电图、心脏彩超、治疗及预后情况,找出心肌致密化不全不良预后的危险因素。2、报道心肌致密化不全合并先天性心脏病的外科手术治疗和介入治疗的效果,明确手术治疗和介入治疗是否有效。3、研究心肌致密化不全的发病机制和探讨治疗方法。研究方法收集2009年4月至2011年10月间山东省立医院0-18岁的所有诊断心肌致密化不全的患者的病历资料,包括住院电子病历资料、门诊电子病历资料、随访资料,超声数据库。收集内容并登记,一般资料：姓名、性别、年龄、诊断、心功能分级,症状、体征、治疗方法、治疗效果等；治疗方法包括药物治疗、介入治疗和手术治疗。症状包括胸闷、憋气、心慌等；体征包括心脏杂音、呼吸困难、水肿、发育迟缓、特殊面容等。胸片资料：计算心胸比率。记录心电图和24小时心电图资料。心脏彩超资料：左室舒张末内径,心功能指数,缩短分数,心肌致密化不全累及部位,合并其他心脏畸形及超声继发改变等情况。随访资料：所有病人在首次就诊3、6、12月后门诊进行随访,其后每年进行随访。随访内容包括：症状、体征、病史,体格检查、心功能分级,胸片、心脏彩超、心电图和24小时心电图。随访至2012年4月31日,完成率100%。所有资料收集后录入Excel表,然后在SPSS16.0建库进行统计学分析。解决以上三个问题需对以下6个方面进行研究。1、通过描述的方法描述全部患儿的整体特征和各个部分的特征描述,包括：一般性描述、合并先心病特征、心电图特征、致密化不全分布特点。通过与其他小儿心肌致密化不全的研究进行比较,明确本研究的有效性；通过与其他成人心肌致密化不全的研究比较,找出小儿心肌致密化不全与成人心肌致密化不全的区别,结果采用Fisher's精确检验或χ2检验。对本组合并先心病和孤立性心肌致密化不全进行比较,判断两种情况的差别,采用Pearson chi-square法或者Fisher's精确检验。2、相关死亡和(或)反复心衰的危险因素分析,采用Kaplan-Meier曲线及Log Rank检验。包括以下几个方面的检验：致密化不全累及部位数量、性别、年龄(1岁)、有无症状、有无严重心律失常、EF(50%)。3各治疗组治疗效果的观察,包括手术组治疗效果观察(手术方法及病人特征、心脏彩超结果、心电图发现、胸部x-线发现、临床数据、手术结果、术后早期结果、术后6月结果、术后远期结果、手术组危险因素筛选),介入组治疗结果(病人特征、术后6月患儿的EF,CTR和NYHA分级),孤立性NVM的药物治疗加心律治疗结果。4三个治疗组免除死亡和反复心衰的生存情况。5手术效果进一步评价。6合并先天性心脏病与致密化不全部位的关系。使用SPSS16.0进行统计分析,计量资料用均数±标准差表示,分类变量使用计数和百分比表示,全部47例患者危险因素分析采用Kaplan-Meier曲线配合Log Rank检验逐个因素检验进行直观表达,部分变量采用单因素分析。时间和时间相关事件使用图标和Kaplan-Meier曲线表示,在首诊6个月时心功能指数、心胸比率、心功能分级结果采用t检验,分析挑选出来的危险因素使用Fisher's精确检验,与其他组进行比较使用Fisher's精确检验或胞检验。P值0.05为有统计学意义。 [结果] 结果1：2009年4月至2011年10月,确诊NVM患儿47例,占同期超声心动图检查患儿的0.17%,占心肌病的12.2%,占先天性心脏病的1.1%。诊断时年龄3.88±3.76岁(1月-12岁),小于1岁19例,占总病例的40.43%,合并其他先天性心脏畸形37例,占NVM的78.72%,孤立性NVM10例,男性略多于女性55.3%：44.7%；《1LV：BV：RV比例为34：9：4(72.3%：19.1%：8.6%)：所有患者平均LVEF为58.94%(20%-78%)其中LVEF500%34例,占72.34%,LVEF≤50%者13例,占27.66%；心胸比率大于55%占76.6%：其中心脏重度扩大17例,占36.2%。心电图异常38例,占80.9%；合并严重心律失常10例,占21.3%；有自觉症状病例24例,占51.1%,23例无症状,占总数的48.9%。特殊面容2例,无严重栓塞病史,病历未记载家族特性。外科手术治疗16例,介入治疗18例,药物治疗13例。随访1.83±0.90年,恢复良好30例(62.5%),心衰复发4例(8.3%),反复心衰5例(10.4%),死亡8例(16.7%)。心肌致密化不全几乎可合并所有类型先天性心脏病动脉,导管未闭和室间隔缺损是最常见的心脏畸形,发生率分别为59.4%和27%。心电图异常发生率80.9%,主要为ST-T改变,异常Q波；严重心律失常发生率为21.3%。心肌致密化不全最常见发生部位为左室尖,发生率为76.6%,其他部位依次为左室中段后壁48.9%,左室中段侧壁34.0%,室间隔中段25.5%,右室尖23.4%,右室侧壁6.4%。与其他小儿NVM研究的比较结果显示：小儿心肌致密化不全临床特征相对稳定；与成人NVM在心电图改变方面存在差异。本组合并先心病和孤立性NVM在多个方面存在差异,可能与样本选择有关。结果2：心肌致密化不全累及范围(P0.01)、EF(P0.01)、合并严种心律失常(P=0.038)、有临床症状(P0.01)是死亡和反复心衰的危险因素,年龄(P=0.27)、性别(P=0.94)不是。结果3：手术组：手术死亡2(12.5%)例,术后6月,EF没有明显改善(61.77±12.29%vs64.85±6.07%,P=0.207),但是NYHA心功能分级改善明显(2.38±0.89vs1.62±0.65,P=0.009),心胸比率显示心脏有缩小(P0.001)。EF50%为手术组危险因素(P=0.018)。介入组患儿病情轻,效果良好,无远期死亡,随访效果良好。孤立性NVM药物治疗组病情重,NVM累及范围较广,平均累及2.7个部位；合并较严重心律失常5例,EF较低,为44.2±14.6%,7(70%)例小于50%；心脏扩大明显,CTR为0.67±0.05,7例≥0.65,70%重度心脏扩大。死亡4例,反复心衰心功能持续下降2例,心功能无恶化或改善4例。结果4：三组生存曲线存在差别。与样本选择有关。结果5：对手术效果进一步评价发现,手术能够有效免除有症状的NVM合并先心病的死亡和反复心衰的发生率(P=0.037)。结果6：致密化不全发生部位与先天性心脏病发生部位存在关系。结论小儿心肌致密化不全临床特征相对稳定,以往报道差异与样本选择有关,小儿心肌致密化不全与成人心肌致密化不全在心电方面存在差异。心肌致密化不全累及范围、EF50%、合并严种心律失常、有临床症状是死亡和反复心衰的危险因素。手术和介入治疗可有效改善预后,心律失常的治疗也可改善预后。心脏微循环障碍可能是发病基础,致密化不全发生部位与先天性心脏病发生部位存在关系。心肌致密化不全的研究还需要各方面进一步研究进行统一、达成共识。
[Abstract]:background
Noncompaction of the ventricular myocardium (NVM) is a rare and special type of congenital cardiomyopathy with congenital ventricular myogenesis. The cause of the disease is not yet clear, and the lesions mainly involve the left ventricle or (and) right ventricle of the heart, mainly characterized by a large number of large projections in the left ventricle or (and) right ventricle. Muscle trabeculae and deep recess can merge with other cardiac malformations (congenital heart disease, CHD) and cause a series of related clinical symptoms. The symptoms are mainly cardiac insufficiency, arrhythmia and systemic embolism. However, the initial age of the symptoms varies greatly, and the clinical manifestations are different and the prognosis is different. So far, domestic There are only a few reports outside, and the results are quite different from each other.
With the deepening of people's understanding of this disease and the continuous improvement of imaging technology, more and more myocardial densification has been reported in recent years. The main cases are reported mainly by case reports. The number of cases reported in a few cases is between 10 cases of -50 cases, and more than 50 cases reported in more than 2-3 cases. Vagueness, which makes the effectiveness of large sample reports greatly reduced, and reports from clinical symptoms, abnormal rates of electrocardiogram, the incidence of embolism to mortality, and so on, such as mortality from 2% to 60%, which has a very negative impact on the understanding of the disease. The more patients with myocardial densification and congenital heart disease have been found, the effect of surgical treatment and interventional therapy is not much observed, mainly in case reports. Although great efforts have been made in the study of myocardial densification, the etiology is not clear and the pathogenesis is not very clear, treatment is not very clear. Most of these methods are symptomatic treatment and heart transplantation. To solve these problems, we will make people's understanding of this disease advance greatly.
Because of the problems of myocardial densification, the purpose of this study is to study the medical records of large cardiac centers and to analyze them, to solve the above problem.1, to clarify the clinical characteristics and the characteristics of various types of myocardial densification, including the incidence, symptoms, signs, electrocardiogram, cardiac color Doppler, treatment and treatment. To find out the risk factors for the prognosis of myocardial densification,.2, the effect of surgical and interventional therapy on myocardial densification and congenital heart disease is reported. It is clear whether surgical treatment and interventional therapy are effective.3, study the pathogenesis of myocardial densification and study the method of treatment.
The medical records of all patients with myocardial densification at the age of 0-18 in Shangdong Province-owned Hospital from April 2009 to October 2011 were collected, including in-patient electronic medical records, outpatient medical records, follow-up data, and ultrasonic databases.
Collection of contents and registration, general information: name, sex, age, diagnosis, classification of heart function, symptoms, signs, treatment, treatment effect and so on; the treatment includes drug treatment, interventional therapy and surgical treatment. Symptoms include chest distress, suffocation, and palpitation. Symptoms include heart murmur, dyspnea, dropsy, retardation, special facial features, etc. Chest data: computed cardiothoracic ratio, record ECG and 24 hour electrocardiogram data. Cardiac color Doppler data: left ventricular end diastolic diameter, cardiac function index, shortening score, myocardial densification involvement, other cardiac malformation and secondary ultrasound changes. Follow-up information: all patients were in the outpatient for the first time after 3,6,12 months. Follow up, followed up every year, followed up each year, including symptoms, signs, medical history, physical examination, heart function classification, chest film, heart color Doppler, electrocardiogram and 24 hour electrocardiogram. Follow up to April 31, 2012, complete rate of 100%. data collection, Excel form, and then statistical analysis in SPSS16.0 database.
To solve the above three problems, we need to study the following 6 aspects:.1, describing the overall characteristics and the characteristics of all the parts of the children, including the general description, the characteristics of the congenital heart disease, the characteristics of electrocardiogram, and the densification of the children. Comparing with the study of other adult myocardial densification, the difference between the densification of the myocardium in children and the densification of the adult myocardium was compared with that of the adult myocardial densification. The results were compared with the Fisher's test or the chi 2 test. Two cases were judged by the comparison of the combination of congenital heart disease and the densification of isolated cardiac muscle. The difference, using Pearson chi-square or Fisher's accurate test of.2, analysis of risk factors for related death and / or recurrent heart failure, using Kaplan-Meier curve and Log Rank test, including the following tests: densification not involved in the number of parts, sex, age (1 years), symptoms, severe arrhythmia, EF (50%).3 The observation of the therapeutic effect of the treatment group included the observation of the therapeutic effect of the operation group (surgical method and patient characteristics, cardiac color Doppler results, electrocardiogram discovery, chest x- line discovery, clinical data, surgical results, early postoperative results, June results after operation, postoperative long-term results, screening of risk factors in hand group), intervention group treatment results (patient characteristics, operation) The EF, CTR and NYHA classification of children in June, the drug treatment of isolated NVM and the result of cardiac arrhythmia,.4 three treatment groups were relieved of death and the survival of repeated heart failure. The effect of.5 operation was further evaluated in the relationship between.6 combined with congenital heart disease and densification area. Statistical analysis was performed with SPSS16.0, and the measurement data used mean standard deviation. The classification variables were expressed by counting and percentage. The analysis of risk factors in all 47 patients was expressed by Kaplan-Meier curve and Log Rank test by one by one factor test. Some variables were analyzed by single factor analysis. The time and time related events were represented by icons and Kaplan-Meier curves, and the heart function was indicated at the first 6 months of first visit. Number, cardiothoracic ratio, and cardiac function classification results were t test, and the selected risk factors were analyzed by Fisher's accurate test. Compared with other groups, Fisher's accurate test or cell test.P value 0.05 were statistically significant.
[results]
Results from April to October 2011 1:2009, 47 children were diagnosed with NVM, accounting for 0.17% of children with echocardiography, accounting for 12.2% of cardiomyopathy, accounting for 3.88 + 3.76 years of age (January -12 years) for congenital heart disease (-12 years in January), 19 cases younger than 1 years old, accounting for 40.43% of the total cases, 37 cases of other congenital heart malformations, 78.72% of NVM, isolated NVM In 10 cases, the male was slightly more than the female 55.3%:44.7%; the proportion of <1LV:BV:RV was 34:9:4 (72.3%:19.1%:8.6%). The average LVEF of all patients was 58.94% (20%-78%) in LVEF500%34 cases, 72.34%, LVEF < 50%, 13, 27.66%, and the heart rate greater than 55%, 76.6%: 38 cases of severe cardiac enlargement, accounting for 38 cases of 36.2%. ECG, 80. .9%, 10 cases with severe arrhythmia, accounted for 21.3%, 24 cases of conscious symptoms, 51.1%, 23 asymptomatic, 2 cases of 48.9%. special face, no history of severe embolism, no family history, 16 cases of surgical treatment, 18 cases of interventional therapy, 13 cases, 1.83 + 0.90 years, recovery of 30 cases (62.5%), heart failure recovery. 4 cases (8.3%), 5 cases of recurrent heart failure (10.4%), 8 cases of death (16.7%). Myocardial densification was almost combined with all types of congenital heart disease. The most common cardiac malformation was ductus arteriosus and ventricular septal defect, the incidence rate was 59.4% and 27%. electrocardiogram was 80.9%, mainly ST-T change, abnormal Q wave, and serious arrhythmia. The incidence is 21.3%.
The most common site of myocardial densification was left ventricular apex, the incidence was 76.6%, the other parts were 48.9% in the posterior wall of the left ventricle, 34% in the middle of the left ventricle, 25.5% in the middle of the ventricular septum, 23.4% in the right ventricular apex, and the comparison between the right ventricular wall 6.4%. and other children NVM showed that the clinical characteristics of the densification of the myocardium were relatively stable in children. There are differences in electrocardiogram changes with adult NVM. There are many differences between this combination, congenital heart disease and isolated NVM, which may be related to the selection of samples. Results 2: myocardial densification is not involved in the range (P0.01), EF (P0.01), combined severe arrhythmia (P=0.038), and the clinical symptoms (P0.01) are the risk factors of death and repeated heart failure. Age (P=0.27), sex (P=0.94) is not.
Results 3: operation group: Operation death 2 (12.5%) cases, after June, EF did not improve (61.77 + 12.29%vs64.85 + 6.07%, P=0.207), but NYHA cardiac function classification improved significantly (2.38 + 0.89vs1.62 + 0.65, P=0.009), cardiac ratio showed that the heart had reduced (P0.001).EF50% as the risk factor of operation group (P=0.018). Good, no long-term death, good follow-up effect. The patients in the isolated NVM treatment group were seriously ill, NVM was involved in a wide range, with an average of 2.7 parts; 5 cases with severe arrhythmia, 44.2 + 14.6%, 7 (70%) less than 50%; the heart enlargement was obvious, CTR was 0.67 + 0.05,7 cases more than 0.65,70% severe heart enlargement. 4 cases died of repeated heart failure. The cardiac function continued to decline in 2 cases, with no deterioration of cardiac function or improvement in 4 cases. Results 4: the survival curve of the three groups was different. The results were related to the selection of samples. Result 5: further evaluation of the effect of the operation showed that the operation could effectively relieve the symptoms of NVM combined with congenital heart disease and the incidence of repeated heart failure (P=0.037). Results 6: densification Location is associated with the site of congenital heart disease.
conclusion
The clinical characteristics of myocardial densification in children are relatively stable. The difference in the previous reports is related to the selection of samples. There is a difference in cardiac densification in children with adult myocardial densification in the field of electrocardiography. Myocardial densification is not involved in the range of myocardial densification, EF50%, combined with severe arrhythmia, and the clinical symptoms are the risk factors of death and repeated heart failure. Surgical and interventional therapy can improve the prognosis effectively, and the treatment of arrhythmia can also improve the prognosis. Cardiac microcirculation may be the basis of the disease. The site of the densification is related to the site of congenital heart disease. The study of myocardial densification need to be unified in all aspects and reach a consensus.

【学位授予单位】：山东大学
【学位级别】：博士
【学位授予年份】：2014
【分类号】：R725.4

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