| BackgroundOver the last decade, noncompaction of the ventricular myocardium (NVM) has been recognized as a distinct form of cardiomyopathy. It is a rare clinical condition characterized by numerous prominent intertrabecular recesses in the ventricle and is an unclassified cardiomyopathy. Clinically, this may be coupled with a loss of contractility, arrhythmias, and thromboembolisms. Ventricular noncompaction may occur in isolation (isolated NVM) or may be associated with other congenital cardiac malformations. Heart failure symptoms, varying from mild to severe, were observed in approximately half of the patients. Arrhythmias were observed in approximately40%of cases. Thromboembolisms occur in20-40%of cases. More and more NVM cases were reported with the improvement level of understanding in NVM and the improvement of imaging technology. But the literatures were mainly case report. The number of case of in few reports was10-50.50and50more cases in literatures wre rarely. It is very difficult in understanding the desease. The coexistence rate of NVM and other CHDs in pediatric patients was78%according to the study, VSD and PDA are the common type. The surgical and intervention treatment of NVM with other CHDs has been only rarely described. The pathogenesis of the desease was unclear despite the best efforts in studing the desease. Objective The aims of this study were to describe the characteristics of the desease and to describe the effects of surgical and intervention treatment and possible risk factors associated with increased mortality and to explore the pathogenesis.MethodBetween April2009and October2011,47patients were given a diagnosis of NVM and were admitted. Ten patients had isolated NVM. Thirty-seven patients (78%) had associated other CHDs.47patients,18of them received catheter interventions,13of them received medical management,16of them received surgical treatment and were enrolled in this study.A set of perioperative and follow-up data were collected prospectively for all the patients at our institution. Medical records were reviewed to document clinical presentations, including symptoms, primary diagnosis, New York Heart Association classification (NYHA), associated dysmorphic features, presence of arrhythmias, a positive family history, the surgical date and the postoperative course. Echocardiograms were analyzed to determine the ejection fraction, the location of the NVM, and features of congenital heart disease. Holter monitors and12-lead ECGs were examined for evidence of arrhythmias. Chest x-rays were also examined for the degree of cardiomegaly based on the cardiothoracic ratio. Statistical analysis was carried out with SPSS16.0software. Continuous variables are presented as median (minimum-maximum). Categorical variables are given as counts and percentages. Time-to-event summaries included graphical displays and Kaplan-Meier curves. Kaplan-Meier curves and Log Rank was used to compare two groups. The results of EF, CTR and NYHA functional class in survivors at6months after surgery were analyzed using the t-test method. To evaluate selected dichotomous risk factors, Fisher’s Exact Test was employed. While a P value of<.05was considered statistically significant. The research content is as follows: 1describe the characteristics of the desease, including the characteristics of congenital heart diseases, the characteristics of ECG, the extent of NVM, testing the effectiveness by comparing others similar studies, testing the difference by comparing others diffent studies.2studying the risk factors involved died and the recurrence of heart failure, including sex, age, the extent of NVM, EF.3the results of surgery group, intervention and drug group.4the survival of three groups5further evaluation in the effects of surgical treatment.6the relationship the range of NVM and congenital heart diseases.ResultsResult1:Between April2009and October2011,47patients were given a diagnosis of NVM, which represented0.17%of the total database;12.2%of cardiomyopathies, and1.1%of congenital heart disease. The median age at diagnosis was3.9years (rage1m to12y).19(40%) of them<1year of age. Thirty-seven patients (78%) had associated other CHDs. More men (55.3%) suffer from the desease than do women. The ratio of left ventricle to biventricular to right ventricular involved was34:9:4(72.3%:19.1%:8.6%). The median EF at diagnosis was58.94%(20to78%),34(72.34%)of them LVEF>50%,13(27.66%) LVEF≤50%.36(76%) patients had cardiac cardiomegaly according to the CTR. Severe cardiac cardiomegaly was found in17patients (36.2%).38of the47(80.9%) patients were noted to have ECG abnormalities. Serious arrhythmias were found in10(21.3%) patients. Asymptomatic:symptomatic was48.9%:51.1%.The follow up time was1.83±0.90years,8(16.7%)died,4(8.3%)recurrence of heart failure,5(10.4%) repeat heart failure,30(62.5%) recovered well. All type of CHDs can be found, patent ductus arteriosus and ventricular septal defects were the most frequent lesions, left ventricle apical was the common site involved. It accounts for76.6%, posterior wall for48.9%, lateral wall for34.0%. NVM in children and adolescents was relative stable and has significant difference compared with the NVM in adults.Result2:the range of NVM (P<0.01), EF (P<0.01), serious cardiac arrhythmias (P=0.038), symptomatic (P<0.01) were the risk factors. Age and sex were not.Result3:surgery group:two patients (12.5%) died of refractory congestive heart failure and serious fast arrhythmias after the operation. At6months after the operation, echocardiography indicated that the left ventricular ejection fraction was not significantly improved (61.77±12.29%vs64.85±6.07%, P=0.207), but NYHA functional class was significantly improved (2.38±0.89vs1.62±0.65, P=0.009). Chest x-ray indicated that the cardiothoracic ratio was significantly reduced compared to before the operation (P<0.001). EF<50%was the risk factor. Intervention group:no deaths occurred and follow-up was good. The INVM drug group:4(40%) of them was died, and2(20%) repeat heart failure,2(20%) recurrence of heart failure2(20%) recovered well.Result4:survival rate was significant differences in three groups.Result5:Surgery for NVM with other CHDs can be effective in relieving heart failure, improving heart function and decreasing heart cavity. Arrhythmias should be treated with an active approach during the operation.Result6:there are relationship in the location of NVM and CHD.ConclusionNVM in children and adolescents was relative stable and has significant difference compared with the NVM in adults. The range of NVM (P<0.01), EF (P<0.01), serious cardiac arrhythmias (P=0.038), symptomatic (P<0.01) were the risk factors. Age and sex were not. Surgery and intervention for NVM with other CHDs can be effective in relieving heart failure, improving heart function and decreasing heart cavity. Arrhythmias should be treated with an active approach during the operation. Microcirculation disturbance may be one of the reasons of NVM and there are relationship in the location of NVM and CHD. |
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