Clinical Characterization And Prognostic Factors Of 33 Patients With Noncompaction Ventricular Myocardium

Background:Non-compaction of ventricular myocardium(NVM),a rare genetic form of cardiomyopathy,is characterized by prominent ventricular trabeculations and deep intertrabecular recesses,or sinusoids,in communicatio n with the ventricular cavity.It can be categorized as various forms,in which the most common type is left ventricular non-compaction cardiomyopathy.Clinical presentations are variable from asymptomatic to symptomatic,but usually displays as a triad of heart failure,arrhythmias,and embolic events from mural thrombi.Echocardiography is common technology for diagnosing NVM and differentiating it from the hypertrophic cardiomyopathy,dilated cardiomyopathy,endocardial fiborelastosis.Treatments depend on the clinical manifestations,and transplantation for prevention of sudden death is mainstay of therapy when deemed necessary and appropriate.The prognosis for NVM is variable,and the asymptomatic patients have a relative improved the clinical outcome and prognosis.Objective:The purpose of this study was to investigate the progress of the study on non-compaction of ventricular myocardium(NVM)in children and to collect relevant clinical data for the preliminary analysis of prognostic risk factorsPopulation and Methods:From January 2009 to January 2019,33 cases of NVM children hospitalized in the department of pediatric of the first hospital of jilin university were selected and some of them were followed up.Relevant clinical data were collected and summarized.Kruskal-Wallis test and ordered Logistic regression analysis were used to analyze the prognostic factors.Results:Among the 33 patients,16 were males and 17 were females.The onset age was between 2 days and 16 years old,and there were 4 patients(14%)with a clear family history of heart disease,there were 1 case of myocardiopathy and 1 case of myocardial hypertrophic trabeculae of the father,and 2 cases of ventricular septal defect of the sister.Cardiac insufficiency,respiratory infection and digestive disorders were the main reasons to see adoctor.Among them,20 cases(61%)received routine or dynamic electrocardiogram,and the results showed that 95% of them had abnormal electrocardiogram,mainly including st-segment or t-wave changes,ventricular arrhythmia,p-wave high peak,atrial premature beat or atrial tachycardia.All the children were diagnosed by echocardiography,including 26 cases(79%)with isolated NVM and 7 cases(21%)with other congenital heart diseases,including 3 cases with ventricular septal defect,2 cases with atrial septal defect and 1 case with PDA.There were 30 cases(91%)of left ventricular type and 3 cases(9%)of double ventricle type.all the children examined chest radiography,and results: 30 cases(91%)had enlarged heart shadow,0.58-0.86 heart chest ratio,and 15 cases(45%)with pneumonia.a kid's genetic examination showed MYPN and AGL gene mutations.A kid's tandem mass spectrometry revealed increased palmitoenylcarnitine,octadecanoenylcarnit ine,and C3/Met.There were 2 cases of death during the first hospitalization,2 cases of transfer,24 cases of clinical manifestations and cardiac function improved after treated,the later follow-up,11 cases(33%)were lost to follow-up,9 cases of improvement,6 cases of no remission,2 cases of aggravation,3 cases of death,the cause of death were heart failure or combined respiratory failure.22 possible prognostic factors were selected for univariate analysis,and the results showed that NC/C,cardiac function grading,ALT and CKMB quality were statistically significant prognostic factors(P < 0.05).No statistically significant independent influencing factors were obtained by Logistic regression analysis.Conclusion:1.The clinical manifestations of non-comonpaction of ventricular myocardium are diverse,non-specific,and prone to be combined with other types of congenital heart disease,some of which have family history.2.non-comonpaction of ventricular myocardium requires attention to the possibility of metabolic diseases.3.Strengthen clinical and ultrasound physicians' understanding of NVM,and suggest to check CMR as far as possible after the exclusion of contraindications.4.Low-dose aspirin after diagnosis can reduce the incidence of thrombosis to some extent.5.The prognosis of NVM is better than before,and partial cure is possible.6.Small infants with clinical have symptoms poor prognosis.7.NC/C? NHYA?ALT and CKMB mass are prognostic factors for NVM.