| Background: Noncompaction of ventricular myocardium is a rare congenital hereditary cardiomyopathy,which is characterized by prominent,thick ventricular trabeculae and deep trabecular spaces,recesses or sinus spaces connected with the cardiac chamber.And there is no evidence to show that it is connected with the epicardial coronary artery system.When it is not associated with other heart diseases,it is called isolated incomplete densification of myocardium.The clinical manifestations are different,often characterized by progressive cardiac insufficiency,arrhythmia and thromboembolism.Cardiac ultrasound and magnetic resonance imaging are of great significance in its diagnosis.The treatment is still symptomatic,which can be referred to dilated cardiomyopathy.A number of studies have shown that NVM in children is often associated with congenital heart disease,and some studies have concluded that NVM in children with congenital heart disease may not have myocardial dysfunction itself.It is a simple morphological change of secondary myocardial compaction insufficiency.However,the above point of view is lack of research support of a large sample.Recent studies on noncompaction of ventricular myocardium have shown that the prognosis of patients is generally better than that shown in the early stage.However,the factors affecting the prognosis are not very clear and lack of related research,which need to be further discussed.Objective: The purpose of this study is to collect the relevant clinical data of NVM in children in order to enhance the understanding of it and to compare the clinical characteristics of incomplete compaction of myocardium with congenital heart disease and isolated noncompaction of myocardium.Finally,it can provide some reference basis for the diagnosis and prognosis of patients with incomplete densification of myocardium in the future.Research objects and methods: Forty-five children with NVM who were hospitalized in the affiliated Children’s Hospital of Chongqing Medical University from May 2015 to October 2019 were selected,and some of them were followed up.The clinical data were collected,collated and summarized.The prognostic factors were analyzed by independent sample rank sum test and ordered Logistic regression analysis.Results: Among the 45 cases diagnosed as incomplete densification of myocardium,there were 38 cases of left ventricular type,2 cases of right ventricular type and 5 cases of double ventricular type.And among the 45 cases,3 cases were discharged automatically,and the rest were discharged from hospital after clinical improvement.11 cases lost follow-up during the follow-up after discharge.The median age of first visit was 6 months,the minimum age was 2 days,the maximum age was 14 years old and 6 months,and the average follow-up time was 2 years.There were 20 males(44.4%)and 25 females(55.6%).The main clinical symptoms were respiratory infection such as cough and shortness of breath,cardiac insufficiency such as hyperhidrosis and decreased activity tolerance.The main signs were heart murmur and right ventricular insufficiency such as hepatomegaly and jugular vein filling(especially hepatomegaly).Among them,cardiac insufficiency is mainly III and IV cardiac function.Secondary respiratory tract infection is more obvious in children with NVM with congenital heart disease than in children with isolated NVM,while cardiac insufficiency in children with isolated NVM is more serious than that in children with NVM with congenital heart disease.The results of laboratory tests show that: CKMB increased in 6 cases(15%),troponin I increased in 21 cases(52.5%),BNP increased in 20 cases(55.6%),serum creatinine increased in 5 cases(11.4%),ALT increased in 10 cases(22.2%),AST increased in 16 cases(35.6%).Among them,the myocardial damage in children with isolated noncompaction of myocardium was more serious than that in children with NVM complicated with congenital heart disease,but there was no significant difference in ALT,AST and blood muscle.Electrocardiogram examination was performed in all 45 cases.The results showed that 41 cases(91.1%)had abnormal ECG,and their ECG manifestations were different,including ventricular extrasystole,T wave and ST-T changes,sinus tachycardia,left ventricular high voltage,tachycardia and so on.Compared with the ECG findings of isolated NVM children and NVM children with congenital heart disease,isolated myocardial compaction insufficiency is more likely to appear conduction abnormalities and ST-T changes.The results of color Doppler ultrasound showed that there were 38 cases of left ventricular type,2 cases of right ventricular type and 5 cases of double ventricular type.The most common site of incomplete densification was apical region in 37 cases(82.2%).Others included lateral wall,especially left ventricular lateral wall in 31 cases(68.9%),left ventricular inferior wall in 29 cases(64.4%),left ventricular posterior wall in 7 cases(15.6%),anterior wall in 5 cases(11.1%),anterior septum in 5 cases(11.1%),right ventricular free wall in 5 cases(11.1%),and interventricular septum in 3 cases(6.7%).Mitral and tricuspid regurgitation are more common in children with isolated NVM and NVM with congenital heart disease,but pulmonary hypertension is more likely to occur in children with NVM with congenital heart disease.All the 45 cases were treated with drugs,including 6 cases of interventional operation and 7 cases of surgical treatment.Most of the patients were treated with digoxin,spironolactone and hydrochlorothiazide diuretic,benazepril or captopril to reverse ventricular remodeling,only carnitine and levocarnitine to protect heart.Based on the analysis of the possible prognostic factors,it was found that the increase of ALT,cardiac function grade,high or prolonged QT were statistically significant prognostic factors,but not independent risk factors for the prognosis of myocardial compaction insufficiency.High or prolonged QT and elevated serum creatinine are statistically significant prognostic indicators in children with NVM complicated with congenital heart disease.High or prolonged QT and left ventricular posterior wall thickness are statistically significant prognostic indicators of isolated noncompaction of myocardium.Conclusion:(1)The early clinical manifestations of myocardial densification insufficiency are not specific,and the main clinical manifestations are cardiac insufficiency and arrhythmias.Thromboembolic events are relatively rare and rare in children.(2)Children with NVM are often complicated with congenital heart disease,and children with NVM with congenital heart disease have more significant secondary respiratory infection and more common right ventricular insufficiency,so it is necessary to guard against the possibility of congenital heart disease in children with pulmonary infection and right ventricular insufficiency as the main manifestations.(3)The left ventricular insufficiency in children with isolated NVM is more serious than that in children with NVM with congenital heart disease,which is considered to be related to the secondary structural changes of uncompacted myocardium in children with NVM with congenital heart disease.(4)Among the ECG abnormalities in children with NVM,tachyarrhythmia is more common than bradyarrhythmia and has characteristic manifestations such as preexcitation syndrome.Isolated NVM is more likely to show abnormal conduction and ST-T changes than NVM with congenital heart disease.(5)In this study,through the analysis of the prognostic factors that may affect myocardial compaction insufficiency,it was found that the increase of ALT,high or prolonged QT and cardiac function grade were statistically significant factors for prognosis,but they could not be used as independent prognostic risk factors for NVM.High or prolonged QT and elevated blood muscle are statistically significant prognostic factors in children with NVM complicated with congenital heart disease.High or prolonged QT and left ventricular posterior wall thickness are statistically significant prognostic indicators of isolated myocardial noncompaction without congenital heart disease. |
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