Clinical Characteristics Of Arrhythmia And Prognosis In Adult With Isolated Left Ventricular Noncompaction Cardiomyopathy

Research BackgroundLeft ventricular non-compaction cardiomyopathy(LVNC)is a relatively rare hereditary cardiomyopathy characterized by prominent trabeculae and the formation of deep intratrabecular recesses.Since LVNC was first named in 1990,people have made some progress in the diagnosis of the disease in the past 30 years,but there are still many unknowns about its pathogenesis,treatment and prognosis.At present,there are relatively few studies on large cases of LVNC.Thus,a single-center clinical analysis with a large size of sample is not only of great significance to improve the cognition of the disease,but to continuously summarize and improve the management of LVNC as well.ObjectiveThis study aims to summarize the clinical symptoms,laboratory examinations,functional examinations and imaging findings of isolated LVNC patients based on the retrospective analysis of a large sample of LVNC from the National Center of Cardiovascular Disease,Beijing Fuwai Hospital.Focusing on the analysis of the characteristics of arrhythmia in patients with isolated LVNC,the risk factors of malignant arrhythmia,and possible prognostic factors of isolated LVNC.Methods and materialsA total number of 255 adult who met Jenni's echocardiography standards and/or Petersen's cardiac magnetic resonance imaging standards of the diagnosis of LVNC were admitted to Beijing Fuwai Hospital from May 2011 to January 2021 were continuously included.Collect clinical information and conduct regular follow-ups,and perform statistical analysis such as chi-square test,mean test,regression analysis,receiver operating characteristic curve,etc.on the collected clinical data.ResultsThe average age at diagnosis of LVNC patients was 45.4 years,with a male to female ratio of 1.87:1.The first three common clinical symptoms of LVNC were dyspnea(82.4%),palpitations(54.9),and lower limb edema(25.5%).96.4%of the patients had electrocardiogram(ECG)abnormalities,66.7%of the patients had left ventricular systolic dysfunction,and 9.8%of the patients had cardioembolism.During a median follow-up of 4.3 years,43 patients(18.1%)died and 41(17.2%)among them were cardiac deaths.15(6.3%)received heart transplants.The 5-year survival rate of LVNC was 71%.The independent risk factor for malignant arrhythmia in LVNC patients is large endothelin-1(OR 3.18,95%CI 1.44-7.04,P=0.004),the predicted cut-off value of it was 0.335 pmol/L(sensitivity 74.5%,specificity 61.0%).The independent poor prognosis risk factors of LVNC were NT-proBNP(adjusted HR 2.39,95%CI 1.51-3.76,P<0.001)and positive late gadolinium enhancement(HR 7.56,95%CI 1.91-29.97,P=0.004).ConclusionsAmong Chinese adult patients with isolated LVNC,males have a higher proportion than females.Heart failure and arrhythmia are the most two common clinical manifestations of isolated LVNC.Isolated LVNC patients have a variety manifestation of arrhythmias,which ventricular arrhythmia is most common.Serum big endothelin-1 level is a risk factor for malignant arrhythmia in isolated LVNC patients.Ventricular fibrosis and left ventricular disfunction predict the adverse outcome of isolated LVNC.Serum big endothelin-1 level,NT-proBNP level and positive late gadolinium enhancement have certain reference value for the management of isolated LVNC.