Clinical Analysis For 12 Cases With Noncompaction Of Ventricular Myocardium

Objective To investigate the clinical and ultrasonic diagnosis features of noncompaction of ventricular myocardium (NVM) patients, in order to reduce the clinical omission diagnostic rate and misdiagnosis, and to raise the awareness and treatment level of noncompaction of ventricular myocardium, to prevent and treat it early.Methods The medical records of 12 hospitalized patients with noncompaction of ventricular myocardium diagnosed by echocardiography (UCG), which were in the First Affiliated Hospital of Guangxi Medical University from September 2007 to October 2009 , were analyzed retrospectively , summarized noncompaction of ventricular myocardium's clinical and imaging features ,and the way of diagnosis and treatment . In addition, we elected 12 cases with normal results of echocardiography at the same term as a control group. we analyzed and compared with the left ventricular systolic function parameters of the of echocardiography results between two groups of patient, and reviewed relevant literature and discussed it.Results In this study, there were a total of 12 patients, 8 males and 4 females, aged 4 to 53 years old, an average of 32.08±19.41 years, males accounted for 66.7%, women account for 33.33%. NVM accounted for 3.23% of primary cardiac disease during the same period. The main clinical manifestation of NVW was charactered with heart failure , cardiac arrhythmia and thromboembolism. 11 patients showed varying degrees of heart failure. 8 patients had palpitations, 1 case with cerebral infarction, 1 case with transient ischemic attack. chest X-ray examination of 12 cases revealed enlargement of the heart in varying degrees. ECG showed abnormal, in which ST-T changes in 8 cases, intraventricular conduction block in 5 cases, ventricular arrhythmia in 4 cases. 1 case for 24-hour Holter shows frequent premature ventricular bigeminy, 1 case for cardiac electrophysiology examination revealed atrial tachycardia. All patients come up to the diagnostic criteria of noncompaction of ventricular myocardium by echocardiography, of which 10 cases'lesions were confined to left ventricle, accounting for 83.33%, 2 cases'left and right ventricular were involved at the same time, nine cases involving the apex, accounting for 75.00 %, 2 cases involving the free wall, 1 case involving the interventricular septum. Thick trabecular muscles and deep-set were seen in all patients, forming of network structure. Between the cryptes , there were bleak and slow blood flow connect with cardiac chamber . disorders were divided into two layers, in which the ratio of adult non-compaction of myocardium and dense myocardium ranged from 2.0 to 3.4, with an average 2.27±0.46, the ratio ranged from 1.6 to 2.2 in children. Regional wall motion analysis of 11 patients showed diffuse wall motion weakened, left ventricular ejection fraction of five cases were less than 30%, accounting for 41.67%, left ventricular ejection fraction of six cases were 30% ~ 50%, and accounted for 50%. Comparing with the normal control group, the average ages were close , P>0.05, there was no significant difference. Comparing the Indicators Reflecting the left ventricular systolic function including the left ventricular end-diastolic diameter (EDV), left ventricular ejection fraction (LVEF), and left ventricular internal diameter shortening (FS), P <0.05, the difference was statistically significant. 1 case for cardiac magnetic resonance (CMR) examination, 1 case for cardiac CT scan plus enhanced MRI ,both of wich were in line with NVM echocardiographic characteristic changes. Follow-up time is 20 to 40 months, with an average follow-up time was 24.86±14.28 months, in 12 patients one cases died during hospitalization, 1 patient died outside the hospital, 2 patients lost to follow, and 1 case heart failure was still recurring, 1 patient symptom was improved, but there is still palpitation, another six cases congestive heart failure symptoms were improvement than before. Conclusion Incidence of noncompaction of ventricular myocardium is low and rare in clinic. Males predominance. For progressive heart failure, arrhythmia, systemic embolism as the main clinical manifestations. Echocardiography is the most important means of inspection and confirmed diagnosis, the heart MRI is its important complement. Patients have high rates of death and sudden death. progressive heart failure and malignant arrhythmia are leading causes of death. Drug treatment can improve symptoms. can reduce mortality. Early diagnosis, heart transplantation, implantation of implantable automatic defibrillators and a screening of relatives can improve the prognosis.