ABSTRACT
Non-compaction of ventricular myocardium (NVM),a rare congenital and inherited cardiomyopathy,is characterized by prominent trabeculations and deep intertrabecular recesses in communication with the ventricular cavity.NVM has diverse clinical presentations without specificity,of which the major characteristics are cardiac insufficiency,arrhythmias and thrombosis.Echocardiography and cardiac magnetic resonance imaging are necessary technologies to diagnose NVM.Symptomatic therapy is the only choice for most patients.Furthermore,if medical treatment fails,the heart transplantation could be performed.As a whole,NVM has a high mortality and entirely different prognosis,however,the prognosis of asymptomatic patients is relatively good.
ABSTRACT
Non-compaction of ventricular myocardium (NVM), a rare congenital and inherited cardiomyopathy, is characterized by prominent trabeculations and deep intertrabecular recesses in communication with the ventricular cavity.NVM has diverse clinical presentations without specificity, of which the major characteristics are cardiac insufficiency, arrhythmias and thrombosis.Echocardiography and cardiac magnetic resonance imaging are necessary technologies to diagnose NVM.Symptomatic therapy is the only choice for most patients.Furthermore, if medical treatment fails, the heart transplantation could be performed.As a whole, NVM has a high mortality and entirely different prognosis, however, the prognosis of asymptomatic patients is relatively good.
ABSTRACT
Objective To study the clinical characteristics and curing methods of non-compaction of ventricular myocardium(NVM) in children with a view to improve its diagnosis and treatment.Methods From Jan.2009 to Dec.2012,the clinical data,treatment and follow-up of 22 patients with NVM in Beijing Children's Hospital Affiliated to Capital Medical University were analyzed.Results The 22 patients included 13 boys and 9 girls aged from 4 months to 13 years and 3 months with an average age of 3 years and 7 months.Five of them had a family history of cardiac diease.Twenty patients suffered from ventricular dysfunction,with 2 cases at level Ⅰ,5 cases at level Ⅱ,7 cases at level Ⅲ and 6 cases at level Ⅳ.Asymptomatic heart murmur was heard in 6 patients and creatine kinase car-diac isoenzymes raise was found in 6 patients.X-ray indicated that heart shadow enlargement and electrocardiographic abnormalities in all 22 patients.Echocardiography features of all cases indicated left ventricular enlargement,and 20 cases of the children had ventricular systolic dysfunction.Left ventricle was detected in 21 patients,while double ventricle dysfunction detected in 1 patient.Thrombus was found in none of the patients.Magnetic resonance imaging was performed in 8 cases of the patients and CT was performed in 14 cases,all of them showed abnormalities.Anti-heart failure medications were administered to 20 patients and aspirin to all of the children.The patients were followed up for 0.5 to 3.0years.Two patients died from heart failure during the follow-up.In 2 cases whose cardiac ejection fraction was more than 60％ had little change of the internal diameter of left ventricle and cardiac function.In 3 cases,the left ventricular diameter narrowed by 3 to 5 millimeter and ejection fraction increased by 5％ to 10％ over the previous.In 5 cases,the left ventricular diameter expanded by 5 to 7 millimeter and ejection fraction decreased by 3％ to 5％.In 10 cases,the left ventricular diameter and heart function had no significant change.Conclusions The main clinical manifestions of NVM in children are cardiac dysfunction,and the prognosis is usually poor.Echocardiography is the principal means of diagnosis.Magnetic resonance imaging can contribute to the diagnosis.
ABSTRACT
Objective To explore the echocardiography characteristics of non-compaction of ventricular myocardium(NVM),and assess its value in the diagnosis of NVM.Methods Philips SONOS 5500 color Doppler ultrasound system with 2-4 MHz transducers was used in 9 patients with NVM.Results In all 9 patients,the presence of multiple trabeculations and multiple deep intertrabecular Space communicating with the ventricular cavity was demonstrated by color Doppler imaging.Both ventricles were involved in 1 patient,only the left ventricle in 7 patients,and right ventricle in 1 patient.Conclusions NVM has typical ultrasonic appearance on echocardiography.It Can find out the NVM manifestations even if patients do not have any symptoms.It is a reliable and convenient way for diagnosing NVM.