Abstract

Left Ventricular Noncompaction (LVNC) is a genetic cardiac disease of emerging importance with a distinct clinical and pathophysiological presentation. The most common clinical manifestations include heart failure, arrhythmias and embolic events, and in children it may be associated with facial dysmorphisms and Wolff-Parkinson-White syndrome. The diagnosis of LVNC, however, is often missed, most often as a consequence of ignorance of the condition. Echocardiography is considered the reference standard for the diagnosis of LVNC. Prognosis remains poor for patients with impaired systolic left ventricular function, as treatment options are very limited. Because of the familial association of LVNC, first-degree relatives should be screened by Echocardiography.

Highlights

  • The persistence of prominent myocardial trabeculation, seen in the normal right ventricle, does not usually occur after birth in the left ventricle.[1]

  • During the early embryonic phase, the left ventricular myocardium consists of a loose network of fibres separated by deep recesses that link the myocardium with the left ventricular cavity

  • Malla et al Left Ventricular Noncompaction died of acute left heart failure, an autopsy proved for the first time the diagnosis of persistent sinusoids, today called LVNC.[8,9]

Read more

Summary

INTRODUCTION

The persistence of prominent myocardial trabeculation, seen in the normal right ventricle, does not usually occur after birth in the left ventricle.[1] During the early embryonic phase, the left ventricular myocardium consists of a loose network of fibres separated by deep recesses that link the myocardium with the left ventricular cavity. Gradual compaction of these fibres and intertrabecular recesses usually occurs within 8 weeks of life, progressing from the epicardium to endocardium and from the base of the heart to the apex.[1]. Malla et al Left Ventricular Noncompaction died of acute left heart failure, an autopsy proved for the first time the diagnosis of persistent sinusoids, today called LVNC.[8,9]

CLASSIFICATION OF CARDIOMYOPATHY
CLINICAL MANIFESTATIONS
TREATMENT OPTIONS
CONCLUSION
Findings
Resonance:A Mini Case
Full Text
Paper version not known

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call

Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.