Left Ventricular Non Compaction : Epidemiology, Clinical Findings, Diagnosis and Therapy

Date: 
Wednesday, December 5, 2018

Left Ventricular Non Compaction : Epidemiology, Clinical Findings, Diagnosis and Therapy

Prof. Dr. Ahmed K. Motaweih

M.Sc. Medicine &Cardiology- M.D Cardioloy & Vasc. Dis .Porf.& Head of Cardio - Vasc. Medicine Dept. Faculty of Medicine Azhar Univ. F.E.S.C & F.A.C.C.

Non compaction of the left ventricle is a genetic cardiomyopathy caused by an embryogenesis defect which determines a non correct compaction of the myocardium and consequently

The persistence of a two layered structure with a wide spongy stratum and a thin compacted stratum. Implicated genetic mutations include mitochondrial, sytoskeletal, Z-line and sarcomeric proteins. The disease is rare, however it is getting more and more recognized due to the increase awareness of the cardiologist and the improvement of imaging technologies. Clinical manifestations include heart failure symptoms, thromboembolic events and arrhythmias. Patients can also be asymptomatic, being occasionally diagnosed during family screening.

Echocardiography is the first line diagnostic tool, allowing the visualization of trabeculae separated by deep recesses and measurement of the compacted/non compacted layer ratio. New technologies such as contrast echocardiography, speckle tracking and 3D echocardiography could improve diagnostic accuracy of conventional echocardiography. Cardiac MRI is a very useful adjunctive technique allowing a more precise quantification of the spongy tissue and giving prognostic

Information.

Prognosis was traditionally considered poor, however recent study showed that it is better than previously reported, being the worst prognostic marker the occurrence of symptoms. A close monitoring of patients by the cardiologist is strongly warranted in all cases.

A close monitoring of patients by the cardiologist is strongly warranted in all cases

 

Left Ventricular Non Compaction : Epidemiology, Clinical Findings, Diagnosis and Therapy