Left ventricular noncompaction: a new form of heart failure.
In this article the newly classified cardiomyopathy known as left ventricular noncompaction is discussed. This genetic inherited form of heart disease has substantial risk of heart failure, stroke, metabolic derangement, arrhythmias, and sudden cardiac death. The disorder seems to occur because of an arrest of the normal process of development, and the genes identified to date seem to encode for cytoskeletal or sarcomeric proteins. These features are outlined.
Towbin
Jeffrey A
JA
The Heart Institute, Pediatric Cardiology, Heart Failure, Cardiomyopathy & Heart Transplant Service, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, USA. jeffrey.towbin@cchmc.org
D048868:Adaptor Proteins, Signal Transducing / Q000235:genetics; D000818:Animals; D001145:Arrhythmias, Cardiac / Q000150:complications; D056889:Barth Syndrome / Q000150:complications; D009202:Cardiomyopathies / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D002352:Carrier Proteins / Q000235:genetics; D004272:DNA, Mitochondrial / Q000235:genetics; D004195:Disease Models, Animal; D004452:Echocardiography; D004562:Electrocardiography; D006330:Heart Defects, Congenital / Q000150:complications; D006352:Heart Ventricles / Q000473:pathology; D018398:Homeodomain Proteins / Q000235:genetics; D006801:Humans; D015994:Incidence; D007625:Kearns-Sayre Syndrome / Q000150:complications; D060588:LIM Domain Proteins; D017243:MERRF Syndrome / Q000150:complications; D019028:Magnetic Resonance Imaging, Cine; D017240:Mitochondrial Myopathies / Q000150:complications; D009135:Muscular Diseases / Q000235:genetics; D009154:Mutation; D009206:Myocardium / Q000166:cytology; D050778:NFATC Transcription Factors / Q000235:genetics; D058570:TOR Serine-Threonine Kinases / Q000235:genetics; D018487:Ventricular Dysfunction, Left / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*
D048868:Adaptor Proteins, Signal Transducing; D002352:Carrier Proteins; D004272:DNA, Mitochondrial; D018398:Homeodomain Proteins; C120696:LDB3 protein, human; D060588:LIM Domain Proteins; C485639:Ldb3 protein, mouse; D050778:NFATC Transcription Factors; D058570:TOR Serine-Threonine Kinases