Biomaterial Database

Sudden unexpected death due to left ventricular noncompaction of myocardium: case report and review of the literature. 2010

Ling Li, and Allen Burke, and Xiang Zhang, and David Fowler

Key Laboratory of Evidence Science, China University of Political Science and Law, Beijing, China. Ling001@aol.com

Left ventricular noncompaction of the myocardium is a rare genetic cardiac disease characterized by a prominent meshwork and deep intertrabecular recesses of the left ventricle. The condition has frequently been misdiagnosed as other cardiomyopathies. Sudden deaths due to the disease are rarely reported in the literature of forensic sciences. We report 2 cases of sudden death due to undiagnosed left ventricular noncompaction. One was a 30-year-old man who suddenly collapsed while eating breakfast. He was pronounced dead on arrival at the hospital. The other 24-year-old man had a witness collapse at home. Despite immediate resuscitation, he expired 11 hours after hospitalization. Postmortem examination revealed that both patients died of left ventricular noncompaction. The key pathologic findings are described. Additionally, the literature is reviewed, and the incidence, clinical and pathophysiologic presentation, pathogenesis, and diagnostic criteria for the left ventricular noncompaction are discussed.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D009202	Cardiomyopathies	A group of diseases in which the dominant feature is the involvement of the CARDIAC MUSCLE itself. Cardiomyopathies are classified according to their predominant pathophysiological features (DILATED CARDIOMYOPATHY; HYPERTROPHIC CARDIOMYOPATHY; RESTRICTIVE CARDIOMYOPATHY) or their etiological/pathological factors (CARDIOMYOPATHY, ALCOHOLIC; ENDOCARDIAL FIBROELASTOSIS).	Myocardial Disease,Myocardial Diseases,Myocardial Diseases, Primary,Myocardial Diseases, Secondary,Myocardiopathies,Primary Myocardial Disease,Cardiomyopathies, Primary,Cardiomyopathies, Secondary,Primary Myocardial Diseases,Secondary Myocardial Diseases,Cardiomyopathy,Cardiomyopathy, Primary,Cardiomyopathy, Secondary,Disease, Myocardial,Disease, Primary Myocardial,Disease, Secondary Myocardial,Diseases, Myocardial,Diseases, Primary Myocardial,Diseases, Secondary Myocardial,Myocardial Disease, Primary,Myocardial Disease, Secondary,Myocardiopathy,Primary Cardiomyopathies,Primary Cardiomyopathy,Secondary Cardiomyopathies,Secondary Cardiomyopathy,Secondary Myocardial Disease
D009206	Myocardium	The muscle tissue of the HEART. It is composed of striated, involuntary muscle cells (MYOCYTES, CARDIAC) connected to form the contractile pump to generate blood flow.	Muscle, Cardiac,Muscle, Heart,Cardiac Muscle,Myocardia,Cardiac Muscles,Heart Muscle,Heart Muscles,Muscles, Cardiac,Muscles, Heart
D003645	Death, Sudden	The abrupt cessation of all vital bodily functions, manifested by the permanent loss of total cerebral, respiratory, and cardiovascular functions.	Sudden Death
D004695	Endocardial Fibroelastosis	A condition characterized by the thickening of ENDOCARDIUM due to proliferation of fibrous and elastic tissue, usually in the left ventricle leading to impaired cardiac function (CARDIOMYOPATHY, RESTRICTIVE). It is most commonly seen in young children and rarely in adults. It is often associated with congenital heart anomalies (HEART DEFECTS CONGENITAL;) INFECTION; or gene mutation. Defects in the tafazzin protein, encoded by TAZ gene, result in a form of autosomal dominant familial endocardial fibroelastosis.	Endomyocardial Fibroelastosis,Endocardial Fibroelastoses,Fibroelastoses, Endocardial,Fibroelastosis, Endocardial
D006330	Heart Defects, Congenital	Developmental abnormalities involving structures of the heart. These defects are present at birth but may be discovered later in life.	Congenital Heart Disease,Heart Abnormalities,Abnormality, Heart,Congenital Heart Defect,Congenital Heart Defects,Defects, Congenital Heart,Heart Defect, Congenital,Heart, Malformation Of,Congenital Heart Diseases,Defect, Congenital Heart,Disease, Congenital Heart,Heart Abnormality,Heart Disease, Congenital,Malformation Of Heart,Malformation Of Hearts
D006352	Heart Ventricles	The lower right and left chambers of the heart. The right ventricle pumps venous BLOOD into the LUNGS and the left ventricle pumps oxygenated blood into the systemic arterial circulation.	Cardiac Ventricle,Cardiac Ventricles,Heart Ventricle,Left Ventricle,Right Ventricle,Left Ventricles,Right Ventricles,Ventricle, Cardiac,Ventricle, Heart,Ventricle, Left,Ventricle, Right,Ventricles, Cardiac,Ventricles, Heart,Ventricles, Left,Ventricles, Right
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults

Related Publications

Ling Li, and Allen Burke, and Xiang Zhang, and David Fowler

[Noncompaction of the left ventricular myocardium. Case report and review of the literature].

June 2002, Zeitschrift fur Kardiologie,

Ling Li, and Allen Burke, and Xiang Zhang, and David Fowler

Isolated noncompaction of the left ventricular myocardium: a case report and literature review.

August 2008, Echocardiography (Mount Kisco, N.Y.),

Ling Li, and Allen Burke, and Xiang Zhang, and David Fowler

Left ventricular noncompaction: case report and literature review.

December 2007, Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology,

Ling Li, and Allen Burke, and Xiang Zhang, and David Fowler

Sudden unexpected intrapartum death and left ventricular noncompaction involving the right ventricle.

January 2024, Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology,

Ling Li, and Allen Burke, and Xiang Zhang, and David Fowler

Sudden, unexpected death due to cardiac fibroma. Report of a case and review of the literature.

June 1987, The American journal of forensic medicine and pathology,

Ling Li, and Allen Burke, and Xiang Zhang, and David Fowler

Sudden, Unexpected Death Due to Pseudo-Meigs Syndrome: A Case Report and Review of the Literature.

March 2019, The American journal of forensic medicine and pathology,

Ling Li, and Allen Burke, and Xiang Zhang, and David Fowler

Left ventricular noncompaction cardiomyopathy: a case report and literature review.

January 2014, International journal of clinical and experimental medicine,