Biomaterial Database

Left and right ventricular dysplasia and Uhl's anomaly. A case report. 1996

A Sutter, and H R Gujer

University Institute of Legal Medecine, Lausanne, Switzerland.

Myocardial dysplasia involves a quasi-complete or localized absence of myocardial fibers in the right or left ventricle, with corresponding partial or complete filling by fibroadipose tissue. This disorder, in its full form called "Uhl's anomaly", was first described in 1952 as an anomaly of the heart conferring a parchment-like appearance to its walls. Incomplete forms of the disorder fall into the category of right or left arrhythmogenic ventricular dysplasia. Although approximately 50 cases of right ventricular dysplasia have been documented, only two reports concern left ventricular dysplasia. Having encountered the latter anomaly, we first present the case and our observations, and then review the etiology, pathogenesis, clinical aspects, and evolution of this disorder.

UI	MeSH Term	Description	Entries
D008297	Male		Males
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
D000367	Age Factors	Age as a constituent element or influence contributing to the production of a result. It may be applicable to the cause or the effect of a circumstance. It is used with human or animal concepts but should be differentiated from AGING, a physiological process, and TIME FACTORS which refers only to the passage of time.	Age Reporting,Age Factor,Factor, Age,Factors, Age
D012737	Sex Factors	Maleness or femaleness as a constituent element or influence contributing to the production of a result. It may be applicable to the cause or effect of a circumstance. It is used with human or animal concepts but should be differentiated from SEX CHARACTERISTICS, anatomical or physiological manifestations of sex, and from SEX DISTRIBUTION, the number of males and females in given circumstances.	Factor, Sex,Factors, Sex,Sex Factor
D016757	Death, Sudden, Cardiac	Unexpected rapid natural death due to cardiovascular collapse within one hour of initial symptoms. It is usually caused by the worsening of existing heart diseases. The sudden onset of symptoms, such as CHEST PAIN and CARDIAC ARRHYTHMIAS, particularly VENTRICULAR TACHYCARDIA, can lead to the loss of consciousness and cardiac arrest followed by biological death. (from Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 7th ed., 2005)	Cardiac Sudden Death,Sudden Cardiac Death,Cardiac Arrest, Sudden,Sudden Cardiac Arrest,Arrest, Sudden Cardiac,Cardiac Arrests, Sudden,Cardiac Death, Sudden,Death, Cardiac Sudden,Death, Sudden Cardiac,Sudden Death, Cardiac
D017809	Fatal Outcome	Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept.	Fatal Outcomes,Outcome, Fatal,Outcomes, Fatal
D018487	Ventricular Dysfunction, Left	A condition in which the LEFT VENTRICLE of the heart was functionally impaired. This condition usually leads to HEART FAILURE; MYOCARDIAL INFARCTION; and other cardiovascular complications. Diagnosis is made by measuring the diminished ejection fraction and a depressed level of motility of the left ventricular wall.	LV Diastolic Dysfunction,LV Dysfunction,LV Systolic Dysfunction,Left Ventricular Diastolic Dysfunction,Left Ventricular Dysfunction,Left Ventricular Systolic Dysfunction,Diastolic Dysfunction, LV,Dysfunction, LV,Dysfunction, LV Diastolic,Dysfunction, LV Systolic,Dysfunction, Left Ventricular,LV Diastolic Dysfunctions,LV Dysfunctions,LV Systolic Dysfunctions,Left Ventricular Dysfunctions,Systolic Dysfunction, LV