LEFT VENTRICULAR NONCOMPACTION, GARDNER SYNDROME, AND DESMOID TUMORS, A GENETIC CONNECTION?
نویسندگان
چکیده
منابع مشابه
Isolated left ventricular noncompaction syndrome.
Isolated left ventricular noncompaction (ILVNC) is a rare congenital cardiomyopathy characterized by prominent trabeculae, deep intertrabecular recesses, and thickened myocardium with 2 distinct layers (compacted and noncompacted). Clinical characteristics, outcomes, and appropriate therapies remain poorly defined. Data were collected on patients diagnosed with ILVNC by echocardiographic criter...
متن کاملLeft Ventricular Noncompaction: A Distinct Genetic Cardiomyopathy?
Left ventricular noncompaction (LVNC) describes a ventricular wall anatomy characterized by prominent left ventricular (LV) trabeculae, a thin compacted layer, and deep intertrabecular recesses. Individual variability is extreme, and trabeculae represent a sort of individual "cardioprinting." By itself, the diagnosis of LVNC does not coincide with that of a "cardiomyopathy" because it can be ob...
متن کاملLeft Ventricular Noncompaction
Ma Whether left ventricular noncompaction (LVNC) is a distinct cardiomyopathy or a morphologic trait shared by different cardiomyopathies remains controversial. Current guidelines from professional organizations recommend different strategies for diagnosing and treating patients with LVNC. This state-of-the-art review discusses new insights into the basic mechanisms leading to LVNC, its clinica...
متن کاملLeft ventricular noncompaction.
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, mos...
متن کاملLeft ventricular noncompaction.
Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy of emerging importance. We report a case of a 3-months-old boy who presented with congestive heart failure due to LVNC.
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ژورنال
عنوان ژورنال: Chest
سال: 2019
ISSN: 0012-3692
DOI: 10.1016/j.chest.2019.08.533