Isolated Noncompaction of the Left Ventricle in Adults.
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2015-08-04
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Isolated left ventricular noncompaction (ILVNC) is a cardiomyopathy that was first described in 1926 as a "spongy myocardium." The disorder results from intrauterine arrest of compaction of the loose interwoven meshwork of the fetal myocardial primordium and subsequent persistence of deep trabecular recesses in the myocardial wall. The classical clinical presentation is a triad of heart failure, arrhythmias, and embolic events from mural thrombi. ILVNC has been associated with several autosomal dominant, X-linked, and mitochondrial genetic mutations that are also shared among other cardiomyopathies. Over the past decade, ILVNC has been subject to intensive research, as it increases the risk for sudden cardiac death. This review focuses on the current understanding of ILVNC in adult populations and attempts to provide organized insight into the disease process, screening, diagnosis, management, role of device therapy, and prognosis.
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Hussein, Ayman, Ahmadreza Karimianpour, Patrick Collier and Richard A Krasuski (2015). Isolated Noncompaction of the Left Ventricle in Adults. J Am Coll Cardiol, 66(5). pp. 578–585. 10.1016/j.jacc.2015.06.017 Retrieved from https://hdl.handle.net/10161/10986.
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Richard Andrew Krasuski
Dr. Richard Krasuski is an Adjunct Professor of Medicine and Pediatrics at Duke University Medical Center. He was formerly the Director of the Adult Congenital Heart Center, Director of Hemodynamic Research, and the Medical Director of the CTEPH Program at Duke. He is considered a thought leader in the fields of pulmonary hypertension and congenital heart disease. His research focus is in epidemiologic and clinical studies involving patients with pulmonary hypertension and patients with congenital heart disease. He is involved in multiple multicenter studies through the Alliance for Adult Research in Congenital Cardiology (AARCC). He has also helped to develop multiple research databases in these patient populations. He is Co-PI in the EPIPHANY Study examining the impact of medical and transcatheter interventions on RV-PA coupling in patients with chronic thromboembolic pulmonary hypertension. Over his career he has mentored over 100 students, residents and fellows and has published over 350 peer reviewed articles, book chapters and meeting abstracts. He is also the Chief Editor of Advances in Pulmonary Hypertension and on the editorial boards of >20 leading medical journals. He has been an invited speaker at over 100 medical centers worldwide.
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