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Cardiac Hypertrophy, Heart Failure and Cardiomyopathy
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
Noncompaction cardiomyopathy (NCCM), which is also known as left ventricular noncompaction (LVNC), is a disease with excessive trabeculations of the LV, with a >2-fold thickening of the endocardial noncompacted layer compared with the epicardial compacted layer of the myocardium (NC/C >2). It has deep intertrabecular recesses communicating with the ventricular cavity, and a thin compact epicardial layer. While LVNC is considered a genetic cardiomyopathy by the American Heart Association, the European Society of Cardiology categorizes it as an unclassified cardiomyopathy. This is labelled as a cardiomyopathy but is a controversial entity. LVNC has previously been regarded as a rare disease, and has been called by a variety of names including spongy myocardium, fetal myocardium and noncompaction of the left ventricular myocardium. Some consider it to be a congenital or genetic entity while others consider it as acquired in common cardiomyopathy such as HCM, ACM (Fig. 5.48), DCM and RCM.
Prenatal Diagnosis of Isolated Right Ventricular Non-Compaction Cardiomyopathy with an MYH7 Likely Pathogenic Variant
Published in Fetal and Pediatric Pathology, 2023
Weiming Yu, Mary Ann Thomas, Lindsay Mills, James R. Wright
Noncompaction of ventricular myocardium (NVM) is a cardiomyopathy characterized by the presence of excessive ventricular trabeculae, deep intertrabecular recesses, and a thin compacted myocardial layer [1]. NVM usually involves the left ventricle, and if isolated is known as left ventricular noncompaction cardiomyopathy (LVNC); however, NVM may also involve the right ventricle as either biventricular or isolated right ventricular (RVNC) [2–6]. The European Society of Cardiology defines LVNC as unclassified cardiomyopathy [7]. The American Heart Association has classified LVNC as genetic cardiomyopathy based largely on molecular and genomic information [8]. We report here a case of isolated RVNC in a stillborn fetus at 19 weeks gestational age related to an MYH7 likely pathogenic variant.
Usefulness of a left ventricular assist device in patients with left ventricular noncompaction
Published in Baylor University Medical Center Proceedings, 2018
Helen Hashemi, Fayez S. Raza, David M. Harmon, Tony Alias, Joost Felius, Melody J. Sherwood
Noncompaction cardiomyopathy was first described in 1969 by Feldt et al2 but the etiology remains uncertain.1 It is hypothesized that compaction of the trabeculae in utero is interrupted, resulting in a spongy, hypertrabeculated myocardium with large intratrabecular spaces.3 Many genetic mutations have been identified; however, due to the high heterogeneity of mutations and various associations with neuromuscular diseases, no single gene has been identified in familial cases.1,3–5 Reports of acquired LVNC in the postpartum period have been published, as well as reports of acquired LVNC in highly trained athletes.1,5–8 Though often asymptomatic, LNVC can present with a classic triad of symptoms, including heart failure, thromboembolism, or arrhythmias.1,3 Electrocardiogram abnormalities are seen in up to 94% of adult LVNC cases, with ventricular arrhythmias occurring in 18% to 47% of diseased adults.1,9,10 Thrombus formation often occurs in the ventricular cavity as a result of severe systolic dysfunction or from stagnation of blood flow in the deep intratrabecular spaces. Arrhythmias, such as atrial fibrillation, may also be a source of thrombi.1,11
Sudden cardiac arrest as the initial presentation for left ventricular noncompaction cardiomyopathy
Published in Baylor University Medical Center Proceedings, 2019
Avaneesh Jakkoju, Rakesh Jakkoju, Vishnupriya Kuchana, Pedro R. Cox-Alomar, Frank W. Smart, D. Luke Glancy
Left ventricular noncompaction cardiomyopathy (LVNCC) is an uncommon primary genetic cardiomyopathy that often presents as heart failure. Typically the LV is dilated and hypocontractile with a thin compacted outer muscular layer and a thicker, heavily trabeculated inner layer with deep intratrabecular recesses that are continuous with the LV cavity. (Unlike the normal right ventricle, the normal LV is not heavily trabeculated.) The condition is thought to arise from intrauterine arrest of compaction of fetal myocardium. Malignant arrhythmias or sudden cardiac arrest is a rare presentation and constitutes about 2% to 5% of cases.2