Noncompaction of myocardium is a rare and recently defined entity that may cause cardioembolism during childhood. We report an 18-month-old girl with noncompaction of the left ventricular myocardium presenting with fatal cardioembolic stroke. The patient had a high factor VIII level, which is known to cause an increased tendency to thromboembolic events. To our knowledge, this is the youngest case with stroke associated with noncompaction of the myocardium. Patients with noncompaction should be considered for prophylactic antithrombotic treatment to prevent mortality and morbidity owing to systemic thromboembolic events, especially if they carry additional risk factors that make them prone to hypercoagulation.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1177/08830738030180061501 | DOI Listing |
J Clin Med
January 2025
Hospital Virgen de la Arrixaca, 30120 Murcia, Spain.
Accurate segmentation of the left ventricular myocardium in cardiac MRI is essential for developing reliable deep learning models to diagnose left ventricular non-compaction cardiomyopathy (LVNC). This work focuses on improving the segmentation database used to train these models, enhancing the quality of myocardial segmentation for more precise model training. We present a semi-automatic framework that refines segmentations through three fundamental approaches: (1) combining neural network outputs with expert-driven corrections, (2) implementing a blob-selection method to correct segmentation errors and neural network hallucinations, and (3) employing a cross-validation process using the baseline U-Net model.
View Article and Find Full Text PDFFront Transplant
December 2024
Pediatric Cardiology and Adult with Congenital Heart Disease Unit, Instituto do Coração (InCor) do Hospital das Clinicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
Background: Cardiomyopathy is a disease that affects the myocardium and can be classified as dilated, restrictive, or hypertrophic cardiomyopathy. Among the subtypes, restrictive cardiomyopathy is characterized by restriction of ventricular filling and its uncommon cause is a disease due to mutation on Filamin C (FLNC) gene. Filamin C is an actin-binding protein encoded by FLNC gene and participates in sarcomere stability maintenance, which is expressed on the striated muscle.
View Article and Find Full Text PDFBMC Cardiovasc Disord
November 2024
Department of Cardiovascular Medicine, Children's Hospital of Chongqing Medical University, Chongqing, China.
Background: The absence of other structural heart disease is a prerequisite for the diagnosis of non-compaction of the ventricular myocardium (NVM). We also observed that the phenomenon of non-compaction in ventricular muscle in some large patent ductus arteriosus (PDA) patients in children. This study was aimed to explore the prognosis of NVM associated with large PDA in children and provide a better understanding of the interplay between genetic and hemodynamic factors that lead to the phenotype of NVM.
View Article and Find Full Text PDFJ Int Med Res
November 2024
Department of Cardiology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei Province, China.
Desmin is a type III intermediate filament protein specifically expressed in muscle cells, which is encoded by the gene. Defects in the desmin protein and cytoskeletal instability may interfere with cardiac muscle conduction signals, a fundamental mechanism for arrhythmias in patients with desmin-related myopathy. This current case report presents a female patient in her early 20s who presented with early-onset complete atrioventricular block and complete left bundle branch block over the previous decade.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!