Background: Non-compaction of the left ventricle (NCLV) is an unclassified cardiomyopathy due to intrauterine arrest of compaction of the loose interwoven meshwork. Only a few studies involving sub-Saharan Africans insmall numbers have been published. The aim of our study was to determine the diagnostic, therapeutic and prognostic aspects as well as the clinical course of NCLV in a black African population.
Methodology: A multicentre retrospective study was carried out between November 2007 and June 2012 in two cardiology departments in Dakar. Patients who met the echocardiographic criteria for NCLV were included in the study.
Results: 35patients with the diagnosis of NCLV were evaluated in the study. Their mean age was 47±18.4 years. Heart failure was found in 77.1% of the patients. The most frequent electrocardiographic abnormalities were left ventricular hypertrophy (LVH) (46%) and sinus tachycardia (43%). Mean non-compaction/compaction ratio was 2.84±0.68 with preferential localization in the apex of the left ventricle. The main complications observed were cardiogenic shock (23.5%), pulmonary embolism (6.3%) and ventricular tachycardia (5.9%). Diuretics and ACE inhibitors were the medications most often prescribed. Age >60 years (p=0.04), male gender (p=0.03) and the occurrence of complications during follow-up (p=0.04) were noted to be predictors of poor prognosis.
Conclusion: Contrary to previous beliefs, NCLV may not be less common in black Africans than in other ethnic subgroups. Clinicians in Africa should be made aware of NCLV so that it can be diagnosed at earlier stages.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5818043 | PMC |
http://dx.doi.org/10.1136/heartasia-2017-010884 | DOI Listing |
J Vet Cardiol
December 2024
Department of Diagnostic Imaging, The Animal Hospital at Murdoch University, Perth, WA, Australia.
Two juvenile (five- and 10-month-old), male, neutered, Jack Russel terrier cross dogs, each presented primarily for respiratory signs of varying severity, were included in the study. The first presented for coughing and weakness, and the other presented in respiratory distress. A cardiac murmur was absent in both.
View Article and Find Full Text PDFEur J Med Genet
December 2024
CHU Lille, Institut de Génétique Médicale, F-59000 Lille, France; Univ. Lille, ULR7364 - RADEME - Maladies RAres du DEveloppement embryonnaire et du Métabolisme, F-59000 Lille, France. Electronic address:
The X-linked NONO gene encodes Non-Pou Domain-Containing Octamer-Binding Protein, a multifunctional member of the DBHS family involved in transcriptional regulation, RNA splicing and DNA repair. Pathogenic variants in NONO cause Intellectual Developmental Disorder, X-linked Syndromic (MIM #300967), characterised by intellectual disability, neurodevelopmental delay, cardiomyopathy, such as left ventricular non-compaction (LVNC), and congenital heart defects such as including atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), and patent foramen ovale (PFO). This study reports three new patients with pathogenic hemizygous frameshift variants in NONO identified with exome sequencing, broadening the clinical presentation.
View Article and Find Full Text PDFInt J Mol Sci
December 2024
Research Center of Biotechnology, A.N. Bach Institute of Biochemistry, Russian Academy of Sciences, Moscow 119071, Russia.
Pediatric dilated cardiomyopathy (DCM) is a rare heart muscle disorder leading to the enlargement of all chambers and systolic dysfunction. We identified a novel de novo variant, c.88A>G (p.
View Article and Find Full Text PDFLeft ventricular non compaction is a genetic cardiomyopathy with a high occurence in individuals of African ancestry and may present in adulthood with diagnostic challenges when there is advanced heart failure. Echocardiography and Magnetic Resonance Imaging have mostly been used in making a diagnosis. However, there is a lack of these diagnostic tools required to aid in early diagnosis in Low and Middle Income Countries.
View Article and Find Full Text PDFBiomolecules
November 2024
Cardiogenetic Center, Rare Diseases and Medical Genetics Units, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.
Inherited cardiac channelopathies are major causes of sudden cardiac death (SCD) in young people. Genetic testing is focused on the identification of single-nucleotide variants (SNVs) by Next-Generation Sequencing (NGS). However, genetically elusive cases can carry copy number variants (CNVs), which need specific detection tools.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!