Objectives: In single cases left-ventricular-hypertrabeculation/noncompaction (LVHT) develops after birth (acquired-LVHT). This study aimed to determine the prevalence of acquired-LVHT, to look for echocardiographic similarities between acquired-LVHT cases, to determine the interval between the last normal echocardiography and detection of acquired-LVHT, and which neuromuscular-disorders are associated with acquired-LVHT.
Design: We retrospectively looked for acquired-LVHT among a cohort of 22 LVHT-patients, diagnosed during 3 years. We compared cardiac and extra-cardiac findings between patients with acquired-LVHT.
Results: Among the 22 patients with LVHT, acquired-LVHT was found in four of them. One of the four was echocardiographically normal when investigated before the detection of LVHT. At diagnosis of LVHT echocardiography showed cardiac abnormalities in addition to LVHT in three of the four patients. The last echocardiography without LVHT was carried out on the average 3.8 y before LVHT was detected. Two patients had a dystrophinopathy and two patients a metabolic myopathy.
Conclusion: Acquired-LVHT is more frequent than previously thought, requires review of previous echocardiographies, is largely associated with other cardiac abnormalities, and occurs most frequently together with neuromuscular-disorders.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1080/14017430701744451 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Prognostic Value of Signal Abnormalities on Brain MRI in Post-Anoxic Super-Refractory Status Epilepticus: A Single-Center Retrospective Study.
Eur J Neurol
January 2025
Epilepsy Center, Department of Neurology, Fondazione IRCCS San Gerardo Dei Tintori, Monza, Italy.
Background: Epileptiform activity, including status epilepticus (SE), occurs in up to one-third of comatose survivors of cardiac arrest and may predict poor outcome. The relationship between SE and hypoxic-ischemic brain injury (HIBI) is not established.
Methods: This is a single-center retrospective study on consecutive patients with post-anoxic super-refractory SE.
Inhibition of aortic CX3CR1+ macrophages mitigates thoracic aortic aneurysm progression in Marfan syndrome in mice.
J Clin Invest
January 2025
Department of Physiology and Pathophysiology, School of Basic Medical Sciences, Peking University; State Key Laboratory of Vascular Homeostasis and Remodeling, Peking University, Beijing, China.
The pathogenesis of thoracic aortic aneurysm (TAA) in Marfan syndrome (MFS) is generally attributed to vascular smooth muscle cell (VSMC) pathologies. However, the role of immune cell-mediated inflammation remains elusive. Single-cell RNA sequencing identified a subset of CX3CR1+ macrophages mainly located in the intima in the aortic roots and ascending aortas of Fbn1C1041G/+ mice, further validated in MFS patients.
View Article and Find Full Text PDFAn atrial septal defect (ASD) is a common congenital heart anomaly that results in irregular blood flow between the systemic and pulmonary circulations due to an opening in the atrial septum. Ostium secondum ASD accounts for a large proportion of these defects and often goes unnoticed during childhood and adolescence. Pulmonary hypertension (PH), affecting a significant number of patients with ostium secondum ASD, is associated with functional limitations, heart failure, and tachyarrhythmias.
View Article and Find Full Text PDFAssociations With Adverse Postoperative Outcomes in Children Undergoing Atrial Septal Defect Closure in Lower-Resource Settings.
JACC Adv
December 2024
Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA.
Background: Atrial septal defects (ASDs) are a common cause of congenital heart disease worldwide.
Objectives: The purpose of the study was to assess change over time in surgical outcomes for ASD repair and identify patient-level risk factors for adverse postoperative outcomes.
Methods: We analyzed cases of isolated ASD in patients <18 years from 2010 to 2020 from 71 sites participating in the International Quality Improvement Collaborative for Congenital Heart Disease.
Catheter ablation of atrial flutter in an adult with a univentricular heart, common atrium, and single atrioventricular valve: a case report-'complex things don't always require a complex solution'.
Eur Heart J Case Rep
January 2025
Cardiac Electrophysiology, National Medical Center 'November 20th', ISSSTE, Av. Felix Cuevas #540, Col. Del Valle Del. Benito Juarez, C.P. 03100 Mexico City, Mexico.
Background: The 'univentricular' heart encompasses a variety of congenital cardiac defects characterized by a single functional ventricle and an underdeveloped ventricular chamber. Surgical intervention, typically in infancy or childhood, aims to regulate pulmonary blood flow volume. In adulthood, untreated patients may experience limitations in physical activity and elevated morbidity due to persistent cyanosis and arrhythmias, notably after the Fontan procedure.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!