Heart Transplantation in Patients With Loeys-Dietz Syndrome: Is It a Novel Cardiomyopathy?

Publication date: January 2020Source: The Annals of Thoracic Surgery, Volume 109, Issue 1Author(s): Muhammad Aftab, T. Brett Reece, Lars G. Svensson
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research

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Conclusions: We demonstrate that R406W-desmin provokes its severe cardiotoxic potential by a novel pathomechanism, where the concurrent dual functional states of mutant desmin assembly complexes underlie the uncoupling of desmin filaments from intercalated discs and their structural disorganization. PMID: 33023321 [PubMed - as supplied by publisher]
Source: Circulation - Category: Cardiology Authors: Tags: Circulation Source Type: research
AbstractRacially disparate health outcomes exist for a multitude of populations and illnesses. It is unknown how race and ethnicity impact mortality for children with cardiomyopathy or myocarditis. This retrospective cross-sectional study employed the Kids ’ Inpatient Database to analyze 34,617 hospital admissions for patients ≤ 18 years old with cardiomyopathy, myocarditis, or both, without concomitant congenital heart disease. Multivariate logistic regression models investigated the impact of race/ethnicity on in-hospital mortality adjustin g for age, calendar year, sex, insurance type, diagno...
Source: Pediatric Cardiology - Category: Cardiology Source Type: research
Conclusions: There is an association between PVB19 and HHV-6 infection and DCM in children. The study suggests the persistence of PVB19 and HHV-6 in the host can lead to subsequent viral reactivation in the transplanted heart, even in those recipients who do not have active myocarditis. PVB19 in the cardiac allograft tended toward higher adverse post-HT events.
Source: Annals of Pediatric Cardiology - Category: Cardiology Authors: Source Type: research
We describe a case of cobalt-induced cardiomyopathy that progressed to end stage heart failure requiring orthotopic heart transplantation. A 67 year old woman with history of bilateral cobalt-chromium hip replacements presented with dyspnea of 3 weeks duration. She was tachycardic and hypotensive, with elevated NT- proBNP. Electrocardiogram demonstrated sinus tachycardia, low voltage and right bundle branch block. Transthoracic echocardiogram revealed left ventricular ejection fraction of 20-25% with global hypokinesis and a moderate circumferential pericardial effusion.
Source: Journal of Cardiac Failure - Category: Cardiology Authors: Tags: 466 Source Type: research
The purpose of this analysis is to provide up to date, summary data around post cardiac transplant survival in patients with Adriamycin associated cardiomyopathy (ACM).
Source: Journal of Cardiac Failure - Category: Cardiology Authors: Tags: 438 Source Type: research
We present a case of high-grade AV block and septal ventricular tachycardia (VT) requiring orthotopic heart transplantation with post-transplant genetic testing showing LMNA-related dilated cardiomyopathy.
Source: Journal of Cardiac Failure - Category: Cardiology Authors: Tags: 298 Source Type: research
An estimated 3.1 million cases of myocarditis/cardiomyopathy were diagnosed in 2017. Myocarditis, inflammation of the myocardium, is a leading cause of sudden death from heart failure in children and young adults worldwide. Patients can progress to dilated cardiomyopathy, heart and heart transplant. Studies have found that Vitamin D binding protein (DBP) could be instrumental in response to tissue injury and activation of the immune response. This chemotaxis activation has been shown to be due to activation of the complement cascade via C5a.
Source: Journal of Cardiac Failure - Category: Cardiology Authors: Tags: 274 Source Type: research
ConclusionOur results demonstrate an excellent outcome with the use of the CentriMag ™ device in this seriously ill population. Despite requiring multiple procedures, over 58% of patients were discharged from hospital with 5-year survival of 46%.
Source: Indian Journal of Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
X-linked dilated cardiomyopathy (XLDCM) is a serious condition caused by abnormalities in the DMD gene (dystrophin gene). The affected patients did not have major skeletal weakness but presented with early signs of heart failure, and deteriorated quickly despite medical treatment resulting in early death. Currently there is no curative treatment and heart transplantation is often required. We study the pathomechanism of the DMD-associated XLDCM with mutation in the first exon-intron boundary, through examination of dystrophin isoforms expression and the functional characterization of the manifested cardiomyocytes generated...
Source: Neuromuscular Disorders - Category: Neurology Authors: Source Type: research
Hypertrophic cardiomyopathy (HCM) is the most common inherited disease, with a prevalence of 1:200 worldwide. The cause of HCM usually presents with an autosomal dominant mutation in the genes encoding one of more than 20 sarcomeric proteins, incomplete penetrance, and variable expressivity. HCM classically manifests as an unexplained thickness of the interventricular septum (IVS) and left ventricular (LV) walls, with or without the obstruction of the LV outflow tract (LVOT), and variable cardiac arrhythmias. Here, we present a rare case of mixed cardiomyopathy (cardiac hypertrophy and dilation) and erythrocytosis in a you...
Source: Frontiers in Pharmacology - Category: Drugs & Pharmacology Source Type: research
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