Pediatric patient with restrictive cardiomyopathy on staged biventricular assist device support with Berlin Heart EXCOR ® underwent heart transplantation successfully: the first case in Japan

AbstractAbstractPure restrictive cardiomyopathy is a strong risk factor for poor outcomes in children with cardiomyopathy on ventricular assist devices. Owing to concomitant right heart failure, children with end-staged restrictive cardiomyopathy who are supported with a ventricular assist device often require a biventricular assist device, which is another risk factor for waitlist mortality in heart transplantation candidates. Herein, we report the case of a 3-year-old boy with pure restrictive cardiomyopathy who successfully underwent heart transplantation after 12  months of support with staged biventricular assist devices. Owing to the progression of diastolic dysfunction, the left ventricular assist device could not provide adequate circulation support. Despite the provision of biventricular assist device support, the patient required a complex management strategy that involved balancing the left and right ventricular assist device supports. We were able to stabilize the patient by careful synchronization of the supports and proceeded to heart transplantation.Trial registrationClinical Registration No.: Institutional Review Board of Osaka University Hospital, approval no. 16105.
Source: Journal of Artificial Organs - Category: Transplant Surgery Source Type: research

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We report a case of successful percutaneous edge‐to‐edge tricuspid valve repair for severe tricus pid regurgitation after cardiac transplantation. A 27‐year‐old man with a history of idiopathic restrictive cardiomyopathy status after orthotopic heart transplant presented with severe right‐sided heart failure symptoms. A transthoracic echocardiogram showed bi‐atrial enlargement and modera te‐to‐severe tricuspid regurgitation, and an increase to the severe range with exercise. Percutaneous edge‐to‐edge tricuspid valve repair was performed. The patient's symptoms improved, and follow‐up echocardiogram sh...
Source: ESC Heart Failure - Category: Cardiology Authors: Tags: Case Report Source Type: research
Authors: Eicher JC, Audia S, Damy T Abstract Transthyretin (TTR) cardiac amyloidosis results from the dissociation of the tetrameric, liver-synthetized transport protein, either because of a mutation (hereditary CA), or spontaneously due to ageing (wild type CA). Monomers self-associate into amyloid fibrils within the myocardium, causing heart failure, arrhythmias and conduction defects. This overlooked disease must be recognized in case of unexplained increased thickness of the myocardium, particularly in subjects of African descent, in patients with heart failure and preserved ejection fraction, and in those with...
Source: Revue de Medecine Interne - Category: Internal Medicine Tags: Rev Med Interne Source Type: research
Abstract Transthyretin amyloid cardiomyopathy (ATTR-CM) results in a restrictive cardiomyopathy caused by extracellular deposition of transthyretin, normally involved in the transportation of the hormone thyroxine and retinol-binding protein, in the myocardium. Enthusiasm about ATTR-CM has grown as a result of 3 simultaneous areas of advancement: Imaging techniques allow accurate noninvasive diagnosis of ATTR-CM without the need for confirmatory endomyocardial biopsies; observational studies indicate that the diagnosis of ATTR-CM may be underrecognized in a significant proportion of patients with heart failure; an...
Source: Circulation - Category: Cardiology Authors: Tags: Circulation Source Type: research
Amyloid transthyretin (ATTR) cardiac amyloidosis (CA) is an increasingly recognized cause of restrictive cardiomyopathy and associated heart failure with preserved ejection fraction (HFpEF). Despite improved diagnostic techniques to identify this condition, many patients experience delayed diagnosis. Interpretation of routine cardiac biomarkers (i.e. troponins and brain natriuretic peptide (BNP)) and echocardiography in ATTR-CA is limited, particularly in relation to pyrophosphate (PYP) scintigraphy.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (64) Source Type: research
AbstractPurpose of reviewThis review summarizes the clinical characteristics and updated outcomes of primary pediatric cardiomyopathies including dilated (DCM), hypertrophic (HCM), and restrictive cardiomyopathy (RCM), and briefly discusses left ventricular non-compaction (LVNC) and arrhythmogenic cardiomyopathy (ACM), primarily arrythmogenic right ventricular cardiomyopathy (ARVC).Recent findingsPediatric cardiomyopathies are diseases of the heart muscle with an estimated annual incidence of 1.1 –1.5 cases per 100,000. They are progressive in nature and are frequently caused by a genetic mutation causing a structura...
Source: Current Treatment Options in Cardiovascular Medicine - Category: Cardiology Source Type: research
CONCLUSION: As a result of the difficulty in performing arterial puncture, the small vessel calibre and the limitation in the use of iodated contrast, there are certain limitations to endovascular treatment of strokes in children that can lead to complications. A multidisciplinary approach to managing such cases would be helpful. PMID: 31072251 [PubMed - as supplied by publisher]
Source: Interventional Neuroradiology - Category: Radiology Tags: Interv Neuroradiol Source Type: research
Cardiac amyloidosis causes significant morbidity and mortality, leading to restrictive cardiomyopathy, heart failure, and death. Many patients are diagnosed late when irreversible amyloid fibril deposition has occurred. Heart transplantation may be considered in such patients. Historically, heart transplant outcomes were worse in cardiac amyloid patients compared with other types of heart failure, in part due to the systemic nature of the disease. However, effective therapies, particularly for light chain (AL) amyloidosis, have emerged, and disease remission is now possible.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: 514 Source Type: research
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Source: Pediatric Cardiology - Category: Cardiology Source Type: research
In conclusion, prolonged AI and/or LS infusions in HF are safe and beneficial even in small infants, allowing stabilization and reasonable social and family life out of the hospital. It may provi de precious time for heart transplantation or myocardial remodeling, improvement, and possible discontinuation even after long periods of support.
Source: Pediatric Cardiology - Category: Cardiology Source Type: research
We describe the case of a middle-aged man diagnosed with a myeloproliferative neoplasm associated with hypereosinophilia and treated with imatinib. He was diagnosed with cardiac involvement by hypereosinophilic syndrome at a late stage, with an established restrictive cardiomyopathy. Because of end-stage heart failure, he successfully received a heart transplant. This disease might not be considered a contraindication for heart transplantation.RésuméLe syndrome hyperéosinophilique est caractérisé par une surproduction d’éosinophiles qui s’infiltrent dans de nombreux org...
Source: Canadian Journal of Cardiology - Category: Cardiology Source Type: research
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