336 Thromboembolic event and it’s management in a girl with restrictive cardiomyopathy and implanted Berlin Heart Excor device

Ventricular assist devices are increasingly used as an emerging therapy in children with different heart condition, mostly cardiomyopathy. Introduction of pulsatile devices for bridge to heart transplantation contributed to significant improvements in the survival and discharge rate of these patient...

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Bibliographic Details
Published in:Archives of disease in childhood 2021-10, Vol.106 (Suppl 2), p.A141-A141
Main Authors: Šestan, Mario, Matković, Hana, Dessardo, Sandro, Matić, Toni, Cvitković, Miran, Galić, Slobodan, Bartoniček, Dorotea, Radoš, Marko, Ozretić, David, Jovanović, Ivan, Rubić, Filip
Format: Article
Language:English
Subjects:
Abstracts
Acetylsalicylic acid
Anatomy
Angiography
Anticoagulants
Calcium-binding protein
Cardiomyopathy
Central nervous system
Clopidogrel
Clotting
Computed tomography
Congestive heart failure
Femoral artery
Heart
Heart transplantation
Neuroimaging
Oxygenation
Paresis
Patients
Pediatrics
Reperfusion
Thromboembolism
Thrombosis
Troponin
Troponin I
Ventricle
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Summary:Ventricular assist devices are increasingly used as an emerging therapy in children with different heart condition, mostly cardiomyopathy. Introduction of pulsatile devices for bridge to heart transplantation contributed to significant improvements in the survival and discharge rate of these patients. Berlin Heart EXCOR Pediatric (BH) is a pulsatile device with different chamber sizes and is registered for use in pediatric patients. When such devices are used during longer period of time, many complications can occur, most of which are linked to clotting disorders. Therefore, Edmonton protocol was introduced for patients on BH where detailed instructions are given regarding institution and maintenance of anticoagulation and antiaggregation therapy. Despite proper management, patients could have devastating complications, particularly cerebral arterial thromboses.We present a case of a 2,5-year-old girl who was diagnosed with restrictive cardiomyopathy caused by the p.Arg192Cys variant in the gene encoding cardiac troponin I (TNNI3). When her condition worsened and end-stage heart failure developed, she was placed on Venoarterial extracorporeal membrane oxygenation (VA-ECMO), by which she was supported for 3 weeks. Since she did not tolerate separation from mechanical support, a left ventricular assist device BH was implanted. Afterwards her anticoagulation and antiaggregation therapy was managed according Edmonton protocol thus she was given warfarin (target INR 2.8-3.5), acetylsalicylic acid (target value of ASPI test
ISSN:0003-9888
1468-2044
DOI:10.1136/archdischild-2021-europaediatrics.336