Document Detail


Extensive thrombus formation in the right ventricle due to a rare combination of arrhythmogenic right ventricular cardiomyopathy and heterozygous prothrombin gene mutation G20210 A.
MedLine Citation:
PMID:  10894919     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Arrhythmogenic right ventricular cardiomyopathy is a rare heart muscle disease characterized by right and often left ventricular myocardial atrophy and fibrofatty replacement. Heart failure, arrhythmias and sudden death are characteristic complications. We observed a female in whom arrhythmogenic right ventricular cardiomyopathy was diagnosed due to presyncopes and dyspnea on exertion. A left ventricular thrombus was found echocardiographically, which disappeared with oral anticoagulation. Subsequently, however, extensive thrombus formation in the dilated akinetic right ventricle occurred which was resistant to combined treatment with heparin and oral anticoagulation. Thrombophilia screening showed a mutant prothrombin 20210A allele which is an inherited coagulopathy associated with increased plasma levels of prothrombin and increased risks of mainly venous thrombosis. The patient developed endstage biventricular heart failure and underwent heart transplantation within 3 months after thrombus formation in the right ventricle was diagnosed. In the explanted heart, the thrombus in the right ventricle was impressively large and calcified. In patients with unusual thrombus formation in the heart, coagulopathy may be associated and should be excluded.
Authors:
C H Attenhofer Jost; T Bombeli; C Schrimpf; E Oechslin; W Kiowski; R Jenni
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Cardiology     Volume:  93     ISSN:  0008-6312     ISO Abbreviation:  Cardiology     Publication Date:  2000  
Date Detail:
Created Date:  2000-09-13     Completed Date:  2000-09-13     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  1266406     Medline TA:  Cardiology     Country:  SWITZERLAND    
Other Details:
Languages:  eng     Pagination:  127-30     Citation Subset:  IM    
Copyright Information:
Copyright 2000 S. Karger AG, Basel
Affiliation:
Division of Cardiology, University Hospital Zurich, Switzerland. karatc@usz.unizh.ch
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MeSH Terms
Descriptor/Qualifier:
Adult
Alleles
Arrhythmogenic Right Ventricular Dysplasia / blood,  complications*,  genetics,  surgery
Female
Heart Diseases / blood,  etiology,  surgery
Heart Transplantation
Heterozygote*
Humans
Point Mutation*
Prothrombin / genetics*,  metabolism
Thrombosis / blood,  etiology*,  surgery
Chemical
Reg. No./Substance:
9001-26-7/Prothrombin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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