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Congenital Idiopathic Dilatation of the Right Atrium: Antenatal Appearance, Postnatal Management, Long-Term Follow-Up and Possible Pathomechanism.
MedLine Citation:
PMID:  22722771     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Introduction: Idiopathic dilatation of the right atrium (IDRA) is a rare abnormality usually detected by chance at any time between antenatal and adult life. It is defined as isolated enlargement of the right atrium in the absence of other cardiac lesions causing right atrial dilatation. IDRA can be associated with atrial arrhythmia and systemic embolism. The clinical presentation shows high variability ranging from the lack of any symptoms up to cardiac failure. Methods/Results: We describe 2 children with antenatally diagnosed IDRA, the intrauterine course in 1 case, the postnatal management and its long-term follow-up. There has been no need for surgical intervention so far because of the lack of arrhythmias and no further progression of right atrial diameters. Thrombus formation in the right atrium, which is a potential risk for pulmonary embolism, led us to initiate anticoagulation in our cases to prevent such complications. Furthermore, we suggest one possible pathomechanism of congenital right atrial dilatation. Conclusion: Optimal management of severe IDRA depends on the individual case. Long-term follow-up of these patients is necessary to monitor a possible further progression of right atrial size and occurrence of arrhythmias. As a possible pathomechanism, a functional partial anomalous pulmonary venous insertion may imitate a structural abnormal pulmonary vein connection in some idiopathic cases of congenital right atrial dilatation.
Authors:
Sigrun R Hofmann; Antje Heilmann; Hans J Häusler; Ingo Dähnert; Gabriele Kamin; Robert Lachmann
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-6-21
Journal Detail:
Title:  Fetal diagnosis and therapy     Volume:  -     ISSN:  1421-9964     ISO Abbreviation:  -     Publication Date:  2012 Jun 
Date Detail:
Created Date:  2012-6-22     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9107463     Medline TA:  Fetal Diagn Ther     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 S. Karger AG, Basel.
Affiliation:
Department of Pediatrics, University Hospital Carl Gustav Carus, Technical University of Dresden, Dresden, Germany.
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