Document Detail

Anomalous mitral arcade in twin-twin transfusion syndrome.
MedLine Citation:
PMID:  20876435     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Echocardiography has documented acquired pulmonary stenosis and cardiomyopathy in recipient fetuses in twin-twin transfusion syndrome. At autopsy, we also have identified anomalous mitral arcade, a rare valve deformity associated with mitral regurgitation. METHODS AND RESULTS: To identify a profile for anomalous mitral arcade, we compared clinicopathological data from 11 sets of autopsied twin-twin transfusion syndrome fetuses, including 4 twin pairs in whom the recipient had anomalous mitral arcade (affected) and 7 pairs in whom both had structurally normal mitral valves (unaffected). Anomalous mitral arcade was characterized by a thick fibrous band at the free margin of the leaflets tethering papillary muscles and absent/short tendinous cords. One affected recipient also had pulmonary stenosis and tricuspid valve dysplasia. In all 11 sets, recipient hearts were larger than paired donor hearts. All 11 recipients had moderate to severe cardiac dysfunction by echocardiography. Echocardiography disclosed left atrial enlargement in all affected recipients but none of the unaffected recipients. Mitral regurgitation was present before demise in all affected recipients evaluated with color Doppler. Progressive decrease in mitral leaflet mobility was noted in those affected recipients with serial echocardiography. CONCLUSIONS: Previously unreported in twin-twin transfusion syndrome, anomalous mitral arcade was identified in 4 of 11 recipient fetuses (36%) in this autopsy series. Ultrasound or echocardiographic evidence of left atrial dilation, mitral regurgitation, and decreased leaflet mobility in recipients should raise suspicion for anomalous mitral arcade. Development of anomalous mitral arcade in twin-twin transfusion syndrome recipients suggests that the lesion is an acquired valve deformity in this setting, not a malformation.
Elizabeth Losada; Anita J Moon-Grady; William C Strohsnitter; Danny Wu; Philip C Ursell
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Publication Detail:
Type:  Journal Article; Twin Study     Date:  2010-09-27
Journal Detail:
Title:  Circulation     Volume:  122     ISSN:  1524-4539     ISO Abbreviation:  Circulation     Publication Date:  2010 Oct 
Date Detail:
Created Date:  2010-10-12     Completed Date:  2010-11-02     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0147763     Medline TA:  Circulation     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1456-63     Citation Subset:  AIM; IM    
Department of Pathology, University of California, San Francisco, 513 Parnassus Ave, Box 0511, San Francisco, CA 94143-0511, USA.
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MeSH Terms
Echocardiography, Doppler
Fetofetal Transfusion / epidemiology*,  ultrasonography
Fetus / abnormalities*
Heart Defects, Congenital / epidemiology*,  ultrasonography
Mitral Valve / abnormalities*,  ultrasonography
Mitral Valve Insufficiency / epidemiology*,  ultrasonography
Papillary Muscles / abnormalities
Pregnancy Outcome
Retrospective Studies
Ultrasonography, Prenatal

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

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