| Dextrocardia in patients with Poland syndrome: phenotypic characterization provides insight into the pathogenesis. | |
| | |
MedLine Citation:
|
PMID: 19909992 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
OBJECTIVE: Poland syndrome is a rare congenital anomaly characterized by complete or partial agenesis of the pectoralis major muscle variably associated with other thoracic malformations, upper limb malformations, or both. More than 20 patients with dextrocardia and left-sided Poland syndrome have been previously described. The association between these 2 rare anomalies suggests a causal relationship, but the etiopathogenetic mechanism has not been clarified yet. We studied the clinical correlation between these 2 anomalies, and we tried to elucidate whether dextrocardia or Poland syndrome comes first. METHODS: This is a multicentric multidisciplinary study conducted over the last 5 years. We identified 122 patients with Poland syndrome, and we investigated heart position through different imaging techniques. Logistic regression statistical analyses were carried out. RESULTS: We observed dextrocardia in 14 (11.5%) patients, which was never associated with situs inversus. All of them presented with left-sided Poland syndrome and partial agenesis of 2 or more ribs. Conversely, all patients with Poland syndrome with partial agenesis of 2 or more ribs presented with dextrocardia, whereas dextrocardia was never associated with partial agenesis of a single rib. Three patients with dextrocardia presented with simple congenital heart defects. CONCLUSIONS: These findings suggest that mechanical factors during embryonic life could explain the strong association between left-sided Poland syndrome and dextrocardia. According to this hypothesis, partial agenesis of 2 or more ribs is needed to displace the heart toward the right side. The peculiar features of dextrocardia when associated with Poland syndrome (neither associated with situs inversus nor complex intracardiac anomalies) support our hypothesis. |
| | |
Authors:
|
Michele Torre; Anwar Baban; Anna Buluggiu; Sara Costanzo; Lara Bricco; Margherita Lerone; Sebastiano Bianca; Gian Luca Gatti; Filippo Maria S?n?s; Maura Valle; Maria Grazia Calevo |
Publication Detail:
|
Type: Journal Article; Multicenter Study Date: 2009-11-11 |
Journal Detail:
|
Title: The Journal of thoracic and cardiovascular surgery Volume: 139 ISSN: 1097-685X ISO Abbreviation: J. Thorac. Cardiovasc. Surg. Publication Date: 2010 May |
Date Detail:
|
Created Date: 2010-04-23 Completed Date: 2010-05-06 Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 0376343 Medline TA: J Thorac Cardiovasc Surg Country: United States |
Other Details:
|
Languages: eng Pagination: 1177-82 Citation Subset: AIM; IM |
Copyright Information:
|
2010 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved. |
Affiliation:
|
Pediatric Surgery Department, Gaslini Children Hospital, Genova, Italy. micheletorre@hotmail.com |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Abnormalities, Multiple* Adolescent Adult Child Child, Preschool Dextrocardia / etiology*, pathology, radiography Female Humans Infant Italy Logistic Models Male Middle Aged Odds Ratio Phenotype Poland Syndrome / complications*, pathology, radiography, ultrasonography Risk Assessment Risk Factors Young Adult |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Evolution of treatment options and outcomes for hypoplastic left heart syndrome over an 18-year peri...
Next Document: Influence of timing of intraaortic balloon placement in cardiac surgical patients.