Association Between Tetralogy of Fallot and Tracheobronchial Branching Abnormalities: A New Clue for Pathogenesis?

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease

卷:7

Mathilde Meot¹  Alain Chantepie¹  Bruno Lefort¹  Baptiste Morel²  Dominique Sirinelli²  Elodie Carpentier²  Guillaume Chassagnon² 

[1] Pediatric Cardiology Department, Hôpital Clocheville, Université François Rabelais de Tours, France;

[2] Radiology Department, Hôpital Clocheville, Université François Rabelais de Tours, France;

关键词: computed tomography;    congenital heart disease;    pulmonary atresia;    tetralogy of Fallot;    tracheobronchial branching abnormalities;   

DOI  :  10.1161/JAHA.117.006921

来源: DOAJ

【 摘 要 】

BackgroundIn our practice, we noticed an increased frequency of tracheobronchial branching abnormalities (TBAs) in patients with tetralogy of Fallot (ToF). This study aimed to determine whether an association exists between congenital TBAs and ToF with or without pulmonary atresia. Methods and ResultsThe frequency of TBAs on chest computed tomography was assessed in 55 patients with ToF without pulmonary atresia, 34 patients with ToF with pulmonary arteria, and 100 control patients. We then looked for a possible association between TBAs and pulmonary artery branch hypoplasia, the presence of major aortopulmonary collateral arteries, and the presence of the chromosome 22q11 deletion. TBAs were significantly more frequent in patients with ToF with or without pulmonary atresia than in the control group (any TBAs, 21% versus 2% [P<0.001]; bronchial situs anomalies, 6% versus 0% [P=0.002]; right tracheal bronchus, 4% versus 0% [P=0.04]; left eparterial bronchus, 8% versus 0% [P=0.005]); and tended to be more frequent in those with ToF without pulmonary atresia than in those with ToF with pulmonary atresia (any TBAs, 27% versus 12% [P=0.11]; left eparterial bronchus, 13% versus 0% [P=0.04]). TBAs were readily multiple (8 patients of 19 with TBA) and concerned essentially the upper lobes. TBAs were not associated with pulmonary branch hypoplasia, major aortopulmonary collateral arteries, or the chromosome 22q11 deletion. ConclusionsWe demonstrated a significantly increased frequency of tracheobronchial abnormalities in patients with ToF with or without pulmonary atresia compared with a control group. These results suggest an interaction between abnormalities in conotruncal septation and tracheobronchial branching and may provide a new clue to the pathogenesis of conotruncal heart diseases.

【 授权许可】

Unknown