Open AccessAssociation Between Tetralogy of Fallot and Tracheobronchial Branching Abnormalities: A New Clue for Pathogenesis?
Author(s) -
Chassag Guillaume,
Lefort Bruno,
Meot Mathilde,
Carpentier Elodie,
Sirinelli Dominique,
Chantepie Alain,
Morel Baptiste
Publication year - 2018
Publication title -
journal of the american heart association
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.494
H-Index - 85
ISSN - 2047-9980
DOI - 10.1161/jaha.117.006921
Subject(s) - medicine , pulmonary atresia , tetralogy of fallot , hypoplasia , bronchus , cardiology , atresia , pulmonary artery , left pulmonary artery , lung , heart disease , respiratory disease
Background In our practice, we noticed an increased frequency of tracheobronchial branching abnormalities ( TBA s) in patients with tetralogy of Fallot (ToF). This study aimed to determine whether an association exists between congenital TBA s and ToF with or without pulmonary atresia. Methods and Results The frequency of TBA s 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 TBA s and pulmonary artery branch hypoplasia, the presence of major aortopulmonary collateral arteries, and the presence of the chromosome 22q11 deletion. TBA s were significantly more frequent in patients with ToF with or without pulmonary atresia than in the control group (any TBA s, 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 TBA s, 27% versus 12% [ P =0.11]; left eparterial bronchus, 13% versus 0% [ P =0.04]). TBA s were readily multiple (8 patients of 19 with TBA ) and concerned essentially the upper lobes. TBA s were not associated with pulmonary branch hypoplasia, major aortopulmonary collateral arteries, or the chromosome 22q11 deletion. Conclusions We 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.