Open AccessFrequent Dilatation of the Descending Aorta in Children With Hypoplastic Left Heart Syndrome Relates to Decreased Aortic Arch Elasticity
Author(s) -
Voges Inga,
JeroschHerold Michael,
Wegner Philip,
Hart Christopher,
Gabbert Dominik,
Al Bulushi Abdullah,
Fischer Gunther,
Andrade Ana Cristina,
Pham Hoang Minh,
Kristo Ines,
Kramer HansHeiner,
Rickers Carsten
Publication year - 2015
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.115.002107
Subject(s) - medicine , cardiology , hypoplastic left heart syndrome , aortic arch , descending aorta , aorta , thoracic aorta , magnetic resonance imaging , radiology , heart disease
Background Patients with hypoplastic left heart syndrome after a Norwood operation show dilatation and reduced distensibility of the reconstructed proximal aorta. Cardiac magnetic resonance imaging ( CMR ) and angiographic examinations indicate that the native descending aorta ( DA o) is also dilated, but this has not been studied in detail. Methods and Results Seventy‐nine children with hypoplastic left heart syndrome in Fontan circulation (aged 6.3±3.2 years) and 18 control participants (aged 6.8±2.4 years) underwent 3.0‐tesla CMR . Gradient‐echo cine and phase‐contrast imaging was applied to measure cross‐sectional areas ( CSA s), distensibility, pulse wave velocity, and the incremental elastic modulus of the thoracic aorta. CSA of the DA o in patients was also compared with published percentiles for aortic CSA . Patients had significantly larger CSA of the DA o at the level of pulmonary artery bifurcation (229.1±97.2 versus 175.7±24.3 mm/m 2 , P =0.04) and the diaphragm (196.2±66.0 versus 142.6±16.7 mm/m 2 , P <0.01). In 41 patients (52%), CSA of the DA o was >95th percentile level for control participants, and the incremental elastic modulus of the aortic arch and the DA o was higher than in patients with normal CSA s (arch: 90.1±64.3 versus 45.6±38.9 m/s; DA o: 86.3±53.7 versus 47.1±47.6 m/s; P <0.01). Incremental elastic modulus of the aortic arch and the DA o correlated with the CSA of the DA o (arch: r =0.5; DA o: r =0.49; P <0.01). Conclusions Children with hypoplastic left heart syndrome frequently show dilatation of their DA o associated with increased stiffness of the aortic arch. Higher aortic impedance increases the afterload of the systemic circulation and likely contributes to the burden of the systemic right ventricle.