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Congenital heart disease
Original article
Factors associated with severe aortic dilation in patients with Fontan palliation
  1. Yuli Y Kim1,2,
  2. Rahul H Rathod1,2,
  3. Kimberlee Gauvreau1,2,
  4. Ellen M Keenan1,2,
  5. Pedro del Nido3,4,
  6. Tal Geva1,2
  1. 1Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA
  2. 2The Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA
  3. 3Department of Surgery, Harvard Medical School, Boston, Massachusetts, USA
  4. 4Department of Cardiovascular Surgery, Boston Children's Hospital, Boston, Massachusetts, USA
  1. Correspondence to Dr Tal Geva, Department of Cardiology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA; Tal.Geva{at}cardio.chboston.org

Abstract

Objective To describe the range of aortic dimensions and severity of aortic valve regurgitation (AR) in patients after the Fontan operation, examine rate of growth over time and identify risk factors for severe aortic dilation.

Methods This was a single-centre retrospective study of Fontan patients who underwent magnetic resonance angiography.

Results Between 2005 and 2014, there were 235 patients (median age 18.8 years). The aortic root (median Z-score 3.2) and ascending aorta (AAo) (median Z-score 4.1) were dilated. Severe aortic root or AAo dilation (Z-score ≥6.0) was present in 8.5% and 22%, respectively. Multivariable analysis identified older age at Fontan (adjusted odds ratio (AOR): 1.1 per year; 95% CI 1.04 to 1.2; p=0.002), male gender (AOR: 16.3; 95% CI 1.8 to 144.9; p=0.01) and higher mean blood pressure (AOR: 1.5 per 10 mm Hg; 95% CI 1.01 to 2.3; p=0.05) as factors associated with severe aortic root dilation. Older age at Fontan (AOR: 1.1 per year; 95% CI 1.02 to 1.1; p=0.01), male gender (AOR: 3.3; 95% CI 1.5 to 7.5; p=0.004) and left ventricular morphology (AOR: 2.6; 95% CI 1.3 to 5.1; p=0.007) were associated with severe AAo dilation. Over a median of 3.3 years, there was no significant increase in aortic dimension. Most patients (96%) had mild or less AR.

Conclusions The Fontan aorta is enlarged at the aortic root and AAo. Older age at Fontan, male gender, elevated blood pressure and left ventricular morphology are associated with severe aortic dilation. Although trivial-mild AR is common, significant regurgitation is infrequent.

https://doi.org/10.1136/heartjnl-2016-309615

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    Footnotes

    • YYK and RHR contributed equally to this study.

    • Contributors YYK planned and designed the study, collected data, analysed data, wrote the manuscript and provided critical revisions and is responsible for the overall content as a guarantor. RHR collected data, analysed data, wrote the manuscript, provided critical revisions and finalised the manuscript. KG analysed the data, performed all statistics, provided critical revisions and finalised the manuscript. EMK collected data and finalised the manuscript. PdN planned the study, provided critical revisions and finalised the manuscript. TG conceived of and designed the study, analysed data, provided critical revisions, finalised the manuscript and is responsible for the overall content as a guarantor.

    • Competing interests None declared.

    • Ethics approval The Scientific Review Committee of the Department of Cardiology and the Boston Children's Hospital Committee on Clinical Investigation approved review of the medical records.

    • Provenance and peer review Not commissioned; externally peer reviewed.