Looks Do Matter! Aortic Arch Shape After Hypoplastic Left Heart Syndrome Palliation Correlates With Cavopulmonary Outcomes

Ann Thorac Surg. 2017 Feb;103(2):645-654. doi: 10.1016/j.athoracsur.2016.06.041. Epub 2016 Sep 1.


Background: Aortic arch reconstruction after hypoplastic left heart syndrome (HLHS) palliation can vary widely in shape and dimensions between patients. Arch morphology alone may affect cardiac function and outcome. We sought to uncover the relationship of arch three-dimensional shape features with functional and short-term outcome data after total cavopulmonary connection (TCPC).

Methods: Aortic arch shape models of 37 patients with HLHS (age, 2.89 ± 0.99 years) were reconstructed from magnetic resonance data before TCPC completion. A novel, validated statistical shape analysis method was used to compute a three-dimensional anatomic mean shape from the cohort and calculate the deformation vectors of the mean shape toward each patient's specific anatomy. From these deformations, three-dimensional shape features most related to ventricular ejection fraction, indexed end-diastolic volume, and superior cavopulmonary pressure were extracted by partial least-square regression analysis. Shape patterns relating to intensive care unit and hospital lengths of stay after TCPC were assessed.

Results: Distinct deformation patterns, which result in an acutely mismatched aortic root and ascending aorta, and a gothic-like transverse arch, correlated with increased indexed end-diastolic volume and higher superior cavopulmonary pressure but not with ejection fraction. Specific arch morphology with pronounced transverse arch and descending aorta mismatch also correlated with longer intensive care unit and hospital lengths of stay after TCPC completion.

Conclusions: Independent of hemodynamically important arch obstruction, altered aortic morphology in HLHS patients appears to have important associations with higher superior cavopulmonary pressure and with short-term outcomes after TCPC completion as highlighted by statistical shape analysis, which could act as adjunct to risk assessment in HLHS.

MeSH terms

  • Aorta, Thoracic / diagnostic imaging*
  • Aorta, Thoracic / surgery
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Fontan Procedure / methods*
  • Humans
  • Hypoplastic Left Heart Syndrome / diagnosis
  • Hypoplastic Left Heart Syndrome / physiopathology
  • Hypoplastic Left Heart Syndrome / surgery*
  • Imaging, Three-Dimensional
  • Magnetic Resonance Imaging, Cine / methods*
  • Male
  • Palliative Care / methods*
  • Retrospective Studies
  • Stroke Volume / physiology*
  • Treatment Outcome