title: Ventricular function deteriorates with recurrent coarctation in hypoplastic left heart syndrome.
abstract: BACKGROUND
  Recurrent coarctation (re-CoA) after stage I palliation in hypoplastic left heart syndrome (HLHS) is deleterious. We studied whether re-CoA had an effect on ventricular systolic function.
  
  
  METHODS
  Retrospectively reviewed were HLHS patients surviving stage I Norwood palliation (stage I) and cavopulmonary shunt (CPS) between January 2004 and February 2007. Echocardiographic right ventricular fractional area change (RV-FAC) was used to evaluate ventricular systolic function after stage I, before CPS, and before Fontan procedures. Cardiac catheterization and magnetic resonance imaging data before CPS were reviewed to assess re-CoA, using a coarctation index (CI = isthmus diameter/descending aortic diameter).
  
  
  RESULTS
  Fifty-one patients were included, and 21 had a CI of less than 0.75 (mean, 0.82 +/- 0.19; 21). Twelve patients required arch balloon dilation between CPS and Fontan. The change of RV-FAC for all patients between stage I and CPS was -2.2% +/- 9.6%. Pearson correlation coefficient demonstrated a significant correlation between lower CI values and lower RV-FAC at the pre-CPS echocardiogram (r = .35, p = 0.03); and lower CI values and greater decrease in RV-FAC between stage I and pre-CPS evaluation (r = 0.40, p = 0.018). At follow-up pre-Fontan, RV-FAC for patients who underwent balloon dilation for re-CoA recovered to a level that was inferior but not significantly different from that of patients who did not need balloon dilation.
  
  
  CONCLUSIONS
  Recurrent aortic arch obstruction after stage I for HLHS is associated with worse RV systolic function at the time of stage II operation. Timely intervention on the re-CoA results in recovery of RV function.
authors: Department of Cardiac Surgery, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA.
authors: Luis Alesandro
authors: LA
authors: Larrazabal
authors: Elif Seda
authors: ES
authors: Selamet Tierney
authors: David W
authors: DW
authors: Brown
authors: Kimberlee
authors: K
authors: Gauvreau
authors: Vladimiro L
authors: VL
authors: Vida
authors: Lisa
authors: L
authors: Bergersen
authors: Frank A
authors: FA
authors: Pigula
authors: Pedro J
authors: PJ
authors: del Nido
authors: Emile A
authors: EA
authors: Bacha
mesh_terms: D001017:Aortic Coarctation / Q000503:physiopathology*; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000150:complications* / Q000628:therapy; D007231:Infant, Newborn; D010166:Palliative Care; D012008:Recurrence; D012189:Retrospective Studies; D016278:Ventricular Function, Right / Q000502:physiology*