Ventricular function deteriorates with recurrent coarctation in hypoplastic left heart syndrome.
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.
Larrazabal
Luis Alesandro
LA
Department of Cardiac Surgery, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA.
Selamet Tierney
Elif Seda
ES
Brown
David W
DW
Gauvreau
Kimberlee
K
Vida
Vladimiro L
VL
Bergersen
Lisa
L
Pigula
Frank A
FA
del Nido
Pedro J
PJ
Bacha
Emile A
EA
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*