BACKGROUND

Outcome status in patients with hypoplastic left heart syndrome (HLHS) is partially dependent on right ventricular (RV) systolic function. In other disease states, ventricular function is impacted by anatomy and physiology of the contralateral ventricle. In HLHS, it is suggested that a relatively larger left ventricular (LV) size may negatively impact RV function because it becomes a "passenger" without providing any systolic or diastolic physiologic benefit. The purpose of this study was to determine whether LV size adversely affects RV systolic function in surviving patients with HLHS.

METHODS

The hospital database was searched for all patients with HLHS and technically adequate echocardiograms born in the last 6 years and who had survived at least the Norwood procedure. LV size was assessed by echocardiographic measurement of LV end-diastolic short-axis and apical area. RV function was assessed by short-axis and apical fractional area change as well as the myocardial performance index (Tei). Measurements were made at up to 4 time points depending on duration of follow-up (1 - pre-Norwood; 2 - pre-Glenn; 3 - pre-Fontan; and 4- post-Fontan).

RESULTS

A total of 48 patients were studied. LV size showed sufficient variability in the patient population (1.0-21 cm(2)/body surface area, pre-Norwood). RV function tended to worsen across the time periods but these changes did not reach statistical significance. Regression analysis showed no effect of LV size on RV function before Norwood operation. Significant correlations existed between LV size indices and RV functional indices before Glenn shunt but these were inconsistent in the direction of their effect. Only before Fontan operation did the correlation between LV size and RV function become both consistent and statistically significant; specifically larger LV size correlated significantly with poor RV systolic function (short-axis RV fractional area change vs LV area r = -0.4, P = .03 and RV Tei vs LV area r = 0.5, P = .02). These relationships were not apparent after Fontan operation.

CONCLUSION

In surviving patients with HLHS, larger LV size does not seem to negatively impact RV function before or after Norwood procedure nor does it seem to have an adverse effect on RV function chronically (after Fontan). However, further study with larger population size will be necessary to see whether these findings remain negative and are true for nonsurvivors as well.