Regional Myocardial Dysfunction following Norwood with Right Ventricle to Pulmonary Artery Conduit in Patients with Hypoplastic Left Heart Syndrome

Background: Improved early survival has led many centers to use the right ventricle-to-pulmonary artery (RVPA) conduit instead of the modified Blalock-Taussig shunt for Norwood palliation of hypoplastic left-heart syndrome. However, there is concern regarding the potential deleterious effects of the required right ventriculotomy for placement of the RVPA conduit on global and regional right ventricular (RV) function. The purpose of this study was to investigate global and regional RV wall motion abnormalities after Norwood palliation with RVPA conduit using Velocity Vector Imaging (VVI). Methods: Thirty consecutive patients with hypoplastic left-heart syndrome who underwent stage 2 palliation between January 2007 and December 2009 were identified from the surgical database. VVI was performed on two-dimensional echocardiographic images obtained before second-stage palliation. Peak systolic circumferential and radial velocity, strain, and strain rate were measured from parasternal short-axis and apical four-chamber views. RV ejection fraction was measured using the biplane modified Simpson's rule. Regional RV systolic deformations were compared between different RV segments. VVI measures were also compared with RV systolic function. In a subgroup (n = 14), VVI was repeated on follow-up after stage 2 palliation to evaluate changes in regional and global RV deformation. Results: A total of 30 patients (20 males) were studied. The median age at the time of interstage echocardiography was 12 weeks (range, 8-18 weeks). In the short axis, average peak systolic circumferential strain values for the anterior, posterior, septal, and RV free wall segments were 3.79 +/- 2.52%, 11.4 +/- 5.2%, 13.3 +/- 6.5%, and 11.1 +/- 5.0%, respectively. From the short-axis view, the anterior RV segment (ventriculotomy site) exhibited significantly reduced circumferential velocity, peak systolic strain, and strain rate (P < .0001). Mean global VVI measurements were correlated with RV ejection fraction. On follow-up after stage 2 palliation, the ventriculotomy region showed persistently reduced velocity, peak systolic strain, and strain rate compared with all other segments. Conclusions: In patients with hypoplastic left-heart syndrome after Norwood palliation with RVPA conduit, RV myocardial deformation was significantly reduced at the ventriculotomy site, which persisted after stage 2 palliation. VVI-derived measures demonstrating impairment of global systolic myocardial deformation were correlated with RV systolic function. Long-term multicenter studies to evaluate the effects of ventriculotomy scar on single systemic right ventricle are required. (J Am Soc Echocardiogr 2011; 24: 826-33.)