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Left Ventricular Rehabilitation is Effective in Maintaining Two-Ventricle Physiology in the Borderline Left Heart
Sitaram Emani, Emile A. Bacha, Doff McElhinney, Gerald Marx, Wayne Tworetsky, Frank A. Pigula, Pedro J. del Nido; Childrens Hospital Boston, Boston, MA

Objective: In borderline left heart (BLH) disease, there is generally some degree of endocardial fibroelastosis (EFE), mitral valve dysfunction, and/or aortic stenosis. The multilevel obstruction and impaired left ventricular (LV) systolic and diastolic function place such patients at high risk for biventricular repair. We studied the effects of EFE resection with mitral and/or aortic valvuloplasty on LV diastolic and systolic function.
Methods: All patients with BLH structures and EFE who underwent an LV rehabilitation procedure (LV rehab) consisting of EFE resection and mitral valve repair, with or without aortic valvuloplasty, were retrospectively analyzed to determine operative mortality, reintervention rates, and hemodynamic status. Echocardiographic measures obtained pre- and post-operatively included ejection fraction, LV end diastolic volume (EDV), LV mass/volume ratio, and estimated right ventricular (RV) pressure. At cardiac catheterization, left atrial (LAp) and RV/LV pressure ratios were obtained. Postoperative LAp was obtained from the LA line early after LV rehab. Pre- and post-operative values were compared by paired t-test.
Results: Between 1999 and 2007, 9 patients with EFE and BLH structures underwent LV rehab at a median age of 5.6 months (range 1-38 months). None had associated ventricular septal defects. Interventions prior to LV rehab included coarctation repair (4/9) and aortic valve balloon dilation either in utero (5/9) or postnatally (7/9). LV rehab consisted of mitral valvuloplasty and EFE resection (9/9 patients), aortic valvuloplasty (4/9), and subaortic resection (2/9). There was no operative mortality, and at a median follow up of 13 months (1 to 95 months), there was one death from non cardiac causes (motor vehicle collision). Two patients required reoperations, one for mitral valve replacement, and another for aortic and mitral valve repairs. No patients required single ventricle palliation or heart transplantation. Table 1 summarizes average pre- and postoperative hemodynamic data. Significant increase in EF and LVEDV were observed, whereas LAp, and RV/LV ratios decreased postoperatively.
Conclusion: In patients with BLH disease, LV rehab with surgical EFE resection and mitral and aortic valvuloplasty results in improved LV systolic and diastolic performance and decreased RV pressures. This approach may provide an alternative to single ventricle management in this difficult patient group.

Table 1
Preoperative Postoperative
Ejection fraction (%) 36±12 58±10 P<0.01
LVEDV z score -0.17±1.7 2.72±1.8 P<0.05
Mass/Vol ratio z score 0.68±1.15 0.10±2.1
LA pressure (mmHg) 27.5+6.3 11+2.4 P<0.01
RV/LV systolic pressure ratio 0.78±0.36 0.32±0.11 P<0.05


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