Chronic pulmonary regurgitation (PR) is an important cause of morbidity and mortality in long-term survivors of tetralogy of Fallot (TOF) repair. Although early results of pulmonary valve replacement (PVR) in patients with repaired TOF and severe PR have demonstrated that surgery is associated with very low mortality, our data and that of other investigators have demonstrated that despite successful restoration of pulmonary valve competence and elimination of the RV volume overload, RV dysfunction often persists or even worsens. We hypothesize that persistence of aneurysmal and akinetic RV wall segments, which are not addressed by PVR alone, contribute to persistent RV dysfunction in long-term survivors of TOF repair. This prospective, randomized, single-center trial aims to compare the effects of two surgical strategies, PVR alone (standard treatment) vs. PVR and surgical RV remodeling, on RV mechanics in patients with repaired TOF and chronic PR. Patients who are eligible for enrollment based on standardized preoperative quantitative cardiac MRI criteria will be randomly assigned to PVR alone or to PVR and surgical RV remodeling. In both study arms patients will receive a bioprosthetic valve in the pulmonary position (standard treatment). In the PVR + RV remodeling group, the aneurysmal and akinetic portions of the RV free wall will be resected to reduce RV size in addition to PVR. The first specific aim is to compare the effects of the two surgical strategies on RV mechanics six months after surgery. The primary outcome variable is the change in RV ejection fraction six months postoperatively compared with preoperatively as measured by cardiac MRI. Secondary outcome variables are changes in RV and LV volumes, mass, mass-to-volume ratio, and recruitable ventricular stroke volume and ejection fraction in response to submaximal Dobutamine stress (by cardiac MRI) measured six months postoperatively compared with preoperative values. Other secondary outcome variables include a functional status score (SF-36), exercise capacity (treadmill test), pulmonary function, and a comprehensive noninvasive assessment of arrhythmias and conduction. The second specific aim is to compare the incidence of postoperative adverse events occurring in the PVR plus surgical RV remodeling group to those in the PVR alone group. The total sample size is 100 patients, to be recruited over four years.
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