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Given the paucity of available literature, we sought to evaluate the mechanisms of tricuspid regurgitation and the outcomes of tricuspid valve surgery in the presence of permanent pacemakers.We retrospectively reviewed the records of 622 adult patients who underwent tricuspid valve surgery in the presence of permanent pacemakers between January 1993 and December 2013. Those with prosthetic tricuspid valve or tricuspid valve endocarditis and those undergoing concomitant heart transplant were excluded (n = 23). Patients were divided into 2 etiologic groups: pacemaker-associated tricuspid regurgitation (n = 349, 58%) and pacemaker-induced tricuspid regurgitation (n = 249, 42%). One patient was not categorized, because permanent pacemaker involvement was unknown.Mean age was 69.5 ± 12.0 years; 312 patients (52%) were female. In pacemaker-associated tricuspid regurgitation, the most common cause was functional (n = 304, 87%). The most common mechanism leading to pacemaker-induced tricuspid regurgitation was restricted leaflet mobility (n = 101, 41%), followed by adherent leaflet to the leads (n = 93, 37%), leaflet perforation (n = 30, 12%), scarring of leaflets (n = 19, 8%), and chordal entrapment (n = 18, 7%). The most common leaflet involved was septal leaflet (n = 182, 73%). Tricuspid valve repair (n = 215, 62%) was higher in the pacemaker-associated tricuspid regurgitation group. In multivariable analysis, pacemaker-induced tricuspid regurgitation was found to be protective with improved survival (hazard ratio [HR], 0.79; 95% confidence interval [CI], 0.68-0.98). Other independent risk factors of mortality included tricuspid valve replacement (HR, 1.50; 95% CI, 1.20-1.87), nonelective surgery (HR, 1.66; 95% CI, 1.33-2.08), diabetes (HR, 1.37; 95% CI, 1.09-1.73), severe tricuspid regurgitation (HR, 1.42; 95% CI, 1.04-1.95), and older age when there was a concomitant aortic valve surgery (HR, 1.44; 95% CI, 1.15-1.79).Several mechanisms lead to pacemaker-induced tricuspid regurgitation. Pacemaker-induced tricuspid regurgitation when compared with pacemaker-associated tricuspid regurgitation carries a better prognosis with improved survival.