Objective To explore the characteristics and clinical outcomes of patients with Heyde syndrome (HS) who undergo aortic valve replacement (AVR). Methods Electronic databases including PubMed, Embase, Ovid, WANFANG, VIP and CNKI were searched to identify all case reports of HS patients undergoing AVR surgery, using different combinations of search terms "Heyde syndrome", "gastrointestinal bleeding", "aortic stenosis", and "surgery". Three authors independently extracted the clinical data including the patients' characteristics, aortic stenosis severity, gastrointestinal bleeding sites, surgical treatments and prognosis. Results Finally, 46 case reports with 55 patients aging from 46 to 87 years, were determined eligible and included. Of them, 1 patient had mild aortic stenosis, 1 had moderate aortic stenosis, 42 had severe aortic stenosis, and 11 were not mentioned. Gastrointestinal bleeding was detected in colon (n=8), jejunum (n=6), ileum (n=4), cecum (n=3), duodenal (n=3) and multiple sites (n=8). No specific bleeding site was identified in 23 patients. Preoperative hemoglobin level ranged from 43 to 117 g/L. All but one of 16 patients showed decreased level of high molecule weight von Willebrand factor. Of the 55 patients, 43 underwent AVR, and 12 received transcatheter AVR. Aortic valves of 14 cases were replaced by mechanical valves, and 33 cases by biological valves. All patients recovered well during the follow-up, except 5 patients. One patient who had perivalvular leakage and gastrointestinal bleeding after AVR underwent the second AVR. Two patients had recurrent gastrointestinal bleeding. Two patients died of life-threatening acute subdural hematoma and multiple organ failure, respectively. Conclusions HS is a rare syndrome characterized by aortic stenosis and gastrointestinal bleeding. AVR is an effective treatment for HS.
Keywords: Heyde syndrome; aortic valve stenosis; gastrointestinal bleeding.