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Published on: August 8, 2025
Heyde Syndrome Complicated by Pulmonary Embolism Before Transcatheter Aortic Valve Replacement: A Clinical Dilemma
Mingying Cao1, Lingli Zhang1, Ying Ren1
1Department of Cardiology, Tianjin Union Medical Center, The First Affiliated Hospital of Nankai University, Tianjin, China.
BACKGROUND Heyde syndrome is an uncommon clinical entity characterized by severe aortic stenosis (AS) and acquired von Willebrand syndrome, typically presenting with recurrent gastrointestinal bleeding secondary to angiodysplasia. Although most reported cases involve isolated gastrointestinal bleeding, the coexistence of thromboembolic events is exceedingly rare and poses a significant therapeutic challenge in balancing hemostatic and anticoagulant strategies. CASE REPORT We report a 70-year-old woman who initially presented with massive hematochezia and subsequently developed dyspnea, requiring hospitalization. Physical examination revealed a prominent systolic murmur over the aortic area. Transthoracic echocardiography confirmed severe AS, with an aortic valve area of 0.8 cm² and a mean transvalvular pressure gradient of 71 mm Hg. Together with profound anemia (hemoglobin 44 g/L) and markedly reduced von Willebrand factor ristocetin cofactor activity (vWF: RCo, 25.3%), these findings supported the diagnosis of Heyde syndrome. During the preprocedural evaluation for transcatheter aortic valve replacement (TAVR), acute pulmonary embolism was incidentally identified on computed tomography pulmonary angiography. After hemostatic stabilization, anticoagulant therapy was cautiously initiated, resulting in complete resolution of the pulmonary embolism after 1 month. Given the elevated surgical risk (EuroSCORE II, 8.05%), the patient underwent successful TAVR. Following the procedure, the transvalvular pressure gradient normalized (mean, 13.8 mm Hg), with restoration of normal vWF activity. CONCLUSIONS This case underscores the therapeutic complexity of simultaneously managing hemorrhagic and thrombotic risks in Heyde syndrome. TAVR remains the definitive treatment for acquired von Willebrand syndrome, while a staged, individualized anticoagulation approach is crucial in patients with concomitant thromboembolic complications. Correcting the underlying AS remains the cornerstone of management.
BACKGROUND Heyde syndrome is an uncommon clinical entity characterized by severe aortic stenosis (AS) and acquired von Willebrand syndrome, typically presenting with recurrent gastrointestinal bleeding secondary to angiodysplasia. Although most reported cases involve isolated gastrointestinal bleeding, the coexistence of thromboembolic events is exceedingly rare and poses a significant therapeutic challenge in balancing hemostatic and anticoagulant strategies. CASE REPORT We report a 70-year-old woman who initially presented with massive hematochezia and subsequently developed dyspnea, requiring hospitalization. Physical examination revealed a prominent systolic murmur over the aortic area. Transthoracic echocardiography confirmed severe AS, with an aortic valve area of 0.8 cm² and a mean transvalvular pressure gradient of 71 mm Hg. Together with profound anemia (hemoglobin 44 g/L) and markedly reduced von Willebrand factor ristocetin cofactor activity (vWF: RCo, 25.3%), these findings supported the diagnosis of Heyde syndrome. During the preprocedural evaluation for transcatheter aortic valve replacement (TAVR), acute pulmonary embolism was incidentally identified on computed tomography pulmonary angiography. After hemostatic stabilization, anticoagulant therapy was cautiously initiated, resulting in complete resolution of the pulmonary embolism after 1 month. Given the elevated surgical risk (EuroSCORE II, 8.05%), the patient underwent successful TAVR. Following the procedure, the transvalvular pressure gradient normalized (mean, 13.8 mm Hg), with restoration of normal vWF activity. CONCLUSIONS This case underscores the therapeutic complexity of simultaneously managing hemorrhagic and thrombotic risks in Heyde syndrome. TAVR remains the definitive treatment for acquired von Willebrand syndrome, while a staged, individualized anticoagulation approach is crucial in patients with concomitant thromboembolic complications. Correcting the underlying AS remains the cornerstone of management.
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