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Effectiveness of Aortic Valve Replacement in Heyde Syndrome
Quick Takes
- In a meta-analysis of studies of patients with Heyde syndrome who underwent AVR, 87% of patients had recovery from acquired von Willebrand syndrome and 73% of patients had resolution of gastrointestinal bleeding (GIB).
- Residual aortic valve disease (paravalvular leak or prosthesis-patient mismatch) was associated with lower likelihoods of recovery for both acquired von Willebrand syndrome and GIB.
- Subanalysis revealed faster resolution of acquired von Willebrand syndrome and a higher rate of cessation of GIB after SAVR compared with TAVR.
Study Questions:
Among patients with Heyde syndrome, what are the effects of aortic valve replacement (AVR) on acquired von Willebrand syndrome and gastrointestinal bleeding (GIB)?
Methods:
The literature search identified 33 studies (32 observational studies and one randomized controlled trial) on acquired von Willebrand syndrome (total 1,054 patients who underwent AVR between 2000 and 2022) and 11 observational studies on GIB (total 300 patients who underwent AVR between 1968 and 2022). One study (six patients) reported on both disorders. The pooled proportion of Heyde syndrome patients with acquired von Willebrand syndrome recovery was 86% (95% confidence interval [CI], 79-91%) at T1, 90% (95% CI, 74-96%) at T2, 92% (95% CI, 84-96%) at T3, and 87% (95% CI, 67-96%) at T4. The pooled proportion of Heyde syndrome patients with GIB cessation was 73% (95% CI, 62-81%). Residual aortic valve disease (paravalvular leak or prosthesis-patient mismatch) was associated with lower recovery rates of acquired von Willebrand syndrome (relative risk [RR], 0.20; 95% CI, 0.05-0.72; p = 0.014) and GIB (RR, 0.57; 95% CI, 0.40-0.81; p = 0.002).
In studies that distinguished surgical (SAVR) and transcatheter AVR (TAVR), acquired von Willebrand syndrome recovery occurred earlier after SAVR than after TAVR (91.5% [95% CI, 61.9-98.6%] vs. 85.3% [95% CI, 77.3-90.9%] at T1, p = 0.092; 95.0% [95% CI, 87.4-98.1%] vs. 52.7% [95% CI, 47.2-58.2%] at T2, p = 0.002; and 93.9% [95% CI, 86.1-97.5%] vs. 91.1% [95% CI, 72.5-97.6%] at T3; p = 0.739), and GIB cessation was higher after SAVR than after TAVR (82.0% [95% CI, 71.8-89.1%] vs. 64.2% [95% CI, 49.8-76.5%]; p = 0.003). An intensified antithrombotic regimen had a relative risk for GIB of 0.94 (95% CI, 0.70-1.27; p = 0.704).
Results:
The literature search identified 33 studies (32 observational studies and one randomized controlled trial) on acquired von Willebrand syndrome (total 1,054 patients who underwent AVR between 2000 and 2022) and 11 observational studies on GIB (total 300 patients who underwent AVR between 1968 and 2022). One study (six patients) reported on both disorders. The pooled proportion of Heyde syndrome patients with acquired von Willebrand syndrome recovery was 86% (95% confidence interval [CI], 79-91%) at T1, 90% (74-96%) at T2, 92% (84-96%) at T3, and 87% (67-96%) at T4. The pooled proportion of Heyde syndrome patients with GIB cessation was 73% (95% CI, 62-81%). Residual aortic valve disease (paravalvular leak or prosthesis-patient mismatch) was associated with lower recovery rates of acquired von Willebrand syndrome (relative risk [RR], 0.20; 95% CI, 0.05-0.72; p = 0.014) and GIB (RR, 0.57; 95% CI, 0.40-0.81; p = 0.002).
In studies that distinguished surgical (SAVR) and transcatheter AVR (TAVR), acquired von Willebrand syndrome recovery occurred earlier after SAVR than after TAVR (91.5% [95% CI, 61.9-98.6%] vs. 85.3% [95% CI, 77.3-90.9%] at T1, p = 0.092; 95.0% [95% CI, 87.4-98.1%] vs. 52.7% [95% CI, 47.2-58.2%] at T2, p = 0.002; and 93.9% [95% CI, 86.1-97.5%] vs. 91.1% [95% CI, 72.5-97.6%] at T3; p = 0.739), and GIB cessation was higher after SAVR than after TAVR (82.0% [95% CI, 71.8-89.1%] vs. 64.2% [95% CI, 49.8-76.5%]; p = 0.003). An intensified antithrombotic regimen had a relative risk for GIB of 0.94 (95% CI, 0.70-1.27; p = 0.704).
Conclusions:
Among patients with Heyde syndrome, AVR is associated with rapid recovery of acquired von Willebrand syndrome and the cessation of GIB, but residual valve disease was associated with lower rates of improvement for both.
Perspective:
Acquired von Willebrand syndrome and GIB in the setting of aortic stenosis (Heyde syndrome) previously have been shown to improve following AVR. This meta-analysis found that recovery from acquired von Willebrand syndrome occurred in 87% of patients and resolution of GIB occurred in 73% of patients following AVR; with residual aortic valve disease (paravalvular leak or prosthesis-patient mismatch) associated with lower likelihoods of recovery for both, and no influence of antithrombotic therapy intensity on the cessation of GIB. Subanalysis revealed faster resolution of acquired von Willebrand syndrome and a higher rate of cessation of GIB after SAVR compared with TAVR (possibly related to a higher rate of residual aortic valve disease in the form of paravalvular leak after TAVR). Large, prospective studies would be of interest to address the potential role of residual valve disease on outcomes and any differences between TAVR and SAVR on acquired von Willebrand syndrome and GIB among patients with Heyde disease who undergo intervention.
Clinical Topics: Cardiac Surgery, Invasive Cardiovascular Angiography and Intervention, Valvular Heart Disease, Cardiac Surgery and VHD, Interventions and Structural Heart Disease
Keywords: Cardiac Surgical Procedures, Heart Valve Diseases, Hemorrhage, Transcatheter Aortic Valve Replacement, von Willebrand Diseases
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