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Remission of recurrent gastrointestinal bleeding after septal reduction therapy in patients with hypertrophic obstructive cardiomyopathy-associated acquired von Willebrand syndrome.
J Thromb Haemost 2015; 13(2):191-6JT

Abstract

BACKGROUND

Gastrointestinal hemorrhage is considered to be a severe complication of von Willebrand disease. The optimal therapy for acquired von Willebrand syndrome and severe gastrointestinal bleeding with hypertrophic cardiomyopathy is undefined.

PATIENTS/METHODS

Seventy-seven patients (median age, 67 years; interquartile range [IQR], 56-75 years; 49% women) with hypertrophic cardiomyopathy underwent von Willebrand factor multimer testing and acquisition of bleeding history. Bleeding was detected in 27 (36%) (median age, 74 years; IQR 66-76 years; 74% women), 20 with gastrointestinal bleeding, including 11 women with transfusion dependence. In these 11 women, the median duration of transfusion dependency was 36 months (IQR 18-44 months), and the median number of transfusions required was 25 (IQR 20-38). Two patients had undergone bowel resection for bleeding, one of them twice. Seven patients showed angiodysplasia, and the remainder had no endoscopic lesion. Bleeding recurred after bowel surgery or endoscopic intervention and medical therapy for hypertrophic cardiomyopathy in 10 of 11 patients. Two patients had septal myectomy, and six patients underwent alcohol septal ablation. With the exception of one patient in whom a significant gradient persisted after septal ablation, after the periprocedural period, patients after septal reduction therapy remained free of recurrent bleeding and need for transfusions.

CONCLUSION

Acquired von Willebrand syndrome is common in hypertrophic cardiomyopathy. Gastrointestinal bleeding often recurs after endoscopic therapy, but may be relieved by structural cardiac repair.

Authors+Show Affiliations

Division of Cardiovascular Diseases, Mayo Clinic Florida, Jacksonville, FL, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural

Language

eng

PubMed ID

25387993

Citation

Blackshear, J L., et al. "Remission of Recurrent Gastrointestinal Bleeding After Septal Reduction Therapy in Patients With Hypertrophic Obstructive Cardiomyopathy-associated Acquired Von Willebrand Syndrome." Journal of Thrombosis and Haemostasis : JTH, vol. 13, no. 2, 2015, pp. 191-6.
Blackshear JL, Stark ME, Agnew RC, et al. Remission of recurrent gastrointestinal bleeding after septal reduction therapy in patients with hypertrophic obstructive cardiomyopathy-associated acquired von Willebrand syndrome. J Thromb Haemost. 2015;13(2):191-6.
Blackshear, J. L., Stark, M. E., Agnew, R. C., Moussa, I. D., Safford, R. E., Shapiro, B. P., ... Chen, D. (2015). Remission of recurrent gastrointestinal bleeding after septal reduction therapy in patients with hypertrophic obstructive cardiomyopathy-associated acquired von Willebrand syndrome. Journal of Thrombosis and Haemostasis : JTH, 13(2), pp. 191-6. doi:10.1111/jth.12780.
Blackshear JL, et al. Remission of Recurrent Gastrointestinal Bleeding After Septal Reduction Therapy in Patients With Hypertrophic Obstructive Cardiomyopathy-associated Acquired Von Willebrand Syndrome. J Thromb Haemost. 2015;13(2):191-6. PubMed PMID: 25387993.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Remission of recurrent gastrointestinal bleeding after septal reduction therapy in patients with hypertrophic obstructive cardiomyopathy-associated acquired von Willebrand syndrome. AU - Blackshear,J L, AU - Stark,M E, AU - Agnew,R C, AU - Moussa,I D, AU - Safford,R E, AU - Shapiro,B P, AU - Waldo,O A, AU - Chen,D, Y1 - 2014/12/12/ PY - 2014/08/05/received PY - 2014/11/13/entrez PY - 2014/11/13/pubmed PY - 2015/11/17/medline KW - cardiomyopathy hypertrophic KW - endoscopy KW - gastrointestinal KW - hemorrhage KW - von Willebrand diseases KW - von Willebrand factor SP - 191 EP - 6 JF - Journal of thrombosis and haemostasis : JTH JO - J. Thromb. Haemost. VL - 13 IS - 2 N2 - BACKGROUND: Gastrointestinal hemorrhage is considered to be a severe complication of von Willebrand disease. The optimal therapy for acquired von Willebrand syndrome and severe gastrointestinal bleeding with hypertrophic cardiomyopathy is undefined. PATIENTS/METHODS: Seventy-seven patients (median age, 67 years; interquartile range [IQR], 56-75 years; 49% women) with hypertrophic cardiomyopathy underwent von Willebrand factor multimer testing and acquisition of bleeding history. Bleeding was detected in 27 (36%) (median age, 74 years; IQR 66-76 years; 74% women), 20 with gastrointestinal bleeding, including 11 women with transfusion dependence. In these 11 women, the median duration of transfusion dependency was 36 months (IQR 18-44 months), and the median number of transfusions required was 25 (IQR 20-38). Two patients had undergone bowel resection for bleeding, one of them twice. Seven patients showed angiodysplasia, and the remainder had no endoscopic lesion. Bleeding recurred after bowel surgery or endoscopic intervention and medical therapy for hypertrophic cardiomyopathy in 10 of 11 patients. Two patients had septal myectomy, and six patients underwent alcohol septal ablation. With the exception of one patient in whom a significant gradient persisted after septal ablation, after the periprocedural period, patients after septal reduction therapy remained free of recurrent bleeding and need for transfusions. CONCLUSION: Acquired von Willebrand syndrome is common in hypertrophic cardiomyopathy. Gastrointestinal bleeding often recurs after endoscopic therapy, but may be relieved by structural cardiac repair. SN - 1538-7836 UR - https://www.unboundmedicine.com/medline/citation/25387993/Remission_of_recurrent_gastrointestinal_bleeding_after_septal_reduction_therapy_in_patients_with_hypertrophic_obstructive_cardiomyopathy_associated_acquired_von_Willebrand_syndrome_ L2 - https://doi.org/10.1111/jth.12780 DB - PRIME DP - Unbound Medicine ER -