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Original research
Prognostic value of von Willebrand factor in adult patients with congenital heart disease
  1. Hideo Ohuchi1,
  2. Yohsuke Hayama1,
  3. Hikari Miike1,
  4. Dai Suzuki1,
  5. Kimiko Nakajima1,
  6. Toru Iwasa1,
  7. Nao Konagai1,
  8. Heima Sakaguchi1,
  9. Aya Miyazaki2,
  10. Isao Shiraishi1,
  11. Ken-ichi Kurosaki1,
  12. Michikazu Nakai3
  1. 1 Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan
  2. 2 Department of Congenital Heart Disease, Division of Transitional Medicine, Shizuoka General Hospital, Shizuoka, Shizuoka, Japan
  3. 3 Preventive Medicine and Epidemiologic Informatics, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan
  1. Correspondence to Dr Hideo Ohuchi, Pediatric Cardiology, National Cerebral and Cardiovascular Center, Suita 564-8565, Japan; hohuchi{at}


Objectives von Willebrand factor (vWF) has prognostic value in patients with heart failure (HF) and in those with liver disease. Liver congestion, due to right-sided HF (RHF), is one of the major clinical pathophysiologic manifestations in adults with congenital heart disease (ACHD). The present study’s purpose was to clarify the prognostic value of plasma levels of vWF antigen (vWF:Ag) in ACHD.

Methods We measured vWF:Ag (%) in 382 consecutive patients (20 unrepaired cyanotic ACHD, 172 Fontan patients and 190 ACHD after biventricular repair) and compared the results with the clinical profiles and prognosis.

Results The plasma vWF:Ag level was 130±53 (normal range: 55%–190%), and 48 patients (13%) showed high levels of vWF:Ag (≥190%). Older age, Fontan circulation, higher central venous pressure, lower arterial oxygen saturation and lower plasma levels of albumin were independently associated with high log (vWF:Ag) (p<0.05–0.0001). During the follow-up of 2.4±1.4 years, 15 patients died. High log (vWF:Ag) predicted the all-cause mortality (HR 1.63 per 0.1, 95% CI 1.40 to 1.96, p<0.0001). Specifically, patients with high vWF:Ag (≥165%) had a substantially higher risk of all-cause mortality (HR 56.4, 95% CI 11.4 to 1020, p<0.0001), and this prognostic value was independent of plasma levels of brain-type natriuretic peptide.

Conclusions High vWF:Ag may reflect RHF severity and related liver dysfunction with a strong prognostic value of all-cause mortality in ACHD. Thus, vWF:Ag might be an excellent biomarker for monitoring ACHD with RHF.

  • complex congenital heart disease

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  • Contributors HO: PI/drafting article/data analysis/interpretation. YH: data collection/data analysis/interpretation. HM, DS, KN, TI, NK, HS and AM: data collection/interpretation. IS and KK: interpretation. MN: statistic analysis/Interpretation.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement All data relevant to this study are presented in the article.

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