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Thrombotic microangiopathy in a very young infant with mitral valvuloplasty

Yuka Matsunaga1, Masataka Ishimura1, Hazumu Nagata1

  • 1Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Japan.

Pediatrics and Neonatology
|March 11, 2018
PubMed
Abstract

Insights

Post-operative thrombotic microangiopathy (TMA) in an infant with congenital heart disease resolved after mitral valve repair. This case highlights TMA

Area of Science:

  • Cardiovascular Surgery
  • Pediatric Cardiology
  • Hematology

Background:

  • Thrombotic microangiopathies (TMA) are vascular disorders characterized by platelet aggregation, thrombocytopenia, and red cell fragmentation.
  • Post-operative TMA typically affects adults after cardiovascular surgery, with unclear pathophysiology distinct from TTP.
  • Congenital heart disease surgery presents unique challenges for TMA development in infants.

Observation:

  • A one-month-old infant developed TMA following double outlet right ventricle surgery.
  • ADAMTS13 activity was normal, but VWF multimer analysis showed absent high-molecular-weight multimers.
  • Severe mitral regurgitation was noted, suggesting a link to valvulopathy.

Findings:

  • TMA in this infant was associated with valvulopathy-induced turbulent shear flow, mechanical hemolysis, and endothelial damage.
  • Consumption of large VWF multimers likely contributed to vascular shear stress, similar to Heyde syndrome.
  • Mitral valve repair led to prompt resolution of TMA, confirming the association.

Implications:

  • This is the youngest reported case of post-operative TMA.
  • It underscores the critical coagulopathy risks following initial surgical interventions for congenital heart disease.
  • Early recognition and intervention for TMA in infants undergoing cardiac surgery are crucial.
Keywords:
cardiac surgerythrombotic microangiopathyvon Willebrand factor multimers

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