Yuka Matsunaga1, Masataka Ishimura2, Hazumu Nagata1, Kiyoshi Uike1, Tadamune Kinjo1, Masayuki Ochiai1, Kenichiro Yamamura1, Hidetoshi Takada3, Yoshihisa Tanoue4, Masaki Hayakawa5, Masanori Matsumoto5, Toshiro Hara6, Shouichi Ohga1. 1. Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Japan. 2. Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Japan. Electronic address: ischii@pediatr.med.kyushu-u.ac.jp. 3. Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Japan; Department of Perinatal and Pediatric Medicine, Graduate School of Medical Sciences, Kyushu University, Japan. 4. Department of Cardiovascular Surgery, Graduate School of Medical Sciences, Kyushu University, Japan. 5. Department of Blood Transfusion Medicine, Nara Medical University, Japan. 6. Fukuoka Children Hospital, Japan.
Abstract
BACKGROUND: Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by systemic or intrarenal platelet aggregation, thrombocytopenia, and red cell fragmentation. Post-operative TMA mostly occurs in adult patients with cardiovascular surgery, with the distinct pathophysiology from classical thrombotic thrombocytopenic purpura (TTP) although the exact pathophysiology remains unclear. CASE PRESENTATION: A one-month-old infant developed TMA after the initial surgery of double outlet right ventricle. ADAM metallopeptidase with thrombospondin type 1 motif 13 (ADAMTS13) activity was sustained (64%) with the undetectable inhibitor. Von Willebrand factor (VWF) multimer analyses showed absent high-molecular weight multimers. Echocardiography disclosed severe mitral regurgitation. The mitral valve repair 32 days after the initial valvuloplasty led to prompt resolution of TMA. These suggested that TMA occurred in association with valvulopathy-triggered turbulent shear flow, mechanical hemolysis and endothelial damage. The consumption of large VWF multimers might account for the vascular high shear stress shown in Heyde syndrome. CONCLUSION: The youngest case of post-operative TMA underscores the critical coagulopathy after the first surgical intervention for congenital heart disease.
BACKGROUND:Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by systemic or intrarenal platelet aggregation, thrombocytopenia, and red cell fragmentation. Post-operative TMA mostly occurs in adult patients with cardiovascular surgery, with the distinct pathophysiology from classical thrombotic thrombocytopenic purpura (TTP) although the exact pathophysiology remains unclear. CASE PRESENTATION: A one-month-old infant developed TMA after the initial surgery of double outlet right ventricle. ADAM metallopeptidase with thrombospondin type 1 motif 13 (ADAMTS13) activity was sustained (64%) with the undetectable inhibitor. Von Willebrand factor (VWF) multimer analyses showed absent high-molecular weight multimers. Echocardiography disclosed severe mitral regurgitation. The mitral valve repair 32 days after the initial valvuloplasty led to prompt resolution of TMA. These suggested that TMA occurred in association with valvulopathy-triggered turbulent shear flow, mechanical hemolysis and endothelial damage. The consumption of large VWF multimers might account for the vascular high shear stress shown in Heyde syndrome. CONCLUSION: The youngest case of post-operative TMA underscores the critical coagulopathy after the first surgical intervention for congenital heart disease.