44 Fig. 1 Fluoroscopy showing that A) both leaflets functioned well after the first atrioventricular valve replacement; B) one leaflet was fixed at th

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1 症例報告 PEDIATRIC CARDIOLOGY and CARDIAC SURGERY VOL. 27 NO. 1 (43 48) 房室弁置換術後の血栓弁に対し recombinant tissue plasminogen activator による血栓溶解療法を施行した単心室幼児例 Key words: prosthetic valve thrombosis, thrombolytic therapy, tissue plasminogen activator, single ventricle, atrioventricular valve replacement Thrombolytic Therapy with Recombinant Tissue Plasminogen Activator for Prosthetic Valve Thrombosis in a Two-year-old Boy with a Single Ventricle after Atrioventricular Valve Replacement Kazushi Yasuda, 1) Shiro Ishikawa, 1) Yuichi Ishikawa, 1) Makoto Nakamura, 1) Hiroya Ushinohama, 1) Koichi Sagawa, 1) Eiji Morihana, 2) Naoki Fusazaki, 2) Kazuhiro Hinokiyama, 3) Toshihide Nakano, 3) and Hideaki Kado 3) Departments of 1) Pediatric Cardiology, 2) Neonatal Cardiology, 3) Cardiovascular Surgery, Fukuoka Children s Hospital, Fukuoka, Japan We report on thrombolytic therapy with a recombinant tissue plasminogen activator (rt-pa) for prosthetic valve thrombosis in a two-year-old boy with a single ventricle after an atrioventricular valve replacement (SJM Regent, 23 mm). An increase in urine volume and improvements of symptoms and signs of congestive heart failure were observed about 30 minutes after an intravenous injection of rt-pa (alteplase 40,000 I.U./kg of body weight). Fluoroscopy showed that an immobile leaflet remained fixed in the closed position before and after the therapy, while the other leaflet, which was hypomobile, improved to the normal range. Only slight nasal bleeding was observed as an adverse effect. After some improvement of congestive heart failure, he underwent atrioventricular valve replacement again, this time using an On-X mitral 23-mm valve. We conclude that thrombolytic therapy with rt-pa for the prosthetic valve thrombosis in this patient with a deteriorated hemodynamic condition was effective as a bridge therapy toward the valve re-replacement. 要旨 2 SJM Regent 23 mm recombinant tissue plasminogen activator rt-pa rt-pa 40 /kg 30 X On-X mitral 23 mm rt-pa bridge therapy

2 44 Fig. 1 Fluoroscopy showing that A) both leaflets functioned well after the first atrioventricular valve replacement; B) one leaflet was fixed at the closed position, and the other leaflet was hypomobile due to valve thrombosis; and C) after thrombolytic therapy, the hypomobile leaflet was normalized, but the immobile leaflet remained fixed. A B C はじめに Fontan recombinant tissue plasminogen activator rt-pa 症例提示 g Darling Ib 2 I 57 BT 3.5 mm L/min/m L/min/m mmhg 3.8 Wood 319 EF mmhg II III II IV 1 6 SJM Regent 23 mm PT-INR 2.51 FDP 1.5 μg/ml d-dimer 0.8 μg/ml 2.0 m/s X Fig. 1A PT-INR

3 m/s 2 PT-INR cm 10.4 kg 2 Levine III/VI 3 cm WBC 8030/μl RBC 574 /μl Hb 10.7 g/dl Ht 36.6 Plt 48.5 /μl PT-INR 5.35 Fib 277 mg/dl AT3 61 FDP 0.7 μg/ml d-dimer 0.5 μg/ml TP 6.7 g/dl Alb 4.2 g/dl BUN 54 mg/dl Cre 0.6 mg/dl UA 15.2 mg/dl Na 133 meq/l K 6.2 meq/l Cl 101 meq/l AST 38 U/l ALT 13 U/l LD 836 U/l γgtp 19 U/l CK 76 U/l TB 0.7 mg/dl CRP 0.54 mg/dl ANP 1220 pg/ml BNP 1460 pg/ml 4 -T1 -T3 6 cm 11.5 kg +1.1 kg m/s X immobile leaflet hypomobile leaflet Fig. 1B PT-INR EF=50 5 rt-pa mg /kg /kg/day m/s mg /kg 1 Fig cm X hypomobile leaflet immobile leaflet Fig. 1C 6 ANP BNP pg/ml Fig On-X mitral 23 mm

4 46 Fig. 2 Time course of thrombolytic therapy and findings of Doppler echocardiography and fluoroscopy. Doppler echocardiography revealed that the peak velocity (Vp) across the prosthetic valve into the ventricle decelerated after thrombolytic therapy. Fluoroscopy before thrombolytic therapy (F * ) showed that one leaflet was hypomobile and the other leaflet was immobile at the closed position. After the therapy (F ** ), the motion of the hypomobile leaflet improved to normal, but the other was still immobile. Fig m/s ANP BNP pg/ml Fig. 3 考 察 NYHA class NYHA class NYHA class III IV Fig. 3 Changes in plasma levels of atrial natriuretic peptide (ANP) and type B natriuretic peptide (BNP). rt-pa 15 16) t-pa t-pa t-pa t-pa

5 47 Fig. 4 A white thrombus was macroscopically observed around the hinge of one leaflet (arrows), and the diagnosis of a white thrombus was confirmed microscopically. 17 rt-pa rt-pa mg/kg/h mg/kg/h American Society of Hematology rt-pa mg/kg/10 min 5 10 unit/kg/h mg/kg/24 h Montorsi 19 X hypomobile immobile rt-pa hypomobile immobile 21 hypomobile leaflet 8 7 immobile leaflet 21 immobile hypomobile NYHA class IV 2 immobile leaflet hypomobile leaflet bridge therapy 2 Montorsi immobile leaflet hypomobile leaflet

6 48 1 leaflet rt-pa 2 rt-pa 3 4 結語 rt-pa hypomobile leaflet bridge therapy 参考文献 1 Kontos GJ Jr, Schaff HV, Orszulak TA, et al: Thrombotic obstruction of disc valves: clinical recognition and surgical management. Ann Thorac Surg 1989; 48: Roudaut R, Labbe T, Lorient-Roudaut MF, et al: Mechanical cardiac valve thrombosis. Is fibrinolysis justified? Circulation 1992; 86: II8 II15 3 Bortolotti U, Milano A, Mossuto E, et al: Early and late outcome after reoperation for prosthetic valve dysfunction: analysis of 549 patients during a 26-year period. J Heart Valve Dis 1994; 3: Rizzoli G, Guglielmi C, Toscano G, et al: Reoperations for acute prosthetic thrombosis and pannus: an assessment of rates, relationship and risk. Eur J Cardiothorac Surg 1999; 16: Silber H, Khan SS, Matloff JM, et al: The St. Jude valve. Thrombolysis as the first line of therapy for cardiac valve thrombosis. Circulation 1993; 87: St. Jude Medical 1995; 24: ; 28: Lengyel M: Thrombolysis should be regarded as first-line therapy for prosthetic valve thrombosis in the absence of contraindications. J Am Coll Cardiol 2005; 45: 325; author reply Das M, Twomey D, Al Khaddour A, et al: Is thrombolysis or surgery the best option for acute prosthetic valve thrombosis? Interact Cardiovasc Thorac Surg 2007; 6: ; 20: Horigome H, Hiramatsu Y, Shigeta O, et al: Overproduction of platelet microparticles in cyanotic congenital heart disease with polycythemia. J Am Coll Cardiol 2002; 39: Kajimoto H, Nakazawa M, Murasaki K, et al: Increased thrombogenesity in patients with cyanotic congenital heart disease. Circ J 2007; 71: Mahle WT, Gaynor JW, Spray TL: Atrioventricular valve replacement in patients with a single ventricle. Ann Thorac Surg 2001; 72: Lubiszewska B, Rozanski J, Szufladowicz M, et al: Mechanical valve replacement in congenital heart disease in children. J Heart Valve Dis 1999; 8: Albisetti M: Thrombolytic therapy in children. Thromb Res 2006; 118: Raffini L: Thrombolysis for intravascular thrombosis in neonates and children. Curr Opin Pediatr 2009; 21: Schneppenheim R, Greiner J: Thrombosis in infants and children. Hematology Am Soc Hematol Educ Program 2006; Montorsi P, Cavoretto D, Alimento M, et al: Prosthetic mitral valve thrombosis: can fluoroscopy predict the efficacy of thrombolytic treatment? Circulation 2003; 108: II79 II

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