Cyanotic congenital heart disease (CCHD): Focus on hypoxemia, secondary erythrocytosis, and coagulation alterations

Paediatric Anaesthesia

Volumn 25, Issue 10, 2015, Pages 981-989

  Zabala, Luis M ^a   Guzzetta, Nina A ^b  

^a Department of Anesthesiology   (United States)

^b EMORY UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

antifibrinolytic; blood transfusion; coagulation; congenital heart disease; heparin; neonate

Indexed keywords

AMINOCAPROIC ACID; APROTININ; HEPARIN; PROTAMINE; TRANEXAMIC ACID;

ARTICLE; BLOOD CLOTTING DISORDER; BLOOD CLOTTING TIME; BRAIN BLOOD FLOW; CARDIOPULMONARY BYPASS; CEREBROVASCULAR ACCIDENT; CYANOTIC HEART DISEASE; DISSEMINATED INTRAVASCULAR CLOTTING; ERYTHROCYTE; ERYTHROCYTOSIS; HEMATOCRIT; HEMOSTASIS; HUMAN; HYPOXEMIA; LEUKOCYTOSIS; OXYGEN SATURATION; PARTIAL THROMBOPLASTIN TIME; PHLEBOTOMY; POLYCYTHEMIA; POSTOPERATIVE HEMORRHAGE; PRIORITY JOURNAL; PROTHROMBIN TIME; RISK ASSESSMENT; SECONDARY ERYTHROCYTOSIS; SYSTEMIC VASCULAR RESISTANCE; THROMBOCYTOPENIA; THROMBOCYTOSIS; THROMBOELASTOGRAPHY; ANOXIA; BLOOD CLOTTING TEST; CHILD; COMPLICATION; CONGENITAL HEART MALFORMATION; CYANOSIS; PATHOPHYSIOLOGY;

ANOXIA; BLOOD COAGULATION DISORDERS; BLOOD COAGULATION TESTS; CHILD; CYANOSIS; HEART DEFECTS, CONGENITAL; HUMANS; POLYCYTHEMIA;

EID: 84941167558     PISSN: 11555645     EISSN: 14609592     Source Type: Journal    
DOI: 10.1111/pan.12705     Document Type: Article

References (57)

1. Oechslin E,. Hematological management of the cyanotic adult with congenital heart disease. Int J Cardiol 2004; 97: 109-115.
2. Perloff JK, Rosove MH, Sietsema KE, et al,. Cyanotic congenital heart disease: a multisystem disorder. In:, Perloff JK, Child JS, eds. Congenital Heart Disease in Adults. Philadelphia: W.B Saunders Company, 1998: 199-226.
3. Rosove MH, Perloff JK, Hocking WG, et al,. Chronic hypoxaemia and decompensated erythrocytosis in cyanotic congenital heart disease. Lancet 1986; 2: 313-315.
4. Perloff JK, Rosove MH, Child JS, et al,. Adults with cyanotic congenital heart disease: hematologic management. Ann Intern Med 1988; 109: 406-413.
5. Tyndall MR, Teitel DF, Lutin WA, et al,. Serum erythropoietin levels in patients with congenital heart disease. J Pediatr 1987; 110: 538-544.
6. Gaiha M, Sethi HP, Sudha R, et al,. A clinicohematological study with hyperviscosity symptoms in cyanotic congenital heart disease. Ind Heart J 1993; 45: 53-55.
7. Broberg C, Jayaweera AR, Diller GP, et al,. Seeking optimal relation between oxygen saturation and hemoglobin concentration in adults with cyanosis from congenital heart disease. Am J Cardiol 2011; 107: 595-599.
8. Grotta J, Ackerman R, Correia J, et al,. Whole blood viscosity parameters and cerebral blood flow. Storke 1982; 13: 296-301.
9. Phornphutkul C, Rosenthal A, Nadas AS, et al,. Cerebrovascular accidents in infants and children with cyanotic congenital heart disease. Am J Cardiol 1973; 32: 329-334.
10. Thomas DJ, du Boulay GH, Marshall J, et al,. Cerebral blood flow in polycythemia. Lancent 1977; 2: 161-163.
11. Ammash N, Warnes CA,. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J Am Coll Cardiol 1996; 28: 768-772.
12. Thorne SA,. Management of polycythemia in adults with cyanotic congenital heart disease. Heart 1998; 78: 315-316.
13. Nadeem O, Gui J, Ornstein DL,. Prevalence of venous thromboembolisms in patients with secondary polycythemia. Clin Appl Thromb Hemost 2013; 19: 363-366.
14. Perloff JK, Marelli AJ, Miner PD,. Risk of stroke in adults with cyanotic congenital heart disease. Circulation 1993; 87: 1954-1959.
15. Rudolph AM, Nadas AA, Borges WH,. Hematologic adjustments to cyanotic congenital heart disease. Pediatrics 1953; 11: 454-465.
16. Bahnson HT, Ziegler RF,. A consideration of the cause of death following operation for congenital heart disease of the cyanotic type. Surg Gynecol Obstet 1950; 90: 60-76.
17. Maurer HM, McCue CM, Robertson JC, et al,. Correction of platelet dysfunction and bleeding in cyanotic congenital heart disease by simple red cell volume reduction. Am J Cardiol 1975; 35: 831-835.
18. Niwa K, Perloff JK, Kaplan S, et al,. Eisenmenger syndrome in adults. Ventricular septal defect, truncus arteriosus, and univentricular heart. J Am Coll Cardiol 1999; 34: 223-232.
19. Territo MC, Perloff JK, Rosove MH, et al,. Acquired von Willebrand factor abnormalities in adult with congenital heart disease. Clin Appl Thromb Hemost 1998; 4: 257-261.
20. Peters AM, Rozkocev A, Bell RN, et al,. Platelet kinetics in congenital heart disease. Cardiovasc Res 1982; 16: 391-397.
21. Goldschmidt B, Sarkadi B, Gardos G, et al,. Platelet production and survival in cyanotic congenital heart disease. Scand J Haematol 1974; 13: 110-115.
22. 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: 1072-1077.
23. Lill MC, Perloff JK, Child JS,. Pathogenesis of thrombocytopenia in cyanotic congenital heart disease. Am J Cardiol 2006; 98: 254-258.
24. Cui Y, Hei F, Long C, et al,. Perioperative monitoring of thromboelastograph on hemostasis and therapy for cyanotic infants undergoing complex cardiac surgery. Artif Organs 2009; 33: 909-914.
25. Jensen AS, Johansson PI, Bochsen L, et al,. Fibrinogen function is impaired in whole blood from patients with cyanotic congenital heart disease. Int J Cardiol 2013; 167: 2210-2214.
26. Goel M, Shome DK, Sing ZN, et al,. Haemostatic changes in children with cyanotic and acyanotic congenital heart disease. Indian Heart J 2000; 5: 559-563.
27. Wedemeyer AL, Edson JR, Krivit W,. Coagulation in cyanotic congenital heart disease. Am J Dis Child 1972; 124: 656-660.
28. Johnson CA, Abildgaard CF, Schulman I,. Absence of coagulation abnormalities in children with cyanotic congenital heart disease. Lancet 1968; 2: 660-662.
29. Heriksson P, Varendh G, Lundstrom NR,. Haemostatic defects in cyanotic congenital heart disease. Br Heart J 1979; 41: 23-27.
30. Levin E, Wu J, Devine DV, et al,. Hemostatic parameters and platelet activation marker expression in cyanotic and acyanotic padiatric patients undergoing cardiac surgery in the presence of tranexamic acid. Thromb Haemost 2000; 83: 54-59.
31. Naiman JL,. Clotting and bleeding in cyanotic congenital heart disease. J Pediatr 1970; 76: 333-335.
32. Oechslin E,. Eisenmenger's syndrome. In:, Gatzoulis MA, Webb GD, Daubeney PEF, eds. Diagnosis and Management of Adult Congenital Heart Disease. London: Churchill Livingstone, 2003: 363-377.
33. Rosenthal A, Nathan DG, Marty A, et al,. Acute hemodynamic effects of red cell volume reduction in polycythemia of cyanotic congenital heart disease. Circulation 1970; 42: 297-307.
34. Humphrey PR, Michael J, Pearson TC,. Management of relative polycythemia: studies of cerebral blood flow and viscosity. Br J Haematol 1980; 46: 427-433.
35. Tempe DK, Virmani S,. Coagulation abnormalities in patients with cyanotic congenital heart disease. J Cardiothorac Vasc Anesth 2002; 16: 752-765.
36. Takahashi Y, Tatsuno K, Kikuchi T,. Open heart surgery with bloodless priming for cyanotic congenital heart disease: the role of autologous blood donation after induction of anesthesia. Nippon Kyobu Geka Gakkai Zasshi 1996; 44: 1075-1081.
37. Takahashi Y, Tatsuno K, Kikuchi T, et al,. Open heart surgery without blood transfusion for complete atrioventricular septal defect associated with tetralogy of Fallot. Nippon Kyobu Geka Gakkai Zasshi 1997; 45: 170-174.
38. Gruenwald CE, Manlhiot C, Chan AK, et al,. Randomized controlled trial of individualized heparin and protamine management in infants undergoing cardiac surgery with cardiopulmonary bypass. J Am Coll Cardiol 2010; 56: 1794-1802.
39. Maiviya S,. Monitoring and management of anticoagulation in children requiring extracorporeal circulation. Semin Thromb Hemost 1997; 223: 563-567.
40. D'Errico C, Shayevitz JR, Martindale SJ,. Age related differences in heparin sensitivity and heparin-protamine interactions in cardiac surgical patients. J Cardiothorac Vasc Anesth 1996; 10: 451-457.
41. Saini A, Spinella PC,. Management of anticoagulation and hemostasis for pediatric extracorporeal membrane oxygenation. Clin Lab Med 2014; 34: 655-673.
42. Dietrich W, Braun S, Spannagle M, et al,. Low preoperative antithrombin activity causes reduced response to heparin in adult but not in infant cardiac-surgical patients. Anesth Analg 2001; 92: 66-71.
43. Guzzetta NA, Miller BE, Todd K, et al,. An evaluation of the effects of a standard heparin dose on thrombin inhibition during cardiopulmaonary bypass in neonates. Anesth Analg 2005; 100: 1276-1282.
44. Gautam NK, Schmitz ML, Harrison D, et al,. Impact of protamine dose on activated clotting time and thromboelastography in infants and small children undergoing cardiopulmonary bypass. Paediatr Anaesth 2013; 23: 233-241.
45. Paramo JA, Rifon J, Llorens R, et al,. Intra and postoperative fibrinolysis in patients undergoing cardiopulmonary bypass surgery. Haemostasis 1991; 21: 58-64.
46. Ray MJ, Marsh NA, Hawson GA,. Relationship of fibrinolysis and platelet function to bleeding after cardiopulmonary bypass. Blood Coagul Fibrinolysis 1994; 5: 679-685.
47. Ortmann E, Besser MV, Klein AA,. Antifibrinolytic agent in current anaesthetic practice. Br J Anaesth 2013; 111: 549-563.
48. Fergusson DA, Hebert PC, Mazer CD, et al,. A comparison of aprotinin and lysine analogues in high-risk cardiac surgery. N Engl J Med 2008; 358: 2319-2331.
49. Health-Canada. Health Canada Decision on Trasylol (aprotinin). 2011
50. European-Medicines-Agency. Questions and answers on the review of antifibrinolytic medicines (aprotinin, aminocaproic acid and tranexamic acid). 2013
51. Eaton MP,. Antifibrinolytic therapy in surgery for congenital heart disease. Anesth Analg 2008; 106: 1087-1100.
52. Pasquali SK, Li JS, He X, et al,. Comparative analysis antifibrinolytic medications in pediatric heart surgery. J Thorac Cardiovasc Surg 2012; 143: 550-557.
53. Martin K, Breuer T, Gartler R, et al,. Tranexamic acid vs epsilon aminocaproic acid: efficacy and safety in pediatric cardiac surgery. Eur J Cardiothorac Surg 2011; 39: 892-897.
54. Chan KL, Summerhayes RG, Ignjatovic V, et al,. Reference values for kaolin-activated thromboelastography in healthy children. Anesth Analg 2007; 105: 1610-1613.
55. Haizinger B, Gombotz H, Rehak P, et al,. Activated thromboelastogram in neonates and infants with complex congenital heart disease in comparison with healthy children. Br J Anaesth 2006; 97: 545-552.
56. Miller BE, Guzzetta NA, Tosone SR, et al,. Rapid evaluation of coagulopathies after cardiopulmonary bypass in children using modified thromboelastography. Anesth Analg 2000; 90: 1324-1330.
57. Cui Y, Hei F, Long C, et al,. Perioperative monitoring of thromboelastograph on blood protection and recovery for severely cyanotic patient undergoing complex cardiac surgery. Artif Organs 2010; 34: 955-960.