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Eur J Cardiothorac Surg 2004;26:1058-1059
© 2004 Elsevier Science NL

Letter to the Editor

Paroxysmal nocturnal hemoglobinuria – pre- and perioperative rationale during cardiac surgery using extracorporeal circulation

K. Knoblocha,*, A. Lichtenberga, J. Schubertb, A. Havericha

a Thoracic and Cardiovascular Surgery, Medical School Hannover, Carl-Neuberg-Str. 1, 30625 Hannover, Germany
b Hematology/Oncology, Saarland University, Homburg/Saar, Germany

Received 10 May 2004; accepted 17 August 2004.

* Corresponding author. Tel.: +49 511 532 6581; fax: +49 511 532 5404. (E-mail: kknobi{at}yahoo.com).

We read with interest the report of Naito et al. [1] presenting a successful on-pump coronary artery revascularisation in a patient with paroxysmal nocturnal hemoglobinuria (PNH), an acquired hemolytic anemia associated with thrombocytopenia and an abnormal susceptibility to venous thromboses based on an unregulated complement activation [2]. In cardiac surgery, PNH-induced granulocytopenia increases the risk of postoperative infection. PNH-induced complement activation is further exaggerated by extracorporeal circulation leading to potential hemolytic crises. In addition, resulting thrombocytopenia would lead to an increase risk of bleeding.

In a previous report [3] we could already present the feasibility of a combined aortic valve replacement and simultaneous coronary revascularisation using extracorporeal circulation in a 72-year-old male patient with renal insufficiency who developed PNH after severe aplastic anemia. Preoperative therapy included oral cyclosporin (CsA levels 100–150ng/ml) for trileanage cytopenia with anemia, neutropenia, and thrombocytopenia. Seven days before the scheduled procedure treatment with G-CSF (Neupogen® 300µg, Amgen©, subcutaneously three times a week) was started. On-pump porcine aortic valve replacement and revascularisation of the left anterior descending artery with the left internal mammary artery were performed. Postoperative complement analysis were in a normal ranges. No signs of hemolysis were detected with normal haptoglobin values over all days and no thrombosis was evident under intravenous heparin prophylaxis for 11 days with PTT ranging between 50 and 60s.

To the best of our knowledge another successful coronary revascularisation has been reported [4] in a 62-year-old-woman with triple vessel coronary artery disease and PNH with severe intravascular haemolytic anemia, but no neutropenia or thrombocytopenia. Preoperative management included correction of anemia (in the five months prior to surgery), pulses of corticosteroids during haemolytic flareups, and low dose aspirin. Cardiopulmonary bypassing using a standard heparin protocol with protamine reversal was used followed by enoxaparin until discharge and aspirin. Other supportive measures included folate supplementation, hydrocortisone and prophylactic cefazolin.

Cardiac surgery in PNH-patients is associated with several possible complications:

(1) PNH-induced granulocytopenia increases the risk of postoperative infection. Therefore, the prophylactic use of antibiotics appears to be mandatory. Furthermore, the use of G-CSF (NeupogenTM 300µg, Amgen©, subcutaneously) to stimulate neutrophil counts is an option in this setting.
(2) The aggravation of hemolysis by extracorporeal circulation in cardiac surgery due to complement activation from either contact of blood with the foreign material surfaces during cardiopulmonary bypass circuit [5]. Therefore, such a treatment in patients with PNH is expected to result in consecutive hemolytic crisis. We mainly prevented intraoperative hemolytic crisis by preoperative transfusion up to a normal hemoglobin level in order to decrease GPI-deficient red blood cells. As a result, over the postoperative course we even observed a normal haptoglobin level indicating that hemolysis was almost absent due to the preoperative treatment.
(3) The risk of acute renal failure after cardiac surgery due to hemolysis in PNH is further increased by preexisting renal insufficiency. In this clinical setting, adequate fluid administration and the use of diuretics, in this case mannitol pre- and perioperatively, appears to be essential.
(4) Thrombocytopenia in PNH and the use of extracorporeal circulation may lead to an increased risk of bleeding. Furthermore, substitution of platelets perioperatively supposedly reduced bleeding complications.

References

  1. Naito Y, Nakajima M, Inoue H, Tsuchiya K. Successful CABG in a patient with paroxysmal nocturnal hemoglobinuria. Eur J Cardiothorac Surg 2004;25:468-470.[Abstract/Free Full Text]
  2. Hillmen P, Lewis SM, Bessler M, Luzzatto L, Dacie JV. Natural history of paroxysmal nocturnal hemoglobinuria. N Engl J Med 1995;333:1253-1258.[Abstract/Free Full Text]
  3. Knobloch K, Zardo P, Gohrbandt B, Fischer S, Leyh RG, Tiede A, Ganser A, Schubert J. Cardiac surgery in a patient with paroxysmal nocturnal hemoglobinuria. Haematologica 2002;87(8):ECR29.[Medline]
  4. Lopez PG, Garg A, Cote S. Cardiac surgery and paroxysmal nocturnal hemoglobinuria. Haematologica 2003;88(6):ELT19.[Free Full Text]
  5. Kirklin JK, Westaby S, Blackstone EH, Kirklin JW, Chenoweth DE, Pacifico AD. Complement and the damaging effects of cardiopulmonary bypass. J Thorac Cardiovasc Surg 1983;86:845-857.[Abstract]




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