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OPCAB treatment option for Coronary Artery Disease in Myelodisplastic Syndrome

Management of Coronary Artery Disease in Myelodisplastic Syndrome.
Articles > Cardiovascular Surgery
Submitted : 12.06.2016
Accepted : 26.07.2016
Published : 02.08.2016

Abstract

The aim of this study is evaluate the outcomes of myelodysplastic syndrome on multivessel coronary artery disease. A 72-year-old male who had been diagnosed as myelodysplastic syndrome (MDS) referred for effort angina. On coronary angiography, critical 80% stenosis of the left main coronary artery and multivessel coronary artery disease (MVCAD) determined. On complete blood count the number of platelets was about 10-20^3/µL. We administered platelet replacement twice daily to increase the number of platelets to prevent hemorrhage for a week. This case was characterized with hemodynamic instability and difficulties in MDS after off pump coronary artery bypass grafting (OPCAB) due to thrombocytopenia of varying severity. He was stabilized after increasing the number of platelets. Herein, we have published this case due to its rarity and difficulty in decision-making.

Introduction

Myelodysplastic syndrome (MDS) is a rare disorder of hematopoietic stem cells that results with ineffective hematopoiesis. Clinically, MDS is presented with anemia, thrombocytopenia and neutropenia which is an independent risk factor for heart surgery , additively is known as to increase the risk of hemorrhage and infection or anemia during cardiac surgery. We reported here a case with MDS and severe multivessel coronary artery disease with hemodynamic instability underwent OPCAB operation. In similar cases, surgeons avoid operating because of disasterios complications. Rarely patients with left main coronary artery and multivessel coronary artery disease and MDS has been reported.  Our extremely rare approach succeeded without any harmful complications in such a difficult case.

Case Report

A 72-years-old male who had been diagnosed as myelodysplastic syndrome (MDS) referred for effort angina. His physical examination was usual. Routine blood count and also biochemical measurements were normal. In PA chest radiograph, cardiothoracic ratio was increased with widened aortic knob (Figure-1). 

Figure 1
X ray image

 

Echocardiography revealed moderate aortic regurgitation and 40% ejection fraction. His pancytopenia improved gradually after reducing synthetic corticosteroids (danazol 200 mg/day). After blood and platelet transfusions the surgery was performed within appropriate hemogram values (Table1). Prophylactic antibiotics and infusion of blood products were used for pre-operative state.

Table 1
hemogram results

 

Coronary artery disease (CAD) diagnosed by coronary angiography showed right coronary artery (RCA) 90%, RCA PDA branch 80%(Figure 3A), left anterior descending  (LAD) 90%, circumflex artery (CX) 80%  proximal stenoses (Figure 3B).

Figure 3A
Coronary angiography image
Figure 3B
Coronary angiography image 2

 

Treatment of pancytopenia with transfusion of red blood cells and platelets preoperatively followed with off-pump coronary artery bypass grafting. 3-vessels OPCAB (LIMA-LAD with left internal thoracic artery, AO-RCA PDA (posterior descending branch) and AO-CX OM1(obtuse marginal branch) with saphenous vein grafts) establised. Proximal ones performed after the distal anastomoses to protect the stabilization. We preferred OPCAB in order to decrease activation of complements. A large amount of transfusion of red blood cells or platelets in intra-operative and postoperative states was not required. Major bleeding was avoided and controlled during the OPCAB. Heparin reversal established and granulocyte colony stimulating factor was not used. The amount of chest drainage was totally 400 ml and drain was taken 3 days postoperatively to avoid bleeding.

He was hemodynamically stable without inotropic support and had no any high fever in intansive care unit (ICU) period. Post-operative chest radiograph showed as usual lungs (Figure-2).

Figure 2
X ray image 2

 

In the follow-up period our patient developed any comorbidites. Patient treated with the standard anticoagulant therapy (acetyl - salicylic acid 100 mg/day and clopidogrel 75 mg/day per oral) was given to protect graft patency. Patient was discharged without any complication. 

Discussion

This rare case was important for hemodynamic instability and difficulties in MDS  after OPCAB due to thrombocytopenia. Furthermore, many surgeons avoid surgery in  cases with MDS because of disastrous complications. MDS increases the risk of hemorrhage and infection or anemia in cardiovascular surgery. We preferred surgery for critical stenosis of the left main coronary artery and MVCD. We succeeded operation by platelet transfusion twice daily. We saw pancytopenia was improved gradually by reducing the synthetic corticosteroids (danazol 200 mg/day). Prophylactic antibiotics and infusion of blood products were used peri-operative state.

Three percent  to 4% of platelet groups have been detected on blood count and microscobic examination. Acute MDS was diagnosed as “unclassified (MDS-U)” according to WHO classification of MDS. The Revised Prognostic Scoring System (IPSS-R) was calculated to be <1.5 (very low). He was interpreted as acute myofibrosis. Medical treatment with danazol was continued. Department of Hematology has concluded the case as a high risk patient for open heart surgery and suggested platelet and appropriate blood products transfusion to keep platelet level above 100000 /mm3. Daily blood counts and microscopic examinations were performed during postoperative period.

We preferred OPCAB in order to reduce activation of complements and cardiopulmonary bypass related coagulopathy. Perioperative and surgical follow-up is important in patients with MDS. Management of internal emergencies was also carried out by consultation with the internal medicine and hematology specialists. Modern surgical techniques and experiences has  reduced the importance of MDS in heart surgery. Treatment of post-OPCAB with MDS can be managed successfully with effective physician-to-physician communication. Herein, we have shared this case due to its rarity and difficulty in decision-making.

MDS presented with refractory anemia,  accompanied by various degrees of granulocytopenia and pancytopenia 1,2. Thrombocytopenia and neutropenia caused by MDS can complicate cases with major infections and bleeding during cardiac surgery. We prefer OPCAB for hemodynamically unstable patients with other organ-system problems. For cardiac surgery, MDS is an independent risk factor for postoperative mortality and morbidity. OPCAB is indicated in the following  problems for the fear of complications: severe aortic calcification, carotid artery stenosis, history of stroke, renal dysfunction, pulmonary artery diseases and other independent risk factors 3-5. Shortened length of hospital stay, decreased respiratory period and decreased pulmonary complications, less bleeding and blood transfusion, decreased inflammatory response, decreased arrhythmias have associated with OPCAB outcomes 4,5.

However, limited cases were reported about MDS with MVCD. Ganti and colleagues have demonstrated that the major myeloproliferative syndromes (MPS) [polycythemia vera (PV), essential thrombocythemia (ET), chronic myeloid leukemia (CML) and myelofibrosis (MF)], PV and ET were reported to be associated with increased thrombotic complications 6. According to this study, the relationship between these MPS’s  and coronary artery disease (CAD) is unclear. In addition, they showed that after adjusting for age, sex, dyslipidemia, diabetes, hypertension and tobacco use, the difference in the prevalence of CAD between the various categories of MPS was not significant 6. Gangwani and colleagues have described that the perioperative management was challenging because of associated anaemia, pancytopenia and immunosuppression 6. These patients also have the tendency to bleed not only due to associated thrombocytopenia but also due to the cardiopulmonary bypass related coagulopathy 7. Enblom and colleagues conclude that a large proportion of MDS patients suffer severe thromboembolic complications prior to diagnosis 8. According to this study, if MDS was diagnosed earlier, a large proportion of these events can be prevented 8. Clinicians who care for patients with MDS should be aware of upper risk of severe thromboembolic complications and cardiac disorders in this population 9,10. Thromboembolic complications and bleeding stand as the main complications in MDS.

His coronary angiography revealed critical stenosis of the left main coronary artery and severe proximal stenoses of 3 vessel disease. We presented OPCAB on triple vessels using left internal thoracic artery and saphenous vein grafts (SVG). Standard doses (1-1.5 mg/kg) of systemic heparin had been applied. The target ‘activated clotting time’ (ACT) was greater than 300-350 seconds. Left internal mammary artery (LIMA) was prepared as long as possible. The grafts were harvested with scissors and hemoclips used for bleeding. The grafts had protected inside of ‘perlingalit’ (1.0 mmol/L) mixed with heparinized blood. Also, heparinized blood was injected intraluminally into the saphenous vein grafts after harvesting. SVG was commonly used for the obtuse marginal branches and/or the posterior descending coronary artery. Heparin reversal establised. Red blood cells and platelets transfusions had used twice daily to increase the number of the granulocytes in peri-operative state. We avoided major bleeding and severe sternal infections. His follow-up was uneventful. The patient discharged with acetylsalicylate 100 mg / day and clopidogrel 75 mg / day peroral treatment. In the 1st postoperative month, functional capacity improved dramatically. New York Heart Association functional class (NYHA) IV (preoperatively) moved to II.

Hearth surgery can be performed in cases with myelodysplastic syndrome who have significant comorbidites that highlights the technical considerations to be kept in mind. OPCAB with MDS cases should be performed by experienced teams.

We think, it was an interesting case of successful management in MDS who underwent open-heart surgery. Consequently, in MDS, cardiovascular surgery with OPCAB can give simple and successful results.

References

  1. Cavolli R, Uymaz B, Memishaj S, et al. A rare case of left main coronary stenosis and neutropenia treated with G-CSF prior to CABG. Thorac Cardiovasc Surg 2010;58(8):488-9.
  2. Miyagi Y, Yamauchi S, Suzuki S, et al. Investigation of coronary artery bypass grafting for a patient with myelodysplastic syndrome. Ann Thorac Cardiovasc Surg. 2001;7(4):250-3.
  3. Ng CY, Ramli MF, Awang Y. Coronary bypass surgery in patients aged 70 years and over: mortality, morbidity, length of stay and hospital cost. Asian Cardiovasc Thorac Ann 2004;12(3): 218-23.
  4. Bakaeen FG, Chu D, Kelly RF, et al. Perioperative outcomes after on- and off-pump coronary artery bypass grafting. Tex Heart Inst J 2014;41(2):144-51.
  5. Sepehripour AH, Harling L, Ashrafian H, et al. Does off-pump coronary revascularization confer superior organ protection in re-operative coronary artery surgery? A meta-analysis of observational studies. J Cardiothorac Surg. 2014 24;9(1):115.
  6. Ganti AK, Potti A, Koka VK, et al. Myeloproliferative syndromes and the associated risk of coronary artery disease. Thromb Res. 2003;110(2-3):83-6.
  7. Gangwani AL, Hamid M, Naz F. Aortic valve replacement in a patient with pancytopenia secondary to myelodysplastic syndrome. J Pak Med Assoc. 2014;64(2):207-9.
  8. Enblom A, Lindskog E, Hasselbalch H, et al. High rate of abnormal blood values and vascular complications before diagnosis of myeloproliferative neoplasms. Eur J Intern Med. 2015;26(5):344-7.
  9. Wu J, Zhang L, Vaze A, et al. Risk of Wernicke's encephalopathy and cardiac disorders in patients with myeloproliferative neoplasm. Cancer Epidemiol. 2015;39(2):242-9.
  10. Wang Y, Zuo A, Liu Y, et al. Complications and conversions in myeloproliferative disorders: an analysis of 356 cases. Zhonghua Xue Ye Xue Za Zhi. 2002;23(6):314-7.

 

 

Keywords : Trombositopeni , Myelodisplastik sendrom , Koroner stenoz , Kardiyak hastalıklar , Myelofibrosis.

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