Akgül, Ebru

Aylı, Meltem

Yörükoğlu, Yavuz

Anadolu Kardiyoloji Dergisi/The Anatolian Journal of Cardiology

English

Ufuk Universitesi Akademik Acikerisim Sistemi

IntroductionEssential thrombocytemia (ET) is a rare myeloproliferative diseasecharacterized by a persistent increase in platelet numbers and platelet dysfunction. The disease manifests clinically by hemorrhage and/or thromboembolic complications. Coronary artery involvement is moreprevalent than previously thought but there are only 9 cases of coronarybypass published in the literature. We present a case of essentialthrombocytemia presenting with an acute coronary syndrome treated surgically employing the off-pump beating heart surgery.Case reportA 61-year-old man with an established diagnosis of ET was referred toour hospital for rapidly progressing episodes of chest pain. On admission,he was in a stable hemodynamic condition. Laboratory investigationrevealed a hemoglobin of 8.9 g/dl, Hct - 30% and a platelet count of 930 x 109 /L.Electrocardiogram (ECG), creatine kinase-myocardial band isoenzyme andtroponin levels were normal. Inherited thrombophilia tests (Protein C,Protein S, antithrombin III deficiency, Factor V Leiden, prothrombin 20210 A,MTHFR mutations, ant›iphospholipid antibodies) were normal. Following aninitial medical treatment with nitrates, beta-blockers, angiotensin converting enzyme inhibitors, aspirin, statins, sedatives and continuinganti-platelet therapy with Anagrelide (ThromboreductinTM - AOP OrphanPharmaceuticals, Austria) a coronary angiogram was performed, revealingan irregular 90% stenotic lesion of the proximal left anterior descending(LAD) and first diagonal coronary arteries. The other coronary arterieswere disease-free. A coincidental finding during the angiogram was segmental total occlusion of the right external iliac artery with extensivecollateral vessel development filling the femoral arteries. The coronarylesion was considered unsuitable for an angioplasty and a decision for surgical therapy was made. The platelet count was found to be high (851 x 109 /L) despite therapy so the patient was maintained on IV heparinand nitroglycerin infusions to relieve the unstable angina while anincreased dose of anti-platelet therapy with Anagrelide (2 mg/day) wasadministered until the platelet count was reduced to 326 x 109 /L.At this point; hemostasis parameters and plasma Von Willebrand factor (VWF) levels were normal but epinephrine-induced secondaryplatelet aggregation was inhibited.In view of the deleterious effects of cardiopulmonary bypass (CPB) andthe anemia and history of gastrointestinal bleeding of the patient we choseto perform the operation with the off-pump, beating heart method. A singleinternal mammary artery graft (IMA) was used to bypass the lesion of theLAD coronary artery. The quality of the IMA graft, LAD and anastomosiswas excellent and the operation was completed without complications.The patient was transferred to the intensive care unit in a stable conditionrequiring no inotropic support.During the operation the patient was anticoagulated with heparin(5mg/kg) and the activated clotting time (ACT) was maintained at 350 secduring occlusion of the LAD and IMA. After completion of the anastomosisthe heparinization was partially reversed with protamine sulphate and theoperation was completed with the ACT at 150 sec.The early postoperative course was uneventful. All hemodynamic parameters were stable requiring no inotropic support. The ECG was normaland the first postoperative hemogram revealed a Hb of 9.1 g/dl, Hct of 32% anda platelet count of 262 x 109/L. Chest tube drainage was 300 cc in the first twohours with a slight increase of 200 cc in the third hour. The ACT at this time was126 seconds, the platelet count was 232 x 109/L and hemostasis parameterswere normal. Chest tube drainage loss was replaced with packed red cells.On the fifth postoperative hour, the patient was hemodynamically stable, awake and being weaned off the ventilator when he suddenly wentinto ventricular fibrillation which was reverted to normal rhythm and hemodynamic state with a brief period of cardiac resuscitation. A few minutes later a second cardiac arrest accompanied by a long period ofgeneralized convulsions occurred and the patient expired despite allefforts.Discussion Essential thrombocytemia is a rare, clonal chronic myeloproliferativedisease of unknown origin characterized by a persistent increase inplatelet numbers (>600x109/L) and platelet dysfunction. Originally describedas a hemorrhagic disorder, recent studies have shown it is manifested withthrombotic and thromboembolic complications. The incidence of thromboticand hemorrhagic complications and the overall rates of thrombosis andhemorrhage at diagnosis range from 9% to 84% and from 3.9% to 63%,respectively. Age, a previous thrombotic event and long duration of thrombocytosis were identified as major risk factors for thrombosis in thecontrolled series. Recent studies have suggested that vascular complica-tions can also be predicted from the biological characteristics of the disease, markers of hypercoagulability (thrombophilia, Factor V Leiden,antiphospholipid antibodies) and general cardiovascular risk factors(hypertension, hypercholesterolemia, smoking). Paradoxically, a highplatelet count (≥1500x109/L) is a major predictor of bleeding rather thanthrombotic complications. Normalization of the platelet count is accompa-nied by restoration of a normal plasma VWF multimeric distribution .Patients with a high risk of thromboembolic complications or bleeding arecandidates for interventions to lower platelet counts. Several agents are nowavailable for initial treatment of ET to prevent these complications. Anagrelideand Hydroxyurea are effective in reducing high platelet counts in ET.Platelets have a well-defined critical role in coronary thrombosis. Theincidence of coronary artery disease in ET has been reported as 9.4 % inpatients 40 years and older with a high incidence of acute myocardialinfarction (AMI) (1). In addition, many patients with ET initially present withsymptoms related to small or large vessel thrombosis.Experience in coronary revascularization in patients with ET is very limited. Our search of the medical literature revealed only nine cases of ETtreated surgically for acute coronary syndromes (2-10). All cases were operated utilizing cardiopulmonary bypass. Six of thestudies report early successful outcomes (2-4, 6-8). The long-term results ofsurgery however are not available. One patient suffered an AMI and cerebral infarct in the early postoperative period (5) One patient died in the postoperative sixth month, again due to an AMIand cerebrovascular accident (CVA) (8). Another case, a 26-year-old previously healthy man suffered an AMI and despite coronary arteryAnadolu Kardiyol Derg2007; 7: 436-48Olgu Sunumlar›Case Reports444Coronary bypass surgery in essential thrombocytemia Esansiyel trombositemide koroner baypas cerrahisiYavuz Yörüko¤lu, Meltem Ayl›*, Ebru Akgül**From Departments of Cardiovascular Surgery, *Hematology and **Cardiology, Medical School, Ufuk University, Ankara, TurkeyAddress for Correspondence/Yaz›flma Adresi: Yavuz Yörüko¤lu, MD, Ufuk Üniversitesi T›p Fakültesi, Mevlana Bulvar› No. 86-88 06520 Balgat, Ankara, TurkeyPhone: +90 312 204 41 57 E-mail: yyorukoglu@ufuk.edu.trbypass grafting (CABG) required left ventricular assist device (LVAD)implantation due to deterioration of hemodynamic state. The patient waslater transported to specialized center with LVAD support and eventuallyunderwent successful heart transplantation (10). Three patients presentedclinically with AMI (2, 9, 10) and two others had history or evidence ofperipheral artery thrombosis; one of the common iliac artery and the otherof the carotid artery (4, 7). Surprisingly none of the cases have encounteredany problems regarding hemorrhage postoperatively. One would expectthat the deleterious effects of CPB would aggravate the inherent tendencies of bleeding in ET. Only one of these patients required bloodtransfusions for hematoma of the groin after a coronary angiogram (7).The limited surgical experience with ET demonstrates that this entitycarries a high rate of morbidity and mortality mainly due to thromboemboliccomplications despite the meticulous efforts to monitor and manipulateplatelet numbers and function.Reports of successful surgery stress the importance of a combinationtherapy with cytoreductive agents and antiaggregants to prevent thromboembolic events (6) There is, however a delicate balance of hemorrhage versus thromboembolism to be considered in the case of surgical interventions. Platelet functions are very unpredictable in ET causing hemorrhage and thrombosis in the same patient during the sametime frame. Cardiopulmonary bypass causes severe reductions in plateletnumbers and function (aggregation) alongside depletion of coagulationfactors rendering the patient prone to postoperative bleeding. Theoreticallythe combination of CPB and an ET patient with sub-normal platelet functions is a potential hematological disaster. Keeping this in mind, wedecided to utilize the off-pump beating heart method for revascularization.It is clear in this case that in an attempt to avoid the possible hemorrhagiccomplications of ET and CPB we have run straight into another devastating(thrombotic) complication. Despite our efforts to normalize platelet numbers preoperatively with Anagrelide and maintain an acceptable levelof anticoagulation perioperatively the patient suffered a massive thromboembolic episode. Although an autopsy was not available we pos-tulate that this thromboembolic episode must have happened almostinstantaneously because at no stage were there any indications of a problem, such as deteriorating hemodynamics, change in ECG or consciousness.The lack of response to all resuscitation efforts and the development ofcardiac standstill in a matter of minutes alongside convulsions during car-diac arrest suggests a massive thrombosis of not only the bypass vessel but probably all the coronary arteries and major cerebral vessels.A number of studies have demonstrated that platelet function tests canpredict ‘major adverse cardiac events’ in cardiovascular disease but noneof these assays have yet been sufficiently studied in large clinical trials tobecome part of standard clinical evaluation . Further research is necessaryto understand the unpredictable nature of platelet function in patients withET undergoing major surgical procedures.References1. Rossi C, Randi ML, Zerbinati P, Rinaldi V, Girolami A. Acute coronary disease inessential thrombocytemia and polycythemia vera. J Intern Med 1998; 244: 49-53.2. Pick RA, Glover MU, Nanfro JJ, Dubbs WF, Gibbons JA, Vieweg WV. Acutemyocardial infarction with essential thrombocytemia in a young man. Am Heart J1983; 106: 406-7.3. Kotani N, Sakai T, Ohshima S, Kudoh A, Dobashi N, Matsuki A. Anesthetic experience of a patient with essential thrombocytemia. Masui 1990; 39: 114-7.4. Iemura J, Sato T, Yamada T, Ando F. A case report: coronary artery bypass grafting for the patient with essential thrombocytemia. Nippon Kyobu GekaGakkai Zasshi 1991; 39: 1237-41.5. Momiyama T, Hiranaka T, Nomura F, Tominaga H, Nishioka T. Coronary arterybypass grafting for left main trunk coronary artery lesion associated with essential thrombocytemia. Clin Cardiol 1993; 16: 691-3.6. Schott U. Essential thrombocytemia and coronary bypass surgery. J CardiothoracVasc Anesth 1994; 8: 552-5.7. Kohno K, Takeuchi Y, Gomi A, Nakatani H, Yamanaka J. A case report of aorto-coronary bypass surgery in a patient with essential thrombocytemia.Nippon Kyobu Geka Gakkai Zasshi 1997; 45: 55-8.8. Ohto T, Shihara H, Miyauchi Y, Nakajima N. A case of coronary artery bypasssurgery using left internal thoracic artery and right gastroepiploic artery for apatient with essential thrombocytemia. Jpn J Thorac Cardiovasc Surg 1998; 46:767-71.9. Klinzig P, Markert UR, Liesaus K, Peiker G. Case report: successful pregnancy anddelivery after myocardial infarction and essential thrombocytemia treated withclopidrogel. Clin Exp Obstet Gynecol 2001; 28: 215-6.10. Haddad M, Veinot JP, Masters RG, Hendry PJ. Essential thrombocytosis causinga massive myocardial infarction. Cardiovasc Pathol 2003; 12: 216-8.IntroductionAortic coarctation is a serious pathology required surgical treatment. About 50% of uncorrected isolated aortic coarctation casesare lost up to the age of 10, only 10% may reach the age of 50 (1). Themost common reason for death from untreated aortic coarctation is theaneurysm or rupture of aorta or side branches with a rate of 23% (1).Aortic insufficiency resulting from annuloaortic ectasia and ascendingaortic aneurysm together with aortic coarctation rarely occur, and surgical treatment is difficult. It is very important to decide whether surgical operation will be of one and two stage, and to determine intraoperative strategy.Aortic coarctation is a congenital vessel disease that can causesuch complications as myocardial infarction, congestive cardiac failure,Anadolu Kardiyol Derg2007; 7: 436-48Olgu Sunumlar›Case Reports 445Address for Correspondence/Yaz›flma Adresi: Cevdet U¤ur Koço¤ullar›, Assist Prof, MD, Derviflpafla Mh. Dr. Mahmut Hazar Cd. Buse Apt. No: 43 A Blok B B Girifl D: 4 Afyon, TurkeyPhone: +90 542 235 56 03 E-mail: cevdetkoc@hotmail.com Two-stage successful surgery in an aortic coarctation case operated initially for ascending aortic aneurysmÖncelikli olarak assandan aort anevrizmas›na müdahale edilen aort koarktasyonlu bir olguda iki evreli baflar›l› cerrahi tedaviBilgehan Erkut, Cevdet U¤ur Koço¤ullar›, Ahmet Özyaz›c›o¤lu, Hikmet KoçakDepartment of Cardiovascular Surgery, Medical Faculty, Atatürk Üniversity, Erzurum, Turkey

Esansiyel trombositemide koroner baypas cerrahisi

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Esansiyel trombositemide koroner baypas cerrahisi

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