We present the case of a 61-year-old patient with a history of essential thrombocythemia (ET) who was diagnosed as having aortic valve stenosis and dilatation of his ascending aorta. His aortic valve and ascending aorta were replaced under hypothermic circulatory arrest (HCA). No clear guideline exists for preoperative, perioperative, and postoperative management of cardiac surgery using HCA for ET patients. After performing risk assessment, we prescribed preoperative aspirin therapy and postoperative care was planned as usual for cardiovascular surgery in our establishment. Unexpectedly, activated clotting time did not exceed 400 seconds, but the course of treatment was otherwise uneventful.

Cardiac Surgery Using Hypothermic Circulatory Arrest in a Case of Essential Thrombocythemia.

本態性血小板血症(ET)は慢性骨髄増殖性疾患に属し,血栓形成傾向と易出血性傾向の相反する二面を併 せ持つ複雑な病態を有する.本症を合併する症例に対する人工弁置換術の報告はきわめて稀であり,その病 態から術中の出血傾向と術後の血栓症が懸念される.今回,ETを合併した大動脈弁狭窄症(AS)に対し, 生体弁による大動脈弁置換術を経験した.症例は 69歳女性.検診で心雑音を指摘され,近医の心エコー検 査で ASを指摘された.弁口面積は 0.73 cm,左室-上行大動脈の圧較差 113 mmHgの ASであり,手術の 方針となった.入院時血小板数は 70万/μlで,ヒドロキシカルバミドは術直前まで内服し,シロスタゾール は術前 3日に中止し,術前 2日よりヘパリンを開始した.術中はヘパリンで活性化凝固時間(ACT)を 400 秒以上に維持し,生体弁を用いて大動脈弁人工弁置換術を施行した.術中易出血性は認めず問題なく手術終 了した.術後血小板数は 30〜40万/μlで推移し,出血も問題なくドレーンは術後 2日目で抜去可能であっ た.また血栓による合併症も認めず,術後 13日目に退院となった.ET合併例に対して開心術を施行する場 合,周術期の出血と術後の血栓症が問題となり,術前の血小板増多に対する処置と術後の抗血栓療法が重要 となる.日心外会誌 43巻 2号:49-52(2014) キーワード:本態性血小板血症;大動脈弁置換術;開心術;血栓症;出血

Aortic Valve Replacement in a Patient with Essential Thrombocythemia

Increased incidence of pulmonary hypertension (PH) has been reported in patients with chronic myeloproliferative disorders. The exact incidence of PH in essential thrombocythemia (ET) is unknown. Most of the reported literature consists of case reports or small studies. We designed this study to asses the incidence of PH in patients with ET and reactive thrombocytosis. Previously or newly diagnosed 46 patients with ET, and 40 patients with reactive thrombocytosis secondary to iron deficiency anemia were found to be eligible for this study. Diagnosis of PH was established via transthoracic echocardiography. PH was found in 22 (47.8%) out of 46 patients with ET. Seven patients with PH were newly diagnosed ET, 5 patients with PH were in low, and the other patients with PH were in intermediate or high risk category. We found statistically significant difference in terms of platelet counts between ET patients with PH and without PH (p = 0.027). None of the patients with reactive thrombocytosis had PH. In conclusion, PH appears to be common in patients with ET. Therefore, all patients with ET should be evaluated for PH. Larger and prospective studies are required to clarify the long-term impact of PH on the survival of these patients. Future studies are also needed to determine whether cytoreductive treatment and aspirin prevent the development of PH, and to determine the effects of cytoreductive treatments and aspirin on the prognosis of PH. The effect of PH on ET prognosis should also be determined in low risk ET patients.

Pulmonary hypertension in patients with essential thrombocythemia and reactive thrombocytosis

https://www.internationaljournalofcardiology.com/article/S0167-5273(03)00573-4/pdf

Functional and morphological cardiac changes in myeloproliferative disorders (clinical study).

yeloproliferative disorders (MPDs) are defined as neoplastic diseases that involve a proliferation of the myeloid stem cell or its derivatives. MPDs include chronic myeloid leukemia (CML), polycythemia rubra vera (PV), essential thrombocythemia (ET), and agnogenic myeloid metaplasia/myelofibrosis (AMM/MF). Bleeding and thrombosis are the primary causes of morbidity and mortality in patients with chronic MPDs. Thrombosis occurs in approximately one third of cases, contributing to a mortality of 15% to 40%. 1 Thrombosis may involve arteries or veins and, in some cases, involves unusual sites such as hepatic, mesenteric, or portal systems. The cardiovascular system is involved in 4% to 21% of cases of MPDs, 2,3 and acute ischemic coronary artery disease is the presenting symptom in these cases. 4‐9 Other cardiovascular complications in patients with MPDs are valvular heart involvement, pericardial involvement, aortitis, thrombosis of major vessels (eg, inferior vena cava), pulmonary embolism, and systemic and pulmonary hypertension. This article reviews case reports to document the different cardiovascular manifestations of MPDs and to highlight this association, particularly in younger patients who present with cardiac insult but who have no known risk factors including family history. In these cases, hematologic and oncologic causes should be sought, especially when pertinent history and clinical evaluation raise a high degree of suspicion. Indications for treatment modalities such as antiplatelet or antimitotic agents should be recognized. This article also emphasizes the need for further studies to evaluate cardiovascular manifestations of MPDs. Results of the literature search are listed in Table 1.

Cardiovascular Manifestations of Myeloproliferative Disorders: A Review of the Literature

A 62-year-old man with heart murmur and recent cerebral thrombosis was admitted to our hospital. Examination of his heart revealed mitral stenosis and hematological study confirmed the diagnosis of essential thrombocytosis (ET). After 1100 ml pre-operative phlebotomy, mitral valve replacement with a 29 mm Bjork-Shiley prosthesis was performed utilizing routine cardio-pulmonary bypass using only his prestored blood and moderate hypothermia. We had no difficulty in hemostasis at the operation. Acute gastric bleeding began suddenly as much as 1500 ml on the 4th postoperative day. As medical care was in vain, emergent distal gastrectomy was performed. As we used urokinase for fear of thrombosed valve formation, residual gastric bleeding was continued on. Then, we began to use Gabexate-Mesilate (FOY) instead of urokinase. Retrospectively, Gabexate-Mesilate was very useful drug for preventing the left atrium and prosthesis from thrombo-formation until the start of oral intake of Warfarin and Busulfan. To our knowledge, this would be the first case report of open-heart valve replacement surgery in a ET patient in Japan.

[A case report: mitral valve replacement for the patient with essential thrombocythemia].

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[A case report: mitral valve replacement for the patient with essential thrombocythemia].