Ann Thorac Surg 2002;73:1307-1310
© 2002 The Society of Thoracic Surgeons
Case report
Eosinophilic heart disease with vasculitis: supported by HeartMate left ventricular assist device and heart transplantation
Chiao-Po Hsu, MDa,
Shiao-Hwang Chang, MDc,
Jih-Shiuan Wang, MDa,
Chun-Che Shih, MDa,
Shiau-Ting Lai, MD*a,
An-Hang Yang, MDb
a Division of Cardiovascular Surgery, Department of Surgery, National Yang-Ming University, School of Medicine and Taipei Veterans General Hospital, Taipei, Taiwan, Republic of China
b Department of Pathology, National Yang-Ming University, School of Medicine and Taipei Veterans General Hospital, Taipei, Taiwan, Republic of China
c Department of Surgery, Veterans Hospital—Tauyuan, Tauyuan, Taiwan, Republic of China
Accepted for publication June 28, 2001.
* Address reprint requests to Dr Lai, Division of Cardiovascular Surgery, Department of Surgery, Taipei Veterans General Hospital, 201 Sec 2, Shih-Pai Rd, Taipei, Taiwan 112, ROC China
e-mail: laist{at}vghtpe.gov.tw
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Abstract
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Eosinophilic heart disease is rarely seen in clinical practice. We report a patient with eosinophilic heart disease who presented with acute myocardial infarction and congestive heart failure. Because of lack of a donor organ and progressive deterioration of his general condition in spite of intraaortic balloon pumping, a left ventricular assist device (HeartMate 1000 IP; Thermo Cardiosystems Inc, Woburn, MA) was implanted before development of irreversible multiple organ failure and was followed by heart transplantation.
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Introduction
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Eosinophilic heart disease is rarely encountered in clinical practice. The natural history of the disease is usually rapid and unfavorable. The optimal therapy is undetermined and death is the usual outcome. In this report, we present a young patient who suffered from the disease and is surviving today after undergoing implantation of a left ventricular assist device, followed by heart transplantation.
A 27-year-old man was admitted to our hospital on October 17, 1998 because of chest tightness, palpitation, and increasing dyspnea for 3 days. The patient was found to have a 2-year history of bronchial asthma. Myositis of extraocular muscles was suspected 4 months earlier. No major risk factors of atherosclerosis were noted. Physical examination on admission showed tachycardia, mild fever, wheezing, and moist rales over the bilateral lung field. The leukocyte count was 10,100/cumm; the plasma levels of creatine kinase and creatine kinase-MB fraction were 157 U and 17 U, respectively. The chest roentgenography showed alveolar process over the bilateral central lung field. Under the impression of bronchial asthma with acute exacerbation and complicated pneumonia, he was treated with empiric antibiotics and bronchodilators.
Unfortunately, the clinical condition deteriorated rapidly because of acute pulmonary edema 1 day after admission. Electrocardiography (ECG) showed sinus tachycardia, right axis deviation, low voltages in limb leads, and anterolateral infarction (deep Q-wave over V1 to V5). Echocardiography revealed dilated left ventricle (LV) left ventricular internal diameter (LVID)s/d: 61/69 mm; akinesis of mid to apical anterior ventricular septum, anterior wall, lateral and posterior wall, and old extensive anterior wall myocardial infarction with severe LV dysfunction; incomplete closure of mitral valve with moderate-to-severe mitral regurgitation; moderate pulmonary hypertension with moderate tricuspid regurgitation and mild pulmonary regurgitation; patent foramen ovale (PFO) with left to right shunt. The patient was transferred to the coronary care unit for further management on October 19, 1998 under the presumptive diagnosis of: (1) acute myocardial infarction with pulmonary edema, killip III; and (2) bronchial asthma complicated pneumonia.
Cardiac catheterization performed later revealed proximal left anterior descending coronary artery total occlusion; in the middle segment of the left circumflex coronary artery (LCX-M), total occlusion with thrombus formation (Fig 1A),
and normal right coronary artery. During examination, intraaortic balloon pumping (IABP) was inserted due to cardiogenic shock. On October 26, 1998, percutaneous transluminal coronary angiography (PTCA) over LCX-M was performed and urokinase was used for thrombolytic therapy intracoronarily and systemically. Three days later, PTCA with stent was done while thrombus was decreased in the coronary artery. The LCX-M was successfully dilated, but haziness in the stent was still noted in coronary angiography (Fig 1B).
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Fig 1. Left coronary system in right anterior oblique view. (A) Pre- and (B) post-percutaneous transluminal coronary angiography with stent insertion.
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On October 30, 1998, eosinophilia was found by routine blood count (leukocyte count: 11,700/cumm; total eosinophilic count: 2,200/cumm). However, serial investigations found no evidence of parasite infection, human immunodeficiency virus infection, atypical pneumonia, drug-induced allergy, or malignancy. Total eosinophilic count surged persistently (white blood cells: 11,500/cumm; eosinophils: 59%) on November 10, 1998. Methylprednisolone, 30 mg intravenously q6h, was tried under the suspicion of eosinophilic pulmonary syndrome and eosinophilic myocarditis. After 6-day steroid treatment, the eosinophilic count became normal and clinical condition greatly improved. Steroid was tapered and stopped on November 30, 1998.
Though PTCA with stent insertion was attempted, the patient still suffered from congestive heart failure. Position emission tomographic scans showed nonviable myocardium in the anterior and inferolateral walls, and myocardial ischemia in the anterolateral and inferior walls of the right ventricle (RV). Therefore, heart transplantation was considered the best choice of treatment. Later, eosinophilia recurred and his condition deteriorated. He was transferred to the intensive care unit for IABP support; steroid was given again. On January 5, 1999, the endomyocardial biopsy (EMB) was done which showed thrombus attached to endocardium; a small amount of eosinophils were also noted in the thrombus.
Because of continuing deterioration after IABP insertion, HeartMate 1000 IP (Thermo Cardiosystems Inc, Woburn, MA) implantable pneumatic left ventricular assist device (LVAD) was implanted on an urgent basis as a bridge to heart transplantation to prevent irreversible multiple organ failure on January 14, 1999. Meanwhile, PFO was closed and tricuspid valve was repaired with Devega’s method. The patient was weaned off of the ventilator 1 month later, and then he was transferred to an ordinary ward for further care. The myocardium of the LV apex during LVAD implantation showed extensive myocardial and endocardial fibrosis with mural thrombosis; some scattered eosinophils were also noted in the myocardium and endocardial thrombus (Fig 2).
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Fig 2. Light microscopy of left ventricle apex removed during left ventricular assist device implantation. (A) Myocardium; (B) endocardium and mural thrombus. (Hematoxylin and eosin stain; original magnification, x133.)
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Later, he began a rehabilitation program with the LVAD to restore muscle mass and strength. Serial follow-up echocardiography evidenced partial recovery of heart function (LV ejection fraction, 56%, RV ejection fraction, 49%, under LVAD support). Prior to heart transplantation, another four attacks of hypereosinophilia induced cardiopulmonary embarrassment and unstable LVAD flow that often dropped below 4 L/min (about 1/2 to 2/3 of stable flow). Whenever hypereosinophilia recurred, steroid was administered and clinical condition was improved. The exit site of drive-line infection appeared 2 months postimplantation; wound culture showed coagulase negative staphylococcus persistently.
After 266-day support by HeartMate LVAD, the patient received orthotopic heart transplantation on October 6, 1999. During the operation, infection of the pump pocket was found. Because of severe mediastinal fibrotic adhesion, total ischemic time of the donor heart was 230 minutes; total bypass time was 360 minutes. Both extracorporeal membrane oxygenation and IABP were used for postoperative circulatory support. The pathology of the explanted heart disclosed conspicuous vasculopathic changes including wall thickening of proximal coronary arteries (Fig 3A), organized thrombosis with recanalization of the middle segment of the left anterior descending coronary artery (Fig 3B), and advanced wall thickening with nearly complete obliteration of intramuscular branches of the coronary arteries (Fig 3C).
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Fig 3. Light microscopy of (A) proximal segment of left anterior descending coronary artery, (B) middle segment, and (C) intramuscular branches of coronary artery. (Hematoxylin and eosin stain; original magnification, x50.)
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One week later, mediastinitis was found. Postoperative pneumonia and adult respiratory distress syndrome also complicated recovery. After several operations and frequent debridements, successful reconstruction of the chest wall was performed with muscular flaps and a split-thickness skin graft.
One month posttransplantation, EMB showed International Society for Heart Lung Transplantation grade 0, with no evidence of acute rejection. Overall, after 6-week intensive care posttransplantation, the patient was transferred to an ordinary ward. He was finally discharged on December 30, 1999. One year posttransplantation, he was living very well and had returned to work. Neither rejection nor eosinophilia were noted during this period.
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Comment
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Some cases of eosinophilic heart disease were reported in the past. Eosinophilic myocardial infiltration has been reported in association with pronounced eosinophilia in the circulating peripheral blood [1–4], caused by an allergic diathesis [1], parasitic infestation [3], drug hypersensitivity [4], or various types of vasculitis (such as Churg-Strauss syndrome) [2]. The heart may be involved with coronary artery vasculitis in addition to eosinophilic myocardial infiltration. Although eosinophilic heart disease may manifest with chest pain [2, 4], ECG abnormalities suggestive of myocardial infarction and echocardiographic observation of regional wall motion abnormalities are unusual in the absence of coronary artery involvement [4]. Coronary arteritis is considered responsible for most myocardial infarction [2]. In our case, the symptoms of chest pain, abnormal ECG and echocardiography are compatible with the finding of coronary artery angiography. Hypereosinophilia was additionally found. Although numerous eosinophiles were not found in the pathologic examination of EMB and LV apex because of steroid interference or inflammation subsidence, eosinophilic heart disease with vasculitis was highly suspected from the evidence of the clinical course and pathologic examination. Finally, it was supported by the pathologic finding of diffuse vasculopathy of the coronary artery in the explanted heart. So, vasculitis is considered to be the etiology of the coronary artery stenosis or occlusion and the haziness of coronary angiography post-PTCA. Vasculitis may be associated with the symptoms and signs of bronchial asthma.
The natural history of eosinophilic heart disease is usually rapid and unfavorable. The usual outcome is untreatable congestive heart failure [4] and death, which may be sudden [1]. So early suspicion [5] by clinical symptoms with or without elevation of eosinophilic count and diagnostic biopsy [2, 3] are extremely important. Clinical improvement is uncommon but possible with early corticosteroid treatment [2, 5]. In this case, PTCA was tried but failed. Coronary artery bypass grafting was even considered but was not feasible because of underlying vasculitis. Eventually, we implanted the HeartMate LVAD because of the progressive deterioration of congestive heart failure.
The wound care of the LVAD patient is critically important, because it might progressively induce pump pocket infection, graft infection, or even tissue valve endocarditis and sepsis. In our patient, the exit site wound did not heal well in the early phase possibly due to poor nutrition, poor general condition, bad drive-line fixation, and relatively short subcutaneous tunnel (the patient’s height is 165 cm). If there had been no infection of the exit site and pump pocket, mediastinitis may not have occurred. The importance of wound care of LVAD patients cannot be emphasized more.
Heart transplantation could be a rational and effective treatment of severe eosinophilic heart disease before its fatal event [6]. Yet, there was no literature found regarding the treatment of such disease with LVAD. Based on our experience in this extremely rare case, we found that the eosinophilic heart disease did relapse during the LVAD support and also impact the cardiac function during relapse when it resulted in the significant decrement of LVAD pump flow, though the condition could be controlled with steroid administration. While characteristics of the disease should be kept in mind, long-term low-dose steroid treatment should be considered in such patients. After heart transplantation, serial investigations including echocardiography, ECG, EMB, immunoglobulin E, and leukocyte with differential count showed no evidence of rejection of, nor recurrence of eosinophilic heart disease in, the transplanted heart. Similar to previous reports, this could be attributed to the immunosuppressant treatment after transplantation. On this particular patient, we conclude that LVAD and heart transplantation is a life-saving and effective treatment of eosinophilic heart disease, though long-term follow-up is still needed.
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References
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Touze J.E., Debonne J.M., Scheiner C., et al. Acute necrotizing eosinophilic myocarditis. Favorable clinical course after heart transplantation. Presse Med 1992;21:565-568.
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Abstract
Introduction
