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Interact CardioVasc Thorac Surg 2008;7:928-930. doi:10.1510/icvts.2008.175687 © 2008 European Association of Cardio-Thoracic Surgery
Hypereosinophilic syndrome and myocardial fibrosisDepartment of Cardiac Surgery, Hospital Clínico San Carlos, Madrid, Spain Received 16 January 2008; received in revised form 11 March 2008; accepted 12 March 2008
*Corresponding author. Secretaria de Cirugía Cardiaca, Hospital Clínico San Carlos, Plaza Cristo Rey s/n, Madrid, P. Code: 28040, Madrid, Spain. Tel.: +34913303691.
A 62-year-old man was accepted to our institution because of hypereosinophilia, severe tricuspid regurgitation and isolated right restrictive myocardiopathy, with thrombi inside the right atrium and ventricle. Based on the diagnosis of hypereosinophilic syndrome plus eosinophilic myocarditis, the patient underwent a tricuspid valve repair and endomyocardiectomy. We briefly discuss hypereosinophilic syndrome myocardiopathy, and its management.
Key Words: Inflammation; Myocardium; Right ventricle
Eosinophilic myocarditis (EM) occurs in up to 60% of patients diagnosed with hypereosinophilic syndrome (HES) [1–3]. EM prognosis is poor, mainly in its advanced stages.
A 62-year-old man was admitted to our institution because of two months progressive dyspnea and atypical thoracic pain. Frank signs of right cardiac failure and tricuspid regurgitation (TR) murmur were observed. The electrocardiogram (ECG) showed atrial fibrillation (AF) at 130 bpm. Transthoracic (TTE) and transesophageal echocardiography (TEE) showed moderate pericardial effusion, severe TR, diffuse thickening of the right ventricle (RV) with evident signs of myocardial restriction (filling pressures >25 mmHg), and organized intracavitary thrombi that partially occluded both cavae veins, right atrium (RA) and RV. Coronary angiography revealed no significant lesions. Biopsy of the RV was not made due to the high risk of embolization. A computerized tomography showed normal pulmonary arteries. A deep venous system echo-Doppler of the inferior members was informed as normal. Eosinophilia (>1600 eosinophils/µl in three different samples) was present in the blood count. Parasite detection and coagulation studies showed no pathological data. Bone aspiration revealed a normal maturation of the three series with an increased population of polymorph eosinophils. A diagnosis of myocardial fibrosis associated to HES was settled. Medical therapy with corticosteroids, digitalis, betablockers and anticoagulation was started. After one week of treatment, the clinical situation of the patient got worse. TTE showed signs of cardiac tamponade and a growing presence of intraluminal thrombi. Given the bad result to the treatment, we decided on surgery. In Trendelenburg position, through median sternotomy, ascending aorta and both cavae cannulation, moderate hypothermia, aortic cross-clamping and anterograde perfusion of cardioplegia, the right cavities of the heart were explored. A great mass of organized thrombi had grown upon the entire surface of the RA and RV walls. Myocardium was thickened and fibrotic, with retraction of the tricuspid subvalvular apparatus (Fig. 1). Endomyocardium of RV was aggressively extirpated and a tricuspid repair (chordae tendinae resection, and modified De Vega annuloplasty) was performed. Mild residual TR was observed by TEE after extracorporeal circulation interruption.
Six hours after surgery the patient died because of a massive refractory right ventricle failure. The clinical autopsy revealed a HES with EM, and eosinophil infiltration of the liver, spleen, kidney, stomach, intestine and pleura. RV (Fig. 2) had large areas of endomyocardial fibrosis with inflammatory infiltration of fibrin and eosinophils. Endocardial fibrin thrombi were observed in the RA, RV and cavae veins.
HES is characterized by a maintained overproduction of eosinophils with organic affectation [1]. HES is defined by [4, 5]: a) eosinophilia in blood >1500/µl during six months; b) absence of any other cause of hypereosinophilia; and c) signs and symptoms of organic affectation. In our case, we lacked previous blood counts of the patient. Thus, the settled diagnosis was of suspicion. The etiology of the HES has not been clarified yet [6]. HES affects men more frequently than women (9 to 1) [1]. The beginning is usually asymptomatic, being discovered by chance. Injury to the heart, skin, nervous system, lungs and spleen occurs in up to 45–60% of the cases [7]. Eosinophilic myocarditis may be secondary to HES or other causes of maintained hypereosinophilia [1]. Cardiac damage follows extracellular protein deposit and activation of eosinophils by IL-5. It evolves through three stages [1]: 1) Necrotic stage: it is characterized by endocardial damage, eosinophilia and linfocitic infiltration in the myocardium with necrosis and formation of microabscesses. At this moment, the disease is usually asymptomatic. 2) Mural thrombi develop. By now, symptoms derived from pulmonary embolism may appear, and TEE may demonstrate the presence of thrombi in the LV or RV. 3) In the fibrotic phase a progressive scarring of the endocardium takes place and gives rise to clinical and echo signs of restrictive myocardiopathy with atrioventricular valve dysfunctions. Clinical suspicion usually settles down in the advanced stages of the disease. The definitive diagnosis is made by endomyocardial biopsy, although it is necessary to consider the risk of embolism. Corticosteroids are the base of the treatment of HES [1]. Anticoagulation is only justified if thrombi are present, and not for prophylactic use [7].
Medical treatment in advanced stages of EM is symptomatic and does not alter the progression of cardiac failure nor completely avoid the risk of embolism. Surgical indication is given by these facts. The operative technique was first described by Dubost et al. In our case, we made the endomyocardiectomy under extracorporeal circulation, aortic cross-clamping and antegrade cardioplegic perfusion. In cases of isolated right cavities' fibrosis without evident RV thrombosis, it is feasible to undergo a beating-heart operation, since the risk of embolism is low. When right cavities' exploration is needed, selective cavae veins cannulation should be accomplished. In our case, a relatively ample free margin existed in both cavae, 2 cm beyond the RA, that allowed us to directly cannulate them. If cavae thrombosis is massive, it is necessary to establish a peripheral venous drainage, inserting cannulas in jugular and femoral veins. When macroscopic pulmonary thromboembolism exists, a direct extraction can be made exploring the pulmonary artery and its branches. In these circumstances, a Trendelenburg technique should be taken into account. Endomyocardiectomy of the right cavities can be accomplished via transatrial if the area of fibrosis of the RV is accessible. On the contrary, when fibrosis of the RV is extensive or inaccessible, it is necessary to make a right ventriculotomy, by means of a perpendicular incision to the trajectories of the LAD and posterior interventricular artery. Then the thrombi are extracted until the fibrosed endocardium is identified. Metras et al. recommend to make a decortication until finding healthy myocardium. Bands of fibrosis that penetrate deep into the myocardium may be observed. In these cases, it is agreed to section them without trying to extract them. Whenever possible, it is necessary to preserve AV valves, since early thrombosis and prosthesis dysfunction by pannus are very frequent. It is mandatory to make a TEE after interrupting extracorporeal circulation to assure the valve competence. The risk of recurrence of EM is high. Long-term anticoagulation and steroids must be considered to minimize this risk.
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Abstract
1. Introduction
