Endomyocardial Fibrosis Secondary to Hypereosinophilic Syndrome

Abstract

We present the case of a 27-year-old man from Paraguay who was referred to our centre because of pain, paresthesias and ulcered lesions in both lower limbs. An arteriogram showed diffuse arterial disease with areas of stenosis and occlusion as well as important collateral circulation. With the diagnosis of advanced lower extremity peripheral artery disease, medical treatment was started, but he finally required a left limb supracondylar amputation. During the postoperative period, he suffered an acute pulmonary edema requiring intravenous diuretics and non invasive mechanical ventilation. An echocardiogram was performed, revealing severe left ventricular dysfunction with global hypokinesia and a diastolic restrictive pattern, secondary severe mitral regurgitation and mild right ventricular dysfunction. Several complementary tests were performed, finding a constant elevation of eosinophiles and IgE levels. A cardiac resonance revealed circumferential subendocardial late gadolinium enhancement in both ventricles, suggesting endomyocardial fibrosis, without areas of edema in STIR sequences. A wide differential diagnosis was attempted, dismissing most common etiologies. We ruled out infectious diseases, mainly parasitic infections, but also HIV, Lyme and Chagas disease, among others. We also considered celiac disease and other endocrinal diseases or nutritional deficits, Buerger disease, sarcoidosis, amyloidosis, hemochromatosis and acute myocarditis. Coronary artery disease was ruled out with an angio-CT. Hypereosinophilic syndrome (HES) is a multisystemic disease as a result of mantained blood elevation of eosinophiles and IgE. Primary causes (hematologic disease) were dismissed by means of a bone marrow biopsy, as well as secondary causes (hypersensitivity, atopia, parasite infection, autoimmune disease, collagen disease, malignancy, eosinophilic pulmonary diseases, hyper-IgE syndrome, etc.). Finally, an idiopathic HES with cardiac and vascular - but also pulmonary and hematologic - involvement was diagnosed. We initiated treatment with bisoprolol, enalapril, eplerenone and ivabradine. We also started corticosteroids and, later on, azathioprine, with analytical improvement but with no impact on left ventricular ejection fraction. A CRT-D was implanted for primary prevention of sudden cardiac death. The patient is currently in a stable situation with good functional class and no hospital readmissions. Collaboration among different specialities is mandatory for the managment of these patients with complex multisystemic diseases. (Figure presented).