Surgical resection of restrictive left ventricular endomyocardial fibrosis

A 60-year-old Swiss male patient born with no foreign ancestors with seropositive rheumatoid arthritis (treatment with sulfasalazine 2 g/day, intermittently with Leflunomid 10 mg, and Golimumab 50 mg showing normal blood cell count) was admitted with secondary left ventricular (LV) endomyocardial fibrosis with deteriorating dyspnoea [New York Heart Association (NYHA) III] throughout 1 year. Endomyocardial fibrosis and intraventricular thrombus were first detected during diagnostic echocardiographic work-up of an aortoiliac thromboembolic event. Follow-up transthoracic echocardiography (TTE, Panel A) revealed a progressive LV endomyocardial fibrosis causing a restrictive LV filling pattern (E/A 2.8, e/e’ 47) with a severely dilated left atrium (LA volume indexed 65 mL/m2) and post-capillary pulmonary hypertension (sPAP 55 mmHg). Biventricular systolic function was normal (LV ejection fraction 55%, tricuspid annular plane systolic excursion (TAPSE) 18 mm). Cardiac magnetic resonance imaging (cMRI) showed a characteristic almost circumferential sub-endocardial mid-ventricular to apical late enhancement characterizing the endomyocardial fibrosis and the big intraventricular thrombus (Panel B), which progressed over time despite oral anticoagulation. The patient underwent transapical resection of endomyocardial fibrosis with subsequent LV closure using a Dacron patch. Combined direct and video-assisted surgery allowed complete controlled resection and exclusion of a LV outflow-tract involvement (Panels C–E). Histo-pathological resected specimen examination confirmed an eosinophilic myocardial fibrosis characteristic for the rare presence of Loeffler’s endocarditis (Panel F). Six months of follow-up revealed an improvement of dyspnoea (NYHA I), diastolic function (E/A 2.0, e/e’ 20), and pulmonary hypertension (sPAP 40 mmHg). Due to normalization of end-diastolic LV volume after thrombectomy, stroke volume also increased markedly. This case is uncommon in Europe and direct and video-assisted surgery allowed complete resection of endomyocardial fibrosis. Panel A: preoperative TTE with LV endomyocardial fibrosis (arrow) and apical thrombus (arrowhead). Panel B: cardiac magnetic resonance imaging of the left ventricle with apical thrombus (arrow) and endomyocardial fibrosis (arrowhead). Panel C: intra-operative transapical view of the LV endomyocardial fibrosis (arrow) and hypertrophic left ventricle. Panel D: intra-operative image of nearly resected LV endomyocardial fibrosis. Panel E: intra-operative transapical view of LV after total resection of endomyocardial fibrosis. Panel F: histo-pathological specimen of endomyocardial fibrosis after surgical resection.