Endomyocardial substrate of ventricular arrhythmias in patients with autoimmune rheumatic diseases.

INTRODUCTION: Delayed enhancement-magnetic resonance imaging (DE-MRI) has demonstrated that nonischemic cardiomyopathy is mainly characterized by intramural or epicardial fibrosis whereas global endomyocardial fibrosis suggests cardiac involvement in autoimmune rheumatic diseases or amyloidosis. Conduction disorders and sudden cardiac death are important manifestations of autoimmune rheumatic diseases with cardiac involvement but the substrates of ventricular arrhythmias in autoimmune rheumatic diseases have not been fully elucidated. METHODS AND RESULTS: 20 patients with autoimmune rheumatic diseases presenting with ventricular tachycardia (VT) (n = 11) or frequent ventricular extrasystoles (n = 9) underwent DE-MRI and/or endocardial electroanatomical mapping of the left ventricle (LV). Ten patients with autoimmune rheumatic diseases underwent VT ablation. Global endomyocardial fibrosis without myocardial thickening and unrelated to coronary territories was detected by DE-MRI or electroanatomical voltage mapping in 9 of 20 patients with autoimmune rheumatic diseases. In the other patients with autoimmune rheumatic diseases, limited regions of predominantly epicardial (n = 4) and intramyocardial (n = 5) fibrosis or only minimal fibrosis (n = 2) were found using DE-MRI. Endocardial low-amplitude diastolic potentials and pre-systolic Purkinje or fascicular potentials, mostly within fibrotic areas, were identified as the targets of successful VT ablation in 7 of 10 patients with autoimmune rheumatic diseases. CONCLUSION: Global endomyocardial fibrosis can be a tool to diagnose severe cardiac involvement in autoimmune rheumatic diseases and may serve as the substrate of ventricular arrhythmias in a substantial part of patients.

Surgical Management of The Endomyocardial Fibrosis of Right Ventricle Mimicking Tumor with Recurrent Pulmonary Embolism.

Endomyocardial fibrosis is a neglected tropical disease that leads to restrictive cardiomyopathy. Its etiopathogenis is unclear and involves the progression of 3 stages of the disease. Compared with echocardiography, cardiac magnetic resonance imaging shows better apical visualization of obliteration and thrombus and provides an early diagnosis. However, there is no specific drug therapy, although surgery can increase survival. Therefore, surgical resection of the fibrous and thickened endocardium is recommended for symptomatic patients. The risk of mortality increases as the ratio of endocardial fibrous tissue per body surface rises. The aim of this manuscript is to describe the surgical management of the right-sided endomyocardial fibrosis mimicking tumor with recurrent pulmonary embolism.

Endomyocardial fibrosis of the right ventricle: A case report of successful surgery.

AIMS: The case we report, shows a successful treatment of right ventricle endomyocardial fibrosis. MATERIALS AND METHODS: Surgical therapy by endocardial decortication seems to be beneficial for many patients with advanced disease who are in functional-therapeutic class III or IV. The operative mortality rate is high, but successful surgery has a clear benefit on symptoms and seems to favourably affect survival as well.

A case report of a 40-year-old woman with endomyocardial fibrosis in a non-tropical area: from initial presentation to high urgent heart transplantation.

BACKGROUND: Endomyocardial fibrosis (EMF) represents the most common cause of restrictive cardiomyopathy worldwide. Despite a high prevalence in tropical regions, it occasionally occurs in patients who have never visited these areas. While researches have proposed various possible triggers for EMF, etiology and pathogenesis remain largely unknown. Diagnosis is based on patient history, heart failure symptoms, and echocardiographic signs of restrictive ventricular filling, atrioventricular valve regurgitation and frequently apical thrombus. Following is a case report of an Austrian patient with EMF who eventually had to undergo a heart transplant. This case report strives to promote awareness for this in non-tropical areas uncommon but nevertheless detrimental disease. CASE PRESENTATION: A 40-year-old woman was presented at our emergency department with chest pain and fever up to 38.1° Celsius. Plasma troponin-T levels and inflammatory markers were slightly elevated, but the echocardiogram was without pathological findings. The patient was hospitalized on the suspicion of acute myocarditis and discharged soon after improvement. Eight months later, she was presented again with chest pain and symptoms of heart failure. The echocardiogram showed normal systolic left ventricular (LV) function with LV wall thickening and severe restrictive mitral regurgitation as well as aortic and tricuspid regurgitation. Coronary angiogram was normal but right heart catheterization showed pulmonary hypertension due to left heart disease. Further diagnostic workup with cardiac magnetic resonance imaging revealed subendocardial late enhancement and apical thrombus formation in the left ventricle compatible with the diagnosis of EMF. A comprehensive diagnostic workup showed no evidence of infection, systemic immunologic or hematological disease, in particular hypereosinophilic syndrome. After a multidisciplinary consideration of several therapeutic options, the patient was listed for heart transplantation. On the waiting list, she deteriorated rapidly due to progressive heart failure and finally underwent a heart transplantation. Histological examination confirmed the diagnosis of EMF. Six years after her heart transplantation, the patient was presented in an excellent clinical condition. CONCLUSIONS: Even in non-tropical regions, the diagnosis of EMF should always be considered in restrictive cardiomyopathy. Knowledge of the distinct phenotype of EMF facilitates diagnosis, but comprehensive workup and therapeutic management remain challenging and require a multidisciplinary approach.

Tricuspid valve relocation with endomyocardial fibrosis removal for Löeffler's endocarditis.

Löffler endocarditis with hypereosinophilic syndrome is rare but can cause critical ventricular obliteration by endomyocardial fibrosis. A 52-year-old woman experienced severe right heart failure with extreme shrinkage of her right ventricle, severe tricuspid regurgitation and marked right atrial enlargement. Preoperative tests showed identical pressures in the right atrium and pulmonary artery. Endocardial stripping was done, and to enlarge the right ventricle, we relocated the anterior and posterior tricuspid leaflets cephalad, up the right atrium wall, to 'ventricularize' a portion of the right atrium, with autologous pericardial augmentation of the tricuspid leaflets. An annuloplasty ring was added to reinforce the relocated tricuspid attachment. Right heart pressures normalized postoperatively. The patient recovered uneventfully. She has received corticosteroid therapy continuously and has shown no recurrence of heart failure in the 5 years since surgery.

Endomyocardial Fibrosis With End-Stage Heart Failure as a Consequence of a Myeloproliferative Neoplasm With Hypereosinophilia.

Hypereosinophilic syndrome is characterized by an overproduction of eosinophils that infiltrate and damage multiple organs. Cardiac dysfunction occurs frequently and is a main cause of morbidity and mortality. We describe the case of a middle-aged man diagnosed with a myeloproliferative neoplasm associated with hypereosinophilia and treated with imatinib. He was diagnosed with cardiac involvement by hypereosinophilic syndrome at a late stage, with an established restrictive cardiomyopathy. Because of end-stage heart failure, he successfully received a heart transplant. This disease might not be considered a contraindication for heart transplantation.

Ventricular endomyocardial fibrosis in a pregnant female with Behçet's disease.

A 32-year-old pregnant woman, diagnosed with Behçet's disease 6 months earlier, presented with recent mild hemoptysis and exertional dyspnea. Transthoracic echocardiography showed an enlarged dysfunctional right ventricle. A large hypoechoic triangular-shaped mass was seen attached to the inner right ventricular wall, filling the cavity. No change in the size of the mass was noted after anticoagulant administration, and right heart failure progressed. Surgery was performed to remove the mass and repair the tricuspid valve. This was a very rare presentation of Behçet's disease in pregnancy, which resulted in delivery of a completely healthy baby despite corticosteroid pulse therapy and cyclophosphamide.

A Rare Case of Mitral Valve Prolapse in Endomyocardial Fibrosis.

Mitral valve prolapse in endomyocardial fibrosis (EMF) is an unusual entity. Literature search reveals only 1 report of mitral valve prolapse assosiated with EMF. A 32-year-old woman, of African origin, who presented with features of right heart failure, was diagnosed to have mitral valve prolapse of rheumatic origin with severe mitral regurgitation and severe pulmonary hypertension (PAH). Intraoperative findings lead to the diagnosis of EMF. We report this rare case of mitral valve prolapse in EMF, in a geographical area where rheumatic heart disease is endemic, to showcase how a rare manifestation of EMF can be misdiagnosed as that of rheumatic heart disease.

Cardiac surgery for patients with heart failure due to structural heart disease in Uganda: access to surgery and outcomes.

OBJECTIVE: Few data are available on heart failure (HF) in sub-Saharan Africa. We aimed to provide a current picture of HF aetiologies in urban Uganda, access to heart surgery, and outcomes. METHODS: We prospectively collected clinical and echocardiographic data from 272 consecutive patients referred for suspected heart disease to a tertiary hospital in Kampala during seven non-governmental organisation (NGO) missions from 2009 to 2013. We focused the analysis on 140 patients who fulfilled standardised criteria of HF by echocardiography. RESULTS: Rheumatic heart disease (RHD) was the leading cause of HF in 44 (31%) patients. Among the 50 children included (age ≤ 16 years), congenital heart disease (CHD) was the first cause of HF (30 patients, 60%), followed by RHD (16 patients, 32%). RHD was the main cause of HF (30%) among the 90 adults. All 85 patients with RHD and CHD presented with an indication for heart surgery, of which 74 patients were deemed fit for intervention. Surgery was scheduled in 38 patients with RHD [86%, median age 19 years (IQR: 12-31)] and in 36 patients with CHD [88%, median age 4 years (IQR 1-5)]. Twenty-seven candidates (32%) were operated on after a median waiting time of 10 months (IQR 6-21). Sixteen (19%) had died after a median of 38 months (IQR 5-52); 19 (22%) were lost to follow up. CONCLUSION: RHD still represents the leading cause of HF in Uganda, in spite of cost-efficient prevention strategies. The majority of surgical candidates, albeit young, do not have access to treatment and present high mortality rates.

Long-term survival following surgery for endomyocardial fibrosis.

We report a successful follow-up after 28 years of a woman with obliterative restrictive endomyocardial fibrosis (EMF) that underwent complete surgical decortication with simultaneous mitral and tricuspid bioprosthetic valve replacement in 1982 and underwent successful reoperation for the structural failure of biological prostheses after 25 years.

Eosinophilic endocarditis and Strongyloides stercoralis.

A 40-year-old woman from El Salvador presented with 3 months of abdominal pain and diarrhea followed by 2 weeks of atypical chest pain and exertional dyspnea and was diagnosed with eosinophilic endocarditis secondary to Strongyloides stercoralis infection. Transthoracic echocardiogram revealed apical masses in the left and right ventricles and a thickened posterior mitral valve leaflet and cardiac magnetic resonance imaging confirmed the presence of a left ventricular apical mass with diffuse subendocardial delayed enhancement consistent with endocardial fibrosis. In conclusion, eosinophilic endocarditis is a rare cause of restrictive cardiomyopathy characterized by endomyocardial fibrosis and apical thrombosis and fibrosis with frequent involvement of the posterior mitral valve leaflet.