Endomyocardial Fibrosis Research Articles

Cardiovascular operations in Behçet's disease

Cardiac involvement of Behçet disease (BD) representing a spectrum of valve disorder, recurrent aneurysm, intracardiac and great vessel thrombosis, and coronary artery disease with different presentations including pericarditis, cardiomyopathy, endocarditis, endomyocardial fibrosis, intracavitary thrombosis, and coronary artery disease is very rare. However, great vessel involvement more commonly involves the aorta, pulmonary artery, superior vena cava, inferior vena cava and hepatic veins, etc.

Endomyocardial Fibrosis and the Prothrombotic State: From Hypercoagualability to Protien C Deficiency

Thrombosis and endomyocardial fibrosis have coexisted for a long time. Earlier authors like Shaper, De Arbela and Davies have commented on the prevalence of large intracardiac thrombi in endomyocardial fibrosis. In three different reports Gupta PN, has also commented on intracardiac thrombi and their sequelae as thromboembolism in the past. We review here the possibility that apart from the basic intracavitary stasis due to chamber dilatation there may be an additional haematological defect in endomyocardial fibrosis. We review our previous studies on Protein C in endomyocardial fibrosis and the reduced platelet aggregation with collagen (but normal aggregation with ADP) that we had seen in our endomyocardial fibrosis patients. We postulated that these defects would add to the intracardiac thrombosis seen in endomyocardial fibrosis.

Endomyocardial fibrosis in hypereosinophilic syndrome

Endomyocardial fibrosis in hypereosinophilic syndrome

Non-tropical endomyocardial fibrosis associated with sarcoidosis

A 50-year-old female was admitted with a 2-year history of significant weight loss and a pyrexia of unknown origin. Hepatosplenomegaly and mildly enlarged retroperitoneal and mediastinal lymph nodes were seen on a computed tomography scan. The patient was profoundly cachectic with severe muscle wasting. Following an episode of acute pulmonary oedema, a transthoracic echocardiogram (TTE) was performed, revealing …

Endomyocardial fibrosis in patients with confirmed Churg-Strauss syndrome

Endomyocardial fibrosis in patients with confirmed Churg-Strauss syndrome

Strongyloides-Associated Left Ventricular Thrombus

Aexertional dyspnea, and a history of diarrhea. Echocardiography revealed a large intracavitary thrombus occupying the apical two thirds of the left ventricular (LV) cavity (Fig 1A) and severely elevated LV filling pressures. The mitral valve was mildly thickened, with significant subvalvular tethering. Further imaging with computed tomography and magnetic resonance imaging confirmed these findings (Fig 1B, C, D). Stool samples tested positive for Strongyloides stercoralis larvae. She was taken to the operating room with the diagnosis of endomyocardial fibrosis secondary to hypereosinophilia caused by the Strongyloides infection. Right and left ventricular apical thrombus and fibrotic tissue were identified and aggressively excised. The mitral valve was immobile because of the intense reaction and involvement of the thrombus, with the subvalvular apparatus necessitating

Cardiac involvement in hypereosinophilic syndromes detected by cardiac magnetic resonance imaging

Purpose: The hypereosinophilic syndromes (HES) are rare diseases characterized by blood hypereosinophilia and eosinophil-related organ damage, with the Churg-Strauss syndrome as the vasculitic subtype. Cardiac involvement, reported in 20-50% of patients in earlier series, carries poor prognosis and justifies aggressive immunosuppressive therapy. Cardiac magnetic resonance imaging (MRI) is the reference method for assessment of left ventricular (LV) function and allows evaluation of myocardial inflammation with consecutive oedema or myocardial fibrosis. We therefore sought to evaluate the frequency and patterns of cardiac manifestation of HES using cardiac MRI. Methods: 37 patients with definite HES were referred to our department for cardiac MRI because of possible cardiac involvement according to clinical, echocardiography and laboratory findings. MRI analysis included cine sequences for the assessment of LV function. T2-weighted imaging was conducted for assessment of myocardial oedema. Gadolinium late enhancement (LE) imaging was conducted for detection of fibrosis. Results: Of the 37 patients 14 were male. The mean age was 51.9±2.4 years. 20 patients also underwent coronary angiography, and only one patient had a significant stenosis of a marginal branch. Four patients (11%) had a cardiac MRI scan without pathological findings; three patients (8%) a non-circumferential PE of ≤ 5mm and an otherwise normal MRI. In 12 patients a PE ranging from 4 to 30mm was associated with other pathological findings. Quantitative analysis of LV function revealed a mean ejection fraction (EF) of 51.9±2.3%, EF was reduced (<55%) in 20 patients (54%). 7 patients (19%) showed hyperintense areas in T2-weighted images indicative of myocardial oedema. LE of the myocardium was observed in 23 patients (62%), all demonstrating a non-ischemic pattern of LE or multiple small endocardial LE zones not compatible with myocardial infarction due to coronary artery disease. Lastly, 4 patients (11%) showed the typical pattern of endomyocadial fibrosis with oedema and contrast enhancement of the myocardial/endocardial border zone and thrombotic material at the left and right ventricular apex. Conclusions: Cardiac MRI revealed pathologies in 90% of the study cohort. Yet, the pattern of cardiac manifestations varies significantly. More than half of the patients showed cardiomyopathy with reduced systolic LV function. Non-ischemic LE was present in the majority of patients and combined in one third of cases with myocardial oedema indicating acute inflammation. The unique pattern of endomyocardial fibrosis was observed in 11% of the patients.

Right‐Sided Endomyocardial Fibrosis with a Right Atrial Thrombus: Three‐Dimensional Transthoracic Echocardiographic Evaluation

Endomyocardial fibrosis is a form of restrictive cardiomyopathy mainly affecting poor children and young adults in geographically restricted areas of Latin America, Africa, and Southeast Asia. The pathophysiological hallmark of the disease is focal or diffuse endocardial thickening involving mainly the inflow, the apices, and the subvalvular region leading to valvular regurgitation, diastolic dysfunction and obliteration of the ventricular apex. Advanced right-sided disease has slow flow of blood through chambers with propensity of thrombus formation especially in the right atria. Although two-dimensional transthoracic echocardiography remains the cornerstone for the diagnosis of this disease, the case presented here shows how three-dimensional transthoracic echocardiography can add substantial information regarding the region of involvement of the right ventricle as well as the various characteristics of the right atrial thrombus.

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.

AB0240 Prevalence and severity of systemic vasculitis in various organs of patients with progressive system

ObjectivesThe aim of this study was to determine: the prevalence and severity of systemic vasculitis (SV) with or without fibromuscular and/or intimal proliferation (FIP) in various organs of SSc patients.Methods12...

AB0225 Severity of systemic vasculitis in progressive systemic sclerosis at death

BackgroundThe basic pathological process in progressive systemic sclerosis (SSc) is non-specific or fibrinoid necrotic systemic vasculitis (SV) with or without fibromuscular and/or intimal proliferation and successive adventitial fibrosis (FIP) (1)....

THU0069 Increased rate of diastolic heart failure in rheumatoid arthritis correlates with systemic inflammation and persistent disease activity, independent from treatment strategy

BackgroundIncreased prevalence of chronic heart failure is observed in patients with rheumatoid arthritis (RA) and heart failure with normal ejection fraction (HFNEF) is the predominant type. Although traditional cardiovascular risk...

HEART's Original [症例] 心内膜心筋線維症を合併した好酸球増多症の1例

HEART's Original [症例] 心内膜心筋線維症を合併した好酸球増多症の1例

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.

Heart failure in sub-Saharan Africa.

The heart failure syndrome has been recognized as a significant contributor to cardiovascular disease burden in sub-Saharan African for many decades. Seminal knowledge regarding heart failure in the region came from case reports and case series of the early 20th century which identified infectious, nutritional and idiopathic causes as the most common. With increasing urbanization, changes in lifestyle habits, and ageing of the population, the spectrum of causes of HF has also expanded resulting in a significant burden of both communicable and non-communicable etiologies. Heart failure in sub-Saharan Africa is notable for the range of etiologies that concurrently exist as well as the healthcare environment marked by limited resources, weak national healthcare systems and a paucity of national level data on disease trends. With the recent publication of the first and largest multinational prospective registry of acute heart failure in sub-Saharan Africa, it is timely to review the state of knowledge to date and describe the myriad forms of heart failure in the region. This review discusses several forms of heart failure that are common in sub-Saharan Africa (e.g., rheumatic heart disease, hypertensive heart disease, pericardial disease, various dilated cardiomyopathies, HIV cardiomyopathy, hypertrophic cardiomyopathy, endomyocardial fibrosis, ischemic heart disease, cor pulmonale) and presents each form with regard to epidemiology, natural history, clinical characteristics, diagnostic considerations and therapies. Areas and approaches to fill the remaining gaps in knowledge are also offered herein highlighting the need for research that is driven by regional disease burden and needs.

Loeffler's Endocarditis Presenting with Acute Abdominal Aortic Obstruction

Loeffler's endocarditis is a complication of diseases associated with the idiopathic hypereosinophilic syndrome, which is characterised by persistently elevated blood eosinophil counts with symptoms and signs of organ involvement especially in the heart, vascular system, nervous system and bone marrow. We report the involvements of the endocardium and aorta, without endomyocardial fibrosis and the complete resolution of the endocardial eosinophilic infiltration with steroids and anticoagulation therapy.

Right ventricular endomyocardial fibrosis – A case report

Endomyocardial fibrosis (EMF) is a progressive type of restrictive cardiomyopathy. It affects inflow portion of right and/or left ventricle and apex. It is a neglected tropical disease. Here we report a rare case of right ventricular endomyocardial fibrosis. A 70-year-old female presented to us with history suggestive of right-sided heart failure of two months duration. There was no eosinophilia. Chest X-ray showed cardiomegaly. Echocardiogram showed dilated right atrium and obliteration of the apex of the right ventricle. A diagnosis of Right ventricular Endomyocardial fibrosis was made. She was treated with diuretics and anticoagulants and she improved.

Decreased Protein C Levels in Endomyocardial Fibrosis: The Possible Significance

The occurrence of frequent intracardiac thrombi in Endomyocardial fibrosis, and the embolization of these thrombi contributing to the morbidity is an established fact. Since previous studies on endomyocardial fibrosis revealed normal prothrombin time, clotting times, bleeding time, it was considered worthwhile to investigate whether an inherited deficiency of protein C contributed to thrombosis in these patients. Measurements of protein C functional levels revealed that 17/18 (94.4%) of patients tested and 4/18 of the healthy controls were functionally deficient (p<.001) while the antigenic levels of 7 patients and 3 controls were deficient. It was observed that 5.55% and 44.4% of patients had normal profiles and Type 1 deficiency respectively, while 44.4% had Type II deficiency. This association between protein C deficiency and endomyocardial fibrosis may contribute to the formation of intracardiac thrombi in these patients.

Cardiomyopathies in Tropical Countries: Causes and Nosological Perspective

Background: Cardiomyopathy is the main cause of heart failure in developing countries, mainly in Africa. In those areas the concept of “tropical cardiomyopathy” is still used to design all unexplained cardiomyopathy. The primary aim of this review is first to review the main etiologies of cardiomyopathies observed in tropical countries and second to gain a better understanding of the nosological place of the so-called “tropical cardiomyopathies” in the current framework of cardiomyopathies. Methods and Results: We reviewed relevant references over the last forty years (June, 1976 to May 2012). Given literature data, endomyocardial fibrosis (EMF) is mainly diagnosed in sub-Saharan countries, as well as Brazil and India. Peripartum cardiomyopathy (PPCM) is observed with a higher prevalence than in temperate countries. Sickle cell anemia does not induce specific cardiomyopathy in all echocardiographic studies. Malnutrition and chronic anemia can induce reversible cardiac dysfunction. Myocardial involvement in parasitic infections is restricted to Chagas disease and probably to human African trypanosomiasis. Helminthiasis is not involved in the pathogenesis of cardiomyopathy except for the deleterious effect of high eosinophilia induced by some endemic diseases (filariasis, schistosomiasis). Primary cardiomyopathies (dilated, hypertrophic, and restrictive cardiomyopathy) have no specificity. Arrhythmogenic right ventricular dysplasia and left ventricular noncompaction are also reported and do not differ from elsewhere. Conclusions: The concept of tropical cardiomyopathy is no longer relevant as most of the cardiomyopathies observed in tropical countries have no specificity, with few exceptions (PPCM, EMF, Chagas disease). In this context, the European Society of Cardiology classification offers a simpler clinical approach and allows the inclusion of the rare tropical specificities.

Hypereosinophilic syndrome induced cystitis mimicking bladder tumor

Hypereosinophilic syndrome induced cystitis mimicking bladder tumor