Endomiocardiofibrose como causa rara de insuficiência mitral: relato de caso e revisão da literatura / Endomyocardial fibrosis as a rare cause of mitral insufficiency: case study and literature review

Paciente M.B.L., feminina, 62 anos, portadora de insuficiência cardíaca prévia (etiologia indefinida), apresentou-se agudamente com dispneia aos esforços e déficit neurológico súbito. O ecocardiograma evidenciou insuficiência mitral moderada e obliteração infiltrativado ventrículo esquerdo. Realizou-se ventriculografia, confirmando aspecto sugestivo de endomiocardiofibrose. Optou-se por tratamento clínico (atual NYHA I), com boa resposta. Objetiva-se descrever caso clínico incomum e aspectos gerais desta entidade de acordo com as mais recentes atualizações.

Case study of a female patient, 62 years old, with prior heart failure (etiology not defined) presenting dyspnea acutely on exertion, and sudden neurological deficit. An echocardiogram showed moderate mitral failure and left ventricular infiltrative obliteration. Ventriculography confirmed an appearance suggestive of endomyocardial fibrosis. Medical treatment (current NYHA I) was selected, with good response. The intention is to present a clinical study of an unusual case with general aspects of this entity according to the latest updates.

A population study of endomyocardial fibrosis in a rural area of Mozambique.

BACKGROUND: Endomyocardial fibrosis is the most common restrictive cardiomyopathy worldwide. It has no specific treatment and carries a poor prognosis, since most patients present with advanced heart failure. On the basis of clinical series, regional variations in distribution have been reported within several countries in Africa, Asia, and South America, but large-scale data are lacking on the epidemiologic features and early stages of the disease. METHODS: We used transthoracic echocardiography to determine the prevalence of endomyocardial fibrosis in a rural area of Mozambique. We screened a random sample of 1063 subjects of all age groups selected by clustering. Major and minor diagnostic criteria were defined, and a severity score was developed and applied. Cases were classified according to the distribution and severity of the lesions in the heart. RESULTS: The estimated overall prevalence of endomyocardial fibrosis was 19.8%, or 211 of 1063 subjects (95% confidence interval [CI], 17.4 to 22.2). The prevalence was highest among persons 10 to 19 years of age (28.1%, or 73 of 260 subjects [95% CI, 22.6 to 33.6]) and was higher among male than among female subjects (23.0% vs. 17.5%, P=0.03). The most common form was biventricular endomyocardial fibrosis (a prevalence of 55.5%, or 117 of 211 subjects [95% CI, 48.8 to 62.2]), followed by right-sided endomyocardial fibrosis (a prevalence of 28.0%, or 59 of 211 subjects [95% CI, 21.9 to 34.1]). Most affected subjects had mild-to-moderate structural and functional echocardiographic abnormalities. Only 48 persons with endomyocardial fibrosis (22.7%) were symptomatic. The frequency of familial occurrence was high. CONCLUSIONS: Endomyocardial fibrosis is common in a rural area of Mozambique. By using echocardiography, we were able to detect early, asymptomatic stages of the disease. These findings may aid in the study of the pathogenesis of the disease and in the development of new management strategies.

[The heart and the eosinophil]. / Le coeur et l'eosinophile.

The beneficial effects of polynuclear eosinophils (PE) are well known. However, under certain circumstances, PE can be harmful. The heart is a prime target for PE toxicity which is due to release of basic proteins by eosinophils including major basic protein, cationic protein, and peroxidase. The most common manifestation of PE toxicity is chronic parietal endocarditis (CPE) which regroups two entities: Loeffler's fibroplastic endocarditis and Davies' endomyocardial fibrosis. Loeffler's fibroplastic endocarditis occurs mainly in temperate climates. Patients present high, persistent eosinophil levels similar to those observed in essential hypereosinophilic syndrome (EHS) or Chusid syndrome. Davies' endomyocardial fibrosis occurs in tropical countries where eosinophilic helminthiasis are endemic. The incidence of eosinophilic myocarditis (EM) is low but probably underestimated. EM can be observed in any case involving PE and has been described in many cases of drug-induced atopy, in Churg and Strauss syndrome, and in EHS. The most common cause of death is short-term occurrence of cardiogenic shock or dilated hypokinetic cardiomyopathy. Some patients have been successfully treated by early, intensive corticosteroid therapy and/or heart transplantation. The nosological classification of EM and CPE remains controversial. The two disorders may form a continuum with CPE as the second phase. Other authors have suggested that EM and CPE result from the action of PE on two distinct targets, i.e. endothelial cells for EM and myocytes for CPE. In the future, it may be possible to identify subjects with a predisposition to PE-induced heart disease by studying of genes coding for interleukins (IL-5, IL-4, IL-3) and GM-CSF in the 5q31-q33 region of chromosome 5.

Determinants of survival in endomyocardial fibrosis.

This study describes the clinical course of 108 patients with endomyocardial fibrosis. There were 76 females and 32 males, with a mean age of 35 years. All patients underwent cardiac catheterization. The angiographic data show that 64 (59%) patients had biventricular involvement. From a clinical point of view, 91 (84%) were in New York Heart Association functional classes III and IV. According to the right and left ventricular intensity of fibrosis, the patients were classified as mild (40 [37%]), moderate (36 [33%]), or severe (32 [30%]) for the right ventricle and mild (19 [18%]), moderate (61 [56%]), or severe (28 [26%]) for the left ventricle. Tricuspid regurgitation was observed in 63 (58%) patients and mitral regurgitation in 65 (60%) patients. Fifty patients in functional classes III and IV underwent surgical treatment. Survival curves constructed according to functional classes showed better prognosis for those in classes I and II (p = 0.0452). Survival curves for patients in classes III and IV for clinical and surgical treatment showed a trend toward better results for the surgical group. Analysis of factors that may have influenced the course of endomyocardial fibrosis showed that biventricular involvement (moderate or severe), right ventricular fibrosis, and presence of tricuspid and mitral regurgitation were associated with greater mortality.

The reliability of the tracing-method of fine cardiac fibrosis at a magnification of x10--preliminary study for quantitative analysis of fibrosis in large tissue sections of hearts with cardiomyopathy.

to define the reliability of the tracing method of fine fibrosis at low magnification (x10), the percentage area of fine fibrosis was compared between the traced pictures at magnifications of x10 and x250, using an image analyzer (Olympus VIP-21). A total of 25 tissue areas, each approximately 4 x 7 mm, from the inner and middle thirds of the left ventricular free wall and ventricular septum were selected from ten hearts with hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) and were stained with Masson trichrome. The percentage areas of fine fibrosis traced at a magnification of x10 correlated well with those traced at a magnification of x250 (Y = 1.08X - 1.1, r = 0.95, P less than 0.01). It is concluded that the overall percentage area of fibrosis is the same at magnifications of x10 and x250, despite the fact that at x10 one is unable to detect individual fibers which can be detected at x250. The tracing method of fine cardiac fibrosis at a magnification of x10 is reliable. Therefore, fine fibrosis in large tissue sections of hearts with cardiomyopathy, such as entire transverse sections of the left ventricular wall, can be quantitatively analyzed by this method.

[Distomatosis and cardiac disease. Apropos of 2 new cases. Trials of classification and pathogenetic hypotheses]. / Distomatose et cardiopathie. A propos de deux nouvelles observations. Essais de classification et hypothèses pathogéniques.

The authors report two new cases of cardiac disease associated with distomatosis: one case of biventricular fibroblastic parietal endocarditis affecting mainly the left side in a young female of 26 years, and one case of cardiomyopathy with atrial endocardial fibrosis, affecting especially the left ventricle in a man of 47. Bearing in mind the five cases reported in the literature, the authors propose a classification of cardiac disorders supposed to be due to distomatosis using three groups (endocardial fibrosis, cardiomyopathy, myocarditis) and relate them to a common pathogenesis based on immuno-allergic theory.

Löffler's endocarditis and endomyocardial fibrosis--a nosologic entity?

The problem whether Löffler's endocarditis and endomyocardial fibrosis belong to the same disease spectrum or are separate entities is still under debate. Until recently it was believed that endomyocardial fibrosis was a disorder restricted to tropical areas. Three Danish patients are presented, two showing a continuous disease spectrum, one patient showing the fully developed endomyocardial fibrosis which is indistinguishable from endomyocardial fibrosis described from the tropical areas. Eosinophilia was present in all three patients. The findings described in these three patients lend support to the unitarian hypothesis that Löffler's endocarditis and endomyocardial fibrosis belong to the same disease spectrum, and that the eosinophilic granulocyte may be the underlying cause.