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PubMed1979

Endomyocardial fibrosis and rheumatic heart disease in Mozambique.

The first five cases of endomyocardial fibrosis to be reported from Mozambique are described in an autopsy series over the years 1975-77, together with 85 cases of rheumatic heart disease in patients who died and were autopsied during the same period. Outside the central parts of the African continent, endomyocardial fibrosis does not seem as rare as previously thought. Observations on the age and sex of the patients correspond with what is already known about both diseases. The strikingly higher frequency of rheumatic heart disease discussed in this review is at variance with the findings of SHAPER et al. in Uganda over the years 1950-65 and poses problems as to the hypothesis of a common aetiological process in both diseases, i.e. a hypersensitivity mechanism acting on cardiac connective tissue.

Adolescent↗

PubMed1976

The variety in endomyocardial fibrosis. A necropsy study.

In a necropsy series of 75 patients with endomyocardial fibrosis who died during the years 1967 to 1971 in Mulago Hospital, Kampala, the site of the lesions in the heart was studied alongside with the age, clinical history, geographical distribution, heart weight and concurrent diseases. Most frequently involved were the posterior cusp of the mitral valve, the left ventricular wall (upper-mid and/or apical region) and the right ventricular wall wheras othter localisations turned out to be relatively rare. Intracardial thrombi were also often described. Rheumatic heart disease was the most important concurrent cardiac disorder. The range and variety of the lesions is discussed together with relations to age, sex and geographical distribution of the patients. The results give support to Shaper's hypothesis concerning the aetiology of endomyocardial fibrosis; however many unanswered questions about the disease remain.

Adolescent↗

PubMed1989

Clinical course of endomyocardial fibrosis.

The survival pattern, morbidity, and clinical course of 145 patients with endomyocardial fibrosis who were followed up between November 1975 and June 1987 were studied. The diagnosis was confirmed in all cases by cardiac angiography, or echocardiography, or necropsy. Percentage survival at the end of one and 9.5 years was 76.11 and 26.35 respectively. History, physical examination, electrocardiography, and cardiac catheterisation were studied at the first presentation. The determinants of early mortality were studied by univariate Kaplan-Meier estimates compared by the log rank test and Cox proportional hazards multiple regression analysis. Significant univariate predictors of early mortality were QRS axis above +90 degrees, intraventricular conduction delay (QRS duration greater than 0.12 s), duration of symptoms before presentation, New York Heart Association functional classes III and IV, presence of embolic episodes, right atrial mean pressures greater than 20 mm Hg, right ventricular end diastolic pressure greater than 20 mm Hg, and aortic oxygen saturation less than 85%. The significant multivariate predictors of mortality were cyanosis, New York Heart Association functional class at first presentation, and right atrial mean pressure greater than 20 mm Hg. The bleak prognosis of endomyocardial fibrosis did not substantially improve despite advances in the medical management of congestive cardiac failure during the period of the study.

Adult↗

PubMed1984

[The course of endomyocardial fibrosis following surgical endocardial decortication].

Between 1971 and 1983 the authors observed 10 patients with left ventricular (n = 3) and biventricular (n = 7) endomyocardial fibrosis (7 women and 3 men). Seven of the 10 patients underwent open heart surgery with endocardial decortication of the left (n = 5) or left and right (n = 2) ventricle combined with mitral (n = 6) and tricuspid (n = 2) valve replacement. In 1 patient left ventricular endocardial decortication was performed without valve replacement. Three of the 10 patients were treated medically because functional limitation was only mild. One of the medically treated patients died 4 years later from congestive heart failure. Postoperative follow-up was 4.4 years. Two of the 7 patients who had undergone surgery died due to recurrence of endomyocardial fibrosis with blood eosinophilia of 46% (Löffler's endocarditis) in one, and due to severe left ventricular heart failure in the other. Annual mortality was 6.4%. NYHA classification was 3.4 pre- and 2.0 (p less than 0.005) postoperatively. Four patients were recatheterized 10 months after surgery: left ventricular end-diastolic pressure had decreased significantly from 24.6 to 13.6 mm Hg, cardiac index had increased slightly from 1.9 to 2.4 l/min/m2, left ventricular end-diastolic volume had increased from 69 to 84 ml/m2 (ns) and left ventricular ejection fraction remained unchanged pre- and postoperatively (59% and 57% respectively). It is concluded that endomyocardial fibrosis involves both ventricles in 70% of all patients, and that women are affected more frequently than men. Endocardial decortication with AV-valve replacement is regarded as the therapy of choice.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed2004

Tropical endomyocardial fibrosis (Davies' disease): case report demonstrating the role of magnetic resonance imaging.

Tropical endomyocardial fibrosis (TEMF), a restrictive cardiomyopathy of unclear etiology, is an endemic disease in equatorial Africa, South America and India. The patients are usually young, the onset of the disease and its clinical manifestations insidious, and the prognosis poor. We currently present a 50-year-old Congolese female who was referred with symptoms of progressive right-sided heart failure due to isolated TEMF of the right ventricle. Surgical resection of regional endomyocardial fibrosis was not possible and our patient was referred for cardiac transplantation. Cardiac magnetic resonance imaging (CMR) demonstrated the primary and secondary structural and functional abnormalities. CMR seems ideally suited to diagnose this condition and monitor response to medical and/or surgical therapy.

Cardiac Output, Low↗

PubMed2002

[Geographic origin of endomyocardial fibrosis treated at the central hospital of Maputo (Mozambique) between 1987 and 1999].

Endomyocardial Fibrosis (EMF) is a restrictive myocardiopathy of unknown aetiology, which is specific to tropical areas. In order to document the geographical distribution of this disease in Southern Mozambique, we reviewed the medical records of patients treated at Maputo Central Hospital between 1987 and 1999. Analysis was restricted to cases originating from the 3 provinces of Southern Mozambique. Cumulative incidences were computed for the period of 1987-1999, by age group, sex and residential district at disease onset. The geographical distribution of the disease in the 34 districts (administrative subdivisions) of the study area was mapped. Between 1987 and 1999, 118 cases of EMF originating from Maputo, Gaza and Inhambane provinces were diagnosed at Maputo Central Hospital. Seventy seven patients (65%) were living in Inhambane Province at the time of disease onset. Cumulative incidence for this province was 6.9/100,000 over the study period of 1987-1999. Comparatively, cumulative incidences were respectively 1.7/100,000 for Maputo and 1.0/100,000 for Gaza Province (chi 2 = 64.3, p < 10(-5)). All the patients were native from 22 out of the 34 districts of these 3 provinces. Affected districts were all located in the coastal area and higher cumulative incidence was computed for Inharrime district (18.3/100,000). This study confirmed that endomyocardial fibrosis is endemic in some areas of South Mozambique, but did not allow to measure the true magnitude of the disease which is probably grossly underestimated. The geographical distribution of EMF in South Mozambique reinforce the environmental hypothesis of aetiologic disease.

Adolescent↗

PubMed1998

Short report: endomyocardial fibrosis and cardiomyopathy in an area endemic for schistosomiasis.

An association of schistosomiasis and endomyocardial fibrosis or cardiomyopathy has been suggested. Clinical, stool, abdominal ultrasound, electrocardiogram, and Doppler echocardiogram examinations were performed on 226 patients from an area endemic for schistosomiasis. The diagnosis of schistosomiasis was made in 152 patients (69%) but clinical, electrocardiographic, or Doppler echocardiographic evidence of a schistosome cardiomyopathy or endomyocardial fibrosis could not be detected in any patient.

Abdomen↗

PubMed1990

[Pericardial disease erroneously diagnosed as endomyocardial fibrosis. Report of 2 cases].

Two patients who were sent to operation with diagnosis of endomyocardial fibrosis (EMF) turned out to have pericardial disease. EMF had been suspected on the basis of clinical history, electrocardiographic, radiologic and echocardiographic data. The hemodynamic studies were "typical", and included apex amputation, flat ventricular surface and mitral and tricuspid insufficiencies. Thus, ventriculography should not be considered specific, but rather suggestive of endomyocardial fibrosis.

Adult↗

PubMed1987

[Right ventricular endomyocardial fibrosis].

The clinical course, non-invasive and invasive diagnostic findings from a 50-year-old patient with endomyocardial fibrosis are reported. The patient died in cardiogenic shock. In Europe, endomyocardial fibrosis is a rare event. An exclusively right ventricular involvement is a seldom manifestation. The clinical, echocardiographic and computed tomographic features are diagnostic; therefore a satisfactory differentiation from other cardiac disorders with restrictive hemodynamic signs is possible. An echodense mass as cavity obliteration, with predominantly apical localization, was present at echocardiography; the computed tomography showed a hypodense area instead of the right ventricular cavity.

Echocardiography↗

PubMed1995

Endomyocardial fibrosis in Egypt: an illustrated review.

The detailed features of right sided endomyocardial fibrosis are described in 15 out of 10,000 consecutive patients who all had infection with Schistosoma mansoni and came from rural Egypt. Laboratory investigations, 12 lead electrocardiography, chest radiography, and Doppler echocardiography were performed in all patients. Cardiac catheterisation and angiography were performed in eight. Endomyocardial biopsy specimens were obtained from the right ventricles of two patients and pericardial biopsy specimens from two. Pericardiocentesis was performed in all patients. All patients were infected with S mansoni and had schistosomal hepatic fibrosis and ascites. Eleven had splenomegaly. All patients had raised cervical venous pressure with prominent Y descent and atrial fibrillation. Eosinophilia was notably absent. Echocardiography showed apical fibrosis in the right ventricle, obliteration of the ventricle, and moderate to massive exudative pericardial effusion in all patients. Calcification and fibrosis extended into the right ventricular outflow tracts in two patients. Huge right atrial thrombi occurred in five patients. Tricuspid regurgitation (grades I-II) was detected in 11 patients by Doppler ultrasonography. Haemodynamic and angiographic data confirmed the pure right sided restrictive pathophysiology. Pericardial biopsy specimens showed perivascular inflammatory infiltrates in two patients and a schistosomal granuloma in one. Endocardial biopsy specimens showed dense fibrosis with many fibroblasts. Endomyocardial fibrosis in Egypt is unique in several aspects. It always affected only the right side of the heart. Calcification and fibrosis extended to the right ventricular outflow tract. Pericardial inflammatory reaction was present. The relation to schistosomiasis and the link to periportal hepatic fibrosis in these patients is intriguing.

Adolescent↗

PubMed2000

[Endomyocardial fibrosis: report of 22 Congolese cases].

A study has been made of 22 cases of endomyocardial fibrosis (EMF) observed over a 12-year period. The epidemiological characteristics have been described, and the importance of the echocardiographic findings in the diagnosis of the disease has been emphasized. EMF constitutes 0.3% of the total admissions in the department, 0.9% of the cases of cardiac failure, and 3% of the cases involving subjects under 40 years old. The patient population consisted of 13 men and 9 women with an average age of 35.6 +/- 16.4 years (age range: 8.5-77 years). The diagnosis of EMF was based on clinical, radiological, electrocardiographic, and echocardiographic findings (22 cases), and surgical examination (1 case). In 8 cases, parasitosis (filariasis: the patients came from a known endemic area) with hypereosinophilia was observed. Three of these patients had associated high blood pressure. In 13 subjects, a severe right adiastolic syndrome was noted. Two patients presented with tachycardia, 2 others had signs of overall cardiac failure, and 5 subjects suffered from palpitations connected with arrhythmia. Thoracic X-ray showed cardiomegaly in all cases, lung involvement in 15 cases, normal lungs in 5 cases, and bilateral hilar stasis in 2 cases. Electrocardiography mainly showed endomyocardial fibrosis (15 cases), supraventricular arrhythmia, notably auricular fibrillation (13 cases), and conductive disorders (12 cases), which were frequently associated. Echocardiography showed the presence of EMF in 21 cases (95.5%). Doppler (n = 9 cases) detected tricuspid failure in 9 subjects, and mitral failure in 1 subjects. EMF was exclusively located on the right side in 19 cases, bilateral in 2 cases, and on the left in 1 case, which required surgery. Four patients died (i.e., 1 case of sudden death, 1 case of pulmonary embolism, 1 case of neurological coma, and 1 case of cardiac arrhythmia). The authors, like many others, note the clinical polymorphism of EMF, the predisposition to the disease caused by the presence of helminthiasis, which should be eradicated, the diagnostic value of echocardiography-Doppler, and the efficiency of surgery in the treatment of this condition.

Adolescent↗

PubMedDate not supplied

Recent trends in the surgical treatment of endomyocardial fibrosis.

Several modifications of the traditional treatment of endomyocardial fibrosis have been made based on a personal experience of 51 surgical cases and on the reports of others in the surgical literature during the last decade. Description of these techniques and the author's current concept of the pathological processes are reported herein.

Endocardium↗

PubMedDate not supplied

Pericardioperitoneal shunt for massive recurrent pericardial effusion in patients with endomyocardial fibrosis.

Massive recurrent pericardial effusion in patients with endomyocardial fibrosis presents a therapeutic challenge. Repeated pericardiocentesis and creation of a pericardial window is associated with significant morbidity and mortality in these critically ill patients. A new pericardioperitoneal shunt using a Holter valve and Harris catheter has been devised to solve some of these problems.

Adolescent↗

PubMed1989

A geochemical basis for endomyocardial fibrosis.

Analysis of hearts of patients dying from endomyocardial fibrosis showed a significantly greater concentration of cerium, a lanthanide present in leafy vegetables and root tubers, than in control hearts. This may be important in the pathogenesis of the disease and provides a geographical basis for the prevalence of the disease in the tropics.

Cerium↗

PubMed1993

Endomyocardial fibrosis in a scuba diving death.

A death of an amateur SCUBA diver with undiagnosed late phase endomyocardial fibrosis is reported. This 27-year-old man found himself in difficulties during a Sub-Aqua Club outing and drowned. Autopsy revealed features of drowning in addition to severe endomyocardial fibrosis (EMF) with collagenization of the left ventricle and interventricular septum with foci of chronic myocarditis. Other organs were essentially normal and toxicological studies were negative. Examination of the air tank revealed a normal oxygen circulation with elevation of the carbon dioxide content above recommended limits. Sudden ventricular arrhythmia underwater due to EMF is believed to have precipitated drowning. Stricter medical monitoring of amateur divers should prevent similar accidents in the future.

Adult↗

PubMed1984

[Echocardiographic, angiographic and hemodynamic development of surgically treated endomyocardial fibrosis].

The echocardiographic, angiographic and haemodynamic evolution of endomyocardial fibrosis (EMF) was assessed in 19 patients: 8 patients with isolated right sided EMF, 3 with predominantly right-sided EMF, and 8 with isolated left sided EMF with mitral regurgitation. Echocardiographic controls were preformed in 16 patients, on average 11.3 months after surgery; angiographic and haemodynamic controls were preformed in 14 patients, on average 14 months after surgery. In isolated or predominantly right-sided EMF, echocardiographic abnormalities of septal motion (paradoxal) disappeared. Although the volume of the right heart chambers decreased, they remained dilated. The syndrome of adiastole disappeared in patients operated early. In left EMF with mitral regurgitation, the M-shaped motion of the septum regressed in the 4 cases in which this abnormality was observed preoperatively. The size of the left heart chambers decreased on post-operative echo and angiographic studies. Left ventricular function returned to normal after surgery. The author underline the differences between right and left-sided EMF with mitral regurgitation. In right-sided EMF the evolution is that of adiastole and surgery should be preformed early because the right ventricular reserve is small. In left-sided EMF with mitral regurgitation, the evolution is that of a valvular lesion with a good postoperative result.

Adolescent↗

PubMed2006

[Endomyocardial fibrosis: a rare case of restrictive cardiomyopathy in a Caucasian female].

The authors report the case of endomyocardial fibrosis diagnosed in a young Caucasian female presenting with progressive congestive cardiac failure. The diagnosis was suspected on the echocardiographic, magnetic resonance imaging and cardiac catheterisation findings in association with the clinical presentation. After a short course of symptomatic medical therapy, the patient underwent the only curative treatment of this pathology, surgical endocardectomy and combined valvular surgery. The confirmation of the diagnosis was obtained a posteriori by histopathological examination of the operative findings which showed appearances of endomyocardial fibrosis similar to those observed in tropical regions. The patient was discharged on the eighth postoperative day, much improved clinically, and follow-up at one year was very satisfactory.

Adult↗

PubMed1977

Primary restrictive cardiomyopathy. Non-tropical endomyocardial fibrosis and hypereosinophilic heart disease.

Eleven patients with a restrictive cardiomyopathy are described. Seven of these had biventricular disease and in them the haemodynamic abnormality resembled that in constrictive pericarditis but the condition was distinguishable because of unequal involvement of the two ventricles, murmurs of atrioventricular valve regurgitation, or characteristic ventricular deformity on angiography. Two of these patients had eosinophilia with the clinical features of Löffer's endocarditis. In 4 patients the disease was apparently confined to the left ventricle; they were investigated on account of atypical chest pain, third heart sound, or abnormal electrocardiogram. Simultaneous measurements of left ventricular pressure and volume throughout diastole were made in 3 patients and showed rapid but abruptly curtailed left ventricular filling. Transvenous endomyocardial biopsy in 2 patients showed fibrous tissue with collagen and irregular elastic fibrils. Surgical biopsy in 3 patients excluded pericardial constriction but was diagnostically unhelpful because of failure to obtain endocardium. Necropsy in one patient showed that the heart had features indistinguishable from tropical endomyocardial fibrosis. It is suggested that the spectrum of ventricular abnormalities in these patients results from endomyocardial fibrosis of varying severity and probably of differing cause. This study has shown that "tropical" endomyocardial fibrosis may occur outside the tropics and suggests that eosinophilia may play a leading role or associated part in the genesis of some cases.

Adult↗