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Successful surgical correction was accomplished in a 12-year-old boy with proved endomyocardial fibrosis. Pathological features and the role of surgical therapy in the management of this lesion are discussed. This report presents one of the youngest patients who has undergone endocardiectomy and valve replacement for right-sided endomyocardial fibrosis.
Left ventricular endomyocardial fibrosis accompanied by severe mitral insufficiency occurring in 4 adolescent African patients is reported. Mitral valve repair was successfully performed in all 4 cases including annuloplasty in 3 and chordal shortening in one. Short-term follow-up of up to 10 months indicated substantial clinical improvement and decrease of the cardiothoracic ratio in all. It is emphasized that mitral valve repair appears to be the method of choice in treating mitral incompetence in the localized form of endomyocardial fibrosis, where endocardiectomy is not required in the area of the papillary muscles.
19 cases of endomyocardial fibrosis were studied. Angiocardiography localises the site of fibrosis and seems to be the best diagnostic method. All cases in this series had left ventricular involvement which resulted in changes of the silhouette (square, polylobulated or deformed like the shape of a heart on a playing card) and of the ventricular contour (smooth, lacunar or "doubled"). Ten patients had mitral incompetence. The ejection fraction was normal in 8 patients but significantly reduced in the series as a whole (EF = 0,56, p less than 0,05). 15 patients had right ventricular involvement. Apart from the smooth contour of the anterior wall, the only abnormality in the mild cases, the most suggestive feature was an amputation of the ventricular apex, giving rise to a ventricular appearance of a narrow, akinetic (apart from the infundibular region) tube. The catheter data demonstrated the haemodynamic changes due to the fibrosis. A constrictive syndrome was observed in all the severe poorly tolerated cases. This was not apparent under basal conditions in milder cases. The value of pharmacodynamic testing and endomyocardial biopsy in cases where the diagnosis is uncertain should be stressed. The results of resection of the fibrosis and valvular replacement in severe cases depend to a large extent on the degree of myocardial involvement.
This is the report of a rare case of endomyocardial fibrosis associated with massive calcification of the left ventricle in a male patient with dyspnea on great exertion, which began 5 years earlier and rapidly evolved. Due to lack of information and the absence of clinical signs that could characterize impairment of other organs, the case was initially managed as a disease with a pulmonary origin. With the evolution of the disease and in the presence of radiological images of heterogeneous opacification in the projection of the left ventricle, the diagnostic hypothesis of endomyocardial disease was established. This hypothesis was later confirmed on chest computed tomography. The patient died on the 16th day of the hospital stay, probably because of lack of myocardial reserve, with clinical findings of refractory heart failure, possibly aggravated by pulmonary infection. This shows that a rare disease such as endomyocardial fibrosis associated with massive calcification of the left ventricle may be suspected on a simple chest X-ray and confirmed by computed tomography.
OBJECTIVE: To assess whether exercise capacity is related to left atrial dimension (LAD) in patients with biventricular endomyocardial fibrosis. METHODS: This study comprised 38 patients in sinus rhythm, with a mean age of 37.5 +/- 11.5 years (range, 11 to 59 years), 25 of whom were women. They were divided into 2 groups according to the NYHA functional class on hospital admission as follows: group A (12 patients) and group B (26 patients). All patients underwent cardiopulmonary exercise testing to determine their maximum oxygen consumption (VO2 max), and their left atrial dimension was determined on echocardiography. RESULTS: The VO2max values for groups A and B were 21.8 +/- 4.8 mL.kg(-1).min(-1) and 13.7 +/- 3.5 mL.kg(-1).min(-1), respectively, and the left atrial dimensions were 3.7 +/- 0.7 cm and 4.4 +/- 0.7 cm for groups A and B, respectively. A significant and inverse correlation was found between VO2max and LAD in the groups studied. CONCLUSION: The increase in left atrial dimension is associated with impairment in exercise capacity in patients with endomyocardial fibrosis. Our findings support the use of left atrial dimension to estimate a more complex and difficult to assess index of functional capacity, such as VO2max.
The authors have conducted research on conduction disturbances in the endomyocardial fibrosis, synonymous with chronic parietal endocarditis, about 170 cases at the Institute of Cardiology in Abidjan, from January 1977 to June 1991. The anatomical and/or angiographic examination have permitted to describe 64 cases of right fibrosis, 24 cases of left fibrosis and 82 cases of bilateral fibrosis. Conduction anomalies have been observed among 42.9% of the patients. Among 92 anomalies recorded, the most frequent has been the first degree heart block (43.5%) and the incomplete right bundle branch block (30.4%). The old age of the patients and the right localization were the factors associated with conduction disturbances in endomyocardial fibrosis (difference not significant). Yet, fibrosis surgery, especially the decortication of the fibrous endocardium of the right ventricle, have generated one or many conduction anomalies among most of our operated patients. The right branch of the fasciculus of the His has been the most injured by the fragmented techniques of METRAS who had, therefore, the merit to have minimized the incidence of the complete post-operative heart blocks still high in European and Brazilian series. In term of prognosis, no conduction disturbance has directly caused a patient's death, even if those anomalies cannot be totally ruled out in the 16 cases of sudden death.
A case of endomyocardial fibrosis with intramyocardial calcification was characterized by symptoms of restrictive cardiomyopathy resembling constrictive pericarditis. Pathological findings were typical of those found in classical Davies' disease, originally encountered and described in Uganda.
One-and-a-half ventricle repair, consisting of endocardiectomy with tricuspid valve replacement and bidirectional cavopulmonary shunt, was performed on a patient with right ventricular endomyocardial fibrosis and right ventricular outflow tract obstruction. The patient made a smooth recovery. We believe that this repair provides good palliation for a subset of patients with right ventricular endomyocardial fibrosis.
The case reported in this paper, besides illustrating many of the clinical and pathological aspects of chronic dermatomyositis, documents a yet undescribed feature of involvement of the heart in dermatomyositis, namely endomyocardial fibrosis. The pathogenetic mechanism of such a happening is discussed, and its possible relationship to tropical endomyocardial fibrosis is considered.
A 26 year old Saudi man with features of both Loeffer's endocarditis and endomyocardial fibrosis presented with mild symptoms and pulmonary emboli. Echocardiographic examination showed obliteration of the right ventricular apex by an attached mass. The results of haemodynamic studies were somewhat abnormal and medical treatment was started. Despite anticoagulation with warfarin the patient's condition deteriorated rapidly over a four month period after a further episode of pulmonary embolism and the development of pulmonary hypertension. Two haemodynamic studies performed four months apart were typical of pulmonary hypertension and later right ventricular failure; they showed none of the characteristics of restriction. Pulmonary embolectomy was attempted but there was no cleavage plane between the organised thrombi and the endothelium of the pulmonary artery. The patient died of severe pulmonary hypertension and right ventricular failure several days after operation. Surgical intervention in the early stages of right-sided endomyocardial fibrosis might have prevented the development of pulmonary embolism and pulmonary hypertension.
The studies leading up to the unitarian concept suggesting that endomyocardial fibrosis, described in the tropics, and Löffler's endocarditis parietalis fibroplastica (Löffler's endomyocardial disease), described in the temperate zone, belong to the same disease spectrum are detailed. Evidence that the eosinophil is involved in the pathogenesis of endomyocardial diseases, irrespective of the geographical origin, is presented. The findings of a similar abnormality in these cells obtained from patients in the tropical and temperate zones are briefly mentioned. In view of the evidence it is proposed that endomyocardial disease, associated with abnormal eosinophils, hitherto classified under the restrictive type of cardiomyopathy, be removed from this classification and reclassified under 'specific heart muscle disease'.
Massive endocardial calcific deposits in the left ventricular chamber is such an uncommon lesion that only three cases have been reported previously. This report describes an apparently asymptomatic patient with endomyocardial fibrosis and massive endocardial calcific deposits. The results of electrocardiography, echocardiography, angiography, and endomyocardial biopsy findings are presented, and the pathogenesis of massive endocardial calcification is speculated upon. The interesting point is that the clinical history and manifestation of this patient suggests that endomyocardial fibrosis may occur in a benign form and long-term survival may be expected.
A case of right ventricular endomyocardial fibrosis simulating Ebstein's anomaly is described. The clinical features, chest x-ray, electrocardiogram, echocardiogram, intracardiac electrogram, and the angiogram were all compatible with Ebstein's malformation. A correct diagnosis was subsequently made in the operating room. Reasons for the difficulties in the differential diagnoses of these two entities are discussed.
Controversial aspects of, and recent advances in endomyocardial fibrosis are reviewed. Available data indicate that the disease has a worldwide distribution but is most prevalent in tropical Africa. Several factors have been incriminated in different parts of the world in its aetiopathogenesis but in tropical Africa subclinical viral myocarditis and eosinophilia caused by parasitic infections appear to be the most likely causes. Despite diversity in its aetiology, however, the clinicopathological manifestations are fairly uniform. This has facilitated the recognition of characteristic electrocardiographic and echocardiographic signs as well as the use of similar surgical techniques for its treatment in different countries.
OBJECTIVE: To identify life expectancy after surgery for endomyocardial fibrosis (EMF) and the events that influence it. METHODS: Eighty-three patients with EMF underwent endocardial decortication and atrioventricular valve replacement or repair, between December 1977 and December 1997. There were 66 (79.6%) female and 17 (20.4%) male patients, ranging in age from 4 to 59 years (mean, 31). Thirty-seven (44.5%) had biventricular disease, 34 (41.0%) had disease of the right ventricle alone and 12 (14.5%) had EMF confined to the left ventricle. All were in functional class III or IV (New York Heart Association classification). RESULTS: Sixty-eight (81.9%) patients survived the operation and were followed up for periods ranging from 2 months to 17 years. The total follow-up time was 6290 patient/months (mean, 92 months). There were 15 late deaths, but in six, the cause was not related to the underlying disease. Four (5.8%) patients presented recurrence of the fibrosis and were reoperated on and in six (8.8%), EMF appeared in the other ventricle. Five (7.3%) patients were reoperated on to replace either a valve prosthesis or a native valve which had been preserved during the first procedure. Only 24 (45%) of the 53 surviving patients are in functional class I or II. The actuarial probability of survival at 17 years, including operative mortality, was 55%. CONCLUSION: Surgical treatment of EMF should be considered a palliative procedure because surgery does not alter the progressive nature of the disease. However, surgical therapy is recommended for patients with EMF and heart failure as it is their only hope of survival.
The authors have studied rhythmic events happened during early post-operative period in 84 operated patients for endomyocardial fibrosis, synonym nowadays to chronic parietal endocarditis, in the Institute of Cardiology of Abidjan (Côte d'lvoire), from January 1977 to July 1991. The mean age has been 15.6 +/- 43 years old. Endocardectomy was left in 25 cases, right in 32 and at last bilateral in the 19 others. The surgical way has been a left and/or right atriotomy. Mitral and/ ortricuspide valvular surgery has been always realised. The absolute prevalence of patients having presented one or several arrhythmias, atrial tachycardia and premature ventricular beats. The favouring factors have been the period of exclusion of the aortic root from circulation and number of endomyocardectomy. Atrial fibrillation has been related to auricular volume. Most of nodal arrhythmias have appeared with surgery. Endomyocardial fibrosis surgery has been greatful in rhythmic plan in 16% of patients. Early post-operative arrhythmias have been responsible forone case of the death of the operated patients and they have been present in seven other cases of the death.
Early right ventricular failure following a large endocardiectomy in right endomyocardial fibrosis (EMF) is reported. This diagnosis was confirmed upon echocardiography by the presence of a dilated right ventricle, and hemodynamically (upon postoperative control catheterization) by the presence of right ventricular diastolic dysfunction. The role of the endocardiectomy and of the pericardium in the genesis of this ventricular dysfunction are discussed in the light of this case which was documented by a hemodynamic study and surgically confirmed.
Serial electrocardiographic changes, mostly of a minor nature, occurred in two Zambians with endomyocardial fibrosis.