We describe 10 children with endomyocardial fibrosis who underwent surgical treatment between 1978 and 1999. Seven were male and 3 female, with an age range from 4 to 15 years, having a mean age of 11 years. All were in the final stage of heart failure. Three had biventricular disease, 6 had involvement of the right ventricle alone, and one had endomyocardial fibrosis confined to the left ventricle. There were 3 deaths (30%) in the postoperative period due to low cardiac output. The 7 survivors were followed up for a period ranging from 12 to 168 months, with a mean of 72 months. Two late deaths have occurred resulting from heart failure and infectious endocarditis. Five (50%) children are still alive. Two required 3 reoperations for dysfunction of the inserted valvar prosthesis. One patient is in functional Class IV, and 4 are in Class II to III, despite intensive medical treatment. It is concluded that surgery for endomyocardial fibrosis is an essentially palliative procedure and, especially in children, the results of surgical treatment leave much to be desired.
SummaryThis study aimed to assess the prevalence of “silent” valvar regurgitation in healthy Brazilian children; to determine whether variations in the gain settings of the machine interfered with its documentation; and to compare Doppler color flow mapping with the pulsed wave Doppler. Twenty-five healthy children were selected randomly. Echocardiographic equipment used was ALOKA 870. Fixed parameters were 3.5 MHz transducer, 400 Hz filter, reject of 7/14 and aperture of 30/80. Variable parameters were Doppler color flow mapping and pulsed wave Doppler gains. A protocol for evaluation of each cardiac valve was developed. Regurgitation was noted in 93% for the pulmonary valve, 74% for the tricuspid valve and 27% for the mitral valve. In the aortic valve, jets suggestive of mild valvar regurgitation were observed with the Doppler color flow mapping technique in two children but these findings were not corroborated by pulsed wave Doppler. Only in the aortic valve were findings from Doppler color flow mapping not in agreement with those from pulsed wave Doppler. Variations in the settings for gain did not interfere significantly in the documentation of regurgitation. Inter-observer variations were not significant (two independent operators). The clinical implications of these findings are yet to be established. Labeling these regurgitant jets as “normal” may be oversimplifying their meaning and perhaps overlooking the possibility of predisposition to endocarditis or progression of the valvar abnormality. Conversely, labeling them as “abnormal” can generate a more serious problem, which is that of iatrogenic heart disease. Prospective follow-up studies should be carried out in these subjects.
We describe an eleven-year-old boy with severe retardation in physical growth and severe rheumatic valvar disease. At surgery, a sample of the atrial appendage showed Aschoff nodules, although no antecedent of rheumatic fever was recorded. The patient was HLA-DR5 positive. The case emphasizes the strong association of malnutrition with severe rheumatic heart disease in northeast Brazil.
Endomyocardial fibrosis (EMF) is a severe restrictive cardiomyopathy characterized by the formation of fibrous tissue on the endocardium and to a lesser extent in the myocardium of one or both ventricles. The disease results in endocardial rigidity, atrioventricular valve dysfunction and progressive obliteration of the ventricular cavity. The clinical and pathological findings of EMF have been well established and definitive diagnosis can be made by selective cineangiocardiography. However, there are few reports concerning the phonomechanocardiographic (PMCG) patterns in this condition. In this paper we describe the PMCG findings in 12 patients with EMF which is now recognized with increasing frequency in the Northeast of Brazil.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.