Surgical treatment of endomyocardial fibrosis: a new approach.

Endomyocardial fibrosis has been treated surgically for many years. For complete removal of fibrosis from both ventricles by the classic technique, each atrioventricular (AV) valve was removed and replaced with a prosthesis. Relapse of endomyocardial fibrosis has not been observed after surgical correction. Reoperations have been carried out because of complications of valve prostheses. A new surgical technique for removal of ventricular fibrous tissue with preservation of the mitral and tricuspid valves was used in nine consecutive patients with endomyocardial fibrosis. Initial results show a reduction of pulmonary hypertension, mean right and left atrial pressures and end-diastolic pressures in both ventricles. Tricuspid annuloplasty was performed in seven patients and mitral annuloplasty in five. No valve prosthesis was used. There was no death and New York Heart Association functional class improved from class III or IV in the preoperative period to class I or II in the postoperative period. These data suggest that resection of endocardial fibrous tissue can be indicated early in the clinical course and performed with preservation of the AV valves.

One-year follow-up of the effects of sildenafil on pulmonary arterial hypertension and veno-occlusive disease.

We hypothesized that chronic oral administration of the phosphodiesterase-5 inhibitor sildenafil could improve the exercise capacity and pulmonary hemodynamics in patients with pulmonary arterial hypertension (PAH) on the basis of previous short-term studies. We tested this hypothesis in 14 subjects with PAH, including seven patients with the idiopathic form and seven patients with atrial septal defects, but no other congenital heart abnormalities. Patients were subjected to a 6-min walk test and dyspnea was graded according to the Borg scale. Pulmonary flow and pressures were measured by Doppler echocardiography. Patients were given sildenafil, 75 mg orally three times a day, and followed up for 1 year. Sildenafil therapy resulted in the following changes: increase in the 6-min walk distance from a median value of 387 m (range 0 to 484 m) to 462 m (range 408 to 588 m; P < 0.01), improvement of the Borg dyspnea score from 4.0 (median value) to 3.0 (P < 0.01), and increased pulmonary flow (velocity-time integral) from a median value of 0.12 (range 0.08 to 0.25) to 0.23 (range 0.11 to 0.40; P < 0.01) with no changes in pulmonary pressures. In one patient with pulmonary veno-occlusive disease diagnosed by a lung biopsy, sildenafil had a better effect on the pulmonary wedge pressure than inhaled nitric oxide (15 and 29 mmHg, respectively, acute test). He walked 112 m at baseline and 408 m at one year. One patient died at 11 months of treatment. No other relevant events occurred. Thus, chronic administration of sildenafil improves the physical capacity of PAH patients and may be beneficial in selected cases of veno-occlusive disease.

Clinical predictors of prognosis in severe aortic stenosis in unoperated patients > or = 75 years of age.

In elderly patients with severe aortic stenosis, clinical evaluation can dictate decision making. Asymptomatic patients in normal sinus rhythm, without left atrial enlargement and without bundle branch block, can be safely followed clinically, regardless of echocardiographic findings.

Quality of life after cardiomyoplasty.

Cardiomyoplasty has recently been used as a surgical treatment for refractory heart failure, but its results have not been well described in quality-of-life patterns. We studied the quality of life of 14 patients (13 men, with a mean age of 43.3 +/- 7.4 years) submitted to this procedure for treatment of dilated or ischemic cardiomyopathies. They were approached by personal, structured interviews before and 13 +/- 9 months after the procedure, focusing on the following areas: physical activity, food and sleep patterns, working status, social activity, sexual activity, psychologic state, and perceptions and expectations about the treatment. The presence of limitation descriptors (discomfort, disability, and dissatisfaction) was recorded for all patients. The results showed an important decrease in limitation of physical activity, sleep pattern, social activity, and perceptions and expectations about the treatment. These findings suggest that cardiomyoplasty may improve the quality of life of a selected group of patients.

Correlation between Trypanosoma cruzi parasitism and myocardial inflammatory infiltrate in human chronic chagasic myocarditis: Light microscopy and immunohistochemical findings.

Trypanosoma cruzi parasites are only rarely identified in conventional histological sections of hearts from chronic chagasic patients. This finding suggests that T. cruzi plays no important direct role in the chronic myocarditis that accordingly has been considered mainly an autoimmune process. We reinvestigated this issue using a polyclonal anti-T. cruzi antibody serum to map immunohistochemically the T. cruzi antigen(s) in 9 different regions of 8 necropsy hearts and 24 septal fragments from 24 hearts from chronic chagasic patients. T. cruzi antigen(s) were identified in 7 (87%) of the 8 mapped hearts and in 14 (58%) of the 24 septal fragments. There was a statistically significant correlation between the presence of T. cruzi antigen(s) and moderate or severe inflammatory infiltrate (p = 0.005). When staining revealed amastigotes within intact myocardial fibers, there was no surrounding inflammatory infiltrate. However, when T. cruzi antigen(s) were found in macrophages either as amastigotes, diffusely in the macrophages cytoplasm, or free in the interstitium as round structures similar to amastigotes, there was a heavy inflammatory infiltrate. In the case in which no parasite was detected, a mild inflammatory infiltrate was present in the myocardium. Foci of fibrosis did not stain for T. cruzi antigen. These findings do not exclude a role of autoimmunity in chronic chagasic cardiopathy. However, the striking correlation between the presence of T. cruzi antigen(s) with the severity of site of the inflammatory infiltrate supports a direct role for the parasite in the perpetuation of myocardial inflammation in Chagas' disease. The destruction of microvessels and occasional endothelial cells with parasitism among dense inflammatory infiltrate favors the concept that microcirculatory injury, induced by T. cruzi, also contributes to the lesions of chronic Chagas' disease.

Improvements in routine xenodiagnosis with first instar Dipetalogaster maximus (Uhler 1894) (Triatominae).

First instar Dipetalogaster maximus proved as effective as third instar Triatoma infestans in detecting circulating Trypanosoma cruzi in patients with chronic infections examined in a routine xenodiagnostic laboratory. Since rearing costs are diminished, D. maximus has now replaced T. infestans as the xenodiagnostic agent in our laboratory. However, further work is needed to define the optimal conditions necessary when using this species in xenodiagnosis.

Management of aortic insufficiency in chronic aortic dissection.

From January 1980 to December 1988, 44 patients with chronic aortic dissection and aortic insufficiency underwent operation. This group of patients was analyzed to evaluate the outcome of those in whom the aortic valve was preserved compared with those having valve replacement. The overall preoperative characteristics of the two groups were similar except for the incidence of Marfan's syndrome. Valve replacement was the elected procedure in patients with valve degeneration or annuloaortic ectasia. In patients with leaflet prolapse with or without an enlarged annulus, a plastic procedure was used. In 48% of the patients, it was possible to preserve the valve. There were five hospital deaths (11%): three were due to low-output syndrome, one was due to bleeding, and one was due to neurological complications. There were two late deaths (5%). Follow-up of the 37 surviving patients ranged from 2 to 108 months (mean follow-up, 18 months). Seventy-eight percent of the survivors were in functional class I and the others were in class II. Two patients in whom the aortic valve was preserved had mild aortic insufficiency. Three patients with bioprostheses underwent reoperation because of prosthetic valve dysfunction. One patient who had aortoplasty and an aortic valve plastic procedure was seen with redissection and aortic insufficiency after 60 months and was reoperated on using the Bentall technique. The actuarial survival curves showed that patients who underwent valvoplasty had higher, but not significantly higher, survival rates than the valve replacement patients.(ABSTRACT TRUNCATED AT 250 WORDS)

A histological classification of mucocutaneous leishmaniasis in Brazil and its clinical evaluation.

Biopsies of skin and mucosal lesions were made on 60 well documented Brazilian patients with untreated cutaneous or mucocutaneous leishmaniasis, whose response to treatment was subsequently evaluated in 38 cases. The biopsies were examined with a view to classification after correlation with clinical and immunological findings. Although there was no simple or unified spectrum, five histological groups were defined and found to have some clinico-prognostic significance. In two groups the cases were all cutaneous with a relatively good prognosis. In another two groups they were evolving as mucocutaneous with a poor prognosis. The fifth group showed mixed characteristics with a tendency to relapse. There was no strong correlation with serum antibodies or Montenegro skin test, which were usually positive, or with parasite load, which was always low. The tissue response was distinguished from that in oriental sore by the degree of connective tissue involvement in all groups. It was the primary response in two groups, and subsidiary to a mono-nuclear response in the others. It suggested damage due to extra-cellular parasites or immune complexes. It did not correlate with the distinction between cutaneous and mucocutaneous disease. The single, most favourable, prognostic feature in either the cellular or connective tissue component was necrosis with a reactive response.

Nifurtimox in the treatment of South American leishmaniasis.

A trial of Nifurtimox (Lampit) in 26 patients with mucocutaneous leishmaniasis is reported. 13 patients with cutaneous lesions and 13 patients with mucosal disease were treated with a daily oral divided dose of 10 mg/kg body-weight for 30 days. 46% of the cutaneous cases and only 15% of the mucosal cases apparently responded to this regimen during at least one year of follow up. The difficulties of assessing cure in this disease are briefly discussed. We consider that Nifurtimox remains an investigational drug. While possibly exhibiting some anti-leishmanial activity it cannot be recommended for routine use in either form of the disease.

Dipetalogaster maximus (Hemiptera, Triatominae) for xenodiagnosis of patients with serologically detectable Trypanosoma cruzi infection.

In patients serologically positive for Trypanosoma cruzi infection the three bug species/instar combinations used in xenodiagnosis showed third-instar Dipetalogaster maximus to be more efficient in detecting circulating trypanosomes than the first instar of the same species which, in turn, is more sensitive than third-instar Triatoma infestans. The sensitivity of the pool technique of faecal examination compared with individual dissection was investigated. Four pool examinations (the product of 20 bugs) were equivalent to 10 individual bug dissections. Because of the ease of providing large numbers of bugs for mass xenodiagnosis, first-instar D. maximus has replaced third-instar T. infestans in our routine work. The value of third-instar D. maximus as a xenodiagnostic agent needs further investigation.

A focus of mucocutaneous leishmaniasis in Três Braços, Bahia, Brazil: characterization and identification of Leishmania stocks isolated from man and dogs.

The characterization and identification to species and subspecies of 20 stocks of Leishmania isolated from the region of Três Braços, Bahia, Brazil, are described: 17 stocks were from patients and three from dogs. The following techniques were used (i) biological (growth in culture, hamster tissues and phlebotomine gut), (ii) biochemical (isoenzyme and kinetoplast DNA analysis) and (iii) immunological (using monoclonal antibodies). All except two stocks belong to the L. braziliensis complex. One of these two corresponded to L. mexicana amazonensis but the other, while clearly in the mexicana complex, showed slight differences from the L. mexicana amazonensis reference strain on isoenzyme analysis. Two stocks from different lesions in the same patient and with different growth characteristics in hamster tissues were both identified as L. braziliensis braziliensis. All the fully characterized stocks of the L. braziliensis complex were identified as L. braziliensis braziliensis. L. braziliensis guyanensis was not identified. Dog and human stocks of L. braziliensis braziliensis were indistinguishable. From these findings and other evidence, L. braziliensis braziliensis seems to be the predominant species transmitted in Três Braços.

Long-term follow-up of patients with Leishmania (Viannia) braziliensis infection and treated with Glucantime.

Seventy-nine patients with cutaneous (62) or mucosal (17) infection with Leishmania (Viannia) braziliensis in Três Braços, Bahia, Brazil, were followed for at least 4 years after initiating treatment with antimony. Cutaneous relapses occurred in 6/62 (10%), mucosal relapse after cutaneous infection in 2/62 (3%), and mucosal relapse after mucosal disease in 2/17 (17%). It is concluded that relapse (cutaneous and mucosal) is rare after adequate antimony therapy and that no definite prediction of relapse (clinical, serological or by skin reaction) is possible.

The role of active myocarditis in the development of heart failure in chronic Chagas' disease: a study based on endomyocardial biopsies.

The authors analyze the presence of active myocarditis in endomyocardial biopsies from 38 patients with chronic Chagas' disease diagnosed serologically. The patients were divided into three clinical groups of increasing severity. Group I: 13 patients with normal electrocardiograms, normal chest x-rays, and no symptoms; Group II: 13 patients with abnormal electrocardiograms and no cardiomegaly; and Group III: 12 patients with abnormal electrocardiograms, cardiomegaly and heart failure. In order to diagnose myocarditis activity, two sets of criteria were used: one mainly observing histopathologic aspects of inflammatory cells aggressing cardiac fibers; and the other counting the mean number of lymphocytes per high power microscopic field. The results of both methods showed a higher incidence of active myocarditis in the clinical group with heart failure. The present report clearly shows the important role played by activity of myocarditis in the development of heart failure in chronic Chagas' disease. Therefore, the possibility of using drugs to control early stages of the activity of the inflammatory process is suggested. On the other hand, endomyocardial biopsy (EMB) seems to be an adequate method to evaluate the intensity of the cardiac inflammatory process in Chagas' heart disease.

[Acute Chagas' disease: transmission routes, clinical aspects and response to specific therapy in diagnosed cases in an urban center]. / Doença de Chagas aguda: vias de transmissão, aspectos clínicos e resposta à terapêutica específica em casos diagnosticados em um centro urbano.

The authors report clinical features and therapeutic response of 24 outpatients with acute Chagas' disease, and 3 in the initial chronic phase, referred to the Clinic for Infectious and Parasitic Diseases of the FMUSP "Clínicas" Hospital between 1974 and 1987. The following transmission routes were involved: triatominae in 7 cases, blood transfusion in 9, kidney transplantation and/or blood transfusion in 4, accidental in 1, oral route in 3, probably breast feeding in 1, congenital or breast feeding in 1, and congenital or blood transfusion in 1. Six patients infected by triatominac acquired the disease between 1974 and 1980 and one in 1987. The blood transfusion infected patients acquired the disease in Greater São Paulo, seven of whom after 1983. The acute phase Chagas' disease was oligosymptomatic in 4 patients: three of such patients being immunocompromised by drugs or other diseases. Another two adult immunocompromised patients developed myocarditis and congestive heart failure. Clinical features were severe in 5 from 6 children under two years, irrespective of the transmission route. Evaluation of the acute phase patients treated with benznidazol (4-10 mg/kg/day) showed: therapeutic failure in 4/16 (25.0%); possible cure in 9/16 (53.2%) and inconclusive results in 3/16 (18.8%). The antibody and complement-mediated lysis reaction was in keeping with the xenodiagnosis in 18/22 cases, having shown negative results after treatment earlier than classical serological reactions. One aplastic anaemia patient receiving corticosteroid presented lymphoproliferative disease 6 years after being treated with benznidazol for acute Chagas' disease.

[Beriberi heart disease]. / Beribéri cardíaco.

A man with chronic alcoholism presenting with recent-onset congestive heart failure and hyperdynamic features. Low arteriovenous oxygen content difference suggested high cardiac output. After 10 days of alcohol withdrawal, rest and balanced feeding the patient was asymptomatic and cardiomegaly subsided. No supplementation of thiamine was required.

[Mitral valve insufficiency caused by endomyocardial fibrosis of the left ventricle. A case report]. / Insuficiência da valva mitral por endomiocardiofibrose do ventrículo esquerdo. Relato de caso.

A 38-year old female patient, who had asymptomatic heart disease for seven years, developed progressive and fatal heart failure in eight months. The diagnosis of mitral valve insufficiency was made five months before death. The necropsy revealed that the etiology of mitral insufficiency was isolated left ventricle endomyocardial fibrosis with unusual involvement of the posterior cusp of the mitral valve.

[Evaluation of aortic insufficiency in aortic dissection. Significance of intraoperative echocardiography on the conservation of the valve. A case report]. / Avaliação da insuficiência aórtica na dissecção de aorta. Importância da ecocardiografia intraoperatória na conservação da valva. Relato de caso.

A 34-year old man presented with dyspnea and a new murmur of aortic regurgitation (AR). Two months before he had a episode of acute chest pain. The diagnosis of type A chronic aortic dissection was done on the basis of clinical signs and digital angiography. At surgery, intraoperative two-dimensional echocardiography (Iop Echo) showed a large intimal flap prolapsing into the leaflets during diastole causing AR, without primary involvement of the aortic valve. The aorta was transected just above the valve commissures and a 30 mm woven graft was sutured end to end. The false lumen was closed distally and incorporated into the graft-aorta suture line. Valve replacement was not performed. After the surgical procedure, a Iop Echo indicated competence of the aortic valve by means of contrast injection in the aortic root. AR due to the interference of an intimal flap with the aortic leaflets was not yet been reported. To our knowledge, this is the first case of this mechanism and illustrates the potential value of Iop Echo in diagnosis of aortic dissection.

[Pericardial disease erroneously diagnosed as endomyocardial fibrosis. Report of 2 cases]. / Pericardiopatia com falso diagnóstico de endomiocardiofibrose. Relato de dois casos.

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.

[Malnutrition in dilated cardiomyopathy. Correlation with echocardiographic indices of left ventricular function]. / Desnutrição na miocardiopatia dilatada. Correlação com índices ecocardiográficos da função ventricular esquerda.

PURPOSE: To evaluate the incidence of severe protein-calorie malnutrition in patients with dilated cardiomyopathy (DC), and its correlation with left ventricular contractility. METHODS: Group A--51 patients with DC in decompensated congestive heart failure class III or IV, 36 men, aged 51.9 +/- 15.6 years. Group B--25 patients admitted for elective myocardial revascularization with normal LV contractility, 20 men, aged 57.2 +/- 10.5 years. Tricipital skinfold thickness (TS) and mid-arm muscle circumference (MAMC) were obtained in all patients. Severe protein-calorie malnutrition was defined when both measurements were below the fifth populational percentile (Frisancho tables). In Group Am the echocardiographic left ventricular (LV) diastolic diameter (DD), ejection fraction (EF) and systolic volume (SV) were obtained. Those LV parameters were compared between DC patients with and without severe malnutrition. Correlation analysis were performed between TS, MAMC and LV DD, EF, and SV, in the patients of Group A. RESULTS: Severe malnutrition occurred in 7/51 (13.7%) of Group A, and none in Group B. TS values were of 8.90 +/- 4.47 cm in Gr. A and 23.48 +/- 8.52 in B (p < 0.001). MAMC measured 22.25 +/- 3.13 cm in Gr. A and 23.58 +/- 8.52 in B (p = 0.03), LVEF was of 36.29 +/- 9.43% in severe malnutrition patients and of 37.84 +/- 9.78 in the other patients of Group A (p = 0.70). Conversely, LVDD was of 70.90 +/- 11.3 mm vs. 70.75 +/- 8.54 mm (p = 0.98), and LVSV was of 113.0 +/- 52.7 ml vs. 137.6 +/- 56.8 (p = 0.45), when compared severe malnutrition with the rest of patients of Group A. No correlation was found between TS and MAMC and LV, EF, DD and SV in Group A. CONCLUSION: Severe malnutrition was frequent in patients with DC and heart failure. TS measurements, reflecting caloric reserves, were more affected. Echocardiographic parameters of LV function did not correlate with nutritional status.

Effects of chlorthalidone and diltiazem on myocardial ischemia in elderly patients with hypertension and coronary artery disease.

OBJECTIVE: Antihypertensive therapy with thiazides decreases coronary events in elderly patients. However, the influence of diuretics on myocardial ischemia has not been fully investigated. The aim of this study was to compare the effect of chlorthalidone and diltiazem on myocardial ischemia. METHODS: Following a randomized, double-blind, crossover protocol, we studied 15 elderly hypertensive patients aged 73.6+/-4.6 years with myocardial ischemia. All patients had angiographically documented coronary artery disease. We measured patients using 48- hour ambulatory electrocardiogram monitoring and exercise testing. After a 2-week period using placebo, patients received chlorthalidone or diltiazem for 4 weeks. RESULTS: Both treatments lowered systolic and diastolic blood pressures. The number of ischemic episodes on ambulatory electrocardiogram recordings was reduced with the use of chlorthalidone (2.5+/-3.8) and diltiazem (3.2+/-4.2) when compared with placebo (7.9+/-8.8; p<0.05). The total duration of ischemic episodes was reduced in both treatments when compared with placebo (chlorthalidone: 19.2+/-31.9min; diltiazem: 19.3+/-29.6min; placebo: 46.1+/-55.3min; p<0.05). CONCLUSION: In elderly hypertensive patients with coronary artery disease, chlorthalidone reduced myocardial ischemia similarly to diltiazem. This result is consistent with epidemiological studies and suggests that reduction of arterial blood pressure with thiazide therapy plays an important role in decreasing myocardial ischemia.