Abstract Background Juvenile systemic lupus erythematosus (JSLE) is a chronic inflammatory disease that affects the heart in 50% of cases. The behavior of diastolic function in adolescents and the predictors of its occurrence by conventional echocardiography are poorly established. Objectives This study aimed to evaluate diastolic function in adolescents with JSLE and to identify possible predictors of its occurrence by conventional echocardiography. Methods Cross-sectional, observational, control group study in a tertiary hospital of 49 adolescents with JSLE and 49 controls, using the EACVI 2016 guideline classification. Statistical methods used were Fisher and Mann-Whitney tests. Multivariate logistic regression models were constructed. A significance level of 5% was adopted. Results Among 98 patients, the JSLE group had higher indexed left atrial volume (p <0.001), lower lateral E' value (p<0.001) and lower E/A ratio value (p<0.001). The diagnosis of JSLE was associated with a higher chance of increased left atrial index volume (OR 3.3; p value 0.03). Conclusions Based on the 2016 guideline, no diastolic dysfunction was found in JSLE. However, differences in the analyzed echocardiographic parameters were found in these adolescents.

Resumo Fundamentos: A cardiomiopatia hipertrófica (CMH) é a doença cardíaca de origem genética mais frequente na população mundial, com prevalência de, pelo menos, 1/500. A associação com hipertensão arterial sistêmica (HAS) não é incomum, uma vez que esta acomete aproximadamente 25% da população mundial. A maioria dos estudos objetiva o diagnóstico diferencial entre essas doenças, mas pouco se sabe sobre a magnitude dessa associação. Objetivo: Comparar o strain longitudinal global (SLG) do ventrículo esquerdo em pacientes portadores de CMH com e sem HAS associada. Métodos: Estudo transversal retrospectivo que incluiu 45 pacientes portadores de CMH e fração de ejeção preservada, com diagnóstico confirmado por ressonância magnética, sendo 14 hipertensos. Realizada avaliação ecocardiográfica transtorácica com ênfase na análise da deformação miocárdica do ventrículo esquerdo por meio do SLG. Valores de p < 0,05 foram considerados estatisticamente significativos. Resultados: A deformação do ventrículo esquerdo foi significativamente menor nos hipertensos quando comparada aos normotensos (-10,29 ± 2,46 vs. -12,35% ± 3,55%, p = 0,0303), indicando maior comprometimento da função ventricular naquele grupo. A média de idade também foi significativamente maior nos hipertensos (56,1 ± 13,9 vs. 40,2 ± 12,7 anos, p = 0,0001). A disfunção diastólica foi melhor caracterizada nos pacientes hipertensos (p = 0,0242). Conclusão: A deformação miocárdica foi significativamente menor no grupo de pacientes com CMH e HAS, sugerindo maior comprometimento da função ventricular. Esse achado pode estar relacionado a um pior prognóstico com evolução precoce para insuficiência cardíaca. Estudos prospectivos são necessários para confirmar essa hipótese.

Abstract Background: Hypertrophic cardiomyopathy (HCM) is the most common heart disease of genetic origin in the world population, with a prevalence of at least 1/500. The association with systemic arterial hypertension (SAH) is not uncommon, as it affects approximately 25% of the world population. Most studies aim at the differential diagnosis between these diseases, but little is known about the magnitude of this association. Objective: To compare left ventricular global longitudinal strain (GLS) in HCM patients with and without associated SAH. Methods: Retrospective cross-sectional study that included 45 patients with HCM and preserved ejection fraction, with diagnosis confirmed by magnetic resonance imaging, including 14 hypertensive patients. Transthoracic echocardiography was performed, with emphasis on left ventricular myocardial strain analysis using GLS. In this study, p < 0.05 was considered statistically significant. Results: Left ventricular strain was significantly lower in hypertensive individuals compared to normotensive individuals (-10.29 ± 2.46 vs. -12.35% ± 3.55%, p = 0.0303), indicating greater impairment of ventricular function in that group. Mean age was also significantly higher in hypertensive patients (56.1 ± 13.9 vs. 40.2 ± 12.7 years, p = 0.0001). Diastolic dysfunction was better characterized in hypertensive patients (p = 0.0242). Conclusion: Myocardial strain was significantly lower in the group of patients with HCM and SAH, suggesting greater impairment of ventricular function. This finding may be related to a worse prognosis with early evolution to heart failure. Prospective studies are required to confirm this hypothesis.

Abstract INTRODUCTION: Infective endocarditis (IE) is a systemic infectious disease requiring a multidisciplinary team for treatment. This study presents the epidemiological and clinical data of 73 cases of IE in Rio de Janeiro, Brazil. METHODS This observational prospective cohort study of endocarditis patients during an eight-year study period described 73 episodes of IE in 70 patients (three had IE twice). Community-associated (CAIE) and healthcare-acquired infective endocarditis (HAIE) were diagnosed according to the modified Duke criteria. The collected data included demographic, epidemiologic, and clinical characteristics, including results of blood cultures, echocardiographic findings, surgical interventions, and outcome. RESULTS: Analysis of data from the eight-year study period and 73 cases (70 patients) of IE showed a mean age of 46 years (SD=2.5 years; 1-84 years) and that 65.7% were male patients. The prevalence of CAIE and HAIE was 32.9% and 67.1%, respectively. Staphylococcus aureus (30.1%), Enterococcus spp. (19.1%), and Streptococcus spp. (15.0%) were the prevalent microorganisms. The relevant signals and symptoms were fever (97.2%; mean 38.6 + 0.05°C) and heart murmur (87.6%). Vegetations were observed in the mitral (41.1%) and aortic (27.4%) valves. The mortality rate of the cases was 47.9%. CONCLUSIONS: In multivariate analysis, chronic renal failure (relative risk [RR]= 1.60; 95% confidence interval [CI] 1.01-2.55), septic shock (RR= 2.19; 95% CI 1.499-3.22), and age over 60 years (RR= 2.28; 95% CI 1.44-3.59) were indirectly associated with in-hospital mortality. The best prognosis was related to the performance of cardiovascular surgery (hazard ratio [HR]= 0.51; 95% CI 0.26-0.99).

Resumo Fundamento: A doença arterial coronariana é a principal causa de morte no mundo, e a idade é fator de risco independente de mortalidade em pacientes submetidos à revascularização cirúrgica. Objetivo: Avaliar os fatores preditores de risco de óbito em pacientes submetidos à revascularização miocárdica com mais de 70 anos. Métodos: Trata-se de uma coorte retrospectiva de banco de dados de cirurgia cardíaca. Foi utilizada a regressão logística para avaliar os preditores independentes de óbito. Resultados: Foram 372 pacientes submetidos à revascularização cirúrgica de 2004 a 2012. O principal fator de risco cardiovascular foi a hipertensão arterial sistêmica, seguida do diabetes melito. A mortalidade em 30 dias foi de 19,35%. A presença de doença vascular periférica (OR: 2,47), cirurgia de emergência (OR: 4,86) e procedimento valvular combinado (OR: 3,86) foram os preditores independentes de óbito. Conclusão: O procedimento cirúrgico em pacientes idosos apresentou mortalidade maior que da população geral. Doença vascular periférica, cirurgia de emergência e procedimento valvular combinado aumentaram o risco de óbito nesses pacientes.

Abstract Background: Coronary artery disease is the leading cause of death worldwide, with age being an independent risk factor for mortality in patients submitted to surgical revascularization. Objective: To evaluate the mortality risk predictors in patients older than 70 years submitted to myocardial revascularization. Methods: This is a retrospective cohort study of a cardiac surgery database. Logistic regression was used to assess independent death predictors. Results: A total of 372 patients submitted to surgical revascularization from 2004 to 2012 were assessed. The main cardiovascular risk factor was hypertension, followed by diabetes mellitus. Mortality at 30 days was 19.35%. The presence of peripheral vascular disease (OR: 2,47), emergency surgery (OR: 4,86) and combined valve procedure (OR: 3,86) were independent predictors of death. Conclusion: The surgical procedure in elderly patients showed a higher mortality than in the general population. Peripheral vascular disease, emergency surgery and combined valve procedures increased the risk of death in these patients.

OBJETIVO: Estudar um grupo de pacientes com lesão significativa em apenas uma artéria coronária e demonstrar se a ecocardiografia de estresse com dobutamina (EED) tem boa sensibilidade e especificidade na avaliação de viabilidade miocárdica nesse grupo de pacientes. MÉTODOS: Foram estudados 20 pacientes submetidos a angioplastia coronariana transluminal percutânea (ATC). Esse grupo foi avaliado 2 a 7 (3,65 ± 1,69) dias antes do procedimento, e 2 a 5 (4 ± 0,80) dias depois, realizando-se EED. Para a determinação de viabilidade miocárdica foi utilizado ecocardiograma bidimensional três meses após o procedimento. Doze pacientes foram submetidos a ATC de artéria descendente anterior (DA), 7 de artéria coronária direita (CD) e 1 de circunflexa (CX). Apenas um procedimento (CD) não obteve pleno êxito. RESULTADOS: Dos 340 segmentos estudados, 99 (29,18%) demonstraram alterações contráteis, sendo 63 hipocinéticos (63,4%), 28 acinéticos (28,28%) e 8 discinéticos (8,08%). Quanto aos segmentos envolvidos, obtivemos sensibilidade de 92,59%, especificidade de 84,45%, acurácia de 88,88% para o exame EED. O único caso de ATC de CX demonstrou sensibilidade de 100%; para DA, sensibilidade de 88,58%, especificidade de 95% e acurácia de 90,91%. Para segmentos da CD, sensibilidade de 91,30%, especificidade de 83,33% e acurácia de 88,71%. Todos os segmentos discinéticos eram inviáveis. Dos 63 hipocinéticos, a EED previu recuperação em 91,48%. CONCLUSÃO: A EED é útil na avaliação de viabilidade miocárdica em pacientes com lesão significativa de apenas uma artéria.

OBJECTIVE: To investigate a group of patients that have a significant lesion in a single-vessel and to demonstrate whether or not the sensitivity and specificity of a dobutamine stress echocardiography (DSE) was valuable in the evaluation of myocardial viability for these patients. METHODS: Twenty patients who had undergone percutaneous transluminal coronary angioplasty (PTCA) were studied. This group was evaluated 2 to 7 days (3.65 ± 1.69) before the procedure and 2 to 5 days (4 ± 0.80) after the procedure with a DSE. Myocardial viability was assessed three months after the procedure using a two dimensional echocardiogram. Twelve patients underwent PTCA on the left anterior descending artery (LAD), 7 on the right coronary artery (RC) and 1 on the circumflex artery (CX). Only one right coronary artery procedure was not 100% successful. RESULTS: From the 340 segments that were studied, 99 (29.18%) demonstrated contractile alterations of which 63 were hypokinetic (63.4%), 28 akinetic (28.28%) and 8 dyskinetic (8.08%). In reference to the segments involved, we obtained a sensitivity of 92.59%, specificity of 84.45%, and accuracy of 88.88% for the DSE. The solitary case of PTCA for the circumflex artery demonstrated 100% sensitivity. The LAD demonstrated a sensitivity of 88.58%, specificity of 95% and accuracy of 90.91%. For the RC segments, sensitivity was 91.30%, specificity 83.33% and accuracy 88.71%. All dyskinetic segments were unviable. The DSE predicted a 91.48% recovery rate for the 63 hypokinetic segments. CONCLUSION: The DSE is an effective test for evaluating myocardial viability in patients with a significant single-vessel disease.

OBJETIVO: Determinar os fatores preditores ecocardiográficos de morte em crianças com cardiomiopatia dilatada idiopática. MÉTODOS: Estudo retrospectivo de 148 crianças com cardiomiopatia dilatada idiopática diagnosticadas entre setembro/1979 a março/2003. Critérios para inclusão: insuficiência cardíaca e redução da contratilidade no ecocardiograma, na ausência de cardiopatia congênita ou secundária. Foram analisados 470 exames, durante 244,8 meses de evolução. Parâmetros analisados: dimensão do átrio esquerdo (DAE), relação átrio esquerdo/aorta (DAE/Ao), dimensão sistólica (DSVE) e diastólica (DDVE) do VE, massa do VE (massa), dimensão do ventrículo direito (DVD), fração de ejeção do VE (FE), percentagem de encurtamento do VE (%EC), gravidade da insuficiência das valvas atrioventriculares e da valva pulmonar e medida da pressão sistólica (PsVD) e diastólica (PdVD) do VD. Foi considerado significativo erro alfa <0,05. RESULTADOS: Idade média de 2,37 anos, ocorridos 35 óbitos (23,7% - IC95=17,1% a 31,2%). ANOVA demonstrou: DAE (p<0,0001), DAE/Ao (p<0,0001), DSVE (p=0,0061), DDVE (p=0,0086), massa (p<0,0001), FE (p=0,0074), %EC (p=0,0072) e DVD (p<0,0001). O agravamento da insuficiência mitral (IM) (p=0,0113) e tricúspide (IT) (p=0,0044) foram marcadores de óbito e a presença de IM, IT e insuficiência pulmonar moderada/grave foram deletérias à sobrevida. A análise de Cox demonstrou como preditores independentes da morte: DAE/Ao (p=0,0487), FE (<0,0001) e presença de IM moderada/grave (p=0,0419). CONCLUSÃO: Os pacientes com aumento progressivo da DAE/Ao, redução da FE e agravamento progressivo da IM, a despeito do tratamento clínico, devem ser considerados precocemente em programa de transplante cardíaco.

OBJECTIVE: To determine the echocardiographic predicting factors of death in children with idiopathic dilated cardiomyopathy. METHODS: A retrospective study of 148 children with idiopathic dilated cardiomyopathy diagnosed between September 1979 and March 2003 was carried out. The inclusion criteria were as follows: heart failure and a reduction in contractility on the echocardiogram in the absence of congenital or secondary heart disease. Four hundred and seventy examinations during a period of 244.8 months of evolution were analyzed. The following parameters were assessed: left atrial dimension (LAD); left atrium/aorta ratio (LAD/Ao); left ventricular systolic (LVSD) and diastolic (LVDD) dimensions; left ventricular mass (LVmass); right ventricular dimension (RVD); left ventricular ejection fraction (LVEF); left ventricular shortening fraction (% SH); severity of the insufficiency of the atrioventricular and pulmonary valves; and right ventricular systolic (RVSP) and diastolic (RVDP) pressures. The significance level adopted was alpha < 0.05. RESULTS: The mean age was 2.37 years, and 35 patients died (23.7% - 95 CI = 17.1% to 31.2%). The analysis of variance showed the following: LAD (p<0.0001); LAD/Ao (p<0.0001); LVSD (p=0.0061); LVDD (p=0.0086); LVmass (p<0.0001); LVEF (p=0.0074); %SH (p=0.0072); and RVD (p<0.0001). Worsening of mitral (MI) (p=0.0113) and tricuspid (TI) insufficiencies (p=0.0044) were markers of death, and the presence of MI, TI, and moderate/severe pulmonary insufficiency were deleterious to survival. The Cox proportional hazards regression model showed the following independent predictors of death: LAD/Ao (p=0.0487); LVEF (<0.0001); and the presence of moderate/severe MI (p=0.0419). CONCLUSION: Patients with a progressive increase in LAD/Ao, a reduction in LVEF, and progressive worsening of MI, regardless of the clinical treatment, should be considered for early heart transplantation.

OBJECTIVE: To report and assess the incidence of cardiac tamponade in systemic lupus erythematosus as a cardiac manifestation of the disease. METHODS: We reviewed the medical records of 325 patients diagnosed with systemic lupus erythematosus according to the American Rheumatism Association and their complementary laboratory tests compatible with cardiac tamponade. RESULTS: In the 325 medical recors reviewed, we found 108 patients with pericardial effusions corresponding to 33.2% of the total and 54% of the patients studied in the active phase of the disease. Clinical assessment and transthoracic echocardiogram allowed the clinical diagnosis of cardiac tamponade in only 4 (1.23%) patients, 3 of whom were females, white, with ages ranging from 25 to 44 years. The pericardial fluid was hemorrhagic or serosanguineous with high levels of FAN and positivity for LE cells. In the treatment, we successfully used pericardicentesis associated with high doses of corticosteroids. In clinical and laboratory follow-up performed for a period of 3 years, neither recrudescence of the pericardial effusion nor evolution to constriction occurred. CONCLUSION: Even though rare (1.23%), cardiac tamponade in patients with systemic lupus erythematosus has a benign evolution when properly treated, according to our experience.

OBJETIVO: Avaliar o papel da abstenção alcoólica, no período de 12 meses, na cardiomiopatia alcoólica (CMA) com disfunção ventricular de grau moderado, em pacientes tratados com esquema anticongestivo. MÉTODOS: Estudo observacional prospectivo com 20 pacientes com CMA, 9 (45%) na classe funcional (CF) II e 11(55%) na CF III, 16 (80%) homens, predomínio de negros (55%), de 35 a 56 (x=45) anos, com consumo pesado de álcool (>80g etanol/dia), por período de 51 a 112 (x=85) meses, que concordaram, inicialmente, em participar de programa de apoio, com psicoterapia de grupo, além do acompanhamento clínico com realização de exames não invasivos, antes do início do programa e após 12 meses da terapia, e foram divididos em dois grupos, o 1 (G-I) constituído pelos que atingiram a abstinência e o 2 (G-II) pelos não abstêmios. RESULTADOS: Após 12 meses, 11 (55%) pacientes permaneciam em terapia de apoio, 8 (72,72%) no G-I, enquanto os 9 (45%) que não se mantiveram no programa, apenas 2 (22,22%) tinham logrado abstinência (G-I). Ao fim da avaliação, alcançamos número igual de pacientes entre os grupos. Comparando os grupos observamos: a) menores valores médios dos diâmetros sistólico (DSVE) e diastólico (DDVE) do ventrículo esquerdo no G-I; b) maior número de internações no G-II (3) em relação ao G-I (1); c) na evolução clínica, maior número de pacientes do G-I, entre os que apresentaram melhoras (3 G-I e 1 G-II) e que permaneceram inalterados (6 G-I e 3 G-II), além do maior número de pioras entre o G-II (1 G-I e 5 G-II). CONCLUSÃO: Apesar do desejo inicial favorável, a abstinência só foi obtida em 50% dos pacientes com CMA e disfunção ventricular moderada, porém, quando alcançada, apresentou melhor evolução (melhoras + inalterados = 90%), com maior redução do DSVE e do DDVE (p<0,001), devendo sempre ser perseguida mesmo na presença de moderada disfunção ventricular.

PURPOSE: To evaluate the role of a 12 month alcohol abstinence period in patients with moderate left ventricular dysfunction treated with anticongestive therapy. METHODS: Prospective observational study with 20 patients with alcoholic cardiomyopathy (ACM), 9 (45%) in functional class (FC) II and 11 (55%) in FC III, 16 (80%) men, mostly black (55%), from 35 to 56 (x=45) years old, heavy alcohol users (>80g ethanol for 51 to 112 (x=88) months. At the beginning, all agreed to participate with psychotherapy and clinical evaluation. After 12 months, they were divided in G-I, formed by those who remained abstemious and G-II of non-abstemious. RESULTS: After 12 months, among the 11(55%) who remained in psychotherapy, 8 were in G-I, among those who did not 9 (45%), only 2 (22,22%) remained abstemious (G-I). At the end of the evaluation period, both groups had the same number of patients. Comparing them, we observed: a) lower mean systolic and diastolic left ventricular diameters in G-I; b) more hospitalizations in G-II (3); c) more patients with stable or better clinical evolution in G-I. CONCLUSION: Despite the initial will, only 50% reached abstinence. When it was reached, patients had a better evolution in left ventricular systolic diameter and abstinence should always be tried even in the presence of moderate left ventricular dysfunction.

Homem de 59 anos, portador de cardiomiopatia hipertrófica apical há 12 anos, apresentou agudização de quadro anginoso. Foi detectada doença arterial coronária com lesão trivascular, e indicada cirurgia de revascularização, com anastomose de artéria mamária e a colocação de duas pontes de veia safena, tendo evoluído com melhora do quadro anginoso e redução da dimensão do átrio esquerdo.

A fifty-nine year old man, known to have hypertrophic cardiomyopathy, presented worsening of angina. Multivessel coronary artery disease was diagnosed, and he underwent myocardial revascularization (mammary and two safenous grafts were implanted) with good evolution and reduction of left atrium dimension.

OBJETIVO: Avaliar o papel da doença arterial coronária (DAC) com comprometimento da artéria descendente anterior (ADA) na cardiomiopatia hipertrófica (CMH) e sua repercussão na evolução, visto ser controverso o significado da necrose e fibrose do septo interventricular (SIV) nesta cardiomiopatia. MÉTODOS: Entre 158 pacientes com CMH, selecionamos 6 (3,79%) com CMH e DAC com lesão obrigatória de ADA, sendo 4 homens, entre 52 e 70 (x= 65,16) nos, 4 com a forma obstrutiva da CMH. O tempo de diagnóstico da CMH foi de 78 a 182 (x=141) meses e da DAC de 1 dia a 106 (x= 42) meses. Os pacientes foram acompanhados com avaliações clínicas e exames complementares periódicos. RESULTADOS: A forma de apresentação da DAC foi em 5 com angina instável e um com infarto do miocárdio. A ADA estava comprometida entre 60 a 100%, sendo em um lesão única e nos 5 restantes com lesão em 2 ou mais vasos. Na evolução, 3 foram submetidos a revascularização miocárdica (RM), um associada a miomectomia septal, um a angioplastia e 2 somente a tratamento clínico. No período de observação de 76 a 124 meses após o diagnóstico da DAC, ocorreu um óbito. No fim do estudo observamos redução nos valores médios do SIV de 1,53 para 1,40cm, gradiente de pressão entre o corpo e a via de saída do ventrículo esquerdo (VE) de 56 para 15,75mmHg, com discreto aumento no diâmetro diastólico do VE de 4,55 para 4,85cm e do diâmetro sistólico de 2,83 para 3,13cm, sem alterar a dimensão do átrio esquerdo (4,13cm). CONCLUSÃO: A DAC da ADA é bem tolerada na CMH septal assimétrica, participando do processo fibrótico septal e melhorando o desempenho cardíaco, não representando problema adverso na evolução da CMH.

PURPOSE: The significance of necrosis and fibrosis of the interventricular septum in hypethrophic cardiomyopathy (HCM) is controversial. The purpose of this study was then to evaluate the clinical impact of left anterior descending artery (LAD) disease in HCM. METHODS: Among 158 patients presenting with HCM, 6 (3.79%) had LAD disease. Mean age was 65.16 years (52 to 70), 4 were men and 4 had the obstructive form of HCM. All patients were submitted to complete clinical and laboratory evaluation. Mean time of the diagnosis was 141 months (ranging from 78 to 182) for HCM and 42 months (ranging from one day to 106 months) for LAD disease. RESULTS: Five patients had unstable angina and one had myocardial infarction. LAD disease (60 to 100% coronary narrowing) was present in all patients; one patient had single vessel disease and 5 multivessel disease. During follow-up, 3 patients had coronary artery bypass grafting (CABG), one with associated septal myectomy; one was submitted to coronary angioplasty and 2 were submitted to medical treatment. During a follow-up period ranging from 76 to 124 months after LAD disease diagnosis, one patient died. At the end of the study, a reduction of the thickness of the interventricular septum from 1.53 to 1.40cm was observed and left ventricular outflow pressure gradient decreased from 56 to 16mmHg. Left ventricular diastolic diameter increased from 4.55 to 4.85cm and systolic diameter from 2.83 to 3.13cm. Left atrium diameter was unchanged. CONCLUSION: LAD disease is well tolerated in the asymmetrical form of HCM and may contribute to septal fibrosis, improving cardiac function. It does not represent an adverse factor in the evolution of HCM.

OBJETIVO: Investigar a existência de diferença na forma de apresentação clínica e evolutiva da cardiomiopatia hipertrófica apical (CMHA), vista em uma amostra na cidade do Rio de Janeiro e a observada no oriente, onde foi descrita. MÉTODOS: Entre 156 pacientes com cardiomiopatia hipertrófica diagnosticados em nossa instituição, foram identificados 13 (8,34%) com a forma apical, sendo estudadas as suas manifestações clínicas, os meios de diagnóstico e a sua evolução. RESULTADOS: Eram 8 homens e 5 mulheres, com idades entre 19 a 75 anos, todos da raça branca, a maioria sintomática. O eletrocardiograma revelou ondas T gigantes em precordiais em 10 pacientes (76,92%), o ecocardiograma (ECO), a presença de hipertrofia apical em todos os casos, sendo em 10 (76,92%) restrita a ponta do ventrículo esquerdo (VE), em dois (15,4%) a ponta do ventrículo direito (VD) e, em 1 (7,68%), acometendo ambos os ventrículos. A cineventriculografia confirmou os achados do ECO. A evolução variou de 6 a 264 meses (x=95,4). Ocorreram dois óbitos (15,4%) em pacientes com o comprometimento associado ao VD, devido ao aumento significativo da dimensão dos átrios, ocorrência de fibrilação atrial, insuficiência valvar mitral e/ou tricúspide e tromboembolismo. Entre os vivos, a paciente com acometimento do VD evolui com restrição diastólica, e os restantes com lesão localizada do VE , 9 estão assintomáticos em uso de propranolol (8) ou amiodarona (1) e um permanece assintomático sem medicação. CONCLUSÃO: presentação, diagnóstico e evolução, porém notamos em 3 pacientes ( 23,08%) o acometimento da ponta do VD, não descrito no oriente, mas com péssimo prognóstico evolutivo.

PURPOSE: To investigate the differences between clinical aspects and evolution of apical hypertrophic cardiomyopathy (AHCM) seen in Rio de Janeiro and the Oriental form, which was the first described. METHODS: In 156 patients with AHCM diagnosed in our institution, there were 13 (8.34%) with the apical form of the disease, whose clinical settings, diagnostic procedures and evolution were studied. RESULTS: There were 8 males and 5 females, between 19 and 75 years old, all white. Electrocardiogram (EKG) showed giant T waves in precordial leads in 10 (76.92%), echocardiogram (ECHO) demonstrated apical hypertrophy in all, 10 (76.92%) had only in the left ventricle, 2 in right ventricle and one involving both. Cineangiography corroborated ECHO findings. Follow-up ranged from 6 to 294 months (x= 95.4). Two deaths occurred in patients with RV involvement, due to large atria, atrial fibrillation, tricuspid or mitral insufficiency and thromboembolism. Among the survivors, the patient with RV disease has diastolic restriction and the ones with LV involvement, 9 are asymptomatic using either propranolol (8) or amiodarone (1), and one uses no medication. CONCLUSION: A HCM seen in Rio de Janeiro is similar to that found in the Orient (Japan) regarding presentation, diagnosis and evolution; but in 3 patients we have found RV disease, not described in Japan, characterizing a distinct group with a worse evolution.