ABSTRACT
We studied four children who were born with congenital rubella syndrome, in whom cardiac malformations including semilunar valvar stenosis were present. These children attended the pediatric cardiology unit of Hospital Fundacao Santa Casa de Misericordiórdia do Pará. The diagnosis was made clinically and by laboratory results. Specific anti-rubella antibodies IgM and IgG were measured using a capture-antibody enzyme immunoassay. Three patients presented with aortic stenosis associated with patient ductus arteriosus, and 1 patient had isolated pulmonary valvar stenosis. Rubella-specific IgM antibodies were detected in 3 of the 4 children and IgG antibodies in 1. We review the role of rubella virus as a teratogenic agent implicated in a pathogenesis of aortic and pulmonary valvar stenosis. These pathologic lesion are part of the diffuse ateriopathy that affects the fetus with rubella virus infection.
Subject(s)
Humans , Male , Infant , Heart Defects, Congenital/etiology , Aortic Valve Stenosis/etiology , Pulmonary Valve Stenosis , Rubella/complications , Rubella/diagnosis , Rubella Syndrome, Congenital/complications , Rubella Syndrome, Congenital/diagnosis , Heart Ventricles/injuriesABSTRACT
PURPOSE: To evaluate clinical symptoms and echocardiographic findings in elderly patients with severe aortic stenosis and possible gender differences. METHODS: We studied 54 patients, 24 (44.5) males and 30 (55.5) females aged 80.7 +/- 5.2 years with severe aortic stenosis. The following variables were analyzed: presence of clinical manifestations (dyspnea, angina, and syncope) and echocardiographic indices (left ventricular [LV] dimensions, ejection fraction [EF], and mass index). RESULTS: Dyspnea was the most frequent symptom with overall prevalence of 44. EF was lower than 50in only 2 patients. There were no gender differences in the prevalence of any of the clinical manifestations. Male patients had higher LV volumes (p < 0.05) and lower EF (p = 0.03). CONCLUSION: The data showing dyspnea as the most common clinical manifestation; EF > 50; lower LV volumes and greater EF in female patients suggest that the adaptive mechanisms to this condition may be different between the two sexes.
Subject(s)
Humans , Male , Female , Aged , Aortic Valve Stenosis , Aged, 80 and over , Aortic Valve Stenosis/physiopathology , Severity of Illness IndexABSTRACT
Determinadas doenças maternas, ocorrendo durante a gravidez, associam-se a cardiopatias congênitas em filhos em uma relaçäo causal. Neste artigo säo analisadas moléstias infecciosas, perturbaçöes do metabolismo e afecçöes difusas do tecido conectivo, que acarretam diversas alteraçöes congênitas: malformaçöes cardiovasculares, cardiomiopatias e bloqueio cardíaco. As viroses, principalmente a rubéola, säo muito importantes, porque teratogênicas e causadoras de miocardite. Dentre as doenças metabólicas destaca-se o diabete, eventualmente relacionado a malformaçöes cardiovasculares e a uma forma peculiar de cardiomiopatia hipertrófica. A fenilcetonúria, um erro inato do metabolismo, pode ser responsável por anomalias estruturais cardíacas. As afecçöes difusas do tecido conectivo, principalmente o lúpus eritematoso disseminado, constituem uma das causas de bloqueio atrioventricular completo fetal, dependente de reaçäo imunitária.
Subject(s)
Humans , Infant, Newborn , Pregnancy , Cardiomyopathy, Hypertrophic , Communicable Diseases , Heart Block , Heart Defects, Congenital , Metabolic Diseases , Mixed Connective Tissue Disease , Parasitic Diseases , Time FactorsABSTRACT
PURPOSE--To analyze some morphological aspects of the tetralogy of Fallot which have been raising controversies in the literature, due to the different approach to the nomenclature of congenital heart defects by different authors regarding the definition of double outlet right ventricle and its concomitance with tetralogy. METHODS--We reviewed the original description of the anomaly, and also analyzed the morphology of 22 anatomical specimens, describing the degree of aortic overriding, the type of ventricular septal defect, the degree of sub-pulmonary stenosis, etc. RESULTS--Regarding the degree of aortic overriding, there was only one case where that vessel connected predominantly to the left ventricle. In the remaining hearts, the degree of overriding was between 50 per cent and 75 per cent in 10 cases and greater than 75 per cent in 11. The intensity of infundibular stenosis was evaluated as mild in half the available hearts, and the pulmonary valve was bicuspid in 11 cases. Right aortic arch was present in 40 per cent of the available hearts and the ventricular septal defect had muscular borders in just one case (5 per cent). CONCLUSION--We could conclude that if double outlet is only a type of ventricle-arterial connexion, and so, depending on the degree of overriding of the aorta over the trabecular septum, we may have the malformation coexisting with the tetralogy
Subject(s)
Humans , Tetralogy of Fallot/pathology , Double Outlet Right Ventricle/pathology , Heart Ventricles/pathologyABSTRACT
Objetivo - Estudar a evoluçäo clínica de portadoras de estenose mitral no ciclo gravídico-puerperal que iniciaram a gravidez em classe funcional (CF) I/II. Métodos - Noventa e três mulheres, divididas em três grupos: Grupo GE - 30 gestantes portadoras de estenose mitral com média de idade de 27,9 anos. 26(86,7%) apresentavam sobrecarga atrial esquerda e nove (30%) ventricular direita no eletrocardiograma. A área valvar mitral ao ecodopplercardiograma variou entre 0,7 a 1,9 (1,26) cm*. Grupo GN - 32 gestantes näo cardiopatas, com média de idade de 25,5 anos com eletrocardiograma e área valvar mitral ao ecodopplercardiograma normais. Grupo EM - 31 portadoras de estenose mitral, näo grávidas com média de idade de 25 anos. Dezenove (61,3%) apresentavam sobrecarga atrial esquerda e quatro (13%) sobrecarga ventricular direita no eletrocardiograma. A área valvar mitral variou entre 0,50 a 1,80 (média = 1,19) cm*. Em cada consulta foram anotados os dados sobre CF e complicaçöes. As complicaçöes investigadas foram congestäo pulmonar, endocardite infecciosa, arritmia cardíaca e tromboembolismo. Resultados - Vinte e seis (86,7%) pacientes do grupo GE modificaram a CF: 16 evoluíram para CF III e 10 para IV, no grupo GN, 18 (56,2%) pacientes evoluíram de CF I para II no decorrer da gestaçäo e no grupo EM, cinco (16%) evoluíram da CF I/II para III durante o estudo. Arritmia cardíaca, endocardite infecciosa näo foram registradas. Fenômeno tromboembólico foi identificado em um (3,2%) caso no grupo EM. Näo houve mortalidade. Conclusäo - A grande maioria das gestantes portadoras de estenose mitral, que iniciaram a gravidez em CF I/II evoluíram para CF III/IV, no decorrer da gestaçäo. Medidas como repouso, farmacoterapia e avalvoplastia por cateter-baläo contribuíram, na totalidade dos casos, para o termo da gestaçäo e ausência de mortalidade
Purpose The clinical evolution of women with mitral stenosis was studied during pregnancy, delivery and puerperium in inicial function (FCJ class I/II. Methods Ninety-three women were divided in three groups. Group GE Pregnant women with mitral stenosis (n = 30, mean age 28 years); 26 (86.7%) patients had electrocardiographic signs of left atrial enlargement and nine (30%) had signs of right ventricular hypertrophy. The mitral valvar area was between 0.7 and 1.9 (mean = 1.26) cm2 at echod opplercardiogram, Group GM Normal pregnant women (n = 32; aged 25.4 years), the electrocardiogram and echodopplercardiogram were normal. Group EM - non pregnant patients, with mitral stenosis (n = 31.33 years); 19 (61,3%) had left atrial enlargement andfour (13%) had right ventricular hypertrophy. The mitral valvar area between 0.50 and 1.80 (mean = 1.19) cm2. The variables analyzed were FC and occurrence of the following complications. infective endocarditis, cardiac arrhythmias and thromboembolism. Results In GE group, 26 (86,7%) patients worsened the FC during gestation, 16 to FC III and 10 to FC IV. In GN group, 18 (56,2% ) patients changed from FC I to FC II during the gestation and in EM group 5 (16,2%) patients changed from FC I/II to III during the study. Cardiac arrhythmias and infective endocarditis were not observed; thromboembolic event was registered in one (3.2%) patients from EM group. There were no death in all groups. Conclusion The large majority of pregnant with mitral stenosis that started pregnancy in FC I/II worsened to FC III/IV during gestation. Medical treatment and eventually balloon valvuloplasty were successfull measure to allow a full-term gestation without mortality
Subject(s)
Humans , Female , Pregnancy , Adolescent , Adult , Middle Aged , Mitral Valve Stenosis/physiopathology , Pregnancy Complications, Cardiovascular/physiopathology , Catheterization , Prospective Studies , Postpartum Period/physiology , Mitral Valve Stenosis/drug therapy , Mitral Valve Stenosis/therapy , Heart RateABSTRACT
Em revisäo crítica säo estudados os diferentes conceitos sob os quais têm sido admitidas as miocardiopatias. Denominaçöes antigas (miocardites primitivas ou primárias, miocardias, miocardoses) säo discutidas, com avaliaçäo da expressividade de cada uma. A concepçäo de cardiomiopatias é analisada em funçäo de sua significaçäo inicial e em relaçäo à sua amplitude atual. É ressaltada a heterogeneidade das formas aceitas hoje sob esse nome, o que praticamente anula seu caráter normativo básico. É proposta uma classificaçäo das miocardiopatias baseada: a) na presença de afecçöes sem causa aparente (cardiomiopatias) ou dependentes de estado mórbido conhecido (miocardiopatias específicas), b) na ocorrência, ou näo, de estado essencialmente inflamatório e c) no reconhecimento de processos etiológicos e/ou patogênicos
Subject(s)
Humans , Cardiomyopathies , Cardiomyopathies/classificationSubject(s)
Humans , Hominidae/genetics , Genome, Human , Life Style , Anthropology, Physical , Diet , Biological Evolution , Genotype , Genetics, Medical , Genetics, PopulationSubject(s)
Erythromycin/therapeutic use , Rheumatic Fever/prevention & control , Penicillin G Benzathine/therapeutic use , Rheumatic Diseases/prevention & control , Sulfadiazine/therapeutic use , Streptococcal Infections/complications , Streptococcal Infections/drug therapy , Recurrence , Rheumatic Diseases/drug therapy , Streptococcus pyogenesABSTRACT
De um total de 225 valvas mitrais obtidas cirurgicamente de pacientes reumáticos em fase de quiescência clínica foram selecionadas 70 que apresentavam calcificaçäo. Estas foram analisadas histológica e histoquimicamente para avaliaçäo da existência ou näo de correlaçäo entre calcificaçäo, inflamaçäo e depósito amilóide. Como controle, foram estudadas 20 valvas que näo apresentavam calcificaçäo. Observou-se infiltrado inflamatório moderado ou intenso em 59 (84,2%) valvas e discreto ou ausente em 11 casos (15,8%), sempre em íntima relaçäo com as áreas de calcificaçäo. A pesquisa para amilóide feita pela coloraçäo do vermelho Congo mostrou-se positiva em 47 (67,1%) das 70 valvas com calcificaçäo, sendo que 44 (93,6%) apresentavam intenso infiltrado inflamatório, sempre próximo à calcificaçäo. Apenas três valvas apresentavam amilóide junto as áreas calcificadas, sem inflamaçäo adjacente. No grupo controle, a pesquisa para amilóide foi negativa em todos os casos. A análise ao microscópio eletrônico de duas valvas com calcificaçäo mostrou presença de elementos fibrilantes muito semelhantes a filamentos do tipo intermediário em localizaçäo extracelular. O presente trabalho demonstrou evidente associaçäo entre inflamaçäo, calcificaçäo e depósito amilóide em valva mitral cronicamente lesada pela doença reumática. Sugere-se que a inflamaçäo deve desempenhar papel relevante na progressäo das lesöes valvares crônicas de etiologia reumática favorecendo a deposiçäo extracelular do citoesqueleto, ulterior amiloidose e calcificaçäo