RESUMO
Isolated non-compaction cardiomyopathy is a rare disease that is likely to develop in the embryonic period. It is caused by the intrauterine arrest of the myocardial compaction process in the beginning of the fetal development. It is characterized by prominent myocardial trabeculations and deep intertrabecular recesses, as well as the thickening of the myocardium into two distinct layers (compacted and not compacted). Even though this disease is said to be prevalent in the pediatric population or together with congenital heart disease, one can understand that this disease occurs in isolation, because the diagnosis is becoming more common in adult patients that have no other heart disease. The clinical manifestations vary greatly, because they range from absence of symptoms to congestive heart failure, arrhythmias and systemic thromboembolism. Echocardiography is the most widely used diagnostic procedure, but the little knowledge about this disease, its similarity to other myocardial diseases and the limitation of the echocardiographic technique used delay the diagnosis. The purpose of this review is to show that that other imaging techniques, such as MRI, CT and left ventriculography have emerged as diagnostic alternatives.
Assuntos
Miocárdio Ventricular não Compactado Isolado/diagnóstico , Diagnóstico Diferencial , Ecocardiografia , Humanos , Miocárdio Ventricular não Compactado Isolado/etiologia , Miocárdio Ventricular não Compactado Isolado/terapia , Imageamento por Ressonância MagnéticaRESUMO
A miocardiopatia não compactada isolada é uma doença rara, que provavelmente surge no período embrionário, com a parada intrauterina da compactação miocárdica no início do desenvolvimento fetal, e que determina trabeculações miocárdicas proeminentes com recessos intertrabeculares profundos e espessamento do miocárdio em duas camadas distintas (compactado e não compactado). Embora descrita inicialmente na população pediátrica ou em conjunto com cardiopatia congênita, pode-se compreender que essa doença ocorre de forma isolada, porque o diagnóstico é cada vez mais comum em pacientes adultos que não apresentam outra doença cardíaca. As manifestações clínicas são altamente variáveis, porque partem da ausência de sintomas à insuficiência cardíaca congestiva, arritmias e tromboembolismo sistêmico. A ecocardiografia é o procedimento diagnóstico mais utilizado, porém o pouco conhecimento sobre essa doença, sua semelhança com outras afecções miocárdicas e a limitação da técnica ecocardiográfica empregada fazem com que o diagnóstico seja postergado. Esta revisão objetiva mostrar que outras modalidades de imagem como ressonância magnética, tomografia computadorizada e ventriculografia esquerda são alternativas diagnósticas.
Isolated non-compaction cardiomyopathy is a rare disease that is likely to develop in the embryonic period. It is caused by the intrauterine arrest of the myocardial compaction process in the beginning of the fetal development. It is characterized by prominent myocardial trabeculations and deep intertrabecular recesses, as well as the thickening of the myocardium into two distinct layers (compacted and not compacted). Even though this disease is said to be prevalent in the pediatric population or together with congenital heart disease, one can understand that this disease occurs in isolation, because the diagnosis is becoming more common in adult patients that have no other heart disease. The clinical manifestations vary greatly, because they range from absence of symptoms to congestive heart failure, arrhythmias and systemic thromboembolism. Echocardiography is the most widely used diagnostic procedure, but the little knowledge about this disease, its similarity to other myocardial diseases and the limitation of the echocardiographic technique used delay the diagnosis. The purpose of this review is to show that that other imaging techniques, such as MRI, CT and left ventriculography have emerged as diagnostic alternatives.
La miocardiopatía no compactada aislada es una enfermedad rara, que probablemente surge en el período embrionario, con la parada intrauterina de la compactación miocárdica en el comienzo del desarrollo fetal, y que determina trabeculaciones miocárdicas prominentes con recesos intertrabeculares profundos y espesamiento del miocardio en dos capas distintas (compactado y no compactado). Aunque descripta inicialmente en la población pediátrica o en conjunto con cardiopatía congénita, se puede comprender que esa enfermedad ocurre de forma aislada, porque el diagnóstico es cada vez más común en pacientes adultos que no presentan otra enfermedad cardíaca. Las manifestaciones clínicas son altamente variables, porque parten de la ausencia de síntomas a la insuficiencia cardíaca congestiva, arritmias y tromboembolismo sistémico. La ecocardiografía es el procedimiento diagnóstico más utilizado, sin embargo el poco conocimiento sobre esa enfermedad, su semejanza con otras afecciones miocárdicas y la limitación de la técnica ecocardiográfica empleada hacen que el diagnóstico sea postergado. Esta revisión pretende mostrar que otras modalidades de imagen como resonancia magnética, tomografía computarizada y ventriculografía izquierda son alternativas diagnósticas.
Assuntos
Humanos , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Diagnóstico Diferencial , Ecocardiografia , Miocárdio Ventricular não Compactado Isolado/etiologia , Miocárdio Ventricular não Compactado Isolado/terapia , Imageamento por Ressonância MagnéticaRESUMO
As doenças cardiovasculares e neoplásicas tornaram-se, nos últimos anos, temas prioritários na maioria dos países, pelo impacto na mortalidade, na morbidade e nos custos decorrentes da assistência médica. No Brasil, esse cenário está bem documentado nas estatísticas oficiais da vigilância epidemiológica de doenças crônicas do Ministério da Saúde e, principalmente, na série de estudos realizados para determinar a frequência de fatores de risco relacionados a essas doenças. As taxas de câncer, de acordo com relatórios publicados pela Organização Mundial de Saúde (OMS), irão duplicar até 2020, o que tornará as doenças neoplásicas as mais incidentes e prevalente, ultrapassando as doenças cardiovasculares. Atualmente, são diagnosticados, a cada ano, mais de 10 milhões de casos novos de câncer no mundo e a tendência, para a próxima década, é de que esse número ultrapasse os 20 milhões anuais.
Cardiovascular and neoplastic diseases have become priority issues in most countries due to their impact on mortality, morbidity and healthcare costs in the last few years. In Brazil, this scenario is well documented by official epidemiological surveillance statistics of chronic diseases, performed by the Ministry of Health and by the series of studies carried out to determine the frequency of risk factors related to these diseases. Cancer rates, according to estimates reported by the World Health Organization (WHO), will double by 2020, which will make neoplastic diseases the most prevalent and incident diseases, surpassing cardiovascular diseases. Over 10 million new cases of cancer are diagnosed worldwide every year, and the trend for the next decade is that this figure will exceed 20 million per year.
Assuntos
Humanos , Masculino , Feminino , Doenças Cardiovasculares/mortalidade , Neoplasias/epidemiologia , Neoplasias/mortalidade , Tabagismo/epidemiologia , Fatores de RiscoRESUMO
OBJECTIVES: We compared left ventricular regional wall motion, the global left ventricular ejection fraction, and the New York Heart Association functional class pre- and postoperatively. INTRODUCTION: Endomyocardial fibrosis is characterized by fibrous tissue deposition in the endomyocardium of the apex and/or inflow tract of one or both ventricles. Although left ventricular global systolic function is preserved, patients exhibit wall motion abnormalities in the apical and inferoapical regions. Fibrous tissue resection in New York Heart Association FC III and IV endomyocardial fibrosis patients has been shown to decrease morbidity and mortality. METHODS: We prospectively studied 30 patients (20 female, 30+/-10 years) before and 5+/-8 months after surgery. The left ventricular ejection fraction was determined using the area-length method. Regional left ventricular motion was measured by the centerline method. Five left ventricular segments were analyzed pre- and postoperatively. Abnormality was expressed in units of standard deviation from the mean motion in a normal reference population. RESULTS: Left ventricular wall motion in the five regions did not differ between pre- and postoperative measurements. Additionally, the left ventricular ejection fraction did not change after surgery (0.45+/-0.13% x 0.43+/-0.12% pre- and postoperatively, respectively). The New York Heart Association functional class improved to class I in 40% and class II in 43% of patients postoperatively (p<0.05). CONCLUSIONS: Although endomyocardial fibrosis patients have improved clinical symptoms after surgery, the global left ventricular ejection fraction and regional wall motion in these patients do not change. This finding suggests that other explanations, such as improvements in diastolic function, may be operational.
Assuntos
Fibrose Endomiocárdica/fisiopatologia , Complicações Pós-Operatórias/fisiopatologia , Disfunção Ventricular Esquerda/fisiopatologia , Adulto , Diástole/fisiologia , Fibrose Endomiocárdica/complicações , Fibrose Endomiocárdica/cirurgia , Feminino , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Estudos Prospectivos , Índice de Gravidade de Doença , Estatísticas não Paramétricas , Volume Sistólico/fisiologia , Disfunção Ventricular Esquerda/etiologiaRESUMO
OBJECTIVES: We compared left ventricular regional wall motion, the global left ventricular ejection fraction, and the New York Heart Association functional class pre- and postoperatively. INTRODUCTION: Endomyocardial fibrosis is characterized by fibrous tissue deposition in the endomyocardium of the apex and/or inflow tract of one or both ventricles. Although left ventricular global systolic function is preserved, patients exhibit wall motion abnormalities in the apical and inferoapical regions. Fibrous tissue resection in New York Heart Association FC III and IV endomyocardial fibrosis patients has been shown to decrease morbidity and mortality. METHODS: We prospectively studied 30 patients (20 female, 30±10 years) before and 5±8 months after surgery. The left ventricular ejection fraction was determined using the area-length method. Regional left ventricular motion was measured by the centerline method. Five left ventricular segments were analyzed pre- and postoperatively. Abnormality was expressed in units of standard deviation from the mean motion in a normal reference population. RESULTS: Left ventricular wall motion in the five regions did not differ between pre- and postoperative measurements. Additionally, the left ventricular ejection fraction did not change after surgery (0.45±0.13 percent x 0.43±0.12 percent pre- and postoperatively, respectively). The New York Heart Association functional class improved to class I in 40 percent and class II in 43 percent of patients postoperatively (p<0.05). CONCLUSIONS: Although endomyocardial fibrosis patients have improved clinical symptoms after surgery, the global left ventricular ejection fraction and regional wall motion in these patients do not change. This finding suggests that other explanations, such as improvements in diastolic function, may be operational.
