Assuntos
Cardiomiopatia Restritiva , Neoplasias Cardíacas , Hemangiossarcoma , Humanos , Hemangiossarcoma/patologia , Hemangiossarcoma/complicações , Hemangiossarcoma/diagnóstico , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/diagnóstico por imagem , Cardiomiopatia Restritiva/patologia , Cardiomiopatia Restritiva/etiologia , Masculino , Pessoa de Meia-Idade , Feminino , Evolução FatalRESUMO
A 28 year-old male with restrictive cardiomyopathy (RCM) and endocardium thickening, conduction disorders, heart failure, and depressive disorder treated with paroxetine was admitted to the clinic. Blood tests revealed an increase in serum iron level, transferrin saturation percentage, and slightly elevated liver function tests. Sarcoidosis, storage diseases and Loeffler endocarditis were ruled out. Mutations in desmin (DES) and hemochromatosis gene (HFE1) were identified. Liver biopsy was obtained to verify the hemochromatosis, assess its possible contribution to the RCM progression and determine indications for treatment. Biopsy revealed signs of drug-induced injury, subcompensated heart failure, and hemosiderin accumulation. Thus, even if one obvious cause (desmin mutation) of RCM has been identified, other less likely causes should be taken into consideration.
Assuntos
Cardiomiopatia Restritiva , Insuficiência Cardíaca , Hemocromatose , Neoplasias Hepáticas , Adulto , Cardiomiopatia Restritiva/etiologia , Cardiomiopatia Restritiva/genética , Desmina/genética , Insuficiência Cardíaca/genética , Hemocromatose/complicações , Hemocromatose/genética , Humanos , MasculinoRESUMO
BACKGROUND The cardiotoxic effects of chemotherapy in cancer treatment can damage cardiomyocytes. A common link in the pathogenesis is the proliferation of fibroblasts and the increase of collagen synthesis, leading to development of common endomyocardial fibrosis. The walls of ventricles become rigid and their inability to relax prevents them from carrying the required amount of blood. The myocardial contractility gradually decreases and leads to ventricular dysfunction and signs of heart failure. CASE REPORT A 29-year-old woman with reduced exercise tolerance, dyspnea, and heart rhythm disorders was admitted to our hospital. Lymphoblastic leukemia had been diagnosed at the age of 8 years, and she underwent 8 courses of polychemotherapy. She had normal heart anatomy. At the current admission, the diagnostic protocol included echocardiography, computed tomography, cardiac catheterization, and angiocardiography. She was diagnosed with restrictive cardiomyopathy with isolated endomyocardial fibrosis of the right ventricle, and moderate tricuspid valve insufficiency NYHA class III. The patient underwent a right-sided bidirectional cavopulmonary connection with tricuspid valve repair. The early postoperative period was uneventful, and SVCp decreased to 14 mmHg. At discharge, the patient's clinical condition had improved and tricuspid regurgitation was minimal. CONCLUSIONS The one-and-a-half ventricular correction, commonly used in patients with Ebstein's anomaly and RV dysfunction or in patients with congenital heart defects associated with RV hypoplasia, is proposed as the method of choice for cardiomyopathy type RV dysfunction.
Assuntos
Cardiomiopatias , Cardiomiopatia Restritiva , Anomalia de Ebstein , Leucemia-Linfoma Linfoblástico de Células Precursoras , Adulto , Cardiomiopatia Restritiva/etiologia , Criança , Feminino , Ventrículos do Coração/diagnóstico por imagem , Humanos , Resultado do TratamentoAssuntos
Neuropatias Amiloides Familiares , Benzoxazóis/administração & dosagem , Cardiomiopatia Restritiva , Ensaios de Uso Compassivo/métodos , Imagem Cinética por Ressonância Magnética/métodos , Imagem de Perfusão do Miocárdio/métodos , Neuropatias Amiloides Familiares/complicações , Neuropatias Amiloides Familiares/diagnóstico , Neuropatias Amiloides Familiares/tratamento farmacológico , Neuropatias Amiloides Familiares/genética , Cardiomiopatia Restritiva/diagnóstico por imagem , Cardiomiopatia Restritiva/etiologia , Cardiomiopatia Restritiva/fisiopatologia , Cardiomiopatia Restritiva/terapia , Aprovação de Drogas , Drogas em Investigação/administração & dosagem , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/terapia , Humanos , Resultado do TratamentoAssuntos
Cardiomiopatia Restritiva/etiologia , Cardiomiopatia Restritiva/terapia , Terapia Baseada em Transplante de Células e Tecidos/métodos , Transplante de Células-Tronco , Animais , Células da Medula Óssea , Transplante de Medula Óssea , Cardiomiopatia Restritiva/diagnóstico , Doença Crônica , Modelos Animais de Doenças , Feminino , Fibrose , Isquemia/complicações , Masculino , Camundongos , Roedores , Falha de Tratamento , Resultado do TratamentoRESUMO
OBJECTIVE: To investigate the potential association between neutrophil degranulation and patterns of myocardial dysfunction in a cohort of patients with type 2 diabetes mellitus (T2DM). BACKGROUND: Two distinct phenotypes of diabetic cardiomyopathy have been described: a restrictive phenotype with diastolic dysfunction (restrictive/DD) and a dilative phenotype with systolic dysfunction (dilative/SD). However, the underlying determinants of these two patterns are not yet recognized. METHODS: In this single-centre, observational, cross-sectional study, 492 patients were recruited. Ultrasonographic measurements were performed by two experienced sonographers, blinded to the clinical data of the participants. Serum biomarkers of neutrophil degranulation were measured by enzyme-linked immunosorbent sandwich assay (ELISA). RESULTS: After adjustment for confounders, resistin, myeloperoxidase, matrix metalloproteinase 8 and matrix metalloproteinase 9/tissue inhibitor of metalloproteinases 1 complex were positively associated with the restrictive/DD pattern compared with the normal pattern. Similarly, MPO was positively associated with the dilative/SD pattern compared with the normal pattern, and resistin was negatively associated with the dilative/SD pattern compared with the restrictive/DD pattern. CONCLUSIONS: Neutrophil degranulation is associated with the restrictive/DD echocardiographic pattern in patients with T2DM, but not with the normal pattern and dilative/SD patterns. Neutrophils could have a pivotal role in the pathogenesis of myocardial dysfunction, and particularly diastolic dysfunction, in patients with T2DM.
Assuntos
Cardiomiopatia Dilatada/metabolismo , Cardiomiopatia Restritiva/metabolismo , Diabetes Mellitus Tipo 2/metabolismo , Cardiomiopatias Diabéticas/metabolismo , Ativação de Neutrófilo , Idoso , Biomarcadores/metabolismo , Cardiomiopatia Dilatada/diagnóstico por imagem , Cardiomiopatia Dilatada/etiologia , Cardiomiopatia Dilatada/fisiopatologia , Cardiomiopatia Restritiva/diagnóstico por imagem , Cardiomiopatia Restritiva/etiologia , Cardiomiopatia Restritiva/fisiopatologia , Diabetes Mellitus Tipo 2/complicações , Diabetes Mellitus Tipo 2/fisiopatologia , Cardiomiopatias Diabéticas/diagnóstico por imagem , Cardiomiopatias Diabéticas/etiologia , Cardiomiopatias Diabéticas/fisiopatologia , Ecocardiografia , Feminino , Insuficiência Cardíaca Diastólica/diagnóstico por imagem , Insuficiência Cardíaca Diastólica/etiologia , Insuficiência Cardíaca Diastólica/metabolismo , Insuficiência Cardíaca Diastólica/fisiopatologia , Insuficiência Cardíaca Sistólica/diagnóstico por imagem , Insuficiência Cardíaca Sistólica/etiologia , Insuficiência Cardíaca Sistólica/metabolismo , Insuficiência Cardíaca Sistólica/fisiopatologia , Humanos , Masculino , Metaloproteinase 8 da Matriz/metabolismo , Metaloproteinase 9 da Matriz/metabolismo , Pessoa de Meia-Idade , Peroxidase/metabolismo , Resistina/metabolismo , Inibidor Tecidual de Metaloproteinase-1/metabolismo , Inibidor Tecidual de Metaloproteinase-2/metabolismoRESUMO
Resumen Objetivo: La fibrosis endomiocárdica (FE) es una cardiomiopatía restrictiva infrecuente. En América Latina son escasos los reportes. En el presente trabajo se realiza una descripción de una serie de pacientes diagnosticados de FE en Colombia. Método: Realizamos una búsqueda en los registros de imágenes de resonancia magnética (RM) cardiaca realizadas en nuestra institución entre 2016 y 2019 en busca de pacientes con diagnóstico de FE. Se describieron sus características sociodemográficas, clínicas y de imagen. Resultados: Nueve pacientes fueron diagnosticados de FE (el 66.7% mujeres), con una edad promedio de 69 años. Los pacientes presentaron un promedio de 2.6 años de evolución. El principal síntoma referido fue disnea, seguido de síncope, dolor torácico y palpitaciones. En ninguno de ellos se sospechó FE como diagnóstico inicial. En cuanto a los hallazgos ecocardiográficos, se identificó compromiso predominante del ventrículo izquierdo, seguido de compromiso biventricular. Todos los pacientes presentaron patrón de llenado restrictivo con dilatación auricular izquierda severa. En el análisis retrospectivo se cumplieron los criterios de Mocumbi para el diagnóstico de FE en el 100% de los pacientes con gravedad moderada (77.8%). Las imágenes de RM mostraron función sistólica biventricular y volúmenes preservados. Se observó depósito focal de gadolinio subendocárdico a nivel apical y se confirmó la presencia de trombo en el 66% de los casos Conclusión:: La FE es una cardiomiopatía restrictiva infrecuente circunscrita a países tropicales. La mayoría de los pacientes en nuestra serie presentaron compromiso aislado del ventrículo izquierdo, seguido de compromiso biventricular, con función ventricular usualmente preservada.
Abstract Objective: Endomyocardial fibrosis (EF) is an unusual restrictive cardiomyopathy. In Latin America there are few reports. Here, we made a description of patients diagnosed with EF in Colombia. Method: We conducted a search in the records of cardiac magnetic resonance imaging (MRI) performed in our institution between 2016-2019 looking for patients with a diagnosis of EF; sociodemographic, clinical and imaging characteristics were described. Results: Nine patients were diagnosed with EF (66.7% female), with an average age of 69 years. Patients presented an average evolution of 2.6 years. The main reported symptom was dyspnea, followed by syncope, chest pain, and palpitations. None of them was initially suspected for EF. Regarding echocardiographic findings, predominant left ventricular involvement was identified, followed by bi-ventricular involvement. All the patients presented a restrictive filling pattern with severe left atrial dilation. In a retrospective analysis, Mocumbi criteria for diagnosis of EF were met in 100% of the patients, majority with moderate severity (77.8%). Cardiac MRI showed biventricular systolic function and volumes preserved. Focal subendocardial late gadolinium enhancement was observed on the apex and apical thrombus was confirmed in 66% of the patients Conclusion: FE is an uncommon restrictive cardiomyopathy limited to tropical countries. Most of patients in our series presented isolated involvement of left ventricle, followed by bi-ventricular involvement, with ventricular function usually preserved.
Assuntos
Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Cardiomiopatia Restritiva/etiologia , Cardiomiopatia Restritiva/diagnóstico por imagem , Fibrose Endomiocárdica/diagnóstico por imagem , Insuficiência Cardíaca , Imageamento por Ressonância Magnética , Estudos Retrospectivos , Colômbia , Meios de Contraste , GadolínioRESUMO
Cardiomyopathies are a heterogeneous group of cardiac diseases for which diagnosis and treatment are not always simple. The diagnosis of cardiomyopathy, in particular the etiology, comes from an integration between symptoms and results collected by several instrumental exams. The brain storming for the diagnosis includes also the identification of the "red flags", i.e. the pathognomonic features for each etiology that can drive the choice of appropriate diagnostic tests and therapy. In this review, we provide a step by step approach in order to help cardiologists, not specifically dedicated to cardiomyopathies, to draw the diagnosis, therapy and follow-up. This approach will be accompanied by the consultation of other specialists to discuss together the results of the exams performed and to deepen extracardiac signs and symptoms.
Assuntos
Cardiomiopatias/diagnóstico , Cardiomiopatias/genética , Fenótipo , Avaliação de Sintomas , Displasia Arritmogênica Ventricular Direita/diagnóstico , Displasia Arritmogênica Ventricular Direita/genética , Displasia Arritmogênica Ventricular Direita/terapia , Cardiomiopatias/terapia , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/genética , Cardiomiopatia Dilatada/terapia , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/terapia , Cardiomiopatia Restritiva/diagnóstico , Cardiomiopatia Restritiva/etiologia , Cardiomiopatia Restritiva/terapia , Diagnóstico Diferencial , Ecocardiografia , Eletrocardiografia , Humanos , Imagem Cinética por Ressonância Magnética , Tomografia por Emissão de Pósitrons , Encaminhamento e Consulta , Sarcoidose/diagnósticoRESUMO
Constrictive Pericarditis (CP) is a curable and reversible form of severe diastolic heart failure. We aimed to investigate the diagnostic accuracy of published echocardiographic Mayo Clinic Criteria in differentiating 107 patients with surgically proven CP from 30 patients with restrictive cardiomyopathy due to cardiac Amyloidosis. Five principal echocardiographic and Doppler variables were remeasured on preoperative transthoracic echocardiogram namely (1) respiration-related ventricular septal shift; (2) respiratory variation in mitral inflow E pulsed Doppler velocity; 3) tissue Doppler medial mitral annular e' velocity; (4) ratio of medial mitral annular e' to lateral mitral annular e' velocity; and 5) hepatic vein (HV) pulsed Doppler diastolic flow reversal ratio. Etiology of CP included viral/idiopathic or autoimmune (75%), postcardiac surgery (13%) and postradiation (7%). Univariate logistic regression analysis showed that (1) respiration related ventricular septal shift, (2) percentage change in Mitral E velocity, (3) medial e' velocity ≥9 cm/sec, (4) medial e'/lateral e' ratio ≥0.91, (5) HV diastolic reversal ratio ≥0.79 were associated with the diagnosis of CP. Multivariable logistic regression analyses showed that medial e' velocity ≥9 cm/s was independently associated with the diagnosis of CP. Respiration related ventricular septal shift had the highest sensitivity, whereas medial e' velocity ≥9 cm/s has the highest specificity to diagnose CP (Areas under curves 0.99, p 0.001). Combining respiration related ventricular septal shift with medial e' velocity ≥9 cm/s gave a desirable sensitivity (80%) and specificity (92%). Adding reversal ratio to this combination further increased the specificity (97%) but dropped the sensitivity (70%) to diagnose CP.
Assuntos
Amiloidose/complicações , Cardiomiopatia Restritiva/diagnóstico , Ecocardiografia Doppler/métodos , Ventrículos do Coração/fisiopatologia , Miocárdio/patologia , Pericardite Constritiva/diagnóstico , Função Ventricular Esquerda/fisiologia , Idoso , Biópsia , Cardiomiopatia Restritiva/etiologia , Diagnóstico Diferencial , Diástole , Feminino , Seguimentos , Ventrículos do Coração/diagnóstico por imagem , Humanos , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Estudos RetrospectivosRESUMO
RATIONALE: Hypereosinophilic syndrome (HES) is a rare disease characterized by hypereosinophilia and its ensuing organ damage. Cardiac involvement is divided into 3 chronological stages: an acute necrotic stage; a thrombus formation stage; and a fibrotic stage. Infiltration of the myocardium by eosinophilic cells followed by endomyocardial fibrosis is known as "Loeffler endocarditis." PATIENT CONCERNS: We report a case of a 60-year-old man diagnosed with left-sided restrictive cardiomyopathy. DIAGNOSIS: The patient experienced heart failure with preserved ejection fraction. The cardiac MRI showed intense, linear, delayed gadolinium enhancement of the endocardium of the lateral wall of the left ventricle, and obliteration of the LV apex. He was ultimately identified as Loeffler endocarditis. INTERVENTION: A bone marrow smear and biopsy revealed the FIP1L1-PDGFRA fusion gene was positive in 82% of segmented nucleated cells. OUTCOME: Our patient responded well to prednisone at 1âmg/kg/d. LESSONS: HES is a rare disease that often afflicts the heart. Cardiac involvement in hypereosinophilia, especially Loeffler endocarditis, carries a poor prognosis and significant mortality. Early detection and treatment of the disease is therefore essential. Further studies are needed to ascertain therapeutic corticosteroid dosages and develop targeted gene therapies, both important steps to ameliorate the effects of Loeffler endocarditis and improve patient outcomes.
Assuntos
Medula Óssea/patologia , Cardiomiopatia Restritiva , Insuficiência Cardíaca , Ventrículos do Coração , Síndrome Hipereosinofílica , Proteínas de Fusão Oncogênica , Prednisona/administração & dosagem , Receptor alfa de Fator de Crescimento Derivado de Plaquetas , Fatores de Poliadenilação e Clivagem de mRNA , Biópsia/métodos , Cardiomiopatia Restritiva/diagnóstico , Cardiomiopatia Restritiva/etiologia , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/etiologia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Síndrome Hipereosinofílica/diagnóstico , Síndrome Hipereosinofílica/tratamento farmacológico , Síndrome Hipereosinofílica/fisiopatologia , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Proteínas de Fusão Oncogênica/análise , Proteínas de Fusão Oncogênica/genética , Receptor alfa de Fator de Crescimento Derivado de Plaquetas/análise , Receptor alfa de Fator de Crescimento Derivado de Plaquetas/genética , Volume Sistólico , Resultado do Tratamento , Fatores de Poliadenilação e Clivagem de mRNA/análise , Fatores de Poliadenilação e Clivagem de mRNA/genéticaRESUMO
Restrictive cardiomyopathies are the least common form of heart muscle disease. They are characterized as infiltrative and noninfiltrative, storage diseases, and endomyocardial disorders. Genetic diseases commonly present during childhood or adolescence. However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having forms of restrictive cardiomyopathy, particularly cardiac amyloidosis. Noninvasive evaluation has replaced endomyocardial biopsy in the diagnostic evaluation of most suspected etiologies. The detection of infiltrative cardiomyopathies, including lysosomal and glycogen storage disorders, iron overload, and amyloidosis (both light chain amyloidosis and transthyretin amyloidosis variants), as well as inflammatory diseases such as sarcoidosis has slowly led to improved outcomes via disease-specific therapies.
Assuntos
Cardiomiopatia Restritiva , Gerenciamento Clínico , Amiloidose/diagnóstico , Cardiomiopatia Restritiva/diagnóstico , Cardiomiopatia Restritiva/etiologia , Cardiomiopatia Restritiva/fisiopatologia , Humanos , Miocárdio/patologiaRESUMO
Restrictive cardiomyopathies are the least common form of heart muscle disease. They are characterized as infiltrative and noninfiltrative, storage diseases, and endomyocardial disorders. Genetic diseases commonly present during childhood or adolescence. However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having forms of restrictive cardiomyopathy, particularly cardiac amyloidosis. Noninvasive evaluation has replaced endomyocardial biopsy in the diagnostic evaluation of most suspected etiologies. The detection of infiltrative cardiomyopathies, particularly primary and secondary forms of iron overload, as well as inflammatory diseases such as sarcoidosis has slowly led to improved outcomes via disease-specific therapies.
Assuntos
Cardiomiopatia Restritiva , Gerenciamento Clínico , Cardiomiopatia Restritiva/diagnóstico , Cardiomiopatia Restritiva/etiologia , Cardiomiopatia Restritiva/fisiopatologia , Humanos , Miocárdio/patologia , Sarcoidose/diagnósticoAssuntos
Amiloidose de Cadeia Leve de Imunoglobulina/complicações , Amiloidose de Cadeia Leve de Imunoglobulina/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Cardiomiopatia Restritiva/etiologia , Feminino , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Amiloidose de Cadeia Leve de Imunoglobulina/patologia , Análise de Intenção de Tratamento , Masculino , Pessoa de Meia-Idade , Peptídeo Natriurético Encefálico/sangue , Fragmentos de Peptídeos/sangue , Análise de SobrevidaAssuntos
Cardiomiopatia Restritiva/diagnóstico por imagem , Fibrose Endomiocárdica/diagnóstico por imagem , Infecções por Vírus Epstein-Barr/complicações , Imagem Cinética por Ressonância Magnética/métodos , Peritonite/diagnóstico por imagem , Dor Abdominal/diagnóstico , Dor Abdominal/etiologia , Corticosteroides/uso terapêutico , Cardiomiopatia Restritiva/etiologia , Quimioterapia Combinada , Serviço Hospitalar de Emergência , Fibrose Endomiocárdica/tratamento farmacológico , Fibrose Endomiocárdica/etiologia , Infecções por Vírus Epstein-Barr/diagnóstico , Seguimentos , Furosemida/uso terapêutico , Humanos , Aumento da Imagem/métodos , Mesilato de Imatinib/uso terapêutico , Linfoma/complicações , Linfoma/virologia , Masculino , Peritonite/tratamento farmacológico , Terapia Trombolítica/métodos , Tomografia Computadorizada por Raios X/métodos , Adulto JovemAssuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Fibroelastose Endocárdica , Anuloplastia da Valva Mitral/métodos , Insuficiência da Valva Mitral , Adulto , Cardiomiopatia Restritiva/etiologia , Cardiomiopatia Restritiva/fisiopatologia , Cardiomiopatia Restritiva/cirurgia , Dissecação/métodos , Ecocardiografia Transesofagiana/métodos , Fibroelastose Endocárdica/complicações , Fibroelastose Endocárdica/diagnóstico , Fibroelastose Endocárdica/fisiopatologia , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Insuficiência da Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Mitral/etiologia , Insuficiência da Valva Mitral/fisiopatologia , Insuficiência da Valva Mitral/cirurgia , Resultado do Tratamento , Disfunção Ventricular/etiologia , Disfunção Ventricular/fisiopatologiaRESUMO
Hypereosinophilia-associated syndrome is a rare group of systemic diseases without certain underlying causes. Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), are initial considerations, when underlying causes remains unexplained despite of complete evaluation of hypereosinophilia. In this study, we report two rare cases, one case of HES with Loeffler endocarditis, and the other one of EGPA with restrictive cardiomyopathy mimicking myocardial infarction, to further address differential chief cardiac manifestations between HES and EGPA. Key roles of echocardiography played in detection of cardiac involvements, diagnosis, and prognosis prediction are also highlighted.
Assuntos
Medula Óssea/patologia , Cardiomiopatia Restritiva/etiologia , Síndrome Hipereosinofílica/complicações , Miocárdio/patologia , Biópsia , Cardiomiopatia Restritiva/diagnóstico , Diagnóstico Diferencial , Ecocardiografia , Seguimentos , Humanos , Síndrome Hipereosinofílica/diagnóstico , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X , UltrassonografiaAssuntos
Cardiomiopatia Restritiva/etiologia , Ecocardiografia Doppler em Cores/métodos , Fibrose Endomiocárdica/diagnóstico por imagem , Insuficiência da Valva Mitral/etiologia , Adulto , Biópsia por Agulha , Cardiomiopatia Restritiva/diagnóstico por imagem , Cardiomiopatia Restritiva/fisiopatologia , Progressão da Doença , Fibrose Endomiocárdica/complicações , Fibrose Endomiocárdica/patologia , Feminino , Seguimentos , Humanos , Imuno-Histoquímica , Insuficiência da Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Mitral/fisiopatologia , Medição de Risco , Fatores de Tempo , Clima TropicalRESUMO
Here, we report a rare case of isolated leukemic infiltrate of the myocardium (extramedullary involvement) presenting as restrictive cardiomyopathy in a patient in complete remission of acute myeloid leukemia. It was evaluated with multimodality imaging studies (echocardiography and cardiac MRI) and further confirmed by pathology. The present case highlights the importance of maintaining a high degree of clinical suspicion when evaluating patients with progressive ventricular hypertrophy of unknown cause, including recognition of the potential involvement by recurrent hematologic malignancy.
Assuntos
Cardiomiopatia Restritiva/diagnóstico , Ecocardiografia/métodos , Leucemia Mieloide Aguda/complicações , Infiltração Leucêmica/diagnóstico , Miocárdio/patologia , Cardiomiopatia Restritiva/etiologia , Humanos , Hipertrofia , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Indução de RemissãoAssuntos
Cardiomiopatia Restritiva/diagnóstico , Biomarcadores/sangue , Biomarcadores/urina , Exame de Medula Óssea , Cardiomiopatia Restritiva/etiologia , Cardiomiopatia Restritiva/terapia , Tomada de Decisão Clínica , Ecocardiografia Doppler , Eletrocardiografia , Humanos , Imageamento por Ressonância Magnética , Valor Preditivo dos Testes , Prognóstico , Cintilografia , Fatores de RiscoRESUMO
A 74-year-old female patient was referred to our department in 2015 with dyspnea, cough and dysphagia. She had been diagnosed with adenocarcinoma of the right breast in 1986 and underwent mastectomy. When she presented with a local recurrence in 1988, she was receiving high-voltage radiation therapy. Transthoracic echocardiography and magnetic resonance imaging revealed tricuspid regurgitation grade III and unclear right heart failure with a massively dilated right atrium. Coronary heart disease could be ruled out. In summary, the patient's findings represented right ventricular myocardial restriction which we attributed to irradiation of the right anterior chest 17 years previously.