RESUMO
Hypertrophic cardiomyopathy (HCM) is a form of genetically caused heart muscle disease, characterized by the thickening of the ventricular walls. Diagnosis requires detection through imaging methods (Echocardiogram or Cardiac Magnetic Resonance) showing any segment of the left ventricular wall with a thickness > 15 mm, without any other probable cause. Genetic analysis allows the identification of mutations in genes encoding different structures of the sarcomere responsible for the development of HCM in about 60% of cases, enabling screening of family members and genetic counseling, as an important part of patient and family management. Several concepts about HCM have recently been reviewed, including its prevalence of 1 in 250 individuals, hence not a rare but rather underdiagnosed disease. The vast majority of patients are asymptomatic. In symptomatic cases, obstruction of the left ventricular outflow tract (LVOT) is the primary disorder responsible for symptoms, and its presence should be investigated in all cases. In those where resting echocardiogram or Valsalva maneuver does not detect significant intraventricular gradient (> 30 mmHg), they should undergo stress echocardiography to detect LVOT obstruction. Patients with limiting symptoms and severe LVOT obstruction, refractory to beta-blockers and verapamil, should receive septal reduction therapies or use new drugs inhibiting cardiac myosin. Finally, appropriately identified patients at increased risk of sudden death may receive prophylactic measure with implantable cardioverter-defibrillator (ICD) implantation.
La miocardiopatía hipertrófica (MCH) es una forma de enfermedad cardíaca de origen genético, caracterizada por el engrosamiento de las paredes ventriculares. El diagnóstico requiere la detección mediante métodos de imagen (Ecocardiograma o Resonancia Magnética Cardíaca) que muestren algún segmento de la pared ventricular izquierda con un grosor > 15 mm, sin otra causa probable. El análisis genético permite identificar mutaciones en genes que codifican diferentes estructuras del sarcómero responsables del desarrollo de la MCH en aproximadamente el 60% de los casos, lo que permite el tamizaje de familiares y el asesoramiento genético, como parte importante del manejo de pacientes y familiares. Varios conceptos sobre la MCH han sido revisados recientemente, incluida su prevalencia de 1 entre 250 individuos, por lo tanto, no es una enfermedad rara, sino subdiagnosticada. La gran mayoría de los pacientes son asintomáticos. En los casos sintomáticos, la obstrucción del tracto de salida ventricular izquierdo (TSVI) es el trastorno principal responsable de los síntomas, y su presencia debe investigarse en todos los casos. En aquellos en los que el ecocardiograma en reposo o la maniobra de Valsalva no detecta un gradiente intraventricular significativo (> 30 mmHg), deben someterse a ecocardiografía de esfuerzo para detectar la obstrucción del TSVI. Los pacientes con síntomas limitantes y obstrucción grave del TSVI, refractarios al uso de betabloqueantes y verapamilo, deben recibir terapias de reducción septal o usar nuevos medicamentos inhibidores de la miosina cardíaca. Finalmente, los pacientes adecuadamente identificados con un riesgo aumentado de muerte súbita pueden recibir medidas profilácticas con el implante de un cardioversor-desfibrilador implantable (CDI).
A cardiomiopatia hipertrófica (CMH) é uma forma de doença do músculo cardíaco de causa genética, caracterizada pela hipertrofia das paredes ventriculares. O diagnóstico requer detecção por métodos de imagem (Ecocardiograma ou Ressonância Magnética Cardíaca) de qualquer segmento da parede do ventrículo esquerdo com espessura > 15 mm, sem outra causa provável. A análise genética permite identificar mutações de genes codificantes de diferentes estruturas do sarcômero responsáveis pelo desenvolvimento da CMH em cerca de 60% dos casos, permitindo o rastreio de familiares e aconselhamento genético, como parte importante do manejo dos pacientes e familiares. Vários conceitos sobre a CMH foram recentemente revistos, incluindo sua prevalência de 1 em 250 indivíduos, não sendo, portanto, uma doença rara, mas subdiagnosticada. A vasta maioria dos pacientes é assintomática. Naqueles sintomáticos, a obstrução do trato de saída do ventrículo esquerdo (OTSVE) é o principal distúrbio responsável pelos sintomas, devendo-se investigar a sua presença em todos os casos. Naqueles em que o ecocardiograma em repouso ou com Manobra de Valsalva não detecta gradiente intraventricular significativo (> 30 mmHg), devem ser submetidos à ecocardiografia com esforço físico para detecção da OTSVE. Pacientes com sintomas limitantes e grave OTSVE, refratários ao uso de betabloqueadores e verapamil, devem receber terapias de redução septal ou uso de novas drogas inibidoras da miosina cardíaca. Por fim, os pacientes adequadamente identificados com risco aumentado de morta súbita podem receber medida profilática com implante de cardiodesfibrilador implantável (CDI).
RESUMO
ABSTRACT Inferior vena cava filter embolization is not uncommon and can reach 11.8%. However, device migration to the heart is not frequent and occurs in cases after inferior vena cava filter fracture. We present the case of a young woman who was submitted to a routine inferior vena cava filter placement three days before and presented with hemodynamic instability. Since the device was not retrievable, the surgical team opted for an open cardiac surgery under cardiopulmonary bypass to remove the inferior vena cava filter and avoid other further complications.
RESUMO
Inferior vena cava filter embolization is not uncommon and can reach 11.8%. However, device migration to the heart is not frequent and occurs in cases after inferior vena cava filter fracture. We present the case of a young woman who was submitted to a routine inferior vena cava filter placement three days before and presented with hemodynamic instability. Since the device was not retrievable, the surgical team opted for an open cardiac surgery under cardiopulmonary bypass to remove the inferior vena cava filter and avoid other further complications.
Assuntos
Filtros de Veia Cava , Feminino , Humanos , Filtros de Veia Cava/efeitos adversos , Ponte Cardiopulmonar , Veia Cava Inferior , Resultado do TratamentoRESUMO
Heart allotransplantation has become one of the methods of choice in the treatment of severe heart failure. In the face of its difficulties, such as the unmet balance between organ supply and demand, the use of xenotransplantation (XTx) might be an attractive option shortly, even more with the ongoing progress achieved regarding the avoidance of hyperacute rejection and primary organ disfunction, maintenance of xenograft function and control of xenograft growth. To make possible this translational challenge, some points must be taken into account indeed, and they are the equipoise of human benefit and animal suffering, the risk of unknown infections, a well prepared informed consent, ethical and religious beliefs, and the role of cardiac XTx in a ventricular assistance device era.
Assuntos
Transplante de Coração , Animais , Rejeição de Enxerto/prevenção & controle , Xenoenxertos , Humanos , Transplante HeterólogoRESUMO
OBJECTIVES: This observational, cross-sectional study based aimed to test whether heart failure (HF)-disease management program (DMP) components are influencing care and clinical decision-making in Brazil. METHODS: The survey respondents were cardiologists recommended by experts in the field and invited to participate in the survey via printed form or email. The survey consisted of 29 questions addressing site demographics, public versus private infrastructure, HF baseline data of patients, clinical management of HF, performance indicators, and perceptions about HF treatment. RESULTS: Data were obtained from 98 centers (58% public and 42% private practice) distributed across Brazil. Public HF-DMPs compared to private HF-DMP were associated with a higher percentage of HF-DMP-dedicated services (79% vs 24%; OR: 12, 95% CI: 94-34), multidisciplinary HF (MHF)-DMP [84% vs 65%; OR: 3; 95% CI: 1-8), HF educational programs (49% vs 18%; OR: 4; 95% CI: 1-2), written instructions before hospital discharge (83% vs 76%; OR: 1; 95% CI: 0-5), rehabilitation (69% vs 39%; OR: 3; 95% CI: 1-9), monitoring (44% vs 29%; OR: 2; 95% CI: 1-5), guideline-directed medical therapy-HF use (94% vs 85%; OR: 3; 95% CI: 0-15), and less B-type natriuretic peptide (BNP) dosage (73% vs 88%; OR: 3; 95% CI: 1-9), and key performance indicators (37% vs 60%; OR: 3; 95% CI: 1-7). In comparison to non- MHF-DMP, MHF-DMP was associated with more educational initiatives (42% vs 6%; OR: 12; 95% CI: 1-97), written instructions (83% vs 68%; OR: 2: 95% CI: 1-7), rehabilitation (69% vs 17%; OR: 11; 95% CI: 3-44), monitoring (47% vs 6%; OR: 14; 95% CI: 2-115), GDMT-HF (92% vs 83%; OR: 3; 95% CI: 0-15). In addition, there were less use of BNP as a biomarker (70% vs 84%; OR: 2; 95% CI: 1-8) and key performance indicators (35% vs 51%; OR: 2; 95% CI: 91,6) in the non-MHF group. Physicians considered changing or introducing new medications mostly when patients were hospitalized or when observing worsening disease and/or symptoms. Adherence to drug treatment and non-drug treatment factors were the greatest medical problems associated with HF treatment. CONCLUSION: HF-DMPs are highly heterogeneous. New strategies for HF care should consider the present study highlights and clinical decision-making processes to improve HF patient care.
Assuntos
Gerenciamento Clínico , Insuficiência Cardíaca , Brasil , Estudos Transversais , Insuficiência Cardíaca/terapia , Humanos , Inquéritos e QuestionáriosRESUMO
OBJECTIVES: This observational, cross-sectional study based aimed to test whether heart failure (HF)-disease management program (DMP) components are influencing care and clinical decision-making in Brazil. METHODS: The survey respondents were cardiologists recommended by experts in the field and invited to participate in the survey via printed form or email. The survey consisted of 29 questions addressing site demographics, public versus private infrastructure, HF baseline data of patients, clinical management of HF, performance indicators, and perceptions about HF treatment. RESULTS: Data were obtained from 98 centers (58% public and 42% private practice) distributed across Brazil. Public HF-DMPs compared to private HF-DMP were associated with a higher percentage of HF-DMP-dedicated services (79% vs 24%; OR: 12, 95% CI: 94-34), multidisciplinary HF (MHF)-DMP [84% vs 65%; OR: 3; 95% CI: 1-8), HF educational programs (49% vs 18%; OR: 4; 95% CI: 1-2), written instructions before hospital discharge (83% vs 76%; OR: 1; 95% CI: 0-5), rehabilitation (69% vs 39%; OR: 3; 95% CI: 1-9), monitoring (44% vs 29%; OR: 2; 95% CI: 1-5), guideline-directed medical therapy-HF use (94% vs 85%; OR: 3; 95% CI: 0-15), and less B-type natriuretic peptide (BNP) dosage (73% vs 88%; OR: 3; 95% CI: 1-9), and key performance indicators (37% vs 60%; OR: 3; 95% CI: 1-7). In comparison to non- MHF-DMP, MHF-DMP was associated with more educational initiatives (42% vs 6%; OR: 12; 95% CI: 1-97), written instructions (83% vs 68%; OR: 2: 95% CI: 1-7), rehabilitation (69% vs 17%; OR: 11; 95% CI: 3-44), monitoring (47% vs 6%; OR: 14; 95% CI: 2-115), GDMT-HF (92% vs 83%; OR: 3; 95% CI: 0-15). In addition, there were less use of BNP as a biomarker (70% vs 84%; OR: 2; 95% CI: 1-8) and key performance indicators (35% vs 51%; OR: 2; 95% CI: 91,6) in the non-MHF group. Physicians considered changing or introducing new medications mostly when patients were hospitalized or when observing worsening disease and/or symptoms. Adherence to drug treatment and non-drug treatment factors were the greatest medical problems associated with HF treatment. CONCLUSION: HF-DMPs are highly heterogeneous. New strategies for HF care should consider the present study highlights and clinical decision-making processes to improve HF patient care.
Assuntos
Humanos , Gerenciamento Clínico , Insuficiência Cardíaca/terapia , Brasil , Estudos Transversais , Inquéritos e QuestionáriosRESUMO
O transplante cardíaco está indicado para pacientes cominsuficiência cardíaca grave com sintomas incapacitantes, a despeitode estarem recebendo tratamento farmacológico otimizado e deterem se esgotado as possibilidades cirúrgicas de tratamento. Entreoutros fatores, as limitações referentes à disponibilidade de doadoresde coração efetivos, principalmente no Brasil, tornam a escolhacriteriosa do receptor cardíaco, além de um desafio, uma granderesponsabilidade para a prática diária do cardiologista clínico.Hiponatremia, insuficiência renal, caquexia cardíaca, anemia, baixosníveis de colesterol, queda dos níveis de hemoglobina e dependênciade inotrópicos são fatores clínico-laboratoriais associados a mauprognóstico em pacientes com insuficiência cardíaca (IC) avançada.Os escores de risco [HFSS (Heart Failure Survival Score) eSHFM (Seattle Heart Failure Model)] devem ser utilizados comoferramentas acessórias para avaliar a gravidade dos pacientes comIC avançada. VO2 pico < 10 ml/kg/min e VE/VCO2 > 34 no teste doexercício cardiopulmonar estão relacionados com mortalidade empacientes ambulatorialmente avaliados para transplante cardíaco. Ahemodinâmica pulmonar, assim como o perfil imunológico, deveser rotineiramente acessada em pacientes candidatos a transplantecardíaco. Os pacientes com hipertensão pulmonar fixa estão sobrisco de desenvolverem insuficiência cardíaca direita fatal apóso transplante cardíaco. Por sua vez, a presença de anticorposcirculantes contra os antígenos HLA (Human Leukocyte Antigen)é um fator de risco para rejeição do enxerto após o transplantede coração. Finalmente, a indicação do transplante cardíaco emsituações clínicas extremas vem aumentando e se tornando umdesafio na prática clínica cardiológica.
The heart transplantation is indicated for patients with severeheart failure and disabling symptoms despite being receivingoptimal pharmacological treatment and having exhausted thepossibilities of surgical treatment. Among other factors, thelimitations related to the availability of effective heart donor,mainly in Brazil, make a judicious choice of the heart receptorin addition to a challenge, a huge responsibility for the dailypractice of clinical cardiologists. Hyponatremia, renal failure,cardiac cachexia, anemia, low cholesterol levels, decrease inhemoglobin levels and dependence on inotropes are clinical andlaboratory factors associated with poor prognosis in patientswith advanced heart failure. The risk scores [HFSS (HeartFailure Survival Score) and SHFM (Seattle Heart FailureModel)] should be used as ancillary tools to assess the severityof patients with advanced HF. Peak of VO2 < 10 ml/kg/minand VE/VCO2 > 34 during the cardio-pulmonary exercise testare related to mortality in ambulatory patients evaluated forcardiac transplantation. Pulmonary hemodynamics as wellas the immunological profile should be routinely accessed inpatients who are candidates for cardiac transplantation. Patientswith fixed pulmonary hypertension are at risk for fatal rightheart failure after heart transplantation. In turn, the presenceof circulating antibodies to Human Leukocyte Antigen HLA)is a risk factor for graft rejection after heart transplantation.Finally, the indication of cardiac transplantation in extremeclinical situations are increasing and becoming a challenge incardiology clinical practice.
