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[SciELO Preprints] - Guidelines on the Diagnosis and Treatment of Hypertrophic Cardiomyopathy – 2024
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Fernandes, Fabio
Simões, Marcus V.
Correia, Edileide de Barros
Marcondes-Braga, Fabiana G.
Coelho-Filho, Otavio Rizzi
Mesquita, Cláudio Tinoco
Mathias-Junior, Wilson
Rochitte, Carlos Eduardo
Ramires, Felix José Alvarez
Alves, Silvia Marinho Martins
Montera, Marcelo Westerlund
Lopes, Renato Delascio
Oliveira-Junior, Mucio Tavares
Scolari, Fernando L.
Avila, Walkiria Samuel
Canesin, Manoel Fernandes
Bacal, Fernando
Bocchi, Edimar Alcides
Moura, Lídia Ana Zytynski
Saad, Eduardo Benchimol
Scanavacca, Mauricio I.
Valdigem, Bruno Pereira
Cano , Manuel Nicolas
Abizaid , Alexandre
Ribeiro, Henrique Barbosa
Lemos-Neto, Pedro Alves
Ribeiro, Gustavo Calado de Aguiar
Jatene, Fabio Biscegli
Dias, Ricardo Ribeiro
Beck-da-Silva, Luis
Rohde, Luis Eduardo P.
Bittencourt, Marcelo Imbroinise
Pereira, Alexandre
Krieger, José Eduardo
Villacorta, Humberto
Martins, Wolney de Andrade
Figueiredo-Neto, José Albuquerque de
Cardoso , Juliano Novaes
Pastore, Carlos Alberto
Jatene, Ieda Biscegli
Tanaka, Ana Cristina Sayuri
Hotta, Viviane Tiemi
Romano, Minna Moreira Dias
Albuquerque, Denilson Campos de
Mourilhe-Rocha, Ricardo
Hajjar, Ludhmila Abrahão
Brito, Fabio Sandoli de
Caramelli , Bruno
Calderaro, Daniela
Farsky, Pedro Silvio
Colafranceschi , Alexandre Siciliano
Pinto, Ibraim Masciarelli
Vieira , Marcelo Luiz Campos
Danzmann, Luiz Claudio
Barberato , Silvio Henrique
Mady, Charles
Martinelli-Filho, Martino
Torbey , Ana Flavia Malheiros
Schwartzmann, Pedro Vellosa
Macedo, Ariane Vieira Scarlatelli
Ferreira , Silvia Moreira Ayub
Schmidt, Andre
Melo , Marcelo Dantas Tavares de
Lima-Filho, Moysés Oliveira
Sposito, Andrei C.
Brito, Flavio de Souza
Biolo, Andreia
Madrini-Junior, Vagner
Rizk, Stéphanie Itala
Mesquita, Evandro Tinoco
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).
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).
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.
2.
Vena Cava Filter Misplacement: A Killer Traveler
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Garcia, Leonardo Rufino
; Garzesi, André Monti
; Martins, Antonio Sérgio
; Sobreira, Marcone Lima
; Brito, Flavio de Souza
; Felicio, Marcello Laneza
.
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Brazilian Journal of Cardiovascular Surgery
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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.
3.
Implications for Clinical Practice from a Multicenter Survey of Heart Failure Management Centers
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Bocchi, Edimar Alcides
; Moreira, Henrique Turin
; Nakamuta, Juliana Sanajotti
; Simões, Marcus Vinicius
; Casas, Alberto de Almeida Las
; Costa, Altamiro Reis da
; Assis, Amberson Vieira de
; Durães, André Rodrigues
; Pereira-Barretto, Antonio Carlos
; Ravessa, Antonio Delduque de Araujo
; Macedo, Ariane Vieira Scarlatelli
; Biselli, Bruno
; Pinto, Carolina Maria Nogueira
; Filho, Conrado Roberto Hoffmann
; Costantini, Costantino Roberto
; Almeida, Dirceu Rodrigues
; Santos Jr, Edval Gomes dos
; Soliva Junior, Erwin
; Figueiredo, Estevão Lanna
; Albuquerque, Felipe Neves de
; Paulitsch, Felipe
; Neuenschwander, Fernando Carvalho
; Figueiredo Neto, José Albuquerque de
; Brito, Flavio de Souza
; Lopes, Heno Ferreira
; Villacorta, Humberto
; Souza Neto, João David de
; Sepulveda, João Mariano
; Ayoub, José Carlos Aidar
; Vilela-Martin, José F.
; Cardoso, Juliano Novaes
; Uemura, Laercio
; Moura, Lidia Zytynski
; Maia, Lilia Nigro
; Oliveira, Lucia Brandão de
; Maia, Lucimir
; Silva, Luís Beck da
; Gowdak, Luís Henrique Wolff
; Danzmann, Luiz Claudio
; Andrade, Marcus
; Braile-Sternieri, Maria Christiane Valeria Braga
; Moreira, Maria da Consolação Vieira
; França Neto, Olimpio R
; Filho, Otavio Rizzi Coelho
; Esteves, Paulo Frederico
; Raupp-da-Rosa, Priscila
; Silva, Ricardo Jorge de Queiroz e
; Mourilhe-Rocha, Ricardo
; Viégas, Ruy Felipe Melo
; Rassi, Salvador
; Mangili, Sandrigo
; Kaiser, Sergio Emanuel
; Martins, Silvia Marinho
; Kawabata, Vitor Sergio
.
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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.
https://doi.org/10.6061/clinics/2021/e1991
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