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1.
Almeida, André Luiz Cerqueira; Melo, Marcelo Dantas Tavares de; Bihan, David Costa de Souza Le; Vieira, Marcelo Luiz Campos; Pena, José Luiz Barros; Del Castillo, José Maria; Abensur, Henry; Hortegal, Renato de Aguiar; Otto, Maria Estefania Bosco; Piveta, Rafael Bonafim; Dantas, Maria Rosa; Assef, Jorge Eduardo; Beck, Adenalva Lima de Souza; Santo, Thais Harada Campos Espirito; Silva, Tonnison de Oliveira; Salemi, Vera Maria Cury; Rocon, Camila; Lima, Márcio Silva Miguel; Barberato, Silvio Henrique; Rodrigues, Ana Clara; Rabschkowisky, Arnaldo; Frota, Daniela do Carmo Rassi; Gripp, Eliza de Almeida; Barretto, Rodrigo Bellio de Mattos; Silva, Sandra Marques e; Cauduro, Sanderson Antonio; Pinheiro, Aurélio Carvalho; Araujo, Salustiano Pereira de; Tressino, Cintia Galhardo; Silva, Carlos Eduardo Suaide; Monaco, Claudia Gianini; Paiva, Marcelo Goulart; Fisher, Cláudio Henrique; Alves, Marco Stephan Lofrano; Grau, Cláudia R. Pinheiro de Castro; Santos, Maria Veronica Camara dos; Guimarães, Isabel Cristina Britto; Morhy, Samira Saady; Leal, Gabriela Nunes; Soares, Andressa Mussi; Cruz, Cecilia Beatriz Bittencourt Viana; Guimarães Filho, Fabio Villaça; Assunção, Bruna Morhy Borges Leal; Fernandes, Rafael Modesto; Saraiva, Roberto Magalhães; Tsutsui, Jeane Mike; Soares, Fábio Luis de Jesus; Falcão, Sandra Nívea dos Reis Saraiva; Hotta, Viviane Tiemi; Armstrong, Anderson da Costa; Hygidio, Daniel de Andrade; Miglioranza, Marcelo Haertel; Camarozano, Ana Cristina; Lopes, Marly Maria Uellendahl; Cerci, Rodrigo Julio; Siqueira, Maria Eduarda Menezes de; Torreão, Jorge Andion; Rochitte, Carlos Eduardo; Felix, Alex.
Arq. bras. cardiol ; 120(12): e20230646, dez. 2023. tab, graf
Artigo em Português | LILACS-Express | LILACS, SESSP-IDPCPROD, Sec. Est. Saúde SP | ID: biblio-1527794
2.
Arq Bras Cardiol ; 120(12): e20230646, 2023 Dec.
Artigo em Português, Inglês | MEDLINE | ID: mdl-38232246

RESUMO

Central Illustration : Position Statement on the Use of Myocardial Strain in Cardiology Routines by the Brazilian Society of Cardiology's Department Of Cardiovascular Imaging - 2023 Proposal for including strain in the integrated diastolic function assessment algorithm, adapted from Nagueh et al.67 Am: mitral A-wave duration; Ap: reverse pulmonary A-wave duration; DD: diastolic dysfunction; LA: left atrium; LASr: LA strain reserve; LVGLS: left ventricular global longitudinal strain; TI: tricuspid insufficiency. Confirm concentric remodeling with LVGLS. In LVEF, mitral E wave deceleration time < 160 ms and pulmonary S-wave < D-wave are also parameters of increased filling pressure. This algorithm does not apply to patients with atrial fibrillation (AF), mitral annulus calcification, > mild mitral valve disease, left bundle branch block, paced rhythm, prosthetic valves, or severe primary pulmonary hypertension.


Figura Central : Posicionamento do Departamento de Imagem Cardiovascular da Sociedade Brasileira de Cardiologia sobre o Uso do Strain Miocárdico na Rotina do Cardiologista ­ 2023 Proposta de inclusão do strain no algoritmo integrado de avaliação da função diastólica, adaptado e traduzido de Nagueh et al. 67 AE: átrio esquerdo; Ap: duração da onda A reversa pulmonar; Am: duração da onda A mitral; DD: disfunção diastólica; FEVEr: fração de ejeção do ventrículo esquerdo reduzida; IT: insuficiência tricúspide; SAEr: strain do AE de reservatório; SLGVE: strain longitudinal global do ventrículo esquerdo. Se remodelamento concêntrico, confirmar com SLGVE. Na presença de FEVEr, tempo de desaceleração da onda E mitral (TDE) < 160 ms e onda S < D pulmonar também são parâmetros de pressão de enchimento aumentada. Esse algoritmo não se aplica a pacientes com fibrilação atrial (FA), calcificação do anel mitral ou valvopatia mitral maior que discreta, bloqueio de ramo esquerdo (BRE), ritmo de marca-passo, próteses valvares ou hipertensão pulmonar (HP) primária grave.


Assuntos
Fibrilação Atrial , Cardiologia , Disfunção Ventricular Esquerda , Humanos , Ecocardiografia Doppler , Brasil , Fibrilação Atrial/diagnóstico por imagem , Átrios do Coração/diagnóstico por imagem , Função Ventricular Esquerda
3.
Melo, Marcelo Dantas Tavares de; Paiva, Marcelo Goulart; Santos, Maria Verônica Câmara; Rochitte, Carlos Eduardo; Moreira, Valéria de Melo; Saleh, Mohamed Hassan; Soares, Brandão, Simone Cristina; Gallafrio, Claudia Cosentino; Goldwasser, Daniel; Gripp, Eliza de Almeida; Piveta, Rafael Bonafim; Silva, Tonnison Oliveira; Santo, Thais Harada Campos Espirito; Ferreira, Waldinai Pereira; Salemi, Vera Maria Cury; Cauduro, Sanderson A; Barberato, Silvio Henrique; Lopes, Heloísa M Christovam; Pena, José Luiz Barros; Rached, Heron Rhydan Saad; Miglioranza, Marcelo Haertel; Pinheiro, Aurélio Carvalho; Vrandecic, Bárbara Athayde Linhares Martins; Cruz, Cecilia Beatriz Bittencourt Viana; Nomura, César Higa; Cerbino, Fernanda Mello Erthal; Costa, Isabela Bispo Santos da Silva; Coelho-Filho, Otavio Rizzi; Carneiro, Adriano Camargo de Castro; Burgos, Ursula Maria Moreira Costa; Fernandes, Juliano Lara; Uellendahl, Marly; Calado, Eveline Barros; Senra, Tiago; Assunção, Bruna Leal; Freire, Claudia Maria Vilas; Martins, Cristiane Nunes; Sawamura, Karen Saori Shiraishi; Brito, Márcio Miranda; Jardim, Maria Fernanda Silva; Bernardes, Renata Junqueira Moll; Diógenes, Tereza Cristina; Vieira, Lucas de Oliveira; Mesquita, Claudio Tinoco; Lopes, Rafael Willain; Neto, Elry Medeiros Vieira Segundo; Rigo, Letícia; Marin, Valeska Leite Siqueira; Santos, Marcelo José; Grossman, Gabriel Blacher; Quagliato, Priscila Cestari; Alcantara, Monica Luiza de; Teodoro, José Aldo Ribeiro; Albricker, Ana Cristina Lopes; Barros, Fanilda Souto; Amaral, Salomon Israel do; Porto, Carmen Lúcia Lascasas; Barros, Marcio Vinícius Lins; Santos, Simone Nascimento dos; Cantisano, Armando Luís; Petisco, Ana Cláudia Gomes Pereira; Barbosa, José Eduardo Martins; Veloso, Orlando Carlos Glória; Spina, Salvador; Pignatelli, Ricardo; Hajjar, Ludhmilla Abrahão; Filho, Roberto Kalil; Lopes, Marcelo Antônio Cartaxo Queiroga; Vieira, Marcelo Luiz Campos; Almeida, André Luiz Cerqueira.
Arq. bras. cardiol ; 117(4): 845-909, Oct. 2021. graf, ilus, tab
Artigo em Português | CONASS, Sec. Est. Saúde SP, SESSP-IDPCPROD, Sec. Est. Saúde SP | ID: biblio-1344557
4.
Arq Bras Cardiol ; 117(4): 845-909, 2021 10.
Artigo em Inglês, Português | MEDLINE | ID: mdl-34709307
5.
J Am Soc Echocardiogr ; 31(5): 578-586, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-29426649

RESUMO

BACKGROUND: Exercise intolerance is the most common symptom in hypertrophic cardiomyopathy (HCM). We examined whether inability to augment myocardial mechanics during exercise would influence functional performance and clinical outcomes in HCM. METHODS: Ninety-five HCM patients (32 nonobstructive, 32 labile-obstructive, 31 obstructive) and 26 controls of similar age and gender distribution were recruited prospectively. They underwent rest and treadmill stress strain echocardiography, and 61 of them underwent magnetic resonance imaging. Mechanical reserve (MRES) was defined as percent change in systolic strain rate (SR) immediately postexercise. RESULTS: Global strain and SR were significantly lower in HCM patients at rest (strain: nonobstructive, -15.6 ± 3.0; labile-obstructive, -15.9 ± 3.0; obstructive, -13.8 ± 2.9; control, -17.7% ± 2.1%, P < .001; SR: nonobstructive, -0.92 ± 0.20; labile-obstructive, -0.94 ± 0.17; obstructive, -0.85 ± 0.18; control, -1.04 ± 0.14 s-1, P = .002); and immediately postexercise (strain: nonobstructive, -15.6 ± 3.0; labile-obstructive, -17.6 ± 3.6; obstructive, -15.6 ± 3.6; control, -19.2 ± 3.1%; P = .001; SR: nonobstructive, -1.41 ± 0.37; labile-obstructive, -1.64 ± 0.38; obstructive, -1.32 ± 0.29; control, -1.82 ± 0.29 s-1, P < .001). MRES was lower in nonobstructive and obstructive compared with labile-obstructive and controls (51% ± 29%, 54% ± 31%, 78% ± 38%, 77% ± 30%, P = .001, respectively). Postexercise SR and MRES were associated with exercise capacity (r = 0.47 and 0.42, P < .001 both, respectively). When adjusted for age, gender, body mass index, E/e', and resting peak instantaneous systolic gradient, postexercise SR best predicted exercise capacity (r = 0.74, P = .003). Postexercise SR was correlated with extent of late gadolinium enhancement (r = 0.34, P = .03). By Cox regression, exercise SR and MRES predicted ventricular tachycardia/ventricular fibrillation (VT/VF) even after adjustment for age, gender, family history of sudden cardiac death, septum ≥ 3 cm and abnormal blood pressure response (P = .04 and P = .046, respectively). CONCLUSIONS: Nonobstructive and obstructive patients have reduced MRES compared with labile-obstructive and controls. Postexercise SR correlates with LGE and exercise capacity. Exercise SR and MRES predict VT/VF.


Assuntos
Cardiomiopatia Hipertrófica/fisiopatologia , Ecocardiografia sob Estresse/métodos , Tolerância ao Exercício/fisiologia , Ventrículos do Coração/diagnóstico por imagem , Descanso/fisiologia , Função Ventricular Esquerda/fisiologia , Cardiomiopatia Hipertrófica/diagnóstico , Progressão da Doença , Feminino , Seguimentos , Ventrículos do Coração/fisiopatologia , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Prognóstico , Curva ROC , Estudos Retrospectivos , Sístole
6.
Am Heart J ; 184: 47-54, 2017 02.
Artigo em Inglês | MEDLINE | ID: mdl-27892886

RESUMO

BACKGROUND: Exercise echocardiography in the evaluation of hypertrophic cardiomyopathy (HCM) provides valuable information for risk stratification, selection of optimal treatment, and prognostication. However, HCM patients with left ventricular outflow tract gradients ≥30mm Hg are often excluded from exercise testing because of safety considerations. We examined the safety and utility of exercise testing in patients with high-gradient HCM. METHODS: We evaluated clinical characteristics, hemodynamics, and imaging variables in 499 consecutive patients with HCM who performed 959 exercise tests. Patients were divided based on peak left ventricular outflow tract gradients using a 30-mm Hg threshold into the following: obstructive (n=152), labile-obstructive (n=178), and nonobstructive (n=169) groups. RESULTS: There were no deaths during exercise testing. We noted 20 complications (2.1% of tests) including 3 serious ventricular arrhythmias (0.3% of tests). There was no difference in complication rate between groups. Patients with obstructive HCM had a higher frequency of abnormal blood pressure response (obstructive: 53% vs labile: obstructive: 41% and nonobstructive: 37%; P=.008). Obstructive patients also displayed a lower work capacity (obstructive: 8.4±3.4 vs labile obstructive: 10.9±4.2 and nonobstructive: 10.2±4.0, metabolic equivalent; P<.001). Exercise testing provided incremental information regarding sudden cardiac death risk in 19% of patients with high-gradient HCM, and we found a poor correlation between patient-reported functional class and work capacity. CONCLUSION: Our results suggest that exercise testing in HCM is safe, and serious adverse events are rare. Although numbers are limited, exercise testing in high-gradient HCM appears to confer no significant additional safety hazard in our selected cohort and could potentially provide valuable information.


Assuntos
Arritmias Cardíacas/etiologia , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Ecocardiografia sob Estresse/efeitos adversos , Teste de Esforço/efeitos adversos , Síncope/etiologia , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem , Adulto , Idoso , Angina Pectoris/etiologia , Cardiomiopatia Hipertrófica/fisiopatologia , Dispneia/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taquicardia Ventricular/etiologia , Fibrilação Ventricular/etiologia , Obstrução do Fluxo Ventricular Externo/fisiopatologia
7.
Am J Cardiol ; 117(11): 1815-20, 2016 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-27084053

RESUMO

To date, there has not been a large systematic examination of the hypertrophic cardiomyopathy (HC) phenotype in blacks versus whites. In this study, we investigate differences in presentation of HC between blacks and whites. We included 441 consecutive patients with HC seen at the Johns Hopkins HC clinic in the period from February 2005 to June 2012. We compared 76 blacks for clinical presentation, electrocardiogram, exercise capacity, left ventricular morphology, and hemodynamics by echocardiography to 365 whites. Black patients with HC more often presented with abnormal electrocardiogram (93% vs 80%, p = 0.009), driven by a significant difference in repolarization abnormalities (79% vs 56%, p <0.001). Apical hypertrophy was more common in blacks (26% vs 9%, p <0.001); however, blacks had less severe systolic anterior movement of the mitral valve and had significantly lower left ventricular outflow tract gradients at rest (9 mm Hg; interquartile range [IQR] 7 to 19 vs 16 mm Hg; IQR 8 to 40, p <0.001) and during provocation (36 mm Hg; IQR 16 to 77 vs 59 mm Hg; IQR 26 to 110, p = 0.002). Despite the nonobstructive pathophysiology, blacks had lower exercise capacity (adjusted difference 1.45 metabolic equivalents [0.45 to 2.45], p = 0.005). In conclusion, blacks have an HC phenotype characterized by lower prevalence of the well-recognized echocardiographic features of HC such as systolic anterior movement of the mitral valve and left ventricular outflow tract obstruction and display worse exercise capacity.


Assuntos
Negro ou Afro-Americano , Cardiomiopatia Hipertrófica/etnologia , Ecocardiografia , Ventrículos do Coração/diagnóstico por imagem , Sistema de Registros , População Branca , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/fisiopatologia , Estudos Transversais , Eletrocardiografia , Feminino , Seguimentos , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Maryland/epidemiologia , Pessoa de Meia-Idade , Morbidade/tendências , Fenótipo , Prognóstico , Estudos Prospectivos , Fatores de Tempo
8.
Am J Cardiol ; 116(6): 938-44, 2015 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-26239580

RESUMO

Patients with nonobstructive hypertrophic cardiomyopathy (HC) are considered low risk, generally not requiring aggressive intervention. However, nonobstructive and labile-obstructive HC have been traditionally classified together, and it is unknown if these 2 subgroups have distinct risk profiles. We compared cardiovascular outcomes in 293 patients HC (96 nonobstructive, 114 labile-obstructive, and 83 obstructive) referred for exercise echocardiography and magnetic resonance imaging and followed for 3.3 ± 3.6 years. A subgroup (34 nonobstructive, 28 labile-obstructive, 21 obstructive) underwent positron emission tomography. The mean number of sudden cardiac death risk factors was similar among groups (nonobstructive: 1.4 vs labile-obstructive: 1.2 vs obstructive: 1.4 risk factors, p = 0.2). Prevalence of late gadolinium enhancement (LGE) was similar across groups but more non-obstructive patients had late gadolinium enhancement ≥20% of myocardial mass (23 [30%] vs 19 [18%] labile-obstructive and 8 [11%] obstructive, p = 0.01]. Fewer labile-obstructive patients had regional positron emission tomography perfusion abnormalities (12 [46%] vs nonobstructive 30 [81%] and obstructive 17 [85%], p = 0.003]. During follow-up, 60 events were recorded (36 ventricular tachycardia/ventricular fibrillation, including 30 defibrillator discharges, 12 heart failure worsening, and 2 deaths). Nonobstructive patients were at greater risk of VT/VF at follow-up, compared to labile obstructive (hazed ratio 0.18, 95% confidence interval 0.04 to 0.84, p = 0.03) and the risk persisted after adjusting for age, gender, syncope, family history of sudden cardiac death, abnormal blood pressure response, and septum ≥3 cm (p = 0.04). Appropriate defibrillator discharges were more frequent in nonobstructive (8 [18%]) compared to labile-obstructive (0 [0%], p = 0.02) patients. In conclusion, nonobstructive hemodynamics is associated with more pronounced fibrosis and ischemia than labile-obstructive and is an independent predictor of VT/VF in HC.


Assuntos
Cardiomiopatia Hipertrófica/fisiopatologia , Insuficiência Cardíaca/fisiopatologia , Taquicardia Ventricular/etiologia , Fibrilação Ventricular/etiologia , Obstrução do Fluxo Ventricular Externo/fisiopatologia , Adulto , Idoso , Cardiomiopatia Hipertrófica/classificação , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/diagnóstico , Estudos de Coortes , Progressão da Doença , Ecocardiografia sob Estresse , Feminino , Coração/diagnóstico por imagem , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/diagnóstico , Humanos , Estimativa de Kaplan-Meier , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Imagem de Perfusão do Miocárdio , Miocárdio/patologia , Modelos de Riscos Proporcionais , Obstrução do Fluxo Ventricular Externo/classificação , Obstrução do Fluxo Ventricular Externo/diagnóstico , Obstrução do Fluxo Ventricular Externo/etiologia
9.
Echocardiography ; 31(9): 1131-7, 2014 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-25070288

RESUMO

UNLABELLED: Cardiac disease frequently occurs in children with chronic kidney disease (CKD) undergoing dialysis (DI), but it is not well studied in patients undergoing conservative treatment (CT). The aim of our study was to use echocardiography to analyze and compare the cardiac involvement of children with CKD undergoing DI or CT. METHODS: Seventy-one children with CKD were included; 41 undergoing DI and 30 undergoing CT. There were 33 controls. Measurements of arterial pressure and structural and functional echocardiographic variables were obtained; the children were followed up for 18 months. Tests of comparison and multiple regression were used; significant if P < 0.05. RESULTS: Arterial hypertension (AH) was present in 37 of 71 (52%) children with CKD: 27 (65.8%) in DI and 10 (33.3%) in CT (X2 = 8.7; P = 0.003). An abnormal left ventricular geometric pattern was present in 37/41 (90.3%) undergoing DI, 33 had left ventricular hypertrophy (LVH), and in 14/30 (46.7%) undergoing CT, 5 had LVH. Ejection fraction was normal in all groups; diastolic function alteration (DFA) occurred in 28/41 (68.3%) children on DI and in 10/30 (33.3%) on CT (X2 = 9.2; P = 0.002). For children with CKD, DI (P = 0.002) and hypertension (P = 0.04) were associated with LVH; among those on DI, only AH was associated with LVH (P = 0.02). During the follow-up, 18 (43.9%) children undergoing DI had at least one cardiovascular event. CONCLUSION: Children with CKD undergoing CT had less cardiac involvement than those undergoing DI. LVH was associated with DI and AH in all children with CKD and with AH in those on DI.


Assuntos
Ventrículos do Coração/diagnóstico por imagem , Hipertensão/complicações , Hipertrofia Ventricular Esquerda/complicações , Diálise Renal/métodos , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/terapia , Análise de Variância , Pressão Sanguínea , Criança , Ecocardiografia Doppler/métodos , Feminino , Seguimentos , Humanos , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Masculino
10.
Am J Cardiol ; 112(8): 1182-9, 2013 Oct 15.
Artigo em Inglês | MEDLINE | ID: mdl-24079444

RESUMO

Obesity is independently associated with left ventricular (LV) hypertrophy and thus may be an important modifier of the hypertrophic cardiomyopathy (HC) phenotype. We examined if obesity modifies the clinical presentation, LV morphology, outflow hemodynamics, and exercise tolerance in HC. In this cross-sectional study, 88 obese (body mass index [BMI] ≥30 kg/m(2)) and 154 nonobese (BMI <30 kg/m(2)) patients from the Johns Hopkins HC clinic were compared with respect to a variety of clinical and LV echocardiographic measurements. Obese patients (36.4%) were more likely to report exertional dyspnea (p = 0.04) and chest pain (p = 0.002) and had greater prevalence of hypertension (p = 0.008). LV posterior wall thickness (p = 0.01) but not the septal wall (p ≥0.21) was significantly greater in obese patients, resulting in an increased LV mass index (p = 0.003). No significant differences in LV systolic and diastolic functions were observed, but obesity was associated with higher LV stroke volume (p = 0.03), inducible LV outflow tract gradients (p = 0.045), and chance of developing LV outflow tract obstruction during stress (p = 0.035). In multivariate analysis, BMI was associated with increased posterior (but not septal) wall thickness (ß = 0.15, p = 0.02) and LV mass index (ß = 0.18, p = 0.005), particularly in those with hypertension. Obesity was also associated with reduced exercise time and functional capacity, and BMI independently correlated with reduced exercise tolerance. In conclusion, obesity is associated with larger LV mass, worse symptoms, lower exercise tolerance, and labile obstructive hemodynamics in HC. The association with increased outflow tract gradients has particular importance as contribution of obesity to the pressure gradients may influence clinical decisions in labile obstructive HC.


Assuntos
Cardiomiopatia Hipertrófica/diagnóstico por imagem , Tolerância ao Exercício/fisiologia , Ventrículos do Coração/fisiopatologia , Hemodinâmica/fisiologia , Hipertrofia Ventricular Esquerda/fisiopatologia , Obesidade/complicações , Índice de Massa Corporal , Cardiomiopatia Hipertrófica/etiologia , Cardiomiopatia Hipertrófica/fisiopatologia , Estudos Transversais , Ecocardiografia , Feminino , Seguimentos , Ventrículos do Coração/diagnóstico por imagem , Humanos , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Hipertrofia Ventricular Esquerda/etiologia , Masculino , Pessoa de Meia-Idade , Obesidade/fisiopatologia , Estudos Prospectivos
11.
Am J Cardiol ; 112(6): 861-6, 2013 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-23751935

RESUMO

A lethal and extensively characterized familial form of hypertrophic cardiomyopathy (HC) is due to a point mutation (Arg403Gln) in the cardiac ß-myosin heavy chain gene. Although this is associated with abnormal energy metabolism and progression to heart failure in an animal model, in vivo cardiac energetics have not been characterized in patients with this mutation. Noninvasive phosphorus saturation transfer magnetic resonance spectroscopy was used to measure the adenosine triphosphate supplied by the creatine kinase (CK) reaction and phosphocreatine, the heart's primary energy reserve, in 9 of 10 patients from a single kindred with HC caused by the Arg403GIn mutation and 17 age-matched healthy controls. Systolic and diastolic function was assessed by echocardiography in all 10 patients with HC. The patients with HC had impairment of diastolic function and mild systolic dysfunction, when assessed using global systolic longitudinal strain. Myocardial phosphocreatine was significantly decreased by 24% in patients (7.1 ± 2.3 µmol/g) compared with the controls (9.4 ± 1.2 µmol/g; p = 0.003). The pseudo-first-order CK rate-constant was 26% lower (0.28 ± 0.15 vs 0.38 ± 0.07 s⁻¹, p = 0.035) and the forward CK flux was 44% lower (2.0 ± 1.4 vs 3.6 ± 0.9 µmol/g/s, p = 0.001) than in the controls. The contractile abnormalities did not correlate with the metabolic indexes. In conclusion, myocardial phosphocreatine and CK-ATP delivery are significantly reduced in patients with HC caused by the Arg403Gln mutation, akin to previous results from mice with the same mutation. A lack of a relation between energetic and contractile abnormalities suggests the former result from the sarcomeric mutation and not a late consequence of mechanical dysfunction.


Assuntos
Trifosfato de Adenosina/sangue , Cardiomiopatia Hipertrófica/sangue , Creatina Quinase/sangue , Metabolismo Energético , Fosfocreatina/sangue , Adulto , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/fisiopatologia , DNA/genética , Análise Mutacional de DNA , Progressão da Doença , Ecocardiografia , Feminino , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Espectroscopia de Ressonância Magnética , Masculino , Mutação , Cadeias Pesadas de Miosina/genética , Prognóstico
12.
J Cardiovasc Transl Res ; 6(4): 604-15, 2013 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-23771430

RESUMO

We examined whether there is a relationship between repolarization abnormalities on electrocardiography (EKG) and deformation abnormalities by echocardiography. Analysis of baseline EKGs and mechanical (echo-based deformation) changes was performed in 128 patients with a clinical diagnosis of hypertrophic cardiomyopathy (HCM). Patients with left ventricular hypertrophy (LVH) or repolarization abnormalities had higher septal thickness when compared to patients with normal EKG. Patients with EKG evidence of LVH or QTc prolongation had lower systolic velocity, systolic strain, systolic strain rate, late diastolic velocity, and late diastolic strain rate than patients with a normal EKG. Patients with strain pattern or ST depression/T-wave inversion had lower systolic velocity, systolic strain, systolic strain rate, early diastolic velocity, and late diastolic velocity when compared to patients with normal EKGs. LVH and repolarization abnormalities on surface EKG are markers of impaired systolic and diastolic mechanics in HCM.


Assuntos
Cardiomiopatia Hipertrófica/fisiopatologia , Sistema de Condução Cardíaco/fisiopatologia , Hipertrofia Ventricular Esquerda/fisiopatologia , Função Ventricular Esquerda , Potenciais de Ação , Adulto , Idoso , Fenômenos Biomecânicos , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Distribuição de Qui-Quadrado , Diástole , Ecocardiografia Doppler , Eletrocardiografia , Feminino , Sistema de Condução Cardíaco/diagnóstico por imagem , Humanos , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Sistema de Registros , Sístole , Fatores de Tempo
13.
Circ Cardiovasc Imaging ; 6(2): 210-7, 2013 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-23418294

RESUMO

BACKGROUND: Presence of delayed enhancement (DE) on cardiac magnetic resonance (CMR) is associated with worse clinical outcomes in hypertrophic cardiomyopathy. We investigated the relationship between DE on CMR and myocardial ischemia in hypertrophic cardiomyopathy. METHODS AND RESULTS: Hypertrophic cardiomyopathy patients (n=47) underwent CMR for assessment of DE and vasodilator stress ammonia positron emission tomography to quantify myocardial blood flow and coronary flow reserve. The summed difference score for regional myocardial perfusion was also assessed. Patients in the DE group (n=35) had greater left ventricular wall thickness (2.09±0.44 versus 1.78±0.34 cm; P=0.03). Stress myocardial blood flow (2.25±0.46 versus 1.78±0.43 mL/min per gram; P=0.01) and coronary flow reserve (2.78±0.32 versus 2.01±0.52; P<0.001) were significantly lower in DE-positive patients. Summed difference score (7.3±6.6 versus 0.9±1.4; P<0.0001) was significantly higher in patients with DE. A coronary flow reserve <2.00 was seen in 18 patients (51%) with DE but in none of the DE-negative patients (P<0.0001). CMR and positron emission tomography showed visually concordant DE and regional myocardial perfusion abnormalities in 31 patients and absence of DE and perfusion defects in 9 patients. Four DE-positive patients demonstrated normal regional myocardial perfusion, and 3 DE-negative patients had (apical) regional myocardial perfusion abnormalities. CONCLUSIONS: We found a close relationship between DE by CMR and microvascular function in most of the patients studied. However, a small proportion of patients had DE in the absence of perfusion abnormalities, suggesting that microvascular dysfunction and ischemia are not the sole causes of DE in hypertrophic cardiomyopathy patients.


Assuntos
Cardiomiopatia Hipertrófica/diagnóstico , Circulação Coronária , Imageamento por Ressonância Magnética , Isquemia Miocárdica/diagnóstico , Imagem de Perfusão do Miocárdio/métodos , Tomografia por Emissão de Pósitrons , Adulto , Idoso , Análise de Variância , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Cardiomiopatia Hipertrófica/fisiopatologia , Distribuição de Qui-Quadrado , Estudos Transversais , Feminino , Reserva Fracionada de Fluxo Miocárdico , Humanos , Masculino , Microcirculação , Pessoa de Meia-Idade , Isquemia Miocárdica/diagnóstico por imagem , Isquemia Miocárdica/fisiopatologia , Valor Preditivo dos Testes , Prognóstico , Vasodilatadores
14.
PLoS One ; 7(7): e41974, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22860042

RESUMO

OBJECTIVES: To quantify resting myocardial blood flow (MBF) in the left ventricular (LV) wall of HCM patients and to determine the relationship to important parameters of disease: LV wall thickness, late gadolinium enhancement (LGE), T2-signal abnormalities (dark and bright signal), LV outflow tract obstruction and age. MATERIALS AND METHODS: Seventy patients with proven HCM underwent cardiac MRI. Absolute and relative resting MBF were calculated from cardiac perfusion MRI by using the Fermi function model. The relationship between relative MBF and LV wall thickness, T2-signal abnormalities (T2 dark and T2 bright signal), LGE, age and LV outflow gradient as determined by echocardiography was determined using simple and multiple linear regression analysis. Categories of reduced and elevated perfusion in relation to non- or mildly affected reference segments were defined, and T2-signal characteristics and extent as well as pattern of LGE were examined. Statistical testing included linear and logistic regression analysis, unpaired t-test, odds ratios, and Fisher's exact test. RESULTS: 804 segments in 70 patients were included in the analysis. In a simple linear regression model LV wall thickness (p<0.001), extent of LGE (p<0.001), presence of edema, defined as focal T2 bright signal (p<0.001), T2 dark signal (p<0.001) and age (p = 0.032) correlated inversely with relative resting MBF. The LV outflow gradient did not show any effect on resting perfusion (p = 0.901). Multiple linear regression analysis revealed that LGE (p<0.001), edema (p = 0.026) and T2 dark signal (p = 0.019) were independent predictors of relative resting MBF. Segments with reduced resting perfusion demonstrated different LGE patterns compared to segments with elevated resting perfusion. CONCLUSION: In HCM resting MBF is significantly reduced depending on LV wall thickness, extent of LGE, focal T2 signal abnormalities and age. Furthermore, different patterns of perfusion in HCM patients have been defined, which may represent different stages of disease.


Assuntos
Cardiomiopatia Hipertrófica/fisiopatologia , Vasos Coronários/fisiopatologia , Fluxo Sanguíneo Regional , Feminino , Gadolínio , Humanos , Masculino , Pessoa de Meia-Idade
15.
BMJ Open ; 2(4)2012.
Artigo em Inglês | MEDLINE | ID: mdl-22904333

RESUMO

OBJECTIVE: This study was designed to examine the utility of two-dimensional strain (2DS) or speckle tracking imaging to typify functional adaptations of the left ventricle in variant forms of left ventricular hypertrophy (LVH). DESIGN: Cross-sectional study. SETTING: Urban tertiary care academic medical centres. PARTICIPANTS: A total of 129 subjects, 56 with hypertrophic cardiomyopathy (HCM), 34 with hypertensive left ventricular hypertrophy (H-LVH), 27 professional athletes with LVH (AT-LVH) and 12 healthy controls in sinus rhythm with preserved left ventricular systolic function. METHODS: Conventional echocardiographic and tissue Doppler examinations were performed in all study subjects. Bi-dimensional acquisitions were analysed to map longitudinal systolic strain (automated function imaging, AFI, GE Healthcare, Waukesha, Wisconsin, USA) from apical views. RESULTS: Subjects with HCM had significantly lower regional and average global peak longitudinal systolic strain (GLS-avg) compared with controls and other forms of LVH. Strain dispersion index, a measure of regional contractile heterogeneity, was higher in HCM compared with the rest of the groups. On receiver operator characteristics analysis, GLS-avg had excellent discriminatory ability to distinguish HCM from H-LVH area under curve (AUC) (0.893, p<0.001) or AT-LVH AUC (0.920, p<0.001). Tissue Doppler and LV morphological parameters were better suited to differentiate the athlete heart from HCM. CONCLUSIONS: 2DS (AFI) allows rapid characterisation of regional and global systolic function and may have the potential to differentiate HCM from variant forms of LVH.

16.
J Ultrasound Med ; 31(8): 1233-8, 2012 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-22837287

RESUMO

OBJECTIVES: Invasive measurements of intracardiac hemodynamics in animal models have allowed important advances in the understanding of cardiac disease. Currently they are performed either through a carotid arteriotomy or via a thoracotomy and apical insertion. Both of these techniques have disadvantages and are not conducive to repeated measurements. Therefore, the purpose of this study was to develop a new technique for measuring intracardiac hemodynamics. METHODS: In 13 male rats, hemodynamic measurements were performed using a new echocardiographically guided percutaneous apical technique. An intravenous catheter was percutaneously inserted into the left ventricle (LV) in the direction of the LV long axis. Through this catheter, a micromanometer-tipped pressure catheter was inserted, and invasive hemodynamic traces were recorded. After LV recordings, the pressure catheter was advanced into the aorta where pressures were obtained. In 11 rats, measurements were repeated after 1 week (n = 2), 2 weeks (n = 4), 3 weeks (n = 4), or 4 weeks (n = 1). In 3 rats, invasive measurements were performed using a carotid arteriotomy before the percutaneous technique. RESULTS: Among the 13 rats subjected to the procedure, the survival rate was 85%. Of the 11 rats that had the procedure repeated, 3 died (27%). The mean differences ± SD when comparing the two techniques were 10 ± 4 mm Hg for the LV end-systolic pressure and 1 ± 1 mm Hg for the LV end-diastolic pressure. The mean procedure times were 21 ± 3 and 6 ± 1 minutes for the carotid and percutaneous techniques, respectively. CONCLUSIONS: We have successfully developed a percutaneous technique for insertion of LV microtip catheters in rats.


Assuntos
Cateterismo Cardíaco/métodos , Ecocardiografia/métodos , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/cirurgia , Hemodinâmica/fisiologia , Punções , Animais , Masculino , Manometria/instrumentação , Ratos , Ratos Sprague-Dawley
17.
Am J Cardiol ; 110(7): 1033-9, 2012 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-22748357

RESUMO

Dipyridamole is the most common vasodilator used with positron emission tomography for the evaluation of patients with hypertrophic cardiomyopathy (HC). The aim of this study was to evaluate whether positron emission tomographic quantification of regional myocardial perfusion (rMP), myocardial blood flow (MBF), and coronary flow reserve are comparable between dipyridamole and the newer vasodilator regadenoson in HC. An additional aim was to evaluate the association between vasodilator-induced ST-segment depression on electrocardiography and myocardial flow in HC. Nitrogen-13 ammonia positron emission tomography was performed in 57 patients with symptomatic HC at rest and during vasodilator stress (peak) with either dipyridamole (0.56 mg/kg during 4-minute infusion) or regadenoson (0.4 mg fixed bolus dose) for assessment of electrocardiographic findings, rMP (17-segment American Heart Association summed difference score), MBF, and coronary flow reserve. The dipyridamole and regadenoson groups consisted of 28 and 29 patients respectively. Baseline characteristics, including rest MBF (0.92 ± 0.22 vs 0.89 ± 0.23 ml/min/g, p = 0.60), were similar between the 2 groups. During stress, the presence and severity of abnormal rMP (summed difference score 5.5 ± 5.5 vs 5.8 ± 6.7, p = 0.80), peak MBF (1.81 ± 0.44 vs 1.82 ± 0.50 ml/min/g, p = 0.90), and coronary flow reserve (2.02 ± 0.53 vs 2.12 ± 0.12, p = 0.50) were comparable between the dipyridamole and regadenoson groups. Fewer patients exhibited side effects with regadenoson (2 vs 7, p = 0.06). Vasodilator-induced ST-segment depression showed high specificity (about 92%) but low sensitivity (about 34%) to predict abnormal rMP (summed difference score ≥2). In conclusion, measurement of rMP and quantitative flow with positron emission tomography is similar between regadenoson and dipyridamole in patients with symptomatic HC. Regadenoson is tolerated better than dipyridamole and is easier to administer. Vasodilator-induced ST-segment depression is a specific but nonsensitive marker for the prediction of abnormal rMP in patients with HC.


Assuntos
Cardiomiopatia Hipertrófica/diagnóstico , Circulação Coronária/efeitos dos fármacos , Dipiridamol , Eletrocardiografia/efeitos dos fármacos , Teste de Esforço/métodos , Tomografia por Emissão de Pósitrons/métodos , Purinas , Pirazóis , Antagonistas do Receptor A2 de Adenosina/administração & dosagem , Cardiomiopatia Hipertrófica/fisiopatologia , Dipiridamol/administração & dosagem , Feminino , Seguimentos , Humanos , Injeções Intravenosas , Masculino , Pessoa de Meia-Idade , Purinas/efeitos adversos , Pirazóis/efeitos adversos , Curva ROC , Reprodutibilidade dos Testes , Estudos Retrospectivos , Índice de Gravidade de Doença , Volume Sistólico/efeitos dos fármacos , Vasodilatadores/administração & dosagem
18.
Ultrasound Med Biol ; 38(7): 1284-97, 2012 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-22677256

RESUMO

We describe a novel method for computing dense 3D myocardial motion with high accuracy in four-dimensional (4D) echocardiography (3 dimensions spatial plus time). The method is based on a classic variational optical flow technique but exploits modern developments in optical flow research to utilize the full capabilities of 4D echocardiography. Using a variety of metrics, we present an in-depth performance evaluation of the method on synthetic, phantom, and intraoperative 4D transesophageal echocardiographic data. When compared with state-of-the-art optical flow and speckle tracking techniques currently found in 4D echocardiography, the method we present shows notable improvements in error rates. We believe the performance improvements shown can have a positive impact when the method is used as input for various applications, such as strain computation, biomechanical modeling, and automated diagnostics.


Assuntos
Algoritmos , Técnicas de Imagem de Sincronização Cardíaca/métodos , Ecocardiografia Tridimensional/métodos , Coração/fisiopatologia , Interpretação de Imagem Assistida por Computador/métodos , Movimento , Humanos , Aumento da Imagem/métodos , Reprodutibilidade dos Testes , Sensibilidade e Especificidade
19.
J Magn Reson Imaging ; 36(3): 598-603, 2012 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-22549972

RESUMO

PURPOSE: To evaluate if left ventricular outflow tract/aortic valve (LVOT/AO) diameter ratio measured by cardiac magnetic resonance (CMR) imaging is an accurate marker for LVOT obstruction in patients with hypertrophic cardiomyopathy (HCM) compared to Doppler echocardiography. MATERIALS AND METHODS: In all, 92 patients with HCM were divided into three groups based on their resting echocardiographic LVOT pressure gradient (PG): <30 mmHg at rest (nonobstructive, n = 31), <30 mmHg at rest, >30 mmHg after provocation (latent, n = 29), and >30 mmHg at rest (obstructive, n = 32). The end-systolic dimension of the LVOT on 3-chamber steady-state free precession (SSFP) CMR was divided by the end diastolic aortic valve diameter to calculate the LVOT/AO diameter ratio. RESULTS: There were significant differences in the LVOT/AO diameter ratio among the three subgroups (nonobstructive 0.60 ± 0.13, latent 0.41 ± 0.16, obstructive 0.24 ± 0.09, P < 0.001). There was a strong linear inverse correlation between the LVOT/AO diameter ratio and the log of the LVOT pressure gradient (r = -0.84, P < 0.001). For detection of a resting gradient >30 mmHg, the LVOT/AO diameter ratio the area under the receiver operating characteristic (ROC) curve was 0.91 (95% confidence interval [CI] 0.85-0.97). For detection of a resting and/or provoked gradient >30 mmHg, the LVOT/AO diameter ratio area under the ROC curve was 0.90 (95% CI 0.84-0.96). CONCLUSION: The LVOT/AO diameter ratio is an accurate, reproducible, noninvasive, and easy to use CMR marker to assess LVOT pressure gradients in patients with HCM.


Assuntos
Algoritmos , Aorta/patologia , Cardiomiopatia Hipertrófica/patologia , Interpretação de Imagem Assistida por Computador/métodos , Imagem Cinética por Ressonância Magnética/métodos , Obstrução do Fluxo Ventricular Externo/etiologia , Obstrução do Fluxo Ventricular Externo/patologia , Cardiomiopatia Hipertrófica/complicações , Feminino , Humanos , Aumento da Imagem/métodos , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Índice de Gravidade de Doença
20.
J Nucl Med ; 53(3): 407-14, 2012 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-22315440

RESUMO

UNLABELLED: Patients with obstructive hypertrophic cardiomyopathy (HCM) exhibit elevated left ventricular outflow tract gradients (LVOTGs) and appear to have a worse prognosis than those with nonobstructive HCM. The aim of this study was to evaluate whether patients with obstruction, compared with nonobstructive HCM, demonstrate significant differences in PET parameters of microvascular function. METHODS: PET was performed in 33 symptomatic HCM patients at rest and during dipyridamole stress (peak) for the assessment of regional myocardial perfusion (rMP), left ventricular ejection fraction (LVEF), myocardial blood flow (MBF), and myocardial flow reserve (MFR). Myocardial wall thickness and LVOTG were measured with an echocardiogram. Patients were divided into the following 3 groups: nonobstructive (LVOTG < 30 mm Hg at rest and after provocation test with amyl nitrite), obstructive (LVOTG ≥ 30 mm Hg at rest and with provocation), and latent HCM (LVOTG < 30 at rest but ≥ 30 mm Hg with provocation). RESULTS: Eleven patients were classified as nonobstructive (group 1), 12 as obstructive (group 2), and 10 as latent HCM (group 3). Except for age (42 ± 18 y for group 1, 58 ± 7 y for group 2, and 58 ± 12 y for group 3; P = 0.01), all 3 groups had similar baseline characteristics, including maximal wall thickness (2.3 ± 0.5 cm for group 1, 2.2 ± 0.4 cm for group 2, and 2.1 ± 0.7 cm for group 3; P = 0.7). During peak flow, most patients in groups 1 and 2, but fewer in group 3, exhibited rMP defects (73% for group 1, 100% for group 2, and 40% for group 3; P = 0.007) and a drop in LVEF (73% for group 1, 92% for group 2, and 50% for group 3; P = 0.09). Peak MBF (1.58 ± 0.49 mL/min/g for group 1, 1.72 ± 0.46 mL/min/g for group 2, and 1.97 ± 0.32 mL/min/g for group 3; P = 0.14) and MFR (1.62 ± 0.57 for group 1, 1.90 ± 0.31 for group 2, and 2.27 ± 0.51 for group 3; P = 0.01) were lower in the nonobstructive and higher in the latent HCM group. LVOTGs demonstrated no significant correlation with any flow dynamics. In a multivariate regression analysis, maximal wall thickness was the only significant predictor for reduced peak MBF (ß = -0.45, P = 0.003) and MFR (ß = -0.63, P = 0.0001). CONCLUSION: Maximal wall thickness was identified as the strongest predictor of impaired dipyridamole-induced hyperemia and flow reserve in our study, whereas outflow tract obstruction was not an independent determinant.


Assuntos
Cardiomiopatia Hipertrófica/diagnóstico por imagem , Imagem Multimodal/métodos , Tomografia por Emissão de Pósitrons , Tomografia Computadorizada por Raios X , Adulto , Idoso , Algoritmos , Circulação Coronária/fisiologia , Interpretação Estatística de Dados , Dipiridamol , Eletrocardiografia , Feminino , Coração/diagnóstico por imagem , Hemodinâmica/fisiologia , Humanos , Hiperemia/diagnóstico por imagem , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Processamento de Imagem Assistida por Computador , Masculino , Pessoa de Meia-Idade , Ultrassonografia , Vasodilatadores , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem
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