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1.
Int Heart J ; 61(1): 39-45, 2020 Jan 31.
Artigo em Inglês | MEDLINE | ID: mdl-31956141

RESUMO

Patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) and hypertrophic cardiomyopathy (HCM) implanted with implantable cardioverter-defibrillators (ICDs) may show a large decrease in R-wave amplitude during long-term follow-up. However, it is unclear whether this decrease is higher in these patients than in those without structural heart disease. This study investigated ICD-lead intracardiac parameters over a long duration in patients with ARVC and HCM and compared these parameters with those of a control group. We included 50 patients (mean age, 55.2 ± 17.2 years; 26% female) with ICD leads in the right ventricular apex, and compared 7 ARVC and 14 HCM patients with 29 control patients without structural heart disease. ICD-lead parameters, including R-wave amplitude, pacing threshold, and impedance during follow-up, were compared. The difference in these parameters between the time of implantation and year 5 were also compared. There were no significant differences in R-wave amplitude at implantation among the 3 groups. The change in R-wave amplitude between the time of implantation and year 5 was significantly greater in the ARVC group (-3.3 ± 5.4 mV, P = 0.012) in comparison to the control group (1.3 ± 2.8 mV); the HCM group showed no significant difference (-0.4 ± 2.3 mV, P = 0.06). Thus, in the ARVC group, R-wave amplitude at year 5 was significantly lower than that in the control group (5.7 ± 4.8 mV versus 12.5 ± 4.5 mV, P = 0.001). In ARVC patients with ICDs, ventricular sensing is likely to deteriorate during long-term follow-up; however, in HCM patients, sensing may not deteriorate.


Assuntos
Displasia Arritmogênica Ventricular Direita/terapia , Cardiomiopatia Hipertrófica/terapia , Ventrículos do Coração/fisiopatologia , Adolescente , Adulto , Idoso , Displasia Arritmogênica Ventricular Direita/fisiopatologia , Cardiomiopatia Hipertrófica/fisiopatologia , Estudos de Casos e Controles , Desfibriladores Implantáveis , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
2.
Cardiol Young ; 29(10): 1264-1267, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31475665

RESUMO

OBJECTIVE: The purpose of this study was to assess fetal cardiac function in normal fetuses (control group) compared to those who are exposed to gestational diabetes mellitus using different echocardiographic measurements, and to explore the application of left atrial shortening fraction in determination of fetal diastolic function with gestational diabetes mellitus. METHODS: A total of 50 women with gestational diabetes and 50 women with a healthy pregnancy were included in the study. Fetal echocardiography was performed and structural as well as functional fetal cardiac parameters were measured. Data were compared between with or without fetal myocardial hypertrophy and the control group. RESULTS: In the study group, out of 50 fetuses of gestational diabetic mothers, 18 had myocardial hypertrophy and 32 had normal septal thickness. Gestational age at time of examination did not differ significantly between the control and gestational diabetes group (p = 0.55). Mitral E/A ratio was lower in gestational diabetes group as compared to the control (p < 0.001). Isovolumetric relaxation and contraction times and myocardial performance index were greater in fetuses of gestational diabetic mothers (p < 0.001). In fetuses of gestational diabetic mothers with myocardial hypertrophy, left atrial shortening fraction was lower as compared to those without myocardial hypertrophy and those of the control group (p < 0.001). CONCLUSIONS: The results of this study suggest that fetuses of gestational diabetic mothers have altered cardiac function even in the absence of septal hypertrophy, and that left atrial shortening fraction can be used as a reliable alternate parameter in the assessment of fetal diastolic function.


Assuntos
Cardiomiopatia Hipertrófica/diagnóstico , Diabetes Gestacional/fisiopatologia , Ecocardiografia/métodos , Coração Fetal/diagnóstico por imagem , Ultrassonografia Pré-Natal/métodos , Função Ventricular/fisiologia , Adulto , Cardiomiopatia Hipertrófica/embriologia , Cardiomiopatia Hipertrófica/fisiopatologia , Diástole , Feminino , Coração Fetal/fisiologia , Seguimentos , Idade Gestacional , Humanos , Gravidez , Estudos Retrospectivos
3.
Pediatr Cardiol ; 40(6): 1253-1257, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31263917

RESUMO

Obesity is associated with additional left ventricular hypertrophy (LVH) in adults with hypertrophic cardiomyopathy (HCM). It is not known whether obesity can lead to further LVH in children with HCM. Echocardiographic LV dimensions were determined in 504 children with HCM. Measurements of interventricular septal thickness (IVST) and posterior wall thickness (PWT), and patients' weight and height were recorded. Obesity was defined as a body mass index (BMI) ≥ 99th percentile for age and sex. IVST data was available for 498 and PWT data for 484 patients. Patient age ranged from 2 to 20 years (mean ± SD, 12.5 ± 3.9) and 340 (68%) were males. Overall, patient BMI ranged from 7 to 50 (22.7 ± 6.1). Obesity (BMI 18-50, mean 29.1) was present in 140 children aged 2-19.6 (11.3 ± 4.1). The overall mean IVST was 20.5 ± 9.6 mm and the overall mean PWT was 11.0 ± 8.4 mm. The mean IVST in the obese patients was 21.6 ± 10.0 mm and mean PWT was 13.3 ± 14.7 mm. The mean IVST in the non-obese patients was 20.1 ± 9.5 mm and mean PWT was 10.4 ± 4.3 mm. Obesity was not significantly associated with IVST (p = 0.12), but was associated with increased PWT (0.0011). Obesity is associated with increased PWT but not IVST in children with HCM. Whether obesity and its impact on LVH influences clinical outcomes in children with HCM needs to be studied.


Assuntos
Cardiomiopatia Hipertrófica/complicações , Ventrículos do Coração/patologia , Obesidade/complicações , Septo Interventricular/patologia , Adolescente , Índice de Massa Corporal , Cardiomiopatia Hipertrófica/fisiopatologia , Criança , Pré-Escolar , Ecocardiografia , Feminino , Humanos , Masculino , Adulto Jovem
4.
Int J Cardiovasc Imaging ; 35(12): 2167-2175, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31321652

RESUMO

Treatment of overt form of hypertrophic cardiomyopathy (HCM) is often unsuccessful. Efforts are focused on a possible early identification in order to prevent or delaying the development of hypertrophy. Our aim was to find an echocardiographic marker able to distinguish mutation carriers without left ventricular hypertrophy (LVH) from healthy subjects. We evaluated 28 patients, members of eight families. Three types of mutation were recognized: MYBPC3 (five families), MYH7 (two families) and TNNT2 (one family). According to genetic (G) and phenotypic (Ph) features, patients were divided in three groups: Group A (10 patients), mutation carriers with LVH (G+/Ph+); Group B (9 patients), mutation carriers without LVH (G+/Ph-); Group C (9 patients), healthy subjects (G-/Ph-). Echocardiography examination was performed acquiring standard 2D, DTI and 2D-strain imaging. Global longitudinal strain (GLS) and global radial strain (GRS) at basal and mid-level were measured. GRS was significantly different between group B and C at basal level (32.18% ± 9.6 vs. 44.59% ± 12.67 respectively; p-value < 0.0001). In basal posterior and basal inferior segments this difference was particularly evident. ROC curves showed for both the involved segments good AUCs (0.931 and 0.861 for basal posterior and inferior GRS respectively) with the best predictive cut-off for basal posterior GRS at 43.65%, while it was 38.4% for basal inferior GRS. Conversely, GLS values were similar in the three group. 2D longitudinal strain is a valid technique to study HCM. Radial strain and particularly basal posterior and inferior segmental reduction could be able to identify mutation carriers in a pre-clinical phase of disease.


Assuntos
Cardiomiopatia Hipertrófica/diagnóstico por imagem , Ecocardiografia , Função Ventricular Esquerda , Adolescente , Adulto , Idoso , Miosinas Cardíacas/genética , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/fisiopatologia , Proteínas de Transporte/genética , Estudos de Casos e Controles , Análise Mutacional de DNA , Feminino , Predisposição Genética para Doença , Humanos , Masculino , Pessoa de Meia-Idade , Mutação , Cadeias Pesadas de Miosina/genética , Fenótipo , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Troponina T/genética , Adulto Jovem
6.
Medicina (Kaunas) ; 55(6)2019 Jun 23.
Artigo em Inglês | MEDLINE | ID: mdl-31234582

RESUMO

The concepts underlying hypertrophic cardiomyopathy (HCM) pathogenesis have evolved greatly over the last 60 years since the pioneering work of the British pathologist Donald Teare, presenting the autopsy findings of "asymmetric hypertrophy of the heart in young adults". Advances in human genome analysis and cardiac imaging techniques have enriched our understanding of the complex architecture of the malady and shaped the way we perceive the illness continuum. Presently, HCM is acknowledged as "a disease of the sarcomere", where the relationship between genotype and phenotype is not straightforward but subject to various genetic and nongenetic influences. The focus of this review is to discuss key aspects related to molecular mechanisms and imaging aspects that have prompted genotype-phenotype correlations, which will hopefully empower patient-tailored health interventions.


Assuntos
Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/fisiopatologia , Proteínas de Transporte/genética , Estudos de Associação Genética/métodos , Predisposição Genética para Doença/genética , Humanos , Prognóstico , Ultrassonografia/métodos
7.
BMJ Case Rep ; 12(6)2019 Jun 18.
Artigo em Inglês | MEDLINE | ID: mdl-31217211

RESUMO

There are nine published reports of trileaflet mitral valves globally. As such their implication on health outcomes and associations with other disease is uncertain. This case describes a 62-year-old man presenting with exertional dyspnoea and hypertension. It describes an early misdiagnosis of hypertrophic cardiomyopathy and highlights that clinicians should be alerted in cases of very high left ventricular outflow gradients in the presence of eccentric mitral regurgitation (MR). Here the MR was caused by a rare congenital deformity whereby a deep cleft in the posterior leaflet resulted in a tricuspid appearance. We present the natural disease course of a trileaflet mitral valve and without intervention over 13 years from symptom onset to the development of severe MR.


Assuntos
Cardiomiopatia Hipertrófica/diagnóstico por imagem , Ecocardiografia , Ventrículos do Coração/patologia , Insuficiência da Valva Mitral/diagnóstico por imagem , Cateterismo Cardíaco , Cardiomiopatia Hipertrófica/fisiopatologia , Cardiomiopatia Hipertrófica/cirurgia , Dispneia , Implante de Prótese de Valva Cardíaca , Humanos , Hipertensão , Masculino , Pessoa de Meia-Idade , Insuficiência da Valva Mitral/fisiopatologia , Insuficiência da Valva Mitral/cirurgia , Resultado do Tratamento
8.
Nat Commun ; 10(1): 2685, 2019 06 18.
Artigo em Inglês | MEDLINE | ID: mdl-31213605

RESUMO

Hypertrophic cardiomyopathy (HCM) affects 1 in 500 people and leads to hyper-contractility of the heart. Nearly 40 percent of HCM-causing mutations are found in human ß-cardiac myosin. Previous studies looking at the effect of HCM mutations on the force, velocity and ATPase activity of the catalytic domain of human ß-cardiac myosin have not shown clear trends leading to hypercontractility at the molecular scale. Here we present functional data showing that four separate HCM mutations located at the myosin head-tail (R249Q, H251N) and head-head (D382Y, R719W) interfaces of a folded-back sequestered state referred to as the interacting heads motif (IHM) lead to a significant increase in the number of heads functionally accessible for interaction with actin. These results provide evidence that HCM mutations can modulate myosin activity by disrupting intramolecular interactions within the proposed sequestered state, which could lead to hypercontractility at the molecular level.


Assuntos
Miosinas Cardíacas/metabolismo , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/fisiopatologia , Contração Miocárdica/genética , Cadeias Pesadas de Miosina/metabolismo , Actinas/metabolismo , Animais , Miosinas Cardíacas/genética , Linhagem Celular , Movimento Celular/genética , Coração/fisiopatologia , Humanos , Camundongos , Mutação , Mioblastos , Cadeias Pesadas de Miosina/genética , Proteínas Recombinantes/genética , Proteínas Recombinantes/metabolismo
9.
Am J Cardiol ; 124(4): 604-612, 2019 08 15.
Artigo em Inglês | MEDLINE | ID: mdl-31204037

RESUMO

Right ventricular (RV) dysfunction is a well-known prognostic factor in several cardiac diseases. However, the prevalence of RV dysfunction in hypertrophic cardiomyopathy (HC) is unclear and its prognostic value is unknown. This study aims at addressing these issues assessing RV function with speckle tracking echocardiography. In 267 HC patients (52 ± 15 years, 68% male), standard and advanced echocardiographic measurements of RV function were performed including RV 4-chamber longitudinal strain (RV4CLS) and RV free wall longitudinal strain (RVFWLS). The primary end point was all-cause mortality and heart failure development. RV dysfunction was observed in 9% of patients based on tricuspid annular plane systolic excursion (≤17 mm), 5% based on fractional area change (<35%), 23% based on RVFWLS ≥-19%, 39% based on RVFWLS ≥-23%, and 55% based on RV4CLS ≥-20%. In total 59 (22%) patients reached the primary end point during a median follow-up of 6.7 (interquartile range 4.2 to 9.8) years. Kaplan-Meier survival curve showed a significant worse survival free of the end point for patients with impaired RV4CLS ≥-20% versus patients with preserved RV4CLS <-20% (log-rank 7.0, p = 0.008) and for patients with impaired RVFWLS ≥-19% versus patients with preserved RVFWLS <-19% (log-rank 4.4, p = 0.037). Multivariable Cox regression analysis showed that E/E' (hazards ratio [HR] 2.26 [1.30 to 3.92], p = 0.004), left ventricular global longitudinal strain LV GLS (HR 1.08 (1.01 to 1.17), p = 0.034) and RV4CLS (HR 1.08 (1.02 to 1.15), p = 0.007) were independently associated with the primary end point. In conclusion, RV dysfunction as measured by longitudinal strain is relatively frequent in HC patients. Impaired RV4CLS is - together with LV GLS and E/E' - associated with adverse outcome, which may indicate a more severe form of HC.


Assuntos
Cardiomiopatia Hipertrófica/epidemiologia , Insuficiência Cardíaca/epidemiologia , Mortalidade , Disfunção Ventricular Direita/epidemiologia , Adulto , Idoso , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Cardiomiopatia Hipertrófica/fisiopatologia , Comorbidade , Ecocardiografia , Ecocardiografia Doppler , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Prevalência , Prognóstico , Disfunção Ventricular Direita/diagnóstico por imagem
10.
Int J Cardiovasc Imaging ; 35(10): 1913-1924, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31144257

RESUMO

Hypertrophic cardiomyopathy (HCM) is characterized by myocardial disarray, hypertrophy, and fibrosis. Reduced global longitudinal strain and presence of late gadolinium enhancement (LGE) by cardiac magnetic resonance imaging (CMR) have been associated with an adverse prognosis. This study evaluated 3D principal and conventional strain characteristics of non-enhanced myocardium in patients with HCM. 3D principal and conventional strain analysis was conducted in 51 HCM patients and 38 healthy controls. Principal strain was reduced within the non-enhanced myocardium of HCM as compared with controls (maximum principal: 51.5 ± 23.7 vs. 75.1 ± 21.4%, P < 0.0001; minimum principal: - 18.4 ± 4.0 vs. - 20.1 ± 2.9%, P < 0.05). Principal strain within the non-enhanced myocardium was incrementally reduced in HCM patients with extensive global LGE ( ≥ 15%) (maximum principal: 41.6 ± 17.5 vs. 56.9 ± 25.9%, P < 0.05; minimum principal: - 16.9 ± 3.9 vs. - 19.1 ± 4.0%, P = 0.1), as was longitudinal ( - 10.5 ± 2.6 vs. - 12.7 ± 2.6%, P < 0.05) and circumferential strain ( - 11.0 ± 2.7 vs. - 14.0 ± 2.9%, P < 0.01). Principal strain within non-enhanced myocardium was significantly correlated with indexed LV mass (P < 0.0001), maximum (P = 0.0008), and mean wall thickness (P < 0.0001), but not LGE (P = 0.0841). In adjusted analysis, all strain measures within non-enhanced myocardium were independently associated with indexed LV mass (maximum principal: P = 0.0003; minimum principal: P = 0.0039; longitudinal: P = 0.0015; circumferential: P = 0.0002; radial: P = 0.0023). 3D principal strain of non-enhanced myocardium was significantly reduced in HCM patients as compared with controls, and was incrementally reduced among patients with more extensive global LGE. Comprehensive strain assessment may be considered in routine CMR assessment of HCM patients.


Assuntos
Cardiomiopatia Hipertrófica/diagnóstico por imagem , Imagem Tridimensional/métodos , Imagem Cinética por Ressonância Magnética/métodos , Contração Miocárdica , Adulto , Idoso , Fenômenos Biomecânicos , Cardiomiopatia Hipertrófica/fisiopatologia , Estudos de Casos e Controles , Meios de Contraste/administração & dosagem , Estudos Transversais , Feminino , Humanos , Interpretação de Imagem Assistida por Computador , Masculino , Pessoa de Meia-Idade , Compostos Organometálicos/administração & dosagem , Valor Preditivo dos Testes
11.
Am J Cardiol ; 124(3): 435-441, 2019 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-31130217

RESUMO

PURPOSE: Diastolic dysfunction preceding systolic dysfunction is considered an important interaction in cardiomyopathy with poor prognosis. The aim of this study was to compare left ventricular (LV) isovolumic relaxation with the other parameters as a potential prognostic marker for patients with idiopathic dilated cardiomyopathy (IDC) and hypertrophic cardiomyopathy (HC). METHODS: A total of 145 patients with IDC and 116 with HC were evaluated for hemodynamic parameters; LV pressure was directly measured by a micromanometer catheter, and relaxation half-time (T1/2) was used to determine LV isovolumic relaxation. The median follow-up period was 4.7 years. RESULTS: The mean ages of the patients with IDC and HC were 52.0 ± 12.0 and 57.1 ± 12.4 years, respectively. Each patient group was further divided into 2 groups based on the median value of T1/2: (1) <41.0 ms (D-L group) and ≥41.0 ms (D-H group) (2) <38.5 ms (H-L group) and ≥38.5 ms (H-H group). Kaplan-Meier analysis showed a significantly higher probability of cardiac events in the D-H group than in the D-L group (p = 0.001) and in the H-H group than in the H-L group (p = 0.028). Further, Cox proportional hazard regression analysis revealed that T1/2 was an independent predictor of cardiac events for patients with IDC (hazard ratio 1.109; p = 0.007) and HC (hazard ratio 1.062; p = 0.041). In conclusion, regardless of the type of cardiomyopathy, T1/2 as a measure of LV isovolumic relaxation function was found to be associated with the occurrence of cardiac events.


Assuntos
Cardiomiopatia Dilatada/fisiopatologia , Cardiomiopatia Hipertrófica/fisiopatologia , Disfunção Ventricular Esquerda/fisiopatologia , Cateterismo Cardíaco , Cardiomiopatia Dilatada/diagnóstico por imagem , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Diástole , Ecocardiografia Doppler , Feminino , Hemodinâmica , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Sístole , Disfunção Ventricular Esquerda/diagnóstico por imagem
12.
BMC Cardiovasc Disord ; 19(1): 81, 2019 04 03.
Artigo em Inglês | MEDLINE | ID: mdl-30943916

RESUMO

BACKGROUND: Left ventricular apical hypertrophic cardiomyopathy is a rare presentation of hypertrophic cardiomyopathy associated with thickening of the apical segment of the left ventricle. It was initially described in Japan in 1976 and is characterized by electrocardiogram findings showing giant T wave inversions in the precordial leads as well as a spade shaped appearance of the apical cavity on imaging (Abugroun et al., Cardiol Res 8:265-268, 2017). In this case, we present a patient with a heart transplant with a stable post-transplant course who was found to have apical hypertrophic cardiomyopathy. There have been a few cases of apical hypertrophy in a transplanted heart documented in the literature. Making this case even more unique is that this presentation is evident 17 years after heart transplantation. CASE PRESENTATION: Fifty-four year-old male with a history of orthotropic heart transplant in 2001 on immunosuppressive therapy presented with palpitations and associated lightheadedness. He had a blood pressure of 184/89 mmHg on arrival but otherwise had stable vital signs and physical examination. Cardiac biomarkers revealed a CK of 59 U/L and a troponin of 0.11NG/ML(normal < 0.04NG/ML). B type natriuretic peptide was 371 PG/ML(normal 0-100PG/ML). Routine laboratory studies demonstrated normal sodium, magnesium, serum creatinine, and a potassium of 3.3 mmol/L(normal 3.5-5.1 mmol/L). His hemoglobin and hematocrit were normal. His EKG showed sinus rhythm with old T wave inversions in the anterior and lateral leads. Echocardiogram revealed a left ventricular ejection fraction of 55-65%, left posterior wall of 1.3 cm and interventricular septal wall 1.2 cm, thickened trabeculated apex, with severely dilated left atrium. He had a stress test that showed mild inferior wall thinning and a cardiac MRI performed to further evaluate apical hypertrophy revealed prominent apical hypertrophy of the left ventricle with near obliteration of the apical cavity. He had no events on cardiac monitoring and was discharged with close followup with the transplant team. CONCLUSION: While there are many etiologies of ApHCM, it has not been well described in transplanted patients who are on chronic immunosuppressive therapy. It is unclear if these groups of patients are at an increased risk of developing this condition. The literature suggests that ApHCM is associated with a being prognosis but there is new data suggesting increased mortality in a subset of patients with this condition.


Assuntos
Cardiomiopatia Hipertrófica/etiologia , Transplante de Coração/efeitos adversos , Hipertrofia Ventricular Esquerda/etiologia , Função Ventricular Esquerda , Remodelação Ventricular , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Cardiomiopatia Hipertrófica/fisiopatologia , Humanos , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Hipertrofia Ventricular Esquerda/fisiopatologia , Masculino , Pessoa de Meia-Idade , Imagem Multimodal , Prognóstico , Resultado do Tratamento
15.
J Cardiovasc Med (Hagerstown) ; 20(6): 389-396, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30994509

RESUMO

AIMS: Left ventricular outflow tract (LVOT) obstruction is a key feature of hypertrophic cardiomyopathy (HCM) that identifies patients at increased risk of adverse outcomes. Previous studies have hypothesized that LVOT obstruction enhances myocardial fibrosis and increases left ventricular (LV) filling pressures, producing greater clinical deterioration. However, this hypothesis has not been demonstrated in a clinical cohort comparing obstructive and nonobstructive patients. METHODS: Patients with HCM in whom Doppler echocardiography was performed within 30 days of cardiac MRI were enrolled, using the E/e' ratio to assess LV diastolic function and late gadolinium enhancement to evaluate the extent of fibrosis. Data were assorted according to LVOT obstruction status at rest. RESULTS: The current study enrolled 67 patients who were mostly middle-aged (56.8 ±â€Š13.2 years old) men (75%) with preserved ejection fraction. Obstructive HCM presented a significant association with a high fibrosis extent [odds ratio (OR) 3.33; P = 0.034] which was maintained after adjusting for sex and age (OR 4.37; P = 0.016) but not for maximum LV wall thickness (OR 2.13; P = 0.225). Obstructive HCM was also associated with a clinically significant E/e' ratio more than 14 (OR 7.8; P = 0.001) which decreased slightly after adjusting for age, sex and maximum LV thickness (OR 6.54; P = 0.014). There was a significant association between an E/e' ratio more than 14 and the extent of fibrosis (OR 1.29; P < 0.001) which was maintained after adjusting for age, sex and maximum LV wall thickness (OR 1.36; P = 0.001). CONCLUSION: LVOT obstruction may play a role in the extent of fibrosis in HCM, possibly conditioning greater diastolic dysfunction.


Assuntos
Cardiomiopatia Hipertrófica/diagnóstico por imagem , Ecocardiografia Doppler , Imagem Cinética por Ressonância Magnética , Miocárdio/patologia , Disfunção Ventricular Esquerda/diagnóstico por imagem , Função Ventricular Esquerda , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem , Remodelação Ventricular , Adulto , Idoso , Cardiomiopatia Hipertrófica/patologia , Cardiomiopatia Hipertrófica/fisiopatologia , Estudos Transversais , Feminino , Fibrose , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Volume Sistólico , Fatores de Tempo , Disfunção Ventricular Esquerda/patologia , Disfunção Ventricular Esquerda/fisiopatologia , Obstrução do Fluxo Ventricular Externo/patologia , Obstrução do Fluxo Ventricular Externo/fisiopatologia , Pressão Ventricular
16.
Ann Intern Med ; 170(11): 741-748, 2019 06 04.
Artigo em Inglês | MEDLINE | ID: mdl-31035291

RESUMO

Background: Mavacamten, an orally administered, small-molecule modulator of cardiac myosin, targets underlying biomechanical abnormalities in obstructive hypertrophic cardiomyopathy (oHCM). Objective: To characterize the effect of mavacamten on left ventricular outflow tract (LVOT) gradient. Design: Open-label, nonrandomized, phase 2 trial. (ClinicalTrials.gov: NCT02842242). Setting: 5 academic centers. Participants: 21 symptomatic patients with oHCM. Intervention: Patients in cohort A received mavacamten, 10 to 20 mg/d, without background medications. Those in cohort B received mavacamten, 2 to 5 mg/d, with ß-blockers allowed. Measurements: The primary end point was change in postexercise LVOT gradient at 12 weeks. Secondary end points included changes in peak oxygen consumption (pVO2), resting and Valsalva LVOT gradients, left ventricular ejection fraction (LVEF), and numerical rating scale dyspnea score. Results: In cohort A, mavacamten reduced mean postexercise LVOT gradient from 103 mm Hg (SD, 50) at baseline to 19 mm Hg (SD, 13) at 12 weeks (mean change, -89.5 mm Hg [95% CI, -138.3 to -40.7 mm Hg]; P = 0.008). Resting LVEF was also reduced (mean change, -15% [CI, -23% to -6%]). Peak VO2 increased by a mean of 3.5 mL/kg/min (CI, 1.2 to 5.9 mL/kg/min). In cohort B, the mean postexercise LVOT gradient decreased from 86 mm Hg (SD, 43) to 64 mm Hg (SD, 26) (mean change, -25.0 mm Hg [CI, -47.1 to -3.0 mm Hg]; P = 0.020), and mean change in resting LVEF was -6% (CI, -10% to -1%). Peak VO2 increased by a mean of 1.7 mL/kg/min (SD, 2.3) (CI, 0.03 to 3.3 mL/kg/min). Dyspnea scores improved in both cohorts. Mavacamten was well tolerated, with mostly mild (80%), moderate (19%), and unrelated (79%) adverse events. The most common adverse events definitely or possibly related to mavacamten were decreased LVEF at higher plasma concentrations and atrial fibrillation. Limitation: Small size; open-label design. Conclusion: Mavacamten can reduce LVOT obstruction and improve exercise capacity and symptoms in patients with oHCM. Primary Funding Source: MyoKardia.


Assuntos
Benzilaminas/uso terapêutico , Cardiomiopatia Hipertrófica/tratamento farmacológico , Fármacos Cardiovasculares/uso terapêutico , Uracila/análogos & derivados , Função Ventricular Esquerda/efeitos dos fármacos , Administração Oral , Antagonistas Adrenérgicos beta/efeitos adversos , Antagonistas Adrenérgicos beta/uso terapêutico , Adulto , Idoso , Benzilaminas/efeitos adversos , Cardiomiopatia Hipertrófica/fisiopatologia , Fármacos Cardiovasculares/efeitos adversos , Relação Dose-Resposta a Droga , Quimioterapia Combinada , Tolerância ao Exercício , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Consumo de Oxigênio/efeitos dos fármacos , Estudos Prospectivos , Volume Sistólico/efeitos dos fármacos , Uracila/efeitos adversos , Uracila/uso terapêutico , Adulto Jovem
17.
Int J Cardiovasc Imaging ; 35(6): 1089-1100, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30825136

RESUMO

Surgical therapies in aortic valve stenosis (AVS) and hypertrophic obstructive cardiomyopathy (HOCM) aim to relief intraventricular pressure overload and improve clinical outcome. It is currently unknown to what extent myocardial adaptation concurs with restoration of intraventricular pressures, and whether this is similar in both patient groups. The aim of this study was to investigate changes in myocardial adaptation after surgical therapies for AVS and HOCM. Ten AVS and ten HOCM patients were enrolled and underwent cardiac magnetic resonance cine imaging and myocardial tagging prior to, and 4 months after aortic valve replacement (AVR) and septal myectomy, respectively. Global left ventricular (LV) analyses were derived from cine images. Circumferential strain was assessed from myocardial tagging images at the septal and lateral wall of the mid ventricle. Pressure gradients significantly decreased in both AVS and HOCM after surgery (p < 0.01), with a concomitant decrease in left atrial volume (p < 0.05) suggesting lower diastolic filling pressures. Also, LV volumes, mass and septal wall thickness decreased in both, but to a larger extent in AVS than in HOCM patients. AVR improved wall thickening (p < 0.05) and did not change systolic strain rate. Myectomy did not affect wall thickening and reduced septal systolic strain rate (p = 0.03). Both AVR and myectomy induced positive structural remodeling in line with a reduction of pressure overload. A concomitant recovery in systolic function however was found in AVR only. The systolic functional deterioration in HOCM patients seems to be inherent to myectomy and the ongoing and irreversible disease.


Assuntos
Estenose da Valva Aórtica/cirurgia , Cardiomiopatia Hipertrófica/cirurgia , Implante de Prótese de Valva Cardíaca , Função Ventricular Esquerda , Pressão Ventricular , Remodelação Ventricular , Adaptação Fisiológica , Adulto , Idoso , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/fisiopatologia , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Cardiomiopatia Hipertrófica/fisiopatologia , Feminino , Humanos , Imagem Cinética por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Recuperação de Função Fisiológica , Sístole , Fatores de Tempo , Resultado do Tratamento
18.
Chin Med J (Engl) ; 132(6): 631-637, 2019 Mar 20.
Artigo em Inglês | MEDLINE | ID: mdl-30855343

RESUMO

BACKGROUND: The comparative outcomes of subcutaneous implantable cardioverter-defibrillator (S-ICD) and transvenous ICD (T-ICD) have not been well studied. The aim of this study was to evaluate the safety and efficacy of currently available S-ICD and T-ICD. METHODS: The study included 86 patients who received an S-ICD and 1:1 matched to those who received single-chamber T-ICD by gender, age, diagnosis, left ventricular ejection fraction (LVEF), and implant year. The clinical outcomes and implant complications were compared between the two groups. RESULTS: The mean age of the 172 patients was 45 years, and 129 (75%) were male. The most common cardiac condition was hypertrophic cardiomyopathy (HCM, 37.8%). The mean LVEF was 50%. At a mean follow-up of 23 months, the appropriate and inappropriate ICD therapy rate were 1.2% vs. 4.7% (χ = 1.854, P = 0.368) and 9.3% vs. 3.5% (χ = 2.428, P = 0.211) in S-ICD and T-ICD groups respectively. There were no significant differences in device-related major and minor complications between the two groups (7.0% vs. 3.5%, χ = 1.055, P = 0.496). The S-ICD group had higher T-wave oversensing than T-ICD group (9.3% vs. 0%, χ = 8.390, P = 0.007). Sixty-five patients had HCM (32 in S-ICD and 33 in T-ICD). The incidence of major complications was not significantly different between the two groups. CONCLUSIONS: The efficacy of an S-ICD is comparable to that of T-ICD, especially in a dominantly HCM patient population. The S-ICD is associated with fewer major complications demanding reoperation.


Assuntos
Cardiomiopatia Hipertrófica/terapia , Desfibriladores Implantáveis , Taquicardia Ventricular/terapia , Adulto , Cardiomiopatia Hipertrófica/fisiopatologia , Morte Súbita Cardíaca/prevenção & controle , Eletrocardiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Taquicardia Ventricular/fisiopatologia
19.
PLoS One ; 14(3): e0212573, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30893304

RESUMO

BACKGROUND: Pulmonary hypertension (PH) is a known complication of HCM and is a strong predictor of mortality. We aim to investigate the relationship between microvascular dysfunction measured by quantitative PET and PH in HCM patients. METHODS: Eighty-nine symptomatic HCM patients were included in the study. Each patient underwent two 20-min 13N-NH3 dynamic PET scans for rest and stress conditions, respectively. A 2-tissue irreversible compartmental model was used to fit the segments time activity curves for estimating segmental and global myocardial blood flow (MBF) and myocardial flow reserve (MFR). Echocardiographic derived PASP was utilized to estimate PH. RESULTS: Patients were categorized into two groups across PASP: PH (PASP > 36 mmHg) and no-PH (PASP ≤ 36 mmHg). patients with PH had larger left atrium, ratio of higher inflow early diastole (E) and atrial contraction (A) waves, E/A, and ratio of inflow and peak early diastolic waves, E/e', significantly reduced global stress MBF (1.85 ± 0.52 vs. 2.13 ± 0.56 ml/min/g; p = 0.024) and MFR (2.21 ± 0.57 vs. 2.62 ± 0.75; p = 0.005), while the MBFs at rest between the two groups were similar. There were significant negative correlations between global stress MBF/MFR and PASP (stress MBF: r = -0.23, p = 0.03; MFR: r = -0.32, p = 0.002); for regional MBF and MFR measurements, the highest linear correlation coefficients were observed in the septal wall (stress MBF: r = -0.27, p = 0.01; MFR: r = -0.31, p = 0.003). Global MFR was identified to be independent predictor for PH in multivariate regression analysis. CONCLUSION: Echocardiography-derived PASP is negatively correlated with global MFR measured by 13N-NH3 dynamic PET. Global MFR is suggested to be an index of PH in HCM patients.


Assuntos
Pressão Sanguínea , Cardiomiopatia Hipertrófica , Ecocardiografia , Reserva Fracionada de Fluxo Miocárdico , Tomografia por Emissão de Pósitrons , Artéria Pulmonar , Idoso , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Cardiomiopatia Hipertrófica/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Imagem de Perfusão do Miocárdio , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia
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