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1.
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
2.
Cells ; 8(4)2019 04 08.
Artigo em Inglês | MEDLINE | ID: mdl-30965672

RESUMO

Fabry disease (FD) is a rare inherited disorder characterized by a wide range of systemic symptoms; it is particularly associated with cardiovascular and renal problems. Enzyme replacement therapy and pharmacological chaperone migalastat are the only approved and effective treatment strategies for FD patients. It is well documented that alpha-galactosidase A (GLA) enzyme activity deficiency causes globotriaosylceramide (Gb3) accumulation, which plays a crucial role in the etiology of FD. However, the detailed mechanisms remain unclear, and the lack of a reliable and powerful disease model is an obstacle. In this study, we created such a model by using CRISPR/Cas9-mediated editing of GLA gene to knockout its expression in human embryonic stem cells (hESCs). The cardiomyocytes differentiated from these hESCs (GLA-null CMs) were characterized by the accumulation of Gb3 and significant increases of cell surface area, the landmarks of FD-associated cardiomyopathy. Furthermore, we used mass spectrometry to compare the proteomes of GLA-null CMs and parental wild type CMs and found that the Rab GTPases involved in exocytotic vesicle release were significantly downregulated. This caused impairment of autophagic flux and protein turnover, resulting in an increase of reactive oxygen species and apoptosis. To summarize, we established a FD model which can be used as a promising tool to study human hypertrophic cardiomyopathy in a physiologically and pathologically relevant manner and to develop new therapies by targeting Rab GTPases signaling-related exosomal vesicles transportation.


Assuntos
Autofagia , Cardiomiopatia Hipertrófica/genética , Cardiomiopatia Hipertrófica/patologia , Doença de Fabry/complicações , Modelos Biológicos , alfa-Galactosidase/genética , Apoptose , Sistemas CRISPR-Cas , Cardiomiopatia Hipertrófica/etiologia , Linhagem Celular , Exossomos , Técnicas de Inativação de Genes , Células-Tronco Embrionárias Humanas/metabolismo , Humanos , Miócitos Cardíacos/metabolismo , Espécies Reativas de Oxigênio/metabolismo , Triexosilceramidas/metabolismo
3.
Rev Med Interne ; 40(6): 380-388, 2019 Jun.
Artigo em Francês | MEDLINE | ID: mdl-30739754

RESUMO

Hypertrophic cardiomyopathies represent a heterogeneous group of pathophysiological mechanisms and etiologies (genetic or not), which lead to the development of left ventricular hypertrophy. Left ventricular hypertrophy, when not explained by a significant and prolonged increase in post-load (such as severe poorly controlled arterial hypertension or severe aortic stenosis) justifies etiological exploration. The etiology may range from physiological adaptation in the athlete to myocardial involvement, isolated or integrated as part of a global neuromuscular involvement; metabolic or mitochondrial disease to deposition disease. As cardiac signs are non-specific, the clinical examination should focus on looking for a syndromic entity. Considering this pathophysiological heterogeneity, in addition to the biological assays in search of a metabolic or infiltrative cause, the minimum check-up must include an electrocardiogram and a transthoracic echocardiography, which will most of the time be completed by magnetic resonance imaging, and even bone scintigraphy in the event of suspected amyloidosis. The question of genetic analysis and/or counselling should be systematically considered. The treatment is mainly symptomatic, aimed at controlling congestive signs and/or intraventricular obstruction, with the exception of amyloidosis and Fabry disease for which dedicated treatments have been developed. The rhythmic risk must be evaluated and can justify the implantation of an automatic defibrillator.


Assuntos
Cardiomiopatia Hipertrófica , Algoritmos , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/etiologia , Cardiomiopatia Hipertrófica/terapia , Humanos
4.
Int J Cardiol ; 281: 69-75, 2019 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-30711265

RESUMO

BACKGROUND: Intrinsic myocardial mechanics might have different patterns because of the different etiologies of myocardial hypertrophy. We used layer-specific strain to compare those with aortic stenosis (AS) and hypertrophic cardiomyopathy (HCM) and examined the differences in strain distribution pattern and for their clinical implications. METHODS: Comprehensive echocardiography was done in 3 groups: 129 with moderate-to-severe AS, 172 consecutive patients with HCM, and 58 healthy controls. Left ventricle (LV) layer-specific deformation parameters were obtained using two-dimensional speckle tracking echocardiography. The transmural strain gradient was defined as the strain difference between subendocardial and subepicardial myocardium. Both diseased groups were further divided based on the median value of transmural strain gradient for the hemodynamics correlation. RESULTS: Compared with the HCM group, the AS group had more preserved transmural longitudinal strain gradient (4.49 ±â€¯1.3% vs. 3.61 ±â€¯1.2%, p < 0.001), which was not significantly different from that of the healthy controls (4.49 ±â€¯1.3% vs. 4.54 ±â€¯1.0%, p = 0.975). And only in AS group the transmural circumferential strain correlated with myocardium mass index (r = -0.237, p = 0.008), and the hemodynamic profiles (LV ejection fraction and LA pressure) were correlated well with transmural strain gradient, in that the lower subgroup had a significantly lower LV ejection fraction and higher average E/E'. CONCLUSIONS: Myocardium hypertrophy from different etiology resulted in different layer-specific strain distribution pattern. The loss of an adequate transmural strain gradient correlated with hemodynamics and might reflect intrinsic myocardial dysfunction.


Assuntos
Cardiomiopatia Hipertrófica/diagnóstico por imagem , Cardiomiopatia Hipertrófica/etiologia , Volume Sistólico/fisiologia , Função Ventricular Esquerda/fisiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Cardiomiopatia Hipertrófica/fisiopatologia , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
5.
Acta Clin Belg ; 74(2): 82-85, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29717636

RESUMO

Introduction LEOPARD syndrome is a rare genetic disorder characterised by lentigines, electrocardiographic conduction abnormalities, ocular hypertelorism, pulmonary stenosis, abnormal genitalia, retardation of growth and sensorineural deafness. Clinical manifestations are often mild, which may result in difficult and late diagnosis. Cardiac involvement may have a significant impact on the prognosis, however, appearance of severe abnormalities such as hypertrophic cardiomyopathy usually precedes the occurrence of multiple lentigines and may be asymptomatic. Case presentation We report two cases of LEOPARD syndrome with hypertrophic cardiomyopathy in a 10-year-old girl and an 18-year-old boy. In both cases, multiple lentigines, ocular hypertelorism and growth retardation were present. The first patient was followed up at the paediatric cardiology clinic due to the risk of progression of septal hypertrophy and pressure gradient across the left ventricular outflow tract, the second patient underwent surgery for a moderate obstruction of the left ventricular outflow tract with uncomplicated post-operative follow-up. Conclusion In both presented patients, hypertrophic cardiomyopathy was clinically silent and the murmur over the precordium was the sole cardiac abnormality revealed during routine visit. A detailed cardiologic examination should be considered in the patients with suspicion of LEOPARD syndrome since the ventricular hypertrophy is thought to precede the occurrence of lentigines and progress over time.


Assuntos
Cardiomiopatia Hipertrófica/etiologia , Síndrome LEOPARD/complicações , Adolescente , Cardiomiopatia Hipertrófica/diagnóstico , Criança , Feminino , Humanos , Masculino
6.
Rev Esp Cardiol (Engl Ed) ; 72(6): 479-486, 2019 Jun.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-30108015

RESUMO

INTRODUCTION AND OBJECTIVES: Danon disease (DD) is caused by mutations in the LAMP2 gene. It is considered a multisystemic disease characterized by hypertrophic cardiomyopathy with pre-excitation and extreme hypertrophy, intellectual disability, myopathy, childhood presentation, and worse prognosis in men. There are scarce data on the clinical characteristics and prognosis of DD. METHODS: We analyzed the clinical records of patients with DD from 10 Spanish hospitals. RESULTS: Twenty-seven patients were included (mean age, 31 ± 19 years; 78% women). Male patients showed a high prevalence of extracardiac manifestations: myopathy (80%), learning disorders (83%), and visual alterations (60%), which were uncommon findings in women (5%, 0%, and 27%, respectively). Although hypertrophic cardiomyopathy was the most common form of heart disease (61%), the mean maximum wall thickness was 15 ± 7 mm and dilated cardiomyopathy was present in 12 patients (10 women). Pre-excitation was found in only 11 patients (49%). Age at presentation was older than 20 years in 16 patients (65%). After a median follow-up of 4 years (interquartile range, 2-9), 4 men (67%) and 9 women (43%) died or required a transplant. Cardiac disease and adverse events occurred later in women (37 ± 9 vs 23 ± 16 and 36 ± 20 vs 20 ± 11 years, respectively). CONCLUSIONS: The clinical characteristics of DD differ substantially from traditional descriptions: age at presentation of DD is older, the disease is not multisystemic in women, and pre-excitation is infrequent.


Assuntos
Cardiomiopatia Hipertrófica/etiologia , Doença de Depósito de Glicogênio Tipo IIb/diagnóstico , Sistema de Registros , Síndrome de Wolff-Parkinson-White/etiologia , Adolescente , Adulto , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/epidemiologia , Eletrocardiografia , Feminino , Doença de Depósito de Glicogênio Tipo IIb/complicações , Doença de Depósito de Glicogênio Tipo IIb/genética , Humanos , Incidência , Proteína 2 de Membrana Associada ao Lisossomo/genética , Proteína 2 de Membrana Associada ao Lisossomo/metabolismo , Masculino , Mutação , Fenótipo , Prognóstico , Estudos Retrospectivos , Espanha/epidemiologia , Síndrome de Wolff-Parkinson-White/diagnóstico , Síndrome de Wolff-Parkinson-White/epidemiologia , Adulto Jovem
7.
ESC Heart Fail ; 6(1): 122-130, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30284755

RESUMO

AIMS: Mutant transthyretin (ATTRm) amyloidosis is a systemic disease caused by the deposition of amyloid fibrils derived from mutated transthyretin. Although cardiac involvement impacts the prognosis of patients with ATTRm amyloidosis, the incidence of cardiac events, such as bradyarrhythmia, ventricular tachycardia, and heart failure, has not been fully elucidated. The aim of this study was to evaluate the prognosis and predictors of clinical outcomes, including cardiac events, in patients with ATTRm amyloidosis in Japan. METHODS AND RESULTS: We evaluated 90 consecutive patients with ATTRm amyloidosis at Kumamoto University. ATTRm amyloidosis was diagnosed by the observation of both amyloid fibril deposition on tissue biopsy and a transthyretin mutation on sequential analysis. Sympathetic nerve activity was evaluated in 59 patients using 123-iodine metaiodobenzylguanidine (123 I-MIBG) imaging. The endpoint was a composite of all-cause death, hospitalization for heart failure, and implantation of a pacemaker, implantable cardioverter defibrillator, or cardiac resynchronization therapy defibrillator. Sixty-seven patients had the Val30Met mutation (74%). The composite endpoint occurred in 23 patients (26%): all-cause death (n = 6), hospitalization for worsening heart failure (n = 1), and implantation of an implantable cardioverter defibrillator (n = 6), cardiac resynchronization therapy defibrillator (n = 3), or pacemaker (n = 7). The 5-year incident rate for clinical outcomes was 19%. In a multivariate Cox hazard analysis, age [hazard ratio (HR): 1.07, 95% confidence interval (95% CI): 1.01-1.12, P = 0.015], PQ interval (HR: 1.01, 95% CI: 1.00-1.02, P = 0.042), interventricular septum thickness in diastole (HR: 1.25, 95% CI: 1.09-1.42, P = 0.001), and non-Val30Met mutation (HR: 4.31, 95% CI: 1.53-12.16, P = 0.006) were independent predictive factors of clinical outcomes. Kaplan-Meier analysis demonstrated a significantly higher probability of the composite endpoint in the non-Val30Met group than in the Val30Met group (log-rank test: P = 0.002) and in patients with left ventricular hypertrophy than in patients without left ventricular hypertrophy (log-rank test: P < 0.001). In patients who underwent 123 I-MIBG imaging, a delayed heart-to-mediastinum (HM) ratio <1.6 was a significant predictive factor of the composite endpoint (HR: 4.98, 95% CI: 1.73-14.37, P = 0.003) in the univariate Cox hazard analyses. Kaplan-Meier curve analysis showed that a delayed HM ratio <1.6 was associated with a poor prognosis (log-rank test: P = 0.001). CONCLUSIONS: Non-Val30Met mutation, septal hypertrophy, and a delayed HM ratio are useful predictors of clinical outcomes in patients with ATTRm amyloidosis in Japan. These results suggest that it is important to evaluate cardiac involvement in terms of morphological (left ventricular hypertrophy) and functional (cardiac denervation) perspectives using echocardiography and 123 I-MIBG imaging, respectively.


Assuntos
Neuropatias Amiloides Familiares/genética , Cardiomiopatia Hipertrófica/genética , DNA/genética , Septos Cardíacos/diagnóstico por imagem , Mutação , Pré-Albumina/genética , Simpatectomia/métodos , Idoso , Neuropatias Amiloides Familiares/terapia , Terapia de Ressincronização Cardíaca/métodos , Cardiomiopatia Hipertrófica/etiologia , Cardiomiopatia Hipertrófica/terapia , Análise Mutacional de DNA , Ecocardiografia , Eletrocardiografia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Pré-Albumina/metabolismo , Estudos Retrospectivos
9.
Medicine (Baltimore) ; 97(50): e13369, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30557989

RESUMO

RATIONALE: Catecholamine-producing tumors are rare, occurring in less than 0.2% of patients with hypertension, but can have relevant cardiovascular morbidity and mortality. PATIENT CONCERNS: A 37-year-old woman presented with a history of dyspnea, chest pain, palpitations, and paroxysmal hypertension. Electrocardiogram, echocardiogram, and cardiac magnetic resonance showed severe LVH with a prevalent involvement of the anterior portion of interventricular septum. Endomyocardial biopsy found severe hypertrophy with disarray of cardiomyocytes and ultrastructural evidence of contraction and necrosis of myocytes. Hormone investigations revealed high values of 24-hours urinary metanephrines. Abdominal computed tomography (CT) showed an enlarged left adrenal gland with a strong uptake of I-metaiodobenzylguanidine at scintigraphy scan. INTERVENTIONS: Thus, the adrenal tumor was surgically removed. OUTCOMES: At follow-up examination, the patient's metanephrines levels were normalized and the transthoracic echocardiogram showed a reduction of LVH. DIAGNOSIS AND LESSONS: We report a rare case of catecholamine-induced cardiomyopathy due to an adrenal adenoma mixed with nodules enriched in epinephrine-types secreting granules.


Assuntos
Cardiomiopatia Hipertrófica/etiologia , Catecolaminas/análise , Neoplasias/sangue , Neoplasias/complicações , Adulto , Cardiomiopatia Hipertrófica/sangue , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Catecolaminas/sangue , Dor no Peito/etiologia , Dispneia/etiologia , Eletrocardiografia/métodos , Feminino , Humanos , Hipertensão/etiologia , Neoplasias/diagnóstico por imagem , Cintilografia/métodos , Tomografia Computadorizada por Raios X/métodos
10.
Europace ; 20(suppl_3): iii102-iii112, 2018 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-30476051

RESUMO

Aims: To identify key structural and electrophysiological features explaining distinct electrocardiogram (ECG) phenotypes in hypertrophic cardiomyopathy (HCM). Methods and results: Human heart-torso anatomical models were constructed from cardiac magnetic resonance (CMR) images of HCM patients, representative of ECG phenotypes identified previously. High performance computing simulations using bidomain models were conducted to dissect key features explaining the ECG phenotypes with increased HCM Risk-SCD scores, namely Group 1A, characterized by normal QRS but inverted T waves laterally and coexistence of apical and septal hypertrophy; and Group 3 with marked QRS abnormalities (deep and wide S waves laterally) and septal hypertrophy. Hypertrophic cardiomyopathy abnormalities characterized from CMR, such as hypertrophy, tissue microstructure alterations, abnormal conduction system, and ionic remodelling, were selectively included to assess their influence on ECG morphology. Electrocardiogram abnormalities could not be explained by increased wall thickness nor by local conduction abnormalities associated with fibre disarray or fibrosis. Inverted T wave with normal QRS (Group 1A) was obtained with increased apico-basal repolarization gradient caused by ionic remodelling in septum and apex. Lateral QRS abnormalities (Group 3) were only recovered with abnormal Purkinje-myocardium coupling. Conclusion: Two ECG-based HCM phenotypes are explained by distinct mechanisms: ionic remodelling and action potential prolongation in hypertrophied apical and septal areas lead to T wave inversion with normal QRS complexes, whereas abnormal Purkinje-myocardial coupling causes abnormal QRS morphology in V4-V6. These findings have potential implications for patients' management as they point towards different arrhythmia mechanisms in different phenotypes.


Assuntos
Potenciais de Ação , Cardiomiopatia Hipertrófica/diagnóstico , Simulação por Computador , Eletrocardiografia , Acoplamento Excitação-Contração , Frequência Cardíaca , Modelos Cardiovasculares , Contração Miocárdica , Ramos Subendocárdicos/fisiopatologia , Cardiomiopatia Hipertrófica/etiologia , Cardiomiopatia Hipertrófica/fisiopatologia , Humanos , Imagem por Ressonância Magnética , Fenótipo , Valor Preditivo dos Testes , Remodelação Ventricular
11.
J Pediatr Endocrinol Metab ; 31(12): 1371-1376, 2018 Dec 19.
Artigo em Inglês | MEDLINE | ID: mdl-30352041

RESUMO

Background Hypertrophic cardiomyopathy (HCM) in childhood is a rare diagnosis, and associations with adrenocortical tumors (ACTs) have been rarely reported in the pediatric literature. Case presentation We present a case of a 5-month-old who presented with HCM and during the evaluation for hypertension was found to have elevated glucocorticoids, mineralocorticoids, androgens and urine metanephrines. During preoperative evaluation, he developed shock followed by cardiogenic collapse requiring extracorporeal membrane oxygenation (ECMO); however, he did not survive. Pathology revealed an ACT with hormone production that contributed to his demise. Conclusions Adrenocortical tumors associated with hypertrophic cardiomyopathy can be life-threatening. We discuss the complex interplay of unrestricted cortical hormone production in the setting of hypertrophic cardiomyopathy that may lead to rapid decline and poor clinical outcomes.


Assuntos
Neoplasias do Córtex Suprarrenal/complicações , Cardiomiopatia Hipertrófica/etiologia , Choque Cardiogênico/terapia , Neoplasias do Córtex Suprarrenal/sangue , Androgênios/sangue , Cardiomiopatia Hipertrófica/sangue , Oxigenação por Membrana Extracorpórea , Evolução Fatal , Glucocorticoides/sangue , Humanos , Lactente , Masculino , Mineralocorticoides/sangue , Choque Cardiogênico/sangue
12.
Arq Bras Cardiol ; 110(6): 524-531, 2018 Jun.
Artigo em Inglês, Português | MEDLINE | ID: mdl-30226910

RESUMO

BACKGROUND: The new European Society of Cardiology guidelines for hypertrophic cardiomyopathy (HCM) define the estimation of sudden cardiac death (SCD) risk as an integral part of clinical management. An implantable cardioverter defibrillator (ICD) is recommended (class IIa) when the risk is ≥ 6%. OBJECTIVES: To compare the SCD risk stratification according to the 2011 and 2014 recommendations for ICD implantation in patients with HCM. METHODS: Retrospective study including 105 patients diagnosed with HCM. The indication for ICD was assessed using the 2011 and 2014 guidelines. Statistical analysis was performed using SPSS software version 19.0.0.2®. The tests performed were bilateral, considering the significance level of 5% (p < 0.05). RESULTS: Regarding primary prevention, according to the 2011 ACCF/AHA recommendations, 39.0% of the patients had indication for ICD implantation (level of evidence IIa). Using the 2014 guidelines, only 12.4% of the patients had an indication for ICD implantation. Comparing the two risk stratification models for patients with HCM, we detected a significant reduction in the number of indications for ICD implantation (p < 0.001). Of the 41 patients classified as IIa according to the 2011 recommendations, 68.3% received a different classification according to the 2014 guidelines. CONCLUSION: Significant differences were found when comparing the SCD risk stratification for ICD implantation in the two guidelines. The current SCD risk score seems to identify many low-risk patients who are not candidates for ICD implantation. The use of this new score results in a significant reduction in the number of ICD implanted.


Assuntos
Cardiomiopatia Hipertrófica/mortalidade , Morte Súbita Cardíaca/prevenção & controle , Desfibriladores Implantáveis/estatística & dados numéricos , Guias de Prática Clínica como Assunto/normas , Medição de Risco/métodos , Adulto , Idoso , Arritmias Cardíacas/complicações , Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/fisiopatologia , Cardiomiopatia Hipertrófica/etiologia , Cardiomiopatia Hipertrófica/fisiopatologia , Cardiomiopatia Hipertrófica/prevenção & controle , Morte Súbita Cardíaca/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Portugal/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Volume Sistólico , Fatores de Tempo
15.
Echocardiography ; 35(8): 1230-1232, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29870575

RESUMO

We successfully treated a patient who was diagnosed of having hypertrophic obstructive cardiomyopathy after the aortic valve replacement surgery for the concomitant aortic stenosis. We report this first in kind new procedure exclusively developed in our center, Liwen procedureTM (percutaneous intramyocardial septal radiofrequency ablation), for patients with left ventricular outflow tract obstruction in spite of maximal medical therapy. The procedure was performed under transthoracic echo guidance. We discussed the technical details, safety, and effectiveness with corresponding images. The patient did well one year after the procedure without LVOT obstruction or arrhythmia.


Assuntos
Estenose da Valva Aórtica/cirurgia , Cardiomiopatia Hipertrófica/cirurgia , Ablação por Cateter/métodos , Ecocardiografia/métodos , Cirurgia Assistida por Computador/métodos , Toracoscopia/métodos , Substituição da Valva Aórtica Transcateter/métodos , Estenose da Valva Aórtica/complicações , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/etiologia , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Miocárdio
20.
Heart Lung Circ ; 27(6): 752-755, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29398473

RESUMO

BACKGROUND: Individuals with hypertrophic cardiomyopathy (HCM) may be asymptomatic or display activity-limiting symptoms. A common cause of symptoms is left ventricular outflow tract obstruction (LVOTO), which may impact the individuals' ability to undertake physical activity. This study sought to examine daily step count as a potential marker of exercise capacity, which may represent a proxy marker of disease severity in HCM. METHODS: A cross-sectional study of 63 HCM patients was conducted from March to November 2015. Participants wore an ActiGraph GT3X+ (Pensacola, Florida, USA) accelerometer for 7 days. Minutes per day of light, moderate and vigorous physical activity and step count were calculated, and those with LVOTO were compared to those without. Similarly, those with good functional capacity (New York Heart Association; NYHA class I) were compared to those with NYHA class II-IV. RESULTS: The majority of HCM patients were male (n=45, 71%) with mean age of 48.8±14.9years. Hypertrophic cardiomyopathy patients with history of LVOTO and those NYHA class II-IV took significantly fewer steps per day (LV obstruction: 5527±2370 versus 7027±2095, p=0.01 and NYHA: 5346±1898 versus 6801±2339, p=0.03). No differences were observed across the different intensities of physical activity. CONCLUSIONS: Measurement of daily step count may be a useful and simple tool to determine exercise capacity and provide an indicator of disease severity in individuals with HCM.


Assuntos
Acelerometria/métodos , Cardiomiopatia Hipertrófica/fisiopatologia , Tolerância ao Exercício/fisiologia , Obstrução do Fluxo Ventricular Externo/complicações , Caminhada/fisiologia , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/etiologia , Estudos Transversais , Feminino , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Índice de Gravidade de Doença , Obstrução do Fluxo Ventricular Externo/diagnóstico , Obstrução do Fluxo Ventricular Externo/fisiopatologia
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