Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 835
Filtrar
1.
J. bras. econ. saúde (Impr.) ; 15(3): 190-199, Dezembro/2023.
Artigo em Inglês, Português | LILACS, ECOS | ID: biblio-1553993

RESUMO

Objective: To generate data on the costs associated with the diagnosis and treatment of obstructive ypertrophic cardiomyopathy (HCM) from the perspective of the private health system in Brazil. Methods: A modified Delphi panel including seven different specialists (three clinical cardiologists with experience in obstructive HCM, two hemodynamicists with experience in septal ablation and two cardiac surgeons with expertise in myectomy), from two Brazilian states (São Paulo and Pernambuco), was conducted between August and November 2022. Two rounds of questions about the use of healthcare resources according to the functional class (NYHA I-IV) and a panel in a virtual platform were conducted to obtain the final consensus. Micro-costing defined costs and unit values were determined based on official price lists. Results: The total diagnosis cost per patient was estimated at BRL 11,486.81. The obstructive HCM management costs analysis showed average annual costs per patient of BRL 17,026.74, BRL 19,401.46, BRL 73,310.07, and BRL 94,885.75 for the functional classes NYHA I, NYHA II, NYHA III, and NYHA IV, respectively. The average costs per patient related to procedures in a year were BRL 12,698.53, BRL 13,462.30, BRL 58,841.67, and BRL 75,595.90 for the functional classes NYHA I, II, III, and IV, respectively. Conclusions: The annual costs of HCM management increased according to the functional class, highlighting the need for safe and effective strategies to improve patient's NYHA functional class while promoting a decrease in the need for invasive therapies.


Objetivo: Gerar dados acerca dos custos associados ao diagnóstico e tratamento da cardiomiopatia hipertrófica (CMH) obstrutiva, sob a perspectiva do sistema de saúde privado no Brasil. Métodos: Um painel Delphi modificado incluindo sete especialistas (três cardiologistas clínicos com experiência em CMH obstrutiva, dois hemodinamicistas com experiência em ablação de septo e dois cirurgiões cardíacos com experiência em miectomia) de dois estados brasileiros (São Paulo e Pernambuco) foi conduzido entre agosto e novembro de 2022. Foram realizadas duas rodadas de perguntas acerca da utilização de recursos de acordo com a classe funcional (NYHA I-IV) e uma reunião virtual para obtenção do consenso final. Os custos foram definidos por meio de microcusteio, e os valores unitários foram definidos com base em listas de preço oficiais. Resultados: O custo total do diagnóstico por paciente foi estimado em R$ 11.486,81. A análise de custos de manejo da CMH obstrutiva mostrou custos médios anuais por paciente de R$ 17.026,74, R$ 19.401,46, R$ 73.310,07 e R$ 94.885,75 para as classes funcionais NYHA I, NYHA II, NYHA III e NYHA IV, respectivamente. Os custos médios por paciente relacionados a procedimentos em um ano foram de R$ 12.698,53, R$ 13.462,30, R$ 58.841,67 e R$ 75.595,90 para as classes NYHA I, II, III e IV, respectivamente. Conclusões: Os custos anuais com o manejo da CMH aumentam de acordo com a classe funcional, destacando a necessidade de estratégias seguras e eficazes capazes de melhorar a classe funcional NYHA do paciente, ao mesmo tempo que promove diminuição da necessidade de terapias invasivas.


Assuntos
Cardiomiopatia Hipertrófica , Técnica Delphi , Custos e Análise de Custo , Saúde Suplementar
2.
Journal of Southern Medical University ; (12): 660-666, 2023.
Artigo em Chinês | WPRIM | ID: wpr-986976

RESUMO

OBJECTIVE@#To quantitatively assess cardiac functions in patients with cardiac amyloidosis (CA) and hypertrophic cardiomyopathy (HCM) using cardiac magnetic resonance-feature tracking (CMR-FT) technique and evaluate the prognostic value of CMR-FT in patients with CA.@*METHODS@#We retrospectively collected the data from 31 CA patients with systemic amyloidosis confirmed by Congo red staining and serum immunohistochemistry after extracardiac tissue biopsy undergoing CMR at our hospital from March, 2013 to June, 2021.Thirty-one age and gender matched patients with asymmetric left ventricular wall hypertrophy and 31 healthy individuals without organic or functional heart disease served as the controls.Radial, circumferential and longitudinal strains and strain rates of the left ventricle at the global level and in each myocardial segment (basal, middle and apical) were obtained with CMR-FT technique and compared among the 3 groups.The predictive value of myocardial strains and strain rates for all-cause mortality in CA patients was analyzed using a stepwise COX regression model.@*RESULTS@#The left ventricular volume, myocardial mass, ejection fraction and cardiac output differed significantly among the groups (P < 0.05).Except for apical longitudinal strain, the global and segmental strains were all significantly lower in CA group than in HCM group (P < 0.05).The global and segmental strains were all significantly lower in CA group than in the healthy individuals (P < 0.05).The basal strain rates in the 3 directions were significantly lower in CA group than in the healthy individuals (P < 0.05), but the difference in apical strain rates was not statistically significant between the two groups.Multivariate stepwise COX analysis showed that troponin T (HR=1.05, 95%CI: 1.01-1.10, P=0.017) and middle peak diastolic circumferential strain rate (HR=6.87, 95%CI: 1.52-31.06, P=0.012) were strong predictors of death in CA patients.@*CONCLUSION@#Strain and strain rate parameters derived from CMR-FT based on cine sequences are new noninvasive imaging markers for assessing cardiac impairment in CA and cardiac function changes in HCM, and provide independent predictive information for all-cause mortality in CA patients.


Assuntos
Humanos , Estudos Retrospectivos , Imagem Cinética por Ressonância Magnética/métodos , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Função Ventricular Esquerda , Volume Sistólico , Amiloidose/diagnóstico por imagem , Espectroscopia de Ressonância Magnética , Prognóstico , Valor Preditivo dos Testes
3.
Chinese Journal of Contemporary Pediatrics ; (12): 425-430, 2023.
Artigo em Chinês | WPRIM | ID: wpr-981974

RESUMO

Hypertrophic cardiomyopathy (HCM) is the most common monogenic inherited myocardial disease in children, and mutations in sarcomere genes (such as MYH7 and MYBPC3) are the most common genetic etiology of HCM, among which mutations in the MYH7 gene are the most common and account for 30%-50%. MYH7 gene mutations have the characteristics of being affected by environmental factors, coexisting with multiple genetic variations, and age-dependent penetrance, which leads to different or overlapping clinical phenotypes in children, including various cardiomyopathies and skeletal myopathies. At present, the pathogenesis, course, and prognosis of HCM caused by MYH7 gene mutations in children remain unclear. This article summarizes the possible pathogenesis, clinical phenotype, and treatment of HCM caused by MYH7 gene mutations, in order to facilitate the accurate prognostic evaluation and individualized management and treatment of the children with this disorder.


Assuntos
Criança , Humanos , Cardiomiopatia Hipertrófica/terapia , Fenótipo , Troponina T/genética , Mutação , Proteínas de Transporte/genética , Cadeias Pesadas de Miosina/genética , Miosinas Cardíacas/genética
4.
Chinese Journal of Medical Genetics ; (6): 452-457, 2023.
Artigo em Chinês | WPRIM | ID: wpr-981769

RESUMO

OBJECTIVE@#To explore the clinical and genetic characteristics of a patient with hypertrophic cardiomyopathy as the initial manifestation of Mucopolysaccharidosis type Ⅲ A (MPS Ⅲ A).@*METHODS@#A female patient with MPS Ⅲ A who was admitted to the Affiliated Hospital of Jining Medical University in January 2022 and her family members (seven individuals from three generations) were selected as the study subjects. Clinical data of the proband were collected. Peripheral blood samples of the proband was collected and subjected to whole exome sequencing. Candidate variants were verified by Sanger sequencing. Heparan-N-sulfatase activity was determined for the disease associated with the variant site.@*RESULTS@#The proband was a 49-year-old woman, for whom cardiac MRI has revealed significant thickening (up to 20 mm) of left ventricular wall and delayed gadolinium enhancement at the apical myocardium. Genetic testing revealed that she has harbored compound heterozygous variants in exon 17 of the SGSH gene, namely c.545G>A (p.Arg182His) and c.703G>A (p.Asp235Asn). Based on guidelines from the American College of Medical Genetics and Genomics (ACMG), both variants were predicted to be pathogenic (PM2_Supporting +PM3+PP1Strong+PP3+PP4; PS3+PM1+PM2_Supporting +PM3+PP3+PP4). Sanger sequencing confirmed that her mother was heterozygous for the c.545G>A (p.Arg182His) variant, whilst her father, sisters and her son were heterozygous for the c.703G>A (p.Asp235Asn) variant. Determination of blood leukocyte heparan-N-sulfatase activity suggested that the patient had a low level of 1.6 nmol/(g·h), whilst that of her father, elder and younger sisters and son were all in the normal range.@*CONCLUSION@#The compound heterozygous variants of the SGSH gene probably underlay the MPS ⅢA in this patient, for which hypertrophic cardiomyopathy is an associated phenotype.


Assuntos
Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Cardiomiopatia Hipertrófica , Meios de Contraste , População do Leste Asiático , Gadolínio , Mucopolissacaridose III , Mutação , Linhagem
5.
Chinese Journal of Medical Genetics ; (6): 337-343, 2023.
Artigo em Chinês | WPRIM | ID: wpr-970929

RESUMO

OBJECTIVE@#To explore the clinical phenotype and genetic features of a child with dilated cardiomyopathy (DCM).@*METHODS@#Clinical data of the child who had presented at the Zhengzhou Children's Hospital on April 28, 2020 was collected. Trio-whole exome sequencing (trio-WES) was carried out for the child and her parents, and candidate variants were validated by Sanger sequencing. "FHL2" was taken as the key word to retrieve related literature from January 1, 1997 to October 31, 2021 in the PubMed database and was also searched in the ClinVar database as a supplement to analyze the correlation between genetic variants and clinical features.@*RESULTS@#The patient was a 5-month-old female infant presented with left ventricular enlargement and reduced systolic function. A heterozygous missense variant c.391C>T (p.Arg131Cys) in FHL2 gene was identified through trio-WES. The same variant was not detected in either of her parents. A total of 10 patients with FHL2 gene variants have been reported in the literature, 6 of them had presented with DCM, 2 with hypertrophic cardiomyopathy (HCM), and 2 with sudden unexplained death (SUD). Phenotypic analysis revealed that patients with variants in the LIM 3 domain presented hypertrophic cardiomyopathy and those with variants of the LIM 0~2 and LIM 4 domains had mainly presented DCM. The c.391C>T (p.Arg131Cys) has been identified in a child with DCM, though it has not been validated among the patient's family members. Based on the guidelines of the American College of Medical Genetics and Genomics, the c.391C>T(p.Arg131Cys) variant was re-classified as likely pathogenic (PS2+PM2_Supporting+PP3+PP5).@*CONCLUSION@#The heterozygous missense variant of c.391C>T (p.Arg131Cys) in the FHL2 gene probably predisposed to the DCM in this child, which has highlighted the importance of WES in the clinical diagnosis and genetic counseling.


Assuntos
Feminino , Humanos , Cardiomiopatia Dilatada/genética , Cardiomiopatia Hipertrófica , Aconselhamento Genético , Genômica , Heterozigoto , Proteínas Musculares/genética , Fatores de Transcrição , Proteínas com Homeodomínio LIM/genética
6.
Chinese Journal of Surgery ; (12): 214-219, 2023.
Artigo em Chinês | WPRIM | ID: wpr-970183

RESUMO

Objective: To examine the early effect of thoracoscopic trans-mitral myectomy for hypertrophic cardiomyopathy patients with left midventricular obstruction. Methods: From April 2020 to July 2021, 10 hypertrophic cardiomyopathy patients with left midventricular obstruction underwent thoracoscopic trans-mitral myectomy at Guangdong Provincial People's Hospital. The whole group of patients consisted of 7 males and 3 females aged (52.0±16.4) years (range: 18 to 68 years). The EuroSCORE Ⅱ predicted mortality rate was 1.78% (1.20%) (M(IQR)) (range: 0.96% to 4.86%). The clinical data were collected and analyzed retrospectively to evaluate the clinical efficacy by comparing preoperative and postoperative echocardiographic parameters using paired t-test, paired Wilcoxon test or Fisher exact test, including left ventricular outflow tract peak pressure gradient, maximum interventricular septum thickness, systolic anterior motion of the anterior mitral leaflet and so on. The safety was determined by summarizing the incidence of perioperative and follow-up complications. Results: All the procedures successed with no conversion to median sternotomy, septal defect, ventricular rupture. There was no in-hospital 30-day death, neither serious complications like permanent pacemaker implantation, re-sternotomy for bleeding, low cardiac output syndrome, stroke, or multiple organ dysfunction syndrome. The left ventricular outflow tract obstruction was effectively relieved in all patients expect a patient developed residual obstruction. Compared with that of pre-operation, the thickness of the interventricular septum was significantly reduced from (22.1±4.0) mm to (10.3±1.7) mm (t=10.693, P<0.01), while the left ventricular outflow tract peak pressure gradient was significantly reduced from (81.7±21.1) mmHg to 12.3 (11.5) mmHg (Z=-2.805, P<0.01) (1 mmHg=0.133 kPa). Conclusion: Thoracoscopic trans-mitral myectomy is an effective and safe procedure for hypertrophic cardiomyopathy patients with left midventricular obstruction.


Assuntos
Masculino , Feminino , Humanos , Estudos Retrospectivos , Ecocardiografia , Resultado do Tratamento , Cardiomiopatia Hipertrófica/complicações , Septo Interventricular
7.
Chinese Journal of Surgery ; (12): 209-213, 2023.
Artigo em Chinês | WPRIM | ID: wpr-970182

RESUMO

Objective: To examine the short-term curative effect with minimally invasive right infra-axillary thoracotomy for transaortic modified Morrow procedure. Methods: The clinical data of 60 patients who underwent video-assisted thoracoscopic transaortic modified Morrow procedure from August 2021 to August 2022 at Department of Cardiovascular Surgery, Zhejiang Provincial People's Hospital were retrospectively analyzed. There were 31 males and 29 females, with the age (M (IQR)) of 54.0(22.3) years (range: 15 to 71 years). The echocardiography confirmed the diagnosis of moderate mitral regurgitation in 30 patients, and severe mitral regurgitation in 13 patients. Systolic anterior motion (SAM) was present preoperatively in 54 patients. All 60 patients underwent transaortic modified Morrow procedure through a right infra-axillary thoracotomy using femorofemoral cardiopulmonary bypass. Surgical procedures mainly included transverse aortic incision, exposure of left ventricular outflow tract (LVOT), septal myectomy, and correction of the abnormal mitral valve and subvalvular structures. Results: All 60 patients underwent the programmatic procedures successfully without conversion to full sternotomy. The cardiopulmonary bypass time was (142.0±32.1) minutes (range: 89 to 240 minutes), while the cross-clamp time was (95.0±23.5) minutes (range: 50 to 162 minutes). The patients had a postoperative peak LVOT gradient of 7.0 (5.0) mmHg (range: 0 to 38 mmHg) (1 mmHg=0.133 kPa). A total of 57 patients were extubated on the operating table. The drainage volume in the first 24 h was (175.9±57.0) ml (range: 60 to 327 ml). The length of intensive care unit stay was 21.0 (5.8)h (range: 8 to 120 h) and postoperative hospital stay was 8 (5) days (range: 5 to 19 days). The postoperative septal thickness was 11 (2) mm (range: 8 to 14 mm). All patients had no iatrogenic ventricular septal perforation or postoperative residual SAM. The patients were followed up for 4 (9) months (range: 1 to 15 months), and none of them needed cardiac surgery again due to valve dysfunction or increased peak LVOT gradient during follow-up. Conclusion: Using a video-assisted thoracoscopic transaortic modified Morrow procedure through a right infra-axillary minithoracotomy can provide good visualization of the LVOT and hypertrophic ventricular septum, ensure optimal exposure of the mitral valve in the presence of complex mitral subvalvular structures, so that allows satisfactory short-term surgical results.


Assuntos
Masculino , Feminino , Humanos , Insuficiência da Valva Mitral/cirurgia , Toracotomia , Estudos Retrospectivos , Cardiomiopatia Hipertrófica/cirurgia , Septo Interventricular/cirurgia , Resultado do Tratamento , Procedimentos Cirúrgicos Minimamente Invasivos/métodos
8.
Chinese Journal of Surgery ; (12): 201-208, 2023.
Artigo em Chinês | WPRIM | ID: wpr-970181

RESUMO

Objectives: To examine the short-term and mid-term effects of surgical treatment of obstructive hypertrophic cardiomyopathy (HCM) in one center. Methods: The perioperative data and short-term follow-up outcomes of 421 patients with obstructive HCM who received surgical treatment at Department of Cardiac Surgery, Zhongshan Hospital, Fudan University from January 2017 to December 2021 were analyzed retrospectively. There were 207 males and 214 females, aged (56.5±11.7) years (range: 19 to 78 years). Preoperative New York Heart Association (NYHA) classification included 45 cases of class Ⅱ, 328 cases in class Ⅲ, and 48 cases in class Ⅳ. Fifty-eight patients were diagnosed with latent obstructive HCM and 257 patients had moderate or more mitral regurgitation with 56 patients suffering from intrinsic mitral valve diseases. All procedures were completed by a multidisciplinary team, including professional echocardiologists involving in preoperative planning for proper mitral valve management strategies and intraoperative monitoring. A total of 338 patients underwent septal myectomy alone, and 59 patients underwent mitral valve surgery along with myectomy. A single transaortic approach was used in 355 patients, and a right atrial-atrial septal/atrial sulcus approach was used in 51 other patients. Long-handled minimally invasive surgical instruments were used for the procedures. Student t test, Wilcoxon rank sum test, χ2 test or Fisher exact test were used to compare the data before and after surgery. Results: The aortic cross-clamping time of septal myectomy alone was (34.3±8.5) minutes (range: 21 to 94 minutes). Eighteen patients had intraoperative adverse events and underwent immediate reoperation, including residual obstruction (10 patients), left ventricular free wall rupture (4 patients), ventricular septal perforation (3 patients), and aortic valve perforation (1 patient). Four patients died during hospitalization, and 11 patients developed complete atrioventricular block requiring permanent pacemaker implantation. After discharge, 384 (92.1%) patients received a follow-up visit with a median duration of 9 months. All follow-up patients survived with significantly improved NYHA classifications: 216 patients in class Ⅰ and 168 patients in class Ⅱ (χ2=662.73, P<0.01 as compared to baseline). At 6 months after surgery, follow-up echocardiography showed that the thickness of the ventricular septum ((13.6±2.5) mm vs. (18.2±3.0) mm, t=23.51, P<0.01) and the peak left ventricular outflow tract gradient ((12.0±6.3) mmHg vs. (93.4±19.8) mmHg, 1 mmHg=0.133 kPa, t=78.29, P<0.01) were both significantly lower than baseline values. Conclusion: The construction of the surgical team (including echocardiography experts), proper mitral valve management strategies, identification and management of sub-mitral-valve abnormalities, and application of long-handled minimally invasive surgical instruments are important for the successful implementation of septal myectomy with satisfactory short-and medium-term outcomes.


Assuntos
Masculino , Feminino , Humanos , Estudos Retrospectivos , Fibrilação Atrial , Resultado do Tratamento , Cardiomiopatia Hipertrófica/cirurgia , Insuficiência da Valva Mitral/cirurgia , Septo Interventricular
9.
Chinese Journal of Surgery ; (12): 196-200, 2023.
Artigo em Chinês | WPRIM | ID: wpr-970180

RESUMO

Septal reduction therapies, which include septal myectomy and alcohol septal ablation and so on, are the current treatment strategies for patients with obstructive hypertrophic cardiomyopathy and drug-refractory symptoms. With the deepening of theoretical understanding and the rapid development of interventional therapies, some researchers have tried to perform transcatheter mitral valve edge-to-edge repair to treat high-risk patients with hypertrophic cardiomyopathy, including obstructive and non-obstructive. The reported results are relatively satisfactory, but many urgent problems need to be solved, such as the lack of data on animal experiments and large cohort studies, and the unknown medium- and long-term outcomes. However, transcatheter mitral valve edge-to-edge repair brings new ideas for the diagnosis and treatment of patients with hypertrophic cardiomyopathy. On one hand, it can be used as a monotherapy, on the other hand, it can be combined with novel molecular targeted drug therapy or emerging minimally invasive surgical procedures targeting hypertrophic ventricular septum, which deserves our further attention and exploratory research.


Assuntos
Humanos , Resultado do Tratamento , Cardiomiopatia Hipertrófica/cirurgia , Valva Mitral/cirurgia , Septo Interventricular/cirurgia , Hipertrofia
10.
Chinese Journal of Surgery ; (12): 181-186, 2023.
Artigo em Chinês | WPRIM | ID: wpr-970178

RESUMO

After more than 60 years of development, with the deepening of the pathophysiological understanding of obstructive hypertrophic cardiomyopathy, the extent and resection thickness of myectomy have increased significantly. Myectomy combined with the correction of anomalies of the mitral valve apparatus has become the standard treatment of obstructive hypertrophic cardiomyopathy. Only a few centers worldwide can routinely perform it due to the difficulty. Because of the advances of new drugs and interventional therapy, the development of surgical treatment faces many challenges. At the same time, generations of cardiovascular surgeons are constantly trying to promote septal myectomy, including developing devices and the surgical field, as well as improving surgical planning by advanced technology. At present, the superior long-term efficacy of septal myectomy has been confirmed. It is necessary to work together to promote the treatment of hypertrophic obstructive cardiomyopathy, so as to guard people's health.


Assuntos
Humanos , Cardiomiopatia Hipertrófica/cirurgia , Septos Cardíacos/cirurgia , Valva Mitral/cirurgia , Insuficiência da Valva Mitral/cirurgia , Resultado do Tratamento
11.
Chinese Journal of Surgery ; (12): 54-60, 2023.
Artigo em Chinês | WPRIM | ID: wpr-970173

RESUMO

Objective: To examine the clinical efficacy of myectomy guided by personalized three-dimensional reconstruction and printing for patients with obstructive hypertrophic cardiomyopathy. Methods: The clinical data of 28 patients with obstructive hypertrophic cardiomyopathy, who underwent septal myectomy guided by personalized three-dimensional reconstruction and printing in the Department of Cardiaovascular Surgery, Guangdong Provincial People's Hospital from May 2020 to December 2021, were retrospectively analyzed. There were 14 males and 14 females, aging (51.1±14.0) years (range: 18 to 72 years). Enhanced cardiac computed tomography images were imported into Mimics software for preoperative three-dimensional reconstruction. The direction of the short axial plane of each segment was marked perpendicularly to the interventricular septum on the long axial plane of the digital cardiac model, then the thickness was measured on each short axial plane. A figurative digital model was used to determine the extent of resection and to visualize mitral valve and papillary muscle abnormalities. Correlation between the length, width, thickness, and volume of the predicted resected myocardium and those of the surgically resected myocardium was assessed by Pearson correlation analysis or Spearman correlation analysis. The accuracy of detecting mitral valve and papillary muscle abnormalities of transthoracic echocardiography and three-dimensional reconstruction was also compared. Results: There was no death or serious complications like permanent pacemaker implantation, re-sternotomy for bleeding, low cardiac output syndrome, stroke, or multiple organ dysfunction syndromes in the whole group. Namely, the obstruction of the left ventricular outflow tract was effectively relieved. The systolic anterior motion of the anterior mitral valve leaflet was absent in all patients after myectomy. The length, width, and thickness of the predicted resected myocardium by three-dimensional reconstruction were significantly positively correlated with the length (R=0.65, 95%CI: 0.37 to 0.82, P<0.01), width (R=0.39, 95%CI: 0.02 to 0.67, P<0.01), and thickness (R=0.82, 95%CI: 0.65 to 0.92, P<0.01) of the surgically resected myocardium, while the relation of the volume of the predicted resected myocardium and the volume of the surgically resected myocardium was a strong positive correlation (R=0.88, 95%CI: 0.76 to 0.94, P<0.01). Importantly, the interventricular septal myocardial thickness measured by preoperative transthoracic echocardiography showed a moderate positive correlation with the volume of surgically resected myocardium (R=0.52, 95%CI: 0.19 to 0.75, P<0.01). During a follow-up of (14.4±6.8) months (range: 3 to 22 months), no death occurred, and 1 patient was readmitted for endocardial radiofrequency ablation due to atrial fibrillation. Conclusion: Personalized three-dimensional reconstruction and printing can not only visualize the intracardiac structure but also guide septal myectomy by predicting the thickness, volume, and extent of resected myocardium to achieve ideal resection.


Assuntos
Feminino , Humanos , Masculino , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Cardiomiopatia Hipertrófica/diagnóstico , Imageamento Tridimensional , Impressão Tridimensional , Estudos Retrospectivos , Resultado do Tratamento , Septo Interventricular
12.
In. Acquistapace Peroni, Federico Andres; Agorrody Vidal, Guillermo; Arocena, María; Cuesta Holgado, Alejandro Nicolás; Dell'Oca Runco, Nicolás; Raggio Risso, Víctor Enrique; Reyes Cabrera, María Ximena; Ríos Valdez, Mateo; Tortajada Belocon, Gustavo. Pautas de prevención en las principales cardiopatías hereditarias. [Montevideo], Comisión Honoraria para la Salud Cardiovascular, [2023?]. p.25-31.
Monografia em Espanhol | LILACS, UY-BNMED, BNUY | ID: biblio-1436197
13.
Chinese Journal of Cardiology ; (12): 619-625, 2023.
Artigo em Chinês | WPRIM | ID: wpr-984694

RESUMO

Objective: To explore the value of cardiac magnetic resonance imaging (CMR) in the risk stratification of hypertrophic cardiomyopathy (HCM). Methods: HCM patients who underwent CMR examination in Fuwai Hospital between March 2012 and May 2013 were retrospectively enrolled. Baseline clinical and CMR data were collected and patient follow-up was performed using telephone contact and medical record. The primary composite endpoint was sudden cardiac death (SCD) or and equivalent event. The secondary composite endpoint was all-cause death and heart transplant. Patients were divided into SCD and non-SCD groups. Cox regression was used to explore risk factors of adverse events. Receiver operating characteristic (ROC) curve analysis was used to assess the performance and the optimal cut-off of late gadolinium enhancement percentage (LGE%) for the prediction of endpoints. Kaplan-Meier and log-rank tests were used to compare survival differences between groups. Results: A total of 442 patients were enrolled. Mean age was (48.5±12.4) years and 143(32.4%) were female. At (7.6±2.5) years of follow-up, 30 (6.8%) patients met the primary endpoint including 23 SCD and 7 SCD equivalent events, and 36 (8.1%) patients met the secondary endpoint including 33 all-cause death and 3 heart transplant. In multivariate Cox regression, syncope(HR=4.531, 95%CI 2.033-10.099, P<0.001), LGE% (HR=1.075, 95%CI 1.032-1.120, P=0.001) and left ventricular ejection fraction (LVEF) (HR=0.956, 95%CI 0.923-0.991, P=0.013) were independent risk factors for primary endpoint; Age (HR=1.032, 95%CI 1.001-1.064, P=0.046), atrial fibrillation (HR=2.977, 95%CI 1.446-6.131, P=0.003),LGE% (HR=1.075, 95%CI 1.035-1.116, P<0.001) and LVEF (HR=0.968, 95%CI 0.937-1.000, P=0.047) were independent risk factors for secondary endpoint. ROC curve showed the optimal LGE% cut-offs were 5.1% and 5.8% for the prediction of primary and secondary endpoint, respectively. Patients were further divided into LGE%=0, 0<LGE%<5%, 5%≤LGE%<15% and LGE%≥15% groups. There were significant survival differences between these 4 groups whether for primary endpoint or secondary endpoint (all P<0.001) and the accumulated incidence of primary endpoint was 1.2% (2/161), 2.2% (2/89), 10.5% (16/152) and 25.0% (10/40), respectively. Conclusion: LGE is an independent risk factor for SCD events as well as all-cause death and heart transplant. LGE is of important value in the risk stratification in patients with HCM.


Assuntos
Humanos , Feminino , Adulto , Pessoa de Meia-Idade , Masculino , Meios de Contraste , Estudos Retrospectivos , Volume Sistólico , Gadolínio , Função Ventricular Esquerda , Imageamento por Ressonância Magnética , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Morte Súbita Cardíaca , Medição de Risco
14.
Chinese Journal of Cardiology ; (12): 513-520, 2023.
Artigo em Chinês | WPRIM | ID: wpr-984683

RESUMO

Objective: To compare the prognosis of mildly or severely symptomatic patients with obstructive hypertrophic cardiomyopathy (OHCM) who underwent alcohol septal ablation (ASA). Methods: This retrospective study cohort consisted of patients with OHCM who received ASA treatment in Beijing Anzhen Hospital, Capital Medical University from March 2001 to August 2021. These patients were divided into mildly and severely symptomatic groups according to the severity of clinical symptoms. Long-term follow-up was conducted, and the following data were collected: duration of follow-up, postoperatire treatment, New York Heart Association (NYHA) classification, arrhythmia events and pacemaker implantation, echocardiographic parameters, and cause of death. Overall survival and survival free from OHCM-related death were observed, and the improvement of clinical symptoms and resting left ventricular outflow tract gradient (LVOTG) and the incidence of new-onset atrial fibrillation were evaluated. The Kaplan-Meier method and log-rank test were used to determine and compare the cumulative survival rates of the different groups. Cox regression analysis models were used to determine predictors of clinical events. Results: A total of 189 OHCM patients were included in this study, including 68 in the mildly symptomatic group and 121 in the severely symptomatic group. The median follow-up of the study was 6.0 (2.7, 10.6) years. There was no statistical difference in overall survival between the mildly symptomatic group (5-year and 10-year overall survival were 97.0% and 94.4%, respectively) and the severely symptomatic group (5-year and 10-year overall survival were 94.2% and 83.9%, respectively, P=0.405); there was also no statistical difference in survival free from OHCM-related death between the mildly symptomatic group (5-year and 10-year survival free from HCM-related death were 97.0% and 94.4%, respectively) and the severely symptomatic group (5-year and 10-year survival free from HCM-related death were 95.2% and 92.6%, respectively, P=0.846). In the mildly symptomatic group, NYHA classification was improved after ASA (P<0.001), among which 37 patients (54.4%) were in NYHA class Ⅰ, and the resting left ventricular outflow tract gradient (LVOTG) decreased from 67.6 (42.7, 90.1) mmHg (1 mmHg=0.133 kPa) to 24.4 (11.7, 35.6) mmHg (P<0.001). In severely symptomatic group, NYHA classification was also improved post ASA (P<0.001), among which 96 patients (79.3%) improved by at least one NYHA classification, and the resting LVOTG decreased from 69.6 (38.4, 96.1) mmHg to 19.0 (10.6, 39.8) mmHg (P<0.001). The incidence of new-onset atrial fibrillation was similar between the mildly and severely symptomatic groups (10.2% vs. 13.3%, P=0.565). Cox multivariate regression analysis showed that age was an independent predictor of all-cause mortality in OHCM patients post ASA (HR=1.068, 95%CI 1.002-1.139, P=0.042). Conclusions: Among patients with OHCM treated with ASA, overall survival and survival free from HCM-related death were similar between mildly symptomatic group and severely symptomatic group. ASA therapy can effectively relieve resting LVOTG and improve clinical symptoms in mildly or severely symptomatic patients with OHCM. Age was an independent predictor of all-cause mortality in OHCM patients post ASA.


Assuntos
Humanos , Estudos Retrospectivos , Fibrilação Atrial , Septos Cardíacos/cirurgia , Resultado do Tratamento , Cardiomiopatia Hipertrófica/cirurgia
15.
Chinese Journal of Cardiology ; (12): 497-503, 2023.
Artigo em Chinês | WPRIM | ID: wpr-984681

RESUMO

Objective: To observe the association between clinical phenotypes of hypertrophic cardiomyopathy (HCM) patients and a rare calcium channel and regulatory gene variation (Ca2+ gene variation) and to compare clinical phenotypes of HCM patients with Ca2+ gene variation, a single sarcomere gene variation and without gene variation and to explore the influence of rare Ca2+ gene variation on the clinical phenotypes of HCM. Methods: Eight hundred forty-two non-related adult HCM patients diagnosed for the first time in Xijing Hospital from 2013 to 2019 were enrolled in this study. All patients underwent exon analyses of 96 hereditary cardiac disease-related genes. Patients with diabetes mellitus, coronary artery disease, post alcohol septal ablation or septal myectomy, and patients who carried sarcomere gene variation of uncertain significance or carried>1 sarcomere gene variation or carried>1 Ca2+ gene variation, with HCM pseudophenotype or carrier of ion channel gene variations other than Ca2+ based on the genetic test results were excluded. Patients were divided into gene negative group (no sarcomere or Ca2+ gene variants), sarcomere gene variation group (only 1 sarcomere gene variant) and Ca2+ gene variant group (only 1 Ca2+ gene variant). Baseline data, echocardiography and electrocardiogram data were collected for analysis. Results: A total of 346 patients were enrolled, including 170 patients without gene variation (gene negative group), 154 patients with a single sarcomere gene variation (sarcomere gene variation group) and 22 patients with a single rare Ca2+ gene variation (Ca2+ gene variation group). Compared with gene negative group, patients in Ca2+ gene variation group had higher blood pressure and higher percentage of family history of HCM and sudden cardiac death (P<0.05); echocardiographic results showed that patients in Ca2+ gene variation group had thicker ventricular septum ((23.5±5.8) mm vs. (22.3±5.7) mm, P<0.05); electrocardiographic results showed that patients in Ca2+ gene variation group had prolonged QT interval ((416.6±23.1) ms vs. (400.6±47.2) ms, P<0.05) and higher RV5+SV1 ((4.51±2.26) mv vs. (3.50±1.65) mv, P<0.05). Compared with sarcomere gene variation group, patients in Ca2+ gene variation group had later onset age and higher blood pressure (P<0.05); echocardiographic results showed that there was no significant difference in ventricular septal thickness between two groups; patients in Ca2+ gene variation group had lower percentage of left ventricular outflow tract pressure gradient>30 mmHg (1 mmHg=0.133 kPa, 22.8% vs. 48.1%, P<0.05) and the lower early diastolic peak velocity of the mitral valve inflow/early diastolic peak velocity of the mitral valve annulus (E/e') ratio ((13.0±2.5) vs. (15.9±4.2), P<0.05); patients in Ca2+ gene variation group had prolonged QT interval ((416.6±23.1) ms vs. (399.0±43.0) ms, P<0.05) and lower percentage of ST segment depression (9.1% vs. 40.3%, P<0.05). Conclusion: Compared with gene negative group, the clinical phenotype of HCM is more severe in patients with rare Ca2+ gene variation; compared with patients with sarcomere gene variation, the clinical phenotype of HCM is milder in patients with rare Ca2+ gene variation.


Assuntos
Humanos , Adulto , Procedimentos Cirúrgicos Cardíacos/métodos , Cardiomiopatia Hipertrófica/genética , Ecocardiografia , Eletrocardiografia , Fenótipo , Sarcômeros/genética
16.
Chinese Journal of Contemporary Pediatrics ; (12): 1156-1160, 2023.
Artigo em Chinês | WPRIM | ID: wpr-1009863

RESUMO

OBJECTIVES@#To investigate the clinical phenotype and genotype characteristics of children withcardiomyopathy (CM) associated with MYH7 gene mutation.@*METHODS@#A retrospective analysis was conducted on the medical data of five children with CM caused by MYH7 gene mutation who were diagnosed and treated in the Department of Cardiology, Hebei Children's Hospital.@*RESULTS@#Among the five children with CM, there were three girls and two boys, all of whom carried MYH7 gene mutation. Seven mutation sites were identified, among which five were not reported before. Among the five children, there were three children with hypertrophic cardiomyopathy, one child with dilated cardiomyopathy, and one child with noncompaction cardiomyopathy. The age ranged from 6 to 156 months at the initial diagnosis. At the initial diagnosis, two children had the manifestations of heart failure such as cough, shortness of breath, poor feeding, and cyanosis of lips, as well as delayed development; one child had palpitation, blackness, and syncope; one child had fever, runny nose, and abnormal liver function; all five children had a reduction in activity endurance. All five children received pharmacotherapy for improving cardiac function and survived after follow-up for 7-24 months.@*CONCLUSIONS@#The age of onset varies in children with CM caused by MYH7 gene mutation, and most children lack specific clinical manifestations at the initial diagnosis and may have the phenotype of hypertrophic cardiomyopathy, dilated cardiomyopathy or noncompaction cardiomyopathy. The children receiving early genetic diagnosis and pharmacological intervention result in a favorable short-term prognosis.


Assuntos
Masculino , Feminino , Criança , Humanos , Estudos Retrospectivos , Cardiomiopatia Dilatada/genética , Linhagem , Fenótipo , Genótipo , Mutação , Cardiomiopatia Hipertrófica/diagnóstico , Cadeias Pesadas de Miosina/genética , Miosinas Cardíacas/genética
17.
Chinese Journal of Medical Genetics ; (6): 1246-1251, 2023.
Artigo em Chinês | WPRIM | ID: wpr-1009283

RESUMO

OBJECTIVE@#To explore the genetic basis for a Chinese pedigree affected with rare type heart disease.@*METHODS@#A pedigree identified at Shenzhen Maternity and Child Health Care Hospital Affiliated to Southern Medical University on July 9, 2021 was selected as the study subject. Clinical data were collected. Trio-whole exome sequencing (WES) was carried out for the proband and his parents. Candidate variants were validated by Sanger sequencing of his family members and bioinformatic analysis.@*RESULTS@#The proband, a 5-month-old male, was found to have Barth syndrome (dilated myocardiopathy and left ventricular non-compaction). Trio-WES revealed that he has harbored a hemizygous c.542G>A (p.G181A) variant of the TAZ gene, which was inherited from his mother. In addition, his mother, aunt and maternal grandmother were also found to harbor a c.557G>A (p.R186Q) variant of the TNNI3 gene. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the c.542G>A (p.G181A) variant of the TAZ gene was classified as likely pathogenic (PS2_Strong+PM2_Supporting+PP3), whilst the c.557G>A (p.R186Q) variant of the TNNI3 gene was classified as pathogenic (PP1_Strong+PS4_Strong+PP3+PP4+PM2_Supporting).@*CONCLUSION@#The c.542G>A (p.G181A) variant of the TAZ gene probably underlay the Barth syndrome in the proband, and the c.557G>A (p.R186Q) variant of the TNNI3 gene may be responsible for the hypertrophic cardiomyopathy in his mother, aunt and maternal grandmother. Above finding has expanded the mutational spectrum of the TAZ gene and facilitated the diagnosis of this pedigree.


Assuntos
Feminino , Humanos , Lactente , Masculino , Gravidez , Síndrome de Barth , Cardiomiopatia Hipertrófica , População do Leste Asiático , Cardiopatias , Linhagem
18.
Chinese Journal of Medical Genetics ; (6): 1211-1216, 2023.
Artigo em Chinês | WPRIM | ID: wpr-1009277

RESUMO

OBJECTIVE@#To explore the clinical and genetic characteristics of eight children with Primary hypertrophic cardiomyopathy (HCM).@*METHODS@#Eight children with HCM admitted to the Department of Cardiology of Henan Children's Hospital from January 2018 to December 2021 were selected as the study subjects. Clinical data of the children were collected. Whole exome sequencing was carried out on two children, and trio whole exome sequencing was carried out on the remainder 6 children. Sanger sequencing was used to verify the candidate variants in the children and their parents, and the pathogenicity of the variants was evaluated based on the guidelines from the American College of Medical Genetics and Genomics (ACMG).@*RESULTS@#The patients had included 5 males and 3 females, with their ages ranging from 5 to 13 years old. The average age of diagnosis was (7.87 ± 4.8) years old, and the cardiac phenotype showed non-obstructive HCM in all of the patients. WES has identified variants of the MYH7 gene in 4 children, including c.2155C>T (p.Arg719Trp), c.1208G>A (p.Arg403Gln), c.1358G>A (p.Arg453His), and c.1498G>A (p.Glu500Lys). Based on the guidelines from the ACMG, the first 3 variants were classified as pathogenic, while c.1498G>A (p.Glu500Lys) was classified as likely pathogenic (PM1+PM2_Supporting+PM6+PP3), which was also unreported previously. The remaining four children had all harbored maternal variants, including MYL2: c.173G>A (p.Arg58Gln; classified as pathogenic), TPM1: c.574G>A (p.Glu192Lys) and ACTC1: c.301G>A (p.Glu101Lys)(both were classified as likely pathogenic), and MYBPC3: c.146T>G (p.Ile49Ser; classified as variant of uncertain significance). Seven children were treated with 0.5 ~ 3 mg/(kg·d) propranolol, and their symptoms had improved significantly. They were followed up until September 30, 2022 without further cardiac event.@*CONCLUSION@#Genetic testing can clarify the molecular basis for unexplained cardiomyopathy and provide a basis for clinical diagnosis and genetic counseling. Discovery of the c.1498G>A (p.Glu500Lys) variant has also expanded the spectrum of MYH7 gene mutations underlying HCM.


Assuntos
Feminino , Masculino , Humanos , Criança , Pré-Escolar , Adolescente , Proteínas do Citoesqueleto , Família , Aconselhamento Genético , Testes Genéticos , Cardiomiopatia Hipertrófica/genética
19.
Arq. bras. cardiol ; 118(5): 861-872, maio 2022. graf, ilus, tab
Artigo em Inglês, Português | LILACS, CONASS, SES-SP, SESSP-IDPCPROD, SES-SP | ID: biblio-1374358

RESUMO

BACKGROUND: Hypertrophic cardiomyopathy (HCM) can cause obstruction in the left ventricular outflow tract (LVOT), and be responsible for the onset of limiting symptoms, such as tiredness. When such symptoms are refractory to pharmacological treatment, interventionist alternative therapies can be useful, such as septal ablation through the infusion of alcohol in the coronary artery or through myectomy. Recently, the use of a radiofrequency (RF) catheter for endocardial septal ablation guided by electroanatomic mapping has proven to be efficient, despite the high incidence of complete atrioventricular block. An alternative would be the application of RF at the beginning point of the septal gradient guided by the transesophageal echocardiography (TEE). The echocardiography is an imaging method with high accuracy to determine septal anatomy. OBJECTIVE: To assess the long term effect of septal ablation for the relief of ventricular-arterial gradient, using TEE to help place the catheter in the area of larger septal obstruction. Besides, to assess the effects of ablation on the functional class and echocardiographic parameters. METHODS: Twelve asymptomatic patients, with LVOT obstruction, refractory to pharmacological therapy, underwent endocardial septal ablation with 8mm-tip catheters, whose placement was oriented in the region of larger obstruction, assisted by the TEE. Temperature-controlled and staggered RF applications were performed. After each application, the gradient was reassessed and a new application was performed according to the clinical criterion. The effects of RF applications were assessed both for the gradient at rest and for that provoked by the Valsalva maneuver, and considering the gradient. The differences were significant when p-value was lower than or equal to 0.05. RESULTS: It was possible to observe that the mean reduction of the maximum gradients was from 96.8±34.7 mmHg to 62.7±25.4 mmHg three months after the procedure (p=0.0036). After one year, the mean of maximum gradient was 36.1±23.8 mmHg (p=0.0001). The procedure was well tolerated, without records of complete atrioventricular block nor severe complications. CONCLUSION: The TEE-guided septal ablation was efficient and safe, and the results were maintained during the clinical follow-up period. It is a reasonable option for the interventionist treatment of LVOT obstruction in HCM.


FUNDAMENTOS: A cardiomiopatia hipertrófica (CMH) pode causar obstrução da via de saída do ventrículo esquerdo (VSVE) e ser responsável pelo surgimento de sintomas limitantes, como cansaço físico. Quando tais sintomas são refratários ao tratamento farmacológico, os tratamentos alternativos intervencionistas podem ser úteis, como a ablação septal por meio da infusão de álcool na artéria coronária ou por meio da miectomia cirúrgica. Recentemente, o uso de cateter de radiofrequência (RF) para ablação do septo endocárdico guiado por mapeamento eletroanatômico mostrou-se eficaz apesar da elevada incidência de bloqueio atrioventricular total. Uma alternativa seria a aplicação de radiofrequência no ponto de início do gradiente septal guiada pelo ecocardiograma transesofágico (ETE). O ecocardiograma é um método de imagem com elevada acurácia para determinação da anatomia septal. OBJETIVO: Avaliar o efeito em longo prazo da ablação septal para alívio do gradiente ventrículo-arterial, utilizando o ETE para auxiliar no posicionamento do cateter na área de maior obstrução septal. Avaliar também os efeitos da ablação na classe funcional e parâmetros ecocardiográficos. MÉTODOS: Doze pacientes sintomáticos com obstrução da VSVE, refratários à terapia farmacológica, foram submetidos à ablação endocárdica septal com cateteres com ponta de 8 mm, cujo posicionamento foi orientado na região de maior obstrução com auxílio do ETE. Foram realizadas aplicações de radiofrequência (RF) termocontrolada e escalonadas sobre a área alvo. Após cada aplicação, o gradiente era reavaliado e nova aplicação era realizada de acordo com critério clínico. Foram avaliados os efeitos das aplicações de RF tanto para o gradiente em repouso como para o provocado por meio da manobra de Valsalva, e considerado o gradiente. As diferenças foram significativas quando o valor de p foi menor ou igual a 0,05. RESULTADOS: Observou-se que a redução da média dos gradientes máximos obtidos foi de 96,8±34,7 mmHg para 62,7±25,4 mmHg ao final de três meses do procedimento (p=0,0036). Após um ano, a média dos gradientes máximos obtidos foi de 36,1±23,8 mmHg (p=0,0001). O procedimento foi bem tolerado e não houve registro de bloqueio atrioventricular total e nem complicações graves. CONCLUSÃO: A ablação septal guiada pelo ETE foi eficaz e segura, com resultados mantidos durante o período de seguimento clínico. É uma opção razoável para o tratamento intervencionista da obstrução da VSVE em CMH.


Assuntos
Cardiomiopatia Hipertrófica
20.
Arq. bras. cardiol ; 118(1): 77-87, jan. 2022. tab, graf
Artigo em Inglês, Português | LILACS | ID: biblio-1360119

RESUMO

Resumo Fundamento A cardiomiopatia hipertrófica (CMH) e a hipertrofia ventricular esquerda (HVE) secundária à hipertensão arterial sistêmica (HAS) podem estar associadas a anormalidades funcionais do átrio esquerdo (AE). Objetivos Caracterizar a mecânica do AE na CMH e na HAS e avaliar qualquer correlação com a extensão da fibrose ventricular esquerda medida por ressonância magnética cardíaca (RMC) em pacientes com CMH. Métodos A função longitudinal do AE derivada do ecocardiograma bidimensional com speckle tracking foi adquirida a partir de cortes apicais de 60 pacientes com CMH e 34 indivíduos controles, pareados por idade. Pacientes com CMH também foram submetidos à RMC, com medida da extensão do realce tardio por gadolínio. A associação com parâmetros de strain do AE foi analisada. Valores p < 0,05 foram definidos como estatisticamente significativos. Resultados A média da fração de ejeção do ventrículo esquerdo não foi diferente entre os grupos. A razão E/e' estava comprometida no grupo CMH e preservada no grupo controle. A mecânica do AE estava significativamente reduzida na CMH em comparação aos pacientes com HAS. O strain rate do AE nas fases de reservatório (SRrAE) e na fase contrátil (SRctAE) foram os melhores parâmetros de discriminação de CMH com uma área sob a curva (AUC) de 0,8, seguido do strain do AE na fase de reservatório (SrAE) (AUC 0,76). O SRrAE e o SRctAE apresentaram elevada especificidade (89% e 91%, respectivamente), e o SrAE apresentou sensibilidade de 80%. Um decréscimo de 2,79% no strain rate do AE na fase de condução (SRcdAE) foi preditor de um aumento de 1 cm na extensão do RT pelo gadolínio (r2=0,42, β 2,79, p=0,027). Conclusões O SRrAE e o SRctAE foram os melhores fatores de discriminação de HVE secundária à CMH. O SRcdAE foi preditor do grau de fibrose ventricular esquerda avaliada por RMC. Esses achados sugerem que a mecânica do AE pode ser um potencial preditor de gravidade de doença na CMH.


Abstract Background Hypertrophic cardiomyopathy (HCM) and left ventricular hypertrophy (LVH) secondary to systemic hypertension (HTN) may be associated with left atrial (LA) functional abnormalities. Objectives We aimed to characterize LA mechanics in HCM and HTN and determine any correlation with the extent of left ventricular (LV) fibrosis measured by cardiac magnetic resonance (CMR) in HCM patients. Methods Two-dimensional speckle tracking-derived longitudinal LA function was acquired from apical views in 60 HCM patients, 60 HTN patients, and 34 age-matched controls. HCM patients also underwent CMR, with measurement of late gadolinium enhancement (LGE) extension. Association with LA strain parameters was analyzed. Statistical significance was set at p<0.05. Results Mean LV ejection fraction was not different between the groups. The E/e' ratio was impaired in the HCM group and preserved in the control group. LA mechanics was significantly reduced in HCM, compared to the HTN group. LA strain rate in reservoir (LASRr) and in contractile (LASRct) phases were the best discriminators of HCM, with an area under the curve (AUC) of 0.8, followed by LA strain in reservoir phase (LASr) (AUC 0.76). LASRr and LASR-ct had high specificity (89% and 91%, respectively) and LASr had sensitivity of 80%. A decrease in 2.79% of LA strain rate in conduit phase (LASRcd) predicted an increase of 1cm in LGE extension (r2=0.42, β 2.79, p=0.027). Conclusions LASRr and LASRct were the best discriminators for LVH secondary to HCM. LASRcd predicted the degree of LV fibrosis assessed by CMR. These findings suggest that LA mechanics is a potential predictor of disease severity in HCM.


Assuntos
Humanos , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Meios de Contraste , Fibrose , Hipertrofia Ventricular Esquerda/complicações , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Gadolínio
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA