Effect analysis of myectomy guided by personalized three-dimensional reconstruction and printing in the treatment of obstructive hypertrophic cardiomyopathy / 中华外科杂志

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.

Myocardial biopsy of Liwen procedure: representability and etiological diagnostic value of cardiac samples obtained by a novel technique in patients with hypertrophic cardiomyopathy / 中华心血管病杂志

Objective: To investigate the representability and etiological diagnostic value of myocardium samples obtained from patients with hypertrophic cardiomyopathy (HCM) by transthoracic echocardiography-guided percutaneous intramyocardial septal biopsy (myocardial biopsy of Liwen procedure). Methods: This study was a retrospective case-series analysis. Patients with HCM, who underwent myocardial biopsy of Liwen procedure and radiofrequency ablation in Xijing Hospital, Air Force Military Medical University from July to December 2019, were included. Demographic data (age, sex), echocardiographic data and complications were collected through electronic medical record system. The histological and echocardiographic features, pathological characteristics of the biopsied myocardium of the patients were analyzed. Results: A total of 21 patients (aged (51.2±14.5) years and 13 males (61.9%)) were enrolled. The thickness of ventricular septum was (23.3±4.5)mm and the left ventricular outflow tract gradient was (78.8±42.6)mmHg (1 mmHg=0.133 kPa). Eight patients (38.1%) were complicated with hypertension, 1 patient (4.8%) had diabetes, and 2 patients (9.5%) had atrial fibrillation. Hematoxylin-eosin staining of myocardial samples of HCM patients before radiofrequency ablation evidenced myocytes hypertrophy, myocytes disarray, nuclear hyperchromatism, hypertrophy, atypia, coronary microvessel abnormalities, adipocyte infiltration, inflammatory cell infiltration, cytoplasmic vacuoles, lipofuscin deposition. Interstitial fibrosis and replacement fibrosis were detected in Masson stained biopsy samples. Hematoxylin-eosin staining of myocardial samples of HCM patients after radiofrequency ablation showed significantly reduced myocytes, cracked nuclear in myocytes, coagulative necrosis, border disappearance and nuclear fragmentation. Quantitative analysis of myocardial specimens of HCM patients before radiofrequency ablation showed that there were 9 cases (42.9%) with mild myocardial hypertrophy and 12 cases (57.1%) with severe myocardial hypertrophy. Mild, moderate and severe fibrosis were 5 (23.8%), 9 (42.9%) and 7 (33.3%), respectively. Six cases (28.6%) had myocytes disarray. There were 11 cases (52.4%) of coronary microvessel abnormalities, 4 cases (19.0%) of adipocyte infiltration, 2 cases (9.5%) of inflammatory cell infiltration,6 cases (28.5%) of cytoplasmic vacuole, 16 cases (76.2%) of lipofuscin deposition. The diameter of cardiac myocytes was (25.2±2.8)μm, and the percentage of collagen fiber area was 5.2%(3.0%, 14.6%). One patient had severe replacement fibrosis in the myocardium, with a fibrotic area of 67.0%. The rest of the patients had interstitial fibrosis. The myocardial specimens of 13 patients were examined by transmission electron microscopy. All showed increased myofibrils, and 9 cases had disorder of myofibrils. All patients had irregular shape of myocardial nucleus, partial depression, mild mitochondrial swelling, fracture and reduction of mitochondrial crest, and local aggregation of myofibrillary interfascicles. One patient had hypertrophy of cardiomyocytes, but the arrangement of muscle fibers was roughly normal. There were vacuoles in the cytoplasm, and Periodic acid-Schiff staining was positive. Transmission electron microscopy showed large range of glycogen deposition in the cytoplasm, with occasional double membrane surround, which was highly indicative of glycogen storage disease. No deposition of glycolipid substance in lysozyme was observed under transmission electron microscope in all myocardial specimens, which could basically eliminate Fabry disease. No apple green substance was found under polarized light after Congo red staining, which could basically exclude cardiac amyloidosis. Conclusion: Myocardium biopsied samples obtained by Liwen procedure of HCM patients are representative and helpful for the etiological diagnosis of HCM.

Automatic detection model of hypertrophic cardiomyopathy based on deep convolutional neural network / 生物医学工程学杂志

The diagnosis of hypertrophic cardiomyopathy (HCM) is of great significance for the early risk classification of sudden cardiac death and the screening of family genetic diseases. This research proposed a HCM automatic detection method based on convolution neural network (CNN) model, using single-lead electrocardiogram (ECG) signal as the research object. Firstly, the R-wave peak locations of single-lead ECG signal were determined, followed by the ECG signal segmentation and resample in units of heart beats, then a CNN model was built to automatically extract the deep features in the ECG signal and perform automatic classification and HCM detection. The experimental data is derived from 108 ECG records extracted from three public databases provided by PhysioNet, the database established in this research consists of 14,459 heartbeats, and each heartbeat contains 128 sampling points. The results revealed that the optimized CNN model could effectively detect HCM, the accuracy, sensitivity and specificity were 95.98%, 98.03% and 95.79% respectively. In this research, the deep learning method was introduced for the analysis of single-lead ECG of HCM patients, which could not only overcome the technical limitations of conventional detection methods based on multi-lead ECG, but also has important application value for assisting doctor in fast and convenient large-scale HCM preliminary screening.

Diferencias electrocardiográficas en miocardiopatía hipertrófica apical e infarto apical sin elevación del segmento ST / Electrocardiographic differences between apical hypertrophic cardiomyopathy and apical non-ST segment myocardial infarction

La miocardiopatía hipertrófica apical (MCHA) es una variante fenotípica dentro de las miocardiopatías hipertróficas que presenta alteraciones de la repolarización ventricular. Estos cambios electrocardiográficos pueden simular en muchos casos un infarto anterior, lo que dispara una serie de estudios y tratamientos que pueden ser innecesarios. El objetivo del trabajo fue describir y comparar las diferencias electrocardiográficas en una serie de pacientes con MCHA e infarto sin elevación del ST apicales (IAMSESTa) que presenten cambios tipo T en el electrocardiograma (ECG). Se realizó un estudio observacional y retrospectivo, incluyendo pacientes con diagnóstico de MCHA (n = 19) e IAMSESTa (n = 19) con ondas T negativas en ECG de ingreso en derivaciones V1-V6. Se excluyeron aquellos con MCHA y enfermedad coronaria asociada. Se analizaron las características clínicas y electrocardiográficas entre ambos grupos. Los pacientes con MCHA presentaron mayor voltaje de ondas T (7 mV vs. 5 mV; p = 0.001) y sumatoria de voltaje de las mismas (29 mV vs. 17 mV; p = 0.003), mayor voltaje de ondas R (25 mV vs. 10 mV; p = 0.0001), con una sumatoria de máximo voltaje de R y T (R+T) significativamente mayor (33 vs. 14; p = 0.00001). Presentaron además mayor asimetría de las ondas T negativas, objetivado mediante una relación TiTp/TpTf > 1. Con un valor de corte de 26.5 mV para la variable R+T, se obtuvo un 68% de sensibilidad y 100% de especificidad para diagnosticar MCHA. El presente trabajo demuestra la existencia de diferencias en el patrón del ECG en MCHA e IAMSESTa.

Apocal hypertrophic cardiomyopathy (AHCM) is a phenotypic variant within hypertrophic cardiomyopathies, in which ventricular repolarization alterations are present. These electrocardiographic disturbances can mimic an anterior infarction which triggers a series of studies and treatments that may be unnecessary. The aim of this study was to describe and compare electrocardiographic differences in a series of patients with AHCM and apical non-ST segment elevation myocardial infarction in patients (NSTEMI) with T-wave changes. We conducted an observational and retrospective study, including patients with diagnosed AHCM (N = 19) and apical NSTEMI (N = 19) with negative T waves in V1 and V6 lead of the EKG. Those with AHCM presented higher T-wave voltage (7 mV vs. 5 mV, p = 0.001) and peak voltage (29 mV vs. 17 mV, p = 0.003), higher R-waves (25 mV vs. 10 mV, p = 0.0001), and a maximum voltage of R and T sum (R + T) significantly higher (33 vs. 14, p = 0.00001). They also showed a greater T-wave asymmetry, with a TiTp / TpTf ratio > 1. At a cut-off value of 26.5 mV for the R + T variable, 68% sensitivity and 100% specificity were obtained to diagnose AHCM. This study shows the existence of major differences in electrocardiographic presentation of AHCM and apical NSTEMI.

Alteraciones electrocardiográficas en una cohorte de pacientes con miocardiopatía hipertrófica septal asimétrica / Electrocardiographic changes in patients with septal asymmetric hypertrophic cardiomyopathy

Introducción: ciertos hallazgos del electrocardiograma (ECG) de superficie sugieren el diagnóstico de miocardiopatía hipertrófica (MCH) y se correlacionan directamente con su expresión fenotípica. Sin embargo, las modificaciones ECG han sido escasamente descritas en series locales y regionales. El objetivo de este estudio fue caracterizar los cambios ECG en la MCH, correlacionándolos con variables clínicas y estructurales. Método: se interpretaron los ECG en una cohorte de 26 pacientes con MCH septal asimétrica referidos a la policlínica especializada del Centro Cardiovascular Universitario del Uruguay. Todos los pacientes fueron estudiados con ecocardiograma Doppler y 13 de ellos, además, con resonancia nuclear magnética cardíaca (RNMC) para evaluar la presencia, extensión y distribución del realce tardío de gadolinio (RTG) como marcador de fibrosis intramiocárdica. La correlación de los hallazgos ECG con los síntomas, severidad de la hipertrofia y presencia y extensión de la fibrosis intramiocárdica se analizó mediante la prueba exacta de Fisher o el test no paramétrico de Mann-Whitney. Resultados: el hallazgo de un ECG normal fue muy infrecuente en nuestra serie (8,0%). Las alteraciones ECG más comunes se observaron en la repolarización ventricular (76,9%) bajo forma de sobrecarga sistólica (42,3%) e inversión de la onda T (30,7%). Los signos de sobrecarga auricular izquierda (53,8%), hipertrofia ventricular izquierda (61,5%), fragmentación del QRS (46,2%) y ondas Q anormales (30,7%) fueron menos frecuentes. Los criterios de hipertrofia no se correlacionaron con los síntomas, el espesor parietal, la obstrucción al tracto de salida del ventrículo izquierdo ni la presencia de RTG en la RNMC. No encontramos asociación significativa entre la fragmentación del QRS o la presencia de ondas Q y el hallazgo de RTG en la RNMC. Aunque los pacientes con hipertrofia septal más severa presentaban extensas áreas de fibrosis, esta relación no alcanzó significación estadística. Conclusión: el trazado ECG es anormal en la gran mayoría (92,0%) de los pacientes con MCH septal asimétrica, y se caracteriza por alteraciones diversas, lo que confirma su utilidad como herramienta de tamizaje. Sin embargo, en nuestra serie los hallazgos ECG no se correlacionan con los síntomas, el espesor parietal ni la presencia de fibrosis intramiocárdica. La contribución diagnóstica de signos ECG combinados y su potencial valor pronóstico en diferentes variantes fenotípicas de MCH merecen ser evaluados en series locales más amplias.

Introduction: in many cases, surface ECG suggests the diagnosis of hypertrophic cardiomyopathy (HCM) and is directly related to phenotypic expression. However, ECG changes have been not fully described in regional and local case series. Our study was aimed to characterize the ECG abnormalities in patients with HCM, correlating these findings with clinical and structural variables. Method: ECG recordings were interpreted in a cohort of 26 subjects with asymmetric septal HCM. All patients were studied by echocardiogram, adding MRI in 13 cases to evaluate the presence, extension and distribution of late gadolinium enhancement (LGE) as a marker of myocardial fibrosis. The correlation of ECG findings with symptoms, severity of hypertrophy and presence and extension of myocardial fibrosis was analyzed using Fisher's exact test or non-parametric Mann-Whitney test. Results: the presence of a normal ECG was very infrequent in our sample (8,0%). The most common ECG abnormalities were observed in ventricular repolarization (76,9%) as systolic overload (42,3%) and T wave inversion (30,7%). ECG signs suggesting left atrium dilation (53,8%), left ventricle hypertrophy (LVH) criteria (61,5%), QRS fragmentation (46,2%) and abnormal Q waves (30,7%) were less frequently found. LVH was not correlated with symptoms, wall thickness, left ventricular outflow tract obstruction or presence of LGE. We found no significant relationship between LGE and fragmented QRS or abnormal Q waves. While patients with more severe septal hypertrophy exhibited large areas of LGE, this relationship did not reach statistical significance. Conclusion: in our cohort, the ECG is abnormal in 92,0% of patients with asymmetric septal HCM and is characterized by various alterations, confirming its usefulness as a screening tool. However, the findings in the ECG did not correlate closely with symptoms, amount of hypertrophy or presence of myocardial fibrosis. Larger local series must evaluate diagnostic contribution of combined ECG findings and its potential prognostic value in different phenotypic variants of HCM.

Cardiomiopatia hipertrófica dilatada com síndrome de Wolff- Parkinson-White / Dilated hypertrophic cardiomyopathy with Wolff-Parkinson-White syndrome

A cardiomiopatia hipertrófica é uma doença genética com apresentação, prognóstico e estratégias de tratamento variáveis. A visibilidade ligada à cardiomiopatia hipertrófica refere-se em grande parte a seu reconhecimento como causa comum de taquiarritmias e morte súbita cardíaca. O cardiodesfibrilador implantável é aceito como tratamento definitivo para prevenção de morte súbita, especialmente em pacientes de alto risco. Apesar da baixa prevalência, a via acessória é encontrada em 5% dos pacientes com cardiomiopatia hipertrófica e a ablação é o método de escolha, em decorrência do risco de taquiarritmias atriais. Relatamos o caso de um paciente com cardiomiopatia hipertrófica, em fase dilatada, com via acessória.

Hypertrophic cardiomyopathy is a genetic disease with variable presentation, prognosis and treatment strategies. The visibility linked to hypertrophic cardiomyopathy to a large extent refers to its recognition as a common cause of tachyarrhythmias and sudden cardiac death. Implantable cardiac defibrillator is accepted as definitive treatment for the prevention of sudden death, especially in high-risk patients. Despite the low prevalence, the accessory pathway is found in 5% of patients with hypertrophic cardiomyopathy and ablation is the method of choice due to risk of atrial tachyarrhythmias. We report the case of a patient with dilated hypertrophic cardiomyopathy and an accessory pathway.

Sensibilidade e especificidade do exame eletrocardiográfico na detecção de sobrecargas atriais e/ou ventriculares em gatos da raça Persa com cardiomiopatia hipertrófica / Sensitivity and specificity of electrocardiographic examination in detecting ventricular or atrial overloads in Persian cats with hypertrophic cardiomyopathy

A cardiomiopatia hipertrófica (CMH) é a principal cardiopatia dos felinos e é caracterizada por hipertrofia miocárdica concêntrica, sem dilatação ventricular. O ecocardiograma é o melhor meio diagnóstico não invasivo para a diferenciação das cardiomiopatias e é considerado padrão ouro para a detecção de hipertrofia ventricular presente na CMH. Alterações eletrocardiográficas também são comuns em animais com CMH e o eletrocardiograma (ECG) é um teste de triagem para detecção de hipertrofia ventricular em humanos, sendo um exame rápido e facilmente disponível. Em gatos, poucos estudos foram realizados quanto à sensibilidade e especificidade do ECG na detecção de hipertrofia ventricular. Com a intenção de avaliar o uso do ECG como ferramenta de triagem para diagnóstico de CMH em felinos, gatos da raça Persa (n=82) foram avaliados por meio de exames ecocardiográfico e eletrocardiográfico. Animais com bloqueios e/ou distúrbios de condução foram excluídos da análise estatística (n=22). Posteriormente, os animais incluídos foram classificados em: normais (n=38), suspeitos (n=6) e acometidos pela CMH (n=16)...

Hypertrophic cardiomyopathy (HCM) is the most common feline heart disease and is characterized by increased cardiac mass with a hypertrophied and not dilated left ventricle. The echocardiography is the best noninvasive diagnostic tool for the differentiation of cardiomyopathies and is considered the gold standard for detection of ventricular hypertrophy present in HCM. Electrocardiographic changes are also common in animals with HCM and the electrocardiogram (ECG) is quick, easy and highly available screening test for the detection of ventricular hypertrophy in humans. In cats, few studies have been conducted regarding the sensitivity and specificity of ECG in detecting ventricular hypertrophy. With the intention of evaluating the use of ECG as a screening tool for diagnosis of HCM in cats, Persian cats (n=82) were evaluated by echocardiographic and electrocardiographic examinations. Animals with blocks and/or conduction disturbances were excluded from statistical analysis (n=22). Subsequently the animals included were classified as normal (n=38), suspicious (n=6) and affected by HCM (n=16)...

Morphological and Electrical Characteristics in Patient with Hypertrophic Cardiomyopathy: Quantitative Analysis of 864 Korean Cohort

PURPOSE: Although several studies have reported the morphological and electrical characteristics in patients with hypertrophic cardiomyopathy (HCM), comparison between asymmetric and apical HCM has not been investigated in a reasonably sized cohort. MATERIALS AND METHODS: Echocardiography and electrocardiography were quantitatively analyzed in patients with HCM in a Korean tertiary referral center. RESULTS: Of 864 patients (mean age 55.4+/-14.2 years, 68.9% men), 255 (29.5%) patients had apical HCM, 553 (64.0%) patients asymmetric HCM, and 56 (6.4%) patients mixed type HCM. In echocardiographic evaluations, about three quarters of patients (75.8%) had left atrial enlargement. Left ventricular (LV) dilatations and systolic dysfunction were observed in 6.1% and 2.4%, respectively. QRS widening, PR prolongation, and pathologic Q wave are frequent in patients with asymmetric HCM, while LV strain is frequent in patient with apical HCM. The prevalence of J-point elevations (9.4% in inferior, 2.2% in lateral leads) were substantially higher than that in general population. Giant negative T wave was observed in 15.0% of total patients (32.2% in apical, 6.2% in asymmetric, 25% in mixed type). There was no significant correlation between the thickness of the apical wall and the amplitude of T wave inversion (r=-0.005, p=0.71). CONCLUSION: In a large cohort of HCM including apical type, repolarization abnormalities, including early repolarization and QT prolongation as well as LV strain, were significantly observed. T wave inversion was not appropriate for screening of HCM and not correlated with apical wall thickness.

Lesão do isolante do cabo-eletrodo ventricular em portadora de cardiodesfibrilador / Lesions of the ventricular electrode insulating carrier cardioverter

Cabos-eletrodos de cardiodesfibriladores implantáveis sofrem mais complicações e falhas que os demarcapassos. Relata-se o caso de paciente portadora de cardiodesfibrilador dependente de estimulação cardíacaartificial, que passou a apresentar sintomas de pré-síncope. Durante a investigação, apresentava eletrocardiogramacom pausas e a telemetria em repouso era aparentemente normal. Optou-se pela abordagem cirúrgica diagnóstica,sendo observada lesão do isolante do cabo-eletrodo ventricular. Com a substituição do cabo-eletrodo ventricular,houve melhora imediata dos sintomas.

Implantable cardioverter-defibrillator leads have more complications and failures than the pacemakers.We report a case of a patient with implantable cardioverter-defibrillator dependent on cardiac pacing, who beganto show pre-syncope symptoms. During investigation, the electrocardiogram presented with pauses and telemetrywas apparently normal at rest. We chose to use a diagnostic surgical approach and observed fracture of theventricular lead isolation. There was immediate improvement of symptoms after ventricular lead replacement.

Cardiomiopatia hipertrófica apical / Apical hypertrophic cardiomyopathy

Homem de 43 anos de idade procurou o ambulatório com queixa de dor precordial em queimação, iniciada há cinco anos, aos médios esforços. Não apresentava fatores de risco para doença arterial coronariana. O eletrocardiograma apresentou ondas T negativas e com amplitude superior a 10 mm. A cintilografia miocárdica evidenciou no mapa polar o sinal “solar polar map”. A ressonância magnética cardíaca revelou importante hipertrofia do ventrículo esquerdo com predomínio do segmento apical, com maior espessura parietal de 22 mm e realce tardio mostrando exuberante captação tardia do contraste com distribuição mesocárdica difusa, confirmando a hipótese de doença de Yamaguchi.

A 43 year-old man with no risk factors for coronary artery disease visited the out-patient clinic complaining of burning chest pain that began five years ago with average effort. The electrocardiogram showed negative T waves wider than 10mm. Myocardial scintigraphy showed the ‘solar polar’ sign in the polar map. Cardiac magnetic resonance imaging disclosed significant left ventricular hypertrophy with predominance of the apical segment with greater wall thickness of 22mm and delayed enhancement, showing ample delayed contrast uptake with diffuse mesocardial distribution, confirming the hypothesis of Yamaguchi disease.

Miocárdio não compactado isolado ventricular esquerdo: relato de caso / Isolated noncompacted left ventricular myocardium: case report

O miocárdio não compactado isolado (MNCI) é uma afecçãocongênita rara, resultante da interrupção da embriogênesenormal do miocárdio, a qual leva à persistência de trabeculaçõesnumerosas e profundas do endocárdio, comunicantescom a cavidade ventricular. Inicialmente descrita como umaentidade exclusiva da população pediátrica, vários casos deapresentação tardia ao longo da vida adulta foram descritosna literatura. Tem como manifestações clínicas principais:insuficiência cardíaca, eventos arrítmicos e episódios detromboembolismo sistêmico. Relata-se o caso de um adultojovem de 27 anos, cuja suspeita clínica foi estabelecida porecocardiograma bidimensional, sendo o diagnóstico definitivode não compactação isolada do miocárdio ventricular obtidopor ressonância magnética cardíaca.

Isolated noncompacted myocardium is a rare congenital disorderresulting from disruption of normal myocardial embryogenesis,leading to the persistence of numerous deep trabeculations of theendocardium, communicating with the ventricular cavity. Initiallydescribed as a congenital pathology of the pediatric population, severalcases of belated presentation during adulthood have been describedin literature. Its main clinical manifestations are heart failure,arrhythmic events and episodes of arterial thromboembolism. Wereport the case of a young adult man of 27-year-old, whose clinicalsuspicion was established by two-dimensional echocardiography,and the definitive diagnosis of isolated noncompaction of ventricularmyocardium obtained by cardiac magnetic resonance imaging.

Ecocardiografia bidimensional convencional pode não detectar cardiomiopatia hipertrófica apical: relato de caso / Conventional bidimensional echocardiography may not detect apical hypertrophic cardiomyopathy: case report

A cardiomiopatia hipertrófica (CMH) é uma doença genética cardíaca, caracterizada por hipertrofia ventricular esquerda. É assimétrica na maioria das vezes. O método diagnóstico considerado padrão para detecção de CMH é o ecocardiograma bidimensional. Os casos de CMH que passam despercebidos por esse método são poucos, e para eles há a necessidade de um outro exame de imagem que melhor visualize o tipo morfológico da doença apresentada: a ressonância magnética cardíaca (RMC). Este relato descreve um paciente com alterações eletrocardiográficas de base e que foi diagnosticado com CMH apical unicamente pela RMC, sem apresentar anormalidade significativa à ecocardiografia convencional.

Miocardiopatia hipertrófica medioventricular associada ao aneurisma apical: estudo ecocardiográfico de dois casos / Mid-ventricular hypertrophic cardiomyopathy associated with apical aneurism: echocardiography study of two cases

A Miocardiopatia Hipertrófica (MCH) é uma doença caracterizada por ampla expressão genotípica e fenotípica, com curso clínico heterogêneo. A diversidade genética e fatores ambientais formam a base dessa heterogeneidade. É a causa mais comum de morte súbita em atletas jovens. A MCH medioventricular corresponde a 1% dos casos; pode estar associada a aneurisma apical e progressão para disfunção sistólica. Logo, o reconhecimento desse subgrupo, pelo estudo ecocardiográfico, é fundamental para melhor estratificação e acompanhamento desses pacientes. Neste relato, serão apresentados dois casos de miocardiopatia hipertrófica medioventricular, com aneurisma apical associado, seguidos de revisão da literatura.

O diagnóstico da obstrução da via de saída do ventrículo esquerdo na cardiomiopatia hipertrófica / The diagnosis of left ventricular outflow tract obstruction in hypertrophic cardiomyopathy

A cardiomiopatia hipertrófica é uma doença genética prevalente caracterizada por hipertrofia ventricular esquerda, em que obstrução dinâmica da via de saída com geração de gradiente subaórtico incide em repouso em 30% dos casos. A obstrução é atribuida complexa interação entre o folheto anterior mitral, o septo interventricular e vetores anômalos de fluxo gerados no ventrículo esquerdo aliada a modificações na geometria da via de saída. Regurgitação mitral em grau variável é detectada associada ou não a deformidades estruturais do aparelho valvar. O ecocardiograma de esforço demonstra obstrução latente facilmente induzida por exercício em 60 a 75% das formas não obstrutivas. A determinação do gradiente nessas condições impõe-se na investigação de rotina dos pacientes com obstrução leve ou ausente em repouso. A avaliação da cardiomiopatia hipertrófica incorpora métodos de imagem baseados no ultrassom, os quais, adicionados ressonância magnética, possibilitam o reconhecimento de mecanismos geradores de obstrução ventricular, de modo a favorecer o diagnóstico e o manejo das formas obstrutivas e obstrutivas latentes.

Hypertrophic cardiomyopathy is a prevalent genetic disease characterized by left ventricular hypertrophy, presenting dynamic obstruction of outflow tract with subaortic gradient happening at rest in 30% of the cases. It is attributed to the intricate interaction between the anterior mitral leaflet, the interventricular septum and altered flow vectors generated in left ventricle along with changes in outflow tract geometry. Mitral regurgitation in varying degrees is found with or without association with structural deformities of the valve apparatus. The exercise echocardiogram evidences latent obstruction easily induced by exercise in 60 to 75% of non-obstructive forms. The determination of the gradient under this condition must be considered in routine investigation of patients with mild or no obstruction at rest. The evaluation of hypertrophic cardiomyopathy incorporates methods based on the ultrasound image, which, along with MRI, allow recognizing ventricular obstruction generating mechanisms, thus facilitating the diagnosis and management of obstructive and latent obstructive forms.

O valor do Doppler tecidual no diagnóstico diferencial da hipertrofia do atleta e da cardiomiopatia hipertrófica / The value of tissue Doppler in the differential diagnosis of athlete's heart and hypertrophic cardiomyopathy

Introdução: A cardiomiopatia hipertrófica é a responsável pelo maior número de mortes súbitas ou inesperadas por problemas cardíacos, em atletas jovens, e, quando tentamos esclarecer o diagnóstico pela ecocardiografia tradicional, a simples presença de hipertrofia, muitas vezes, não permite distinguir a sua etiologia, como a hipertrofia dos atletas e a hipertrofia secundária à hipertensão arterial. O Doppler tecidual (DTI) pode auxiliar na diferenciação das referidas hipertrofias. Objetivo: Avaliar os valores do Doppler tecidual (TDI) no anel mitral, em pacientes com cardiomiopatia hipertrófica, hipertrofia hipertensiva e hipertrofia, induzida pelo exercício, em atletas saudáveis de alta performance. Métodos: Foram estudados 147 pacientes: 30 voluntários saudáveis (grupo controle); 49 atletas de alta performance, jogadores de futebol profissionais do fluminense Futebol Clube , da cidade do rio de Janeiro-RJ (grupo dos atletas), avaliados na classe 1 de weber pela ergoespirometria; 40 portadores de hipertensão arterial sistêmica moderada controlada, com hipertrofia ventricular esquerda e função ventricular preservada (grupo dos hipertensos)...