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Objective:To investigate whether the existence of thoracic artery blood supply in peripheral pulmonary lesions is the key factor affecting the accuracy of contrast-enhanced ultrasound in differentiating benign and malignant lesions.Methods:From June 2020 to December 2021, a total of 170 patients with peripheral pulmonary lesions were consecutively enrolled in Xi′an Chest Hospital, and all patients underwent conventional ultrasound and contrast-enhanced ultrasound(CEUS). Taking ΔAT(lesion-lung arrival time difference ) of 2.5 seconds as the cut-off point for differentiating benign and malignant lesions(ΔAT<2.5 s for benign, ΔAT≥2.5 s for malignant), and the final pathological results as the gold standard, these patiens were divided into correct classification group and wrong classification group, and the main influencing factors of wrong classification were analyzed.Results:Compared with the correct classification group, the proportion of thoracic artery blood supply in the wrong classification group was significantly higher ( P<0.001). After adjusting the dendritic venous reflux, thoracic artery blood supply was an independent influencing factor for CEUS misclassification ( OR=3.531, 95% CI=1.805-6.908, P<0.001). In the patients with thoracic artery blood supply, the sensitivity of the absence of dendritic venous reflux in judging malignant peripulmonary lesions was 75.0%, the specificity was 91.3%, and the area under the ROC curve (AUC) was 0.832 (95% CI=0.715-0.915, P<0.001), while the sensitivity, specificity and AUC of CEUS in judging malignant peripulmonary lesions were 68.7%, 67.4% and 0.659 (95% CI=0.528-0.775, P=0.006), and there was significant statistical difference in the AUC between them( P<0.001). In the group of patients without thoracic arterial blood supply, the sensitivity of the absence of dendritic venous reflux in judging malignant peripulmonary lesions was 82.8%, the specificity was 63.3%, and the AUC was 0.730 (95% CI=0.636-0.811, P<0.001), while the sensitivity, specificity and AUC of CEUS in judging malignant peripulmonary lesions were 62.1%, 81.0% and 0.684 (95% CI=0.587-0.770, P=0.003), and there was no significant difference in the AUC between them ( P=0.425). Conclusions:The presence of thoracic artery blood supply significantly decrease the diagnostic efficiency of CEUS in differentiating benign and malignant of peripheral pulmonary lesions. For peripheral pulmonary lesions with thoracic arterial blood supply, the diagnostic efficiency of dendritic venous reflux is better than CEUS, while for lesions without thoracic artery supply, the diagnostic efficiency of dendritic venous reflux is equivalent to CEUS.
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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.
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Humans , Male , Biopsy/adverse effects , Cardiomegaly/pathology , Cardiomyopathy, Hypertrophic/diagnosis , Eosine Yellowish-(YS) , Fibrosis , Heart Defects, Congenital , Hematoxylin , Lipofuscin , Myocardium/pathology , Retrospective StudiesABSTRACT
Objective:To quantitatively evaluate myocardial microcirculation perfusion changes after percutaneous intracardiac septal radiofrequency ablation (PIMSRA, Liwen procedure) in patients with obstructive hypertrophic cardiomyopathy (HOCM) using myocardial contrast echocardiography (MCE) and to further establish the effect of the procedure and predict the long-term prognosis of patients.Methods:A total of 45 patients with HOCM treated by the Liwen procedure in the First Affiliated Hospital of Air Force Medical University (Xijing Hospital) from July 2019 to June 2020 were included. MCE was performed before and 6 months after surgery, respectively. Time-intensity perfusion curve analysis was performed using QLab 10.8 offline software to obtain quantitative parameters of myocardial microcirculation perfusion including myocardial blood volume (A value), myocardial blood flow velocity (β value), and myocardial blood flow (A×β value), then the changes in parameters before and after the procedure were compared.Results:After the Liwen procedure, the mean septal thickness and mean left ventricular free wall thickness were significantly reduced, accompanied by a significant reduction in the left ventricular outflow tract pressure gradient (LVOT-PG) and mitral regurgitation length, and a significant improvement in mitral systolic antegrade motion (SAM) (all P<0.001). In addition, the left ventricular ejection fraction (LVEF) did not deteriorate significantly ( P=0.560) and the E/e′ ratio decreased after the procedure ( P=0.015). Besides, the A values of both the ventricular septum and the left ventricular free wall were not significantly changed compared to those before procedure ( P>0.05), whereas the β values and A×β values were obviously increased ( P<0.05). Conclusions:Myocardial blood velocity and myocardial blood flow in the septum and left ventricular free wall were significantly increased in patients with HOCM after the Liwen procedure, suggesting a significant improvement in myocardial microcirculation perfusion. MCE provides a non-invasive quantitative evaluation parameters of myocardial microcirculation perfusion for the Liwen procedure for the treatment of HOCM.
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Objective:To investigate the feasibility of ultrasound-guided percutaneous endomyocardial septal cryoablation of in vitro porcine hearts and to compare its effect with the percutaneous endomyocardial radiofrequency ablation.Methods:Experiment 1: Six in vitro porcine hearts were divided into 1 min ( n=2), 3 min ( n=2) and 5 min ( n=2) groups according to the cryoablation time, and all were subjected to ultrasound-guided percutaneous intra-myocardial septal cryoablation at 100% power respectively. After cryoablation, ultrasound images, the size of the solid dissection of the ice ball, and the size of the necrotic area after melting of the frozen ice ball were measured. Experiment 2: The in vitro porcine hearts were divided into cryoablation group ( n=3) and radiofrequency ablation group ( n=3), and ultrasound-guided percutaneous endomyocardial septal cryoablation and radiofrequency ablation were performed with 100% cryo power and 40 W radiofrequency power, and the extent of complete necrotic area and incomplete necrotic area were compared between the two ablation methods after 1 min. Results:Experiment 1: In the 1 min cryoablation time group ( n=2), the short diameter of the puck measured by ultrasound was (8.00±0.84)mm, the short diameter of the puck measured by solid was (8.38±1.19)mm, and the short diameter of the necrotic zone measured by solid was (8.35±0.83)mm; in the 3 min group ( n=2), the short diameter of the puck measured by ultrasound was (19.4±0.28)mm, and the short diameter of the puck measured by solid was (19.03±0.33)mm, solid measurement of the short diameter of the necrotic zone was (19.16±0.25)mm; in the 5 min group ( n=2), the short diameter of the puck measured under ultrasound was (26.4±2.54)mm, solid measurement of the short diameter of the puck was (26.01±0.24)mm, and solid measurement of the short diameter of the necrotic zone was (24.82±0.25)mm. Randomized blocks analysis of variance was performed on this data and the difference of block Factor b (freezing time: 1 min, 3 min, 5 min) among the three groups was statistically significant( F=505.884, P<0.001). The SNK- q test showed that all three groups differed from each other(all P<0.05). The analysis results for the treatment factors K (measurement modality-ultrasound image measurements, solid anatomical measurements of the puck, and measurements of the necrotic area after melting of the frozen puck) was not statistically significant ( F=0.470, P=0.635). Experiment 2: In the RF ablation group ( n=3), the ratio of incomplete necrotic zone to the radius of the RF ablation area was 0.64±0.01; in the cryoablation group ( n=3), the ratio of incomplete necrotic zone to the radius of the ablation area was 0.26±0.02. The difference was statistically significant( P=0.002) and it can be considered that the incomplete necrotic zone of cryoablation was smaller than that of RF ablation. Conclusions:Percutameous intramyocardial septal cryoablation is controllable in scope, ultrasound image evaluation of ablation area is more accurate and incomplete necrosis area is small, which may have potential applications in cardiac ablation.
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Objective:To explore the value of three-dimensional speckle tracking imaging (3D-STI) in the diagnosis of immunoglobulin light-chain cardiac amyloidosis(AL-CA) patients with normal left ventricular ejection fraction (LVEF).Methods:A total of 92 consecutive patients diagnosed with systemic immunoglobulin light chain amyloidosis(sAL) and with normal LVEF from October 2014 to January 2018 in Xijing Hospital were enrolled.Based on the diagnostic criteria of cardiac involvement, the patients were divided into AL-CA group (52 cases) and immunoglobulin light chain amyloidosis (AL) group (40 cases). The clinical data and serological markers of the patients were collected, the conventional echocardiography and full-volume three dimensional dynamic images were acquired, left ventricular global longitudinal strain (GLS), global radial strain (GRS), global circumferential strain (GCS), and global area strain (GAS) were analyzed using off-line TomTec software. The differences between the two groups were compared.Results:Compared with the AL group, the NT-proBNP of AL-CA group was significantly higher ( P<0.05) and there were no significant differences of the other serological indexes between the two groups(all P>0.05). Compared with the AL group, the maximal left ventricular wall thickness, left ventricular mass index, left atrial volume index, and E/e′ in the AL-CA group were significantly increased (all P<0.05). There were no significant differences of other conventional echocardiographic measurements between the two groups(all P>0.05). Compared with the AL group, GLS, GAS, and GRS were significantly lower in AL-CA group (all P<0.05); but there was no significant difference of GCS between the two groups( P>0.05). The ROC curve analysis showed that the cut-off values discriminating cardiac involvement were 16.09% for GLS, 36.54% for GAS and 31.90% for GRS. Conclusions:3D-STI measurements of left ventricular myocardial mechanics could detect cardiac involvement in patients with sAL amyloidosis, and provides a new method for diagnosis of AL-CA.
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Objective:In recent years, circular RNAs (circRNAs) related to stroke has been widely studied, and its change involves multiple pathophysiological aspects of stroke, including angiogenesis, neural plasticity, cell apoptosis, and neuroinflammation. Further exploration of the changes of specific circRNAs after acute ischemic stroke and the molecular biological mechanisms involved will help to develop possible new drug targets and improve the prognosis of patients. This article reviews the main circRNAs that have changed after acute ischemic stroke, and their mechanism of action, functional detection methods, and challenges faced by the current research.
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Objective: To evaluate the cardiac functional changes in hypertrophic cardiomyopathy(HCM) patients with β-myosin heavy chain gene (MYH7) mutations by three-dimensional (3D) speckle tracking imaging(3D-STI) and conventional echocardiography modalities, and then to explore the potential predictors of adverse cardiovascular events in these patients. Methods: A consecutive series of 192 HCM patients admitted in our center from October 2014 to October 2016 were genetically screened to identify MYH7 mutations in this retrospective study. A total of 43 HCM patients with MYH7 mutations were enrolled. The patients were divided into events group(n=13) and no event group(n=30) according to the presence or absence of adverse cardiovascular events(primary and secondary endpoints). All patients were followed up to January 2019 after comprehensive evaluation of 3D-STI, two-dimensional and Doppler echocardiography. The adverse cardiovascular events were recorded. Results: The median follow up time was 1 012 (812, 1 330) days. During follow-up, 13 patients (30.2%) reached endpoints: 6 cases of the primary endpoints(2 cases of sudden cardiac death(SCD), 3 cases of survival after defibrillation, and 1 case of appropriate implantable cardioverter-defibrillator(ICD) discharge); 7 cases of the second endpoints(5 cases of heart failure hospitalization, 1 case of syncope and cardioversion due to supraventricular tachycardia, and 1 case of end-stage HCM). Patients with adverse cardiovascular events had higher prevalence of syncope and risk of SCD, enlarged left atrial volume index(LAVI) and reduced 3D left ventricular global longitudinal train (3D-GLS), as compared to those without adverse events(all P<0.05). The multivariate Cox regression analysis showed that reduced 3D-GLS(HR=0.814, 95%CI 0.663-0.999, P=0.049) was an independent predictor for adverse cardiovascular events. The cutoff value of 3D-GLS≤13.67% was linked with significantly increased risk of adverse cardiovascular events in this patient cohort(AUC=0.753, 95%CI 0.558-0.948, sensitivity 86%, specificity 69%, P<0.05). The Kaplan-Meier analysis indicated that the patients with the 3D-GLS≤ 13.67% faced higher risk of death than those with 3D-GLS>13.67%. Conclusion: 3D-GLS is useful on predicting adverse cardiovascular events in HCM patients with MYH7 mutations.
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Humans , Cardiac Myosins/genetics , Cardiomyopathy, Hypertrophic/genetics , Echocardiography , Mutation , Myosin Heavy Chains/genetics , Predictive Value of Tests , Retrospective Studies , Risk FactorsABSTRACT
Objective@#To evaluate the characteristics of left ventricular structure, function, myocardial mechanics, hemodynamics and synchrony in different phenotypes of hypertrophic cardiomyopathy (HCM) using state-of-the-art echocardiography.@*Methods@#A consecutive series of 85 adult HCM patients who were admitted to the Xi Jing HCM center from January 2016 to November 2017 were collected. According to the peak left ventricular outflow tract pressure gradient in exercise stress echocardiography, the patients were divided into three groups: patients with non-obstructive HCM (n=28), those with labile-obstructive HCM (n=27), and those with obstructive HCM (n=30). In addition, 16 normal family members of HCM patients were included as control group. Two-dimensional speckle tracking imaging, tissue Doppler imaging and exercise stress echocardiography were used to evaluate the left ventricular function in resting and exercise states.@*Results@#①As compared with the control group, left ventricular end-diastolic diameter decreased and left ventricular ejection fraction increased in all three HCM groups(all P<0.05). Left ventricular maximum wall thickness and left ventricular mass index were the highest in obstructive HCM, followed by labile-obstructive and non-obstructive HCM, and the lowest in the control group (all P<0.05). ②At rest, the left ventricular global longitudinal, circumferential and radial strain (GLS, GCS and GRS), as well as the twist of obstructive HCM were significantly lower than the other three groups (all P<0.05). As compared with the control group, the GLS and twist decreased in the labile-obstructive and non-obstructive HCM(all P<0.05), but there were no significant changes of GCS and GRS (all P>0.05). The obstructive HCM had the lowest mitral annular plane systolic excursion (MAPSE) and s′, and the longest systolic peaking time standard deviation(Ts-SD) and early diastolic peaking time standard deviation(Te-SD) (all P<0.05). The left ventricular diastolic function of obstructive HCM (e′, the E/e′ ratio and the left atrial volume index) was the worst, labile-obstruction and non-obstructive HCM were better, and the control group was the best (all P<0.001). ③During exercise, the GLS, GCS, GRS, twist of the left ventricle and the MAPSE were the lowest in the obstructive HCM, which increased in the labile-obstructive and non-obstructive HCM, and were best in the control group. The Ts-SD and Te-SD were the shortest in the control group, were prolonged in non-obstructive and labile-obstruction HCM, and were longest in obstructive HCM (all P<0.05). Additionally, the exercise time of the control group was the longest, followed by non-obstructive and labile-obstruction HCM, and the shortest in the obstructive HCM (all P<0.05). The METs of obstructive HCM were significantly lower than the other three groups (all P<0.05).@*Conclusions@#In obstructive HCM, the left ventricular systolic strain and synchronization, as well as the MAPSE, are significantly impaired in patients both at rest and during exercise. The patients with labile-obstructive and non-obstructive HCM have reduced left ventricular GLS, twist, and e′, but normal left ventricular GCS, GRS, synchrony, and MAPSE at rest, which are all impaired during exercise.
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Objective To evaluate the characteristics of left ventricular structure ,function ,myocardial mechanics ,hemodynamics and synchrony in different phenotypes of hypertrophic cardiomyopathy ( HCM ) using state‐of‐the‐art echocardiography . Methods A consecutive series of 85 adult HCM patients w ho were admitted to the Xi Jing HCM center from January 2016 to November 2017 were collected . According to the peak left ventricular outflow tract pressure gradient in exercise stress echocardiography ,the patients were divided into three groups :patients with non‐obstructive HCM ( n ＝28) ,those with labile‐obstructive HCM ( n ＝27) ,and those with obstructive HCM ( n ＝ 30 ) . In addition ,16 normal family members of HCM patients were included as control group . T wo‐dimensional speckle tracking imaging ,tissue Doppler imaging and exercise stress echocardiography were used to evaluate the left ventricular function in resting and exercise states . Results ① As compared with the control group ,left ventricular end‐diastolic diameter decreased and left ventricular ejection fraction increased in all three HCM groups ( all P ＜ 0 .05 ) . Left ventricular maximum wall thickness and left ventricular mass index were the highest in obstructive HCM , followed by labile‐obstructive and non‐obstructive HCM ,and the lowest in the control group ( all P ＜0 .05) . ②A t rest ,the left ventricular global longitudinal ,circumferential and radial strain ( GLS ,GCS and GRS) ,as well as the twist of obstructive HCM were significantly lower than the other three groups ( all P ＜0 .05) . As compared with the control group ,the GLS and twist decreased in the labile‐obstructive and non‐obstructive HCM ( all P ＜0 .05 ) ,but there were no significant changes of GCS and GRS ( all P ＞ 0 .05 ) . T he obstructive HCM had the lowest mitral annular plane systolic excursion ( M APSE ) and s′,and the longest systolic peaking time standard deviation( T s‐SD) and early diastolic peaking time standard deviation ( Te‐SD) ( all P ＜0 .05) . T he left ventricular diastolic function of obstructive HCM ( e′,the E/e′ratio and the left atrial volume index ) was the worst ,labile‐obstruction and non‐obstructive HCM were better ,and the control group was the best ( all P ＜ 0 .001 ) . ③ During exercise ,the GLS ,GCS ,GRS ,twist of the left ventricle and the M APSE were the lowest in the obstructive HCM ,which increased in the labile‐obstructive and non‐obstructive HCM ,and were best in the control group . T he T s‐SD and Te‐SD were the shortest in the control group ,were prolonged in non‐obstructive and labile‐obstruction HCM ,and were longest in obstructive HCM ( all P ＜ 0 .05 ) . Additionally ,the exercise time of the control group was the longest , followed by non‐obstructive and labile‐obstruction HCM ,and the shortest in the obstructive HCM ( all P ＜0 .05) . T he M ET s of obstructive HCM were significantly lower than the other three groups ( all P ＜0 .05) . Conclusions In obstructive HCM ,the left ventricular systolic strain and synchronization ,as well as the M APSE ,are significantly impaired in patients both at rest and during exercise . T he patients with labile‐obstructive and non‐obstructive HCM have reduced left ventricular GLS , twist ,and e′,but normal left ventricular GCS ,GRS ,synchrony ,and M APSE at rest ,which are all impaired during exercise .
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Objective@#To investigate the safety and efficacy of transthoracic echocardiography-guided percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) in patients with hypertrophic obstructive cardiomyopathy (HOCM).@*Methods@#Nine HOCM patients with interventricular septal thickness ≥15 mm and ≤25 mm who were treated with PIMSRA between October 2016 to March 2017 in the Hypertrophic Cardiomyopathy Center of Xijing Hospital of Air Force Military Medical University were enrolled,and the clinical data were retrospectively analyzed.Interventricular septum thickness, left ventricular outflow tract diameter and maximum gradient were measured by transthoracic echocardiography immediately after procedure, at 1 month, 3 months and 6 months after operation.Symptoms and New York Heart Association (NYHA) functional class were assessed, and provoked left ventricular outflow tract gradient and exercise time were measured after 6 months.@*Results@#The anterior interventricular septum ((21.5±2.6) mm vs. (24.7±2.7) mm, P<0.05) and posterior interventricular septum (21.1±2.5) mm vs. (22.6±3.3) mm, P<0.05) were significantly increased,left ventricular outflow tract diameter was widened ((8.2±3.4) mm vs. (4.8±2.2) mm, P<0.05), left ventricular outflow tract gradient ((26.8±19.6) mmHg (1 mmHg=0.133 kPa) vs. (83.3±32.4) mmHg, P<0.05) and mitral regurgitation (2.0±1.9) ml vs. (3.2±3.0) ml, P<0.05) were significantly decreased immediately after ablation compared with pre-operation values. Anterior interventricular septum, posterior interventricular septum and left ventricular outflow tract gradient further decreased after 1 month ((17.5±2.0) mm vs. (24.7±2.7) mm, P<0.05; (16.9±2.1) mm vs. (22.6±3.3) mm, P<0.05; (11.6±4.0) mmHg vs. (26.8±19.6) mmHg, P<0.05, respectively) compared with values immediately after ablation. Anterior interventricular septum and posterior interventricular septum decreased after 3 and 6 months ((14.8±1.7) mm and (13.4±2.0) mm vs. (17.5±2.0) mm, all P<0.05; (12.9±1.9) mm and (12.3±2.4) mm vs. (16.9±2.1) mm, all P<0.05, respectively) compared with values at 1 month after ablation.There were no significantly difference in left ventricular outflow tract gradient at 3 and 6 months post procedure compared with 1 month after ablation (all P>0.05). Compared with pre-operation, provoked left ventricular outflow tract gradient decreased ((25.5±11.4) mmHg vs. (147.8±58.0) mmHg, P<0.01), and total exercise time increased ((9.3±1.6) minutes vs. (6.7±1.6) minutes, P=0.03) at 6 months after operation.The symptoms were disappeared in 5 patients. There were 2 cases with NYHA class Ⅱ and 7 cases with NYHA class Ⅲ before operation,while there were 6 patients with NYHA classⅠ and 3 patients with NYHA class Ⅱ at 6 months after operation (P<0.01).@*Conclusion@#Transthoracic echocardiography-guided PIMSRA is a safe and effective new treatment approach for patients with obstructive hypertrophic cardiomyopathy.
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Objective To explore the changes of left ventricular torsion function in patients with latent obstructive hypertrophic cardiomyopathy ( HCM ) ,and provide quantitative informations for clinical evaluation of cardiac function . Methods A total of 49 consecutive patients with HCM without left ventricular outflow tract obstruction at rest were enrolled . All subjects underwent exercise stress echocardiography . After exercise left ventricular outflow tract pressure gradient ( LVO T‐PG ) ≥30 mm Hg was positive for exercise stress test ( latent obstruction) ,w hile LVO T‐PG＜ 30 mm Hg was negative for exercise stress test ( non‐obstruction) . An ultrasound system obtained two‐dimensional ultrasound images of resting and moving peaks . The global longitudinal strain ( GLS ) ,global circumferential strain ( GCS ) , global radial strain ( GRS) of the left ventricle 16 segments and left ventricular rotation ,twist were analysis using off‐line EchoPAC software . T he differences of the above parameters were compared between the two groups . Results T here were no significant differences in GLS ,GRS ,GCS and Rotation‐B between the two groups in resting and peak period of exercise ( all P ＞ 0 .05 ) ,GRS in both groups were significantly increased compared with that before exercise ( all P ＜ 0 .05 ) . Compared with the negative exercise stress group ,the left ventricular twist and Rotation‐A were significantly increased in resting and peak period of exercise in the positive exercise stress test group( all P ＜0 .05) . Compared with before exercise ,Rotation‐A and left ventricular twist were significantly decreased in the positive exercise stress test group ( all P ＜0 .05) ,while no significantly difference was found in the negative exercise stress group ( all P ＞ 0 .05 ) . Conclusions Left ventricular torsion function is significantly changed in rest and after exercise in latent obstructive HCM patients ,providing valuable quantitative information for clinical comprehensive evaluation of cardiac function .
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Objective To investigate whether three-dimensional speckle tracking imaging ( 3D-STI) combined with conventional echocardiography can identify cardiac functional characteristics and predict adverse cardiovascular events in patients with hypertrophic cardiomyopathy ( HCM ) . Methods One hundred and eighty HCM patients were involved in the study . All patients were followed up to March 2017 after comprehensive evaluation of 3D-STI and conventional echocardiography for endpoint events . The endpoint events were divided into the primary and secondary endpoints . The primary endpoints included sudden cardiac death ( SCD ) , survival after cardioversion and appropriate implantable cardioverter-defibrillator( ICD) discharge ;the secondary endpoints included acute myocardial infarction ,heart failure hospitalization ,thromboembolism and endstage HCM . The composite endpoints contained primary or secondary endpoints . Results ①Totally 175 HCM patients completed the follow-up [ ( 435 ± 204) days] . During follow-up ,25 patients ( 14 .3% ) reached composite endpoints :8 cases of the primary endpoints ( 3 cases of sudden cardiac death ,3 cases of survival after defibrillation ,and 2 cases of appropriate implantable cardioverter-defibrillator discharge ) ; 17 cases of the second endpoints ( 14 cases of heart failure hospitalization ,2 cases of thromboembolism ,and 1 case of end-stage HCM ) . ② Patients with primary endpoints had higher NYHA class ,reduced systolic and early diastolic mitral annular velocities (MV s′and MV e′) ,decreased systolic tricuspid annular velocity ( TV s′) ,and impaired 3D left ventricular global longitudinal strain ( GLS ) ,as compared to those without primary endpoints ( n ＝ 167 ) ( P ＜ 0 .05 ) . Impaired 3D GLS ( hazard ratio was 0 .72 ,95% CI was 0 .53 －0 .98 , P ＝ 0 .035) and decreased TV s′( hazard ratio was 0 .70 ,95% CI was 0 .54－0 .91 , P ＝0 .007) were independent predictor factor for primary endpoints . 3D GLS≤11 .7% or TV s′≤12 .7 cm/s raised the risk of primary endpoints . ③Similarly ,HCM patients with composite endpoints ( n ＝25) had higher NYHA class ,enlarged left atrial diameter ( LAD) , reduced MV s′,MV e′ and TV s′,as well as impaired 3D GLS ( P ＜ 0 .05) ,when compared to those without composite endpoints ( n＝150) . Moreover ,impaired 3D GLS ( hazard ratio was 0 .68 ,95% CI was 0 .56－0 .81 , P ＝0 .000) was independent predictor for composite endpoints ;and patients with 3D GLS≤12 .9% was more susceptible to composite endpoints . Conclusions 3D GLS combined with TV s′ are of value in predicting adverse cardiovascular events .
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Objective To explore the predictive value of routine echocardiographic and electrocardiographic parameters in late gadolinium enhancement ( LGE ) in hypertrophic cardiomyopathy ( HCM ) . Methods The study population consisted of a consecutive series of 95 HCM patients .According to the presence of LGE on cardiac magnetic resonance (CMR) ,these patients were divided into two groups :HCM patients with LGE ( n = 71) and HCM patients without LGE ( n = 24) . The parameters of routine echocardiography and electrocardiography were compared between the two groups . ROC and Logistic analysis were made to find the predictors of LGE . Results ① As compared to those without LGE ,HCM patients with LGE had higher prevalence of chest pain ( P = 0 .027) ,β-blocker treatment ( P = 0 .024) , increased maximal left ventricular wall thickness ( MLVWT ) ( P < 0 .0001 ) ,non-sustained ventricular tachycardia ( P = 0 .034) ,prolonged the rate-corrected cardiac QT interval ( QTc) ( P = 0 .011) ,T-wave inversion ( TWI) ( P = 0 .009) ,but reduced early diastolic mitral annular velocity ( e′) ( P = 0 .001) . ②Univariate predictors of LGE on CMR were :increased MLVWT ,reduced e′ ,prolonged QTc and more TWI . Only MLVWT ( OR = 1 .23 ,95％ CI = 1 .05 - 1 .44 , P = 0 .013) and e′( OR = 1 .23 ,95％ CI = 0 .52 - 0 .96 , P =0 .028) remained independent after multivariable analysis . Furthermore ,the ROC analysis showed that these two parameters had discriminative ability to identify those with LGE . To be specific ,HCM patients with MLVWT ≥ 21 .5 mm or e′ ≤ 5 .55 cm /s were more likely to present with LGE . ③ The leads number of TWI was positively correlated with percentage of LGE in left ventricular mass ( LGE ％ ) ( r = 0 .220 , P =0 .044) ,but there was no correlation between location of TWI on ECG and territory of LGE on CMR . Conclusions In HCM patients ,MLVWT and e′ are independent predictors of LGE on CMR . Furthermore , although the leads number of TWI is correlated with LGE ％ ,no correlation has been found between location of TWI on ECG and territory of LGE on CMR .
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<p><b>OBJECTIVE</b>To explore the genotype-phenotype correlation of a MYH7-D554Y mutation identified in an ethnic Han Chinese pedigree affected with hypertrophic cardiomyopathy.</p><p><b>METHODS</b>Ninety six cardiovascular disease-related genes were detected in the proband by exonic amplification and high-throughput sequencing. Suspected mutations were verified by Sanger sequencing among 300 healthy controls as well as family members of the proband. The pathogenicity and conservation of the detected mutations were analyzed with ClustalX, MutationTaster, PolyPhen-2, Provean and SIFT software.</p><p><b>RESULTS</b>Four of the 5 first-degree relatives of the proband were diagnosed with hypertrophic cardiomyopathy. The proband has featured extremely hypertrophic left ventricular wall with a maximal thickness of 35 mm. Genetic testing showed that four of them have carried a heterozygous c.1660G>T (p.Asp554Tyr) mutation of the MYH7 gene, who the remaining one was phenotypically normal and did not carry the mutation. The mutation has not been recorded by the Human Gene Mutation Database (HGMD) and other databases. Bioinformatics analysis suggested that the mutation site is highly conserved and that the mutation is pathogenic.</p><p><b>CONCLUSION</b>The p.Asp554Tyr mutation of the MYH7 gene probably underlies the hypertrophic cardiomyopathy in this pedigree.</p>
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Objective To assess whether strain parameters derived from three-dimensional speckle tracking imaging ( 3D-STI) could identify improvement of cardiac function and predict response of patients with immunoglobulin light-chain cardiac amyloidosis ( AL-CA) following chemotherapy . Methods Totally 13 patients with AL-CA [aged (58 .5 ± 8 .9)years;69％ males] were treated with melphalan or bortezomib-based regimens and by regular chemotherapy for 6 months .The clinical data was collected . Maximal left ventricular wall thickness (MLVWT) ,left ventricular mass index (LVMI) ,left ventricular ejection fraction ( LVEF) ,systolic mitral annular velocity ( s′) ,early diastolic mitral annular velocity echocardiography ( e′) , 3D-STI global longitudinal ,circumferential ,radial and area strain (GLS ,GCS ,GRS ,and GAS) ,the standard deviation of time to peak longitudinal strain among 16 left ventricular segments ( TS-SD_GLS ) were obtained by conventional echocardiography and 3D-STI . In addition ,serologic biomarkers including N-terminal pro-brain natriuretic peptide ( NT-proBNP) and free light chains ( FLC) were acquired at baseline and 6 months after chemotherapy . These patients were divided into two groups according to difference of FLC :complete response (CR) group and Non-CR group . The clinical data ,cardiac ultrasound parameters and serological parameters were compared between groups ,each group parameters at baseline and 6 months after chemotherapy were compared within the group.Results ①There were no significant differencein conventional echocardiographic parameters ,GCS ,GRS and GAS ,as well as TS-SD_GLS in either group between before and after chemotherapy . But GLS was improved only in CR group ( P = 0 .036) ,and its improvement was correlated with the decrease in NT-proBNP ( r = -0 .738 , P = 0 .037) . ② In baseline evaluation ,patients in Non-CR group had increased LVMI ,deteriorated e′ and GLS ,and longer Ts-SD as compared to those in CR group ( all P < 0 .05 ) . ③ ROC analysis revealed that these parameters had discriminative ability to forecast those with better therapeutic effectiveness ,esecially for AL-CA patients with baseline LVMI<96 .55 g/m2 ,e′>4 .7 cm/s ,absolute value of GLS>16 .6％ ,and TS-SD_GLS<35 .2 ms ,which may have better hematologic response to chemotherapy . Conclusions GLS can identify early improvement of cardiac function in AL-CA patients after chemotherapy . Moreover ,LVMI ,e′,GLS and TS-SD_GLS are sensitive measurements of pre-treatment ventricular impairment ,and may predict better response to chemotherapy .
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Objective@#To evaluate the predicting value of the 2014 European Society of Cardiology (ESC) guidelines risk prediction model for sudden cardiac death (HCM Risk-SCD) in Chinese patients with hypertrophic cardiomyopathy (HCM), and to explore the predictors of adverse cardiovascular events in Chinese HCM patients.@*Methods@#The study population consisted of a consecutive 207 HCM patients admitted in our center from October 2014 to October 2016. All patients were followed up to March 2017. The 5-year SCD probability of each patient was estimated using HCM Risk-SCD model based on electrocardiogram, echocardiography and cardiac magnetic resonance (CMR) examination results. The primary, second, and composite endpoints were recorded. The primary endpoint included SCD and appropriate ICD therapy, identical to the HCM Risk-SCD endpoint. The second endpoint included acute myocardial infarction, hospitalization for heart failure, thrombus embolism and end-stage HCM. The composite endpoint was either the primary or the second endpoint. Patients were divided into the 3 categories according to 5-year SCD probability assessed by HCM Risk-SCD model: low risk group<4%,intermediate risk group ≥4% to<6%, and high risk group≥6%.@*Results@#(1) Prevalence of endpoints: All 207 HCM patients completed the follow-up (350 (230, 547) days). During follow-up, 8 (3.86%) patients reached the primary endpoints (3 cases of SCD, 3 cases of survival after defibrillation, and 2 cases of appropriate ICD discharge); 21 (10.14%) patients reached the second endpoints (1 case of acute myocardial infarction, 16 cases of heart failure hospitalization, 2 cases of thromboembolism, and 2 cases of end-stage HCM). (2) Predicting value of HCM Risk-SCD model: Patients with primary endpoints had higher prevalence of syncope and intermediate-high risk of 5-year SCD, as compared to those without primary endpoints (both P<0.05). (3) Predicting value of HCM Risk-SCD model: The low risk group included 122 patients (59%), the intermediate risk group 42 (20%), and the high risk group 43 (21%). There was a clear trend towards to higher heart rate, higher values of PTFV1 and plane QRS-T angle, higher left ventricular mass index (LVMI), elevated maximal left ventricular outflow tract pressure gradient (LVOT-PGmax), enlarged left atrial dimension(LAD) and volume index (LAVI), reduced systolic mitral annular velocity (s'), and higher late gadolinium enhancement (LGE) volume and mass in patients with high risk of 5-year of SCD, as compared to those with low-intermediate risk (all P<0.05). Moreover, 5-year SCD probability was positively correlated with heart rate, plane QRS-T angle, LVMI, LAVI, LGE%, and negatively correlated with s'(r=0.161, P=0.019; r=0.669, P=0.001; r=0.206, P=0.004; r=0.284, P=0.000; r=0.351, P=0.000; r= -0.245, P=0.001; respectively). (4) LAD, LAVI, e' and s' were independent predictors for poor outcomes. HCM patients with LAD≥39 mm, LAVI≥49.6 ml/m2, e'≤6.5 cm/s and s'≤6.6 cm/s were more likely to have adverse cardiovascular events (AUC 0.702, 95%CI 0.604-0.799, P=0.001; AUC 0.700, 95%CI 0.567-0.833, P=0.001; AUC 0.716, 95%CI 0.616-0.817, P=0.000; AUC 0.764, 95%CI 0.676-0.853, P=0.000,respectively).@*Conclusions@#The HCM Risk-SCD model is of value in predicting SCD for Chinese HCM patients. The plane QRS-T angle and LGE% are the best predictors of 5-year SCD risk in Chinese HCM patients. Moreover, conventional echocardiographic parameters, including LAD, LAVI, e' and s', are useful to predict adverse cardiovascular events among Chinese HCM patients.
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Objective To evaluate left ventriclular systolic function in patients with obstructive hypertrophic cardiomyopathy (HOCM) after modified Morrow surgery using two-dimensional speckle tracking imaging (2D-STI).Methods Twenty three HOCM patients were recruited in this study.Echocardiographic data from HOCM patients during pre-operation,1-month and 3-month post-operation were analyzed by Qlab software to compare the variation in systolic function indicators 1-month and 3-month post-operation including global and 16 segmental longitudinal strain,circumferential strain and conventional echocardiographic parameters of left ventricle.Results Compared with preoperative data of HOCM patients,postoperative LVOT diameter and pressure gradient,left atrial diameter and volume index were significantly decreased(P<0.05),but there was no significant difference in left ventricular ejection fraction(P<0.05).Compared with the preoperative case,global and segmental longitudinal strain showed significant reduction after 1 week and gradually recovered after 3 months,without significant variation.The longitudinal strain of the anteroseptum reduced significantly and the longitudinal strain of the free wall increased after 3 months,however,the circumferential strain reduced significantly.The circumferential strain of basal and middle segment after 3 months had improved significantly than those of postoperative 1 week.The circumferential strain of surgical site is no obvious change and the strain of free wall was improved after 3 months.Conclusions 2D-STI can effectively evaluate global and regional systolic function of left ventricle for HOCM patients after modified Morrow surgery.
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<p><b>OBJECTIVE</b>To analyze the phenotype-genotype correlation of MYH7-V878A mutation.</p><p><b>METHODS</b>Exonic amplification and high-throughput sequencing of 96-cardiovascular disease-related genes were carried out on probands from 210 pedigrees affected with hypertrophic cardiomyopathy (HCM). For the probands, their family members, and 300 healthy volunteers, the identified MYH7-V878A mutation was verified by Sanger sequencing. Information of the HCM patients and their family members, including clinical data, physical examination, echocardiography (UCG), electrocardiography (ECG), and conserved sequence of the mutation among various species were analyzed.</p><p><b>RESULTS</b>A MYH7-V878A mutation was detected in five HCM pedigrees containing 31 family members. Fourteen members have carried the mutation, among whom 11 were diagnosed with HCM, while 3 did not meet the diagnostic criteria. Some of the fourteen members also carried other mutations. Family members not carrying the mutation had normal UCG and ECG. No MYH7-V878A mutation was found among the 300 healthy volunteers. Analysis of sequence conservation showed that the amino acid is located in highly conserved regions among various species.</p><p><b>CONCLUSION</b>MYH7-V878A is a hot spot among ethnic Han Chinese with a high penetrance. Functional analysis of the conserved sequences suggested that the mutation may cause significant alteration of the function. MYH7-V878A has a significant value for the early diagnosis of HCM.</p>
Subject(s)
Adult , Female , Humans , Male , Middle Aged , Young Adult , Amino Acid Sequence , Asian People , Genetics , Cardiac Myosins , Genetics , Cardiomyopathy, Hypertrophic , Genetics , Genetic Association Studies , Methods , Genotype , Mutation , Genetics , Myosin Heavy Chains , Genetics , Pedigree , PhenotypeABSTRACT
<p><b>OBJECTIVE</b>To explore the relationship between electrocardiographic (ECG) and genetic mutations of patients with hypertrophic cardiomyopathy (HCM), and early ECG changes in HCM patients.</p><p><b>METHODS</b>Clinical, 12-lead ECG and echocardiographic examination as well as genetic examinations were made in a three-generation Chinses HCM pedigree with 8 family members (4 males). The clinical characterization and ECG parameters were analyzed and their relationship with genotypes in the family was explored.</p><p><b>RESULTS</b>Four missense mutations (MYH7-H1717Q, MYLK2-K324E, KCNQ1-R190W, TMEM70-I147T) were detected in this pedigree. The proband carried all 4 mutations and 5 members carried 2 mutations. Corrected QTc interval of KCNQ1-H1717Q carriers was significantly prolonged and was consistent with the ECG characterization of long QT syndrome. MYLK2-K324E and KCNQ1-R190W carriers presented with Q wave and(or) depressed ST segment, as well as flatted or reversed T waves in leads from anterolateral and inferior ventricular walls. ECG results showed ST segment depression, flat and inverted T wave in the gene mutation carriers with normal echocardiographic examination results. ECG and echocardiographic results were normal in TMEM70-I147T mutation carrier.</p><p><b>CONCLUSIONS</b>The combined mutations of the genes associated with cardiac ion channels and HCM are linked with the ECG phenotype changes in this HCM pedigree. The variations in ECG parameters due to the genetic mutation appear earlier than the echocardiography and clinical manifestations. Variation in ECG may become one of the indexes for early diagnostic screening and disease progression of the HCM gene mutation carriers.</p>
Subject(s)
Humans , Brugada Syndrome , Cardiac Conduction System Disease , Cardiac Myosins , Cardiomyopathy, Hypertrophic , Echocardiography , Electrocardiography , Exons , Genetic Testing , Genotype , KCNQ1 Potassium Channel , Long QT Syndrome , Mutation , Mutation, Missense , Myosin Heavy Chains , Myosin-Light-Chain Kinase , Pedigree , PhenotypeABSTRACT
Objective To evaluate the short-term efficacy of cardiac resynchronization therapy(CRT)by three-dimensional speckle tracking imaging (3D-STI).Methods 27 chronic heart failure patients who underwent CRT were enrolled.All following indices were measured respectively:the left ventricular systolic function indices in the 16 segments of 3D-STI including the mean area peak strain,the mean longitudinal peak strain,the mean circumferential peak strain,and the mean radial peak strain(MAS,MLS,MCS,MRS) ; the strain-derived dyssynchrony indices in the 16 segments of 3D-STI including the standard deviation for time to peak area strain,the standard deviation for time to peak longitudinal strain,the standard deviation for time to peak circumferential strain,and the standard deviation for time to peak radial strain (TAS-SD,TCS-SD,TLS-SD,TRS-SD) ;the standard deviation for time to peak area strain,the standard deviation for time to peak circumferential strain,the standard deviation for time to peak longitudinal strain,and the standard deviation for time to peak radial strain(TAS-dif,TCS-dif,TLS-dif,TRS-dif) ;the rotation and twist index of 3D-STI including peak rotation on apical plane,peak rotation on basal plane,peak twist angle(RotA,RotB,Twist),time to peak of the rotation on apical,time to peak of the rotation on bastal,time to peak of the twist (PTRotA,PTRotB,PTtw).The differences of all the indices between before CRT and 3 months after CRT were calculated and the correlation between the changes of all those indices and the changes of left ventricular ejection fraction (△LVEF) were analyzed.Results MAS,MLS,MCS,MRS,RotA and Twist were increased 3 months after CRT (P ＜0.01,P ＜0.05,P ＜0.01,P ＜0.01,P＜ 0.01,P ＜0.01);TRS-SD and PTtw was decreased 3 months after CRT (P ＜0.01,P ＜0.05) ; |△MAS|,| △MLS|,△TRSSD,△RotA and △Twist were correlated with △LVEF 3 months after CRT (r =0.521,P =0.005; r =0.389,P =0.045; r =-0.560,P =0.002; r =0.513,P =0.006; r =0.610,P =0.001).Conclusions The myocardial systolic strain function,dyssynchrony and twist function of left ventricle were improved significantly 3 months after CRT.3D-STI offered a new way for the evaluation of short-term efficacy of CRT.