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Uric acid is associated with cardiac death in patients with hypertrophic obstructive cardiomyopathy
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作者 Jun GAO Chun-Li SHAO +5 位作者 Xiang-Bin MENG Wen-Yao WANG Kuo ZHANG Jing-Jia WANG Ming-Qi ZHENG Yi-Da TANG 《Journal of Geriatric Cardiology》 SCIE CAS CSCD 2021年第4期281-288,共8页
BACKGROUND The role of uric acid(UA) in survival of patients with hypertrophic obstructive cardiomyopathy(HOCM) has not been fully evaluated. This study aimed to determine whether UA could be an independent risk facto... BACKGROUND The role of uric acid(UA) in survival of patients with hypertrophic obstructive cardiomyopathy(HOCM) has not been fully evaluated. This study aimed to determine whether UA could be an independent risk factor of cardiac death in patients with HOCM.METHODS A total of 317 patients with HOCM, who were receiving conservative treatment in Fuwai Hospital from October2009 to December 2014, all of them completed UA evaluations, were analyzed. Patients were divided into three groups according to the UA levels: Tertile 1(≤ 318 μmol/L, n = 106), Tertile 2(319 to 397 μmol/L, n = 105), and Tertile 3(≥ 398 μmol/L, n = 106).RESULTS During a median follow-up of 45 months, 29 cardiac deaths(9.1%) occurred, including 6 sudden cardiac deaths and23 heart failure-related deaths. Cardiac death in Tertile 3(n = 16, 55.2%) was significantly higher than in Tertile 1(n = 6, 20.7%)and Tertile 2(n = 7, 24.1%). In univariate model, UA level(continuous value) showed predictive value of cardiac death [hazard ratio(HR) = 1.006, 95% CI: 1.003-1.009, P = 0.009]. Univariate Cox survival analysis had shown a significant higher property of cardiac death in patients of Tertile 3 when compared with those of Tertile 1, but cardiac death in patients of Tertile 2 did not show significant prognositic value compared with those of Tertile 1(HR = 3.927, 95% CI: 0.666-23.162, P = 0.131). UA was found to be an independent risk factor(HR = 1.005, 95% CI: 1.001-1.009, P = 0.009) of cardiac death in the multivariate regression analysis after the adjustment for age, body mass index, atrial fibrillation, hemoglobin, creatinine, high-sensitivity C-reactive protein, interventricular septum/left ventricular posterior wall ratio, left ventricular outflow tract and left ventricular ejection fraction.CONCLUSIONS UA concentration was found to be independently associated with cardiac death in HOCM patients receiving conservative treatment. Randomized trials of UA-lowering agents for HOCM patients are warranted. 展开更多
关键词 CRP Uric acid is associated with cardiac death in patients with hypertrophic obstructive cardiomyopathy
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Surgical outcomes and strategy of hypertrophic obstructive cardiomyopathy 被引量:1
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作者 朱亚彬 RAJAN S. +1 位作者 KURIAN V.M. 刘志勇 《Journal of Zhejiang University-Science B(Biomedicine & Biotechnology)》 SCIE CAS CSCD 2006年第2期148-153,共6页
Objective: To evaluate the surgical clinical results of hypertrophic obstructive cardiomyopathy. Methods: We retrospectively collected data on 24 patients who underwent surgical management in the past ten years in t... Objective: To evaluate the surgical clinical results of hypertrophic obstructive cardiomyopathy. Methods: We retrospectively collected data on 24 patients who underwent surgical management in the past ten years in two hospitals in China and Madras Medical Mission in India. Myomectomy was carried out on all patients. Among them 3 patients underwent mitral valve replacement; 2 patients underwent mitral valve repair (anterior mitral leaflet plication); 2 patients underwent aortic valve replacement; 1 patient underwent aortic valve repair; 2 patients underwent aortic root replacement; 1 patient underwent Bentall's procedure and 1 patient underwent coronary artery bypass grafting because of a breached muscle bridge, Results: One patient died of post-operative heart failure. The mean follow-up time was 4.3 years, There was significant improvement in the symptomatic status. Sixteen patients were asymptomatic with good effort tolerance and only four patients had New York heart association (NYHA) Classes Ⅰ-Ⅱ due to associated valvular lesions, Conclusion: Our experience proved that symptomatic hypertrophic obstructive cardiomyopathy or non-symptomatic hypertrophic obstructive cardiomyopathy with combined heart disease is indication for surgery as surgical intervention could get better clinical results in this kind of patients compared with other non-surgical method because it beneficially reduces the systolic anterior motion (SAM) of the mitral valve leaflet, which could not be avoided by other non-surgical treatment. 展开更多
关键词 hypertrophic obstructive cardiomyopathy Systolic anterior motion MYOMECTOMY
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Mid-ventricular hypertrophic obstructive cardiomyopathy(MVHOCM) complicated with coronary artery disease:a case report
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作者 Haoming Song Cuimei Zhao Jinfa Jiang Yang Liu Yihan Chen Department of Cardiology,Tongji Hospital, Tongji University, Shanghai, 200065, P.R.China 《Journal of Geriatric Cardiology》 SCIE CAS CSCD 2008年第3期190-192,共3页
Mid-ventricular hypertrophic obstructive cardiomyopathy (MVHOCM) is a rare type ofcardiomyopathy that can be accompanied by apical aneurysm.We presented here a case report of MVHOCM with cornary artery disease.The six... Mid-ventricular hypertrophic obstructive cardiomyopathy (MVHOCM) is a rare type ofcardiomyopathy that can be accompanied by apical aneurysm.We presented here a case report of MVHOCM with cornary artery disease.The sixty-four years old man was sent to hospital because ofventricular tachycardia.Large inversion T wave was showed on electrocardiography in the presence of abnormal coronary arteries and normal cardiac enzyme.Echocardiography showed an hourglass appearance of the left ventricle with an aneurysm in the apex and a pressure gradient between the outflow tract of left ventricle and the middle of the left ventricle was revealed by left-heart catheterization. 展开更多
关键词 hypertrophic obstructive cardiomyopathy coronary artery disease ECHOCARDIOGRAPHY
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Transcatherter chemical myocardial ablation for hypertrophic obstructive cardiomyopathy: short to mid term results.
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作者 赵林阳 陈志营 +1 位作者 王仁飞 严明洲 《海南医学》 CAS 2002年第11期74-75,共2页
Objective: Hypertrophic obstructive cardiomyopathy is still a troublesome clinical problem, lack of good treatment method. We use transcatheter chemical myocardial ablation to treat hypertrophic obstructive cardiomyop... Objective: Hypertrophic obstructive cardiomyopathy is still a troublesome clinical problem, lack of good treatment method. We use transcatheter chemical myocardial ablation to treat hypertrophic obstructive cardiomyopathy and analysis 25 cases results, to search a novel technique for hypertrophic obstructive cardiomyopathy.Method: From Nov.1996 to Oct.2001 25patients with symptomitical and drug resistents hypertrophic obstructive cardiomyopathy acccepted a nonsurgical treatment, that is percutaneous transseptal myocardial ablation (PTSMA) in our department including 16 males and 9 females, with mean age 44.1 years All patients meet echocardiography diagnosis criterias of hypertrophic obstructive cardiomyopathy, With clinical symptoms of angiana pectors, sycope, short breath etc. No improvement to long term Medical theraphy, rest pressure left ventricular outflow tract gradients over 30 mmHg, or over 50mmHg during provocal test either by invasion or indirect measurement with Doppler echocardiography, 5 patients accepted treatment With Sigwart’s method as previous described, other 20 cases with pigtail catheter left ventricular Continuous curve to measure left ventricular outflow tract gradient, percutaneous insert 1.5mm to 2.0mm balloon catheter into first septal branch of left anterior descending coronary artery, via inflated balloon , inject 3-5ml absolute alcohol into the artery , keep inflating balloon for other 5 minutes continue monitoring pressure gradients and ECG, temporary pacemaker and defibrillator were stand by.Results: All patients, left ventricular outflowtract gradients were significantly reduced, more than 50%, 6 cases occurred serve bradycardia, hypotension need immediate administration, 17 cases with trancient complete right branch block, and in five cases, with permonant complete right branch block,2 cases with transient complete AV block. In follow up, symptom and life quality of all patients were dramitically improved, in first month post procedure, septum thickness reduction, 6mm in average and, 6.8 mm reduction were achieved in 1.5 to 2 years.and 12 cases were follow ed up for more than 4 years ,the longest one has been 6 years still in perfect condition. Anther 5 cases chemical myocardial ablation can not be oerforned for vessels anatomy reasons accepted dual chamber pacing,get compariale results with PTSMA.Conclusion: Our results shown that this technique is a safety and reliable, might be an alternative method to patients with hypertrophic obstructive cardiomyopathy and we although should mention, our anther 5 patients whese can not be performed PTSMA received DDDR pacemaker, with symptom improvement and pressure gradients reduction for two years, as demonstrated by other experts, is althought a opitmal therapy for HOCM. 展开更多
关键词 射频消融 心肌疾病 近期疗效 中期疗效
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Angle of Attack between Blood Flow and Mitral Valve Leaflets in Hypertrophic Obstructive Cardiomyopathy: An In Vivo Multipatient CT-based FSI Study
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作者 Long Deng Xueying Huang +4 位作者 Heng Zuo Yuan Zheng Chun Yang Yunhu Song Dalin Tang 《Computer Modeling in Engineering & Sciences》 SCIE EI 2018年第8期115-125,共11页
The mechanisms of systolic anterior motion(SAM)of the mitral valve in hypertrophic obstructive cardiomyopathy(HOCM)remain unclear.To investigate the angle of attack between blood flow and mitral valve leaflets at pre-... The mechanisms of systolic anterior motion(SAM)of the mitral valve in hypertrophic obstructive cardiomyopathy(HOCM)remain unclear.To investigate the angle of attack between blood flow and mitral valve leaflets at pre-SAM time point,patient-specific CT-based computational models were constructed for 5 patients receiving septal myectomy surgery to obtain pre-and post-operative 2D vector flow mapping.The comparisons between pre-and post-operative angles of attack based on 2D vector flow mapping of 5 patients were performed.It was found that there was no statistically significant difference between pre-and post-operative angles of attack(61.1±t wa o vs.56.2±56.o,p=0.306,n=5).Therefore,we propose that the angle of attack might not play an important role in the initiation of SAM. 展开更多
关键词 hypertrophic obstructive cardiomyopathy SYSTOLIC anterior motion mitralvalve LEAFLETS computational model angle of attack
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Right Ventricular Mechanical Changes after Septal Myectomy for Obstructive Hypertrophic Cardiomyopathy: Vector Velocity Imaging Study
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作者 Hala Mahfouz Badran Ghada Soltan +2 位作者 Naglaa Faheem Mohamed Ezzat Enait Magdi H. Yacoub 《World Journal of Cardiovascular Diseases》 2019年第7期467-488,共22页
Objectives: The impairment of right ventricular (RV) myocardial mechanics is evident in hypertrophic cardiomyopathy (HCM). It is independently influenced by LV mechanics and correlated to the severity of LV phenotype.... Objectives: The impairment of right ventricular (RV) myocardial mechanics is evident in hypertrophic cardiomyopathy (HCM). It is independently influenced by LV mechanics and correlated to the severity of LV phenotype. We investigated the changes in RV global and regional deformation following surgical septal myectomy using vector velocity imaging (VVI). Methods: 25 HCM patients, 68% males with mean age (34.5 ± 12 years) were examined before and within two months after surgical myectomy using VVI. In addition to conventional echocardiographic parameters, peak systolic strain (εsys), strain rate (SR) and time to peak εsys (TTP) of regional RV free wall (RVFW) & septal walls were analyzed in longitudinal (long) directions from apical four-chamber view and their (Δ) changes were calculated. Similar parameters were quantified in LV from apical 2 & 4 CH views. Intra-V-delay was defined as SD of TTP and inter-V dyssynchrony was estimated from TTP difference between the most delayed LV segment & RVFW. Results: All study patients showed improvement of their functional class from NYHA class III to class I and reduction of LVOT gradient to below 20 mmHg except one patient who had 30 mmHg gradients at rest. There was significant reduction of septal thickness, left atrial diameter & volume, LVOT gradient, LVMI, severity of mitral regurgitation, tricuspid annular velocities (P < 0.0001), RV diameter (P < 0.02) and increase in LV internal dimensions(P < 0.001) post myectomy. However, there was significant reduction of RV and LV systolic mechanics;RV global εsys % (from -16.1% ± 4.4% to -12.9% ± 2.9%, P < 0.0001) and LV global εsys %: from -11.6% ± 2.8% to -9.4% ± 2.2%, P < 0.0001) respectively. The magnitude of reduction of RV strain (ΔRV εsys %, ΔSRsys) was directly correlated LV maximal wall thickness (r = 0.46, P < 0.01) and ΔRV dyssynchrony (TTP-SD) (r = 0.4, P < 0.05) and negatively correlated to age (r = -0.46, P sys (r = -0.52, P < 0.01) and pre-op LV EF % (r = -0.43, P , the reduction in RV diastolic mechanics: ΔRV SRe & SRa were directly correlated to PAP and LVOT gradient before surgery (r = 0.62, P Conclusion: Despite the improvement of patient functional status and reduction LVOT gradient, RV mechanics shows further deterioration after surgical myectomy. The magnitude of reduction is modestly related to cardiac phenotype and pre-op mechanical function. 展开更多
关键词 RIGHT VENTRICLE Deformation Imaging hypertrophic cardiomyopathy
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Predicting apical hypertrophic cardiomyopathy using T-wave inversion:Three case reports 被引量:1
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作者 Liang Kang Yi-Hua Li +1 位作者 Rong Li Qing-Min Chu 《World Journal of Clinical Cases》 SCIE 2023年第25期5970-5976,共7页
BACKGROUND Apical hypertrophic cardiomyopathy(AHCM)is a subtype of hypertrophic cardiomyopathy.Due to its location,the thickening of the left ventricular apex can be missed on echocardiography.Giant negative T waves(G... BACKGROUND Apical hypertrophic cardiomyopathy(AHCM)is a subtype of hypertrophic cardiomyopathy.Due to its location,the thickening of the left ventricular apex can be missed on echocardiography.Giant negative T waves(GNTs)in left-sided chest leads are the hallmark electrocardiogram(ECG)change of AHCM.CASE SUMMARY The first patient was a 68-year-old woman complaining of recurrent chest tightness persisting for more than 3 years.The second was a 59-year-old man complaining of spasmodic chest tightness persisting for more than 2 years.The third was a 55-year-old woman complaining of recurrent chest pain persisting for 4 mo.In all three cases,GNTs were observed several years prior to apical cardiac hypertrophy after other causes of T-wave inversion were ruled out.CONCLUSION Electrophysiological abnormalities of AHCM appear earlier than structural abnormalities,confirming the early predictive value of ECG for AHCM. 展开更多
关键词 ELECTROCARDIOGRAM Negative T waves hypertrophic cardiomyopathy Apical hypertrophic cardiomyopathy ECHOCARDIOGRAPHY Case report
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Clinical outcome of cardiac resynchronization therapy in dilated-phase hypertrophic cardiomyopathy 被引量:1
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作者 Min GU Han JIN +7 位作者 Wei HUA Xiao-Han FAN Hong-Xia NIU Tao TIAN Li-Gang DING Jing WANG Cong XUE Shu ZHANG 《Journal of Geriatric Cardiology》 SCIE CAS CSCD 2017年第4期238-244,共7页
Backgrounds Clinical trials have demonstrated that cardiac resynchronization therapy (CRT) is effective in patients with "non-is- chemic cardiomyopathy". However, patients with dilated-phase hypertrophic cardiomyo... Backgrounds Clinical trials have demonstrated that cardiac resynchronization therapy (CRT) is effective in patients with "non-is- chemic cardiomyopathy". However, patients with dilated-phase hypertrophic cardiomyopathy (DHCM) have been generally excluded from such trials. We aimed to compare the clinical outcome of CRT in patients with DHCM, idiopathic dilated cardiomyopathy (IDCM), or ischemic cardiomyopathy (ICM). Methods A total of 312 consecutive patients (DHCM: n = 16; IDCM: n = 231; ICM: n = 65) undergoing CRT in Fuwai hospital were studied respectively. Response to CRT was defmed as reduction in left ventricular end-systolic volume (LVESV) _〉 15% at 6-month follow-up. Results Compared with DHCM, IDCM was associated with a lower total mortality (HR: 0.35, 95% CI: 0.13-0.90), cardiac mortality (HR: 0.29; 95% CI: 0.11-0.77), and total mortality or heart failure (HF) hospitalizations (HR: 0.34, 95% CI: 0.17-0.69), independent of known confounders. Compared with DHCM, the total mortality, cardiac mortality and total mortality or HF hospitalizations favored ICM but were not statistically significant (HR: 0.59, 95% CI: 0.22-1.61; HR: 0.59, 95% CI: 0.21-1.63; HR: 0.54, 95% CI: 0.26-1.15; respectively). Response rate to CRT was lower in the DHCM group than the other two groups although the differences didn't reach statistical significance. Conclusions Compared with IDCM, DHCM was associated with a worse outcome after CRT. The clinical outcome of DHCM patients receiving CRT was similar to or even worse than that of ICM patients. These indicate that DHCM behaves very differently after CRT. 展开更多
关键词 Cardiac resynchronization therapy Dilated-phase hypertrophic cardiomyopathy Idiopathic dilated cardiomyopathy Ischemic cardiomyopathy
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Specific alterations of regional myocardial work in strength-trained athletes using anabolic androgenic steroids compared to athletes with genetic hypertrophic cardiomyopathy
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作者 Antoine Grandperrin Frédéric Schnell +4 位作者 Erwan Donal Elena Galli Christophe Hedon Olivier Cazorla Stéphane Nottin 《Journal of Sport and Health Science》 SCIE CAS CSCD 2023年第4期477-485,共9页
Background:Strength-trained athletes using anabolic androgenic steroids(AAS)have left ventricular(LV)hypertrophy and myocardial fibrosis that can lead to sudden cardiac death.A similar feature was described in athlete... Background:Strength-trained athletes using anabolic androgenic steroids(AAS)have left ventricular(LV)hypertrophy and myocardial fibrosis that can lead to sudden cardiac death.A similar feature was described in athletes with hypertrophic cardiomyopathy(HCM),which complicates the diagnosis for clinicians.In this context,we aimed to compare the LV function of the 2 populations by measuring global and regional strain and myocardial work using speckle-tracking imaging.Methods:Twenty-four strength-trained asymptomatic athletes using AAS(AAS-Athletes),22 athletes diagnosed with HCM(HCM-Athletes),and 20 healthy control athletes(Ctrl-Athletes)underwent a resting echocardiography to assess LV function.We evaluated LV global and regional strains and myocardial work,with an evaluation of the constructive work(CW),wasted work,and work efficiency(WE).Results:Compared to Ctrl-Athletes,both AAS-Athletes and HCM-Athletes had a thicker interventricular septum,with maj ored values in HCM-Athletes.LV strain was reduced in AAS-Athletes and even more in HCM-Athletes.Consequently,global WE was significantly diminished in both AAS and HCM-Athletes(93%±2%in Ctrl-Athletes,90%±4%in AAS-Athletes,and 90%±5%in HCM-Athletes(mean±SD);p<0.05).Constructive work and WE regional analysis showed specific alterations,with the basal septal segments preferentially affected in AAS-Athletes,and both septal and apical segments affected in HCM-Athletes.Conclusion:The regional evaluation of myocardial work reported specific alterations of the major LV hypertrophy induced by the regular use of AAS compared to the LV hypertrophy due to HCM.This finding could help clinicians to differentiate between these 2 forms of pathological hypertrophy. 展开更多
关键词 Anabolic androgenic steroids hypertrophic cardiomyopathy Left ventricular function Myocardial work Speckle-tracking echocardiography
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Bicuspid aortic valve with associated aortopathy, significant left ventricular hypertrophy or concomitant hypertrophic cardiomyopathy: A diagnostic and therapeutic challenge
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作者 Ivana Sopek Merkaš Nenad Lakušić +2 位作者 Mladen Predrijevac KrešimirŠtambuk Maja Hrabak Paar 《World Journal of Clinical Cases》 SCIE 2023年第18期4251-4257,共7页
Due to its prevalence of 0.5%to 2%in the general population,with a 75%predominance among men,bicuspid aortic valve is the most common congenital heart defect.It is frequently accompanied by other cardiac congenital an... Due to its prevalence of 0.5%to 2%in the general population,with a 75%predominance among men,bicuspid aortic valve is the most common congenital heart defect.It is frequently accompanied by other cardiac congenital anomalies,and clinical presentation can vary significantly,with stenosis being the most common manifestation,often resulting in mild to moderate concentric hypertrophy of the left ventricle.Echocardiography is the primary diagnostic modality utilized for establishing the diagnosis,and it is often the sole diagnostic tool relied upon by clinicians.However,due to the heterogeneous clinical presentation and possible associated anomalies(which are often overlooked in clinical practice),it is necessary to employ various diagnostic methods and persist in finding the accurate diagnosis if multiple inconsistencies exist.By employing this approach,we can effectively manage these patients and provide them with appropriate treatment.Through a clinical case from our practice,we provide an overview of the literature on bicuspid aortic valve with aortophaty and the possible association with hypertrophic cardiomyopathy,diagnostic methods,and treatment options.This review article highlights the critical significance of achieving an accurate diagnosis in patients with bicuspid aortic valve and significant left ventricular hypertrophy.It is crucial to exclude other possible causes of left ventricular outflow tract obstruction,such as sub-or supra-aortic obstructions,and hypertrophic cardiomyopathy. 展开更多
关键词 Bicuspid aortic valve Left ventricular hypertrophy Left ventricular tract obstruction hypertrophic cardiomyopathy Aortopathy Multimodal imaging
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Mitral Valve Abnormalities during Hypertrophic Cardiomyopathy: Study on a Cohort in Dakar
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作者 Simon Antoine Sarr Hicham Fassi-Fehri +12 位作者 Marguerite Tening Diouf Youssou Diouf Fatou Aw Joseph Salvador Mingou Khadimu Rassoul Diop Serigne Mor Beye Aliou Alassane Ngaidé Malick Bodian Mouhamadou Bamba Ndiaye Alassane Mbaye Adama Kane Maboury Diao Abdoul Kane 《World Journal of Cardiovascular Diseases》 2023年第11期710-717,共8页
INTRODUCTION: Mitral valve abnormalities in hypertrophic cardiomyopathy (HCM) are becoming increasingly well defined, and their role in intra-ventricular obstruction is well defined. The aim of this study was to ... INTRODUCTION: Mitral valve abnormalities in hypertrophic cardiomyopathy (HCM) are becoming increasingly well defined, and their role in intra-ventricular obstruction is well defined. The aim of this study was to evaluate mitral valve abnormalities in patients with HCM. PATIENTS AND METHODS: We conducted a descriptive cross-sectional study from May 1 to July 1, 2022 in the Cardiology Department of Aristide Le Dantec Hospital. All patients with HCM aged at least 18 years old were included. The parameters studied concerned mainly the mitral valvular apparatus (papillary muscles abnormalities, leaflet length, mitral insufficiency). RESULTS: A total of 10 patients were included. Mean age was 58.3. On Doppler echocardiography, mean interventricular septal thickness was 20.6 mm. The mean maximum intra-ventricular gradient was 21.06 mmHg. Two patients had significant intraventricular obstruction. The mean length of the anterior mitral valve leaflet was 28.7 ± 3.55 mm, with extremes of 22 and 33 mm. The posterior mitral leaflet averaged 14.8 ± 3.16 mm. Nine (9) out of 10 patients had an elongated anterior valve leaflet. Elongation of the posterior leaflet was noted in 6 patients. With regard to papillary muscle position, 6 patients had an anterolateral ascending pillary muscle. These patients had a mean intra-ventricular gradient of 25 mmHg, compared with 16.5 mmHg in the others cases. We found no direct insertion on the mitral valve. Mitral insufficiency was noted in 9 patients, including 5 with mild insufficiency and 4 with moderate one. CONCLUSION: Mitral valve abnormalities in HCM appear to be frequent. They should be analyzed for a better diagnostic and therapeutic approach. 展开更多
关键词 hypertrophic cardiomyopathy Mitral Valve OBSTRUCTION
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Myocardial Perfusion Abnormalities in Patients with Hereditary Hypertrophic Cardiomyopathy: A Study of Three Cases and Review of the Literature
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作者 Ngardjibem Djita Ibrahima Sory Sylla +10 位作者 Mamadou Bassirou Bah Felix Collard Sana Samoura Mamadou Aliou Baldé Souleymane Diakité Ibrahima Sory Barry Alpha Koné Elhadj Yaya Baldé Mariame Béavogui Mamadou Dadhi Baldé Philippe Chevalier 《World Journal of Cardiovascular Diseases》 2023年第6期289-296,共8页
Introduction: Hypertrophic cardiomyopathy (HCM) belongs to the very heterogeneous group of cardiomyopathies. This study aimed to study myocardial perfusion abnormalities on scintigraphy and assess the risk of sudden d... Introduction: Hypertrophic cardiomyopathy (HCM) belongs to the very heterogeneous group of cardiomyopathies. This study aimed to study myocardial perfusion abnormalities on scintigraphy and assess the risk of sudden death from ventricular arrhythmia in hereditary sarcomeric HCM. Patients and Methods: This is a retrospective and prospective descriptive study over 18 months (January 01, 2021, to July 31, 2022) on the records of patients over 18 with the diagnosis of hypertrophic sarcomeric cardiomyopathy with or without ventricular rhythm disorders and who have undergone myocardial scintigraphy. Results: Three patients were included. The average age of our patients was 66 years old. Dyspnea is the primary symptomatology found in our patients. One patient presented with syncope and unsustained ventricular tachycardia. His risk score for sudden death from ESC at five years is estimated at 6.45%, and the patient received an ICD in primary prevention. The average sudden death risk score of our patients was 3.78%. The mean LV wall thickness was 20 mm. The mean maximum left intraventricular gradient was 39 mmHg. Myocardial fibrosis was present in all our patients. Myocardial scintigraphy was normal in all cases. Conclusion: Hypertrophic cardiomyopathy is a very heterogeneous group of cardiomyopathies. The rhythmic risk is multifactorial and constitutes a significant prognostic factor. 展开更多
关键词 hypertrophic cardiomyopathy Myocardial Scintigraphy Sudden Death Ven-tricular Arrhythmia
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Multimodality imaging in apical hypertrophic cardiomyopathy 被引量:12
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作者 Rosario Parisi Francesca Mirabella +1 位作者 Gioel Gabrio Secco Rossella Fattori 《World Journal of Cardiology》 CAS 2014年第9期916-923,共8页
Apical hypertrophic cardiomyopathy(AHCM) is a relatively rare morphologic variant of HCM in which the hypertrophy of myocardium is localized to the left ventricular apex. Symptoms of AHCM might vary from none to other... Apical hypertrophic cardiomyopathy(AHCM) is a relatively rare morphologic variant of HCM in which the hypertrophy of myocardium is localized to the left ventricular apex. Symptoms of AHCM might vary from none to others mimic coronary artery disease including acute coronary syndrome, thus resulting in inappropriate hospitalization. Transthoracic echocardiography is the firstline imaging technique for the diagnosis of hypertrophic cardiomyopathies. However, when the hypertrophy of the myocardium is localized in the ventricular apex might results in missed diagnosis. Aim of this paper is to review the different imaging techniques used for the diagnosis of AHCM and their role in the detection and comprehension of this uncommon disease. 展开更多
关键词 Apical hypertrophic cardiomyopathy Imaging techniques Cardiac magnetic resonance Trans-thoracic echocardiography Multidetector computed tomography
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Speckle tracking echocardiography to assess regional ventricular function in patients with apical hypertrophic cardiomyopathy 被引量:8
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作者 María Cristina Saccheri Tomás Francisco Cianciulli +7 位作者 Luis Alberto Morita Ricardo JoséMéndez Martín Alejandro Beck Juan Enrique Guerra Alberto Cozzarin Luciana Jimena Puente Lorena Romina Balletti Jorge Alberto Lax 《World Journal of Cardiology》 CAS 2017年第4期363-370,共8页
AIM To explore regional systolic strain of midwall and endocardial segments using speckle tracking echocardiography in patients with apical hypertrophic cardiomyopathy(HCM).METHODS We prospectively assessed 20 patient... AIM To explore regional systolic strain of midwall and endocardial segments using speckle tracking echocardiography in patients with apical hypertrophic cardiomyopathy(HCM).METHODS We prospectively assessed 20 patients(mean age 53 ± 16 years,range:18-81 years,10 were male),with apical HCM. We measured global longitudinal peak systolic strain(GLPSS) in the midwall and endocardium of the left ventricle. RESULTS The diastolic thickness of the 4 apical segments was 16.25 ± 2.75 mm. All patients had a normal global systolicfunction with a fractional shortening of 50% ± 8%. In spite of supernormal left ventricular(LV) systolic function,midwall GLPSS was decreased in all patients,more in the apical(-7.3% ±-8.8%) than in basal segments(-15.5% ±-6.93%),while endocardial GLPPS was significantly greater and reached normal values(apical:-22.8% ±-7.8%,basal:-17.9% ±-7.5%). CONCLUSION This study shows that two-dimensional strain was decreased mainly confined to the mesocardium,while endocardium myocardial deformation was preserved in HCM and allowed to identify subclinical LV dysfunction. This transmural heterogeneity in systolic strain had not been previously described in HCM and could be explained by the distribution of myofibrillar disarray in deep myocardial areas. The clinical application of this novel finding may help further understanding of the pathophysiology of HCM. 展开更多
关键词 Apical hypertrophic cardiomyopathy Twodimensional strain Speckle tracking ENDOCARDIUM Midwall Regional myocardial systolic function
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Age-related Changes in Familial Hypertrophic Cardiomyopathy Phenotype in Transgenic Mice and Humans 被引量:1
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作者 罗鸿昌 Iraklis Pozios +3 位作者 Styliani Vakrou Lars Sorensen Roselle M.Abraham Theodore Abraham 《Journal of Huazhong University of Science and Technology(Medical Sciences)》 SCIE CAS 2014年第5期634-639,共6页
β-myosin heavy chain mutations are the most frequently identified basis for hypertrophic cardiomyopathy (HCM). A transgenic mouse model (αMHC403) has been extensively used to study various mechanistic aspects of... β-myosin heavy chain mutations are the most frequently identified basis for hypertrophic cardiomyopathy (HCM). A transgenic mouse model (αMHC403) has been extensively used to study various mechanistic aspects of HCM. There is general skepticism whether mouse and human disease features are similar. Herein we compare morphologie and functional characteristics, and disease evolu- tion, in a transgenic mouse and a single family with a MHC mutation. Ten male αWHC403 transgenic mice (at -5 weeks, -12 weeks, and -24 weeks) and 10 HCM patients from the same family with a β-myosin heavy chain mutation were enrolled. Morphometric, conventional echocardiographic, tissue Doppler and strain analytic characteristics of transgenic mice and HCM patients were assessed. Ten male transgenic mice (αMHC403) were examined at ages -5 weeks, -12 weeks, and -24 Weeks. In the transgenic mice, aging was associated with a significant increase in septal (0.59±0.06 vs. 0.64±-0.05 vs. 0.69±0.11 mm, P〈0.01) and anterior wall thickness (0.58±0.1 vs. 0.62±0.07 vs. 0.80-1-0.16 mm, P〈0.001), which was coincident with a significant decrease in circumferential strain (-22%=1=4% vs. -20%-4-3% vs. -19%-4-3%, P=0.03), global longitudinal strain (-19%-4-3% vs. -17%-4-2% vs. -16%±3%, P=0.001) and E/A ratio (1.9±0.3 vs. 1.7-4-0.3 vs. 1.4-4-0.3, P=0.01). The HCM patients were classified into 1st generation (n=6; mean age 534-6 years), and 2nd generation (n=4; mean age 32+8 years). Septal thickness (2.2±0.9 vs. 1.4±0.1 cm, P〈0.05), left atrial (LA) volume (62±16 vs. 41±5 mL, P=0.03), E/A ratio (0.77±0.21 vs. 1.1±0.1, P=0.01), E/e' ratio (25±10 vs. 12±2, P=0.03), global left ventricular (LV) strain (-14%±3% vs. -20%±3%, P=0.01) and global LV early diastolic strain rate (0.76±0.17 s1 vs. 1.3±0.2 s-1, P=0.01) were significantly worse in the older generation. In β-myosin heavy chain muta- tions, transgenic mice and humans have similar progression in morphologic and functional abnormali- ties. The αMHC4±3 transgenic mouse model closely recapitulates human disease. 展开更多
关键词 familial hypertrophic cardiomyopathy transgenic mice AGE-RELATED
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Hypertrophic cardiomyopathy and left ventricular non-compaction:Distinct diseases or variant phenotypes of a single condition?
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作者 Natalia Przytuła Ewa Dziewięcka +3 位作者 Mateusz Winiarczyk Katarzyna Graczyk Agnieszka Stępień Paweł Rubiś 《World Journal of Cardiology》 2024年第9期496-501,共6页
Hypertrophic cardiomyopathy(HCM)is a genetically determined myocardial disease characterized by an increased thickness of the left ventricle(LV)wall that cannot be solely attributed to abnormal loading conditions.HCM ... Hypertrophic cardiomyopathy(HCM)is a genetically determined myocardial disease characterized by an increased thickness of the left ventricle(LV)wall that cannot be solely attributed to abnormal loading conditions.HCM may present with an intraventricular or LV outflow tract obstruction,diastolic dysfunction,myocardial fibrosis and/or ventricular arrhythmias.Differentiating HCM from other diseases associated with LV hypertrophy,such as hypertension,aortic stenosis,or LV non-compaction(LVNC),can at times be challenging.LVNC is defined by excessive LV trabeculation and deep recesses between trabeculae,often accompanied by increased LV myocardial mass.Previous studies indicate that the LVNC phenotype may be observed in up to 5%of the general population;however,in most cases,it is a benign finding with no impact on clinical outcomes.Nevertheless,LVNC can occasionally lead to LV systolic dysfunction,manifesting as a phenotype of dilated or non-dilated left ventricular cardiomyopathy,with an increased risk of thrombus formation and arterial embolism.In extreme cases,where LVNC is associated with a very thickened LV wall,it can even mimic HCM.There is growing evidence of an overlap between HCM and LVNC,including similar genetic mutations and clinical presentations.This raises the question of whether HCM and LVNC represent different phenotypes of the same disease or are,in fact,two distinct entities. 展开更多
关键词 Left ventricle hypertrabeculation hypertrophic cardiomyopathy Left ventricle non-compaction Left ventricle hypertrophy Left ventricle obstruction
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Ventricular fibrillation and sudden cardiac arrest in apical hypertrophic cardiomyopathy:Two case reports
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作者 Yae Min Park Albert Youngwoo Jang +3 位作者 Wook-Jin Chung Seung Hwan Han Christopher Semsarian In Suck Choi 《World Journal of Clinical Cases》 SCIE 2021年第35期11102-11107,共6页
BACKGROUND Apical hypertrophic cardiomyopathy(HCM)is considered to have a benign prognosis in terms of cardiovascular mortality.This serial case report aimed to raise awareness of ventricular fibrillation(VF)and sudde... BACKGROUND Apical hypertrophic cardiomyopathy(HCM)is considered to have a benign prognosis in terms of cardiovascular mortality.This serial case report aimed to raise awareness of ventricular fibrillation(VF)and sudden cardiac death(SCD)in apical HCM.CASE SUMMARY Here we describe two rare cases of apical HCM that presented with documented VF and sudden cardiac collapse.These patients were previously not recommended for primary prevention using implantable cardioverter-defibrillator(ICD)therapy based on current guidelines.However,both received ICD therapy for the secondary prevention of SCD.CONCLUSION These cases illustrate serious complications including VF and aborted sudden cardiac arrest in apical HCM patients who are initially not candidates for primary prevention using ICD implantation based on current guidelines. 展开更多
关键词 Apical hypertrophic cardiomyopathy Ventricular fibrillation Implantable cardioverter-defibrillator Case report
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Clinical Analysis of 42 Patients With Hypertrophic Cardiomyopathy
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作者 Kebbati Abdelhafid 伍卫 刘泽生 《South China Journal of Cardiology》 CAS 2007年第1期17-26,共10页
Objectives To investigate the clinical manifestations of hypertrophic cardiomyopathy (HCM), and to find out the clinical clues to avoid misdiagnosis and provide reference for future clinical diagnosis and treatment. M... Objectives To investigate the clinical manifestations of hypertrophic cardiomyopathy (HCM), and to find out the clinical clues to avoid misdiagnosis and provide reference for future clinical diagnosis and treatment. Methods A retrospective analysis of 42 consecutive patients with HCM hospitalized in our hospital between January 1995 and December 2002 was explored. Based on the family history of HCM, clinical manifestations, electrocar-diogram, echocardiogram, coronary angiography and left ventriculography, the clinical characteristics between HCM patients with left ventricular outflow tract obstruction (HOCM) and HCM patients without obstruction (HNOCM) were compared. The causes of misdiagnosis and losing diagnosis were analysis. Results 13 patients were in HOCM group and 29 patients were in HNOCM group. More patients with syncope were in HOCM group than in HNOCM group (6/13 vs. 2/29, P < 0.05). Patients with ejective murmur were in HOCM group only (P < 0.01). Left ventricular outflow tract pressure gradient (LVOTPG) only observed in HOCM group (P < 0.01). Ventricular tachycardia was seen in both groups. 28 out of 42 patients (66.67%) had misdiagnosis, and 4 out of 42 patients (9.53%) had losing diagnosis. Thus, coronary heart disease (CHD) had the highest rate of misdiagnosis. There were 20 CHD patients (71.43%) among 28 patients with misdiagnosis. Hypertension was in 3, congenital heart in 2, cerebro-embolism in 2, and myocarditis in 1. Conclusions For a patient with family history or sudden death history of HCM, unexplained syncope episodes, chest pain (angina), especially in young, an ejection murmur along the left sternum border, the presence of narrow and deep Q waves, or inversion of giant T waves in V3-V6, atrial fibrillation and /or cerebra-embolism echocardiogram should be given. CAG and LVG are necessary only if the result of echo is negative, and the patients with suspected HCM or CHD. 展开更多
关键词 cardiomyopathy hypertrophic Analysis Retrospective Clinical
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Role of genetic screening in a chinese woman of childbearing age with hypertrophic cardiomyopathy
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作者 Pedro Brugada Ramon Brugada 《岭南心血管病杂志》 2011年第S1期213-213,共1页
Objectives The objective of this study was to evaluate the role of genetic screening in a Chinese woman of childbearing age with hypertrophic cardiomyopathy.Methods and Results One 39 year-old woman with presyncope fo... Objectives The objective of this study was to evaluate the role of genetic screening in a Chinese woman of childbearing age with hypertrophic cardiomyopathy.Methods and Results One 39 year-old woman with presyncope for 10 years was admitted.Both of her father and her son died of sudden death and she strongly desire to get another baby. A series of clinical and laboratory studies were performed. Hypertrophic cardiomyopathy was diagnosed finally and implantable Cardioverter Defibrillator was implanted to prevent sudden cardiac death for her.Genomic DNA was isolated and the most common causal genes for hypertrophic cardiomyopathy were screened.A known pathogenetic heterozygous mutation c.700 g】a(p.Argl86Gln) in TNNI3 was found,which was not found in 100 normal control individuals matched for age,sex and geographical region.Because 50%probability of the pathogenetic mutation is inherited to the offsprings,she will get a healthy baby in vitro fertilization which the egg comes from a healthy female donor to prevent from the inherited HCM.Conclusions We found a pathogenetic TNNI3 mutation in a Chinese woman of childbearing age with hypertrophic cardiomyopathy.The genetic screening definite DNA-based diagnosis and help to design a healthy fertilization in vitro for female with hypertrophic cardiomyopathy. 展开更多
关键词 Role of genetic screening in a chinese woman of childbearing age with hypertrophic cardiomyopathy
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WJC 6~th Anniversary Special Issues(3):Cardiomyopathy Hypertrophic cardiomyopathy in 2013:Current speculations and future perspectives 被引量:9
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作者 Georgios K Efthimiadis Efstathios D Pagourelias +1 位作者 Thomas Gossios Thomas Zegkos 《World Journal of Cardiology》 CAS 2014年第2期26-37,共12页
Hypertrophic cardiomyopathy(HCM),the most variable cardiac disease in terms of phenotypic presentation and clinical outcome,represents the most common inherited cardiomyopathic process with an autosomal dominant trait... Hypertrophic cardiomyopathy(HCM),the most variable cardiac disease in terms of phenotypic presentation and clinical outcome,represents the most common inherited cardiomyopathic process with an autosomal dominant trait of inheritance.To date,more than 1400 mutations of myofilament proteins associated with the disease have been identified,most of them "private" ones.This striking allelic and locus heterogeneity of the disease certainly complicates the establishment of phenotype-genotype correlations.Additionally,topics pertaining to patients' everyday lives,such as sudden cardiac death(SCD)risk stratification and prevention,along with disease prognosis,are grossly related to the genetic variation of HCM.This review incorporates contemporary research findings and addresses major aspects of HCM,including preclinical diagnosis,genetic analysis,left ventricular outflow tract obstruction and SCD.More specifically,the spectrum of genetic analysis,the selection of the best method for obstruction alleviation and the need for a unique and accuratefactor for SCD risk stratification are only some of the controversial HCM issues discussed.Additionally,future perspectives concerning HCM and myocardial ischemia,as well as atrial fibrillation,are discussed.Rather than enumerating clinical studies and guidelines,challenging problems concerning the disease are critically appraised by this review,highlighting current speculations and recommending future directions. 展开更多
关键词 hypertrophic cardiomyopathy PRECLINICAL diagnosis Left ventricular OUTFLOW OBSTRUCTION Sudden cardiac death Genetic analysis
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