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
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
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
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
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
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
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
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
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
基金Supported by The Department of Scientific Research and Structural Funds of Medical College,Jagiellonian University,No.N41/DBS/000594.
文摘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.
文摘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.
文摘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.
文摘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.
文摘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.
文摘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.
基金The authors would like to acknowledge the research support from Natural Science Foundation of Fujian Province of China(Grant No.2017J01009)Fundamental Research Funds for the Central Universities(Grant No.20720180004)National Heart,Lung and Blood Institute grants R01 HL089269,and National Sciences Foundation of China(Grant No.11672001,81571691).
文摘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.
基金supported by the National Key Research and Development Plan of China (2020YFC2004700)the National Natural Science Foundation of China (No.81825003 & No.91957123)+1 种基金the CAMS Innovation Fund for Medical Sciences (CIFMS 2016I2M-1-009)the Beijing Municipal Commission of Science and Technology (Z171100000417021)。
文摘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.
文摘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.
