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
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
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
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
We describe a patient with asymptomatic apical hypertrophic cardiomyopathy(AHCM)who later developed cardiac arrhythmias,and briefly discuss the diagnostic modalities,differential diagnosis and treatment option for thi...We describe a patient with asymptomatic apical hypertrophic cardiomyopathy(AHCM)who later developed cardiac arrhythmias,and briefly discuss the diagnostic modalities,differential diagnosis and treatment option for this condition.AHCM is a rare form of hypertrophic cardiomyopathy which classically involves the apex of the left ventricle.AHCM can be an incidental finding,or patients may present with chest pain,palpitations,dyspnea,syncope,atrial fibrillation,myocardial infarction,embolic events,ventricular fibrillation and congestive heart failure.AHCM is frequently sporadic,but autosomal dominant inheritance has been reported in few families.The most frequent and classic electrocardiogram findings are giant negative T-waves in the precordial leads which are found in the majority of the patients followed by left ventricular(LV)hypertrophy.A transthoracic echocardiogram is the initial diagnostic tool in the evaluation of ACHM and shows hypertrophy of the LV apex.AHCM may mimic other conditions such as LV apical cardiac tumors,LV apical thrombus,isolated ventricular non-compaction,endomyocardial fibrosis and coronary artery disease.Other modalities,including left ventriculography,multislice spiral computed tomography,and cardiac magnetic resonance imagings are also valuable tools and are frequently used to differentiate AHCH from other conditions.Medications used to treat symptomatic patients with AHCM include verapamil,beta-blockers and antiarrhythmic agents such as amiodarone and procainamide.An implantable cardioverter defibrillator is recommended for high risk patients.展开更多
Hypertrophic cardiomyopathy(HCM) is the most common cause of sudden cardiac death(SCD) in the young, particularly among athletes. Identifying high risk individuals is very important for SCD prevention. The purpose of ...Hypertrophic cardiomyopathy(HCM) is the most common cause of sudden cardiac death(SCD) in the young, particularly among athletes. Identifying high risk individuals is very important for SCD prevention. The purpose of this review is to stress that noninvasive diagnostic testing is important for risk assessment. Extreme left ventricular hypertrophy and documented ventricular tachycardia and fibrillation increase the risk of SCD. Fragmented QRS and T wave inversion in multiple leads are more common in high risk patients. Cardiac magnetic resonance imaging provides complete visualization of the left ventricular chamber, allowing precise localization of the distribution of hypertrophy and measurement of wall thickness and cardiac mass. Moreover, with late gadolinium enhancement, patchy myocardial fibrosis within the area of hypertrophy can be detected, which is also helpful in risk stratification. Genetic testing is encouraged in all cases, especially in those with a family history of HCM and SCD.展开更多
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
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
The left ventricular regional systolic functions in patients with hypertrophic cardiomyopathy (HCM) were assessed by using quantitative tissue velocity imaging (QTVI). Left ventricular (LV) regional myocardial v...The left ventricular regional systolic functions in patients with hypertrophic cardiomyopathy (HCM) were assessed by using quantitative tissue velocity imaging (QTVI). Left ventricular (LV) regional myocardial velocity along long- and short-axis in 31 HCM patients and 20 healthy subjects were analyzed by QTVI, and the regional myocardial systolic peak velocities (MVS) were measured. Mean MVS at each level including mitral annular, basal, middle and apical segments were calculated. The ratio of MVS along long-axis to that along short-axis (Ri) at basal and middle segments of the LV posterior wall and ventricular septum were calculated. The results showed that mean MVS was slower at each level including mitral annular, basal, middle and apical segments in the HCM patients than that in the healthy subjects (P〈0.01). There were no significant differences in mean MVS between obstructive and non-obstructive groups in HCM patients. MVS of all regional myocardial segments along long-axis in the HCM patients were significantly slower than that in the healthy subjects (P〈0.05), but there was no significant difference in MVS of all regional myocardial segments along long-axis between hypertrophied and non-hypertrophied group in the HCM patients. Ri was significantly lower in the HCM patients than that in the healthy subjects. The LV regional myocardial contractility along long-axis was impaired not only in the hypertrophied wall but also in the non-hypertrophied one in patients with HCM, suggesting that QTVI can assess accurately LV regional systolic function in patient with HCM and provides a novel means for an early diagnosis before and independent of hypertrophy.展开更多
The value of tissue strain imaging (SI) in regional myocardial systolic and diastolic func tion assessment was studied. In 18 patients with nonobstructive hypertrophic cardiomyopathy (HCM) and 20 age-matched healt...The value of tissue strain imaging (SI) in regional myocardial systolic and diastolic func tion assessment was studied. In 18 patients with nonobstructive hypertrophic cardiomyopathy (HCM) and 20 age-matched healthy subjects, regional myocardial longitudinal peak systolic strain in eject time (represented by εet) was measured at basal, mid and apical segments of septal, lateral and posterior walls of the left ventricle (LV) and compared between groups, εet had no significant difference between segments in control group (P〉0.05), which displayed a decreasing trend from basal segments to apical ones. εet in the HCM group was significantly decreased (P(0. 05) as compared with that in the healthy group. In the HCM group, εet in the midseptum was significantly less than at the basal and apical septum, and was also less than at the rest LV walls in the same group (P〉0.01). The systolic reversed εet was noticed in 35% of the hypertrophic segments in HCM group. Significantly negative correlation existed between the absolute value of εet and wall thickness in the midseptum (r= -0.83). The post-systolic strain(PSS) segment number the and amplitudes in healthy group were significantly less than those in HCM group (P〈0.05). Both regional myocardial systolic and diastolic functions were impaired in hypertrophic or non-hypertrophic segments in patients with the HCM, especially in hypertrophic segments. Strain imaging technique is a sensitive and accura tool in myocardial dysfunction assessment.展开更多
Objective To determine the risk of noncardiac surgery in patients with hypertrophic cardiomyopathy.Methods We reviewed the medical records of all patients who were diagnosed as hypertrophic cardiomyopathy at Peking Un...Objective To determine the risk of noncardiac surgery in patients with hypertrophic cardiomyopathy.Methods We reviewed the medical records of all patients who were diagnosed as hypertrophic cardiomyopathy at Peking Union Medical College Hospital from January 1998 to August 2006 and identified 24 patients who subsequently underwent noncardiac surgery.Results There were no intraoperative cardiac events.Postoperative cardiac events were identified in 3 patients including 1 death due to acute myocardial infarction and 2 episodes of transient hypotension.Conclusions The risk of anesthesia and noncardiac surgery is low in patients with hypertrophic cardiomyopathy.During the perioperative period,beta-blockers and/or calcium channel blockers should be given;vasodilator and inotropic agents should be avoided due to the side effects on hemodynamics.展开更多
Background Atrial fibrillation(AF)is a generally acknowledged turning-point of the natural history of hypertrophic cardiomyopathy(HCM);however,data from the cryoballoon ablation(CBA)for AF in HCM patients are relative...Background Atrial fibrillation(AF)is a generally acknowledged turning-point of the natural history of hypertrophic cardiomyopathy(HCM);however,data from the cryoballoon ablation(CBA)for AF in HCM patients are relatively scarce.The study aimed to evaluate the efficacy and safety of CBA in HCM patients with AF.Methods We retrospectively analyzed HCM patients among 1253 patients with symptomatic AF who underwent CBA for pulmonary vein isolation in a single center.The study analyzed the AF recurrence and assessed the CBA indexes,including nadir temperature,time-to-isolation,CBA failure,pulmonary vein potentials(PVPs),and redo procedure.Results A total of 108 patients were included(mean age:59.0±6.9 years),27 patients(25%)had HCM,with the median follow-up duration of 25.5 months.The one-year AF-free rates were 79.0%vs.63.0%(non-HCM vs.HCM),while the two-year AF-free rates were 77.8%vs.55.1%[hazard ratio(HR)=2.758,log-rank P=0.024].Patients with persistent AF had poor AF-free rates compared to those with paroxysmal AF(P<0.001).The CBA failure was the most common in the right inferior pulmonary veins,which had the lowest PVPs.Multivariate Cox regression analysis indicated that HCM and persistent AF were risk factors for AF recurrence(HR=2.74,95%CI:1.29–5.79,P=0.008;and HR=3.97,95%CI:1.85–8.54,P<0.001,respectively).Conclusions The CBA can be effectively and safely used to treat HCM patients with symptomatic AF.The freedom from AF for HCM patients after CBA is relatively low compared to that for non-HCM patients.展开更多
Objective:Late gadolinium enhancement(LGE) patterns of cardiovascular magnetic resonance (CMR) relying on PSIR(phase-sensitive inversion recovery sequence) techniques had been used to determine the characteristics of ...Objective:Late gadolinium enhancement(LGE) patterns of cardiovascular magnetic resonance (CMR) relying on PSIR(phase-sensitive inversion recovery sequence) techniques had been used to determine the characteristics of LGE in apical hypertrophic cardiomyopathy(ApHCM). Methods:Forty patients pure ApHCM[age,(60.2±10.4) years,31 men]were enrolled.LGE images were acquired using PSIR,and analyzed using a 17-segment model.Summing the LGE areas in all short axis slices yielded the total volume of late enhancement,which was subsequently presented as a proportion of total LV myocardium(%LGE).Results:Mean maximal apical wall thickness was(17.9±2.3) mm,and mean left ventricular(LV) ejection fraction was(67.7±8.0)%.LGE was detected in 130 segments of 30 patients(75.0%),occupying(4.9±5.5)% of LV myocardium.LGE was mainly detected at the junction between left and right ventricles in 12(30%) and at the apex in 28(70%),although LGE-positive areas were widely distributed,and not limited to the apex.Focal LGE at the non-hypertrophic LV segments was found in some ApHCM patients,even without LGE of hypertrophied apical segments.Conclusions:LGE was frequently observed not only in the thickened apex of the heart but also in other LV segments,irrespective of the presence or absence of hypertrophy.The simple presence of LGE on CMR was not representative of adverse prognosis in this population.展开更多
Hypertrophic cardiomyopathy (HCM) is a common genetic cardiovascular disease that affects the left ventricle. HCM can appear at any age, with the majority of the patients remaining clinically stable. When patients com...Hypertrophic cardiomyopathy (HCM) is a common genetic cardiovascular disease that affects the left ventricle. HCM can appear at any age, with the majority of the patients remaining clinically stable. When patients complain of symptoms, these include: dyspnea, dizziness, syncope and angina. HCM can lead to sudden cardiac death (SCD), mainly due to ventricular tachyarrhythmia or ventricular tachycardia. High-risk patients benefit from implantable cardioverter-defibrillators. Left ventricular outflow tract obstruction is not a rare feature in HCM, especially in symptomatic patients, and procedures that abolish that obstruction provide positive and consistent results that can improve longterm survival. HCM is the most common cause of sudden death in young competitive athletes and preparticipation screening programs have to be implemented to avoid these tragic fatalities. The structure of these programs is a matter of large debate. Worldwide registries are necessary to identify the full extent of HCMrelated SCD.展开更多
To assess the left ventricular longitudinal regional myocardial systolic function by strain imaging (SI) echocardiography and to study the relationship between regional myocardial systolic function and left ventricu...To assess the left ventricular longitudinal regional myocardial systolic function by strain imaging (SI) echocardiography and to study the relationship between regional myocardial systolic function and left ventricular structure in patients with hypertrophic cardiomyopathy (HCM). S1 echocardiography were performed in 18 patients with HCM and 17 healthy subjects. For each wall, regional myocardial systolic strain was analyzed at the basal, mid, and apical level respectively. And the peak systolic strain was measured. Our results showed that the patients with HCM had reduced peak systolic strain at almost each segment of different walls when compared with healthy subjects. There was significant correlation between the mid-septum peak systolic strain and the thickness of IVS, so was the correlation between the mid-septum peak systolic strain and the IVS to LVPW thickness ratio. This study demonstrated that the left ventricular longitudinal regional myocardial systolic function was abnormal in HCM, and this kind of abnormalities existed extensively in hypertrophic and non-hypertrophic cardiac segments. The degrees of left ventricle hypertrophy and asymmetry are related to the myocardial regional systolic function in HCM.展开更多
We are reporting a long-time magnetic resonance imaging(MRI) follow-up in a rare case of cardiac left lateral wall hypertrophy. Hypertrophic cardiomyopathy(HCM) is the most common genetic cardiovascular disorder and a...We are reporting a long-time magnetic resonance imaging(MRI) follow-up in a rare case of cardiac left lateral wall hypertrophy. Hypertrophic cardiomyopathy(HCM) is the most common genetic cardiovascular disorder and a significant cause of sudden cardiac death. Cardiac magnetic resonance(CMR) imaging can be a valuable tool for assessment of detailed information on size,localization,and tissue characteristics of hypertrophied myocardium. However,there is still little knowledge of long-term evolution of HCM as visualized by magnetic resonance imaging. Recently,our group reported a case of left lateral wall HCM as a rare variant of the more common forms,such as septal HCM,or apical HCM. As we now retrieved an old cardiac MRI acquired in this patient more than 20 years ago,we are able to provide the thrilling experience of an ultra-long MRI followup presentation in this rare case of left lateral wall hypertrophy. Furthermore,this case outlines the tremendous improvements in imaging quality within the last two decades of CMR imaging.展开更多
To determine the clinical application of pulsed Doppler tissue imaging in assessing the left ventricular diasto-lic function and in discriminating between normal subjects and patients with hypertrophic cardiomyopathy ...To determine the clinical application of pulsed Doppler tissue imaging in assessing the left ventricular diasto-lic function and in discriminating between normal subjects and patients with hypertrophic cardiomyopathy with various stages of diastolic dysfunction. Methods We measured the peak diastolic velocities of mitral annulus in 81 patients with hypertrophic cardiomyopathy with various stages of diastolic dysfunction and 50 normal volunteers by Doppler tissue imaging using the apical window at 2-ch-amber and long apical views, respectively. The myocardial velocities were determined with use of variance F statistical analysis. Results Early diastolic myocardial velocities of mitral annulus were higher in normal subjects than in patients with hy-pertrophic cardiomyopathy with either delayed relaxation, pseudonormal filling, or restrictive filling. However, peak myocar-dial velocities of mitral annulus during atrial contraction were similar in normal subjects and patients with hypertrophic cardiomyopathy. Conclusion Doppler tissue imaging can directly reflect upon left diastolic ventricular function. Early phase of diastole was the best discriminator between control subjects and patients with hypertrophic cardiomyopathy.展开更多
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
Hypertrophic cardiomyopathy(HCM)is a type of hereditary cardiomyopathy caused by gene mutation.Its histological features include cardiomyocyte hypertrophy and disarray as well as myocardial fi brosis.Gene mutation,abn...Hypertrophic cardiomyopathy(HCM)is a type of hereditary cardiomyopathy caused by gene mutation.Its histological features include cardiomyocyte hypertrophy and disarray as well as myocardial fi brosis.Gene mutation,abnormal signal transduction,and abnormal energy metabolism are considered the main mechanisms of myocardial fi brosis.There is a strong correlation between myocardial fi brosis and the occurrence,development,and prognosis of HCM.We review the application of myocardial fi brosis in the diagnosis and treatment of HCM,focusing on research progress and the application of magnetic resonance imaging on the basis of the characteristics of fi brosis in the diagnosis and prognosis of HCM.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
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.
文摘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.
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.
文摘We describe a patient with asymptomatic apical hypertrophic cardiomyopathy(AHCM)who later developed cardiac arrhythmias,and briefly discuss the diagnostic modalities,differential diagnosis and treatment option for this condition.AHCM is a rare form of hypertrophic cardiomyopathy which classically involves the apex of the left ventricle.AHCM can be an incidental finding,or patients may present with chest pain,palpitations,dyspnea,syncope,atrial fibrillation,myocardial infarction,embolic events,ventricular fibrillation and congestive heart failure.AHCM is frequently sporadic,but autosomal dominant inheritance has been reported in few families.The most frequent and classic electrocardiogram findings are giant negative T-waves in the precordial leads which are found in the majority of the patients followed by left ventricular(LV)hypertrophy.A transthoracic echocardiogram is the initial diagnostic tool in the evaluation of ACHM and shows hypertrophy of the LV apex.AHCM may mimic other conditions such as LV apical cardiac tumors,LV apical thrombus,isolated ventricular non-compaction,endomyocardial fibrosis and coronary artery disease.Other modalities,including left ventriculography,multislice spiral computed tomography,and cardiac magnetic resonance imagings are also valuable tools and are frequently used to differentiate AHCH from other conditions.Medications used to treat symptomatic patients with AHCM include verapamil,beta-blockers and antiarrhythmic agents such as amiodarone and procainamide.An implantable cardioverter defibrillator is recommended for high risk patients.
文摘Hypertrophic cardiomyopathy(HCM) is the most common cause of sudden cardiac death(SCD) in the young, particularly among athletes. Identifying high risk individuals is very important for SCD prevention. The purpose of this review is to stress that noninvasive diagnostic testing is important for risk assessment. Extreme left ventricular hypertrophy and documented ventricular tachycardia and fibrillation increase the risk of SCD. Fragmented QRS and T wave inversion in multiple leads are more common in high risk patients. Cardiac magnetic resonance imaging provides complete visualization of the left ventricular chamber, allowing precise localization of the distribution of hypertrophy and measurement of wall thickness and cardiac mass. Moreover, with late gadolinium enhancement, patchy myocardial fibrosis within the area of hypertrophy can be detected, which is also helpful in risk stratification. Genetic testing is encouraged in all cases, especially in those with a family history of HCM and SCD.
文摘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.
文摘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.
文摘The left ventricular regional systolic functions in patients with hypertrophic cardiomyopathy (HCM) were assessed by using quantitative tissue velocity imaging (QTVI). Left ventricular (LV) regional myocardial velocity along long- and short-axis in 31 HCM patients and 20 healthy subjects were analyzed by QTVI, and the regional myocardial systolic peak velocities (MVS) were measured. Mean MVS at each level including mitral annular, basal, middle and apical segments were calculated. The ratio of MVS along long-axis to that along short-axis (Ri) at basal and middle segments of the LV posterior wall and ventricular septum were calculated. The results showed that mean MVS was slower at each level including mitral annular, basal, middle and apical segments in the HCM patients than that in the healthy subjects (P〈0.01). There were no significant differences in mean MVS between obstructive and non-obstructive groups in HCM patients. MVS of all regional myocardial segments along long-axis in the HCM patients were significantly slower than that in the healthy subjects (P〈0.05), but there was no significant difference in MVS of all regional myocardial segments along long-axis between hypertrophied and non-hypertrophied group in the HCM patients. Ri was significantly lower in the HCM patients than that in the healthy subjects. The LV regional myocardial contractility along long-axis was impaired not only in the hypertrophied wall but also in the non-hypertrophied one in patients with HCM, suggesting that QTVI can assess accurately LV regional systolic function in patient with HCM and provides a novel means for an early diagnosis before and independent of hypertrophy.
文摘The value of tissue strain imaging (SI) in regional myocardial systolic and diastolic func tion assessment was studied. In 18 patients with nonobstructive hypertrophic cardiomyopathy (HCM) and 20 age-matched healthy subjects, regional myocardial longitudinal peak systolic strain in eject time (represented by εet) was measured at basal, mid and apical segments of septal, lateral and posterior walls of the left ventricle (LV) and compared between groups, εet had no significant difference between segments in control group (P〉0.05), which displayed a decreasing trend from basal segments to apical ones. εet in the HCM group was significantly decreased (P(0. 05) as compared with that in the healthy group. In the HCM group, εet in the midseptum was significantly less than at the basal and apical septum, and was also less than at the rest LV walls in the same group (P〉0.01). The systolic reversed εet was noticed in 35% of the hypertrophic segments in HCM group. Significantly negative correlation existed between the absolute value of εet and wall thickness in the midseptum (r= -0.83). The post-systolic strain(PSS) segment number the and amplitudes in healthy group were significantly less than those in HCM group (P〈0.05). Both regional myocardial systolic and diastolic functions were impaired in hypertrophic or non-hypertrophic segments in patients with the HCM, especially in hypertrophic segments. Strain imaging technique is a sensitive and accura tool in myocardial dysfunction assessment.
文摘Objective To determine the risk of noncardiac surgery in patients with hypertrophic cardiomyopathy.Methods We reviewed the medical records of all patients who were diagnosed as hypertrophic cardiomyopathy at Peking Union Medical College Hospital from January 1998 to August 2006 and identified 24 patients who subsequently underwent noncardiac surgery.Results There were no intraoperative cardiac events.Postoperative cardiac events were identified in 3 patients including 1 death due to acute myocardial infarction and 2 episodes of transient hypotension.Conclusions The risk of anesthesia and noncardiac surgery is low in patients with hypertrophic cardiomyopathy.During the perioperative period,beta-blockers and/or calcium channel blockers should be given;vasodilator and inotropic agents should be avoided due to the side effects on hemodynamics.
基金supported by the National Natural Science Foundation of China(No.81670309)。
文摘Background Atrial fibrillation(AF)is a generally acknowledged turning-point of the natural history of hypertrophic cardiomyopathy(HCM);however,data from the cryoballoon ablation(CBA)for AF in HCM patients are relatively scarce.The study aimed to evaluate the efficacy and safety of CBA in HCM patients with AF.Methods We retrospectively analyzed HCM patients among 1253 patients with symptomatic AF who underwent CBA for pulmonary vein isolation in a single center.The study analyzed the AF recurrence and assessed the CBA indexes,including nadir temperature,time-to-isolation,CBA failure,pulmonary vein potentials(PVPs),and redo procedure.Results A total of 108 patients were included(mean age:59.0±6.9 years),27 patients(25%)had HCM,with the median follow-up duration of 25.5 months.The one-year AF-free rates were 79.0%vs.63.0%(non-HCM vs.HCM),while the two-year AF-free rates were 77.8%vs.55.1%[hazard ratio(HR)=2.758,log-rank P=0.024].Patients with persistent AF had poor AF-free rates compared to those with paroxysmal AF(P<0.001).The CBA failure was the most common in the right inferior pulmonary veins,which had the lowest PVPs.Multivariate Cox regression analysis indicated that HCM and persistent AF were risk factors for AF recurrence(HR=2.74,95%CI:1.29–5.79,P=0.008;and HR=3.97,95%CI:1.85–8.54,P<0.001,respectively).Conclusions The CBA can be effectively and safely used to treat HCM patients with symptomatic AF.The freedom from AF for HCM patients after CBA is relatively low compared to that for non-HCM patients.
基金Supported by Haikou Key Science and Technology Project (2012-075)Haikou Science and Technology Planning Project(2009-049-1)Science and Technology Fund of Haikou Health Bureau(2010-SWY-13-058)
文摘Objective:Late gadolinium enhancement(LGE) patterns of cardiovascular magnetic resonance (CMR) relying on PSIR(phase-sensitive inversion recovery sequence) techniques had been used to determine the characteristics of LGE in apical hypertrophic cardiomyopathy(ApHCM). Methods:Forty patients pure ApHCM[age,(60.2±10.4) years,31 men]were enrolled.LGE images were acquired using PSIR,and analyzed using a 17-segment model.Summing the LGE areas in all short axis slices yielded the total volume of late enhancement,which was subsequently presented as a proportion of total LV myocardium(%LGE).Results:Mean maximal apical wall thickness was(17.9±2.3) mm,and mean left ventricular(LV) ejection fraction was(67.7±8.0)%.LGE was detected in 130 segments of 30 patients(75.0%),occupying(4.9±5.5)% of LV myocardium.LGE was mainly detected at the junction between left and right ventricles in 12(30%) and at the apex in 28(70%),although LGE-positive areas were widely distributed,and not limited to the apex.Focal LGE at the non-hypertrophic LV segments was found in some ApHCM patients,even without LGE of hypertrophied apical segments.Conclusions:LGE was frequently observed not only in the thickened apex of the heart but also in other LV segments,irrespective of the presence or absence of hypertrophy.The simple presence of LGE on CMR was not representative of adverse prognosis in this population.
文摘Hypertrophic cardiomyopathy (HCM) is a common genetic cardiovascular disease that affects the left ventricle. HCM can appear at any age, with the majority of the patients remaining clinically stable. When patients complain of symptoms, these include: dyspnea, dizziness, syncope and angina. HCM can lead to sudden cardiac death (SCD), mainly due to ventricular tachyarrhythmia or ventricular tachycardia. High-risk patients benefit from implantable cardioverter-defibrillators. Left ventricular outflow tract obstruction is not a rare feature in HCM, especially in symptomatic patients, and procedures that abolish that obstruction provide positive and consistent results that can improve longterm survival. HCM is the most common cause of sudden death in young competitive athletes and preparticipation screening programs have to be implemented to avoid these tragic fatalities. The structure of these programs is a matter of large debate. Worldwide registries are necessary to identify the full extent of HCMrelated SCD.
文摘To assess the left ventricular longitudinal regional myocardial systolic function by strain imaging (SI) echocardiography and to study the relationship between regional myocardial systolic function and left ventricular structure in patients with hypertrophic cardiomyopathy (HCM). S1 echocardiography were performed in 18 patients with HCM and 17 healthy subjects. For each wall, regional myocardial systolic strain was analyzed at the basal, mid, and apical level respectively. And the peak systolic strain was measured. Our results showed that the patients with HCM had reduced peak systolic strain at almost each segment of different walls when compared with healthy subjects. There was significant correlation between the mid-septum peak systolic strain and the thickness of IVS, so was the correlation between the mid-septum peak systolic strain and the IVS to LVPW thickness ratio. This study demonstrated that the left ventricular longitudinal regional myocardial systolic function was abnormal in HCM, and this kind of abnormalities existed extensively in hypertrophic and non-hypertrophic cardiac segments. The degrees of left ventricle hypertrophy and asymmetry are related to the myocardial regional systolic function in HCM.
基金Supported by The German Research Foundation(DFG)the University of Wuerzburg in the funding programme Open Access Publishing
文摘We are reporting a long-time magnetic resonance imaging(MRI) follow-up in a rare case of cardiac left lateral wall hypertrophy. Hypertrophic cardiomyopathy(HCM) is the most common genetic cardiovascular disorder and a significant cause of sudden cardiac death. Cardiac magnetic resonance(CMR) imaging can be a valuable tool for assessment of detailed information on size,localization,and tissue characteristics of hypertrophied myocardium. However,there is still little knowledge of long-term evolution of HCM as visualized by magnetic resonance imaging. Recently,our group reported a case of left lateral wall HCM as a rare variant of the more common forms,such as septal HCM,or apical HCM. As we now retrieved an old cardiac MRI acquired in this patient more than 20 years ago,we are able to provide the thrilling experience of an ultra-long MRI followup presentation in this rare case of left lateral wall hypertrophy. Furthermore,this case outlines the tremendous improvements in imaging quality within the last two decades of CMR imaging.
文摘To determine the clinical application of pulsed Doppler tissue imaging in assessing the left ventricular diasto-lic function and in discriminating between normal subjects and patients with hypertrophic cardiomyopathy with various stages of diastolic dysfunction. Methods We measured the peak diastolic velocities of mitral annulus in 81 patients with hypertrophic cardiomyopathy with various stages of diastolic dysfunction and 50 normal volunteers by Doppler tissue imaging using the apical window at 2-ch-amber and long apical views, respectively. The myocardial velocities were determined with use of variance F statistical analysis. Results Early diastolic myocardial velocities of mitral annulus were higher in normal subjects than in patients with hy-pertrophic cardiomyopathy with either delayed relaxation, pseudonormal filling, or restrictive filling. However, peak myocar-dial velocities of mitral annulus during atrial contraction were similar in normal subjects and patients with hypertrophic cardiomyopathy. Conclusion Doppler tissue imaging can directly reflect upon left diastolic ventricular function. Early phase of diastole was the best discriminator between control subjects and patients with hypertrophic cardiomyopathy.
文摘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.
文摘Hypertrophic cardiomyopathy(HCM)is a type of hereditary cardiomyopathy caused by gene mutation.Its histological features include cardiomyocyte hypertrophy and disarray as well as myocardial fi brosis.Gene mutation,abnormal signal transduction,and abnormal energy metabolism are considered the main mechanisms of myocardial fi brosis.There is a strong correlation between myocardial fi brosis and the occurrence,development,and prognosis of HCM.We review the application of myocardial fi brosis in the diagnosis and treatment of HCM,focusing on research progress and the application of magnetic resonance imaging on the basis of the characteristics of fi brosis in the diagnosis and prognosis of HCM.
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.