Advances in the treatment of hypertrophic cardiomyopathy with cardiac myosin inhibitors / 中国药房

In recent years， with the increasing understanding of the genetic mechanisms of hypertrophic cardiomyopathy， novel molecular-targeted drugs Mavacamten and Aficamten are two cardiac myosin inhibitors currently approved by the FDA for the treatment of hypertrophic obstructive cardiomyopathy. Both of them have a similar mechanism of action and can selectively bind to different variable sites of cardiac myosin to inhibit cardiac myosin， thus reducing myocardial hypercontractility. Relevant clinical studies have also shown that both drugs can reduce patients’ left ventricular outflow tract pressure gradient， the levels of N-terminal pro-B-type natriuretic peptide and cardiac troponin I as cardiac markers， and improve New York Heart Association （NYHA） cardiac function class. They are safe， have mild adverse reactions， and can be tolerated by patients. Compared to Mavacamten， Aficamten， as a structurally optimized product， has a shorter half-life and fewer drug-drug interactions， which is more conducive to drug- targeted dose titration.

Anesthetic management of an adult patient with hypertrophic obstructive cardiomyopathy and chronic kidney disease for minimally invasive video-assisted thoracoscopic surgery

It is not an uncommon situation to encounter a patient with anesthesia having multiple co-morbidities. Here, we report the successful anesthetic management of an immunosuppressed 53-year-old female patient with hypertrophic obstructive cardiomyopathy, stage IV chronic kidney disease, and chronic lung infection who was posted for minimally invasive video-assisted thoracoscopy requiring one lung ventilation. Intraoperative hemodynamic stability was maintained with etomidate, fentanyl, cisatracurium, desflurane, and dexmedetomidine with accurate bispectral depth for sedation and precise fluid guidance with transesophageal echocardiography. The entire anesthetic conduct was planned to avoid the left ventricular outflow tract obstruction and maintain a steady-state hemodynamic balance. This case report is a learning experience of how close vigilance with appropriate use of monitoring and knowledge about disease per se resulted in an uneventful perioperative period.

Evaluation of fluid field of left ventricle outflow tract in hypertrophic obstructive cardiomyopathy with fluid models based on three-dimensional echocardiography with inverted grey values / 中华超声影像学杂志

Objective:To explore the effect of systolic anterior motion (SAM) of mitral valves on the morphology and function of left ventricular outflow tract (LVOT) in patients with hypertrophic obstructive cardiomyopathy (HOCM) using computer fluid dynamics based on three-dimensional echocardiography with inverted grey values.Methods:A total of 40 patients with hypertrophic cardiomyopathy were divided into SAM group (24 cases) and non SAM group (16 cases) in Renmin Hospital of Wuhan University from April 2016 to October 2019. Two dimensional and three-dimensional echocardiographic data of the patients were collected. The LVOT morphological model was constructed based on the post-processing of three-dimensional echocardiography data, and the LVOT flow field model was constructed based on the time-volume curve of left ventricle. LVOT peak velocity was obtained to assess the agreement with echocardiography measurements. Area of LVOT, average velocity, flow rate and iso-surface area of vortex of different levels were obtained and compared between the two groups.Results:There was a good correlation between cardiac fluid model and echocardiographic measurement ( r=0.943, P<0.01). The Bland-Altman consistency interval was -75.0-111.3, and 92.5% of the points were within the consistency limit. Compared with non-SAM group patients, the peak velocity of LVOT increased, the area of LVOT decreased, the flow rate decreased and the area of vortex increased in SAM patients (all P<0.01). In the SAM group, in 16 patients the double orifice LVOT was observed due to the contact between mitral valve and septum, in 1 patient the single orifice LVOT structure was observed with contact between mitral value and septum, and in 7 patients, single orifice LVOT without contact between mitral value and septum. In SAM patients, compared with single orifice LVOT, patients with double orifice LVOT were observed with higher LVOT velocity, smaller LVOT area and higher vortex area with high level(all P<0.05). Conclusions:Accurate fluid models can be obtained using three-dimensional echocardiography with inverted grey values. In SAM patients, contact between mitral valve and septum leads to the formation of double orifice structure and the increase of vortex level in LVOT.

Application of percutaneous transluminal septal branch microsphere embolization in the treatment of hypertrophic obstructive cardiomyopathy / 中华急诊医学杂志

Objective:To compare the clinical efficacy and safety of percutaneous transluminal septal branch anhydrous alcohol ablation (PTSAAA) and percutaneous transluminal septal branch microsphere embolization (PTSBME) in the treatment of patients with symptomatic hypertrophic obstructive cardiomyopathy.Methods:The clinical data of 55 patients with symptomatic hypertrophic obstructive cardiomyopathy treated by PTSAAA and PTSBME were retrospectively analyzed, among whom 27 were treated with PTSAAA and 28 with PTSBME. The changes of postoperative indicators of the two groups of patients were compared, including the improvement degree of symptoms [shortness of breath after activity (cardiac function NYHA classification), chest tightness, chest pain (angina CCS classification) and amaurosis, the decrease of left ventricular outflow tract pressure gradient (LVOTPG)], the ventricular septum thickness shown by color Doppler echocardiography, the incidences of complications at postoperative month 6 and 12, and the incidences of cardiovascular events at follow-up month 12. LSD- t, χ 2 or Fisher exact probability methods were used to compare the differences of indicators between the two groups. Results:Compared to the relative indicators before operation, there were significant differences in shortness of breath after activity, chest pain and amaurosis, LVOTPG, ventricular septum thickness, the incidences of complications at postoperative month 6 and 12 and the incidences of cardiovascular events at follow-up month 12 in both the PTSAAA group and PTSBME group ( P<0.05). The PTSBME group was not inferior to the PTSAAA group in the improvement degree of amaurosis, cardiac function NYHA classification and angina CCS classification and left ventricular ejection fraction (LVEF) at postoperative month 6 and 12 ( P>0.05) as well as in the LVOTPG decrease and the ventricular septum thickness at postoperative month 6 [(16.8±7.5) mmHg vs (15.8±7.3) mmHg, (19.8±4.9) mm vs (17.4±4.1) mm, P>0.05], but was superior to the PTSAAA group in the LVOTPG decrease and the ventricular septum thickness at postoperative month 12 [(15.2±6.7) mmHg vs (9.8±5.4) mmHg, (18.4±5.1) mm vs (12.2±3.2) mm, P<0.05]. There were statistical significances in the incidences of cardiovascular events and third degree atrio-ventricular block and nosocomial mortality between the two groups (6 vs 1; 5 vs 0, P<0.05), and the PTSBME group was superior to the PTSAAA group in safety. Conclusion:PTSBME may be a safe and effective method for the management of patients with symptomatic hypertrophic obstructive cardiomyopathy.

Risk factors and predictive value of estimated glomerular filtration rate for new-onset atrial fibrillation in patients with hypertrophic obstructive cardiomyopathy after modified extended Morrow procedure / 中国胸心血管外科临床杂志

@#Objective    To explore the association between preoperative, perioperative parameters, especially estimated glomerular filtration rate (eGFR) and postoperative atrial fibrillation (POAF) after modified extended Morrow procedure. Methods    A total of 300 hypertrophic obstructive cardiomyopathy (HOCM) patients who underwent modified extended Morrow procedure in our hospital from January 2012 to March 2018 were collected. There were 197 (65.67%) males and 103 (34.33%) females with an average age of 43.54±13.81 years. Heart rhythm was continuously monitored during hospitalization. The patients were divided into a POAF group (n=68) and a non-POAF group (n=232). The general data, perioperative parameters and echocardiographic results were collected by consulting medical records for statistical analysis. Univariate and multivariate logistic regression models were used to analyze the risk factors for POAF. Results    Overall incidence of POAF during hospitalization was 22.67% (68/300). Compared with patients without POAF, patients with POAF were older, had higher incidence of chest pain and syncope, lower level of preoperative eGFR, higher body mass index and heart function classification (NYHA), larger preoperative left atrial diameter and left ventricular end diastolic diameter, and longer ventilator-assisted time, ICU stay and postoperative hospital stay. Age, heart function classification (NYHA)≥Ⅲ, hypertension, syncope history and eGFR were independent risk factors for POAF. Receiver operating characteristic curve analysis showed that the area under the curve of eGFR was 0.731 (95%CI 0.677-0.780, P<0.001), and the sensitivity and specificity were 82.4% and 57.8%, respectively. Conclusion    Increased age, high preoperative heart function classification (NYHA), hypertension, preoperative syncope history and decreased eGFR are independent risk factors for POAF in HOCM patients who underwent surgical septal myectomy. Preoperative decreased eGFR can moderately predict the occurrence of POAF after modified extended Morrow procedure.

Totally endoscopic transmitral myectomy and traditional thoracotomy for hypertrophic obstructive cardiomyopathy: A propensity score matching analysis / 中国胸心血管外科临床杂志

@#Objective    To investigate the effectiveness and safety of totally endoscopic transmitral myectomy (TETM) for hypertrophic obstructive cardiomyopathy (HOCM), comparing with traditional sternotomy modified Morrow procedure (SMMP). Methods    Thirty-eight patients with HOCM who needed surgical intervention were selected from our hospital in 2019, including 14 males and 24 females, with an average age of 56 (44-68) years. According to the operation method, they were divided into a TETM group (n=18) and a SMMP group (n=20). Appropriate patients  were screened by propensity matching scores. Finally, the clinical data of two matched groups were compared and

Ablación septal con alcohol como tratamiento en la miocardiopatía hipertrófica obstructiva. Reporte de caso / Septal alcohol ablation as a treatment for hypertrophic obstructive cardiomyopathy. Case Report

RESUMEN La miocardiopatía hipertrófica obstructiva, es una patología de orden genético, cuyo evento más grave es la muerte súbita. El diagnóstico se basa en los hallazgos imagenológicos, constatándose un ventrículo izquierdo hipertrófico no dilatado, en ausencia de otras patologías que puedan explicar dichas alteraciones. El tratamiento médico tiene como objetivo mejorar la discapacidad funcional y el llenado diastólico, aquellos pacientes que continúan sintomáticos pese al tratamiento farmacológico, con obstrucción del tracto de salida del ventrículo izquierdo significativo, la miomectomía septal es de elección, no obstante, la ablación septal con alcohol es una alternativa a la cirugía. Se presenta una paciente de 39 años con síncope e insuficiencia cardíaca, con diagnóstico confirmado por imágenes de miocardiopatía hipertrófica asimétrica obstructiva, en quien persisten los síntomas a pesar del tratamiento médico, por lo que se realizó la ablación septal con alcohol, obteniendo resultados satisfactorios. La miocardiopatía hipertrófica obstructiva ha sido considerada como una patología poco frecuente, no obstante, en la actualidad es cada vez más diagnosticada, gracias al mayor conocimiento de su expresión fenotípica. La ablación septal con alcohol ha demostrado ser una opción segura y eficaz en nuestro medio, constituyendo una alternativa razonable de tratamiento alternativo a la cirugía, en pacientes seleccionados.

ABSTRACT Hypertrophic cardiomyopathy is a genetic disease, the most serious event of which is sudden death. Diagnosis is based on imaging findings, noting a hypertrophic undilated left ventricle, in the absence of other pathologies that may explain these alterations. The objective of the medical treatment is to improve functional disability and diastolic filling. In those patients who continue to be symptomatic despite drug treatment, with significant left ventricular outflow tract obstruction, septal myomectomy is recommended; however, septal alcohol ablation is an alternative to surgery. We present a 39-year-old patient with syncope and heart failure, with an image-confirmed diagnosis of hypertrophic obstructive cardiomyopathy, in whom symptoms persisted despite medical treatment, for which septal alcohol ablation was performed, obtaining satisfactory results. Hypertrophic obstructive cardiomyopathy has been considered a rare pathology, however, it is currently being diagnosed more and more, thanks to the greater knowledge of its phenotypic expression. Septal alcohol ablation has proven to be a safe and effective option in our setting, constituting a reasonable alternative treatment to surgery in selected patients.

Hypertrophic obstructive cardiomyopathy and its outcome following surgical myectomy: a retrospective study

Background: Hypertrophic cardiomyopathy is highly heterogeneous with a diverse anatomy, pathophysiology, and clinical course. It is obstruction to left ventricular outflow that has become the major hallmark of the disease. Septal myectomy has been the gold standard treatment for the relief of left ventricular outflow tract obstruction and cardiac symptoms in both adults and children with obstructive hypertrophic cardiomyopathy. Objective of the study was to evaluate effect of Myomectomy and its impact on survival for a period of one year.Methods: The study design is a retrospective record based observational study. Data was retrieved from previous records both electronic as well as manual records of all the patients who underwent myectomy with or without concomitant procedures such as mitral valve replacement or aortic valve replacement or coronary artery bypass surgery during 2014 to 2018.Results: Majority of the patients 11 (52.4%) in fourth decade i.e. 40-59 years age group. majority were males i.e. 16 (76.2%) and remaining 5 i.e. 23.8% were females. Male to female ratio was 3.2:1. Dyspnoea was present 81% and chest pain in 76.2%. Preoperative LVOT gradient was 86.86±20.33 and post-operative gradient was 23.47±20.49.Conclusions: Operative techniques have evolved from simple myotomy to the present method of extended septal myectomy which can be done in all adult cases of hypertrophic obstructive cardiomyopathy.

Analysis of surgical result of Cox-maze Ⅳ in the treatment of hypertrophic obstructive cardiomyopathy with persistent atrial fibrillation / 中国胸心血管外科临床杂志

@#Objective    To evaluate the efficacy and safety of modified maze Ⅳ (Cox-maze Ⅳ) in hypertrophic obstructive cardiomyopathy (HOCM) patients. Methods    From June 2016 to June 2019, 30 HOCM and persistent atrial fibrillation (pAF) patients received Cox-maze Ⅳ operation with modified extended Morrow operation, including 21 males and 9 females. The average age was 51.36±10.27 years and the average weight was 72.48±11.29 kg. All patients underwent left atrial appendectomy. Recurrence of AF, improvement of symptoms, cardiac function (NYHA) were assessed during follow-up. Results    There was no death during the perioperative period. Postoperative left ventricular outflow tract gradient was significantly decreased compared with that before operation (P<0.01), and all systolic anterior motion (SAM) signs disappeared after operation. Thirty patients were all effectively followed up for 3-40 (16.24±8.26) months. During the follow-up period, there was no death, and the cardiac function (NYHA) of all patients recovered to gradeⅠ-Ⅱ. At the end of follow-up, twenty-four patients (80.00%) maintained sinus rhythm, and twenty-seven patients (90.00%) maintained sinus rhythm after amiodarone conversion. Univariate analysis showed that the smoking history (P=0.04), left atrial diameter≥55 mm before operation (P=0.03), left atrial diameter≥50 mm after operation (P=0.02), postoperative tricuspid regurgitation (P=0.02) were closely related to postoperative AF recurrence. The increase of left atrial diameter after operation was an independent risk factor for AF recurrence (P=0.02). Conclusion    Morrow/Cox-maze Ⅳ procedure is safe and effective in treatment of patients with HOCM complicated with pAF, which helps to maintain postoperative sinus rhythm, and to improve the cardiac function. The increase of left atrial diameter after operation is an independent risk factor for AF recurrence.

Surgical treatment for hypertrophic obstructive cardiomyopathy with moderate-to-severe mitral regurgitation through right mini-thoracotomy / 中国胸心血管外科临床杂志

@#Objective    To explore the effect and safety of surgical treatment for hypertrophic obstructive cardiomyopathy (HOCM) with mitral regurgitation (MR) through right mini-thoracotomy. Methods    From January 2008 to June 2018, 54 patients with HOCM and moderate-to-severe MR underwent modified Morrow procedure and edge-to-edge mitral valvuloplasty through right mini-thoracotomy, including 31 males and 23 females, with an average age of 47.1±12.6 years. All patients had systolic anterior motion (SAM) phenomenon. Preoperative left ventricular outflow tract pressure gradient (LVOTPG) was 93.6±32.8 mm Hg, interventricular septum thickness (IVST) was 24.8±2.8 mm. Results    Surgeries in all patients were completed successfully. No early death or interventricular septal perforation occurred. One (1.9%) patient received permanent pacemaker implantation due to the complete atrial-ventricular block. At discharge, postoperative LVOTPG (18.1±6.2 mm Hg) and IVST (14.5±2.1 mm) were significantly decreased compared with the preoperative values (P<0.05). No MR or SAM was observed in all patients. The follow-up time was 6-132 months, and during this period, no death, MR or SAM occurred. The average LVOTPG was 19.4±5.7 mm Hg, and the average IVST was 14.2±1.5 mm. Conclusion    Morrow procedure and edge-to-edge mitral valvuloplasty through right mini-thoracotomy is a safe and effective method for treatment of HOCM with moderate-to-severe MR.

Result of surgical treatment of hypertrophic obstructive cardiomyopathy with coronary heart disease / 中国胸心血管外科临床杂志

@#Objective To summarize the perioperative management strategies and early results of modified Morrow expanded operation and coronary artery bypass grafting (CABG) in patients with hypertrophic obstructive cardiomyopathy (HOCM) and coronary atherosclerotic heart disease. Methods Between January 2012 and December 2017, in the Second Inpatient Department of Fuwai Hospital, 32 patients (20 females and 12 males) underwent modified expanded Morrow operation and CABG. The median age was 53.7±8.7 years (interquartile range 37 to 67 years). Preoperative chest distress symptom was found in 24 patients, chest pain symptom was found in 14 patients, history of syncope in 6 patients. Cardiac echocardiography, electrocardiogram, chest X-ray, magnectic resonance imaging (MRI) were performed routinely after operation and follow-up to analyze structure and function of heart and mitral valve. Results All patients underwent modified and expanded Morrow combined with CABG. The preoperative left ventricular outflow tract peak pressure difference (LVOTG) was 40 to 152 (79.6±28.7) mm Hg. Four patients underwent myocardial bridge releasing in the same period, mitral valve replacement in 2 patients, mitral valve angioplasty in 3 patients, Maze operation in 2 patients and tricuspid valveoplasty in 3 patients. There was no hospital mortality. CABG surgery in patients with branches included anterior descending artery in 26 patients, diagonal branch in 16 patients, left circumflex in 8 patients, right coronary artery in 11 patients. There were 15 patients with one coronary artery (CA) bypass graft, 5 patients with two CA bypass grafts, and 12 patients with 3 CA bypass grafts. The average of CA bypass grafts was 1.9±0.6. The postoperative ICU time ranged from 1–13 (4.1±2.8) days and postoperative hospital stay ranged from 7 to 30 (12.6±5.5) days. No severe postoperative complications were found and 1 patient had postoperative incision healing. The postoperative new arrhythmia included left bundle branch block in 6 patients. Compared with the preoperative values, postoperative left ventricular outflow tract peak pressure (79.6±28.7 mm Hg vs. 10.8±5.9 mm Hg, P<0.001), interventricular septum thickness (1.9±0.4 cmvs. 1.3±0.5 cm, P<0.001) were decreased obviously. Mitral valve closure is good or only mild reflux, mitral valve forward movement (SAM sign) disappeared. The patients were followed up for 6-68 months, with an average of 38.8±20.6 months. All patients were followed up with symptoms disappeared or only mild symptoms. NYHA classification decreased Ⅰ to Ⅱ grade after surgery, without long-term mortality, complications or reoperation. Conclusion For patients with hypertrophic obstructive cardiomyopathy with coronary atherosclerotic heart disease, the application of improved expand morrow operation at the same time undergoing coronary artery bypass grafting is safe. It can significantly improve patients' survival and reduce symptoms, play a synergistic effect, and do not increase the patient's surgical complications.

Modified surgery for hypertrophic obstructive cardiomyopathy with concomitantly significant mitral regurgitation through a single transaortic approach / 中华胸心血管外科杂志

Objective@#To summarize the safety and effect of modified surgery for hypertrophic obstructive cardiomyopathy(HOCM) with concomitantly significant mitral regurgitation(MR) through a single transaortic approach.@*Methods@#From January 2008 to June 2018, 93 patients with HOCM and significant MR underwent modified Morrow procedure and edge-to-edge mitral valvuloplasty through a single transaortic approach. Preoperative left ventricular outflow tract pressure gradient(LVOTPG) was 51-199 mmHg(1 mmHg=0.133 kPa). Preoperative interventricular septum thickness(IVST) was 17-30 mm. All patients had significant MR with SAM phenomenon. The modified Morrow procedure and edge-to-edge mitral valvuloplasty through a single transaortic approach was performed under cardiopulmonary bypass and aortic crossclamp.@*Results@#All patients successfully underwent the surgery of modified Morrow procedure and edge-to-edge mitral valvuloplasty through a single transaortic approach. No early death and interventricular septal perforation were occurred. In the early postoperative period, two patient(2.15%) received permanent pacemaker implantation due to the complete atrial-ventricular block. At discharge, postoperative LVOTPG(7-31 mmHg) and IVST(11-19 mm) were significantly decreased compared with the preoperative values(P<0.05). All patients had none or trivial MR. The mitral valve pressure gradient(MVPG) was 0-6 mmHg. SAM phenomenon disappeared in all patients. At a mean follow-up of(40.53±27.11) months, no patient had significant residual left ventricular outflow tract obstruction. All patients had none or trivial MR. No SAM phenomenon occurred.@*Conclusion@#Modified surgery of Morrow procedure and edge-to-edge mitral valvuloplasty through a single transaortic approach could be safely and effectively applied for patients with HOCM and concomitantly significant MR.

Modified surgery for hypertrophic obstructive cardiomyopathy with concomitantly significant mitral regurgitation through a single transaortic approach / 中华胸心血管外科杂志

Objective To summarize the safety and effect of modified surgery for hypertrophic obstructive cardiomyopathy ( HOCM) with concomitantly significant mitral regurgitation( MR) through a single transaortic approach. Methods From Jan-uary 2008 to June 2018, 93 patients with HOCM and significant MR underwent modified Morrow procedure and edge-to-edge mitral valvuloplasty through a single transaortic approach. Preoperative left ventricular outflow tract pressure gradient( LVOT-PG) was 51-199 mmHg(1 mmHg=0. 133 kPa). Preoperative interventricular septum thickness(IVST) was 17 -30 mm. All patients had significant MR with SAM phenomenon. The modified Morrow procedure and edge-to-edge mitral valvuloplasty through a single transaortic approach was performed under cardiopulmonary bypass and aortic crossclamp. Results All patients successfully underwent the surgery of modified Morrow procedure and edge-to-edge mitral valvuloplasty through a single transaortic approach. No early death and interventricular septal perforation were occurred. In the early postoperative period, two patient(2. 15％) received permanent pacemaker implantation due to the complete atrial-ventricular block. At discharge, postoperative LVOTPG(7-31 mmHg) and IVST(11 -19 mm) were significantly decreased compared with the preoperative values(P<0. 05). All patients had none or trivial MR. The mitral valve pressure gradient(MVPG) was 0-6 mmHg. SAM phenomenon disappeared in all patients. At a mean follow-up of(40. 53 ± 27. 11) months, no patient had significant residual left ventricular outflow tract obstruction. All patients had none or trivial MR. No SAM phenomenon occurred. Conclusion Modified surgery of Morrow procedure and edge-to-edge mitral valvuloplasty through a single transaortic approach could be safely and effectively applied for patients with HOCM and concomitantly significant MR.

Surgical treatment for hypertrophic cardiomyopathy in children / 中国实用儿科杂志

Hypertrophic cardiomyopathy(HCM)is the second commonest form of heart muscle disease affecting children and adolescents and is a leading cause of sudden death in young athletes. The aetiology of HCM is heterogeneous in the paediatric population,and includes inborn errors of metabolism,neuromuscular disorders and malformation syndromes.However,most cases of apparently idiopathic HCM in childhood are caused by mutations in cardiac sarcomere protein genes. Hypertrophic obstructive cardiomyopathy(HOCM) is not uncommon in children who don't respond to drug treatment;for such children,surgical treatment is the only solution.Previous routine surgical methods include modified Konno operation,modified extended Morrow operation and interventional alcohol ablation or radiofrequency ablation for pediatric patients. At present,the reports and large-scale clinical experience are rare.

A single-center comparative study of alcohol septal ablation(SA) and surgery myectomy(SM) for refractory hyper-trophic obstructive cardiomyopathy( HOCM) / 中华胸心血管外科杂志

Objective Septal reduction therapies were recommended for drug-refractory patients with hypertrophic ob-structive cardiomyopathy(HOCM).To explore and compare the effectiveness and safety in patients with hypertrophic obstruc-tive cardiomyopathy(HOCM) treated with surgery myectomy(SM) and alcohol septal ablation(SA).Methods The clinical data of 260 patients performed SA(n=184) or SM(n=76)between September 2002 and September 2014 in our institute were retrospectively reviewed.The t-test, rank sum test and chi-square test were used to compare the differences between the two groups, and the Cox regression model was used for multivariate survival analysis.All-cause mortality, cardiac cause death(peri-operative death were included ) , heart function improvement , procedure-related complications and permanent pacemaker de-pendence( PPM) constituted the main contents of this study .Results Compared with patients treated with SM , patients un-dergone SA were poor heart function status(2.97 ±0.29 vs 2.50 ±0.56, P =0.01), more prevalence of atrial fibrillation( 15.14％ vs 6.80％, P=0.046) and longer follow-up period[(5.4 ±3.8) years vs(2.5 ±2.2) years, P =0.01)].All-cause mortality for SA and SM were 3.3％ and 14.5％ respectively(P=0.001).The fatal cardiac events of SA and SM were 1.63％ and 13.16％ respectively(P<0.001).Sudden cardiac arrest were the main cardiac cause death for both patients trea-ted with SA and SM.The cardiac death of left ventricular systolic dysfunction was main found in patients treated with SM . Heart function improvement(NYHA) after SA and SM were 1.23 ±0.61 and 0.88 ±0.64 respectively(all P <0.01).And SA had a lower procedure-related PPM implantation(1.63％ vs 4.20％, P<0.05).Conclusion Our results shown that SA have survival advantage, lower PPM and similar heart function improvement compared with SM for refractory patients with HOCM.

Surgical treatment for hypertrophic obstructive cardiomyopathy associated with aortic stenosis / 中国胸心血管外科临床杂志

@#Objective     To evaluate the clinical and follow-up results of the surgical treatment for hypertrophic obstructive cardiomyopathy associated with aortic stenosis. Methods     We retrospectively analyzed the clinical data of the patients with hypertrophic obstructive cardiomyopathy plus aortic stenosis in our hospital from February 2008 to October 2015. There were 4 males and 3 females aged 55.6 ± 7.5 years. All the patients were received concomitant aortic valvulopasty at the time of modified extended Morrow procedure. Echocardiographic data and major complications were recorded through the outpatient clinic and telephone. Results     The postoperative ventricular septal thickness, left ventricular outflow tract gradient and aortic gradient were significantly lower than those in preoperation with statistical differences (P<0.05). During the mean follow-up 25.6 ± 28.2 months period, 1 patient died of cerebral hemorrhage, 1 patient was implanted a permanent pacemaker, and 1 patient had a postoperative new-onset atrial fibrillation. All patients had a satisfied prosthetic valve function and the left ventricular outflow tract gradient. The patient's symptoms and heart function significantly improved postoperatively. Conclusion     For patients with hypertrophic obstructive cardiomyopathy associated with moderate to severe aortic stenosis, concomitant aortic valvulopasty at the time of modified extended Morrow procedure is an appropriate and effective treatment, which can significantly alleviate the clinical symptoms, and improve quality of life with a satisfied prognosis.

Surgical treatment for hypertrophic obstructive cardiomyopathy complcated with mitral regurgitation abstract / 中华胸心血管外科杂志

Objective Investigate the therapeutic strategies of hypertrophic obstructive cardiomyopathy ( HOCM ) com-bined with mitral regurgitation(MR).Methods From January 2014 to January 2017, 34 patients with HOCM complicated with moderate to severe MR were enrolled.All patients underwent modified Morrow surgery.Compare the clinical data of pa-tients before and after surgery and the results of one year after surgery .Results There is no patient died during hospitaliza-tion, and all were discharged smoothly without serious complications ( ventricular septal perforation, complete atrioventricular block, etc.) .Results of echocardiography 1 week after surgery suggested: Left ventricular outflow tract pressure difference [(93.36 ±7.93) mmHg vs.(16.73 ±2.02) mmHg,1 mmHg=0.133 kPa, P<0.01], left ventricular outflow tract velocity[(472.40 ±22.12)cm/s vs.(188.40 ±14.16)cm/s, P<0.01], interventricular septal thickness [(19.43 ±0.77) mm vs.(16.45 ±0.76) mm, P<0.01], mitral valve structure and function were good, and MR area [(8.41 ±0.69)cm2 vs. (3.04 ±0.73)cm2, P<0.01], all of which were significantly lower than that before surgery, and the differences were statis-tically significant.Although the results of echocardiography 1 week after surgery indicated that the left ventricular ejection frac-tion(LVEF) was significantly lower than that before surgery(0.67 ±0.07 vs.0.65 ±0.07, P=0.01), the symptoms of the patients were significantly improved, and the cardiac function(NYHA classification) was grade I～II.The results of echocar-diography after 1 year of follow-up suggested that: Left ventricular outflow tract pressure difference [(93.36 ±7.93) mmHg vs.(16.98 ±2.33) mmHg, P<0.01], left ventricular outflow tract velocity [(472.40 ±22.12)cm/s vs.(189.33 ±14.23) cm/s, P<0.01], ventricular septal thickness [(19.43 ±0.77) mm vs.(16.55 ±0.83) mm, P <0.01], mitral valve structure and function well, MR area [(8.41 ±0.69) cm2 vs.(2.95 ±0.66) cm2, P<0.01], and the MR area was signifi-cantly decreased compared with that before operation .The difference was statistically significant .Results of echocardiography 1 week and 1 year after surgery suggest:Left ventricular outflow tract pressure difference [(16.73 ±2.02) mmHg vs.(16.98 ± 2.33) mmHg, P>0.05], left ventricular outflow tract velocity [(188.40 ±14.16)cm/s vs.(189.33 ±14.23)cm/s, P>0.05], ventricular septal thickness [(16.45 ±0.76) mm vs.(16.55 ±0.83) mm, P>0.05], MR area [(3.04 ±0.73) cm2 vs.(2.95 ±0.66) cm2, P>0.05], no statistical significance.One year after the operation, the symptoms and quality of life were significantly improved .Conclusion Hypertrophic obstructive cardiomyopathy often combined with mitral regurgita-tion, modified Morrow operation can fully clear the left ventricular outflow tract, which can eliminate MR and SAM signs, and the results are satisfactory.

Tips for Successful Septal Myectomy in Patients with Hypertrophic Cardiomyopathy

Septal myectomy is the gold-standard treatment of hypertrophic cardiomyopathy. However, it involves the risk of incomplete resection of septal muscles or iatrogenic septal perforation depending on the surgeon's practice. Herein, we suggest a few tips for successful septal myectomy and present a relevant case.

Clinical Efficacy and Outcome of Modified Extended Morrow Procedure in Children With Hypertrophic Obstructive Cardiomyopathy / 中国循环杂志

Objectives: Modified extended Morrow procedure for treatment of children with hypertrophic obstructive cardiomyopathy (HOCM) is a complicated and challenging procedure. Our study sought to assess the effect and outcome of modified extended Morrow procedure in children with HOCM. Methods: From January 2010 to July 2017, 50 consecutive pediatric (age≤14 years) patients with HOCM underwent transaortic modified extended Morrow procedure in Fuwai Hospital. Clinical data of these patients were analyzed retrospectively. Pre-operative and post-operative echocardiography data were analyzed, including left atrial size, left ventricular end diastolic diameter, left ventricular ejection fraction, left ventricular outflow tract peak pressure, ventricular septal thickness, mitral systolic anterior motion and grade of mitral regurgitation. Results: Mean age at the time of operation was (84.4±57.7) month (6 month -14 year). Mean body weight at the time of surgery was (28.4±20.7) kg (4.3-92.0) kg. There were 2 deaths after the operation. Three patients had postoperative complete heart block and received permanent pacemaker implantation. After myectomy, left ventricular outflow tract gradient decreased from (74.8±25.0) mmHg to (19.6±17.3) mmHg (P<0.001); the postoperative thickness of ventricular septum decreased from (21.2±9.7) mm to (14.3±6.7) mm (P<0.001); mitral regurgitation degree reduced from 2.2±1.0 to 0.67±0.72, P<0.001). Concomitant surgical procedures were required in 22 patients (44.0%). There was no late death during a follow-up of (27.7±14.0) months. Patients' symptoms were significantly improved post operation and all surviving patients were in New York Heart Association functional class I or II during follow-up. Conclusions: Modified extended Morrow procedure is safe and effective in children with HOCM, post-procedural clinical outcome is excellent, and this procedure can significantly improve the quality of 1ife and the long-term prognosis in these patients.

Surgical treatment with RPR technique for complex hypertrophic obstructive cardiomyopathy / 上海交通大学学报（医学版）

Objective · To evaluate the surgical outcome of RPR composite technique for complex hypertrophic obstructive cardiomyopathy (HOCM). Methods · From June 2009 to December 2015, 9 complex HOCM patients received RPR procedure. There were 6 males and 3 females with age from 22 to 63 years old and the average age of (43±19) years old. Transthorax echocardiography (TTE) showed systolic anterior motion (SAM) and at least moderate mitral valve regurgitation (MR) in all patients before operations. Transesophageal echocardiography (TEE) was used to evaluate the results of procedures during operation. All the patients had been followed up since one week after operation and examined by TTE. Results · All the patients were discharged without complications. Intraoperative TEE indicated that left ventricular outflow tract pressure gradient (LVOTPG) significantly decreased from (92±14) mmHg before operation to (9±3) mmHg after operation (P<0.01). SAM in all the patients disappeared. One week after operation, TTE demonstrated remarkable reduction in the thickness of ventricular septum, LVOTPG and MR than those before operation (P<0.01). Mean follow-up was 26 months. All the patients became asymptomatic. LVOTPG remained low and MR remained mild. There were no deaths, reoperations, or any other adverse consequences. Conclusion · RPR technique is an effective surgical method to relieve LVOTO and MR of complex HOCM to lead a better life.