Objective:To evaluate the clinical efficacy of percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) in the treatment of obstructive hypertrophic cardiomyopathy (HOCM) with mild septal hypertrophy.Methods:Forty-five HOCM patients with mild septal hypertrophy (the maximal left ventricular wall thickness is 15-19 mm) who were treated with PIMSRA between November 2016 to February 2021 in the Hypertrophic Cardiomyopathy Center of Xijing Hospital of Air Force Military Medical University were enrolled, and their clinical datas were collected and analyzed. The clinical symptoms and NYHA functional class before operation, 6 months and 1 year after operation were collected. Interventricular septum thickness, left ventricular outflow tract pressure gradient, left ventricular outflow tract diameter, mitral regurgitation, left ventricular systolic and diastolic function were evaluated by transthoracic echocardiography before operation, 6 months and 1 year after operation, intraoperative complications were monitored and recorded. Postoperative arrhythmias were monitored by routine 12 lead ECG and 24-hour ambulatory ECG.Results:All patients successfully completed PIMSRA procedure.No clinical adverse events such as death, bleeding and stroke occurred during and around the operation.No left bundle branch block, complete atrioventricular block and malignant arrhythmia occurred after the operation. All patients did not need permanent pacemaker implantation.NYHA functional class and clinical symptoms of patients were significantly improved after 6 months compared with values before operation (all P<0.001, respectively), it remained stable for 1 year after operation; Anterior interventricular septum, posterior interventricular septum, maximal left ventricular wall thickness all significantly decreased (all P<0.001, respectively), left ventricular outflow tract diameter widened ( P<0.001), continuous improvement 1 year after operation; left ventricular outflow tract gradient and provoked left ventricular outflow tract gradient all significantly decreased, mitral regurgitation decreased and SAM classification reduced after 6 months compared with values before operation (all P<0.001, respectively); left ventricular end-diastolic diameter widened and left atrial diameter decreased (all P<0.001, respectively), it remained stable for 1 year after operation. Left atrial volume index decreased ( P<0.001), with continuous improvement 1 year after operation; The ratio of early diastolic mitral valve velocity to early diastolic mitral annulus velocity (E/e′) decreased ( P=0.001), it remained stable for 1 year after operation. There were no significant differences in left ventricular end diastolic volume, left ventricular end systolic volume and left ventricular ejection fraction among the three groups (all P>0.05). Conclusions:PIMSRA is effective in the treatment of obstructive hypertrophic cardiomyopathy with mild ventricular septal hypertrophy.

Objective:To investigate the effect of percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) guided by echocardiography on the Lown classification of ventricular arrhythmias in patients with hypertrophic obstructive cardiomyopathy (HOCM).Methods:A total of 85 patients with HOCM who received PIMSRA treatment at Xijing Hospital of Air Force Military Medical University from May 2017 to October 2019 were retrospectively selected. All patients underwent 24-hour Holter examinations before and 1 year after PIMSRA to obtain parameters related to Lown classification. The changes in Lown grades after PIMSRA were analyzed. The patients were divided into improved group and unimproved group according to whether there was significant improvement in Lowen′s grades, and the difference of the parameters related were compared. The influencing factors of the changes in Lown classification were analyzed.Results:Compared with before PIMSRA, there was a significant improvement in the Lown classification after PIMSRA ( P=0.001). The patients with Lown grade Ⅰ increased significantly ( P=0.001), and the patients with grade Ⅲ decreased significantly ( P=0.005). There were no significant changes in patients with Lown grades 0, Ⅱ, and Ⅳ (all P>0.05). The proportion of patients with family history of hypertrophic cardiomyopathy (HCM), the baseline Lown classes, the reduction rate of the maximum left ventricular wall thickness and the reduction rate of the provocative left ventricular outflow tract gradient (LVOTG) were higher in the improved group than the unimproved group (all P<0.05). Multivariate Logistic regression results showed that HCM family history ( OR=3.95, 95% CI=1.34-11.64, P=0.013), baseline Lown classes ( OR=2.01, 95% CI=1.25-3.22, P=0.004) and the reduction rate of the provocative LVOTG gradient ( OR=1.02, 95% CI=1.00-1.04, P=0.041) were independent factors of postoperative Lown classification improvement. Conclusions:The Lown classes of HOCM patients after PIMSRA is significantly improved.HCM family history, the baseline Lown classes, and the reduction rate of postoperative provocative LVOTG are independent influencing factors for the improvement of Lown grade.

The clinical manifestations of subacute combined degeneration of spinal cord (SCD) in children are complex and vary greatly. Due to the fact that some patients with SCD may be complicated with autoimmune diseases, the high early misdiagnosis and missed diagnosis rates are observed. One case of 13-year old female with severe anemia, multiple joint swelling and pain in left limbs and paralysis of bilateral lower limbs with the extremely low level of serum vitamin B12 and poly-glandular involvement as well as a variety of positive auto-antibodies (anti-intrinsic factor antibody, anti-parietal cell antibody, thyroid peroxidase antibody, thyroid globulin antibody and perinuclear anti-neutrophil cytoplasmic antibody) was retrospectively analyzed. The patient was diagnosed as SCD with autoimmune disease (undifferentiated connective tissue disease and autoimmune polyglandular syndrome). The patient′s condition gradually alleviated after high-dose intravenous methylprednisolone, immunoglobulin, naproxen (then changed to hydroxychloroquine 1 month later), vitamin B12 and levothyroxine sodium tablets supplementation, blood transfusion and rehabilitation. SCD with autoimmune diseases is rare in children, and the clinical manifestations vary greatly. Early recognition and early treatment can improve the prognosis of SCD. The clinical data of this child were retrospectively analyzed, so as to improve the understanding of the disease by clinicians.

Objective:To evaluate the left ventricular myocardial strain in patients with light chain cardiac amyloidosis (AL-CA) with normal left ventricular ejection fraction (LVEF) by three-dimensional speckle tracking imaging(3D-STI), and to explore the clinical value of 3D-STI in predicting the prognosis of AL-CA patients with normal LVEF.Methods:A total of 80 patients with AL-CA and LVEF≥50% were retrospectively analyzed in the Xijing Hospital of Air Force Military Medical University from October 2014 to May 2020.According to whether the patients had endpoint events, they were divided into endpoint event group and non-endpoint event group. The clinical data, conventional echocardiographic parameters, 3D-STI related parameters and follow-up results were collected. Cox regression proportional hazards model was used to analyze the survival status of AL-CA patients with univariate and multivariate regression analyses, in order to find the relevant indicators of conventional echocardiography and 3D-STI to predict adverse events.Results:All patients were followed up for 20(7.3, 40.8) months. At the end of follow-up, 25 patients had all-cause deaths. Compared with the non-endpoint group, the endpoint event group had significantly increased left ventricular end diastolic maximum wall thickness (MLVWT), peak early diastolic flow velocity/peak early diastolic velocity at mitral annulus(E/e′) (all P<0.05), and decreased LVEF, left ventricular global longitudinal strain (GLS) and basal segment longitudinal strain (LS) (all P<0.05). Multivariate cox regression analysis after adjusting for age and gender showed that basal segment LS ( HR=0.812, 95% CI=0.675-0.976, P=0.026) was an independent predictor of end-point events in patients with AL-CA. Kaplan-Meier survival curve showed that AL-CA patients with basal segment LS≤13.07% were more likely to have endpoint events. Conclusions:Basal segment LS can be used as a predictor of endpoint events in patients with AL-CA.

Objective:To quantitatively evaluate myocardial microcirculation perfusion changes after percutaneous intracardiac septal radiofrequency ablation (PIMSRA, Liwen procedure) in patients with obstructive hypertrophic cardiomyopathy (HOCM) using myocardial contrast echocardiography (MCE) and to further establish the effect of the procedure and predict the long-term prognosis of patients.Methods:A total of 45 patients with HOCM treated by the Liwen procedure in the First Affiliated Hospital of Air Force Medical University (Xijing Hospital) from July 2019 to June 2020 were included. MCE was performed before and 6 months after surgery, respectively. Time-intensity perfusion curve analysis was performed using QLab 10.8 offline software to obtain quantitative parameters of myocardial microcirculation perfusion including myocardial blood volume (A value), myocardial blood flow velocity (β value), and myocardial blood flow (A×β value), then the changes in parameters before and after the procedure were compared.Results:After the Liwen procedure, the mean septal thickness and mean left ventricular free wall thickness were significantly reduced, accompanied by a significant reduction in the left ventricular outflow tract pressure gradient (LVOT-PG) and mitral regurgitation length, and a significant improvement in mitral systolic antegrade motion (SAM) (all P<0.001). In addition, the left ventricular ejection fraction (LVEF) did not deteriorate significantly ( P=0.560) and the E/e′ ratio decreased after the procedure ( P=0.015). Besides, the A values of both the ventricular septum and the left ventricular free wall were not significantly changed compared to those before procedure ( P>0.05), whereas the β values and A×β values were obviously increased ( P<0.05). Conclusions:Myocardial blood velocity and myocardial blood flow in the septum and left ventricular free wall were significantly increased in patients with HOCM after the Liwen procedure, suggesting a significant improvement in myocardial microcirculation perfusion. MCE provides a non-invasive quantitative evaluation parameters of myocardial microcirculation perfusion for the Liwen procedure for the treatment of HOCM.

Objective:To explore the clinical manifestations of 4 pedigrees with transthyretin related familial amyloid polyneuropathy (TTR-FAP).Methods:The clinical data were collected and analyzed from 4 pedigrees with TTR-AFP, admitted to our hospital from July 2017 to May 2019; 20 patients and 2 asymptomatic carriers of the TTR mutation gene were included. In particular, the detailed data of the 4 probands affected with TTR-FAP came from the 4 different pedigrees were collected. Results:In these 20 patients, the age of onset ranged from 30 to 65; the first symptoms of diarrhea, constipation, alternating episodes of constipation and diarrhea were found; there were damaged peripheral nerve and inexplicable weight loss; cardiomyopathy was noted in 9 patients; orthostatic hypotension was noted in 9 patients, sexual dysfunction in 5, abnormal urination in 6, and blurred vision or corestenoma in 3. TTR mutation gene was confirmed in 7 patients and pathological diagnosis was found in 3 patients. Diflunisal was used in one patient and tafamidis was used 2 patients. Twelve died and 8 patients survived among 20 patients with disease progression. All the 4 probands were male, with an average age of 49.3 years; all patients had different degrees of sensorimotor peripheral neuropathy, autonomic neuropathy and cardiomyopathy; electrophysiological examination suggested length dependent sensory motor peripheral neuropathy of the extremities, with axonal damage as the evidence; and cardiac hypertrophy was noted in echocardiography. The sural nerve biopsy of the 3 probands showed positive Congo red staining. Medical whole exon sequencing indicated that 2 probands had pathogenic mutations (TTR-E74K and TTR-A140S), and 1 proband had likely pathogenic mutation (TTR-S70R). Two asymptomatic carriers of the TTR gene mutation remained normal condition. Conclusion:The clinical manifestations of TTR-FAP include progressive sensorimotor and autonomic neuropathy, and multi-system disorders, such as combining with gastrointestinal problems, hypertrophic myocardium, inexplicable weight loss and blurred vision or corestenoma, which might be important reminders for diagnosis of TTR-FAP.

Objective:To explore the value of three-dimensional speckle tracking imaging (3D-STI) in the diagnosis of immunoglobulin light-chain cardiac amyloidosis(AL-CA) patients with normal left ventricular ejection fraction (LVEF).Methods:A total of 92 consecutive patients diagnosed with systemic immunoglobulin light chain amyloidosis(sAL) and with normal LVEF from October 2014 to January 2018 in Xijing Hospital were enrolled.Based on the diagnostic criteria of cardiac involvement, the patients were divided into AL-CA group (52 cases) and immunoglobulin light chain amyloidosis (AL) group (40 cases). The clinical data and serological markers of the patients were collected, the conventional echocardiography and full-volume three dimensional dynamic images were acquired, left ventricular global longitudinal strain (GLS), global radial strain (GRS), global circumferential strain (GCS), and global area strain (GAS) were analyzed using off-line TomTec software. The differences between the two groups were compared.Results:Compared with the AL group, the NT-proBNP of AL-CA group was significantly higher ( P<0.05) and there were no significant differences of the other serological indexes between the two groups(all P>0.05). Compared with the AL group, the maximal left ventricular wall thickness, left ventricular mass index, left atrial volume index, and E/e′ in the AL-CA group were significantly increased (all P<0.05). There were no significant differences of other conventional echocardiographic measurements between the two groups(all P>0.05). Compared with the AL group, GLS, GAS, and GRS were significantly lower in AL-CA group (all P<0.05); but there was no significant difference of GCS between the two groups( P>0.05). The ROC curve analysis showed that the cut-off values discriminating cardiac involvement were 16.09% for GLS, 36.54% for GAS and 31.90% for GRS. Conclusions:3D-STI measurements of left ventricular myocardial mechanics could detect cardiac involvement in patients with sAL amyloidosis, and provides a new method for diagnosis of AL-CA.

Objective To explore the changes of left ventricular torsion function in patients with latent obstructive hypertrophic cardiomyopathy ( HCM ) ,and provide quantitative informations for clinical evaluation of cardiac function . Methods A total of 49 consecutive patients with HCM without left ventricular outflow tract obstruction at rest were enrolled . All subjects underwent exercise stress echocardiography . After exercise left ventricular outflow tract pressure gradient ( LVO T‐PG ) ≥30 mm Hg was positive for exercise stress test ( latent obstruction) ,w hile LVO T‐PG＜ 30 mm Hg was negative for exercise stress test ( non‐obstruction) . An ultrasound system obtained two‐dimensional ultrasound images of resting and moving peaks . The global longitudinal strain ( GLS ) ,global circumferential strain ( GCS ) , global radial strain ( GRS) of the left ventricle 16 segments and left ventricular rotation ,twist were analysis using off‐line EchoPAC software . T he differences of the above parameters were compared between the two groups . Results T here were no significant differences in GLS ,GRS ,GCS and Rotation‐B between the two groups in resting and peak period of exercise ( all P ＞ 0 .05 ) ,GRS in both groups were significantly increased compared with that before exercise ( all P ＜ 0 .05 ) . Compared with the negative exercise stress group ,the left ventricular twist and Rotation‐A were significantly increased in resting and peak period of exercise in the positive exercise stress test group( all P ＜0 .05) . Compared with before exercise ,Rotation‐A and left ventricular twist were significantly decreased in the positive exercise stress test group ( all P ＜0 .05) ,while no significantly difference was found in the negative exercise stress group ( all P ＞ 0 .05 ) . Conclusions Left ventricular torsion function is significantly changed in rest and after exercise in latent obstructive HCM patients ,providing valuable quantitative information for clinical comprehensive evaluation of cardiac function .

Objective To evaluate left ventriclular systolic function in patients with obstructive hypertrophic cardiomyopathy (HOCM) after modified Morrow surgery using two-dimensional speckle tracking imaging (2D-STI).Methods Twenty three HOCM patients were recruited in this study.Echocardiographic data from HOCM patients during pre-operation,1-month and 3-month post-operation were analyzed by Qlab software to compare the variation in systolic function indicators 1-month and 3-month post-operation including global and 16 segmental longitudinal strain,circumferential strain and conventional echocardiographic parameters of left ventricle.Results Compared with preoperative data of HOCM patients,postoperative LVOT diameter and pressure gradient,left atrial diameter and volume index were significantly decreased(P<0.05),but there was no significant difference in left ventricular ejection fraction(P<0.05).Compared with the preoperative case,global and segmental longitudinal strain showed significant reduction after 1 week and gradually recovered after 3 months,without significant variation.The longitudinal strain of the anteroseptum reduced significantly and the longitudinal strain of the free wall increased after 3 months,however,the circumferential strain reduced significantly.The circumferential strain of basal and middle segment after 3 months had improved significantly than those of postoperative 1 week.The circumferential strain of surgical site is no obvious change and the strain of free wall was improved after 3 months.Conclusions 2D-STI can effectively evaluate global and regional systolic function of left ventricle for HOCM patients after modified Morrow surgery.