Role of Surgery on Growth of Tricuspid Valve in Pulmonary Atresia with Intact Ventricular Septum:Mid-Term Results of Modified Right-Ventricular Overhauling Procedure

Objectives:To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve(TV)growth in patients with pulmonary atresia with intact ventricular septum(PAIVS).Methods:We retrospectively reviewed 21 patients with PAIVS who underwent modified right ventricular overhauling(mRVoh)between 2008 and 2019 at two institutions.Our mRVoh consisted of wide resection of hypertrophied infundibular and trabecular muscle,peeling off fibrotic endocardial tissue in the right ventricle(RV)cavity,surgical pulmonary valvotomy,and Blalock-Taussig shunt or banding of ductus arteriosus under cardiopulmonary bypass.The TV annulus sizes were measured and analyzed using echocardiography before and after mRVoh.Results:No mortalities were observed during a median follow-up of 3 years(interquartile range:1.3–4.7 years)of follow-up were noted.mRVoh was performed at a median age of 163.5 days(range:21–560 days),including seven neonates and two infants(<60 days).During follow-up,the median TV annular z-score increased significantly from−2.24 to−1.15 before and after mRVoh(p=0.004).In ten patients with a prior history of percutaneous interventions for RV outflow tract(RVOT)widening at least 6 months before mRVoh,the TV annular z-score significantly changed during the period after mRVoh(−2.03 to−1.61,p=0.028)compared with the period before mRVoh(−2.51→–2.03,p=0.575)after percutaneous intervention only.Conclusions:mRVoh in PAIVS patients was positively associated with TV annular growth,and it was more effective than percutaneous RVOT widening interventions without mRVoh.

Management of ductal spasm in a neonate with pulmonary atresia and an intact ventricular septum during cardiac catheterization:A case report

BACKGROUND Ductal spasm is a rare but life-threatening complication of cardiac catheterization in neonates with pulmonary atresia and an intact ventricular septum.In patients with ductal-dependent pulmonary blood flow,ductal spasm may lead to refractory hypoxemia and severe hemodynamic instability,which need to be treated in perfect order.CASE SUMMARY We present a male infant with a gestational age of 39 wk,and his fetal echocardiography showed pulmonary atresia.At 28 d of age,transcatheter pulmonary valvuloplasty with balloon dilatation was performed.Two hours after the operation,the patient's pulse oxygen saturation continued to decrease.The patient was then transferred to receive cardiac catheterization.During catheterization,the invasive blood pressure and pulse oxygen saturation suddenly decreased,and repeated aortography revealed partial occlusion of the ductus arteriosus.It no longer changed when pulse oxygen saturation rose to 51%after approximately 20 min of maintenance therapy.Therefore,a ductal stent was used for implantation.Hemodynamics and hypoxemia were improved.CONCLUSION We should know that ductal spasm may occur during pulmonary atresia and intact ventricular septum cardiac catheterization.Understand the pathophysiology of ductal-dependent pulmonary blood flow and make comprehensive perioperative preparations essential to deal with hemodynamic disorders caused by ductal spasm.

Arrhythmic Risk in Paediatric Patients Undergoing Surgical Repair for Pulmonary Atresia with Intact Ventricular Septum

Introduction:While previous studies only focused on the arrhythmic risk associated with specific correction strategies,this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-IVS after surgical repair.Methods:In this single centre observational cohort study,we retrospectively evaluated 165 patients with a diagnosis of PA-IVS and we excluded those with an exclusively percutaneous treatment,patients lost or with insufficient follow-up and those affected by other arrhythmic syndromes.Surgical history and clinical outcomes were reviewed.Results:86 patients were included in the study(54 male[62.8%],mean age 16.4±6.1 years),with median follow-up from definitive repair of 12.8 years(6.4–18.9 years).They underwent three different final repairs:23 patients(26.7%)univentricular palliation,43(50%)biventricular correction,and 20(23.3%)one and a half ventricle correction.Thirteen patients(15%)developed arrhythmia:6 patients(all the subgroups)sinus node disfunction(SND);2(biventricular repair)premature ventricular complexes;2(one and a half ventricle repair)non-sustained ventricular tachycardia;1(biventricular repair)intra-atrial re-entrant tachycardia;1(one and a half ventricle repair)supraventricular tachyarrhythmia;1(biventricular repair)atrial fibrillation.Three patients with SND needed a pacemaker implantation.Only Fontan circulation showed an association with SND,while the other two groups heterogeneous types of arrhythmias.Conclusions:The low arrhythmic risk is related to surgical repair,it does not appear to be associated with native cardiomyopathy,and it appears to increase with length of follow up.Continuous follow-up in specialized centres is necessary to make an early diagnosis and to manage the potential haemodynamic impact at medium-long term.

Hybrid procedure for pulmonary atresia with intact ventricular septum

Objective In patients with pulmonary atresia and intact ventricular septum (PAIVS) without right ventricular-dependent coronaries,catheter techniques including the use of a sniff wire,lasers,and radiofrequency have been the most widely used as initial therapy. However,percutaneous perforation and balloon valvuloplasty were associated with higher rate of procedural failure and

Evaluation of the Effect of Sigmoid-Shaped Interventricular Septum on Left Ventricular Systolic Function in Patients with Essential Hypertension by Two-Dimensional Speckle Tracking Echocardiography

Objective: To evaluate left ventricular regional and global systolic function by measuring left ventricular longitudinal strain (LS) in hypertensive patients with sigmoid-shaped interventricular septum (SIS) by two-dimensional speckle tracking (2D-STE);in order to explore whether the sigmoid-shaped interventricular septum affects the left ventricular systolic function in patients with hypertension. Methods: Routine echocardiographic parameters were measured in 30 hypertensive patients with SIS (SIS group) and 30 hypertensive patients without SIS (non-SIS group). The left ventricular segments and global LS were measured by 2D-STE, and the two sets of parameters were compared. Results: The value of the thickness of the basal segment of the interventricular septum (IVSBT), the thickness of the middle segment of the interventricular septum (IVSMT) and the ratio of the basal segment of the ventricular septum to the middle segment of the interventricular septum (IVSBT/IVSMT) in SIS group was higher than that in non-SIS group. However, the value of left ventricular outflow tract diameter (LVOTD) in SIS group was lower than that in non-SIS group. There was a significant difference between the two groups (all P Conclusion: SIS affects left ventricular regional systolic function of patients with hypertension. 2D-STE can early evaluate left ventricular longitudinal systolic function in hypertensive patients with SIS.

Implantation of Lumenless Pacing Leads at the Inter-atrial Septum and Right Ventricular Outflow Tract with Deflectable Catheter-sheath

Current permanent right ventricular and right atrial endocardial pacing leads are implanted utilizing a central lumen stylet. Right ventricular apex pacing initiates an abnormal asynchronous electrical activation pattern, which results in asynchronous ventricular contraction and relaxation. When pacing from right atrial appendage, the conduction time between two atria will be prolonged, which results in heterogeneity for both depolarization and repolarization. Six patients with Class Ⅰ indication for permanent pacing were implanted with either single chamber or dual chamber pacemaker. The SelectSecure 3830 4-French （Fr） lumenless lead and the SelectSite C304 8.5-Fr steerable catheter-sheath （Medtronic Inc., USA） were used. Pre-selected pacing sites included inter-atrial septum and right ventricular outflow tract, which were defined by ECG and fluoroscopic criteria. All the implanting procedures were successful without complication. Testing results （mean atrial pacing threshold： 0.87 V; mean P wave amplitude： 2.28 mV; mean ventricular pacing threshold： 0.53V; mean R wave amplitude： 8.75 mV） were satisfactory. It is concluded that implantation of a 4-Fr lumenless pacing lead by using a streerable catheter-sheath to achieve inter-atrial septum or right ventricular outflow tract pacing is safe and feasible.

新生儿室间隔完整型肺动脉闭锁治疗结果

目的 总结室间隔完整型肺动脉闭锁患儿的治疗经验。方法 回顾性分析2017年1月至2023年1月收治的16例新生儿室间隔完整型肺动脉闭锁临床资料,男9例,女7例;日龄4 h~25 d;入院体重1.6~4 kg,平均(3.22±0.65) kg。所有患儿初期治疗后进行随访,随访时间3个月至6年,中位随访时间33(12,55)月。初期治疗措施包括以下术式:体外循环下房间隔扩大、B-T分流、镶嵌治疗、非体外下经胸肺动脉瓣切开术等,每例患儿选择一种或者多种组合术式。结果 患儿院内死亡1例,院外无死亡病例。再次治疗6例,其中行经导管肺动脉瓣球囊扩张成形术5例,行双向Glenn术1例。其余9例患儿术中后已5年1例,经皮血氧饱和度>95%,房间隔转为左向右分流,远期免于再干预,其余患儿仍在随访中,存在再次干预可能。首次根治后1个月较大的肺动脉根部直径远期行双心室根治可能性大。结论 室间隔完整型肺动脉闭锁是需要序贯性治疗和观察的疾病,为获得较好的生存质量,初次治疗应依据右心室发育程度选择个性化措施,然后密切主动随访,适时给予再次治疗。

先天性心脏病宫内介入治疗现状与展望

胎儿宫内心脏介入治疗(FCI)技术自1991年起发展至今,随着技术成功率的提高、并发症的减少及对患胎预后的改善,该技术已逐步获得医学界的认可与推广。国际胎儿心脏介入注册数据库的建立促进了该技术的国际间交流和技术发展,国际报道临床病例已逾400例。在国内,迄今已有广东、上海、青岛、重庆等多家医疗机构开展宫内心脏介入治疗,但病例数仍有限。文章从胎儿宫内心脏介入治疗的种类、操作过程、手术并发症、治疗指征以及治疗结局进行综述,同时提出该技术目前存在的问题和未来的展望。

宫内心脏介入治疗胎儿危重型先天性心脏病5例报告

目的探索胎儿心脏介入治疗(FCI)对于胎儿危重型先天性心脏病的可行性及短期疗效。方法纳入2018年8月至2022年5月在宫内诊治中心进行FCI的所有患儿的临床资料,包括围产期至出生后基本信息、FCI手术信息、胎儿超声心动图信息等,追踪病例至出生后3月,分析FCI技术的可行性、有效性及安全性。结果纳入胎儿7例,其中5例实施FCI手术,手术成功率100%(5/5),包括2例重度主动脉瓣狭窄(CAS)和3例室间隔完整型肺动脉闭锁(PA/IVS)。手术时的中位孕周为29^(+2)(28^(+6)~32^(+4))周。5例胎儿FCI术后的超声心动图指标均获得明显改善。5例胎儿均足月出生,中位出生胎龄39^(+2)(38~39^(+4))周。生后2例CAS患儿行外科主动脉瓣成形术,3例PA/IVS患儿完成经皮肺动脉瓣球囊扩张术。1例心功能不全CAS患儿于新生儿期死亡,4例随访中,预后良好。结论FCI是一项安全、有效的技术,有望改善危重型先天性心脏病的预后。

左心室室间隔瘢痕对左束支起搏术实现左束支夺获的影响

目的:通过心脏磁共振探索室间隔瘢痕对左束支起搏实现左束支夺获的影响。方法:回顾性分析52例拟接受左束支起搏的患者,根据是否实现左束支夺获分为失败组和成功组,并通过心脏磁共振对室间隔瘢痕分节段进行评分。结果:上室间隔瘢痕评分是预测左束支夺获成功与否的独立预测因子。当上室间隔的瘢痕<25%,左束支夺获的成功率为100%。而当瘢痕≥50%,左束支夺获的失败率为100%。结论:采用心脏磁共振评估上室间隔瘢痕有助于预测左束支起搏手术成功与否,为手术的顺利实施提供可靠的参考依据。

心脏磁共振评估结缔组织病伴重度肺动脉高压的价值

目的评估心脏磁共振(CMR)对重度结缔组织病相关肺动脉高压(CTD-PAH)患者的诊断价值。方法纳入经右心导管(RHC)检查确诊为CTD-PAH的患者。在CMR图像上手动勾画并经体表面积校正获得右心室(RV)形态、功能及室间隔(IVS)的相关参数;在心肌延迟强化(LGE)图像上手动勾画并计算得到LGE心肌质量(MM)及其占左心室(LV)MM百分比。依照平均肺动脉压(mPAP)将其分为轻中度组和重度组,比较2组患者CMR参数差异,采用受试者工作特征(ROC)曲线分析CMR对重度患者的诊断价值。结果本研究共纳入48例CTD-PAH患者。重度组患者室间隔曲率(CIVS)及RV射血分数(EF)低于轻中度组,IVS形变时间占比、RV舒张末期容积指数(EDVI)、RV收缩末期容积指数(ESVI)及RV MM高于轻中度组(均P<0.05)。ROC曲线分析显示RV MM、IVS形变时间占比及RV ESVI对重度患者具有较好的诊断效能(AUC分别为0.792、0.766、0.731)。联合检测曲线下面积为0.840,敏感度为79.4%,特异度为85.7%。结论CMR测得的RV及IVS参数能够有效地评估重度CTD-PAH患者,从形态和功能学的角度直接反映了重度CTD-PAH患者的心脏受损程度。

Effects of PPAR-y in the Myocardium on the Development of Ventricular Septation

Ventricular septum defects(VSDs)are common types of congenital heart diseases caused by developmental defect;they contribute to 25%-30%of all adult congenital heart diseases.The peroxisome proliferator-activated receptor gamma(PPAR-y)is widely expressed in mammalian tissues and in the immune system,regulating cell differentiation and immune and inflammatory responses.The PPAR-y gene has recently been found crucial for heart development,but the mechanism of action is not clear.This study aims to investigate the effects of the PPAR-y gene in the myocardium on the development of ventricular septation.In this study,we applied Cre-loxP recombination enzyme(CRE)technology to downregulate the expression of the PPAR-y gene in different cardiac tissues,RT-PCR to examine the expression of the c-fos and TGF-B!genes,and histology staining to check the defect of embryonic heart at embryonic day 14.5(E14.5).We found that the downregulation of the PPAR-p gene resulted in a ventricular membranous septation defect of the embryonic heart at E14.5.Furthermore,only conversion of a Tnt:Cre,but not Mef2c:Cre,Tie2:Cre,or Wnt:Cre PPAR-T floxed allele to a null allele resulted in VSD.PPAR-/mi-Orv+embryos showed increascs in atrioventricular(AV)-cushion cells and the expression of c-fos gene but no change in the expression of TGF-B1 at E10.5.Our study demonstrates PPAR-N in the myocardium is required for ventricular septation through regulation of AV-cushion cell proliferation by a Tntc-fos signal.

慢性肾脏病患者左心室肥厚危险因素的研究进展

左心室肥厚(LVH)是慢性肾脏病(CKD)患者中最常见的心肌损害,室间隔增厚是评估LVH的重要定量指标。导致CKD患者LVH的危险因素,包括高血压、糖尿病、容量超负荷、透析方式、贫血、继发性甲状旁腺功能亢进、营养不良、血脂代谢异常及慢性炎症反应等,这些因素互相影响,共同作用于心血管系统,导致CKD患者发生心脏结构及功能改变。目前新的危险因素如骨调节蛋白、成纤维细胞生长因子23,对CKD患者LVH的相关发病机制、治疗的研究仍尚少,将来仍需收集大样本量的研究进一步探讨,寻找对应靶点进行干预,改善CKD患者的预后。

经皮心肌内室间隔射频消融治疗肥厚型梗阻性心肌病进展

肥厚型梗阻性心肌病(HOCM)以左心室流出道动态性梗阻为特征,可引起心源性猝死;早期干预对于改善预后至关重要。经皮心肌内室间隔射频消融(PIMSRA)为室间隔减容治疗HOCM提供了新的选择。本文围绕PIMSRA治疗HOCM进行综述。

心脏永久性起搏器的不同起搏部位对植入患者心功能的影响

目的探析心脏永久性起搏器的不同起搏部位对植入患者心功能的影响。方法200例植入心脏永久性起搏器患者,按照起搏部位不同分为对照组和试验组,每组100例。对照组植入部位为右心室心尖部,试验组植入部位为右心室间隔。对比两组患者植入前后心功能指标[左心室射血分数(LVEF)、左心室舒张末期内径(LVEDD)、左心室收缩末期内径(LVESD)、B型脑钠肽(BNP)]和生活质量[健康调查简表(SF-36)评分]。结果植入6个月后,两组患者LVEF高于本组植入前,LVEDD、LVESD小于本组植入前,BNP低于本组植入前,且试验组患者LVEF(53.06±5.47)%显著高于对照组的(48.45±5.11)%,LVEDD(52.48±3.32)mm、LVESD(49.07±3.56)mm明显小于对照组的(54.88±3.84)、(51.54±3.42)mm,BNP(67.43±8.06)pg/ml明显低于对照组的(82.18±8.84)pg/ml(P<0.05)。植入6个月后,两组患者SF-36评分均较本组植入前显著提升,且试验组患者SF-36评分(80.06±5.11)分高于对照组的(72.12±5.67)分(P<0.05)。结论在进行心脏永久性起搏器植入时,与右心室心尖部相对比,右心室间隔起搏可以更为有效的改善患者心功能,提高生活质量。

Long-term follow-up of right ventricular outflow tract septal pacing

Objective Right ventricular outflow tract septum has become widely used us an electrode placement site. However, data concerning lead performances and complications for lead repositioning with this technique were scant. The purpose of this study was to observe long- term lead performances and complications of right ventricular outflow tract septal pacing and provide evidences for choosing an optimal electrode implantation site. Methods Thirty-six patients with septal active electrode implantation and 39 with apical passive electrode implantation were enrolled in this study. Pacing threshold, R-wave sensing, lead impedance, pacing QRS width and pacing-related compli- cations for two groups at implantation and follow-up were compared. Results There were higher pacing threshold and shorter pacing QRS width at implantation in the septal group compared with the apical group. There were no differences between the septal and the apical groups in pacing threshold, R-wave sensitivity, lead impedance and pace-related complication during a follow-up. Conclusions Right ventricular outflow tract septum could be used as a first choice for implantation site because it had long-term stable lead performances and no serious complications compared with the traditional apical site.

Septic Pulmonary Emboli and Right Ventricular Obstruction in a Membranous Interventricular Septal Aneurysm

Although the majority of patients with perimembranous ventricular septal defect and septal aneurysm remained asymptomatic, some of them presented with serious complications during adulthood and thus required high risky surgery. In accordance with other rare condition, the incidence and natural history have not been well documented. This case describes the occurrence of a septic pulmonary emboli associated with right ventricular outflow tract obstruction in a young child.

Refinement of total 12-lead QRS voltage criteria for diagnosing left ventricular hypertrophy

Objective: We sought to test the hypothesis that the total QRS voltage without either set of the limb leads (I, II, III) or (R, L, F) may be a better indicator of LVH as compared to the total QRS voltage. Background: The total 12 lead QRS voltage has been a validated electrocardiographic criterion for left ventricular hypertrophy (LVH), with an upper limit of175 mm. However, there is some redundancy in this measurement as the output of the limb leads is repeated because leads I, II, III, and R, L, F use the same three electrodes. Methods: 43 unselected, consecutive echocardiograms were examined for evidence of LVH by wall thickness. Electrocardiogram (ECG) of these patients within a week of the echocardiogram were then examined for the total 12 leads QRS voltage, minus I, II, III and total minus R, L, F voltages. ECG findings were then compared with corresponding echocardiographic dimensions. Results: A total QRS voltage of123 mmon ECG yielded a sensitivity of 73% and specificity of 67% for diagnosing LVH with 95% CI = 0.59 - 0.89, p = 0.007. Total minus (R, L and F) value of110 mmon ECG appears to give the best sensitivity (73%), specificity (72%), and accuracy (64% negative predictive value and 82% positive predictive value) for LVH. Conclusion: It appears that total QRS voltage minus either set of the limb leads, especially the total minus R, L and F is a better criterion, with110 mmbeing the best specific, sensitive and accurate index for diagnosing LVH.

超声诊断左冠状动脉-右心室瘘合并室间隔夹层1例

女性患儿,10岁,间断胸痛1年,加重5个月;既往外院诊断为“先天性左冠状动脉瘘(coronary artery fistula, CAF)”, 无特殊家族史。查体:胸骨左缘第3肋间闻及4/6级舒张期杂音,传导广泛。心电图提示窦性心律、右束支传导阻滞。

室间隔缺损合并缺陷乏养菌致感染性心内膜炎——附1例报告

目的报告1例室间隔缺损合并缺陷乏养菌致感染性心内膜炎(IE)患者的诊疗过程。方法回顾并分析厦门大学附属心血管病医院收治的1例先天性室间隔缺损合并IE患者的临床资料以及治疗方案,采用微生物检验与临床诊断相结合的方法,收集患者外周静脉血样本及术中切除的瓣膜赘生物标本进行微生物培养。结果患者为54岁女性,因“反复胸痛3年,再发1个月余”于2021年10月12日入院。入院心脏彩超显示为室间隔缺损,肺动脉瓣上可见长约12.7 mm的赘生物,其外周血及瓣膜赘生物标本均培养出缺陷乏养菌。经抗感染、外科手术治疗,患者好转出院。结论缺陷乏养菌作为正常菌群,当机体免疫力低下时经由血行途径成为致病菌,尤其当患者存在先天性心脏病等危险因素时,会引起IE。