Congenital left ventricular aneurysm coexisting with left ventricular non‐compaction in a newborn

Described herein is the case of a rare combination of congenital left ventricular (LV) aneurysm and left ventricular non‐compaction (LVNC) in a newborn. The patient developed refractory heart failure soon after birth and died at 5 months of age. The etiology of both congenital LV aneurysm and LVNC seems to be maldevelopment of the ventricular myocardium during early fetal life. Treatment should be individually tailored depending on clinical severity, and treatment options are limited. Given that this combination of congenital LV aneurysm and LVNC is significantly associated with poor prognosis, it appears that patients with congenital LV aneurysm and LVNC are candidates for early, aggressive intervention, including surgical aneurysmectomy and evaluation for transplantation. It is important to be aware of this combination of congenital LV aneurysm and LVNC, and to make earlier decisions on therapeutic strategy.     

儿童先天性心脏病合并心肌致密化不全的介入治疗及随访分析

目的探讨儿童先天性心脏病合并心肌致密化不全介入治疗的安全性及预后。方法回顾分析2016年1月至2017年10月收治的先天性心脏病合并心肌致密化不全患儿的临床资料。比较患儿在心脏介入治疗前后,以彩色多普勒超声心动图测量的非致密心肌/致密心肌(N/C)比值及心功能参数的变化;记录并发症发生情况。结果共纳入患儿25例,男9例、女16例,中位年龄为1岁(0.14～8岁);动脉导管未闭22例、房间隔缺损2例、室间隔缺损1例。术前、术后1、6、12个月之间左心室舒张末期内径(LVEDD)-Z值和N/C比值的差异均有统计学意义(P0.05),均以术后12月时为最低;其中6例患儿在随访过程中心肌致密化不全基本消失。但各时间点之间左室射血分数(LVEF)的差异无统计学意义(P0.05)。术前及术后1、6、12个月,患儿N/C比值、LVEDD-Z值与LVEF值均无相关性(P0.05)。术后随访中位时间19个月(12 ～27个月),随访过程中未出现心血管不良事件及心律失常发生。结论儿童先天性心脏病合并心肌致密化不全如满足介入治疗指征,应积极介入治疗以减少左向右分流,减轻心脏负荷,有利于左室心肌发育及心室逆重构。     

新型左心室扩大技术在左心室较小型婴儿法洛四联症根治术中的应用

目的 探讨新型左心室扩大技术在左心室较小型婴儿法洛四联症(TOF)根治术中的应用.方法 2008年8月至2013年2月,一期根治左心室较小型婴儿TOF 41例.其中男23例,女18例;年龄2.5 ～12.0(8.71±2.52)个月;体质量5.2 ～10.8(8.73±2.06) kg;身高55.5 ～78.4(66.38±10.18)cm;左心室舒张末期容积指数(LVEDVI) 24～29 (26.51±2.92) mL/m2.所有患儿术中均采用新型左心室扩大技术增大左心室容积.结果 出院时与术前相比:左心室舒张末前后径(LVEDD)、左心室舒张末容量(LVEDV)、LVEDVI、右心室横径(RVD)、主肺动脉内径(PAD)均增加(t=-2.909,-9.786,-2.105,-2.686,-14.137,P均＜0.05),收缩期主肺动脉血流速度(PAV)、左心室短轴缩短率(FS)减小(t=30.123,2.329,P均＜0.05);术后6个月与术前相比:左心房前后径(LAD)、LVEDD、LVEDV、LVEDVI、RVD、PAD、左右肺动脉内径之和(LP +RP)均显著增加(t=-3.164,-6.441,-28.642,-5.741,-7.022,-20.174,-9.224,P均＜0.05),PAV减小(t=41.147,P ＜0.001);术后6个月与出院时相比:LAD、LVEDD、LVEDV、FS、RVD、LP+RP均增加(t=-3.032,-3.083,-12.661,-2.121,-3.728,-8.229,P均＜0.05).术后低心排出量综合征4例,急性肾功能不全2例,无严重心律失常,均治愈出院,术后随访6个月～5年,无死亡病例,恢复良好.结论 此新型左心室扩大技术可以安全有效应用于左心室较小型婴儿TOF根治术中.     

Changes in left ventricular mass index in children and adolescents after renal transplantation

Recent reports indicate a high prevalence of left ventricular hypertrophy (LVH) in children on dialysis and after renal transplantation (Tx), as identified by cross-sectional analysis. However, the evolution of LVH in pediatric patients with end-stage renal disease after renal Tx is not well established. To assess changes of left ventricular mass (LVM), we prospectively performed echocardiography in 23 children and adolescents between November 1998 and July 2000. Each patient had an echocardiographic evaluation while on dialysis (for at least 6 weeks) and a follow-up evaluation at least 6 months after successful renal Tx (i.e. with a measured glomerular filtration rate [GFR] of at least 40 mL/min/1.73 m2). The LVM index was estimated by indexing LVM to height(2.7). Sixteen patients had a cadaveric transplant and seven had a live donor transplant; the mean duration between the two studies was 1.9 +/- 1.6 yr; and the mean GFR was 55.0 +/- 21.4 mL/min/1.73 m2. There was no significant difference in the mean values of the LVM index while on dialysis and after renal Tx (43.9 +/- 17.8 g/m2.7 and 39.3 +/- 12.0 g/m2.7, respectively, p = 0.19), or in the prevalence of LVH (52% and 56%, respectively). Interval changes in the LVM index in individual subjects between the two studies were significantly associated with interval changes in indexed systolic (r = 0.42, p = 0.04) and diastolic (r = 0.42, p = 0.05) blood pressures. Interval changes in hemoglobin, blood urea nitrogen (BUN), creatinine, and duration after Tx did not correlate with changes in the LVM index. There was no significant difference in LVM index change according to the type of dialysis, donor source, and the cause of renal failure. In multivariate analysis, the baseline LVM index and changes in indexed SBP were independent predictors for LVM index change after renal Tx. We conclude that LVH persists in children and adolescents after renal Tx. Control of blood pressure might be an important factor in regression or prevention of progression of LVH in these patients.     

Left ventricular growth in a patient with critical coarctation of the aorta and hypoplastic left ventricle

An infant is presented who at birth met criteria consistent with hypoplastic left heart syndrome. He was followed clinically and by 11 weeks of age demonstrated substantial growth of the left ventricle. He underwent successful repair of coarctation of the aorta and continues to do well with moderate aortic stenosis. The difficulties of predicting left ventricular growth and function are discussed, and management options are reviewed.     

Constrictive pericarditis developed after childhood repair of ventricular septal defect

The patient was an 18‐year‐old man who had undergone surgical closure of ventricular septal defect at the age of 2 years. The patient was urgently hospitalized due to syncope after longstanding uneventful postoperative course, and diagnosed with atrial flutter. Echocardiography showed poor ventricular motion and markedly dilated both atriums. After termination of atrial flutter, ventricular motion improved remarkably; however, there was little improvement in heart failure. Although we suspected constrictive pericarditis, calcification observed on computed tomography was too localized to fully explain the cause of severe diastolic dysfunction. Based on the waveform of mitral annular tissue Doppler imaging, we were strongly convinced of the underlying physiology of constrictive pericarditis. Pericardial decortications markedly improved heart failure symptoms. This case emphasizes that constrictive pericarditis could be a life‐threatening complication in the remote phase after congenital heart surgery. It also emphasizes the importance of functional assessment by echocardiography to identify the cause–effect relation between imaging and clinical symptoms.     

Physical activity levels in children and adolescents are reduced after the Fontan procedure, independent of exercise capacity, and are associated with lower perceived general health

Objectives

To determine physical activity levels in paediatric patients who underwent the Fontan procedure, and their relationship to functional status and exercise capacity.

Study Design

We studied 147 patients (ages 7–18 years) at a median of 8.1 years after Fontan, as part of the Pediatric Heart Network cross‐sectional study of Fontan survivors. Assessment included medical history, self‐reported physical activity, parent‐completed Child Health Questionnaire (CHQ), cardiopulmonary exercise testing and physical activity level measured by accelerometry (MTI Actigraph).

Results

Measured time spent in moderate and vigorous activity was markedly below normal at all ages, particularly in females, and was not significantly related to self‐reported activity levels, or to maximum Vo₂, Vo₂ at anaerobic threshold or maximum work rate on exercise testing. Lower measured activity levels were significantly related to lower perceived general health but not to self‐esteem, physical functioning, social impact of physical limitations or overall physical or psychosocial health summary scores. Reduced exercise capacity was more strongly related than measured activity levels to lower scores in general health, self‐esteem and physical functioning.

Conclusions

Physical activity levels are reduced after Fontan, independent of exercise capacity, and are associated with lower perceived general health but not other aspects of functional status.     

Persistent left superior vena cava: Experience of a tertiary health‐care center

Background: The aim of this study was to assess the prevalence of persistent left superior vena cava (PLSVC) in patients with all types of congenital heart defects and to determine the congenital heart anomalies accompanying PLSVC. Methods: The present study is based on a retrospective review of 1205 children who consecutively underwent cardiac catheterization from 2000 to 2007. In order to determine the existence of PLSVC, all the subjects routinely underwent superior vena cava (SVC) injection during angiography at the catheter laboratory of the study center. Results: The prevalence of PLSVC was computed to be 6.1% for the present study population. Transthoracic echocardiography was able to detect PLSVC in 32 children (2.6%) whereas angiography diagnosed PLSVC in 74 children (6.1%). The mean age of the patients with PLSVC was 40.09 ± 50.21 months. A communication between the right and left SVC was determined in 27% of the children who were diagnosed with PLSVC after angiography was performed (20 out of 74). A statistically significant association was present between PLSVC and other congenital cardiac anomalies, including ventricular septal defect (n= 42, 56.8%), atrial septal defect (n= 31, 41.9%), pulmonary stenosis (n= 19, 25.7%), atrioventricular septal defect (n= 10, 13.5%), patent ductus arteriosus (n= 6, 8.1%) and cor triatriatum (n= 3, 4.1%). Conclusion: Transthoracic echocardiography usually visualizes dilated coronary sinus in association with PLSVC. However, SVC injection should be performed in patients undergoing angiography so that morbidity and mortality related with persistent left superior vena cava can be avoided during cardiovascular surgery.     

Sub‐acute neonatal hemochromatosis in an infant with hypoplastic left heart syndrome on ventricular assist device awaiting transplantation

Single‐ventricle pediatric patients, amongst other children waiting for OHT, are a vulnerable population, especially if candidacy is established before any palliation. NH is a rare disease with poor prognosis in the post‐natal period. We present a case of sub‐acute NH diagnosed in an infant with HLHS who was listed for OHT while bridged with a pulsatile paracorporeal VAD, with an emphasis on the evolution of the condition throughout the patient's clinical course and the ultimate decision for compassionate deactivation of VAD.     

膜部室间隔缺损封堵术后完全性左束支传导阻滞治疗效果分析

目的探讨儿童膜部室间隔缺损(VSD)封堵术后完全性左束支传导阻滞(CLBBB)的治疗及预后。方法回顾分析2009年2月至2019年6月就诊的18例VSD封堵术后CLBBB患儿的临床资料。结果 18例接受VSD封堵术并经心电图检测确诊CLBBB患儿的平均年龄为5.69±2.33岁(3岁2个月～10岁5个月),男性11例、女性7例,随访时间中位数5年(3个月～10年)。14例无心力衰竭患儿中4例经糖皮质激素治疗CLBBB即恢复,随访3～6个月各项指标均无异常;8例随访5～10年,无不适,心电图无变化,心功能、左室射血分数、心房利钠肽均无异常,但左室舒张末期内径(LVDD)增大;2例接受封堵器取出及VSD修补术,1例术后出现完全性右束支传导阻滞,余无异常,另1例术后出现完全性房室传导阻滞,植入起搏器,随访1年QRS波时限较术前缩短,心功能无异常,LVDD增大。4例合并心力衰竭患儿中3例行心脏再同步化治疗(CRT)植入术,1例行左室起搏,术后QRS波均150 ms;1例行CRT术后3天因急性心力衰竭死亡,另3例随访1～2年心功能明显改善。结论对于VSD封堵术后早期出现CLBBB患儿,糖皮质激素可能有效,若无效,可选择手术取出封堵器,但有发生完全性房室传导阻滞的风险。若合并心力衰竭,CRT或左室起搏治疗可能有一定效果。     

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??Objective To observe the changes of left ventricular voltage??QT interval and QT interval dispersion before and after transcatheter closure for simple ventricular septal defect. Methods The ECG RV5 ??V5 lead r waves????SV1 ??V1-lead s-wave??????RV5+SV1?? voltage??QT interval and corrected QT interval??QT dispersion ??QTd?? and heart rate corrected QT dispersion ??QTcd?? were analyzed in 90 VSD children without arhythmia before transcatheter closure or 1??3??5 days and 1 month after it. All the patients were admitted between January 2014 and January 2015 in Department of Pediatrics West China Second University Hospital of Sichuan University. Results Left ventricular voltage after transcatheter closure for ventricular septal defect ??RV5+SV1?? decreased??being with statistical significance on the third day after operation when compared with the preoperative one ??P??0.05????there were significant differences in QTc before and on the fifth day after operation ??P??0.05??. QTd and QTcd one month after surgery??compared with preoperative and postoperative day??had statistically significant difference ??P ?? 0.05??. Precordial leads RV5??SV1 voltage and QT interval in the preoperative??postoperative 1??3??5 days??1 month??had no statistically significant difference??P??0.05??. Conclusion Cardiac arrhythmia doesn’t occur in simple ventricular septal defect in children after transcatheter closure??and left ventricular voltage will be gradually reduced??corrected QT interval??QTd and QTcd will be shortened.     

Impact of antenatal diagnosis of hypoplastic left heart syndrome on the clinical presentation and surgical outcomes: The Australian experience

Aim:   Antenatal diagnosis of severe congenital heart disease enables planning of perinatal care of affected infants. Congenital heart surgery is highly centralised in Australia, and surgery for hypoplastic left heart syndrome (HLHS) currently takes place at a single institution, in order to ensure case volume. The study aims to review the impact of antenatal diagnosis on the early clinical course of infants with HLHS in Australia.
Methods:   Retrospective review was performed on all neonates who were admitted for management of HLHS between 2001 and 2005 at the Paediatric Cardiac Surgical Unit, The Royal Children's Hospital, Melbourne, Australia.
Results:   Sixty neonates with HLHS were admitted, in whom an antenatal diagnosis was present in 46 (77%). Treatment was withdrawn in seven infants, of whom three had prenatal, and 4 had post-natal diagnoses. Antenatally diagnosed infants were commenced on prostaglandin earlier than post-natally diagnosed infants (age 1 h and 55 h respectively), and on paediatric intensive care unit admission had a higher pH (7.31 vs. 7.20), a lower lactate (3.0 vs. 6.7), a lower inspired oxygen fraction (0.21 vs. 0.96) and were less likely to be ventilated (10.8% vs. 92.9%). Infants with an antenatal diagnosis had lower peak creatinine (70 vs. 120) and alanine aminotransferase (29 vs. 242). The survival to intensive care discharge and stage 2 palliation was 74% and 68% respectively, and was not influenced by timing of diagnosis.
Conclusions:   Antenatal diagnosis of HLHS was strongly associated with a superior pre-operative clinical status, but did not influence early survival after surgical palliation.     

先心病血浆心钠素,环核苷酸与肺血流动力参数关系及术后变化

目的研究先心病患者外用静脉血心钠素、环核苷酸与肺血流动力参数关系及外科矫治术后的变化。方法经临床确诊的３２例先心病人，用放射免疫法同步测定血中ＡＮＰ及环核苷酸浓度。术前对２１例患儿行右心导管检查并监测其血流动力参数。结果左向右分流型先心病人，血浆ＡＮＰ、ｃＧＭＰ明显高于对照组，二者呈正相关；血浆ＡＮＰ、ｃ－ＧＭＰ与肺动脉压相关显著，而与右房、右室压无相关性。术后７～１８天血中ＡＮＰ、ｃＧＭＰ含量无明显下降。结论左向右分流的先心病人，心房扩张，血浆ＡＮＰ值升高；血浆ｃＧＭＰ浓度随ＡＮＰ的不同而变化，提示ｃＧＭＰ是ＡＮＰ的第二信使；手术前后血中ＡＮＰ、ｃＧＭＰ水平无明显差异，可能与肺动脉压及肺血管阻力尚未降至正常有关。     

功能性单心室全腔肺吻合术后运动功能评价

目的研究功能性单心室行全腔肺吻合术后的运动功能,探讨影响运动功能的可能因素。方法15例功能性单心室患儿行全腔肺吻合术后进行平板运动试验。其中男9例,女6例;平均年龄(8.6±3.9)岁;平均随访时间(29.5±20.8)个月。同期选取15例健康儿童为对照组。结果患儿运动耐量、最大心率和最高收缩压均较正常儿明显降低;血氧饱和度(SpO2)显著低于正常;代谢当量与目前年龄、术时年龄、随访时间和静息SpO2等因素无关;无运动诱发严重心律失常。结论功能性单心室行全腔肺吻合术后运动功能明显低于正常儿童。影响运动功能的主要因素可能与心内持续性右向左分流、心率变时性和心肌收缩力减弱等有关。     

High prevalence of the metabolic syndrome and associated left ventricular hypertrophy in pediatric renal transplant recipients

Wilson AC, Greenbaum LA, Barletta GM, Chand D, Lin J-J, Patel HP, Mitsnefes M. High prevalence of the metabolic syndrome and associated left ventricular hypertrophy in pediatric renal transplant recipients.
Pediatr Transplantation 2010: 14: 52–60© 2009 John Wiley & Sons A/S.
Abstract:  The goal of this study was to estimate prevalence of the metabolic syndrome and its association with cardiac abnormalities in children with kidney transplant. A multi-center retrospective review of demographic, clinical, laboratory, and echocardiographic parameters at time of and at one-yr post-transplant was conducted in 234 pediatric recipients between 2000 and 2006. Eighty-eight patients (37.6%) met criteria for metabolic syndrome. Among 55 overweight patients, 40% had metabolic syndrome. Among 51 obese patients, 74.5% had metabolic syndrome. A total of 181 (71.0%) patients had complete data at both time points: prevalence of metabolic syndrome at time of transplant was 18.8%, compared with 37.0% at one-yr post-transplant (p < 0.0001). Among 147 patients without metabolic syndrome at time of transplant, 41 (27.9%) had developed metabolic syndrome by one-yr post-transplant. A total of patients from four centers had routine echocardiogram at one-yr post-transplant. LVH was significantly more common in those with metabolic syndrome (55%) than in those without (32%) (OR 2.6, 95% CI 1.2–5.9). In conclusion: metabolic syndrome is common at time of pediatric kidney transplant, and prevalence rises sharply at one-yr post-transplant. The presence of metabolic syndrome is strongly associated with LVH in these patients.     

应用室间隔缺损封堵器治疗婴幼儿特殊形状动脉导管未闭疗效观察

目的评价室间隔缺损(VSD)封堵器治疗婴幼儿特殊形状动脉导管未闭(PDA)的临床疗效。方法分析2017年7月至2018年7月经主动脉弓降部造影提示窗型PDA或动脉导管长度7 mm管型PDA,并选择VSD封堵器进行介入治疗的27例患儿的临床资料。结果 27例患儿中窗型PDA 12例,男7例、女5例,中位年龄8.7个月(6个月～3岁);管型PDA 15例,男6例、女9例,中位年龄18个月(11个月～5岁)。窗型PDA选择膜部VSD封堵器,管型PDA选择肌部VSD封堵器封堵;按照窗型PDA肺动脉端内径加3～6 mm、管型PDA肺动脉端内径加2～4 mm选择封堵器腰部大小。所有患儿均成功封堵,术后均无残余分流。术后24小时、1个月、3个月、6个月、12个月复查胸片提示封堵器位置良好。术后24小时、1个月、3个月、6个月、12个月降主动脉流速均较术前明显降低,差异有统计学意义(P0.05);术后左肺动脉流速较术前无变化,差异无统计学意义(P0.05)。结论使用VSD封堵器介入治疗特殊形状PDA是一种有效可行的治疗方法。     

法洛四联症根治术后右心室功能的影响因素和评估策略

法洛四联症根治术技术已成熟,在临床应用至今已经50余年.从远期预后来看,猝死、右心扩张及充血性心力衰竭、运动能力下降等现象普遍存在,这些均与右心功能损伤有关.该文主要概括了法洛四联症根治术中有关右心室功能影响因素、随访和评估手段的研究进展.