Double Outlet Right Ventricle with Intact Atrial Septum and Restrictive Ventricular Septal Defect: An Analysis of Two Cases

Two cases of double outlet right ventricle with restrictive ventricular septal defect are described. This is an uncommon presentation that causes left ventricular dysfunction because of left ventricular outflow tract obstruction. The presence of an intact atrial septum leads to severe pulmonary hypertension, which tends to aggravate the right ventricular output. In the presence of a normal left ventricle, the authors suggest the possibility of enlargement of the ventricular septal defect in order to perform a biventricular repair. The association of a supramitral valve ring in both cases, and the isolation of the left subclavian artery and an aortopulmonary fenestration in one of these cases, are also discussed. In addition we explore factors that cause restrictive ventricular septal defects as well as the mechanisms that may lead to spontaneous closure of ventricular septal defect in a double outlet right ventricle.     

浅析心肌肥厚患者左室舒张功能与冠脉循环的关系

目的探讨心肌肥厚患者冠脉血流与左室舒张功能障碍关系。方法采用彩色多普勒超声仪,行经胸超声心动图(TTE)测量40例心肌肥厚患者和20例正常人冠状窦、二尖瓣及肺静脉血流频谱。结果心肌肥厚患者冠状窦直径、前向血流和净前向血流流速积分(VTInet)明显高于正常对照组;冠状窦前向血流和VTInet与左室舒张功能障碍的多项指标存在相关关系;肺静脉舒张末期逆行血流最大流速之间均存在明显正相关。结论冠脉循环血流动力学的改变可能是心肌肥厚患者舒张功能受损的机制之一.     

心脏扭转运动的研究进展

心脏扭转运动形式不但影响心脏正常的收缩功能,而且还能促进舒张早期心室充盈。不正常的扭转运动形式,提示心肌结构和功能改变。因此心脏扭转角度是临床一个非常有价值的诊断指标。本文就心脏扭转的解剖基础、检测方法及其意义作一综述。     

磁共振成像(T2*)检测重型β-地中海贫血患儿心脏、肝脏铁负荷及左心室射血分数的研究

目的观察我院重型β-地中海贫血(β-TM)患儿血清铁蛋白(SF)和体内铁沉积状况及对心脏功能的影响。方法检测我院28例重型β-TM患儿SF水平,并由我院放射科运用磁共振成像T2*(MRI T2*)技术检测心脏及肝脏铁沉积状况及左室射血分数(LVEF)。结果本组28例患儿中男16例,女12例,中位年龄11.2岁(7岁1个月~16岁11个月)。28例患儿中SF轻度升高者4例(14.3%),中度升高者8例(28.6%),高度升高者16例(57.1%)。肝脏MRI T2*检测结果 :肝铁轻度沉积2例(7.1%),中度沉积8例(28.6%),重度沉积18例(54.8%);心脏MRI T2*检测结果 :心铁正常16例(57.1%),轻度铁沉积4例(14.3%),中度铁沉积4例(14.3%),重度铁沉积4例(14.3%);LVEF降低者19例(67.9%),4例心脏重度铁沉积患儿中有3例(75%)LVEF明显降低(≤55%)。本组患儿SF水平与肝脏T2*值(r=-0.467,P=0.036)、心脏T2*值(r=-0.486,P=0.01)呈明显负相关;SF与输血量呈正相关(r=0.634,P=0.043;r=0.067,P=0.124)而与去铁治疗呈负相关(r=-0.526,P=0.043),SF和心脏T2*值与LVEF均无相关性(r=-0.154,P=0.306;r=0.067,P=0.124)。结论重型β-TM患儿SF增高及肝脏重度铁沉积发生率高,虽然心脏重度铁沉积患儿发生率不高,但心脏重度铁沉积患儿多有LVEF明显降低。     

Single-stage off-pump repair of coarctation of the aorta and ventricular septal defects in children

Open in a separate window OBJECTIVESThe appropriate approach for surgical repair of coarctation of the aorta with a ventricular septal defect (VSD) remains controversial. This study evaluated the outcomes of primary repair of VSDs with periventricular device closure without cardiopulmonary bypass through a left thoracotomy in patients without arch hypoplasia.METHODSWe selected 21 patients aged <1 year, including 7 neonates, who underwent repair of coarctation of the aorta with periventricular device closure of a VSD.RESULTSThe median occluder size was 6 (range, 5–8) mm. The median mechanical ventilation time was 14 (range, 2–68) h, and the median duration of hospital stay was 11 (range, 7–16) days. No reoperations were required to correct VSD shunting, and the median residual shunt size was 1 (range, 1–2) mm. The median follow-up period was 13 (range, 4–31) months. No late deaths were reported, and no haemodynamically significant pressure gradient at the anastomotic site was observed. The median distal aortic arch z-score was 0.39 (range, −0.1–to 0.9). Only 1 patient had a permanent pacemaker implanted towards the end of the follow-up period.CONCLUSIONSPeriventricular device closure can be used safely for closure of VSD in children with coarctation of the aorta without a hypoplastic aortic arch, even in neonates, to reduce the risk of prolonged cardiopulmonary bypass. This hybrid approach can be performed with a low incidence of rhythm disturbances and residual shunting. However, a meticulous assessment of the VSD anatomy is essential to avoid any unfavourable events.     

Ablación de taquicardia ventricular asistida con ECMO. Primera experiencia en nuestra institución

Catheter ablation is useful for reducing drug refractory ventricular tachycardia (VT) episodes and can be life-saving when VT is incessant or arrhythmic storm. Left ventricular hemodynamic support may be required in patients with VT and hemodynamic instability. Extracorporeal membrane oxygenation (ECMO) support is an alternative to achieve ventricular tachycardia mapping and ablation over long periods of time. We present a case of successful catheter ablation of substrate in a patient with ischemic heart disease and ventricular tachycardia with hemodynamic instability performed using venous- arterial ECMO support. There were not episodes of ventricular tachycardia after 2 years of follow-up.     

利多卡因在搭桥术中抑制再灌注室颤研究

目的　研究冠脉搭桥手术中 ,开放升主动脉时注入 1 0 0mg利多卡因是否有利于避免再灌注室颤。方法　将 30例CAD患者随机分成 2组 :利多卡因组 (L组 )和对照组 (C组 )。L组 1 5名患者于升主动脉开放前 1分钟给予 1 0 0mg利多卡因 ,对照组给予 5ml的生理盐水 ,通过swan—ganz导管监测全组病人麻醉诱导后以及脱离体外循环后的血流动力学指标。结果　①L组的再灌注室颤的发生率 (1 7% )明显低于对照组 (5 8% )。 (P〈0 .0 5 )。②两组患者应用血管活性药物 ,没有明显差异。③与对照组比较利多卡因在脱离体外循环后心排量明显增加。 (P〈0 .0 1 )。结论　在开放升主动脉时注入利多卡因有利于防止再灌注室颤的发生     

The ‘beating-heart butterfly technique’ for repair of basal post-infarction ventricle septum defect

Ischaemic ventricular septal defect is a serious complication of acute myocardial infarction with poor outcome. We present the ‘beating-heart butterfly’ technique to close the ventricular septal defect with a double-layered pericardial patch sewn to the intact septum under beating-heart cardiopulmonary bypass in 4 highest-risk patients. This technique combined with a liberal postoperative mechanical circulatory support and open-chest treatment allowed excellent results with 12 months of survival in all patients.     

经导管射频消融治疗室性心律失常的疗效观察

目的 探讨经导管射频消融治疗特发性室性心动过速及频发室性期前收缩的疗效及安全性.方法 141例特发室性心动过速及频发室性期前收缩患者(男80例,女61例)采用激动顺序标测和(或)起搏标测方法行导管射频消融治疗.结果 消融成功128例,成功率为90.8%,起源于右室流出道的室性心动过速或室性期前收缩消融成功率为92.0%.20例患者行消融前后的Hoher检查,术前平均(21 824±12 769)次/24 h,术后平均(1 548±2 926)次/24 h,二者间差异有统计学意义(P<0.001).随访3～36个月,10例复发并全部再次消融成功.无并发症发生.结论 导管射频消融治疗症状严重且药物治疗无效的特发性室性心动过速或频发室性期前收缩是安全、有效、可行的方法.     

Refractory hypertensive emergency associated with complete heart block resolved after permanent pacemaker implantation: A case report

Severe hypertension sometimes improves with treatment of bradycardia but this phenomenon is under‐reported. Here, an elderly gentleman with complete heart block and a hypertensive emergency was refractory to medical therapies and blood pressure only improved following pacemaker implantation. We discuss the possible mechanisms relating to heart rate and artificial pacing.