射频消融法洛四联症根治术后室性心动过速二例

患者1,女性,18岁.8岁时行法洛四联症根治术,术后6个月出现阵发性宽QRS波心动过速.入院检查:心电图示窦性心律,完全性右束支阻滞(图1A、B);X线胸片为肺动脉段饱满,心胸比0.66;超声心动图为右心室28mm,右心房、室扩大,心室水平分流消失,左心室43 mm,射血分数(EF)0.54.

小儿法洛四联症修复手术减少肺动脉瓣反流的外科策略及效果

目的 探讨小儿法洛四联症(TOF)修复手术中减少肺动脉瓣反流的治疗策略和效果.方法 2009年1月至2015年12月,211例TOF患儿行肺动脉瓣保护(PVS)的心内修复手术,年龄2～ 36个月,其中术后右心室和左心室压(PRV/PLV)比值≥0.8者114例.114例中前54例全部在体外循环心脏跳动下采用右心室漏斗部保护方法(RVIS),切开肺动脉瓣环或右心室0.5～1.0 cm;后60例中8例采用以下标准决定行RVIS:多巴胺≥10 μg·kg-1·min-1和肾上腺素≥0.05 μg·kg-1·min-1维持下循环不稳定或者跨肺动脉瓣环压差≥30 mmHg(1mmHg =0.133 kPa),余52例未进一步处理.114例患儿按是否行RVIS分组,其中RVIS 62例,PVS组52例,比较两组的临床结果.结果 RVIS组体外循环时间明显延长[(110.3±12.0) min对(77.7±10.0) min,P＜0.01].与RVIS组相比,PVS组术后跨肺动脉瓣环压差[(21.0±5.4)mmHg对(16.0±3.6)mmHg,P＜0.05]、PRV/PLV(0.82 ±0.03对0.67±0.12,P＜0.01)明显高,PVS组术后早期血管活性药物(多巴胺和肾上腺素)的使用时间[(6.03±9.60)天对(4.20±1.90)天,P＜0.01]、呼吸机辅助时间[(81.2±27.6)h对(38.5±33.0)h,P＜0.01]和ICU滞留时间[(6.3±1.7)天对(4.3±1.9)天,P＜0.01]明显长.PVS组术后床旁腹膜透析比例明显高于RVIS组(8/52对4/62,P＜0.01).RVIS组术后1例死于心律失常.随访期间PVS组1例死于感染性心内膜炎.随访资料显示,术后52个月PVS组的肺动脉瓣反流程度明显小于RVIS组,两组的左、右心室功能差异无统计学意义,但是都明显低于健康同龄儿.结论 选择合适的TOF病例,采用保留肺动脉瓣或右心室小切口,能明显减轻修复术后肺动脉瓣的反流.

外科治疗法洛四联症33例临床体会

1996年6月至2006年6月,我院胸心外科共手术治疗法洛四联症(TOF)患者33例,取得较为满意的临床效果,现报道如下.

TOF is generally accepted as the index lesion used for evaluating the overall quality of a congenital cardiac program. To achieve good results, the evaluation and management of TOF requires an intimate collaboration between all members of the cardiac team involved in the care of these children, especially the interventional catheterizer and the surgeon. Each program has to develop its own approach, relying on the collective strengths of their staff. Any weak link in this chain will often have a profoundly negative effect upon the patient, which may be difficult to correct later.

小儿法洛四联症围术期循环和血气的临床观察

法洛四联症(F4)是威胁小儿生命的主要先天性心脏病之一,随着外科技术和麻醉管理水平的提高,围术期F4的矫治手术死亡率明显下降.

Background: Abnormal expression of connexin 43 (Cx43) has been reported to play an important role in the development of conotrunccal anomalies. However, less is known about the underlying reason for its abnormal expression. MicroRNAs (miRNAs), as an important part of gene expression regulation, have been implicated in some cardiac diseases. This study aimed to investigate the expression of Cx43 and its related miRNAs in patients with tetralogy of Fallot (TOF), and illustrate the potential role of abnormal miRNAs regulation to Cx43 expression in the pathology of TOF.
Methods: Real-time polymerase chain reaction was used to detect the expression of Cx43 and 10 Cx43-related miRNAs in the myocardium from 30 TOF patients and 10 normal controls. Immunohistochemistry was used to detect Cx43 protein expression. Putative miRNA binding sites in the 3'UTR of Cx43 were examined in 200 TOF patients and 200 healthy individuals, using Sanger sequencing, to exclude sequence variations resulting in binding diffi culties of miRNAs.
Results: Cx43 mRNA and protein expression in the myocardium tissue was significantly increased in TOF patients. The expression of MiR-1 and 206 was significantly decreased in the TOF patients as compared with the controls (P<0.05). No obvious difference was observed in the expression of the other 7 miRNAs between the TOF patients and controls (P>0.05). No meaningful sequence variation was detected in the putative miR1/206 binding sites in the 3'UTR of Cx43.
Conclusions: This study indicated that miR-1 and 206 is down-regulated in TOF patients, which may cause an up-regulation of Cx43 protein's synthesis. It provided a clue that miR-1 and 206 might be involved in the pathogenesis of TOF, additional experiments are needed to determine if in fact, miR-1 and 206 contribute substantially to TOF.