小儿法洛四联症根治术后早期死亡的危险因素分析

目的 探讨小儿法洛四联症(tetralogy of Fallot,TOF)手术后早期死亡的危险因素,以提高手术成功率,降低手术病死率.方法 2003年1 月至2010年12月期间在成都心血管病医院行TOF根治术患儿191例,其中男142例,女49例;年龄4个月～12岁.收集所有患儿术前、术中和术后的临床资料,分析影响TOF手术后早期死亡的危险因素.结果 191例患儿,围术期死亡6例(3.14％),死于手术后感染合并多器官功能衰竭3例,低心排血量综合征2例,脑部并发症1例.其中≤6个月患儿病死率6.67％(2/30),6个月～3岁患儿病死率1.41％ (1/71),3～12岁患儿病死率3.33％ (3/90).多因素Logistic回归分析结果表明,年龄≤6个月(OR=4.606,95％CI 1.811 ～11.719,P＜0.05)、术前血氧饱和度＜70％(OR=0.982,95％ CI 0.501 ～ 1.932,P＜0.01)、Nakata指数＜140mm2/m2(OR=16.960,95％CI 1.414 ～ 150.390,P＜0.01)、体外循环时间＞150 min(OR=4.398,95％CI2.091 ～9.216,P＜0.01)及术后多器官功能衰竭(OR=4.872,95％CI 2.583 ～ 9.192,P＜0.05)是小儿TOF根治术后早期死亡的危险因素.结论 临床上可根据患儿年龄、术前血氧饱和度、Nakata指数、体外循环时间及术后并发症预测TOF根治术后早期死亡的危险性.     

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.     

选择性心血管造影检查小儿法洛四联症伴随畸形：附384例报告

为探讨法洛四联症患儿的伴随畸形。提高手术成功率，我们于1993年2月-1999年7月对384例法洛四联症患儿行心导管及选择性心血管造影检查。结果表明166例（43.2%)法洛四联症伴有周围肺动脉狭窄。冠状动脉畸形，左室发育不良、完全性房室隔缺损等多种伴随畸形。为此作者认为，法洛四联症伴随畸形并不少见。而且部分伴随畸形对手术治疗及预后估测至关重要，因此对法洛四联症行心血管造影检查十分必要。     

法洛四联症产前心脏超声评估现状

法洛四联症是最常见的青紫型先天性心脏病,随着产前筛查技术的提升,产前诊断率明显升高,法洛四联症的产前评估需求亦日益增加。心脏超声可以评估法洛四联症胎儿的心脏畸形程度,在产前评估中具有独特的优势及价值,是法洛四联症产前评估的重要手段。文章总结了心脏超声技术在法洛四联症胎儿评估中的应用及进展。     

法洛四联症根治术后远期心律失常的随访

目的　了解法洛四联症 (TOF)根治术后远期心律失常的发生率以及影响心律失常发生的相关因素。方法　 54例法洛四联症根治术后患儿 ,男 3 5例 ,女 19例 ;年龄 5～ 14岁 [( 9 4± 2 5)岁 ]。术时年龄 17个月～ 117个月 ,随访年限 2 8～ 9 0年。进行了心电图、2 4小时Holter、二维超声心动图和活动平板运动试验检查。结果　 18例患儿存在不同程度的室性心律失常 ,室性心律失常的发生率为 3 3 % ,其中 1例患儿有短阵室性心动过速发生。 4例右室收缩压增高者均存在明显室性心律失常 ,右室收缩压正常者室性心律失常发生率为 2 8% ( 14 / 50例 ) ,两者差异有显著性 (P =0 0 1)。体外循环时间≥ 90min者室性心律失常发生率为 53 % ( 10 / 19例 ) ,体外循环时间 <90min的患儿室性心律失常发生率为 2 3 % ( 8/ 3 5例 ) ,两者差异有显著性 (P <0 0 5)。轻度肺动脉瓣返流组室性心律失常发生率为 2 1% ( 4 / 19例 ) ,中、重度肺动脉瓣返流组室性心律失常的发生率为 40 % ( 14 / 3 5例 ) ,两组差异无显著性 ( χ2 =1 989,P >0 0 5)。随访年限 5年以内室性心律失常发生率为 3 2 % ( 8/ 2 5例 ) ,5年以上患儿的室性心律失常发生率为 3 4 % ( 10 / 2 9例 ) ,两者差异无显著性 ( χ2 =0 0 3 7,P >0 0 5)。手术年龄     

Aortic valve injury 10 years after tetralogy of Fallot repair

Summary Although recognized by pediatric cardiac surgeons, aortic insufficiency as a technical complication after tetralogy of Fallot repair is poorly documented, especially if it occurs late. The case of a boy with aortic insufficiency 10 years after complete tetralogy repair is described. No documentation in the literature other than summary statements in textbooks of this occurence was found.     

经皮球囊肺动脉瓣成形术姑息治疗法洛四联症13例疗效评价

应用经皮球囊肺动脉瓣成形术(PBPV)姑息治疗法洛四联症13例,其中男8例,女5例;平均年龄(3．7±2．1)岁。SaO     

Haemoptysis: A case report with tetralogy of Fallot with absent pulmonary valve leaflets and anomalous origin of left pulmonary artery

A 6 month old female child with tetralogy of Fallot with absent pulmonary valve leaflets and anomalous origin of left pulmonary artery was admitted to hospital with a mild adenoviral lower respiratory tract infection. She had a large haemoptysis 1 week after admission, and recovered with simple supportive measures. This case illustrates a previously unreported complication of a rare anomaly.     

Mechanism of aortic root dilation and cardiovascular function in tetralogy of Fallot

The aortic root dilation in tetralogy of Fallot (TOF) is a long‐term clinical problem, because a severely dilated aorta can lead to aortic regurgitation, dissection, or rupture, which can be fatal, necessitating surgical intervention. The details of the mechanism of aortic root dilation, however, are unclear. We have shown that aortic stiffness is increased in patients with repaired TOF, and may mirror the histological abnormality of elastic fiber disruption and matrix expansion. This aortic stiffness is related closely to the aortic dilation, indicating that aortic stiffness may be a predictor of outcome of aortic dilation. Furthermore, the aortic volume overload is a very important determinant of aortic diameter in TOF patients before corrective surgery. In addition, a chromosomal abnormality and the transforming growth factor‐β signaling pathway, a major contributor to aortic dilation in Marfan syndrome, also affect this mechanism. In this way, aortic dilation in TOF patients is suggested to be a multifactorial disorder. The aim of this review was therefore to clarify the mechanism of aortic dilation in TOF, focusing on recent research findings. Studies linking histopathology, mechanical properties, molecular/cellular physiology, and clinical manifestations of aortic dilation facilitate appropriate treatment intervention and improvement of long‐term prognosis of TOF.     

Longitudinal psychological assessment in tetralogy of Fallot

Summary To determine whether preoperative psychological testing in children with cyanotic heart disease could provide an accurate estimate of adult performance, we studied 21 subjects (mean age, 30.3±4.8 years) who had undergone surgical repair for tetralogy of Fallot between 1958 and 1966 (mean age, 8.7±3.9 years); 17 received childhood preoperative testing (mean age, 8.9±4.2) and four had early postoperative testing. Follow-up psychological tests administered in 1983 included Wechsler Adult Intelligence Scale Revised, Jackson Personality Research Form E, Rosenberg Self-Esteem Scale, and a questionnaire-interview on educational, occupational, and psychosocial status. Adult IQ (x=93.4±15.6) and child IQ (x=97.5±14.6) scores were highly correlated (r=0.87,p<0.01), revealing considerable stability across 22.3±2.2 years. On 17 of 22 Jackson personality scales, the subjects (S) were within the normal range. Scoring below the 16th percentile were 11/21 S on intellectual curiosity, 9/21 S on adaptability to change, and 8/21 S on leadership skills. Scoring above the 84th percentile were 8/21 S on harm avoidance and 8/21 S on giving nurturance to others. Univariate one-way ANOVAs indicated that those subjects with the higher IQ scores had the higher incomes, job levels, and educational attainments. We conclude that the results of perioperative psychological testing in children with tetralogy of Fallot are congruent with postoperative adult intellectual and psychosocial status.     

尼莫地平对法洛四联症患儿心肌细胞内钙运转及心功能的影响

目的　研究尼莫地平对法洛四联症（ＴＯＦ）患儿心肌细胞钙转运和心功能的影响。方法　２７ 例ＴＯＦ分为对照组（１２例）和治疗组〔１５ 例,尼莫地平１．０～１．５ｍ ｇ／（ｋｇ·ｄ）×１４ｄ〕。测定治疗前后心肌细胞内外钙浓度和心功能。结果　用尼莫地平后治疗组红细胞游离钙和总钙浓度明显降低,钠泵活性升高,而钙泵活性无变化;心肌细胞游离钙（２０４±３３ｎｍ ｏｌ／Ｌ）较对照组（３４７±８５ｎｍ ｏｌ／Ｌ）明显降低（Ｐ＜ ０．０１）;心肌细胞线粒体和内质网肿胀明显减轻或消失,线粒体密度升高;心脏指数和左右心室充盈功能明显改善。结论　尼莫地平可明显减轻ＴＯＦ患儿心肌细胞内外的钙超载,同时亦可改善心功能     

法洛四联症术后右室限制性生理临床转归的研究

目的 研究法洛四联症(tetralogy of Fallot,TOF)术后右室限制性生理临床转归,探讨TOF术后右室限制性生理发生的危险因素、评估指标和转归情况.方法 2011年10月至2012年5月在上海儿童医学中心行TOF根治术的80例患儿随机入选本次研究,通过床边心脏彩超明确30例患儿发生了右室限制性生理.统计患儿围手术期的临床资料,记录患儿最后一次超声随访的结果,比较患儿的右室流出道梗阻情况、肺主动脉梗阻情况、肺动脉分支狭窄情况和肺动脉反流情况.结果 右室限制性生理组患儿比非右室限制性生理组的年龄更小,体外循环时间、主动脉阻断时间、机械通气时间、重症监护时间和住院时间更长(P均<0.01).Logistic多元回归分析表明,术前年龄小(OR=0.48,P=0.01)、术中体外循环时间长(OR=1.7,P=0.04)是患儿发生右室限制性生理的危险因素.术后1周内,右室限制性生理组静脉血氧饱和度的上升和氧摄取分数的下降程度均小于非右室限制性生理组(P均<0.05).右室限制性生理组乳酸水平高于非右室限制性生理组(P=0.03).C-反应蛋白在术后2d内先上升,3~7d内下降,且右室限制性生理组下降的速度要慢于非右室限制性生理组(P=0.08).右室限制性生理组氨基末端脑钠肽(N-terminal pro-BNP,NT-proBNP)术后1周内显著升高, NT-proBNP≥4750pg/ml 提示患儿发生了右室限制性生理.中期随访结果表明两组患儿右室流出道梗阻、肺主动脉梗阻、肺动脉分支狭窄的发生率无明显差别,肺动脉反流程度也无明显差别(P均>0.05).结论 TOF术后发生右室限制性生理与手术年龄和体外循环时间相关,NT-proBNP可作为评估TOF术后患儿发生右室限制性生理的指标.术后早期右室限制性生理的发生在一定程度上会影响患儿转归,然而随访数据表明右室限制性生理对患儿中期转归无明显影响.     

Hemostatic management of an infant with severe hemophilia A and tetralogy of Fallot for cardiac bypass surgery

We present the hemostatic management of severe hemophilia A of an infant with high risk of inhibitor development who underwent cardiac surgery for correction of tetralogy of Fallot. Continuous infusion of recombinant factor VIII resulted in good hemostasis during surgery and postoperatively. Unfortunately, the patient had inhibitor development 2 months after cardiothoracic surgery. Although cardiac surgery is successful, exposure to factor concentrates in severe hemophilia early in life might predispose a patient to inhibitor development.     

The use of pethidine as pre-medication to prevent anoxic spells in patients with tetralogy of Fallot

OBJECTIVE: The aim of this study was to determine whether morphine-HCl or pethidine-HCl differ as pre-medication to prevent anoxic spell in patients with tetralogy of Fallot (TOF) at catheterization. METHODS: A 3 year retrospective case notes review study of 46 consecutive patients who underwent cardiac catheterization from 1998 to 2001 was conducted. In a prospective 1 year randomized control study, 10 patients diagnosed as TOF and admitted to Tokyo Women's Medical University, The Heart Institute of Japan, Tokyo, in 2002 were randomized to receive morphine or pethidine as pre-medication for cardiac catheterization. The incidence of anoxic spell before and after cardiac catheterization was investigated in both studies. RESULTS: In the case notes review, a total of 10 out of the 46 patients showed anoxic spell study: eight out of 24 patients in the morphine group and two out of 22 in the pethidine group. The difference in the incidence was statistically significant (P < 0.05, Fisher's exact test). Four patients in the prospective study showed anoxic spell: three out of six in the morphine group and one out of four in the pethidine group (odds ratio = 3.0). Overall, 14 out of 56 patients had anoxic spell: 11 out of 30 in the morphine group and three out of 26 in the pethidine group (P = 0.03). CONCLUSION: The incidence of hypoxic spells in patients with TOF undergoing cardiac catheterization is reduced by the use of pethidine-HCl as pre-medication compared to morphine. This has important clinical implications.     

Prognosis in fetal tetralogy of Fallot

Summary The outcome in a series of 23 cases of tetralogy of Fallot diagnosed prenatally was compared to published figures for this condition when (a) identified postnatally or (b) treated surgically. There was a marked difference in the survival between these groups, with the highest mortality (75%) occurring when the diagnosis had been made prenatally. The high incidence of chromosomal and extracardiac anomalies in this group (60%) largely accounted for the discrepancy. This, and the potential for progressive changes in the anatomy of the defect during pregnancy, must be taken into account by the pediatric cardiologist offering prognosis in early pregnancy.     

小儿法洛四联症根治术106例临床分析

目的探讨小儿法洛四联症根治术的特点,以提高手术疗效。方法对1998-02—2005-07在河南省郑州市第七人民医院接受法洛四联症根治术的106例患儿的临床资料进行回顾性分析。结果治愈103例,死亡3例,病死率为2.9%。术后随访率100%,随访3～93个月,无晚期死亡。全部病例心功能皆恢复至Ⅰ或Ⅱ级(NYHA)。结论绝大部分法洛四联症患儿可施行一期根治术,提高手术成功率的关键在于合理的体外循环灌注、严密修补室间隔缺损和恰当疏通右室流出道。     

Left and right ventricular volume characteristics in tetralogy of Fallot and their relationship to arterial oxygen saturation and age

Left (LV) and right ventricular (RV) volume characteristics in 43 patients with tetralogy of Fallot (TOF) undergoing no prior surgical intervention, aged 3–50 months, were evaluated. The control group consisted of 45 patients with Kawasaki disease without cardiac lesions, aged 12–82 months. The TOF patients were divided into four groups: those having arterial oxygen saturation < 80% with an age at the time of study < 18 months (group 1a) or with that ≧ 18 months (group 1b), and those with arterial oxygen saturation ≧ 80% with an age < 18 months (group 2a) or with that ≧ 18 months (group 2b). The results were compared with those in control subjects. In group 1a, each of LV end-diastolic volume (EDV), LV ejection fraction (EF), RVEDV and RVEF was reduced. In group 1b, LVEDV, LVEF and RVEF were decreased. In groups 2a and 2b, RVEF alone was depressed. From these results, the severity of hypoxemia was an important risk factor for ventricular dysfunctions. No influence of age on the volume characteristics was found. The investigations suggested that patients with TOF having an arterial oxygen saturation < 80% are probably candidates for early surgical intervention.     

Spondylocostal dysostosis with tetralogy of Fallot and herniation of the spleen through the diaphragm

Spondylocostal dysostosis (SCD) is a very rare syndrome characterized by vertebral malformation and rib deformity. Some of the patients with SCD have other birth defects in the central nervous system, the genitourinary tract, diaphragm or heart and so forth. There have been reported SCD with complex congenital heart disease, such as pulmonary atresia, double outlet right ventricle, and d‐transposition of great arteries. However, there have been no reported SCD patients with confirmed tetralogy of Fallot (TOF). Here, a patient with SCD having a very rare combination of rib defects on the right side and left‐sided scoliosis, tetralogy of Fallot, and diaphragmatic spleen herniation, which had not been reported before, was described.