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.     

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.     

Relation between age at surgery and regression of right ventricular hypertrophy in tetralogy of Fallot

Pre- and postoperative echocardiographic right ventricular wall thickness (RVW), transverse dimension (RVD), and their ratio (W/D) were measured from subcostal views in 29 patients with Tetralogy of Fallot (TOF) in order to assess whether primary reparative surgery without prior palliative shunts in early infancy results in significantly faster regression of RV hypertrophy than repair later in life.Fourteen patients who were repaired before 6 months of age were compared with 15 patients who were repaired after 6 months of age. In the group of patients who were repaired before 6 months of age both RVW and W/D ratio decreased significantly whereas RVD did not change from pre- to postoperative status. The group of patients who were repaired after 6 months of age showed no significant changes in RVW, RVD, or W/D ratio.These findings suggest that early primary reparative surgery of TOF may have a positive effect on faster regression of RVH during the first postoperative year which may reduce the detrimental effect of longstanding hypertrophy on myocardial function and the potential for arrhythmia.     

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。     

Radionuclide evaluation of right ventricular wall motion after surgery in tetralogy of Fallot

Summary Regional wall motion patterns in tetralogy of Fallot and its postoperative modifications by electrical and hemodynamic factors were assessed by Fourier analysis of gated radionuclide angiograms in 24 studies performed in children after surgical correction of tetralogy of Fallot. The range of right ventricular (RV) phase angles (standard deviation of the peak [SDP] of RV) as well as the difference between RV and LV (ΔMPh) were used as indices of the synchronicity of wall motion and were correlated with RV apical electrical activation time determined by endocardial electrical mapping. Postoperative studies were divided into two groups according to apical activation: (a) those involving right bundle branch block (RBBB) (nine patients), and (b) those involving distal RBBB (15 patients). ΔMPh was longer in proximal than in distal RBBB. Best discrimination between the two groups was obtained with SDP of RV (proximal=24°±3°, and distal=17°±2°;p<0.0001). These results showed that the range of ventricular phases measured by the SD of the phase distribution of the right ventricle is a good index for distinguishing between proximal and distal RBBB after cardiac surgery.     

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.     

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.     

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.     

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.     

Sleeping position, oxygen saturation and lung volume in convalescent, prematurely born infants

OBJECTIVE: To determine whether the effects of sleeping position on lung volume and oxygenation are influenced by postmenstrual age (PMA) and oxygen dependency in convalescent prematurely born infants. DESIGN: Prospective study. SETTING: Tertiary neonatal unit. PATIENTS: 41 infants (21 oxygen dependent), median gestational age 28 weeks (range 24-31 weeks) and birth weight 1120 g (range 556-1780 g). INTERVENTION: Infants were studied both supine and prone at two-weekly intervals from 32 weeks' PMA until discharge. Each posture was maintained for 1 h. MAIN OUTCOME MEASURES: Pulse oximeter oxygen saturation (Spo(2)) was monitored continuously, and at the end of each hourly period functional residual capacity (FRC) was measured. RESULTS: Overall, lung volumes were higher in the prone position throughout the study period; there was no significant effect of PMA on lung volumes. Overall, Spo(2) was higher in the prone position (p = 0.02), and the effect was significant in the oxygen-dependent infants (p = 0.03) (mean difference in Spo(2) between prone and supine was 1.02%, 95% CI 0.11% to 1.92%), but not in the non-oxygen-dependent infants. There was no significant influence of PMA on Spo(2). CONCLUSION: In the present study, prone sleeping did not improve oxygenation in prematurely born infants, 32 weeks' PMA or older and with no ongoing respiratory problems. However, the infants were monitored in each position for an hour, thus it is recommended that oxygen saturation should continue to be monitored after 32 weeks' PMA to be certain that longer periods of supine sleeping are not associated with loss of lung volume and hypoxaemia.     

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     

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.     

Left ventricular contractile state after surgical correction of tetralogy of Fallot: Risk factors for late left ventricular dysfunction

Summary The purpose of this study was to analyze potential risk-factors for late left ventricular dysfunction after surgical correction of Fallot's tetralogy (FT). As the ejection-phase indices cannot distinguish abnormalities of contractility from altered loading conditions, the slope values of the end-systolic pressure-length and stress-shortening relationships were analyzed by increasing afterload. Thirty-two patients were studied after surgical correction of FT in infancy. The age at investigation was 19.2±5.6 years, total correction had been performed at the age of 7.7±3.3 years. In 20 patients a one-stage operation was performed, and in 12 patients a two-stage correction. The control group consisted of 30 healthy volunteers, aged 18–30 years. The following potential risk factors for left ventricular dysfunction were evaluated: one-stage vs. two-stage correction, age at total correction, preoperative systemic oxygen saturation, preoperative hematocrit, occurrence of hypoxic spells, preoperative ratio of left-to-right ventricular peak systolic pressure, and preoperative ratio of left-to-right ventricular end-diastolic volume. In most patients the baseline data for end-systolic wall stress lay outside the normal range, indicating abnormal loading conditions. Thus, analysis of load-independent indices of the contractile state seems to be mandatory in these patients. Our data show that the severity of preoperative hypoxemia is an important risk factor for late dysfunction of the left ventricle (p<0.01). Additionally, the relation of left and right ventricular peak systolic pressures and enddiastolic volumes were related to the contractile state (p<0.01). No influence of preoperative hypoxic spells, the need for a palliative aortopulmonary shunt, or the age at surgical correction on the postoperative contractile state was demonstrated. The latter may have been due to the fact that none of the patients were operated on within the first 2 years of life.     

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.     

法洛四联症右心室流出道心肌的超微结构及力学特性

目的 研究法洛四联症（ＴＯＦ）右心室流出道心肌细胞的超微结构变化及心肌组织的生物力学性能。方法 对２４例ＴＯＦ根治术手术前后的右心室及肺动脉的压力变化进行对比研究。对其中７例ＴＯＦ与对照组７例先心病小室缺、小房缺应用透射电镜进行右心室流出道心肌的超微研究。对４例ＴＯＦ与２例非先心病死亡右心室心肌进行多样本量的对比,采用计算机控制的小力学量软组织力学测试症台进行右心室心肌力学的研究。结果 ＴＯＦ右心     

法洛四联症右室流出道心肌超微结构特点

目的　研究法洛四联症 (TOF)右室流出道心肌细胞的超微结构变化及年龄特点。方法　取符合电镜观察条件的小儿TOF 13例 ,对照组为同期年龄匹配的房间隔缺损、室间隔缺损患儿 7例。用透射电镜进行右室流出道心肌超微结构观察。结果　对照组可见心肌的肌原纤维及线粒体结构基本正常。TOF右室流出道的心肌存在很多超微结构异常的心肌细胞 ,主要表现为心肌纤维排列紊乱 ;在心肌纤维内 ,肌原纤维排列方向紊乱 ,丧失同向走行的性质 ;肌膜下方及肌纤维间异常地积聚着大量形态各异的线粒体及线粒体变性 ;部分肌节失去正常结构而出现长度不等及不全肌节 ;Z线改变明显 ;心肌细胞的固有极性被破坏等。上述异常变化随着年龄增加 ,尤其是 1岁后心肌细胞的超微结构异常明显加重。结论　 1.TOF右室流出道的心肌组织存在着很多超微结构异常的心肌细胞 ;2 .TOF患儿应尽早手术治疗 ,1岁内手术矫治能避免不可逆性心肌损伤     

T波电交替预测法洛四联症术后室性心律失常的价值

目的探讨T波电交替（TWA）预测法洛四联症（TOF）术后室性心律失常的价值。方法回顾性分析36例TOF术后儿童及120例年龄相仿正常儿童的TWA特点及持续TWA与室性心律失常发生的关系。结果TOF术后患儿持续TWA发生率（36.1%）明显高于正常对照儿童（15.8%）,差异有统计学意义（χ2=6.98,P<0.05）;TOF术后出现持续性TWA患儿在运动过程中室性心律失常的发生率（76.9%）远高于TOF术后无持续TWA患儿（21.7%）,差异有统计学意义（χ2=10.41, P<0.05）。结论TOF术后患儿存在发生室性心律失常的电生理基础,持续TWA对预测TOF术后室性心律失常具有重要价值。