人造单瓣补片扩大右室流出道根治法洛氏四联症的近期疗效

本对重症法洛氏四联症采用人造单瓣补片扩大右室流出道进行根治术。病例共４０例,随机分为Ａ、Ｂ两组。Ａ组为带瓣组,采用带自体心包单瓣进行跨肺动脉瓣扩大右室流出道。Ｂ组为对照组,用相应大小自体心包进行跨肺动脉瓣扩大右室流出道。观察两组病例在术前、术后一周、术后３个月及６个月的心电图、Ｘ线胸片及心脏超声检查指标,比较两组病例肺动脉瓣返流面积和心功能变化。结果显示带瓣组肺动脉瓣返流面积术后一周、３个月及６     

Functional implications of the right ventricular myocardial performance index in patients after surgical repair of tetralogy of Fallot

The myocardial performance index (MPI) has been proposed to be a simple echocardiographic index of right ventricular (RV) function in patients after surgical repair of tetralogy of Fallot (TOF). However, its functional status remains to be clarified. The functional implications of RV MPI were determined by exploring its relationships with parameters of RV function as derived from cardiovascular magnetic resonance (CMR), and exercise capacity of postoperative TOF patients Thirty patients (11 males), aged 15.6 ± 3.1 years, who have undergone surgical repair of TOF at 4.0 ± 1.8 years, were studied. The RV and left ventricular (LV) MPIs determined using pulsed-wave Doppler echocardiography were related to CMR-derived RV and LV ejection fractions, and pulmonary regurgitant fraction and treadmill exercise testing parameters. Log RV MPI correlated positively with log LV MPI (r = 0.38, P = 0.037) and negatively with CMR-derived RV ejection fraction (r = −0.4, P = 0.028) and pulmonary regurgitant fraction (r = −0.4, P = 0.031). No significant correlations were found between LV MPI and any of the CMR parameters. Using receiver operated characteristics analysis, a cutoff value of 0.30 for RV MPI was found to have a sensitivity of 100% and specificity of 74% in predicting a RV ejection fraction <35%. Right ventricular, but not LV, MPI correlated inversely with exercise duration (r = −0.45, P = 0.013) and peak oxygen consumption (VO₂ max) (r = −0.56, P = 0.001). Multivariate analysis identified RV MPI (β = −0.6, P < 0.001), male sex (β = 0.44, P = 0.01), and duration from surgery (β = −0.30, P = 0.019) as significant determinants of VO₂ max. Increased MPI is a reflection of reduced RV ejection fraction and exercise capacity in patients after TOF repair.     

Impact of pulmonary regurgitation and right ventricular dysfunction on oxygen uptake recovery kinetics in repaired tetralogy of Fallot

BACKGROUND: Patients with repaired tetralogy of Fallot (ToF) featuring severe pulmonary regurgitation (PR) and/or right ventricular (RV) dysfunction have reduced exercise tolerance. AIMS: To assess the impact of PR and of RV function on the ability to recover from exercise in ToF patients. METHODS: 61 consecutive patients aged 23.1+/-12.1 years underwent maximal cardiopulmonary exercise test (CPX), transthoracic echocardiography and magnetic resonance imaging. This data was compared to those of 153 matched healthy subjects. RESULTS: 19 patients (31%) had severe PR. RV dysfunction was noted in 19 patients (31%). Nine patients (15%) had both severe PR and RV dysfunction. Patients had lower peak oxygen uptake (VO2), VO2 slope, carbon dioxide production (VCO2) slope and O2 pulse slope (p < 0.0001), especially those with severe PR and RV dysfunction (p < 0.0001). Heart rate slope was similar between groups. No patient with severe PR and RV dysfunction had a predicted peak VO2 > 40%. CPX had a high sensitivity and specificity to identify patients with severe PR and RV dysfunction. CONCLUSIONS: In ToF patients, severe PR and RV dysfunction lead to delayed recovery from exercise. CPX can identify patients with severe PR and RV dysfunction and may be useful to guide the pulmonary valve replacement decision-making process.     

Circulating biomarkers of collagen type I metabolism mark the right ventricular fibrosis and adverse markers of clinical outcome in adults with repaired tetralogy of Fallot

Background

Right ventricular (RV) fibrosis is common in patients with repaired tetralogy of Fallot (rTOF). Although accumulating evidence indicates the role of circulating biomarkers of collagen metabolism in left ventricular fibrosis, rTOF data are lacking. This study examined the expression profile and clinical relevance of circulating biomarkers of collagen type I metabolism in rTOF patients.

Methods

Serum biomarkers of collagen type I synthesis (carboxy-terminal propeptide of procollagen type I, PICP), degradation (carboxy-terminal telopeptide of collagen type I, CITP), and enzymes regulating collagen degradation (matrix metalloproteinases, and type I tissue inhibitor, TIMP-1) were measured in 70 rTOF and 91 control adults. All patients had complete clinical data and received cardiovascular magnetic resonance scans with late gadolinium enhancement (LGE).

Results

Compared to the controls, rTOF patients had higher PICP levels (p < 0.001), PICP:CITP ratios (p < 0.001), and TIMP-1 concentrations (p < 0.001). Increasing PICP levels correlated with higher RV LGE scores (r = 0.427, p < 0.001), lower VO₂max (r = − 0.428, p = 0.002), and larger RV volumes. Furthermore, stepwise multivariate linear regression analysis identified RV end-diastolic volume index > 150 mL/m² (β = 40.52, p = 0.016), RV LGE score (β = 3.94, p = 0.008), and age (β = − 1.77, p = 0.011) as independent correlates of circulating PICP levels.

Conclusions

Patients with rTOF exhibited a profibrotic state with excessive collagen type I synthesis and dysregulated degradation. Elevated circulating PICP levels might reflect RV fibrosis, and link to adverse markers of clinical outcome.     

Mechanisms of maintained exercise capacity in adults with repaired tetralogy of Fallot

Background

The mechanisms whereby cardiac output is augmented with exercise in adult repaired tetralogy of Fallot (TOF) are poorly characterised.

Methods

16 repaired TOF patients (25 ± 7 years of age) and 8 age and sex matched controls (25 ± 4 years of age) underwent cardiopulmonary exercise testing and then real-time cardiac MRI (1.5 T) at rest and whilst exercising within the scanner, aiming for 30% heart rate reserve (Level 1) and 60% heart rate reserve (Level 2), using a custom-built MRI compatible foot pedal device.

Results

At rest, TOF patients had severely dilated RVs (indexed RV end-diastolic volume: 149 ± 37 mL/m²), moderate-severe PR (regurgitant fraction 35 ± 12%), normal RV fractional area change (FAC) (52 ± 7%) and very mildly impaired exercise capacity (83 ± 15% of predicted maximal work rate). Heart rate and RV FAC increased significantly in TOF patients (75 ± 10 vs 123 ± 17 beats per minute, p < 0.001; 44 ± 7 vs 51 ± 10%, p = 0.025), and similarly in control subjects (70 ± 11 vs 127 ± 12 beats per minute, p < 0.001; 49 ± 7 vs 61 ± 9%, p = 0.003), when rest was compared to Level 2. PR fraction decreased significantly but only modestly, from rest to Level 2 in TOF patients (37 ± 15 to 31 ± 15%, p = 0.002). Pulmonary artery net forward flow was maintained and did not significantly increase from rest to Level 2 in TOF patients (70 ± 19 vs 69 ± 12 mL/beat, p = 0.854) or controls (93 ± 9 vs 95 ± 21 mL/beat, p = 0.648).

Conclusions

During exercise in repaired TOF subjects with dilated RV and free PR, increased total RV output per minute was facilitated by an increase in heart rate, an increase in RV FAC and a decrease in PR fraction.     

对法洛四联症中右心室流出道梗阻的再认识

目的:分析右心室流出道病理肌束的形成和构造,以期完善其外科纠治。方法:我院2002年1月至2007年3月选取50例法洛四联症(tetralogy of Fallot TOF)患者,年龄3个月~15岁,体质量6~38 kg。术中经右心室探查并描述右心室流出道的病理解剖。结果:圆锥隔前上移位是所有病例的共同特征,移位程度和主动脉骑跨均呈正比。所有患者都有隔、壁延伸。漏斗口位于圆锥隔下缘38例。低位漏斗口9例。存在弥散性肌束梗阻3例。所有患者的壁延伸连接于心室漏斗皱褶和游离壁。44例患者的隔延伸连接于室间隔。6例隔延伸和隔缘束间无间隙。3例弥散性肌束梗阻患者的调节束和隔缘束体部上移,挤压圆锥隔。离断解除延伸肌束,保留卵圆孔开放。所有患者有隔壁小梁肥厚,完全离断和切除。结论:法洛四联症中右心室流出道梗阻发生的病理基础是流出隔前、上移位和肌束延伸及隔壁小梁肥厚。术中准确识别病变肌束的性质和结构是维护术后良好心功能的关键。     

The role of echocardiography for quantitative assessment of right ventricular size and function in adults with repaired tetralogy of Fallot

Background: Quantitative assessment of right ventricular (RV) systolic function by echocardiography is challenging in patients with congenital heart disease because of the complex geometry of the RV and the iatrogenic structural abnormalities resulting from prior cardiac surgeries. The purpose of this study was to determine the correla‐ tion between echocardiographic indices of RV systolic function and cardiac magnetic resonance imaging (CMRI) derived RV ejection fraction (RVEF) in adults with repaired tetralogy of Fallot (TOF).
Methods: Quantitative assessment of RV function was performed with RV tissue Doppler systolic velocity (RV s'), tricuspid annular plane systolic excursion (TAPSE), and fractional area change (FAC). These echocardiographic indices were compared to RVEF from CMRI performed on the same day as echocardiogram.
Results: Of 209 patients, the mean RV FAC was 39 ± 9%, TAPSE was 18 ± 4 mm, RV s' was 10 ± 2 cm/s, and RVEF was 40 ± 10%. There was a good correlation be‐ tween TAPSE and RVEF (r = 0.79, P < .001), good correlation between RV s' and RVEF (r = 0.71, P < .001), and modest correlation between FAC and RVEF (r = 0.66, P < .001). TAPSE < 17 mm effectively discriminated between patients with RV systolic dysfunc‐ tion defined as RVEF < 47% (sensitivity 81%, specificity 79%, area under the curve [AUC] 0.805). FAC < 40% was associated with RVEF < 47% (sensitivity 72%, specificity 63%, AUC 0.719). RV s' < 11 cm was associated with RVEF < 47% (sensitivity 83%, specificity 68%, AUC 0.798).
Conclusion: Despite the structural and functional abnormalities of the RV in patients with repaired TOF, quantitative assessment of RV systolic function by echocardiog‐ raphy is feasible and had good correlation with CMRI‐derived RVEF.     

3T磁共振成像相位对比法评价法洛四联症根治术后患者右心室舒张功能△

目的通过3T相位对比磁共振成像（phasecontrast-magneticresonanceimaging,PC-MRI）对法洛四联症（tetralogyofFallot,TOF）术后随访患者j尖瓣血流评估,与超声心动图结果进行对照研究,探讨PC-MRl评价TOF术后患者有心室舒张功能的准确性。方法27例进行了超声心动图及i尖瓣PC-MRI的TOF随访患者纳入研究。PC-MRl测量得到i尖瓣E波峰值流速（Evelocity）、A波峰值流速（Avelocity）;E波峰值流率（Eflow）、A波峰值流牢（Aflow）。超声心动图测量得到E波和A波峰值流速（分别表示为E、A）。Evelocitv／Avelocitv及Eflow／Aflow比值分别与超出心动图测量E／A比值进行相关分析及配对样本t检验。按照E／A〉1（无舒张功能障碍）及E／A≤1（有舒张功能障碍）分为两组,比较PC-MRI两种测量方法与超,旨心动网测量结果符合程度。,结果PC-MRI测量的Evelocjtv／Avelocitv,Eflow／Aflow比值与超声心动图测量E／A比值具有正相关性（r=0．560．P=0．002：r=0．542,P=0．003）。PC-MRI测量结果Evelocitv／Avelocity及Eflow／Aflow比值与超声心动冈测最E／A比值卡H比,差异无统计学意义（1．29／）S．1．41,t=1．624,P=0．116;1．39us1．41,t=0．182,P=0．857）。判断有、无舒伥功能障碍,PC-MRI峰值流速比值（Evelocitv／Avelocjtv）、峰值流率比值（Eflow／Aflow）与超声心动冈符合程度较妤（K=O．697,P〈0．001：K=0．571,P=0．003）。结论PC-MRI测量的i尖瓣峰值流速比值及峰值流牢比值能够正确判断行心事舒张功能。     

Differential effect of right ventricular dilatation on myocardial deformation in patients with atrial septal defects and patients after tetralogy of Fallot repair

Background

Right ventricular (RV) volume overload results in RV dilatation in patients with atrial septal defect (ASD) and after tetralogy of Fallot (ToF) repair with pulmonary regurgitation.

Aim

Study the differential effects of chronic RV volume loading on regional and global RV deformation in patients with ASD and after TOF repair.

Methods

We studied 85 subjects: 50 patients after ToF repair, 15 patients with unrepaired ASD and 20 age-matched controls. The ToF patients and controls underwent an echocardiography at the time of a clinically indicated MRI. The ASD patients had a routine echocardiogram including RV volume calculations. Longitudinal deformation was analyzed using 2-D speckle tracking echocardiography.

Results

RV free wall global and segmental longitudinal deformation was significantly lower in ToF patients compared with ASD and controls (p < 0.001). In ToF patients, there was a progressive decrease in strain values from base to apex (p < 0.001), while in the ASD group there was a progressive increase (p = 0.04). We found strong negative correlations between RV size and RV longitudinal strain measurements, strongest with RV length (R = 0.72). When corrected for RV size, all ASD patients had normal or higher deformation values while half of the TOF patients had significantly lower values.

Conclusion

Global and regional myocardial RV deformation is differently affected by chronic volume loading in ASD versus TOF patients, especially regarding the apical function. This suggests a different adaptation mechanism in both diseases. Our data also suggest that strain measurements are strongly influenced by ventricular size, which should be taken into consideration when interpreting strain values.     

法洛四联症右室流出道疏通的临床经验

目的 探讨法洛四联症（Tetralogy of Fallot,TOF）根治术中右心室流出道疏通的最佳直径。方法 回顾性分析2011年1月至2016年12月我院心脏外科行TOF根治术的儿童病例308例,2011年1月至2013年12月201例患儿（A组）均采取右心室流出道疏通直径等同于标准肺动脉瓣环直径;2014年1月至2016年12月107例患儿（B组）采取右心室流出道疏通直径大于标准肺动脉瓣环直径3 mm。所有出院患者术后随访12~36个月。结果 A组有17例患者术后出现右心室流出道残余梗阻致低心排血量综合征（Low Cardiac Output Syndrome,LCOS）,多普勒测右心室流出道流速3.1~4.8（3.8±0.4）m/s,跨肺动脉瓣压差均大于50 mm Hg,其中6例右心室流出道流速超过4.5 m/s,再次手术行右心室流出道疏通术;术后早期死亡5例,随访余12例未发现右心室流出道残余梗阻进一步发展,且均较术后早期明显改善。B组术后无右心室流出道残余梗阻,跨肺动脉瓣压差均小于50 mm Hg,术后早期死亡2例。随访两组出院患者无三尖瓣、肺动脉瓣大量反流,生长发育良好,心功能均达Ⅰ~Ⅱ级。结论 TOF右心室流出道疏通直径等同于标准肺动脉瓣环直径造成术后残余梗阻发生率较高,大于标准肺动脉瓣环直径3 mm可有更好的疗效。     

Remodelling of the right ventricle after early pulmonary valve replacement in children with repaired tetralogy of Fallot: assessment by cardiovascular magnetic resonance.

AIMS: Correct timing of pulmonary valve replacement (PVR) is crucial for preventing complications of pulmonary regurgitation and right ventricular (RV) dilatation after repair of tetralogy of Fallot. We sought to assess the remodelling of the RV after early PVR in children, using cardiovascular magnetic resonance (CMR). METHODS AND RESULTS: Twenty children with severe pulmonary regurgitation and RV dilatation and mean age 13.9 +/- 3 years underwent CMR evaluation 5.6 +/- 1.8 months before and 5.9 +/- 0.6 months after PVR. PVR was performed when the RV end-diastolic volume exceeded 150 mL/m(2), as measured by CMR. The time interval between primary repair and PVR was 12 +/- 3 years. Post-operative CMR demonstrated a significant reduction of the RV end-diastolic volume from 189.8 +/- 33.4 to 108.7 +/- 25.8 mL/m(2) (P < 0.0001), of the RV end-systolic volume from 102.4 +/- 27.3 to 58.2 +/- 16.3 mL/m(2) (P < 0.0001), and of the RV mass from 48.7 +/- 12.3 to 35.8 +/- 7.7 g/m(2) (P < 0.0001). The RV ejection fraction did not change significantly. CONCLUSION: Prompt RV remodelling, with reduction of RV volume and mass, is observed after performing PVR if the RV end-diastolic volume exceeds 150 mL/m(2). Early PVR may prevent the detrimental complications of severe pulmonary regurgitation.     

A new technique of transannular monocusp patch-repair of the right ventricular outflow tract in repair of Tetralogy of Fallot

Reconstruction of the right ventricular outflow tract (RVOT) is a key procedure in repair of Tetralogy of Fallot (TOF). The procedure creates pulmonary insufficiency (PI) that may compromise the right ventricular (RV) function, particularly during late follow-up. A simple way to reduce PI is to create a monocusp patch from xenografts, homografts, fascia lata, and autologous pulmonary artery or pericardium. Each of those has limitations. The autologous pericardial valve sewn on another pericardial patch is one of the earliest monocusp patches used clinically but loses anti-PI effect soon after the operation presumably due to degeneration or absorption of the monocusp. I have therefore designed and used a new technique (folded monocusp patch) to create a monocusp for TOF repair in children and adults. The technique has been used in nine patients of the 18 TOF patients who needed transannular patch-repair in total 74 TOF repairs. The monocusp patch-repaired patients had minimal or mild PI and good RV function beyond 8-12 months. The long-term results await follow-up and further study in multi-institutions.     

法洛四联症根治术后早期并发症的临床分析与处理

目的:总结法洛四联症(tetralogy of Fallot,TOF)患儿根治术后早期处理经验,以降低TOF根治术后早期并发症的发生率及病死率。方法:回顾性分析2012年1月至12月完成的TOF根治术188例,将其分为并发症组31例,非并发症组157例。对比两组患儿年龄、体质量,术前血色素、血氧饱和度、McGoon比值,术中体外循环时间、主动脉阻断时间,术后呼吸机辅助时间、体质量监护室停留时间,正性肌力药物评分以及术后右心室流出道压力差。结果:并发症组术前McGoon比值(1.54±0.21)vs.(2.01±0.42),体外循环时间(112.54±33.32)vs.(97.03±26.1)min、主动脉阻断时间(65.38±15.41)vs.(61.87±15.38)min,呼吸机辅助时间(85.64±35.38)vs.(44.62±21.84)h、监护室停留时间5.0(2.0,7.0)vs.3.0(1.0,5.0)d,正性肌力药物评分(18.21±6.27)vs.(10.16±3.18)与非并发症组比较差异有统计学意义(P0.05)。术后右心室流出道压力差21.5(12.3,33.8)vs.24.0(17.0,32.0)mmHg(1mmHg=0.133kPa),并发症组与非并发症组比较差异无统计学意义。并发症包括低心排出量综合征(低心排)9例,渗漏综合征12例,心律失常5例,灌注肺损伤2例,感染5例。其中死亡5例(病死率2.66%)。结论:严格把握手术适应证,缩短体外循环时间,术后合理应用正性肌力药物,积极腹膜透析是预防和控制TOF根治术后低心排和渗漏综合征的有效方法。呼吸机辅助通气呼气末正压治疗及高频振荡呼吸机治疗可以控制大多数灌注肺,必要时可介入封堵侧枝。     

法洛四联症右室心肌间质胶原改建与功能的关系

目的 :了解法洛四联症患者肥大右室心肌间质胶原改建与右室功能的关系。方法 :用生化方法定量分析 2 2例法洛四联症患者 (病例组 )和 8例非心血管及胶原系统疾病尸检者 (对照组 )的右室心肌间质中的胶原含量和 / 型胶原比值 ,并用右室穿刺法测定反映法洛四联症患者右室收缩及舒张功能的 dp/ dtmax及 - dp/ dtmax。结果 :病例组心肌每 mg总蛋白内含羟脯氨酸 (14.85± 2 .48)μg,较对照组 (7.5 5± 1.89)μg明显增多 (P <0 .0 5 ) ; / 型胶原比值为 5 .10± 1.84,较对照组 2 .2 7± 0 .5 8显著增高 (P <0 .0 5 ) ;病例组右室心肌间质胶原含量与术前、术后 dp/ dtmax无明显相关 ,而与术前、术后 - dp/ dtmax呈明显的负相关 ;其 / 型胶原比值与术前、术后dp/ dtmax及 - dp/ dtmax无相关。结论 :法洛四联症肥大右室心肌间质存在以胶原含量增多和 / 型比值增高为特点的胶原改建以及右室的舒张功能的下降 ,且后者主要与胶原含量增多有关     

右外侧小切口行法洛四联症根治术346例临床分析

目的:总结右外侧小切口剖胸行法洛四联症根治的经验及技术关键。方法:1997年1月至2013年10月,本手术组经右外侧小切口根治法洛四联症346例。其中男性159例,女性187例。年龄4个月~5岁;体质量6~15kg,平均体质量(9.7±2.4)kg。合并卵圆孔未闭43例、房间隔缺损22例、动脉导管未闭10例、永存左上腔静脉10例、主动脉瓣下隔膜7例、二尖瓣关闭不全1例。跨环补片205例,右心室流出道补片141例。结果:术中体外循环时间(90±24)min;主动脉阻断时间(64±17)min,术后机械通气时间4~165 h,监护室停留时间(3.2±1.7)d,术后当日胸腔引流量(138±91)mL,平均带胸管(2.5±0.9)d。术后并发症36例(1.04%):低心排出量综合征(低心排)17例(死亡5例),严重肺部感染2例(死亡1例),灌注肺5例(死亡1例),右肺损伤7例,膈神经损伤4例,室间隔缺损残余分流2例,乳糜胸2例。死亡共计7例,病死率2.02%。结论:经右外侧小切口行法洛四联症根治安全可靠。