右心室微创切口矫治法洛四联症右心功能的中期随访研究

目的:观察右心室微创切口矫治法洛四联症3～5年的手术效果及右心功能情况。方法:入选2010年5月至2017年10月采用经右心室微创切口根治法洛四联症并按期复诊的患儿20例,年龄6.7～29.0个月,平均(14.3±7.1)个月;体质量6.5～14.0 kg,平均(9.3±1.9)kg。3例经右心房及右心室小切口进行矫治(右心室切口长度20 mm),其中2例跨肺动脉瓣环补片。另17例经右心房及肺动脉切口矫治,其中8例跨肺动脉瓣环补片(右心室切口10 mm),9例无跨环补片。结果:术后早期,经右房及肺动脉切口矫治,右室流出道残存压差平均26.3 mmHg,而经右心室小切口矫治压差明显减小(13 mmHg)。中晚期随访,右室流出道压差均明显下降(15.1 mmHg),右心室无明显扩张,三尖瓣及肺动脉瓣反流无加重。结论:右心室微创切口矫治法洛四联症3～5年随访临床效果满意。     

一期根治术治疗法洛四联症110例

目的总结法洛四联症(TOF)一期根治术的手术经验。方法 TOF患者110例,均施行一期根治术,采用右心房+右心室流出道(ROVT)切口,切除右心室肥厚的肌肉组织,使用自体心包补片重建右心室流出道至远端肺动脉;补片闭合室间隔缺损;带瓣跨瓣环补片重建ROVT 33例。结果 3例因术后出现低心排综合征而死亡,余107例随访1～48个月,均恢复良好,无晚期死亡者。结论加强术前评估、改进手术技术和体外循环管理、完善术后并发症防治措施可以提高TOF患者一期根治术的成功率。     

经右心房/肺动脉与经右心室切口根治法洛四联症的经验总结

目的总结经右心房/肺动脉与经右心室切口根治法洛四联症的经验。方法研究北京儿童医院心脏中心2004年1月至2006年4月74例小儿法洛四联症根治术的临床资料。A组16例经右心房/肺动脉切口,B组58例经右心室切口。结果围术期死亡3例,总死亡率4.05%。A组体外循环时间(108.50±23.27)min,阻断时间(69.56±18.24)min,B组体外循环时间(101.14±36.64)min,阻断时间(68.09±23.48)min。低心排综合征A组2例(12.50%),B组9例(15.52%);心律失常发生率A组1例(6.25%),B组5例(8.62%)。肺部并发症26例(35.14%)。结论严格把握经右心房/肺动脉切口与经右心室切口根治法洛四联症的适应证,注意心肌及肺脏的保护,可获得良好的手术效果。     

双心室矫治完全型房室间隔缺损合并右心室双出口或法洛四联症

目的:总结双心室矫治完全型房室间隔缺损合并右心室双出口或法洛四联症(CAVSDDORV/TOF)的外科经验。方法:全部患者均按常规体外循环方法中低温(28℃~32℃)下手术。11例采用右心房-右心室径路,2例单纯右心房径路。应用逗号状补片修补VSD,其中双片法10例,单片法3例。5例采用单纯补片扩大右心室流出道(RVOT),4例行跨环补片,2例行右心室-肺动脉连接术(Rastelli),1例单纯扩大主肺动脉,1例仅行肺动脉瓣交界切开。结果:2例死亡,均为CAVSD-DORV,VSD远离大动脉,合并镜面右位心。11例存活患者出院前超声心动图检查示左、右心室流出道无狭窄,房室瓣成形满意。10例患者随访3~77个月,心功能均为NYHA I级或II级,无再次手术发生。结论:双心室矫治CAVSD-ROVR/TOF近期及中期效果满意,可作为首选手术策略。采用右心房-右心室径路,应用逗号状补片双片法修补室缺以及精细的房室瓣成形是提高手术成功率,减少并发症的关键。     

双心室矫治法洛四联症合并完全性房室间隔缺损的效果

目的:回顾性总结采用双室性矫治法洛四联症合并完全性房室间隔缺损(TOF/CAVSD)的外科手术经验及其效果分析。方法:自2004年1月至2011年12月期间,上海儿童医学中心连续对16例TOF/CAVSD,其中伴有唐氏综合征2例,手术年龄中位数9个月(4个月~12岁),手术时体质量中位数8 kg(4.7~35 kg),术前有56%患者伴有中度以上左侧房室瓣反流,31%患者伴有中度以上右侧房室瓣反流;术前平均肺动脉指数为(168±23)mm2/m2。合并的CAVSD矫治中均采用了双片法,其中有8例(50%)采用右心室流出道(RVOT)切口径路修补室间隔缺损,另外50%患者采用右心房切口径路修补室间隔缺损,仅有1例室间隔缺损补片上留孔,右心室流出道梗阻(RVOTO)处理中,13例患者(81%)施用心包补片跨瓣扩大成形右心室流出道和肺动脉,其中1例带有单瓣。结果:术后医院生存率为93.75%。术后平均呼吸机辅助时间为(166±22.5)h,平均监护室留驻时间中位数为7d。4例患者出现术后严重并发症,其中发生低心排出量综合征(低心排)者3例,多脏器功能损害者1例。出院时12.5%(2例)患者术后二尖瓣反流(轻-中度),12.5%患者术后轻度三尖瓣反流(TR),无1例存在左心室流出道梗阻(LVOTO)。随访期6个月~5年,随访期无死亡,无1例出现LVOTO,无再手术干预;3例肺动脉远端流速增快者已登记介入治疗;随访发现生存60%患者二尖瓣反流为轻微-轻度;同时发现有1/3生存者三尖瓣反流为轻微-轻度;随访期存在中度肺动脉血反流患者为86%。1/3生存患者的右心室舒张末容积相应正常年龄正常值。结论:双心室矫治法TOF/CAVSD的短中期效果好,推荐采用双片法矫治CAVSD;与单纯TOF或CAVSD手术一样,必须定期随访复查,需关注房室瓣功能、肺动脉发育、肺动脉逆向血流和心室功能。     

双心室矫正法洛四联症合并完全性房室间隔缺损的手术技巧

目的:评价双心室矫治法洛四联症合并完全性房室间隔缺损(TOF/CAVSD)的效果。方法:2007年1月至2012年12月,共行TOF-CAVSD解剖矫正术16例,年龄1~13岁,平均3.3岁,体质量7~36kg,平均9.1kg,其中6例为Down综合征;16例均采用右心房、右心室纵切口,二片法补片修补房室间隔缺损,右心室流出道加宽补片或外管道。结果:术后早期无死亡,无因为室缺残余分流、左侧房室瓣反流和右心室流出道梗阻而再手术。随访6个月至5年,无晚期死亡及再手术;NYHA心功能Ⅰ级或Ⅱ级。结论:双心室法矫正TOF-CAVSD效果良好。     

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。     

肺动脉瓣缺如综合征的外科治疗

目的:探讨肺动脉瓣缺如综合征(APVS)的外科治疗经验。方法:1994年12月至2006年11月共治疗APVS患儿5例,其中男性4例,女性1例。5例均伴法洛四联症,其中1例伴左肺动脉起源异常,1例伴左肺动脉狭窄,发育不良。全组患儿均在中低温体外循环下行根治手术。其中4例患儿肺动脉瓣区置带瓣补片,1例采用牛心包直接补片。结果:4例存活,1例死亡,术后平均随访(13.8±5.76)个月。超声心动图检查示肺动脉瓣少量返流,右心室至肺动脉平均流速及压差较术前明显下降(P<0.01)。结论:APVS的外科治疗应选用带瓣补片重建右心室流出道。肺动脉严重扩张的患儿应行肺动脉成形术,以解除气道压迫症状。     

成人法洛四联症根治术135例临床分析

【】：目的 总结成人法洛四联症(TOF)的外科手术治疗经验。 方法 回顾性分析2006年1月~2016年8月我院135例成人法洛四联症患者手术治疗的临床资料。所有患者在低温体外循环下行根治术,其中91例采用跨瓣环补片加宽右心室流出道及肺动脉,17例仅行右心室流出道扩大补片,27例疏通流出道后直接缝合右室切口。 结果 住院期间死亡率2.2%(3/135),死亡原因均为低心排血量综合征。其余并发症包括室缺残余漏2例,一过性Ⅲ度房室传导阻滞4例未安装永久起博器,心包积液2例,胸腔积液5例,肺不张4例,肺部感染2例,肾衰竭2例,行血液滤过治疗后其中1例恢复1例死亡。随访128例,平均随访16.6(3~55)月,心功能Ⅰ级96例(75%),心功能Ⅱ级32例(25%)。结论 彻底矫正畸形、加强心肌保护、避免发生低心排血量综合征、加强术后管理,成人法洛四联症外科手术可取得良好的疗效。     

经皮室间隔化学消融术治疗肥厚梗阻型心肌病

目的评价室间隔化学消融术治疗肥厚梗阻型心肌病在急性期的效果。方法对52例确诊肥厚梗阻型心肌病患者进行经皮室间隔化学消融术治疗,并于术前、术后急性期进行超声心动观察。结果41例患者介入治疗成功,成功率为78.8%。术前超声观察室间隔平均厚度(22.96±5.15)mm,左室流出道压力阶差(LVOTPG)(92.64±38.69)mm Hg(1mm Hg=0.133kPa),术后急性期(4.8±3.2)d,室间隔平均厚度(21.27±4.64)mm,较术前比变薄,P<0.05;LVOTPG(51.79±38.99)mm Hg,较术前明显降低,P<0.001。结论经皮室间隔化学消融术治疗肥厚梗阻型心肌病,在急性期可使室间隔平均厚度降低,收缩期左室流出道压力阶差显著下降。     

Transatrial resection of the obstructed right ventricular infundibulum.

Obstructions of the right ventricular infundibulum were resected through the orifice of the tricuspid valve in 21 patients, 15 of whom had tetralogy of Fallot. At operation the systolic pressure difference between the right ventricle and pulmonary artery after repair averaged 18 mm Hg (range 0-40 mm Hg). In patients with tetralogy, cardiac index four hours after operation averaged 2.8 L/M2/min. One patient with tetralogy and severe pulmonary hypertension died. Twelve patients with tetralogy were recatheterized 10 to 186 days after operation. The mean systolic pressure difference between right ventricle and pulmonary artery was 23 mm Hg. Residual obstructions were in the pulmonary valvular annulus. Cineangiograms did not show paradoxical motion of the right ventricular wall. Transatrial resection of right ventricular infundibular obstructions carries with it none of the consequences that often follow right ventriculotomy and this surgical approach satisfactorily relieves infundibular obstructions.     

单纯心内修复在法洛四联症矫治的应用

目的 总结用单纯心内修复的方法施行法洛四联症矫治的手术经验。方法 回顾分析2005年1月到2016年7月武汉亚洲心脏病医院对275例法洛四联症患者施行单纯心内修复,在充分疏通右室流出道、修补室间隔缺损后直接缝合右室切口,占同期法洛四联症矫治术的28.61%。 结果 全组无围术期死亡,所有病例术后血流动力学较平稳,术后恢复良好,无重大并发症。 结论 法洛四联症的矫治宜选择个性化的手术方案,对单纯漏斗部狭窄、室间隔缺损为嵴下型的病例,单纯心内修复方法可行,有利于心功能保护及术后恢复。     

Hemodynamic evaluation of 221 patients after intracardiac repair of tetralogy of Fallot

Cardiac catheterization and angiocardiographic studies were performed in 221 patients an average of 2 years (range 2 weeks to 10 years) after intracardiac repair of tetralogy of Fallot. The group represented 32.5 percent of the postoperative survivors. The postoperative result was classified as “excellent” (43 percent), “good” (35 percent), “satisfactory” (7 percent) or “unsatisfactory” (14 percent) on the basis of right ventricular peak systolic pressure, right ventricle to pulmonary artery peak systolic gradient and presence of a persistent large or small left to right shunt at the ventricular level. Unsatisfactory results were associated with obstructive pulmonary vascular disease (four patients), severe malformations with obstruction of the right ventricular outflow tract and/or a left to right shunt with a Qp:Qs ratio of greater than 1.5:1 or with currently uncorrectable obstructive anomalies of the right ventricular outflow tract or pulmonary arteries.The postoperative result depended upon the amount of reconstruction of the right ventricular outflow tract and the caliber of the pulmonary arteries. Patients who required a systemic to pulmonary artery anastomosis before total correction and those who required a prosthetic patch for the right ventricular outflow tract fared worse than younger patients who had minimal resection of the right ventricular outflow tract. Pulmonary insufficiency was detected in 94 percent of patients with a right ventricular outflow tract prosthesis and in 10 percent of those who had infundibulectomy alone. The insufficiency was well tolerated clinically and hemodynamically unless there was distal obstruction of the pulmonary artery or its branches.Postoperative catheterization is necessary to assess the immediate and long-term operative results in patients with tetralogy of Fallot. Total correction of tetralogy of Fallot as a primary procedure provides acceptable hemodynamic results even in children less than 4 years of age, providing that the right ventricular outflow tract obstruction can be relieved and that pulmonary insufficiency is not aggravated by increased main pulmonary arterial pressure.     

Right ventricular diastolic function after repair of tetralogy of Fallot: its relationship to the insertion of a 'transannular' patch.

Examined was the effect of surgical technique, particularly the insertion of a transannular patch, on right ventricular diastolic function, and the relationship of forward flow in the pulmonary arteries during late diastole to right ventricular diastolic function in patients with tetralogy of Fallot. Transtricuspid, superior caval venous and pulmonary arterial Doppler spectrals were obtained and compared between 44 patients who had been repaired with a transannular patch; 14 patients who had been repaired with muscular resection and/or pulmonary valvotomy; six who had been repaired with an infundubular patch; and 32 normal children. The velocities of forward flow during late diastole in the pulmonary arteries of normal children ranged from 19.8 to 29.4 cm s(-1) (mean 24.9 +/- 2.8 cm s(-1)) throughout the respiratory cycle. Restrictive right ventricular physiology, defined on the basis of increased forward flow in the pulmonary arteries during late diastole (> 30 cm s(-1)) was present in 25 (57%) of 44 patients with tetralogy of Fallot repaired using a transannular patch. Right ventricular volume was 50.1 +/- 23.7 cm3 in patients with a restrictive right ventricle and 64.9 +/- 21.4 cm3 in patients in whom the ventricle was non-restrictive (p < 0.03). QRS duration was 140 +/- 18 and 156 +/- 24 ms in patients with restrictive and non-restrictive right ventricular physiology respectively (p < 0.003). Restrictive physiology was not encountered in patients with tetralogy in whom the pulmonary valve had been preserved. It is concluded that right ventricular restriction is present in many patients with tetralogy of Fallot at mid-term follow-up subsequent to repair using a 'transannular' patch. Restriction is associated with smaller right ventricular size and less prolongation of the QRS complex.     

Stress-induced ventricular arrhythmia after repair of tetralogy of fallot

The combination of ventricular arrhythmias found on routine electrocardiography at rest and elevated right ventricular pressures may be associated with sudden death in patients after repair of tetralogy of Fallot. In this study data were reviewed from 104 patients who underwent treadmill exercise testing with a modified Bruce protocol at a mean age of 13.8 years, an average of 7 years after repair of tetralogy of Fallot. The frequency and form of ventricular arrhythmias as manifested in an electrocardiogram at rest as well as before, during and after treadmill exercise tests were tabulated for each patient. Cardiac catheterizatlon was performed within 1 week of the treadmill test in 82 of the 104 patients and the right ventricular systolic pressure, right ventricular end-diastolic pressure, pulmonary arterial systolic pressure and pulmonary to systemic flow ($\text{Qp}\text{Qs}$) ratio were compared in patients with and without ventricular arrhythmias. In 15 (14 percent) of the 104 patients, ventricular arrhythmia was evident in the electrocardiogram at rest and in 31 (30 percent) it was induced by treadmill exercise; 5 of the 15 with arrhythmia at rest had no arrhythmia during exercise. Compared with the remaining 73 patients, the 31 patients who had ventricular arrhythmias during treadmill testing (1) were older (16.2 versus 12.8 years, p < 0.01, t test) and were tested at a longer interval after repair of tetralogy of Fallot, (2) had greater right ventricular systolic pressure (52.7 versus 41.3 mm Hg, p < 0.005), (3) had greater right ventricular end-diastolic pressure (8.0 versus 6.1 mm Hg, p < 0.001), and (4) had no difference in pulmonary arterial systolic pressure or $\text{Qp}\text{Qs}$ ratio. Uniform premature ventricular complexes were found in 28 patients, multiform premature ventricular complexes in two and ventricular tachycardia in one. Patients whose ventricular arrhythmia appeared with exercise had similar hemodynamic findings to-those whose arrhythmia disappeared with exercise. Two patients, each with elevated right ventricular systolic pressure and multiform premature ventricular complexes, had cardiac arrest at home.It is concluded that (1) treadmill exercise testing is a sensitive method for detecting ventricular arrhythmia in patients after repair of tetralogy of Fallot; (2) ventricular arrhythmia during treadmill exercise is related to abnormal hemodynamic status; (3) multiform premature ventricular complexes, couplets or ventricular tachycardia with exercise testing are highly associated with abnormal hemodynamic status and sudden death; (4) suppression of ventricular arrhythmia with exercise is neither the characteristic nor necessarily the benign response in these patients; and (5) ventricular arrhythmia may be a late developing phenomenon and may increase with longer postoperative intervals. Patients after repair of tetralogy of Fallot should be treated for ventricular arrhythmia detected in the electrocardiogram at rest or during treadmill exercise testing.     

Assessment of right ventricular diastolic function: does it predict post-operative course in tetralogy of Fallot

BACKGROUND: In some cases of tetralogy of Fallot the post-operative course is characterized by episodes of low cardiac output, elevated central filling pressures and prolonged ventilation and inotropic support. This may be due to impaired diastolic function of the right ventricle despite preservation of biventricular systolic function. METHODS AND RESULTS: Sixty-four consecutive patients (mean age 7.06+/-4.9 years) undergoing repair of tetralogy of Fallot were prospectively studied to assess right ventricular diastolic function. 'Restrictive physiology' was defined as presence of laminar antegrade diastolic pulmonary artery flow (A wave) throughout the respiratory cycle, which was coincident with atrial systole. Right ventricle restriction was present in 45/64 (70%, Group 1) patients and absent in 19/64 (30%, Group 2) patients. There was a marked inspiratory augmentation of the pulmonary artery A wave velocity, flow integral and duration. Transtricuspid flow revealed significantly lower peak E velocity, lower E/A ratio, shorter E deceleration time and higher A velocity time integral in those with right ventricular restriction. Biventricular systolic function and transmitral flow were normal in all patients. Those with restrictive physiology had significantly longer mean inotrope support duration, longer ventilation and chest drainage times. Correspondingly, the mean intensive care unit stay (56.7+/-9.3 v. 34.7+/-5.38 hours, p<0.01) and mean hospital discharge time (9.3+/-2.3 v. 6.2+/-0.5 days, p <0.001) was also significantly longer in group 1. CONCLUSIONS: Right ventricular restriction (as seen by laminar antegrade diastolic pulmonary artery flow throughout the respiratory cycle) exists in a significant subset of patients with tetralogy of Fallot following operative repair. Following surgery, such patients have higher inotropic requirement, longer ventilation times and longer hospital stay.     

Can the human right ventricle create a negative diastolic pressure suggestive of suction?

Pressure in the right ventricle (RV) as well as the right atrium (RA) and pulmonary artery (PA) were measured in 80 patients with catheter-tip mlcromanometere and evaluated to determine If the pressures are compatible with the concept of RV diastollc suction. In 40 patients with normal PA pressure, minimal RV diastollc pressure that occurred during early filling, was negative (?2 ± 0.3 mm Hg) (mean ± SEM). In 29 patients with PA hypertension, minimal RV dlastolic pressure during expiration also was negative (?2 ± 0.7 mm Hg). In 11 patients with right ventricular failure, however, minimal RV diastolic pressure was positive (9 ± 2 mm Hg). These results indicate that the human right ventricle, in the absence of failure, has a negative early diastollc pressure, which may reflect RV diastollc suction.     

Hemodynamic studies after total correction of tetralogy of Fallot

Twenty patients were studied by right heart catheterization 5 to 23 months after complete surgical correction of tetralogy of Fallot. The ventricular septal defects had been closed with a polyvinyl sponge and a Teflon prosthesis had been used to widen the right ventricular outflow tract in each case.

Residual systolic gradients between the right ventricle and pulmonary artery were small. Fifteen patients had gradients of 15 mm. or less, and the highest found was 34 mm. Arterial oxygen saturation was normal at rest or with exercise, or both, in nearly all cases. Two patients had definite evidence of a persistent ventricular septal defect with a left-to-right shunt.

Pulmonic regurgitation was present in 13 patients. Despite elevated right ventricular end diastolic and right atrial pressures in some patients, clinical evidence of heart failure was absent in all at the time of catheterization.

A comparison was made of the right ventricular and pulmonary arterial pressure measurements obtained in the operating room upon completion of the cardiac repair with those made at catheterization. It was found that pressure measurements at surgery give a reliable indication of the effectiveness of relief of the right ventricular outflow stenosis.

The operation employed in these patients has relieved the physiologic abnormalities of tetralogy of Fallot. Pulmonic regurgitation is present in many of the patients but is tolerated well at present. The long-term effects of this new abnormality will become clear only after extended observation.     

The mechanism of apparent right bundle branch block after transatrial repair of tetralogy of Fallot.

The electrocardiographic pattern of right bundle branch block (RBBB) is routinely observed after transatrial repair of tetralogy of Fallot even though no ventriculotomy has been performed. The mechanism of this conduction disturbance was studied in 16 patients with tetralogy of Fallot and one patient with infundibular pulmonic stenosis. Preoperative ECGs and vectorcardiograms showed right ventricular hypertrophy and no RBBB. Epicardial activation maps were obtained before and after total surgical repair in all patients and after infundibular resection but before closure of ventricular septal defect (VSD) in four of these patients. After infundibular resection, RBBB appeared and activation was markedly delayed (greater than 30 msec) over the pulmonary outflow tract, but was unchanged over the body of the right ventricle. No further changes in ventricular activation occurred after closure of the VSD. This study shows that RBBB after transatrial repair of tetralogy of Fallot is usually produced by infundibular resection, but not by VSD closure, and is associated with delayed activation of the pulmonary outflow tract and base of the right ventricle which results from damage to portions of the right ventricular conduction system.     

经右房、右室流出道小切口或经右房肺动脉行法乐四联症根治术体会

本文报道２５例法乐四联症根治术，经右房径路修补室间隔缺损，通过右室流出道小切口或经右房肺动脉径路重建右室出口。对手术方法和临床意义进行讨论。此方法能减轻对右室心肌的损伤，有利于保护右室功能，从而提高手术疗效。