法洛氏四联症矫治术后中远期肺动脉高压

【】目的 报道一组法洛氏四联症矫治术后中远期肺动脉压力及阻力升高的病例。方法2009.01-2011.04间我院行法洛氏四联症矫治术共181例(我院181例接受法洛氏四联症矫治术患儿),随访2-5年怀疑患出现肺动脉高压者并对其行右心导管检查最终确诊19例,分析单纯TOF矫治术后产生肺动脉高压的因素。结果19例患者在随访的2-5年内,绝大多数没有未出现明显临床症状。WHO心功能评定I/II级17例,III/IV级2例。问卷调查Borg呼吸困难评分为1-7分,中位数为4分。BNP检查为(11-＞300000)pg/ml,BNP的中位数为266pg/ml。右心导管检查示不同程度的肺动脉压力和阻力上升升高,其中轻度肺动脉高压16例,占绝大多数,所有患者肺小动脉楔入压均正常(6-10mmHg),肺动脉-右心室连续测压压力阶差小于10mmHg,右房压力属正常范围(6-11mmHg)。部分患者同时行肺小动脉造影,没有发现肺动脉高压特征性改变。无急性肺血管扩张试验阳性者。讨论部分法洛氏四联症矫治术后患者中远期会出现肺动脉高压及全肺阻力升高,其机制目前并不十分确定清楚,可能是一个多因素交叉的复杂过程。     

法洛氏四联症修补术后室间隔残余漏介入封堵治疗

目的:报道法洛氏四联症修补术后室间隔残余漏介入封堵治疗体会。方法:总结2010-10-2018-11在我科住院的法洛四联症修补术后室间隔残余漏患者9例资料,介绍经导管行左心室造影及介入封堵治疗经验。结果:7例患者封堵成功,成功率77.78%,2例外科再修补治疗;无任何并发症发生,介入患者均5～6 d出院。结论:法洛四联症修补术后室间隔残余漏介入封堵治疗安全有效,并发症少,恢复快。     

小儿法洛氏四联症的围手术期护理配合

<正>法洛氏四联症是常见的一种复杂的先天性紫绀性心脏病畸形,主要病变是肺动脉和(或)肺动脉瓣狭窄、室间隔缺损、主动脉骑跨及右心室肥厚,其发病率占各类先天性心脏病的10%~15%,其中最常见的是主动脉口狭窄和室间隔缺损[1]。法洛氏四联症患儿出生后会出现不同程度的颜面、口唇、甲床青紫,生长发育迟缓,易感乏力,劳累后紫绀加重,呼吸困难与乏力常迫使患儿采取下蹲位休息,部分患儿由于严重缺氧会发生昏厥,甚至有癫痫抽搐,如不及时治疗     

法洛氏四联症根治术中右心室-肺动脉测压与术后心脏超声压力阶差的相关性研究

目的：总结我院近两年法洛氏四联症根治术右心室流出道处理经验,研究右心室测压和心脏超声压力阶差的相关性。 方法：回顾我院2016年1月至2018年1月法洛氏四联症根治手术的连续36例患者临床资料。术中停体外循环后直接测量右心室、肺动脉压力;所有患者术中、术后查心脏超声。 结果：全组男18例,女18例,手术中位年龄0.8（0.2-37）岁,中位体重8.4（3.7-61.5）kg。10例患者（27.8%）保留肺动脉瓣环,其余25例（69.4%）行跨瓣补片,另有1例（2.8%）肺动脉闭锁患者采取心外管道。术毕直接测量右心室-肺动脉压力阶差7.6±6.9mmHg;右心室/左心室压力比值0.52±0.15。术中心脏超声检查肺动脉瓣上压力阶差13.4±12.3 mmHg,显著高于直接测量值（P=0.02）。本组患者无手术死亡,无再次手术干预,ICU停留时间5（2-17）天,术后住院时间12（7-66）天。法四保留肺动脉瓣环患者ICU停留时间及住院时间均较短（4.2±1.7天,10.4±3.7天）,与跨瓣补片患者相比差异接近显著（6.4±3.8天,16.4±12天;P=0.08及P=0.06）。随访0.9（0.2-2）年,无死亡及再次干预,半年后随访心超提示肺动脉瓣上压差较出院时显著降低（8.5±1.7mmHg,P=0.03）。 结论：法洛氏四联症患者术中经食管心脏超声可高估右心室-肺动脉压力阶差,该压力阶差在术后随访过程中逐渐下降。保留肺动脉瓣环的策略可加速术后康复。     

法洛氏四联征矫正手术配合体会

法乐氏四联症（Tetralogy of Fallot，以称法四）是最常见的先天性心脏畸形之一，在先天性心脏病中占12％～14％。其手术意义在于未做手术者自然生存率明显小于已手术者。我院自1995年以来共进行法四手术98例，现将我们手术中配合体会介绍如下。     

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     

法乐氏四联症根治术39例报告

本文报导自１９９２年１２月～１９９５年１２月法乐氏四联症根治术３９例。其中一岁以内１４例；１～３岁１７例；４～６岁４例；成人４例。手术死亡４例（死亡率１０．２５％）。存活的３５例，术后效果满意。文中重点讨论了以心包片作室缺修补连续缝合的体会，右室流出道疏通的经验以及术中测量右室一肺动脉跨瓣差的意义。     

8kg以下婴儿法洛氏四联症根治围术期治疗及并发症分析

目的 探讨8kg以下婴儿法洛氏四联症(TOF)一期根治术围手术期处理及并发症治疗。方法 2011年1月～2015年5月对81例8公斤以下婴儿术前完善检查及准备,术中细致操作,术后加强监护、个性化治疗等处理。结果 术后发生低心排6例(7.5％),死亡1例系严重低心排征并多脏器功能不全(1.2%);急性肾功能不全,腹膜透析2例(2.4%),室上性快速性心律失常5例(6.2%),肺不张4例(4.9%);膈神经麻痹1例(1.2%),心包积液2例(2.4%),均经积极治疗后恢复.结论有效的围手期治疗及积极处理并发症,是保证婴儿TOF根治手术成功的重要因素。     

法乐四联症合并肺动脉瓣缺如的矫治

目的 :探讨法乐四联症合并肺动脉瓣缺如 (TOF/ APV)的外科治疗方法。　　方法 :TOF/ APV患者 5例 ,临床上无明显呼吸道压迫症状 ,均在体外循环下矫治。用同种肺动脉 (4例用带单瓣主肺动脉片 ,1例用带瓣主肺动脉管道 )结合自体心包片重建右心室流出道。　　结果 :无手术和住院死亡。平均随访 2 3± 13个月。超声心动图检查肺动脉瓣功能良好 ,无明显反流。右心室至肺动脉平均流速及压差均较术前明显下降 ,P均 <0 .0 1。　　结论 :TOF/ APV的外科矫治应强调对扩张肺动脉的处理 ,彻底解除气道压迫。应用同种肺动脉重建右心室流出道方法简单 ,效果良好。     

Prenatal Diagnosis and Hemodynamics in Tetralogy of Fallot, Absent Pulmonary Valve with Endocardial Fibroelastosis, and Hydrops Fetalis

Tetralogy of fallot with absent pulmonary valve is rare. We report the antenatal diagnosis of this condition in early mid-semester. In this unusual case, the patient presented with hydrops fetalis. The pathophysiology is discussed. (ECHOCARDIOGRAPHY, Volume 10, November 1993)     

Echocardiographic Parameters and Brain Natriuretic Peptide in Patients after Surgical Repair of Tetralogy of Fallot

Background: Although the residual lesions after surgical correction of tetralogy of Fallot (TOF) can be evaluated by Doppler echocardiography (DE), the relation of DE parameters with the proBNP level, a potential biomarker of right ventricle overload, is not well known. The objective of this study was to evaluate the DE parameters and their relation to proBNP levels. Methods: proBNP plasma level and Doppler echocardiography parameters were obtained on the same day in 49 patients later after repair of TOF (mean age of 14.7 years, 51% female, mean PO time of 9.5 years). The DE parameters studied were the dimensions of the right atrium (RA) and ventricle (RV), RV diastolic and systolic function, and residual pulmonary lesions. The relation between them and proBNP levels were analyzed and the cutoff values of DE parameters for elevated proBNP determined. Results: proBNP was elevated in 53% and correlated with RV diastolic diameter (r = 0.41; P = 0.003), RA longitudinal (r = 0.52; P = 0.0001) and transversal (r = 0.47; P = 0.001) diameters, pressure half time of pulmonary regurgitation (PR) velocity (PHT) (r =?0.42; P = 0.005), and the PR index (r =?0.60; P < 0.001). By multivariate analysis, the PR index (r =?597; P = 0,001; CI: ?913.2 to ?280.8) and RA longitudinal (r = 7.74; P < 0,001; CI 4.18 to 11.31) were independent predictors of elevated proBNP. PHT lower than 64 msec (0.76) and PRi lower than 0.65 (0.81) had the best accuracy for elevated proBNP. Conclusion: proBNP may be increased in patients after surgical repair of TOF, correlated with the size of right cardiac chambers and the severity of PR. (Echocardiography 2010;27:442‐447)     

Pulmonary Regurgitation after Tetralogy of Fallot Repair: Clinical Features,Sequelae, and Timing of Pulmonary Valve Replacement

Pulmonary regurgitation following repair of tetralogy of Fallot is a common postoperative sequela associated with progressive right ventricular enlargement, dysfunction, and is an important determinant of late morbidity and mortality. Although pulmonary regurgitation may be well tolerated for many years following surgery, it can be associated with progressive exercise intolerance, heart failure, tachyarrhythmia, and late sudden death. It also often necessitates re‐intervention. Identifying the appropriate timing of such intervention could be very challenging given the risk of prosthetic valve degeneration and the increased risk of reoperation. Comprehensive informed and regular assessment of the postoperative patient with tetralogy of Fallot, including evaluation of pulmonary regurgitation, right heart structure and function, is crucial to the optimal care of these patients. Pulmonary valve replacement performed in an experienced tertiary referral center is associated with low operative morbidity and mortality and very good long‐term results. Early results of percutaneous pulmonary valve replacement are also promising.     

Pulmonary Valve Replacement after Repair of Tetralogy of Fallot： A Review

Tetralogy of fallot （TOF） occurs in approximately 1 in 5000 live births and accounts for 12% - 14% of congenital heart disease. Surgical repair was first introduced in the 1950s and there is now a large population of adults with repaired TOF. Some of them may suffer from significant pulmonary regurgitation （PR）, progressive right ventricle （RV） dilation, RV dysfunction and restrictive right ventricular physiology（RRVP）.