应用双向Glenn手术治疗儿童功能性单心室

目的　探讨双向Glenn手术在治疗儿童功能性单心室类型的复杂先心病中的应用价值。方法　 1998年 1月～ 2 0 0 0年 10月 ,共有 15例患儿在我院接受双向Glenn手术 ,年龄 1～ 13岁 ,平均 (4 94± 3 18)岁 ,体重 10～ 2 9kg,平均(15 13± 5 71)kg。经超声心动图检查、左右心导管术及心血管造影术确诊 ,结合手术中探查确认不宜一期生理矫治。SVC RPA端 -侧吻合 10例 ;SVC与MPA端 -侧吻合 2例 ;LSVC LPA端 -侧吻合 2例 ;LSVC LPA端 -侧吻合及RSVC RPA端 -侧吻合 1例。同期 1例行ASD扩大、1例行PDA结扎、1例合并TAPVC者行肺静脉回流口扩大。结果　有 1例术后早期死于急性肺水肿 ,全组病死率为 6 7%。术后SaO2较术前明显上升 ,P <0 0 1。 1例于术后 6个半月死于上腔静脉与肺动脉吻合口阻塞。结论　双向Glenn手术效果良好 ,作为姑息手术为二期Fontan类手术创造条件 ;也可作为部分高危患儿的终结性姑息手术     

Fontan手术的微创化发展及研究进展

Fontan手术是目前功能性单心室患者的首选治疗方法,但传统手术多次开胸带来的众多并发症仍难以避免。随着介入技术的飞速发展,外科手术和介入手段混合使用的Hybrid Fontan手术开始被尝试用于替代分期外科手术以建立Fontan循环,为Fontan手术的微创化提供了新的可能。该文介绍Fontan手术微创化的发展过程、常见Hybrid Fontan手术方法及相关研究进展。     

一期全腔静脉-肺动脉连接术治疗功能性单心室患者的中期疗效

目的探讨全腔静脉肺-动脉连接术(total cavopulmonary connection,TCPC)治疗功能性单心室患者的术后临床疗效。方法回顾性总结广东省人民医院2008年1月1日至2016年12月31日一期TCPC治疗功能性单心室患者50例的临床资料。其中女17例(34%),男33例(66%);手术年龄(10.5±7.6)岁,体质量(25.3±13.2)kg。所有患者术前均有活动后发绀、气促等临床表现。术前动脉血气示饱和度为80.1%±7.5%,术前血红蛋白浓度为(176.9±29.3)g/L,术前红细胞压积为0.52%±0.09%,术前肺动脉指数为(345.5±174.0)mm^2/m^2,术前肺动脉压力为(14.4±4.3)mmHg(1 mmHg=0.133 kPa)。随访截至2016年12月31日。结果全组患者住院期间病死率为10%,术后5年、8年生存率均为86%。COX多因素回归分析未发现与术后死亡相关的危险因素。随访期间10例患者需再次干预治疗,主要并发症包括反复胸腔积液、心包积液、房室瓣反流、蛋白丢失性肠病。术后5年、8年未干预率为82%,71%。结论一期TCPC是治疗功能性单心室达到生理性矫治的有效手术方案,但术前应严格把握适应证,具备条件的患者可行一期手术,对高危患者仍需行分期手术降低手术风险,减少死亡及中远期并发症。     

改良Fontan手术治疗功能单心室合并左异构的早中期临床结果

目的:总结分析改良Fontan手术治疗功能单心室合并左异构的早期及中期临床结果。方法:回顾性纳入中国医学科学院阜外医院自2009年1月至2016年1月完成的改良Fontan手术治疗功能单心室合并左异构患者19例,依据是否分期完成改良Fontan手术,分为一期手术组(n=9)和分期手术组(n=10),对比分析两组患者术后早期及中期的临床结果。结果:19例患者中,男性13例(68.4%),女性6例(31.6%)。分期手术组患者行第一期Glenn手术时的平均年龄为(1.87±1.56)岁;两组患儿行Fontan手术时的平均年龄为(5.09±2.01)岁。住院期间死亡1例,为分期手术组患者;平均随访时间4.2(1~9)年,随访期间无死亡患者。两组患者术前在性别、年龄、身高、体重、心室形态、房室瓣反流及上腔静脉形态、肺动脉发育和平均肺动脉压力等方面的差异均无统计学意义(P均>0.05);术中体外循环时间、阻断时间、管道建立方式及术后住院时间的组间差异也均无统计学意义(P均>0.05)。在重症监护病房停留时间、机械辅助通气时间及术后血浆用量、胸腔引流量、胸腔引流时间、腹腔积液发生率等方面,一期手术组均优于分期手术组,差异均有统计学意义(P均<0.05)。两组患者术后均出现进行性血氧饱度下降,各有1例患者经皮血氧饱和度<85%,组间差异无统计学意义(P>0.05)。结论:与分期Fontan手术相比,一期Fontan手术治疗年龄偏大的功能单心室合并左异构患者具有良好的围术期结果,两种手术策略在早期和中期疗效方面无明显差异,术后均会出现氧饱和度进行性下降的问题,需要远期随访。     

一期Fontan手术治疗复杂紫绀型先天性心脏病21例

目的：总结一期房坦（ Fontan）手术治疗复杂紫绀型先天性心脏病的临床经验和疗效。方法2007-06～2012-12该院心血管外科对21例复杂紫绀型先天性心脏病患者施行了一期Fontan单心室手术,同期行房室瓣整形术8例。12例采取心内管道或自体心包内隧道连接,8例采取心外管道连接,1例采取不用管道材料肺动脉直接下拉吻合法。5例保留房间隔缺损或行心房板障开窗。结果20例手术获得成功,1例术后当天因低心排出量综合征死亡。1例房室瓣成形术后随访至25个月出现房室瓣重度反流,行机械瓣置换。术前经皮血氧饱和度为67％～91％,术后经皮血氧饱和度为88％～95％。术后随访（7～52个月）所有患者心功能Ⅰ～Ⅱ级。结论一期Fontan手术治疗复杂紫绀型先天性心脏病安全有效,可以避免多次手术创伤,节约医疗资源,但应注意把握手术适应证。     

下腔静脉与肺动脉直接连接的改良Fontan手术护理配合

目的 总结下腔静脉与肺动脉直接连接的改良Fontan手术护理配合.方法 在体外循环下,对29例发绀型复杂先天性心脏病患者采用下腔静脉与肺动脉直接连接术,术中做好各项护理配合.结果 手术死亡1例,术后死于重度低心排血量综合征.其余28例患者术后血流动力学稳定,术后复查下腔静脉与肺动脉吻合口均通畅.结论 复杂先天性心脏病患者常在开胸探查后才能确定是否采取直接连接法,不同的术式需准备不同的器械和用物,要求护士须熟悉手术方法,提前准备好各种材料;因应手术方式改变,及时提供所需的器械、物品及药品等;熟悉手术步骤,了解主刀医生习惯,术中默契配合,可缩短手术时间,保证手术顺利进行.     

依赖单心室功能进行复杂先天性心脏病矫治手术的进展

依赖单心室功能进行复杂先天性心脏病矫治手术 ,适用于不能安全进行双心室解剖矫治的复杂先天性心脏病。术式不断改进。其目的是提高单心室生理矫治手术的生存率、减少围手术期和术后长期并发症 ,以达到最佳的生理矫治。本文就依赖单心室功能进行复杂先天性心脏病矫治手术进行综述。     

功能性单心室并右心室双出口1例

患者男性，17岁。因口唇发绀14年入院。患者3岁时出现口唇发绀，平时易患感冒，出汗多，剧烈活动时嘴唇、四肢末梢发绀加剧，无缺氧发作、晕厥及心力衰竭史。查体：口唇及肢端发绀，杵状指（趾）。心界不大，未触及震颤。P2音减弱，A2〉P2。肺动脉瓣听诊区可闻及3／6级收缩期吹风样杂音，传导局限。胸骨左缘2～4肋间可闯及3／6级收缩期吹风样杂音，传导局限。血氧饱和度77％～81％。心电图：窦性心律，室内传导阻滞，心房、心室肥大，ST-T改变。胸片：肺血少，肺纹理纤细，主动脉结似呈右位，降主动脉轮廓不清，肺动脉段凹，右心缘较平直，左心缘丰满。心胸比：0．42。心脏彩超：先天性复杂心脏畸形，多叶脾，心房左同型位，单心房、单心室（A型），肺动脉瓣及瓣下流出道狭窄（图1，图2）。     

成人单心室一例

患者,男,20岁。因“反复呼吸困难20年,再发伴发热、咳嗽4天”入院。患者系足月顺产,母孕期体健,否认毒物、放射线接触史及家族遗传病史。患者20年来反复出现呼吸困难、胸闷、气促,活动耐量下降,     

Electrophysiologic findings after Fontan repair of functional single ventricle

Cardiac arrhythmias are well recognized sequelae of the Fontan operation for complex congenital anomalies. In this study the electrophysiologic effects of the Fontan procedure were evaluated in 30 patients who underwent cardiac catheterization with electrophysiologic study 1.9 +/- 1.3 years (mean +/- SD) after modified Fontan repair for functional single ventricle. Abnormalities of sinus node or ectopic pacemaker automaticity were detected in 50% (15 patients) by determination of a prolonged corrected sinus node or pacemaker recovery time. Total sinoatrial conduction time was prolonged in 50% of the patients with normal sinus rhythm. Sinus node or ectopic atrial pacemaker function was entirely normal in only 43% of patients. The predominant atrial rhythm was normal sinus in 70% and ectopic atrial or junctional in 30%. Abnormalities of atrial effective and functional refractory periods were noted in 43% of patients and were most pronounced at faster paced cycle lengths. Atrial endocardial catheter mapping revealed intraatrial conduction delays between adjacent sites in 76% of the patients tested and in eight of nine patients with inducible intraatrial reentry. Programmed atrial stimulation induced nonsustained supraventricular arrhythmias in 10% of the 30 patients and sustained arrhythmias in 27%. Intraatrial reentry was the most common inducible arrhythmia and was present in seven of the eight patients with sustained and two of the three patients with nonsustained atrial arrhythmias. Atrioventricular conduction abnormalities were noted in 10% (three patients). No patient had inducible ventricular arrhythmias with programmed ventricular stimulation. The electrophysiologic findings after Fontan repair include abnormal sinus node function, prolonged atrial refractoriness, delayed intraatrial conduction and inducible atrial arrhythmias.(ABSTRACT TRUNCATED AT 250 WORDS)     

The Fontan procedure in adults

SETTING—Tertiary adult congenital cardiac referral centre.
DESIGN—Retrospective cross sectional analysis.
OBJECTIVES—To report our 20 year experience with adult Fontan operations, and to compare late outcome in patients with single ventricle with definitive aortopulmonary or cavopulmonary shunt palliation.
PATIENTS AND MAIN OUTCOME MEASURES—Patients older than 18 years undergoing Fontan operation between 1 January 1982 and 31 December 1998 were identified. Mortality and late outcome were derived from hospital records. These patients were compared with a cohort of 50 adults with single ventricle who had not undergone a Fontan operation.
RESULTS—61 adults, median age 36 years (range 18-47 years), with a median follow up of 10 years (range 0-21 years) were identified. Actuarial survival was 80% at one year, 76% at five years, 72% at 10 years, and 67% at 15 years. Compared with before the Fontan operation, more patients were in New York Heart Association (NYHA) functional class I or II at the latest follow up (80% v 58%, p < 0.001). Systolic ventricular function deteriorated during follow up such that 34% had moderate to severe ventricular dysfunction at the latest follow up compared with 5% before Fontan (p < 0.001). Arrhythmia increased with time (10% before Fontan v 57% after 10 years, p < 0.001). Fontan patients had improved NYHA functional class, ventricular function, atrioventricular regurgitation, and fewer arrhythmias than the non-Fontan group at the latest follow up.
CONCLUSION—The Fontan operation in adults has acceptable early and late mortality. Functional class, systolic ventricular function, atrioventricular regurgitation, and arrhythmia deteriorate late after surgery but to a lesser degree than in non-Fontan patients with a single ventricle.


     

Flow dynamics in the main pulmonary artery after the Fontan procedure in patients with tricuspid atresia or single ventricle

We analyzed the flow velocity pattern in the main pulmonary artery after Fontan operation in patients with tricuspid atresia (n = 10) or with single ventricle (n = 10) by means of a catheter-mounted velocity probe. The area underneath the velocity signal of the forward flow was integrated, and ratios of the portions during atrial systole and during the diastolic phase to the total area (Fa and Fd) were calculated. The Fa was 0.54 +/- 0.09 in patients with tricuspid atresia and 0.45 +/- 0.05 in those with single ventricle (p less than .01). Cardiac output, obtained by the thermodilution method, was 2.45 +/- 0.48 liters/min/m2 in patients with tricuspid atresia and 2.75 +/- 0.72 liters/min/m2 in those with single ventricle. The forward flow during atrial contraction, calculated by multiplying Fa by cardiac output, was 1.32 +/- 0.35 liters/min/m2 in patients with tricuspid atresia and 1.23 +/- 0.33 liters/min/m2 in those with single ventricle. The diastolic forward flow, calculated from Fd and cardiac output, was 0.99 +/- 0.25 liter/min/m2 in patients with tricuspid atresia and 1.52 +/- 0.45 liters/min/m2 in those with single ventricle (p less than 0.005). The sum of cross-sectional areas of the right and left pulmonary arteries normalized by body surface area (PA index) was 282 +/- 85 cm2/m2 in patients with tricuspid atresia and 462 +/- 65 cm2/m2 in those with single ventricle (p less than .005). The Fa was inversely correlated with the PA index in the whole group (r = -.69) and also in the tricuspid atresia group alone (r = -.87).(ABSTRACT TRUNCATED AT 250 WORDS)     

Influence of myocardial mass of the univentricular heart on exercise capacity in patients with functional single ventricle and Fontan surgery

BACKGROUND: The Fontan operation causes an acute decrease of volume overload of the univentricular heart followed by changes in ventricular geometry. The postoperative increase of myocardial mass-volume-index (MVI) may alter ventricular diastolic function. In this study, we analysed whether the increase in MVI and changes of the ventricular geometry have an effect on the decrease of the exercise capacity in patients with Fontan surgery. METHODS: We examined the cardiopulmonary function of 24 patients with functional single ventricle and Fontan operation 3.63 +/- 1.97 years after surgery (m = 14, w = 10, age: 14.57 +/- 9.74 years) using a bicycle cardiopulmonary exercise testing. The parameters of exercise capacity and cardiopulmonary function were correlated with Magnetic Resonance Imaging (MRI) parameters such as the MVI, enddiastolic ventricular muscle mass (EDMM) und endsystolic volume (ESV). RESULTS: The exercise capacity was 2.06 +/- 0.54 W/kg (60.63 +/- 15.75% of the norm) and VO(2)max was 27.41 +/- 8.87 ml/min/kg (60.91 +/- 19.7% of the norm). There was a positive correlation of exercise capacity and VO(2)max with ESV (r(2) = 0.2572, p = 0.033) and EDMM (r(2) = 0.2544, p = 0.024), but none with the MVI. CONCLUSION: Myocardial hypertrophy may influence the myocardial performance of the univentricular heart and thereby the physical performance in children and adults with Fontan circulation.     

Ventricular function in the single ventricle before and after Fontan surgery

To better delineate the importance of ventricular function in patients with a single ventricle and assess its relation to outcome after the Fontan procedure, 47 patients with a single ventricle were studied. Ventricular ejection fraction was estimated by radionuclide angiocardiography. Before Fontan surgery, ejection fraction was 0.57 ± 0.10 (mean ± standard deviation). This differed significantly from the normal mean left ventricular ejection fraction of 0.68 ± 0.09 (p < 0.001) derived in our laboratory by radionuclide angiocardiographic methods. Age, ventricular morphology and the presence of pulmonary artery band or systemic to pulmonary artery shunts had no statistical relation to ventricular ejection fraction in patients with a single ventricle. Serial preoperative evaluation in 15 patients over 3.8 ±1.3 years revealed no significant change in ventricular ejection fraction; however, increased atrioventricular valve regurgitation was documented in 4 of these 15. Modified Fontan procedure was performed in 24 of the 47 study patients; 7 have died, 1 has undergone cardiac transplantation and 1 faces possible transplantation. No difference was noted in preoperative ejection fraction between survivors and nonsurvivors. Ventricular morphology, age at Fontan surgery and operative factors such as bypass and cross-clamp time were not related to functional outcome. Preoperative ejection fraction of 0.52 ± 0.08 decreased to 0.39 ± 0.11 (p < 0.001) when evaluated 1.16 ± 0.44 years after Fontan surgery. In patients with a single ventricle (1) ventricular ejection fraction is less than that of the normal systemic ventricle; (2) during childhood, ejection fraction is not related to age or ventricular morphology; and (3) ventricular ejection fraction frequently decreases after a Fontan repair. Thus, long-term studies of clinical course and ventricular function are essential before altering recommendations for pursuing the Fontan repair.     

Single ventricle palliation: greater risk of complications with the Fontan procedure than with the bidirectional Glenn procedure alone

BACKGROUND: This study was performed to evaluate and compare the early, intermediate, and long-term outcomes of the bidirectional Glenn procedure and Fontan procedure in patients who live at moderately high altitude. METHODS: The outcome of each method of palliation for patients with a functionally single ventricle was retrospectively evaluated from a review of medical records. RESULTS: The bidirectional Glenn procedure was performed in 177 patients from October 1984 to June 2004. The Fontan procedure was performed in 149 patients from June 1978 to June 2004. Cardiovascular death or heart transplantation occurred in 8% of patients after the bidirectional Glenn procedure and 17% of patients after the Fontan procedure. Complications of systemic thromboembolic events, bleeding associated with anticoagulation therapy, protein losing enteropathy, and arrhythmias requiring implantation of a pacemaker, cardioversion, or radiofrequency ablation occurred in 7% of patients after the bidirectional Glenn procedure and 47% of patients after the Fontan procedure. Cardiovascular deaths and heart transplantation occurred less frequently when the Fontan procedure was performed in patients with a previous bidirectional Glenn procedure. However, the actuarial transplant-free survival and freedom from complications was not superior for a subgroup of patients who had a Fontan procedure after a bidirectional Glenn procedure in comparison to a subgroup of patients who had a bidirectional Glenn procedure alone. CONCLUSIONS: The bidirectional Glenn procedure can be used for long-term palliation of patients with a functionally single ventricle. Additional palliation with a Fontan procedure may increase the risk of stroke, protein losing enteropathy and arrhythmias without improving survival.     

Exercise tolerance and cardiorespiratory response to exercise after the Fontan operation for tricuspid atresia or functional single ventricle

To determine the impact of the Fontan operation on exercise tolerance and on the cardiorespiratory response to exercise, we compared the results of graded exercise to maximal effort of 81 patients with tricuspid atresia or single functional ventricle studied preoperatively with those of 29 patients studied postoperatively. Postoperatively, the values for total work performed, duration of exercise and maximal oxygen uptake increased significantly. Regardless of operative status, the maximal heart rate during exercise was reduced. The cardiac output and stroke volume response to exercise were subnormal after operation. Systemic arterial blood oxygen saturation was reduced markedly preoperatively both at rest and during exercise; postoperatively, it was significantly greater than the preoperative value but it remained slightly abnormal. The ventilatory response to exercise (respiratory rate, minute ventilation and ventilatory equivalent for oxygen) decreased toward normal after operation. Exercise tolerance and the cardiorespiratory responses to exercise improve after the Fontan operation. Formal exercise testing is essential to quantitate the degree of improvement.     

Response to the Valsalva maneuver after the Fontan procedure for tricuspid valve atresia, single ventricle or pulmonic valve atresia

The Valsalva maneuver was performed during cardiac catheterization in 9 patients who had undergone a Fontan operation (Fontan group) and in 10 control subjects. The Fontan group had higher right atrial (RA) pressure (16 +/- 1 vs 4 +/- 0.5 mm Hg, p less than 0.001), lower cardiac index (2.5 +/- 0.1 vs 3.7 +/- 0.2 liters/min/m2, p less than 0.05), lower stroke index (32 +/- 2 vs 44 +/- 2 ml/beat/m2, p less than 0.05), and higher systemic vascular resistance (31 +/- 1 vs 24 +/- 1 units, p less than 0.05) than the control group. Four patients in the Fontan group had a normal 4-phase Valsalva response, and 5 did not (p less than 0.05). RA pressure was similar in those who responded normally and those who did not (16 +/- 0.5 vs 17 +/- 1 mm Hg), whereas in those who responded normally cardiac index was higher (2.9 +/- 0.2 vs 2.2 +/- 0.1 liters/min/m2, p less than 0.05), stroke index was higher (36 +/- 1 vs 28 +/- 1 ml/beat/m2, p less than 0.05), and systemic vascular resistance was lower (28 +/- 1 vs 31 +/- 1, p less than 0.05). It is concluded that cardiac output is a better predictor of a normal response to the Valsalva maneuver than RA pressure in patients after the Fontan procedure. A normal response to the supine Valsalva maneuver suggests a cardiac index greater than 2.4 liters/min/m2 and stroke index greater than 31 ml/beat/m2.     

Impact of early ventricular unloading on exercise performance in preadolescents with single ventricle Fontan physiology.

OBJECTIVES: We sought to determine if early ventricular volume unloading improves aerobic capacity in patients with single ventricle Fontan physiology. BACKGROUND: Surgical strategies for patients with single ventricle include intermediate staging or early Fontan completion to reduce the adverse affects of prolonged ventricular volume load. The impact of this strategy on exercise performance has not been evaluated. METHODS: Retrospectively, we reviewed the exercise stress test results of all preadolescents with single ventricle Fontan physiology. "Volume unloading" was considered to have occurred at the time of bidirectional cavopulmonary anastomosis or at the time of Fontan surgery in those patients who did not undergo intermediate staging. Potential predictors of aerobic capacity were analyzed using multivariate regression. RESULTS: The patients (n = 46) achieved a mean percentage predicted of maximal oxygen consumption (VO2max) of 76.1% +/- 21.1%. The mean age at the time of volume unloading was 2.7 +/- 2.4 years, and the mean age at testing was 8.7 +/- 2 years. Intermediate staging was performed in 16 of 46 patients (35%). In multivariate analysis, younger age at volume unloading was associated with increased aerobic capacity (p = 0.003). Other variables were not predictive. The subgroup of patients who underwent volume unloading before two years of age achieved a mean percentage predicted VO2max of 88.6% +/- 24.1%. CONCLUSIONS: Preadolescents with single ventricle who undergo volume unloading surgery at an early age demonstrate superior aerobic capacity compared with those whose surgery is delayed until a later age.     

The Fontan procedure

The Fontan procedure has afforded improved surgical repair for several complex congenital cardiac defects, including tricuspid atresia and single ventricle. Through surgical creation of a connection between the RA and the RV or PA, adequate pulmonary perfusion can be achieved without an RV. Although it is not an anatomic connection, the Fontan procedure is a more physiologic approach than the previously used shunt procedures. Systemic venous return and PVR are effectively separated within the heart, pulmonary blood flow is assured through an RA-to-PA connection, and ventricular volume overload is avoided. The procedure has been effective in relieving cyanosis and has resulted in improved levels of exercise tolerance after surgery.