婴儿法乐四联症外科治疗的危险因素分析

目的 探讨婴儿法乐氏四联症Ⅰ期矫治手术早期死亡的危险因素。方法 统计56例婴儿法乐氏四联症患儿I期手术治疗的临床资料，对年龄、体重、室缺大小、左室舒张末期容积指数、肺动脉发育、主动脉阻断时间、心肌保护、术后并发症、术后呼吸机辅助时间、手术病死率等15项围手术期指标进行分析。结果 术后早期死亡11例，病死率19．6％(11／56)，单因素分析结果表明术后低氧血症、严重心律失常、低心排血量综合征是手术早期死亡的高危因素，Logistic危险因素多因素回归分析显示，排除病情严重程度的混杂因素后，传统心肌保护方法是婴儿法乐氏四联症Ⅰ期矫治手术早期死亡的主要原因。结论 婴儿法乐氏四联症Ⅰ期根治手术是可行的，改良心肌保护比传统心肌保护疗效好。     

18例小儿先天性冠状动脉瘘的外科治疗

目的：探讨小儿先天性冠状动脉瘘的外科治疗。方法：１８例先天性冠装动脉瘘患儿中，右冠状动脉瘘１３例（７２．２％），最常见的瘘入心脏为右心室（５５．６％）。除１例施行冠状动脉瘘支直接结扎手术，均在体外循环下施行手术矫治。结果：全组无手术死亡和严重术后并发症。结论：冠状动脉瘘在诊断明确后，应及时手术治疗；体外循环下直视修复术是安全、可靠的治疗方法。     

儿童右肺动脉异常起源于主动脉的诊断与外科治疗

目的 评价儿童右肺动脉异常起源于主动脉(aortic origin of the right pulmonary artery,AORPA)的治疗方法及效果,并探讨AORPA的诊断与鉴别诊断方法.方法 回顾性分析1993年5月至2007年7月11例儿童AORPA术前诊断及手术治疗的围术期疗效及中长期随访结果.分别采用右肺动脉经人工血管(2例)或同种主动脉(2例)与主肺动脉吻合术、体外循环下右肺动脉与主肺动脉直接吻合术(3例)、体外循环下(主动脉环及肺动脉环片)双片法右肺动脉与主肺动脉吻合术(2例)、非体外循环下右肺动脉经人工血管与主肺动脉吻合术(2例).结果 11例中10例术前确诊,1例术前漏诊而在术中发现.5例通过右室造影及主动脉造影或多排CT/MRI检查确诊,6例单纯超声心动图检查患儿中确诊5例,1例漏诊.11例手术患儿围术期死亡2例.术后随访2.2～13年,1例于术后4年死于右心功能衰竭.生存8例患儿术后超声心动图复查射血分数(EF)值43%～52%,心功能I级5例,Ⅱ级3例.结论 右室造影及主动脉造影或多排CT/MRI检查可减少漏诊率.手术是治疗AORPA的有效方法,但应尽早手术以进一步提高手术疗效.应用自体组织加宽加长右肺动脉可减少吻合口狭窄的可能性.     

婴幼儿先天性心脏病的外科治疗

自1997年10月～1998年12月在我科接受外科手术治疗的208例心脏病病人中,体重在10ｋｇ以下者34例。现就婴幼儿先天性心脏病外科治疗的体会总结如下。临床资料34例中男23例,女11例,年龄8个月～2.5ａ。体重5.5作者单位:450003　郑州,河南省胸科医院小儿心脏外科～10ｋｇ。法乐四联症根治术7例,法乐三联症根治术1例,部分型房室管畸形矫治术4例,完全型房室管畸形矫治术2例,Ⅰ型无顶冠状静脉窦综合征矫治术1例,三尖瓣闭锁改良Ｆｏｎｔａｎ手术1例,室间隔缺损修补术16例(其中包括合并未闭动脉导管同期缝合术4例,二尖瓣畸形同期直视成形术1例,下腔静…     

右腋下小切口在小儿心内直视手术中的应用研究

目的比较小儿右腋下小切口与胸骨正中切13行心内直视手术的临床资料,探讨右腋下小切口先心病手术的临床效果。方法我们自2012年12月至2013年12月实施右腋下小切口小儿心内直视手术102例,与同期胸骨正中切口手术主要诊断类似患儿102例比较,进行回顾性分析。结果两组主动脉阻断时间、体外循环时间及手术时间比较,差异无统计学意义（P〉0．05）。呼吸机辅助通气时间、术后24h引流量以及术后住院时间比较,右腋下小切口优于胸骨正中切口,差异有统计学意义（P〈0．01）。结论采用右腋下小切口能安全有效地完成先心病心内直视手术,治疗效果好,术后恢复快,切口美观。     

小儿心内直视手术探讨

目的 探讨基层医院开展小儿心内直视手术的经验和教训。方法 自1996年初至2001年5月对103例小儿先天性心脏病行心内直视手术，其中房缺修补25例、室缺修补62例、部分房室管畸形矫治5例、法乐氏四联症根治5例、肺动脉瓣切开4例，其它2例。结果 术后心脏自动复跳65例，电击复跳23例，心脏未停跳15例；术后并发症：出血2例，心包填塞1例，心律失常1例；术后死亡3例。结论 掌握好适应症，选择合适的病例，加强术前、术中及术后各个环节的处理，增强医护人员责任心，基层医院开展小儿心内直视手术还是切实可行的。     

儿童漏斗胸合并先天性心脏病的纠治

目的 探讨儿童漏斗胸合并先天性心脏病的同期手术的可行性及手术方法。方法 采用改良Ravitch术式对 11例漏斗胸合并先天性心脏病患儿在常规体外循环手术后施行了同期纠治术,其中男7例,女4例,平均5.4岁;3例继发孔房间隔缺损,6例室间隔缺损,1例法洛四联症,1例动脉导管未闭,术前心功能Ⅰ级4例,Ⅱ级6例,Ⅲ级1例。心内矫治术操作毕,放置有胸腔引流管。结果 所有患儿均平稳地渡过了体外循环。术中主动脉阻断时间、体外循环时间、术后的出血和住院时间与同期类似体外循环手术患儿相比差异无显著性意义(P>0.05),术后循环 18～48 h拔除气管插管、48～72 h拔除胸腔引流管,无术后感染、肺不张等并发症,未见明显二氧化碳潴留。漏斗容积由术前的(26.09±5.54)ml降到了(6.55±1.37)ml。结论 同期手术术后的过程是平稳的,避免了再次手术的麻醉及在胸骨劈开心内直视手术后行漏斗胸纠治所带来的困难,手术结果证明是安全和满意的。     

心脏不停跳心内直视手术治疗先天性心脏病

目的评价体外循环下不阻断升主动脉心内直视术对心肌的保护作用。方法选择先天性心脏病患儿58例,采用枸橼酸芬太尼、维库溴铵静脉诱导麻醉,常规建立体外循环,阻断上下腔静脉,不阻断升主动脉,心脏在空跳情况下手术。结果58例均无手术死亡,无相关中枢神经系统并发症发生,体外循环时间(36.29±12.74)min,术后9-14 d均痊愈出院。结论心脏不停跳法是一种较接近生理状态的心肌保护方法,能最大程度地减少心肌缺氧缺血损伤、避免再灌注损伤,而获得较理想的心肌保护效果。     

同种带瓣管道治疗复杂先天性心脏病(附31例报告)

目的研究同种带瓣管道治疗复杂先天性心脏病的疗效。方法1997年12月～2003年4月，我科应用同种带瓣管道治疗复杂先天性心脏病31例。病种包括重症法乐氏四联症(TOF)14例，其中合并动脉导管未闭(PDA)1例，合并肺动脉闭锁(PA)、右肺动脉缺如(RPaA)各1例，PDA PA 1例，右冠状动脉畸形(RCaA)2例；右室双出口(DORV)8例，其中合并RCaA2例；三尖瓣闭锁(TA)1例；大动脉转位(L-TGA)并室间隔缺损(VSD)、肺动脉狭窄(Ps)3例；永存动脉干(PTA)2例；主动脉缩窄(CoA)并PDA 1例；VSD并PS、RCaA 1例；亚急性细菌性心内膜炎(SBE)1例。术式：主、肺动脉单瓣作右室流出道跨瓣补片术14例；Rastelli手术11例；右心室-肺动脉连接(RV-PA)3例；肺动脉瓣置换1例：Ross手术1例；改良Fantan手术1例。结果主、肺动脉单瓣作右室流出道跨瓣补片组14例，1例术后3d死于右心功能不全(7．14％)。存活13例，随访3～57月，超声心动图检查显示移植后瓣膜启闭功能良好，无增厚及粘连。同种带瓣管道移植矫治组17例，死亡7例(41．2％)，存活10例，随访9～57月，管道血流通过顺畅，管壁无变薄、钙化。结论同种带瓣管道具有管壁弹性好，易于缝合，术后排斥反应少等优点，可广泛应用于复杂先天性心脏病矫治术、左，右室流出道的重建。同种带瓣管道补片既能加宽右室流出道，且有良好抗反流作用，有利于术后患儿心功能恢复，使部分复杂的病例能够一次手术根治。     

完全性肺静脉异位连接的外科治疗

目的 总结10例完全性肺静脉异位连接的外科治疗经验。方法 全组均在全身麻醉中度低温体外循环下进行手术，6例心上型患儿早期1例经后径法矫治，其他5例中3例经右心房切口径路矫治；另2例采用心上法矫治；4例心内型1例采用自体心包片将冠状静脉窦口经扩大的房间隔缺损隔入左心房，后3例用5-0 Prolene线连续缝合房间隔粗糙边缘后再矫治。结果 无手术死亡，发生心律失常3例，一过性肺水肿2例，均经治疗痊愈。全组随访4个月～5年，心功能正常。结论 提高手术成功率和防止术后并发症的关键为：术中吻合口要足够大，扩大左心房容积及术后及时处理心律失常、肺水肿和低心排血量。心内型矫治时消除房间隔粗糙面可防止术后肺静脉梗阻。     

Ventricular dysrhythmias after congenital heart surgery: a canine model

To examine the possible mechanisms of ventricular dysrhythmias in the presence of right ventricular hypertension and following ventriculotomy, we instrumented 6-month-old puppies. There were four groups: 1) six animals served as controls (instrumentation only); 2) six animals underwent ventriculotomy only; 3) six animals underwent pulmonary artery banding with a pneumatic vessel occluder to produce right ventricular hypertension; 4) six animals had both ventriculotomy and right ventricular hypertension. Each week for 8 wk, 24-h electrocardiograms and electrophysiologic studies were performed with the animals awake and unsedated. We attempted to induce ventricular dysrhythmias with premature extrastimuli, rapid pacing, isoproterenol, and vagal stimulation. The following "chronic" data were obtained in week 8 (p value for overall analysis of variance; values are mean +/- SD): heart rate - 126 +/- 16 beats/min (no significant difference between groups; all animals); right ventricular systolic pressure: control 26 +/- 6 mm Hg, ventriculotomy 30 +/- 3, right ventricular hypertension 65 +/- 5, ventriculotomy and right ventricular hypertension 75 +/- 18 (p less than 0.001); right ventricular end diastolic: control 4 +/- 1 mm Hg, ventriculotomy 4 +/- 3, right ventricular hypertension 11 +/- 5, ventriculotomy and right ventricular hypertension 16 +/- 7 (p less than 0.001); QRS duration: control 22 +/- 5 ms, ventriculotomy 33 +/- 7, right ventricular hypertension 44 +/- 6, ventriculotomy and right ventricular hypertension 49 +/- 4 (p less than 0.01); right ventricular apex-base interval with ventricular pacing: control 20 +/- 3 ms, ventriculotomy 34 +/- 9, right ventricular hypertension 30 +/- 5, ventriculotomy and right ventricular hypertension 31 +/- 6 (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)     

Anatomic confirmation of echocardiographic measurements in neonatal hearts.

Significant correlations were demonstrated between echocardiographic measurements of left ventricular wall thickness, right ventricular wall thickness, septal thickness, left ventricular mass, aortic valve excursion, pulmonary valve excursion, mitral valve excursion, and tricuspid valve excursion and the same measurements made directly on the same hearts at autopsy. A new regression formula was derived for the calculation of echocardiographic right ventricular mass in life and was found to correlate significantly with right ventricular mass measured as the sum of right ventricular wall and septal volumes at postmortem examination.     

Membranous septal aneurysm: an unusual cause for right ventricular outflow tract obstruction in a malaligned ventricular septal defect with aortomitral discontinuity (double-outlet right ventricle) associated with visceral heterotaxy

The development of a septal aneurysm in the natural history of membranous ventricular septal defects usually makes the defect hemodynamically less significant. This report describes a case of severe right ventricular outflow obstruction produced by a membranous septal aneurysm in a patient who had an anterior malaligned ventricular septal defect with aortomitral discontinuity (double-outlet right ventricle). This patient did not have pulmonary stenosis other than the dynamic obstruction produced by the septal aneurysm. In this patient, the septal aneurysm produced both favorable and unfavorable hemodynamic effects. A reduction in the size of the ventricular septal defect produced a favorable effect, whereas a right ventricular outflow obstruction led to the unfavorable situation of right ventricular hypertension and hypertrophy. The large septal aneurysm in the presence of an already compromised right ventricular outflow tract related to an anteriorly malaligned septum resulted in severe obstruction.     

Surgical management of pulmonary atresia with intact ventricular septum. Right ventricular size as a guideline for surgical intervention.

61 infants with critical valvular pulmonary stenosis (21 cases) or pulmonary atresia with intact ventricular septum (40 cases) were operated on between 1975-1989 in the Semmelweis University Medical School. Right ventricular volume, area, outflow tract dimension and tricuspid anulus diameter was measured by angiocardiography and echocardiography to evaluate right ventricular size, and to predict the operative outcome using these data. Our results suggested, that if the normalized right ventricular volume was less than 3 ml/m2, the normalized right ventricular area was less than 2.5 cm2/m2, and the normalized right ventricular area was less than 2.5 cm2/m2 and the normalized tricuspid anulus diameter was less than 1.2 cm2/m2/3 only a systemopulmonary shunt procedure is needed. In all other cases pulmonary valvotomy is necessary to decompress the right ventric and to help increase the right ventricular size. Where a different part of the right ventricle is hypoplastic a systemopulmonary shunt procedure is needed too. In the follow up period the right ventricular dimension and tricuspid valve diameter was measured by echocardiography. This noninvasive assessment can predict the preoperative diagnosis, postoperative outcome and demonstrates an adequate growth of the right ventricle after pulmonary valvotomy.     

Cardiac atrial natriuretic peptide in monocrotaline-induced pulmonary hypertensive rats

To determine the time course and potential triggers for synthesis of atrial natriuretic peptide (ANP) in right ventricle during the development of right ventricular hypertrophy (RVH), we measured mean right atrial pressure, right ventricular systolic pressure (RVSP), immunoreactive ANP (iANP) concentrations in plasma, and cardiac iANP concentrations and contents of monocrotaline (MCT)-induced pulmonary hypertensive rats treated with a subcutaneous injection of MCT (40 mg/kg). Following the development of RVH and pulmonary hypertension, iANP concentrations in plasma and iANP concentrations and contents in ventricular tissues increased with time. At the third week after treatment, iANP concentrations in MCT rats increased 6-fold in plasma, 57-fold in the right ventricular free wall, 20-fold in the ventricular septum and 10-fold in the left ventricular free wall compared with those in controls. At the third week, iANP contents of total ventricular tissues in MCT and control rats were 29% and 0.8%, respectively, of the corresponding atrial contents. The iANP concentrations and contents of right ventricular free walls in MCT rats were greater than those of any other parts of ventricular tissues. The iANP concentrations in right ventricular free walls were very closely related to RVSP (r = 0.93, P < 0.001). In MCT rats, iANP concentrations in right atrial tissues decreased with time. This study shows that ventricular ANP contributes to the amount of ANP stored within the heart in MCT rats and that pressure overload may be one of the triggers for ANP synthesis in the right ventricle.     

Electrocardiographic Standard for the Diagnosis of Right Ventricular Hypertrophy in Complete Right Bundle Branch Block

It is generally considered that in complete right bundle branch block, the electrocardiographic standard for the diagnosis of right ventricular hypertrophy is not applicable. According to Cohen's criteria, right ventricular overload exists if thallium-201 myocardial perfusion demonstrates the right ventricular wall, and in this light we attempted to reassess the validity of the electrocardiographic standard for right ventricular hypertrophy. Twenty-three children with complete right bundle branch block (abbreviated as CRBBB) were evaluated. The agreement and disagreement ratios of the electrocardiographic standard with Cohen's criteria was 20/23 and 3/23 respectively. Even in patients without intracardiac anomalies, right ventricular overload was suspected by Cohen's criteria. The electrocardiographic standard was in agreement with Cohen's criteria in most of these patients.     

Dependence of canine right coronary arterial flow upon heart rate and right ventricular pressure

There is a paucity of knowledge regarding right coronary pulsatile hemodynamics when the right ventricle is under hemodynamic overload as is often the case in pediatric patients with congenital cardiac anomalies. To elucidate the exact mechanisms for the right coronary artery (RCA) to cope with the overload, we studied nine open-chest adult Beagles and analyzed the flow signals of the RCA in relation to independently varied heart rate (pacing) and right ventricular pressure (pulmonary artery banding). Both increased heart rate and right ventricular pressure increased the total volume flow of the RCA. The diastolic over total flow ratio (D/T), however, enlarged on increasing right ventricular pressures while it declined on increasing heart rates. Our data confirmed, as well, that increased flow of RCA on rising heart rate was provided mainly by an increase in systolic phase, while the increase on augmented right ventricular pressure was provided by the increase in diastolic phase. The RCA manages to deliver blood to the right ventricular musculature in two different ways in response to increasing heart rate and right ventricular pressure.     

Duplex versus Unguided Pulsed Doppler Measurements of Right Ventricular Output in Newborn Infants

ABSTRACT. Noninvasive measurement of pulmonary artery blood flow has widespread implications for management of newborn infants requiring intensive care. Using a precordial, unguided, single Doppler technique, we evaluated right ventricular output in 26 preterm and 16 term infants without cardiopulmonary problems and compared it with right ventricular output obtained by duplex Doppler scanning and left ventricular output measured by a suprasternal single Doppler approach. Weights ranged from 1120 to 3960 g and postconceptional ages from 29 to 42 weeks. Unguided measurements of left and right ventricular output and guided and unguided measurements of right ventricular output were highly correlated (r=+0.92 and +0.95 respectively, p<0.001). The precordial single Doppler technique offers a reliable noninvasive estimate of right ventricular output in preterm and term newborn infants.     

Doppler Echocardiographic Evaluation of Right Ventricular Diastolic Function in Children

Right ventricular diastolic function was evaluated by flow velocity pattern in the right ventricular inflow tract by means of pulsed Doppler echocardiography. Traditionally used to evaluate this function are peak velocities obtained during early diastole (peak E wave) and during atrial contraction (peak A wave), their ratio (peak E/A ratio), and the deceleration half-time. We conducted pulsed Doppler echocardiographic studies of right ventricular inflow and outflow patterns in 171 children (105 normal children and 66 children who were undergoing total surgical repair of congenital heart defects without sequelae). Results showed that summation flow was present in the right ventricular inflow tract in 43 (25%) of the 171 subjects, which made it difficult to separate the peak E wave from the peak A wave. We noted the presence of antegrade late diastolic flow (DW) in the right ventricular outflow tract of all subjects. DW, measured in 121 subjects in whom both E and A waves were detected in the right ventricular inflow tract, showed a highly significant correlation (p < 0.0001) with A waves in the right ventricular inflow tract. The ratio of DW to right ventricular outflow tract velocities during systole (SW) showed a highly significant (p < 0.0001) correlation with E/A ratio. When evaluating right ventricular diastolic function by pulsed Doppler, especially in children, the analysis of right ventricular outflow tract patterns is helpful in addition to that of inflow tract patterns. The DW and DW/SW ratio may present good alternatives to traditional parameters in children.     

Tei指数和三尖瓣血流频谱评价左向右分流型先天性心脏病患儿右心功能的价值

目的探讨Tei指数和三尖瓣血流频谱评价左向右分流型先天性心脏病(CHD)患儿右心功能的价值。方法根据心室负荷将47例左向右分流型CHD患儿分为左心型组(30例)和右心型组(17例),应用三尖瓣血流频谱及Tei指数评价右心室功能,并与50例正常儿童进行对比研究。结果与正常组比较,CHD患儿42例三尖瓣血流频谱呈双峰型,均为舒张早期最大流速(EV)/舒张晚期最大流速(AV)>1;左心型组AV、A波流速积分(AVI)升高,右心型组EV、AV、E波流速积分(EVI)、AVI、E波减速时间(EDT)增大;左心型组右室等容舒张时间(IRT)和等容收缩时间(ICT) IRT延长;右心型组ICTI、RT和ICT IRT延长,右室射血时间(RVET)降低;两组CHD患儿右室Tei指数均增大;Tei指数与ICTI、RTI、CT IRT间呈显著性正相关(r=0.642,0.734,0.862 P均<0.01),与RVET间呈负相关(r=-0.481 P<0.01)。结论Tei指数能更简便、敏感、准确、综合评价右心室整体功能,与三尖瓣血流频谱结合可更全面了解CHD患儿右心功能。