Tetralogy of Fallot with anomalous origin of left pulmonary artery.

Between 1971 and 1990, 7 patients of tetralogy of Fallot with anomalous origin of left pulmonary artery underwent intracardiac repairs at Tohoku University Hospital. They were 2 males and 5 females with ages ranging from 4 to 26 years old. The right pulmonary artery connected to right ventricle in all cases, whereas no communications between right ventricle and the left pulmonary artery were found. The left pulmonary artery directly originated from the ascending aorta in 2 patients (group I) and connected to the ductus arteriosus in 5 patients (group II and III). In 2 patients (group II), the left pulmonary artery was separated from the pulmonary arterial trunk by the intraluminal membrane, receiving blood supply through the ductus. In the remaining 3 patients (group III), there were no continuations between both pulmonary arteries. At the correction, communication between the left pulmonary artery and the pulmonary arterial trunk could be reconstructed in groups I and II. However, it was not possible in group III, because the ductus arteriosus and the left pulmonary artery had already been occluded before the intracardiac repair. In group III, ventricular septal defect was closed using a one-way valved patch or a perforated patch to decrease supersystemic right ventricular pressure. Postoperative right ventricular aortic pressure ratio was between 0.5 and 0.8 in groups I and II, and between 0.8 and 1.0 in group III. Three patients (one in each group) died after the operation. Severe pulmonary vascular obstructive disease was found in the left lung of group I.(ABSTRACT TRUNCATED AT 250 WORDS)     

法洛四联症的胎儿期超声心动图特征

目的探讨法洛四联症胎儿期的超声心动图特征,提高产前诊断率。方法对所有受检胎儿均行四腔心切面及四腔心平面头侧偏转法获得左、右心室流出道、主肺动脉长轴切面、心底短轴切面及三血管平面扫查。对产前诊断的15例法洛四联症的胎儿期超声心动图特征进行总结分析。结果产前共诊断15例法洛四联症,漏诊2例,在产前诊断的所有法洛四联症病例均具有肺动脉内径较主动脉内径窄、大于0.40cm的室间隔缺损、主动脉骑跨,但仅5例有右室前壁增厚。漏诊的2例法洛四联症为轻型,肺动脉仅轻度狭窄,室间隔缺损直径小于0.40cm。结论法洛四联症的胎儿期超声心动图特征主要表现为肺动脉狭窄、室间隔缺损、主动脉骑跨,右室前壁增厚不明显,轻型法洛四联症可能漏诊。检查时须四腔心切面及四腔心平面头侧偏转法获得左、右心室流出道、主肺动脉长轴切面、心底短轴切面及三血管平面扫查,才不致漏诊。     

成年人法洛四联症的外科治疗

目的:探讨成年人法洛四联症的外科治疗。方法:回顾性分析1995年5月至2002年12月施行的63例成人法洛四联症,均采用右室纵切口,彻底疏通右室流出道,涤纶补片修补室缺,人造血管单纯加宽右室流出道14例,余均采用带单瓣的跨瓣环补片,合并畸形同期手术。结果:住院死亡2例,死亡率3.17%;1例死于顽固的低心输出量综合征,1例死于气管切开后的反复出血。余61例痊愈出院,随访6个月至7年,恢复良好。结论:成人法洛四联症长期缺氧,心肌肥厚纤维化,术前心功能多已受损,术后易至严重的低心排综合征;术中疏通右室流出道剪除心肌不宜过多。成人法洛四联症均为大室缺,缺损边缘心肌较脆,为防止缝合时切割心肌,采用间断褥式缝合外圈加毡条加固。成人法洛四联症应及早手术矫正,近远期效果好。     

法洛四联症合并右肺动脉起源于升主动脉1例

患儿女，8岁．出生后即发现心脏杂音，9个月龄时曾至外院检查，诊断为法洛四联症，未予处理。近日有咳嗽、咳痰，为求进一步诊治．来我院检查。查体：一般情况可．中央性紫绀，口周明显。心率116次／分．心律齐。胸骨左缘3～4肋间可闻及Ⅲ级收缩期杂音．P2无减弱；未及震颤。X线平片示：心影增大．右侧肺血多，     

Entrainment of Ventricular Tachycardia in Postoperative Tetralogy of Fallot

The mechanism of ventricular tachycardia (VT) in postoperative tetralogy of Fallot has been ascribed to both reentry and triggered automaticity. We performed electrophysiologic studies on a patient with this condition and induced sustained uniform ventricular tachycardia by programmed extrastimulation. Pacing during the tachycardia at multiple cycle lengths from the right ventricular apex (RVA) and outflow tract (RVOT) produced constant but progressive fusion between the paced and tachycardia QRS. With termination of pacing, the last captured complex was unfused but coupled at the paced cycle length and then the tachycardia resumed at its intrinsic rate. Therefore, the VT was entrained. In addition, an area of slow conduction between the RVOT and RVA was demonstrated. These findings support a reentrant mechanism of this arrhythmia.     

Clinical implications of haemoptysis in patients with pulmonary arterial hypertension

Introduction: Pulmonary arterial hypertension (PAH) is a disabling disease that may result in haemoptysis. Patients with congenital heart disease associated PAH (CHD-APAH) may have a survival advantage when compared with patients with other types of PAH presenting with haemoptysis. The effects of aetiology and subsequent management choice of haemoptysis in PAH patients is not well-defined. Methods: We conducted outcome analysis in CHD-APAH vs. all other subtypes of PAH patients presenting with haemoptysis to The Methodist Hospital. Twenty-one patients were identified, 13 patients in the CHD-APAH group and eight patients in the non-CHD group. We evaluated outcomes related to treatment (bronchial artery embolisation (BAE) vs. conservative management), hospital length of stay, mortality rates and survival in this cohort. Results: The CHD-APAH and non-CHD groups had similar baseline demographic, haemodynamic and laboratory values except BMI was higher in the non-CHD group and haematocrit was higher in the CHD-APAH group. Twenty-eight-day mortality (0% vs. 31%) and 1-year mortality (0% vs. 54%) was lower in the CHD-APAH patients as compared with non-CHD group. A statistically significant difference was found in the survival rate in favour of CHD-APAH group for the total follow-up period (p?=?0.02). Although not statistically significant, patients treated with BAE had shorter length of stay (4.0?days?±?4.0 vs. 13.7?days?±?22.5; p?=?0.26). There was recurrent haemoptysis in 43% of patients treated with BAE. Conclusion: Haemoptysis in PAH patients is a serious event with a high mortality rate. CHD-APAH seems to confer a survival advantage, independent of therapy utilised. Termination of haemoptysis with BAE is rapid with relatively few complications except for frequent re-bleeding episodes. Further studies are needed to determine the risk factors that may predispose PAH patients to excessive mortality from haemoptysis and to identify an optimal therapeutic modality.     

重症法洛四联症患儿围手术期的护理

目的 探讨重症法洛四联症患儿围手术期的护理措施，以期提高手术成功率。方法 选择1999年3月～2002年12月我科收治重症法洛四联症患儿90例，在体外循环深低温停循环或深低温低流量下行法洛四联症根治术或姑息术，手术前后实施预防缺氧发作、低心排护理、呼吸道及伤口护理等围手术期护理措施。结果 90例手术患儿中手术成功84例，死亡6例。术后发生低心排32例，胸腔引流偏多5例，经治疗后缓解。随访3个月～3年，患儿均恢复良好。结论 重症法洛四联症患儿术前预防缺氧发作，术后做好各项护理措施，可降低手术后并发症发生，提高手术成功率。     

法乐四联症根治术后残余肺动脉瓣狭窄的介入治疗及疗效评价

目的对法乐四联症根治术后残余肺动脉瓣狭窄进行经皮球囊成形术并评价其疗效。方法对16例患者行经皮肺动脉瓣球囊成形术,年龄1~23岁,平均(7.8±6.3)岁,术前超声心动图测量肺动脉瓣跨瓣峰值收缩压差为(81.83±23.17)mmHg(55~130mmHg)。术后24小时、6个月及1年行超声心动图、X线平片及心电图进行随访。结果 14例使用聚乙烯球囊导管进行扩张,2例使用Inoue球囊导管,球囊平均直径(19.58±4.03)mm;扩张后肺动脉-右心室收缩压差由(80.50±27.49)mmHg降至(46.25±29.19)mm(P0.01),4例(25.0%)肺动脉-右心室收缩压差仍大于50mmHg。随访中肺动脉瓣跨瓣峰值压差与术后早期差异无统计学意义。结论法乐四联症术后75.0%残余肺动脉瓣狭窄的患者可以通过介入治疗有效降低肺动脉-右心室收缩压差,从而避免二次手术。     

多层螺旋CT血管造影在法洛四联征诊断中的应用

目的 分析法洛四联征的多层螺旋CT血管造影(MSCTA)结果,探讨MSCTA在法洛四联征诊断中的应用.方法 分析6例法洛四联征患者的MSCTA图像并与超声或手术结果对照.6例患者均行MSCTA检查,结合薄层图像、多平面重组、薄层最大密度投影以及容积再现法进行观察.结果 MSCTA显示肺动脉主干狭窄2例,左肺动脉狭窄3例,右肺动脉狭窄1例.6例均见室间隔缺损及主动脉骑跨, 还可清楚地显示左心房增大、左心室增大、右心室增大等对室缺诊断有帮助的间接征象.2例可很好显示右室壁增厚.结论 MSCTA可清楚地显示法洛四联征各种畸形的主要表现,包括肺动脉狭窄,室间隔缺损,主动脉骑跨,右心室肥厚等.     

Chronic pulmonary valve insufficiency after repaired Tetralogy of Fallot: diagnostics,reoperations and reconstruction possibilities

Complete correction of Tetralogy of Fallot, the most common cyanotic congenital heart defect, has now become routine. However, late residual lesions, primarily chronic pulmonary valve insufficiency, may have a negative impact on right-ventricular function, leading to the need for reoperation to insert a competent valve at the right-ventricular outflow. The diagnostic modalities pertaining to the failing right ventricle, the timing for eventual reintervention and the various surgical reconstruction possibilities of the right-ventricular outflow tract are still controversial and evolving, and are reviewed with a brief overview on current trends and future outlooks.     

法乐氏四联症动态三维超声心动图研究

为了显示法乐氏四联症心脏的动态立体结构图像，本文采用经胸壁及经食管检查法对１０例正常人及１８例法乐氏四联症患者进行动态三维超声心动图研究，建立了不同切面心脏的立体剖面图，可分别从不同方向观察心脏病变部位的形态结构及活动状况。结果显示：除常规的二维断面观察外，还可从左或右室侧详细观察室间隔缺损及从主动脉瓣口观察主动脉骑跨空间隔的动态情况．心动周期不同时相右室流出道狭窄程度、室间隔缺损大小变化也可准确显示。动态二维图像清晰、直观、立体感强，可以确定病变的空间位置、形态及严重程度。为心外科制定手术方案及即刻评价疗效提供形态学依据。