Long-Term Outcome of Surgically Repaired Unilateral Anomalous Pulmonary Artery Origin

The long-term outcome after surgical reimplantation of a unilateral congenitally anomalous pulmonary artery (UCAPA) origin is inadequately described. We performed a single-center retrospective review of patients with pulmonary artery (PA) sling or anomalous origin of 1 PA from the ascending aorta who underwent surgical repair from 1980 to 2006. Baseline data were available for 20 patients with PA sling and 29 patients with anomalous origin of 1 PA from the ascending aorta. Follow-up data for 38 patients, representing 190 cumulative patient-years, were available. Of 28 (74%) patients who developed pulmonary artery stenosis (PAS), 17 (45%) patients underwent a first intervention for PAS, and 9 (24%) patients underwent a second intervention. Median time from surgical repair to diagnosis of PAS was 0.4 years (95% confidence interval [CI] 0, 0.9). Median time from diagnosis of PAS to first intervention was 2.7 years (95% CI 0.07, 5.4). In the 29 patients initially repaired at our center, no first intervention occurred later than 2.8 postoperative years. Median time from first to second intervention was 2 years (95% CI 0, 4.8). Percutaneous intervention for postoperative PAS resulted in acute hemodynamic and anatomic improvement with modest risk profile. On multivariate analysis, recent surgical repair (after 1993) was a risk factor for PAS (p = 0.03, hazard ratio [HR] 1.9), and operative first intervention (vs. percutaneous) was a risk factor for second intervention (p = 0.05, HR 11.2). After reimplantation of a UCAPA origin, PAS is frequent and occurs early postoperatively. Close follow-up after repair is necessary.     

Spectrum of cardiovascular anomalies in Williams-Beuren syndrome

Summary This study is presented to identify and characterize the spectrum of the cardiovascular anomalies in children presenting with Williams-Beuren syndrome and cardiovascular anomalies at The Hospital for Sick Children, Toronto from 1966 to 1988. Forty-nine children were diagnosed and followed. The female to male ratio was 1.21. The age ranged from 1 month to 14 years at the time of diagnosis (mean 39 months), and follow-up periods were from 9 months to 20 years (mean 10 years). All patients having the typical features were also evaluated by geneticsts. Based on cardiovascular findings four groups were identified. Group 1 had isolated supravalvular aortic stenosis (SVAS) (28 patients). There was follow-up in 24 of these children. Six had worsening of supravalvular narrowing and underwent surgery. One showed an increased gradient from 10–40 mmHg during 7 years. Seventeen had mild narrowing and showed no progression over a period of 75 months. Group 2 had isolated pulmonary artery branch stenosis (8 patients). Seven had mild narrowing which remained unchanged over a mean period of 16 months and one underwent surgery. Group 3 had combined lesions (11 patients). Six showed increased left-side narrowing, while right-side obstruction remained static or improved. Five showed improvement in narrowing in both outflow tracts. Five underwent surgery. Additional cardiovascular anomalies included peripheral artery stenosis in two patients, coronary artery abnormalities in three, mitral valve prolapse in three, and coarctation of the aorta in two. Group 4 had isolated lesions. One patient had isolated coarctation of the aorta and one isolated mitral prolapse. In conclusion, supravalvular aortic stenosis was the most common lesion, whereas pulmonary artery stenosis improved in most patients. The role of coronary artery abnormalities has yet to be defined.     

The role of surgical therapy for pulmonary vein atresia in childhood

The outcome for children with pulmonary vein atresia has been historically poor. This report describes clinical outcomes after surgical treatment of primary and secondary pulmonary vein atresia. All patients undergoing surgery for pulmonary vein atresia at Children??s Hospital Boston from 1 January 2005 to 1 January 2009 were identified. The data available for analysis included demographic characteristics, findings based on cardiac catheterization and other imaging, clinical information, operative surgical intervention, and pulmonary vein histology. The study-specific information included the calculated number of atretic pulmonary veins as well as surgical interventions performed during the study period. The study end points included successful relief of pulmonary vein atresia, recurrence of pulmonary atresia or stenosis, overall pulmonary vein disease progression, and patient survival. A total of 16 patients with one or more pulmonary vein atresias were identified in the cardiac surgery registry database. Of these 16 patients, 5 had primary pulmonary atresia, and 11 had secondary atresia after repair of anomalous pulmonary venous drainage. Surgical recanalization of atretic pulmonary veins was achieved for more than 80% of the patients. At the most recent follow-up assessment, 7 (44%) of the 16 patients had recurrence of atresia. Five of the patients had development of new atresia in other previously healthy pulmonary veins. The median follow-up period was 4?months for primary and 17?months for secondary pulmonary vein atresia. The three main predictors of pulmonary vein disease progression after surgery were the presence of underlying lung disease (P?=?0.036), prematurity (P?=?0.035), and a history of recurrent pulmonary vein atresia. The overall mortality rate for the patients with primary or secondary pulmonary vein atresia was 56% at the most recent follow-up assessment (100% for the patients with primary vein atresia and 36% for the patients with secondary vein atresia). Conclusions: Surgical recanalization of pulmonary vein atresia is possible, but prognosis remains poor. Recurrence of atresia can take place as early as a few months in the presence of underlying lung disease, prematurity, or history of recurrent pulmonary vein atresia. Further research is needed to identify mechanisms for attaining and sustaining pulmonary vein patency after surgery.     

The Application of All-Autologous Three-Sinus Repair for Supravalvular Pulmonary Stenosis

Various surgical techniques have been proposed for the repair of supravalvular pulmonary stenosis (SVPS) in pediatric populations. Whereas growth potential should be promised, excessive expansion under the presence of undiminished high pulmonary arterial pressure should be avoided. The authors applied all-autologous three-sinus repair, the so-called modified Brom or Myers technique, to SVPS and examined the midterm outcomes. Between March 2010 and March 2012, 15 patients (8 boys) with a median age of 12 months who had SVPS underwent all-autologous three-sinus repair. Of the 15 patients, 13 (87 %) had previously undergone pulmonary artery (PA) banding for treatment of high pulmonary vascular resistance. Two patients (13 %) had Noonan syndrome. A follow-up evaluation was completed for all the patients, and the median follow-up period was 13.5 months (range, 1 month to 2.4 years). No mortalities occurred. The diameter of the stenotic part at the main PA increased from 47.0 ± 14.1 % (range 29.1–70.0 %) of the normal PA diameter at the preoperative evaluation to 108.9 ± 25.7 % (range 58.9–148.1 %) at 6 months, and then to 104.7 ± 11.4 % (range 87.7–134.1 %) 1 year after the operation. The estimated mean pressure gradient across the main PA decreased from 76.2 ± 12.2 mmHg (range 57.8–108.2 mmHg) at the preoperative evaluation to 11.3 ± 12.0 mmHg (range 1.4–49.0 mmHg) at 6 months, and then to 6.4 ± 5.5 mmHg (range 2.0–19.4 mmHg) 1 year after the operation. No patients showed moderate or greater pulmonary insufficiency. The midterm outcomes after supravalvular pulmonary stenosis by all-autologous three-sinus repair were acceptable. Although a long-term follow-up evaluation is mandatory, application of this technique may provide a just enough growth of the reconstructed main pulmonary artery with symmetric pulmonary valve geometry.     

Decreased Incidence of Right-Ventricular Outflow Tract Complications Using a Retrograde Snare Technique for Radiofrequency Pulmonary Valve Perforation

Perforation of the atretic pulmonary valve with balloon dilation in infants with pulmonary atresia with intact ventricular septum (PA-IVS) is standard initial therapy for right-ventricular (RV) decompression. This procedure often results in adequate pulmonary blood flow, thus eliminating the need for neonatal surgery. Nonetheless, the incidence of RV outflow-tract complications and mortality for this intervention is significant. We report our experience with retrograde snare-guided radiofrequency (RF) perforation in an attempt to improve accuracy and decrease procedural complications. Medical records were reviewed for the period between March 2007 and May 2010 for all patients with PA-IVS who presented to the catheterization laboratory for attempted RF perforation in infancy. Specific details reviewed included demographics, preprocedural echocardiographic (echo) data, procedural technique and complications, pre- and post-RV pressures and pulmonary valve gradients, need for surgical intervention in the neonatal period, and short- to medium-term follow-up. Eleven neonates with PA-IVS underwent RF perforation using a retrograde snare-guided technique during the study period. The pulmonary valve was successfully perforated and the wire snared in all 11 patients. Six of 11 atretic valves were crossed on the first attempt with low energy (5?W?×?2?s). No episodes of tamponade or RV/PA perforation occurred as confirmed by echocardiogram performed immediately after the procedure. There was no ductal spasm with retrograde catheter manipulations. Sequential dilation of the perforated valve was not necessary. RV pressures decreased from 169?% systemic before dilation to 93?% after dilation (p?<?0.001) with a residual pulmonary valve gradient of 16?±?6?mm Hg. Eight of 11 patients (73?%) were discharged without surgery at an average 16?days after the intervention. Three patients required Blalock?CTaussig shunts to augment pulmonary blood flow. Femoral artery thrombus occurred in 4 of 11 patients treated with anticoagulation, and 2 patients had atrial arrhythmias during the procedure. No other complications occurred. No preprocedural echo parameters predicted requirement for surgical shunt before hospital discharge. Retrograde snare-guided pulmonary valve perforation in infants with PA-IVS is safe and may decrease the incidence of significant procedural complications.     

Intermediate-term follow-up results of pulmonary balloon valvuloplasty in children

Twenty-two patients (10 boys, 12 girls) with pulmonary valve stenosis whose mean age was 6.7 +/- 4.1 years (range 1 to 14 years) at time of the procedure underwent balloon dilatation angioplasty. All patients had isolated pulmonary valve stenosis with no associated cardiac anomalies, and their pulmonary valvular gradients were greater than 50 mmHg. Diagnostic catheterization and balloon valvuloplasty were performed during the same procedure. The mean peak systolic pressure gradient before balloon dilation of 95.8 +/- 29.5 mmHg (54-163 mmHg) was reduced to 30.2 +/- 15.8 mmHg (7-64 mmHg) after balloon dilation (p < 0.001). A linear relation was found between the predilation pressure gradient and the pressure gradient drop (r = 0,86; SE: 28.94) (y = 0.89x - 12.11). Following a successful valvuloplasty, the mean peak systolic pressure in the right ventricle decreased from 119.0 +/- 30.4 mmHg (71-184 mmHg) to 55.2 +/- 16.9 mmHg (29-97 mmHg) (p < 0.001). Continuous wave Doppler was used for follow-up. Pressure gradients were estimated in 14 patients between one and 29 months after the dilation 120 +/- 7.1 months). The mean follow-up gradient was 19.0 +/- 6.0 mmHg (11-32 mmHg). No important complication was noted in the immediate course or throughout the follow-up period, but four patients (28.6%) had minimal pulmonary insufficiency. In conclusion, these data confirm that balloon dilation in valvular pulmonary stenosis is safe and effective, and suggest that stenosis does not recur.     

婴儿先天性心脏病1387例外科治疗结果分析

目的 回顾性分析我院近11年来外科治疗的≤6个月小婴儿先天性心脏病(先心病)1387例,探讨其病种、手术时机和治疗观念的变迁,以期进一步提高小婴儿先心病的就诊和救治率.方法 1997年1月至2007年12月,在我院行手术治疗的≤6个月先心病患儿1387例,主要病种包括:室间隔缺损合并肺动脉高压(VSD/PH)、法洛四联症(TOF)、完全性大动脉转位(TGA)、完全性肺静脉异位引流(TAPVC)、主动脉缩窄或主动脉弓中断合并室间隔缺损[CoA(IAA)/VSD]、右心室双出口(DORV)、合并室间隔缺损的肺动脉闭锁(PA/VSD)、室间隔完整的肺动脉闭锁(PA/IVS)等,根据病情采取相应的手术方法矫治,部分复杂型先心病进行了随访.结果 手术死亡110例,总手术死亡率7.9%.从历年手术治疗分析,手术死亡率1997至2003年为11.5%～14.4%,2004至2005年降至8.6%～&9%,2006至2007年降至3.3%～3.8%.对TGA、TAPVC、TOF、PA/VSD、PA/IVS患儿进行了随访,随访率分别为83.8%(98/117)、87.8%(79/90)、48.2%(68/141)、65%(13/20)和95%(19/20),随访期限为3～86个月.晚期死亡16例.随访中绝大多数患儿无症状,心功能和生长发育正常.结论 绝大部分早期出现症状的危重先心病可以在小婴儿期进行矫治,手术效果接近国际水平.不能进行一期矫治的可以先做姑息手术,改善缺氧、促进肺动脉发育,为以后的根治手术创造条件.     

Pulmonary functions in children with progressive systemic sclerosis

The patterns of pulmonary involvement in 13 children with progressive systemic sclerosis were investigated. Eight patients (61%) had respiratory symptoms or signs and 7 patients (55%) had abnormalities on chest roentgenogram. Twelve patients (92%) had abnormal pulmonary function tests: 7 had restrictive disease, 2 had obstructive disease, 2 had small airway disease, and 1 had an isolated reduction in the diffusing capacity of carbon monoxide. Nine patients had the test performed during the first year of illness, 3 during the second year, and 1 at 5 years. All patients had abnormal pulmonary function tests when first studied. Subsequent pulmonary function tests over a period of 3 to 10 years (mean 6.2 years) showed substantial changes in only 2 patients (1 patient had initial worsening of diffusing capacity of carbon monoxide followed by normalization and another patient showed improvement of obstructive disease). Two patients died during follow-up, 1 of pulmonary hypertension, the other of severe restrictive lung disease and myocardial fibrosis. The major findings of this study were (1) high frequency of pulmonary disease in children with progressive systemic sclerosis, (2) early involvement of the lungs, (3) relatively indolent progression of lung disease, and (4) the prognostic importance of the severity of pulmonary disease. Pulmonary manifestations of progressive systemic sclerosis in children appear to be similar to those of affected adults.     

Initial Experience With Tadalafil in Pediatric Pulmonary Arterial Hypertension

This study aimed to investigate the safety, tolerability, and effects of tadalafil on children with pulmonary arterial hypertension (PAH) after transition from sildenafil or after tadalafil received as initial therapy. A total of 33 pediatric patients with PAH were retrospectively evaluated. Of the 33 patients, 29 were switched from sildenafil to tadalafil. The main reason for the change from sildenafil was once-daily dosing. The average dose of sildenafil was 3.4 ± 1.1 mg/kg/day, and that of tadalafil was 1.0 ± 0.4 mg/kg/day. For 14 of the 29 patients undergoing repeat catheterization, statistically significant improvements were observed after transition from sildenafil to tadalafil in terms of mean pulmonary arterial pressure (53.2 ± 18.3 vs. 47.4 ± 13.7 mmHg; p < 0.05) and pulmonary vascular resistance index (12.2 ± 7.0 vs 10.6 ± 7.2 Units/m(2); p < 0.05). Clinical improvement was noted for four patients treated with tadalafil as initial therapy. The side effect profiles were similar for the patients who had transitioned from sildenafil to tadalafil including headache, nausea, myalgia, nasal congestion, flushing, and allergic reaction. Two patients discontinued tadalafil due to migraine or allergic reaction. One patient receiving sildenafil had no breakthrough syncope after transition to tadalafil. Tadalafil can be safely used for pediatric patients with PAH and may prevent disease progression.     

Weaning of epoprostenol in a small infant receiving concomitant bosentan for severe pulmonary arterial hypertension secondary to bronchopulmonary dysplasia

Endothelin receptor antagonism is an important therapeutic tool of pulmonary arterial hypertension (PAH). Bosentan was the first orally active, dual antagonist of endothelin receptors in human adults, and has been recently considered for children as well. However, little is known about bosentan treatment in children weighing less than 10 kg. We describe the use of bosentan concomitantly to epoprostenol in an infant weighing 3.5 kg and affected with severe bronchopulmonary dysplasia (BPD) and PAH. At 5 months old, when she presented subsystemic PAH secondary to severe BPD, she was treated with oxygen, digoxin and diuretics. At 8 months old, due to severe PAH not responsive to 100% oxygen, high frequency oscillatory ventilation (HFOV) and nitric oxide (NO), we started epoprostenol and bosentan. Bosentan dose was doubled at 9 months old, when HFOV and NO were slowly discontinued due to improved oxygenation index. Regular echocardiographic measurements of systolic right ventricular pressure were recorded by the method of tricuspidal atrio-ventricular gradient. A four-month combined epoprostenol and bosentan treatment decreased systolic right ventricular pressure from 68% to 40% of the systemic level, till its normalization at 11 months old. Later, when bosentan and epoprostenol were discontinued and sildenafil was started, severe PAH was reported again. Our patient died due to septic shock and refractory hypoxia at 14 months old.     

Contribution of pulmonary hemodynamics on manifestation of allergic asthma in patients with congenital heart disease

The purpose of this study was to determine whether pulmonary hemodynamic abnormalities relate to manifestations of allergic asthma. In 448 patients with congenital heart disease the relationships between asthma and age or pulmonary arterial blood (PA) flow were studied. Asthma (allergic and non-allergic) was more common in 39 (19%) of 201 patients with high PA flow, compared with the incidence in those with normal PA flow (6/117, 5%; P < 0.001) and reduced PA flow (1/130, 1%; P < 0.05). In the high PA flow group, the frequency of asthma declined significantly (P < 0.01) with age, from 25–26% in the 6 month-5 year patient group to 5% in the 6–12 year old patients. The frequency of asthma, including allergic type, was significantly (P < 0.01) greater in patients with pulmonary hypertension (15/24, 63%) than in those without (10/77, 13%) at the age of 6 months to 1 year. Asthma in the high PA flow group was associated with other allergic diseases in 30 (77%) of 39 patients, including food allergy in nine (23%), atopic dermatitis in 14 (36%), allergic rhinitis in seven (18%) and abnormally high total IgE levels in 14 (36%). These findings suggest that high pulmonary flow or pulmonary hypertension enhances the manifestation of allergic disease, particularly asthma.     

Bronchial compression in an infant with isolated secundum atrial septal defect associated with severe pulmonary arterial hypertension

Symptomatic pulmonary arterial hypertension (PAH) in patients with isolated atrial septal defect (ASD) is rare during infancy. We report a case of isolated ASD with severe PAH in an infant who developed airway obstruction as cardiomegaly progressed. The patient presented with recurrent severe respiratory insufficiency and failure to thrive before the repair of the ASD. Echocardiography confirmed volume overload on the right side of heart and severe PAH (tricuspid regurgitation [TR] with a peak pressure gradient of 55 to 60 mmHg). The chest radiographs demonstrated severe collapse of both lung fields, and a computed tomography scan showed narrowing of the main bronchus because of an intrinsic cause, as well as a dilated pulmonary artery compressing the main bronchus on the left and the intermediate bronchus on the right. ASD patch closure was performed when the infant was 8 months old. After the repair of the ASD, echocardiography showed improvement of PAH (TR with a peak pressure gradient of 22 to 26 mmHg), and the patient has not developed recurrent respiratory infections while showing successful catch-up growth. In infants with symptomatic isolated ASD, especially in those with respiratory insufficiency associated with severe PAH, extrinsic airway compression should be considered. Correcting any congenital heart diseases in these patients may improve their symptoms.     

A postoperative follow-up study of infantile coarctation of the aorta

Follow-up investigations were performed in 16 patients operated on for coarctation during infancy The follow-up period ranged from 4.5 to 11 years (median 5.5 years) Four different surgical techniques were used: resection with end-to-end anastomosis (REE) (4 patients), subclavian flap aortoplasty (SFA) (10 patients), patch aortoplasty (1 patient) and resection and SFA (1 patient) One patient developed reforestation (6%) She had been operated on by REE at 7 days of age The other three patients operated on by REE had equal pulses in the arms and legs; none had hypertension and all had normal arm/leg pressure gradients at rest Seven (58%) of the 12 patients operated on by SFA or aortoplasty had weak radial pulses in the left arm but no limitation of left arm function The left arm showed a normal bone age but was smaller and shorter than the right arm in 9 (81%) of the patients None of the patients operated on by SFA had hypertension and the arm/leg gradient at rest was normal.     

单中心儿童肺高血压病因组成和住院转归分析

目的　观察分析单中心儿童肺高血压（PH）病因组成和不同类型PH患儿住院转归及影响因素。方法　选取2016年1月至2018年12月在湖南省儿童医院确诊并接受治疗的PH患儿共1157例，根据患儿病历资料及出院诊断，对其PH病因按照2015年发布的《儿童肺动脉高压诊断与治疗专家共识》进行分类。收集并记录其住院期间相关临床资料。结果　本研究中肺动脉高压（PAH）患儿共1099例（94.9%），以先天性心脏病相关性PAH（APAH-CHD）、新生儿持续肺动脉高压（PPHN）为主，分别占84.7%、8.0%。特发性PAH死亡3例，放弃治疗6例，手术1例，余5例好转出院。APAH-CHD中以手术后出院676例（68.9%）。要求出院45例（4.6%），其中放弃治疗出院及死亡80例（8.2%）；服用波生坦共52例，有2例联用西地那非。PPHN患儿因其他器官手术后出院6例（6.5%），好转出院66例（70.9%），放弃出院及死亡14例（15.1%）。左心疾病所致PH好转18例，行左心疾病矫治手术11例（40.7%），放弃及死亡病例7例（25.9%）。呼吸系统疾病PH 27例，1例死亡，3例放弃治疗。未知因素所致PH 2例放弃出院，死亡1例，1例行姑息手术出院。结论　儿童PH分类以APAH-CHD及PPHN为主，特发性PAH、左心疾病相关PH的患儿预后差。     

儿童先天性心脏病血清Apelin水平与肺动脉压关系的初步探讨

目的 初步探讨先天性心脏病患儿血清Apelin 水平与肺动脉压的关系.方法 手术治疗的先心病患儿126 例,检测患儿术前及术后第7 天的血清Apelin 水平.建立体外循环前检测并计算肺动脉收缩压/体循环收缩压(Pp/Ps)的比值,依据Pp/Ps 分组:无肺动脉高组压(PAH)组、轻度PAH 组、中度PAH 组和重度PAH 组;术后第7 天超声心动图估测肺动脉平均压(PAMP).结果 无PAH,以及轻、中、重度PAH 各组术前及术后的血清Apelin 水平依次降低,差异有统计学意义(PPr=-0.51,-0.54,P结论 先心病患儿并发肺动脉高压及其发展与血清Apelin 水平降低有关系,血清Apelin 对诊断先心病患儿是否并发肺动脉高压及其程度的意义值得深入研究.     

新生儿危重肺动脉瓣狭窄及闭锁的经导管介入治疗

目的 探讨新生儿期采用经导管介入方法 治疗危重肺动脉瓣狭窄(critical pulmonarystenosis,CPS)及室间隔完整型肺动脉瓣闭锁(pulmonary atresia with intact ventricular septum.PA/IVS)的可行性.方法 2006年6月至2008年1月,采用经导管介入治疗13例新生儿(其中1例早产儿)危重先天性心脏病(CPS 9例,PA/IVS4例).男10例,女3例,年龄2～30(17.4±10.7)d,体重2.3～4.8(3.4±0.8)kg.术前及术中均使用前列腺素E.开放动脉导管.CPS患儿先采用直径2.5～4.0mm小球囊预扩张肺动脉瓣,再用1.0～1.2倍于肺动脉瓣环的球囊扩张肺动脉瓣.PA/IVS患儿先行射频瓣膜打孔,然后再行球囊瓣膜扩张术.结果 12例(92%)介入治疗成功.右室压力下降50%[术前(120.1±17.0)mm Hg(1 mm Hg=0.133 kPa),术后(58.8±7.7)mln Hg,P＜0.001].右室压力/体动脉压力比值下降44%[术前(1.6±0.2),术后(0.9±0.1),P＜0.001].右室造影显示肺动脉瓣开放.球囊/瓣环径比值1.1±0.1(最大球囊直径6～12 mm).手术时间(127.5±32.6)min,X线-曝光时间(25.2±7.2)min.术中4例(30%)出现并发症,2例心包积血,1例低氧血症,1例室上性心动过速.死亡1例(8%).全组随访1～18(7.2±6.4)个月.术后残余中至重度瓣膜狭窄3例,1例再次行球囊扩张术,另2例等待再次球囊扩张术;残余轻至中度瓣膜狭窄9例,所有患儿仅伴随轻度瓣膜反流,动脉导管自然闭合11例,近闭合1例.结论 经导管介入治疗新生儿CPS及PA/IVS是安全、有效的,部分患儿需二期介入治疗.     

经导管射频打孔并球囊扩张治疗婴儿室间隔完整的肺动脉瓣闭锁

目的探讨经导管射频瓣膜打孔并球囊扩张治疗婴儿室间隔完整的肺动脉瓣闭锁（PA／IVS）有效性和安全性。方法4例患儿年龄分别为11、9、9个月和12d,体重4—10kg[（7．6±2．2）kg]。均具有紫绀和动脉导管未闭连续性杂音的临床表现。超声心动图诊断后,在全麻下作右心室造影确定右心室腔由3部分结构组成,漏斗部呈长管形,无冠状动脉异常。适合经导管射频打孔并球囊扩张术治疗。射频瓣膜打孔能量5—8W,持续时间2—5s,一般2次;预扩球囊3mm×20mm-5mm×20mm,6—14atm;肺动脉瓣扩张球囊10mm×30mm-14mm×30mm,有效扩张1-2次。手术持续时间120—150min,X线暴露时间25．4—43．9min。结果技术成功率100％,除1例新生儿发生心脏穿孔早期死亡外,其他3例无任何并发症。存活3例随访4-12个月,1例需外科解除右室流出道梗阻,2例可择期封堵动脉导管未闭。结论经导管射频瓣膜打孔并球囊扩张术治疗PA／IVS需严格掌握适应证,规范操作,可作为替代或延迟外科开胸手术治疗的一种方法。     

超声心动图对室间隔完整型肺动脉瓣闭锁和极重度肺动脉瓣狭窄胎儿的分型、评估及其出生后随访

背景 室问隔完整型肺动脉瓣闭锁(PA/IVS)和极重度肺动脉瓣狭窄(CPS)是一类较为少见的先天性心脏病,对PA/IVS和CPS胎儿进行详细的超声心动图评估并预测出生后的心脏情况,对于生育咨询非常重要.目的 通过超声心动图对PA/IVS和CPS胎儿、新生儿、手术后病例进行分型与评估.设计病例系列报告.方法 对超声心动图...     

Late Pulmonary Valve Replacement in Congenital Heart Disease Patients Without Original Congenital Pulmonary Valve Pathology

Many congenital heart defects with pulmonary valve pathology are repaired or palliated in childhood. It is anticipated that these patients will need subsequent pulmonary valve replacement due to the pulmonary regurgitation or right ventricular dilation/failure that results from their original operation. Interestingly, some patients with prior congenital heart surgery and no congenital pulmonary valve pathology require pulmonary valve replacement in adulthood. The current study analyzed this subset of patients. The pediatric and adult congenital cardiac surgical databases at a large academic center were reviewed from 2001 to 2008 for pulmonary valve replacements. Patients without congenital pulmonary valve disease were identified. Preoperative, operative, and postoperative data were analyzed. Between 2001 and 2008, five patients with congenital heart disease but no pulmonary valve pathology underwent late pulmonary valve replacement. The initial congenital diagnoses were coarctation/ventricular septal defect (n = 3), complete atrioventricular septal defect (n = 1), and anomalous left coronary artery from the pulmonary artery (PA) (n = 1). All five patients had undergone main PA manipulation during their previous operations. Four of these patients had received PA banding and debanding, and one had received a Takeuchi tunnel repair and revision. All the patients underwent pulmonary valve replacement secondary to pulmonary regurgitation and right ventricular dilation. The average time from the last previous operation to the time of pulmonary valve replacement was 20.8 ± 9.1 years. Bioprosthetic valves were used for all the pulmonary valve replacements, and there were no mortalities. Patients who have undergone previous PA manipulation, regardless whether congenital pulmonary pathology is present, may be at risk for pulmonary valve replacement in adulthood. This report describes five cases of patients with a history of congenital heart surgery but no congenital pulmonary valve pathology who required pulmonary valve replacement due to the consequences of prolonged pulmonary regurgitation. Although pulmonary regurgitation may be well tolerated for many years, it is further evidence for the importance of close follow-up assessment and monitoring of young adults with congenital heart disease.     

Pulmonary artery growth during treatment with oral prostaglandin E2 in ductus dependent cyanotic congenital heart disease.

Growth of the pulmonary arteries was assessed in 11 patients with cyanotic congenital heart disease treated with oral prostaglandin E2. Each patient was dependent on patency of the ductus arteriosus for maintenance of the pulmonary circulation. Measurements were made initially from angiographic data obtained in the neonatal period. Subsequent assessments were made at the time of surgery after an interval of 2-8 months, during which time all the infants had gained weight normally. Comparisons were made with data obtained from 13 normal necropsy specimens. In only 4 of the 11 patients had the pulmonary arteries grown appreciably during the treatment period. In 2 patients no growth occurred, while in 5 patients the pulmonary arteries had actually become smaller. It had been hoped that the pulmonary arteries would grow in all 11 patients during this period, facilitating later surgical intervention. In patients with ductus-dependent cyanotic congenital heart disease, the prolonged use of oral prostaglandin E2 should be restricted to patients in whom the pulmonary arteries are too small to allow a palliative operation to be performed initially.