Immediate outcomes after the Ross operation in children and adults

The Ross procedure has been increasingly utilized to treat various forms of left ventricular outflow tract obstruction and aortic regurgitation. Compared with alternative valve replacement options (mechanical, porcine, homograft), benefits of the Ross procedure include the lack of anticoagulation and the potential growth and durability of the autograft valve. Patients undergoing the Ross procedure represent a heterogeneous group of patients, ranging from neonates to adults with congenital or acquired heart disease. Prior procedures are common, as are additional hemodynamic abnormalities requiring intervention at the time of the Ross procedure. The postoperative course may be complicated by ventricular dysfunction, arrhythmia, bleeding and pulmonary hypertension. The purpose of this review article is to evaluate the short term results of the Ross procedure in children and young adults, including current results at our institution, with an emphasis on management in the intensive care unit after surgery.     

Mitral Valve Replacement with the Pulmonary Autograft in Children: A Word of Caution

A 4½-month-old patient who underwent mitral valve replacement for congenital mitral stenosis using a pulmonary autograft is reported. Failure of the autograft resulted in pulmonary hypertension, leading to pulmonary regurgitation in the reconstructed right ventricular outflow tract, then tricuspid regurgitation, refractory right heart failure, and death. Caution should be exercised in applying this procedure with children, particularly those at risk for pulmonary hypertension.     

An Infant with Subvalvar and Valvar Aortic Stenosis,Subvalvar and Valvar Pulmonary Stenosis,Severe Biventricular Hypertrophy and Pulmonary Hemorrhage: A Case Report

An infant with subvalvar and valvar pulmonary stenosis, subvalvar, and valvar aortic stenosis and hypertrophic cardiomyopathy, who presented with pulmonary hemorrhage, is reported. He had right ventricular hypertrophy, thickened pulmonary valve leaflets, severe asymmetric left ventricular hypertrophy with outflow tract obstruction, and a thickened and dysplastic aortic valve.     

Persistent truncus arteriosus operated during infancy: Long-term follow-up

Summary Between July 1974 and October 1988 19 consecutive infants (mean age 2.7 months, range 5 days to 11.7 months) underwent surgical correction for persistent truncus arteriosus by one surgeon (J.L.M.). Continuity between the right ventricle and pulmonary arteries was achieved with an antibiotic-sterilized aortic homograft (diameter 13–18 mm) together with patch closure of the ventricular septal defect. There were 3 early postoperative deaths (16%): 1 patient had severe aortic regurgitation, the other 2 had preoperative cardiac arrests. Of the latter, 1 had suffered severe cerebral damage, and the other developed recurrent pulmonary hypertensive crises and low cardiac output. The 16 survivors have been followed for 3.1–17.3 years (mean 7.8 years). Four patients required subsequent replacement of the homograft for stenosis, aortic valve replacement for regurgitation, or both (3.0, 4.0, 8.5, and 12.0 years postoperatively). Of the 16 survivors, 15 are in NYHA class I. Of the 13 patients who have not had aortic valve surgery, 9 have no evidence of stenosis or regurgitation. In the 14 children with the original homograft the median of the residual peak gradient across the right ventricular outflow tract is 15 mmHg (range 10–40 mmHg), and no patient has severe homograft regurgitation at follow-up. Repair of persistent truncus arteriosus has been achieved with low early mortality and no late mortality, which reflects excellent long-term function of the homograft. Furthermore, if truncal valve function is good at presentation, patients are unlikely to require aortic valve surgery.     

自体心包修复儿童主动脉瓣狭窄9例病例系列报告

目的：总结并分析以自体心包修复儿童先天性主动脉瓣狭窄的手术方法和早期预后。方法：纳入2013年7月至2015年6月在复旦大学附属儿科医院行自体心包主动脉瓣成形术的先天性主动脉瓣狭窄患儿,收集患儿的一般资料,围手术期情况,并发症,术前、术中、术后和随访时的超声心动图资料。结果：符合本文纳入标准的9例患儿进入本文分析,男6例,女3例,年龄4月龄至9岁。术前超声提示,三叶式和二叶式主动脉瓣分别为3例和6例;重度狭窄6例,中度狭窄1例,重度狭窄伴中度返流2例。9例均以自体心包再造或扩大主动脉瓣瓣叶。术后即刻与术前超声心动图比较：主动脉瓣最大跨瓣压差［（31.6±9.4）mm Hg vs（73.0±22.2）mm Hg, P=0.000］和主动脉瓣平均跨瓣压差［（15.8±18.3）mmHg vs （35.8±18.3）mmHg, P= 0.004］均下降。术后随访24～48（32±8）月,无死亡和再干预病例,未见严重不良事件,未见主动脉瓣重度狭窄或重度反流、升主动脉狭窄或扩张、主动脉瓣瓣环狭窄与扩张、瓣膜脱垂或瓣膜赘生物病例;3例患儿分别在随访18、24和12个月时出现瓣叶活动僵硬,瓣叶开放不完全。术后左室后壁厚径均呈下降趋势;末次随访时,8例患儿左室后壁厚径Z值（Z-LVPWd）均下降至正常水平（＜2）。结论：以自体心包修复主动脉瓣可改善先天性主动脉瓣狭窄患儿的血流动力学,手术风险低,术后早期疗效可,避免或延缓儿童主动脉瓣置换术,减少施行ROSS手术机会。     

A Novel Use of Amplatzer Duct Occluder

This report describes the use of the Amplatzer patent ductus arteriosus occluder to close a left ventricle to descending aorta conduit. The patient was a 10-year-old male who was born with critical aortic stenosis and left ventricular outflow tract obstruction. After initial valvotomy, he underwent left ventricular to descending aorta conduit placement. At the age of 10, he had a Konno procedure to enlarge the left ventricular outflow tract and 21-mm St. Jude aortic valve placement. Closure of the conduit was not addressed because it was inaccessable from median sternotomy. Postoperatively, echocardiogram revealed significant flow through the conduit with a wide pulse pressure. Cardiac catheterization was performed with the premise to close the conduit with an Amplatzer patent ductus arteriosus occluder device.     

A Single Center’s Experience with the Ross Procedure in Pediatrics

The use of a pulmonary autograft for aortic valve replacement (AVR) has become more prevalent than other forms of AVR in the pediatric population. We reviewed the data on pediatric patients who underwent the Ross procedure at our institution from 1993 to 2005. Sixty patients <18 years old who underwent a Ross procedure had available clinical and echocardiographic data collected and statistical analysis performed. Mortality rate was 3.3%, while overall survival and freedom from reoperation of either the homograft or the autograft were 96.7% and 66.2%, respectively, at 10 years. Freedom from reoperation of the left ventricular outflow tract was 60.5% at 10 years. Echocardiographic data showed aortic regurgitation to be mild or less in 76% of patients by last follow-up, while dilation of the sinuses of Valsalva had occurred in 52%. Compared to other AVR options, the Ross procedure in eligible pediatric patients demonstrates good intermediate survival rates and continued growth potential, yet a time-dependent need for reoperation.     

Exercise Stress Echocardiography After Childhood Ross Surgery: Functional Outcome in 26 Patients From a Single Institution

Adult studies suggest a better functional outcome after aortic valve replacement with a pulmonary autograft compared with mechanical or homograft valves. Little is known about functional results after Ross surgery in growing children. This study reports formal exercise stress echocardiographic data from 26 pediatric Ross patients. A retrospective cohort study analyzed stress echocardiographic data of patients who underwent Ross surgery as a child (<17 years old). All patients were operated by a single surgeon and underwent a Bruce protocol stress echocardiogram on the treadmill. Twenty-six patients (4 girls) were 9.3 ± 5.0 years at surgery and 14.9 ± 3.5 years (range 6.6-19.7 years) at follow-up. Mean follow-up was 5.4 ± 3.7 years (median 4.2). All were asymptomatic. The exercise time was normal in 87% of cases at 12.8 ± 2.5 min. On stress echocardiography, the mean right-ventricular outflow tract (RVOT) gradient increased from 38 ± 22 mmHg at rest to 82 ± 33 mmHg after exercise, but this did not correlate with exercise times. Stress echocardiography is useful in evaluating patients after childhood Ross surgery for aortic valve disease. In this pediatric cohort, most patients achieved normal exercise capacity. The presence of mild or moderate RVOT obstruction had no significant impact on exercise capacity.     

Transcarotid Balloon Valvuloplasty with Continuous Transesophageal Echocardiographic Guidance for Neonatal Critical Aortic Valve Stenosis: An Alternative to Surgical Palliation

Neonatal critical aortic valve stenosis is a life-threatening malformation if untreated. Before the late 1980s, the preferred treatment was surgical valvotomy; however, operative mortality was high. Early reports of transcatheter balloon dilation were encouraging, although femoral artery damage and aortic valve insufficiency were procedural limitations. With new balloon catheter technology, transumbilical, transvenous, and transcarotid approaches have been advocated, although a comparison with recent surgical results has not been performed. We compared all neonates who presented to our institution since 1985 with the diagnosis of critical aortic stenosis. Ten patients underwent surgical transventricular valvotomy and 13 patients underwent balloon valvuloplasty via a right carotid cutdown with continuous transesophageal echocardiographic guidance. Prior to intervention, all patients had either left ventricular dysfunction, an aortic valve gradient >100 mmHg, significant mitral valve insufficiency, and/or ductal dependent systemic blood flow. All patients had successful relief of aortic valve obstruction with normalization of left ventricular function and successful discontinuation of prostaglandin E₁. Use of continuous transesophageal echocardiographic guidance resulted in fluoroscopic exposure of only 12 ± 8 minutes. At the latest follow-up, a similar proportion of patients has required additional aortic valve procedures (38% vs 25%) and overall mortality (20% vs 15%) is similar. In the transcarotid group, 9 of 13 patients (69%) have a normal appearing right carotid artery by Duplex imaging, and no neurologic events have been reported. Balloon aortic valvuloplasty via a right transcarotid approach is safe, simplifies crossing the valve, and is effective for the initial palliation of neonatal critical aortic stenosis. The use of transesophageal echocardiographic guidance reduces fluoroscopy exposure, enables accurate assessment of hemodynamics without catheter manipulation or angiography, and avoids femoral artery injury.     

儿童主动脉瓣病变的外科治疗策略

目的 探讨儿童主动脉瓣病变的外科治疗策略.方法 回顾性分析2010年1月至2016年1月间收治的13例主动脉瓣病变患儿的临床资料.其中,男9例,女4例;年龄1～12岁,年龄分布1～3岁6例,3～6岁3例,6～12岁4例;体重12～36 kg,平均24 kg.术前经心脏超声及心脏CT明确诊断,先天性主动脉瓣病变8例,后天获得性主动脉瓣病变5例.手术在全身麻醉、低温、体外循环下进行,结合超声、CT及术中情况决定手术方式.结果 全组13例中,行改良Ross手术4例,主动脉瓣成形术3例,主动脉瓣机械瓣置换手术3例,Bentall术1例,Ross手术2例.全组无手术死亡,术中采用冷血停跳液或冷晶体停跳液灌注,合并主动脉关闭不全时,切开直接灌注,术中均应用超滤.术后有效随访患儿11例,2例患儿未规律随访,随访时间6个月～5年,全部存活,正常生活不受影响.机械瓣置换的长期使用华法林抗凝,目前无并发症出现.2例随诊过程中发现瓣膜压差大于40 mmHg,一般状况可,随访中未发现明显瓣膜严重反流.结论 儿童主动脉瓣病变的外科治疗方法有多样性的特点,本文旨在为心外科医师在儿童主动脉瓣病变的治疗策略方面提供更多的临床经验,儿童主动脉瓣疾病治疗的临床效果还有待进一步探讨.     

Exercise Testing in Children with Pulmonary Valvar Stenosis

Pulmonary valvar stenosis with intact ventricular septum is a common anomaly. This lesion poses a fixed obstruction to the right ventricular outflow. The right ventricle ejects the entire cardiac output across the stenotic valve. Right ventricular systolic pressure and oxygen demand are increased at rest and more so with exercise. Exercise tolerance in children and adults with mild valvar pulmonary stenosis is nearly normal, but is diminished in those with moderate and severe stenosis, indicating impaired ability to sustain adequate cardiac output. Following relief of stenosis, cardiac performance improves in children, but remains abnormal in adults. This appears to be related to postoperative resolution of right ventricular hypertrophy in children, whereas myocardial fibrosis may explain the lack of improvement in adults.     

Severe aortic or pulmonary valve stenosis in premature infants

Successful treatment of severe aortic (AS) or pulmonary valve (PS) stenosis by balloon valvuloplasty in term neonates is well-established. Sometimes, AS or PS diagnosed antenatally, progresses to severe left or right ventricular hypoplasia respectively. Successful fetal balloon valvuloplasty cannot be assumed to significantly change the natural history. In this review of premature infants, balloon valvuloplasty was used in extremely small babies. Five with severe AS (32 to 36 weeks, birth weight 1.4 to 1.9 kg) had percutaneous balloon aortic valvuloplasty during the first 10 days of life. Seven infants with severe/critical PS (28 to 36 weeks, birth weight 1.2 to 1.9 kg) had percutaneous balloon pulmonary valvuloplasty during the first 9 days of life. Two with pulmonary atresia (1.9 and 0.85 kg), underwent successful radiofrequency assisted balloon pulmonary valvuloplasty. There were no procedural deaths but one infant developed severe aortic insufficiency.     

Noonan syndrome with double-chambered right ventricle

The association of Noonan syndrome and a double-chambered right ventricle has not been reported previously in the medical literature. We report two patients with Noonan syndrome associated with pulmonary valve stenosis and double-chambered right ventricle due to anomalous hypertrophied muscle bundles. Pulmonary valve stenosis was operated on. However, hypertrophied muscle bundles were not resected, as they caused no obstruction to blood passage. Postoperative follow-up examinations revealed no symptoms. In conclusion, in those with Noonan syndrome there may be anomalous muscle bundles in the right ventricular cavity. It may not be necessary to resect them if they do not obstruct the right ventricular outflow tract.     

右心室双出口的个体化治疗

目的 探讨右心室双出口外科手术的临床疗效和STS-EACTS分类方法的临床价值.方法 2001年1月至2007年9月期间外科手术的87例右心室双出口的患儿,男65例,女22例;年龄:12d～14岁;体重:3.1～40kg,平均(10.4±7.1)kg;术前SPO2 60%～98%,平均(81±9)%;心功能Ⅰ级5例,心功能Ⅱ级23例,心功能Ⅲ级49例,心功能Ⅳ级10例;按照STS-EACTS分类法,室间隔缺损型26例,法洛四联症型48例,完全性大动脉转位型10例,远离大动脉型3例.进行单心室修补9例,双心室修补74例.包括单纯心内隧道23例,同时补片扩大右室流出道31例,Rastelli 7例,REV 8例,大动脉调转术4例,Kawashima1例,其余为姑息手术4例.同时纠治合并肺动脉分支狭窄15例,主动脉弓病变5例,二尖瓣狭窄1例,主动脉瓣下狭窄伴右乏式窦瘤1例,胸骨裂1例.结果 全组围术期死亡2例,术后3个月死亡1例,术后随访1个月～5年,2例存在轻度左室流出道狭窄,6例存在右室流出道残余梗阻,压差15～75 mm Hg.术后心功能多可恢复至Ⅰ～Ⅱ级,但有7例心功能Ⅲ级,其中5例与右室流出道残余梗阻有关.结论 STS-EACTS分类法对于右心室双出口的个体化治疗具有更好的临床指导意义,而右心室流出道残余梗阻是影响预后的重要因素,值得进一步关注.     

Mitral valve replacement in the neonate: A report of two cases

Summary Mitral valve replacement was performed in two critically ill neonates with severe mitral stenosis and regurgitation. Both patients had coexisting severe aortic stenosis previously treated by transventricular aortic valve dilatation. Preoperative echocardiography showed a mitral annulus measuring between 11 and 12 mm in diameter—too small for any of the commercially available free-standing valve prostheses. Accordingly, the porcine valve from a commercially available 12-mm external-valved conduit was removed and sutured into position. The function of the valve was satisfactory in both cases and cardiopulmonary bypass was discontinued easily with a good cardiac output established in both. Both patients died—one at 20 h and one 36 h after surgery. In each case, the immediate cause of death was acute bleeding secondary to preexisting hepatic failure and depletion of coagulation factors. At autopsy, both prostheses had been correctly placed and were competent. Left ventricular outflow tract obstruction secondary to strut imposition was not evident. Both patients were very ill preoperatively. Although neither patient survived mitral valve replacement, we were encouraged by the relative hemodynamic improvement in the immediate postoperative period. We believe that prosthetic valve replacement could become a viable therapy for the neonate with severe mitral valve abnormalities.     

Utility of Intraoperative Transesophageal Echocardiography in the Assessment of Residual Cardiac Defects

To investigate the accuracy of immediate postbypass transesophageal echocardiography in the assessment of residual cardiac defects, we compared intraoperative transesophageal echocardiograms with intra/postoperative data in 86 patients, aged 4 days to 30.7 years (median = 1.4 years), at risk for a total of 174 postoperative lesions: right (n= 55) or left (n= 26) ventricular outflow tract obstruction, ventricular septal defect (n= 65), aortic (n= 12) or mitral regurgitation (n= 8), or mitral stenosis (n= 8). Accuracy of intraoperative transesophageal echocardiography was evaluated based on comparison with (1) immediate post-bypass left (n= 4) or right (n= 9) ventricular outflow tract pressure gradients by pullback in the operating room, (2) direct surgical inspection of residual ventricular septal defects (n= 3), (3) pulmonary artery oxygen saturation (n= 49), (4) right ventricular outflow tract pullback gradient (n= 24), and (5) transthoracic echocardiogram (n= 51) performed within 40 days of surgery. The results indicate that intraoperative transesophageal echocardiography agreed with intra/postoperative data in 87% of patients at risk for right ventricular outflow tract obstruction, 96% at risk for left ventricular outflow tract obstruction, 97% at risk for ventricular septal defect, and 100% at risk for aortic regurgitation, mitral regurgitation, or mitral stenosis. Significant residual lesions led to immediate surgical revision in 11 cases: 3 ventricular septal defects, 6 right and 2 left ventricular outflow tract obstructions. Of these, intraoperative transesophageal echocardiography confirmed and quantified suspected residual lesions in 7 and identified unsuspected lesions in 4 cases. Immediate postbypass transesophageal echocardiography proved reliable for assessing residual ventricular septal defect, mitral stenosis, and mitral or aortic regurgitation. Although accurate for assessment of the left and right ventricular outflow tracts in most patients, transesophageal echocardiography may not reliably reflect the severity of obstruction in all cases.     

Inoue球囊瓣膜成形术治疗儿童先天性肺动脉瓣狭窄22例

目的探讨先天性肺动脉瓣狭窄病例经皮穿刺Inoue球囊肺动脉瓣成形术的体会。方法患儿22例。男10例,女12例。年龄9.5~14.0(11.2±2.6)岁,先行右室造影及右心导管检查,测量压力及肺动脉瓣环直径,应用Inoue环形导丝建立钢丝轨道,使导丝前端在肺动脉形成自然圆圈状以固定轨道钢丝。选用最大径较肺动脉瓣环直径大20%~40%Inoue球囊进行扩张。结果本组22例均扩张成功。5例术后出现反应性右室流出道狭窄,经药物治疗后好转。1例出现三尖瓣乳头肌断裂,经外科治疗痊愈。结论对于较大儿童应用Inoue球囊进行肺动脉瓣成形术治疗效果良好,但应严格掌握操作程序。     

婴儿法乐四联症一期矫治手术中保留肺动脉瓣功能的意义

目的 随访137例婴儿法乐四联症一期矫治手术中跨环补片患儿的近、中期疗效,总结法乐四联症手术中右室流出道及肺动脉瓣处理的经验.方法 采取一期手术矫治1岁以下法乐四联症患儿238例,其中22例经右房疏通右室流出道,79例补片加宽右室流出道;137例跨肺动脉瓣环补片.术中使用不同方法部分恢复肺动脉瓣功能90例,对合并的...     

Dilatation von Klappen- und Gefäßstenosen

New catheter material and non-traditional access sites increased the efficacy of balloon dilation of stenosed valves and vessels. Right heart lesions such as critical pulmonary stenosis and severe pulmonary valve stenosis in childhood have a success rate of over 90%. In older children, the results of balloon dilation are excellent with a need for reintervention in less than 5% of cases and a very low mortality rate. Dysplastic pulmonary valves will need up to 45% surgical therapy. In case of Tetralogy of Fallot or complex lesions with severe pulmonary stenosis, balloon dilation of the right ventricular outflow tract can help to improve oxygen saturation. Dilatation of peripheral pulmonary arteries is associated with a high complication rate and the outcome is not always rewarding. Stent balloon implantation have a better efficacy and safety. Aortic valve stenosis with significant gradients in childhood are a clear indication for valvuloplasty. Excellent results are usually expected in 80 to 90% of patients. On the long term, valve replacement might still be warranted. In case of the critical aortic stenosis, the transvenous, antegrade access reduced the complication rate significantly. Comparison with surgical results are difficult, but the safety and mortality of both procedures seem to be comparable. Discrete native aortic coarctation as well as recoarctation in childhood can be dilated with a success rate of 65–90%. In the older patient stent implantations are preferred to balloon dilation. In the younger infants surgical management is advisable, because of very high recoarctation rate.     

Midterm Results of the Ross Procedure in a Pediatric Population: Bicuspid Aortic Valve Is Not a Contraindication

Our objective was to analyze retrospectively the short- and midterm results of the Ross operation in children and the impact of bicuspid aortic valve (BAV) disease on outcome. From 1991 to 2003, 41 patients (26 male, 15 female) underwent a Ross procedure. Aortic disease was congenital in all but one. Sixty-six percent had BAV. Mean age at operation was 10.13 ± 5.6 years (range, 0.4–18.3 years). Root replacement technique was performed in all but two (inclusion technique). There were two early deaths. Mean follow-up was 6 ± 3.8 years (range, 0.1–14 years). The autograft (neo-aorta) presented absent, trivial, mild, and moderate regurgitation in 42%, 46%, 10%, and 2%, respectively, at latest follow-up. Root dilation was seen in 64% of the patients (mean Z-score, +3.53 ± 0.04). Four patients (9%) required allograft replacement, two for endocarditis and two for stenosis. Allograft stenosis (gradient >20 mmHg) was detected in 44% of the remaining patients, without symptoms or the need for reintervention. Estimated freedom from allograft replacement at 5 and 10 years was 97% and 89%. Left ventricular dimensions and function were normal in all patients. No difference was found between patients with BAV and those with tricuspid aortic valves in aortic regurgitation or root dilation. BAV was not identified as a risk factor for root dilation. In conclusion, the Ross operation remains an excellent option for aortic valve replacement in children. BAV is not associated with a worse outcome and is not a risk factor for aortic root dilation at medium-term follow-up. Long-term follow-up is, however, required.