Surgical correction of types II and III truncus arteriosus.

In truncus arteriosus types II and III, one or both pulmonary arteries arise independently from either side of the truncus. In the surgical correction of this anomaly, we have utilized on operative technique in which the essential features are as follows: ventricular septal defect (VSD) closure, which directs left ventricular outflow into the truncus: (2) anastomosis of a Dacron tube containing a glutaraldehyde-preserved procine aortic heterograft to the right ventriculotomy: (3) removal of a circumferential band of the truncus containing both pulmonary artery orifices; (4) tailoring of the band of truncus tissue into a generous cuff which is anastomosed to the distal end of the valved Dacron conduit; and (5) restoration of aortic continuity with a tubular Dacron graft. Since 1971, 4 children ages 2 to 9 years have undergone successful correction of truncus arteriosus types II or III by this technique. In one patient with marked pulmonary hypertension and congestive heart failure preoperatively, the pulmonary vascular resistance had reverted to normal by 3 years after the operation. In one patient in whom bronchial collaterals to the right pulmonary artery were present, postoperative left ventricular failure required reoperation for ligation of the collaterals. All 4 patients are asymptomatic and fully active 5 to 60 months postoperatively. None has evidence of stenosis or insufficiency of the heterograft valve.     

Mid-term results after correction of type I and type II persistent truncus arteriosus in older patients

Abstract Objective: This study aims to analyze long‐term results after correction of type I and type II truncus arteriosus in older patients operated in one institution over five years. Methods: Between 2006 and 2010, 12 patients, median age 4 years, underwent repair of truncus arteriosus. Repair with reconstruction of the right ventricular to pulmonary artery continuity was performed using a valved conduit in 12 patients. Results: There was no early mortality. All patients are alive with their original conduit 0.6 to 5 years after correction. No patients required reoperations for conduit dysfunction. Recent clinical examination was undertaken in all patients and they are in good condition. Conclusions: Though mean age at operation was higher in this study than published results, the operation should be performed if the pulmonary vascular resistance is under 8 units.m² before operation .     

Surgical repair of type II truncus arteriosus without a conduit

A technique for reconstructing the right ventricular outflow tract in an infant with type II truncus arteriosus without using a prosthetic conduit is described. The truncal root is transected, and the confluence of branch pulmonary arteries is brought anterior to the aorta by using Lecompte's maneuver. The aorta is then reconstructed directly with an end-to-end anastomosis. The right ventricular outflow tract is reconstructed by anastomosing the posterior wall of the confluence directly to the apex of the vertical right ventriculotomy. A glutaraldehyde-preserved pericardial patch is then placed anteriorly to complete reconstruction of the right ventricular outflow tract.     

小儿永存动脉干外科诊治19例

目的 总结19例小儿永存动脉干(PTA)外科治疗的经验。方法 1989年1月至2002年6月外科手术纠治19例PTA。病儿2月龄～5岁；体重3．5—16．0kg。按Van Praagh分型，A1型8例，A2型9例，A3及A4型各1例。右室-肺动脉流出道重建用涤纶生物瓣管道和同种带瓣肺动脉各2例，同种带瓣主动脉11例，近1年的4例未用外管道。结果 手术死亡率10．5％(2，19例)。晚期死亡1例。结论 PTA易早期并发肺血管梗阻性疾病，应在1岁以内行纠治术。手术需防止室间隔缺损残余分流；离断肺动脉和修复动脉干缺损时，应避免损伤动脉干、瓣膜及冠状动脉口；如条件许可，A1型及A2型可不用外管道重建右室-肺动脉流出道，避免了因更换管道而再次手术。     

Successful correction of a Collett Edwards type II truncus arteriosus after bilateral pulmonary artery banding

A case of a 4 year old girl who underwent successful correction of a Collett Edwards type II truncus arteriosus following bilateral pulmonary artery banding is reported herein. The diagnosis of Collett Edwards type II truncus arteriosus was made when she was 5 months of age, after which the bilateral pulmonary artery banding was performed. This proved very effective and enabled elective radial surgery to be done. The radical operation was performed safely by a modified version of Rastelli’s procedure. The patient had an uneventful recovery and is now well with no symptoms one year following her operation.     

Successful correction of truncal valve regurgitation and conduit obstruction after previous Rastelli operation for truncus arteriosus (type I)

A 9-year-old boy had a successful surgical correction of truncal valve regurgitation and obstruction of extracardiac conduit which ensued previous Rastelli operation. He underwent Rastelli operation at the age of 10 months with the diagnosis of truncus arteriosus (Collet & Edwards Type I). At the age of 8 years, he was admitted because of slow weight gain and cardiomegaly (CTR 64%). Cardiac catheterization showed elevated RV pressure, increased pressure gradient across the conduit and severe truncal valve regurgitation. The extracardiac conduit was replaced with a 22 mm Hancock valved conduit and the truncal valve with 23 mm St. Jude Medical aortic valve. Postoperative catheterization revealed a normal RV pressure, only slight pressure gradient across the conduit and no truncal valve regurgitation. He is doing quite well 2 years after the operation.     

Successful correction of a Collett Edwards type II truncus arteriosus after bilateral pulmonary artery banding--a case report

A case of a 4-year-old girl who underwent successful correction of a Collett Edwards type II truncus arteriosus following bilateral pulmonary artery banding is reported herein. The diagnosis of Collett Edwards type II truncus arteriosus was made when she was 5 months of age, after which the bilateral pulmonary artery banding was performed. This proved very effective and enabled elective radial surgery to be done. The radical operation was performed safely by a modified version of Rastelli's procedure. The patient had an uneventful recovery and is now well with no symptoms one year following her operation.     

Total correction of truncus arteriosus with severe truncal valve insufficiency in neonate]

The prognosis of infants with truncus arteriosus associated with severe truncal valve insufficiency is quite poor. Total correction was successfully performed in a neonate with such a complicated anomaly. The patient was 21 days old female with anuria due to severe congestive heart failure preoperatively in spite of medical treatment. She underwent Rastelli operation and pulmonary artery was reconstructed using autologous pericardial 3 valved conduit. Truncal valve was 4 cusps with the malformed nodular margins and one cusp had cleft. This cleft was closed suturing the cleft cusp and adjacent cusp each other and annuloplasty was added in 4 commissures. She survived and her truncal valve insufficiency was still mild at 2 years post-operative period. Although total correction with truncal valve repair for such a severely ill neonate and young infant with truncus arteriosus as this patient has not been reported, our experience suggests that severe truncal valve insufficiency could be fairly well repaired by valvulo-annuloplasty.     

Reverse subclavian flap and aorto-pulmonary window technique for repair of interrupted aortic arch and truncus arteriosus

We report a surgical strategy for repairing an interrupted aortic arch (IAA) with truncus arteriosus (TA) by using a reverse subclavian flap and an aorto-pulmonary (A-P) window technique for preserving the pulmonary artery architecture. A 10-day-old neonate with type B IAA and type I TA with echocardiographic evidence of a significant distance between the ascending and descending aorta underwent surgical repair at the Bristol Royal Hospital for Children. The superior part of the arch was reconstructed using a reverse subclavian flap and the undersurface with a pulmonary homograft patch. The ascending aorta was separated from the pulmonary arteries using a Gore-Tex patch (A-P window type of repair) without disconnecting the branch pulmonary arteries, in order to preserve their architecture. The continuity between the right ventricle and the pulmonary artery bifurcation was established using a 12 mm Contegra conduit. The postoperative course was uneventful, and the neonate was discharged after 12 days. At follow-up, the patient remains well, gaining weight, with no echocardiographic evidences of obstruction. Reverse subclavian flap with homograft patch combined with and 'A-P window' technique for preservation of the pulmonary artery architecture is a useful and effective surgical strategy for neonates presenting with IAA associated with TA.     

The effect of repair technique on postoperative right-sided obstruction in patients with truncus arteriosus

OBJECTIVES: We reviewed our experience with repair of truncus arteriosus to assess the effect of type of right ventricular outflow tract reconstruction on perioperative morbidity, survival, and freedom from catheter-based interventions and reoperation. METHODS: Patients undergoing repair of truncus arteriosus from June 1990 through February 2004 were evaluated on the basis of operative procedure regarding preoperative and postoperative variables, the need for postoperative catheter-based intervention or reoperation, and survival on the basis of univariate, multivariable, and actuarial analyses. RESULTS: Of 54 study patients, 15 (28%) received a valved homograft, and 39 (72%) received a direct connection with a variety of hood materials. Five (9.1%) patients died. Valved homograft recipients were more likely to require reoperation than patients receiving direct connections (40% vs 15%, P = .046); however, valved homograft and direct connection recipients had a similar incidence of the combined end point of reoperation or catheter-based intervention (40.0% vs 37.5%, P = .865). Univariate and multivariable modeling demonstrated use of valved homografts or direct connections with an autologous pericardial hood to be predictive of the need for later catheter-based intervention or reoperation. Actuarial analysis demonstrated a trend toward improved outcomes in the direct connection group and within the direct connection cohort, a statistically significant difference on the basis of hood type. CONCLUSIONS: Although the direct connection technique might not prevent later catheter-based intervention, it does reduce the need for reoperation. Outcomes among direct connection recipients were associated with hood type: polytetrafluoroethylene hoods (W. L. Gore & Associates, Inc, Tempe, Ariz) had the lowest rate of reintervention, and untreated autologous pericardial hoods had the highest rate of reintervention. We report excellent outcomes with primary repair of truncus arteriosus. Where anatomically appropriate, we advocate the direct connection technique.     

Internal banding for palliation of truncus arteriosus in the neonate

A method for surgically limiting pulmonary blood flow in the critically ill neonate with truncus arteriosus is described. Two recent cases utilizing this technique are presented. Comparisons are made between this and other palliative surgical procedures used in truncus arteriosus.     

A case report of Rastelli operation in 2-month-old infant with truncus arteriosus (type II)

A two-month-old girl with heart failure from truncus arteriosus (Collett & Edwards Type II) underwent a total correction by the Rastelli procedure using 12-mm-diameter Hancock valved conduit. The orifice of pulmonary arteries was closed from inside without detachment of the pulmonary artery from truncus. The distal anastomosis of the conduit was made to left pulmonary artery. Primary sternal closure was difficult and delayed closure was performed using splint with a resin plate. The skin was primarily closed using bilateral advancement myocutaneous flaps. Complete closure of the sternum was made on the 11th postoperative day. A rotation flap of the right abdominal rectal muscle was used to cover the partially necrotic skin over the sternum. The patient had persistent respiratory and cardiac problems, but was discharged 14 months after surgery.     

Late results of the peel operation for replacement of failing extracardiac conduits

BACKGROUND: Pulmonary ventricle to pulmonary artery conduits have made repairing many complex congenital cardiac anomalies possible. Late patient outcome is adversely affected by the hemodynamic consequences of conduit failure and the need for reoperation for conduit replacement. METHODS: We retrospectively reviewed 102 patients (65 males, 37 females) who underwent operation with autologous tissue reconstruction ("peel operation") between May 1983 and November 2001, in which a prosthetic roof was placed over the fibrous bed of the explanted conduit. Ages ranged from 5 to 58 years old (median age 19 years old). Explanted conduits were Hancock (n = 54), homograft (n = 21), Tascon (n = 11), and other (n = 16). The conduit roof was constructed with pericardium (n = 91) and other (n = 11). A prosthetic pulmonary valve was utilized in 68 patients: porcine in 65 patients and mechanical in 3 patients. A nonvalved reconstruction was performed in 34 patients. Concomitant cardiac procedures were performed in 66 patients. RESULTS: Early mortality overall was 2% (n = 2) and was 0% for patients who underwent isolated conduit replacement (n = 36). Mean follow-up was 7.6 years (maximum, 19 years). Overall survival at 10 and 15 years was 91% (84.7, 97.2) and 76% (62.8, 91.7), respectively. Nine patients required reoperation related to the peel operation: regurgitation in nonvalved conduit (n = 7); moderate pulmonary bioprosthesis stenosis and regurgitation with atrial arrhythmia (n = 1); and pulmonary bioprosthesis endocarditis (n = 1). Overall survivorship free of reoperation for peel reconstruction failure at 10 and 15 years was 90.7% (82.6, 99.6) and 82% (69.4, 97.0), respectively. Survivorship free of reoperation for patients with a prosthetic valve was 93.7%, and for those with no prosthetic valve was 80.0% at 15 years (p = 0.57). At late follow-up, 89% of patients were in New York Heart Association functional class I or II. CONCLUSIONS: The peel operation simplifies conduit replacement, can be performed with low risk, and provides a generous-sized flow pathway. In our experience late results demonstrate a lower freedom from reoperation than conventional prosthetic or homograft conduits.     

The extracardiac Fontan procedure without cardiopulmonary bypass: technique and intermediate-term results

BACKGROUND: The extracardiac Fontan procedure (ECF) usually requires cardiopulmonary bypass (CPB). In this report, the results and techniques of this procedure without CPB at a single institution are presented. METHODS: Between August 1992 and December 2001, ECF without CPB was achieved in 24 of 44 patients undergoing an ECF. Mean age at surgery was 5.9 +/- 2.9 years, and mean weight was 20.7 +/- 12.6 kg. Diagnoses were tricuspid atresia in 9 patients, single-ventricle with pulmonary outflow tract obstruction in 7, pulmonary atresia/intact septum in 5, and other complex single-ventricle physiology in 3. Initial palliation was by arterial to pulmonary artery shunt in 21 and pulmonary artery banding in 1. A bidirectional cavopulmonary connection was created in 23 patients. A temporary inferior vena caval-to-atrial shunt was used to complete the procedure without CPB. Median graft size was 16 mm (range 14 to 20 mm). RESULTS: There was no early mortality, and 68% of patients were discharged without complications. Complications included persistent cyanosis in 4 patients, persistent pleural effusions in 2 (one chylous), and phrenic nerve injury in 1. Median postoperative hospital stay was 16 days (range 10 to 50) days. At a mean follow-up of 44 +/- 28 months, there was no conduit obstruction. One patient died 11 months postoperatively, and 1 patient received a heart transplant 26 months post-ECF. CONCLUSIONS: At intermediate term follow-up, the ECF without CPB appears to be safe and technically reproducible in selected cases. Ongoing follow-up of these patients is necessary to document the theoretical advantages of avoiding CPB.     

Excessive bleeding on extracorporeal membrane oxygenation after surgical repair of type I truncus arteriosus: A case report

Severe bleeding remains the most common complication of extracorporeal membrane oxygenation (ECMO) following surgical repair of congenital heart defects. We present a case of excessive hemorrhage within the first hours on ECMO support after repair of a type I truncus arteriosus. Bleeding control was achieved by surgical repair following failure of conventional interventions to control hemorrhage despite normalization of laboratory coagulation parameters. Aspects associated with bleeding and bleeding control during extracorporeal circulation after cardiac surgery are discussed.     

小儿永存动脉干右室流出道重建

目的 总结小儿永存动脉干(PTA)右室流出道重建治疗经验.方法 2000年1月至2007年12月共行PTA根治手术治疗43例,男26例,女17例.年龄1.5个月～3.8岁;体重3.2～23.0kg.Ⅰ型、Ⅱ型和Ⅲ型PTA分别为26例、11例和6例.18例PTA的肺动脉直接下拖至右室流出道切口上缘相吻合,前壁再用心包补片扩大;8例用Homograft管道、14例用牛颈静脉管道连接远端肺动脉和右心室,重建右室流出道;3例Ⅱ型者,肺动脉后壁用左心耳壁与右室流出道上缘做吻合,前壁再用心包补片扩大.结果 术后所有病儿均生存,5例表现为右心功能不全,2例右肺动脉(RPA)压差37.5～47.3 mm Hg(1mm Hg=0.133kPa),术后17d恢复至35.3mm Hg以下.左心室流出道阶差均小于20.3mm Hg.残余VSD(2mm)1例,乳糜胸1例.5例病儿肺血管阻力高,吸一氧化氮(NO)治疗7d左右后好转.随访3个月～3年,2例病儿RPA残余压差24.0～29.3 mm Hg,均无明显右心室或左心室流出道梗阻.结论 肺总动脉后壁直接与右心室切口作吻合重建PTA的右室流出道,早期和远期效果良好.牛颈静脉的带瓣管道的应用,解决了小尺寸同种带瓣管道来源不足的问题,操作简便.