Correction of truncus arteriosus with truncal valvar stenosis or insufficiency using two homografts

Surgical correction of truncus arteriosus requires the creation of right ventricular to pulmonary artery continuity and closure of the ventricular septal defect. A variety of conduits have been used including valved and nonvalved. Despite a significant incidence of truncal valvar stenosis and insufficiency, this valve has seldom been replaced. We present 4 cases of truncus arteriosus with truncal valvar stenosis or insufficiency that were repaired using two valved homografts: one to create the pulmonary outflow tract and the other to replace the abnormal truncal valve. Two of these patients are doing well after 4 months. Another child survived the operation and did well for 2 months when she died suddenly. The last child died 14 hours postoperatively from low cardiac output syndrome secondary to diabetic hypertrophic cardiomyopathy. When truncal valvar abnormalities are present, the primary repair of truncus arteriosus in an infant should include replacement of the truncal valve. Total correction can be successfully achieved using two valved homografts, resulting in long-term palliation and freedom from thromboembolic events and the use of anticoagulants.     

Truncal Valve Repair: Initial Experience in Neonates

Background. The identification of moderate to severe preoperative truncal valve regurgitation has been synonymous with significant postoperative mortality after neonatal repair of truncus arteriosus. Spurned by the deficiencies of current truncal valve substitutes surgeons are once again reexamining the option of truncal valve reparative techniques.

Methods. From May 1996 until June 1997, 8 children underwent correction of truncus arteriosus. A retrospective analysis was conducted.

Results. There was one in-hospital death secondary to a postoperative massive coronary air embolism. Moderate-to-severe truncal valve regurgitation was identified clinically and confirmed with cardiac ultrasound in 3 neonates with a mean age of 7 days (range, 4 to 12 days) all with quadracusp truncal valves. Successful truncal valve repair was accomplished in 2 infants, with a third neonate requiring homograft replacement with coronary reimplantation for failure in achieving valvular competence after attempted valvuloplasty. Postoperative echocardiograms in those neonates who underwent truncal valve repair confirmed a functional “tricuspid” valve with only mild to mild-plus regurgitation.

Conclusions. We give further credence to the hypothesis that primary neonatal truncal valve repair is feasible and may be successful in the avoidance and delay of serial truncal valve replacements using either mechanical or allograft substitutes.     

Improving early and intermediate results of truncus arteriosus repair: a new technique of truncal valve repair.

BACKGROUND: Despite improved surgical results for truncus arteriosus, overall mortality rates, remain higher than those reported for other complex congenital heart diseases, especially with truncal valve regurgitation or an interrupted aortic arch. METHODS: Seventeen patients had complete repair of the truncus arteriosus at the Cleveland Clinic Foundation between August 1993 and June 1997. The age at operation ranged from 2 days to 4.5 years. Associated abnormalities included interrupted aortic arch in 3 patients and abnormal coronary artery anatomy in 3. Four patients had more than moderate truncal valve insufficiency requiring concomitant truncal valve repair. RESULTS: There were no early deaths and only one late death at a mean follow-up of 24 months. The death occurred 3 months postoperatively and resulted from refractory pulmonary vascular obstructive disease in a patient who was referred at 1 year of age. Reoperation was required in 4 patients. CONCLUSIONS: Even in the presence of associated anomalies complete repair was performed with a low mortality rate. Truncal valve repair can be performed safely in the neonate with good results.     

Management of Truncal Valvular Regurgitation

The presence of significant truncal valvular regurgitation presents a technically difficult and life-threatening challenge during operations for correction of the truncus arteriosus anomaly. The problem was encountered in 19 of 71 patients (26%) who underwent such operations. A simple technique for managing this problem, namely, temporary cusp approximation, has evolved and has proved successful in all 11 patients in which it was used. The indications for replacement of the regurgitant truncal valve have not yet been defined. The suggestion is made that in all but the most severe instances of truncal valvular regurgitation, persistence of the regurgitation postoperatively may currently be preferable to replacement of the valve.     

Surgical management of severe truncal insufficiency: experience with truncal valve remodeling techniques

BACKGROUND: Truncal valve insufficiency has been a significant short- and long-term risk factor for repair of truncus arteriosus. Recent reports have documented the virtues of truncal valve repair. The purpose of this report is to review our experience with truncal valve repair and illustrate our techniques. METHODS: Between 1995 and 2000, 8 patients had interventions for severe truncal valve insufficiency at primary repair (3 patients) or in conjunction with conduit replacement (5 patients). One neonate had truncal valve replacement at initial repair early in the experience. The other 7 patients had truncal valve repair, 3 by valvar suture techniques. The remaining 4 patients had leaflet excision and annular remodeling in 3 (coronary reimplantation was required in 2) and commissure resuspension in 1 patient. RESULTS: Trivial to mild truncal valve insufficiency is present in the patients who had leaflet excision and annular remodeling (n = 3) and commissure resuspension (n = 1). Of the 3 patients who had valvar suture truncal valve repair, there was one death and 2 patients required acute valve replacement. The 7 survivors are doing well 1 month to 6 years postoperatively. CONCLUSIONS: Truncal valve repair by valvar suture techniques has not been successful in our practice. Truncal valve remodeling by leaflet excision and reduction annuloplasty is an effective method for truncal valve repair. When leaflet excision of a coronary sinus of Valsalva is required, coronary artery translocation can be accomplished.     

Repair of truncus arteriosus in the neonate and young infant

Infants with truncus arteriosus present a difficult management issue. Because of the high operative mortality, repair is often delayed beyond the first 3 to 6 months of age. We reviewed our experience with 11 neonates and young infants with truncus arteriosus undergoing repair (median age, 21 days). Five patients also had major truncal valve insufficiency, and 2 required valve replacement. Right ventricle-pulmonary artery continuity was established with a porcine valved conduit in 3 patients and an aortic or pulmonary homograft in 8. There was 1 operative death (9%; 70% confidence limits, 3%-22%) and 1 late death over a mean follow-up of 21 months (range, 4 to 32 months). Eight of the 9 late survivors are growing normally. Echocardiographic examination revealed normal ventricular function in all patients (mean shortening fraction, 39%). Doppler assessment demonstrated trivial prosthetic or homograft valve regurgitation in 7 patients and mild to moderate obstruction in 5 patients. This recent experience with repair of truncus arteriosus indicates that the operative risk is low even in the neonate. Repair in the first month of life should be recommended before the development of critical congestive heart failure or irreversible pulmonary vascular disease.     

Repair of the truncal valve and associated interrupted arch in neonates with truncus arteriosus

OBJECTIVE: Truncal valve regurgitation and interrupted aortic arch have frequently been identified as risk factors in the repair of truncus arteriosus. We wished to examine these factors in the current era including the impact of truncal valve repair. METHODS: Between January 1992 and August 1998, 50 patients underwent surgical repair of truncus arteriosus. Their ages ranged from 2 days to 6 months (median, 2 weeks). Nine patients had associated interrupted aortic arch. Of the 14 patients (28%) in whom truncal valve regurgitation was diagnosed preoperatively, 5 had mild regurgitation, 5 had moderate regurgitation, and 4 had severe regurgitation. Five underwent truncal valve repair and 1 underwent homograft replacement of the truncal valve with coronary reimplantation. RESULTS: The actuarial survival was 96% at 30 days, 1 year, and 3 years. There were no deaths in patients with associated interrupted aortic arch. The 2 deaths in the series occurred in patients with truncal valve regurgitation, neither of whom underwent repair. Postoperative transthoracic echocardiography in patients who underwent valve repair showed minimal residual valvular regurgitation. None of the patients has required reoperation because of truncal valve problems or aortic arch stenosis at a median follow-up of 23 months (range, 1-60 months). Conduit replacement has been done in 17 patients (34%) after a mean duration of 2 years. The freedom from reoperation for those who had an aortic homograft was 4 years and for those who had a pulmonary homograft was 3 years. CONCLUSION: Despite the magnitude of the operation, excellent results can be achieved in complex forms of truncus arteriosus. In the current era interrupted aortic arch is no longer a risk factor for repair of truncus. Aggressive application of truncal valvuloplasty methods should neutralize the traditional risk factor of truncal valve regurgitation.     

儿童永存动脉干的外科治疗

目的　总结儿童永存动脉干外科治疗经验。　方法　近９ 年来共收治永存动脉干５例,年龄３～１２ 岁,平均６－３ 岁。Ⅰ型２ 例（肺动脉均起于动脉干远侧）,Ⅱ型２ 例, Ⅳ型１ 例。肺循环时间和肺动脉排空时间均无明显延长。手术在体外循环心内直视下进行,室间隔缺损（ＶＳＤ） 补片缝合于动脉干瓣环并使其环缩。右心外管道采用缝制自体新鲜心包瓣涤纶人工血管４ 例,同种异体主动脉１ 例。　结果　术毕肺动脉压均明显下降,跨外管道压差７～３５ ｍｍ Ｈｇ。２ 例术后早期死亡与肺动脉病变无关。存活３ 例中１ 例并发低心排出量和呼吸功能不全,２ 例术后恢复顺利。分别随访１１４、９６ 和３４ 个月,心功能均为Ⅰ级,１ 例术后８ 年死于外管道梗阻。　结论　儿童永存动脉干仍可有手术指征; 带自体心包瓣人工血管远期效果良好; 纠正动脉干瓣关闭不全,防止ＶＳＤ 残余漏,避免过大的跨外管道压差和缩短手术时间,可提高手术成功率和远期效果     

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.     

Transplantation in truncus arteriosus combined with interrupted aortic arch

We present the case of an infant with truncus arteriosus combined with a dysplastic truncal valve not amenable to repair and interrupted aortic arch. Due to the disappointing results of truncal valve replacement we decided to perform cardiac transplantation. The infant survived transplantation and was discharged 4 weeks after surgery.     

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.     

Hyperammonemia and neonatal cardiac failure

Severe hyperammonemia (hyperNH3) in neonatal cardiac failure after cardiac surgery is rare. We report a case of a 2470-g female infant born at the week 37 of gestation with complex congenital heart disease (truncus arteriosus type III, interrupted aortic arch and tricuspid valve insufficiency) and hemodynamically non-significant intrahepatic arterio-venous malformation. She developed hyperNH3 (highest NH3 blood level: 467 μmol/L) without severe liver failure (INR of 1.9). The origin of the hyperNH3 was multifactorial including limited capacity of liver detoxification function due to congenital porto-caval shunt, liver ischemia, excessive protein intake and increased protein catabolic rate. HyperNH3 treatment partially succeeded in decreasing ammonia level and included discontinuation of protein intake, administration of phenylacetate and sodium benzoate. This case highlights the fact that NH3 detoxification by the liver has limitations for a neonate with multifactorial causes that decrease liver perfusion.     

Total repair for truncus arteriosus]

Total repair for truncus arteriosus using an external conduit was performed in 12 patients from 1978 through 1989. Six cases were infants (mean age: 3.4 months) and 6 were children (mean age; 1 years 9 months). Two cases had Collet-Edwards type II truncus and the other 10 cases had type I truncus. One of the infants was associated with an interruption of the aorta and another had a severe regurgitation of the truncal valve (TrV). For external conduits, we used a non-valved conduit in one infant, a composite valved conduit of Dacron containing a heterograft valve in 4 children and a valved pericardial roll made of an autologous or porcine pericardium in 5 infants and 2 children. One infant with a severe regurgitation of the TrV needed valve replacement along with enlargement of the annulus of the TrV. One infant who had replacement of the TrV died early postoperatively. Another infant died 10 months after total repair due to an infection of an external conduit. Cardiac catheterization was performed in all 10 survivors. The mean value for the systolic pulmonary/systemic pressure ratio decreased from 0.98 +/- 0.09 preoperatively to 0.36 +/- 0.09 postoperatively. Replacement of an external conduit was performed due to a conduit stenosis in 2 children and 1 infant, 10 years and 2 months, 7 years and 9 months, and 1 year and 8 months after the total repair, respectively. In one of these 2 children, replacement of the aortic valve was performed due to a severe aortic regurgitation. We conclude that our results of total repair for truncus arteriosus were satisfactory. However, it remains to be solved how to manage an infant with truncus arteriosus associated with a severe regurgitation of the TrV.     

Neonatal repair of truncus arteriosus: continuing improvement in outcomes

BACKGROUND: Repair of truncus arteriosus in the neonatal and early infant periods has become standard practice at many centers. We reviewed our recent experience with repair of truncus arteriosus in neonates, with a focus on early and intermediate outcomes. METHODS: From July 1992 to December 1999, 65 patients 1 month of age or less underwent primary complete repair of truncus arteriosus. Median age was 10 days, and median weight was 3.2 kg. Major associated anomalies included moderate or severe truncal valve regurgitation in 15 patients (23%), interrupted aortic arch in 8 (12%), coronary artery abnormalities in 12 (18%), and nonconfluence of the pulmonary arteries in 3 (5%). Median durations of cardiopulmonary bypass and cardioplegic arrest were 172 minutes and 90 minutes, respectively. Circulatory arrest was employed only in 7 patients undergoing concomitant repair of interrupted arch. Reconstruction of the right ventricular outflow tract was achieved with an aortic (n = 39) or pulmonary (n = 26) allograft valved conduit (median diameter, 12 mm). Replacement (n = 6) or repair (n = 5) of a regurgitant truncal valve was performed in 11 patients, and interrupted arch was repaired in 8. RESULTS: There were three early deaths (5%). Early reoperations included reexploration for bleeding in 3 patients, emergent replacement of a pulmonary outflow conduit that failed acutely in 1 patient, and placement of a permanent pacemaker in 1. Mechanical circulatory support was required in 1 patient. During the median follow-up of 32 months, there were two deaths. The Kaplan-Meier estimate of survival was 92% at 1 year and beyond. The only demographic, diagnostic, or operative factors significantly associated with poorer survival over time were operative weight of 2.5 kg or less (p = 0.01) and truncal valve replacement (p = 0.009). Actuarial freedom from conduit replacement among early survivors was 57% at 3 years. CONCLUSIONS: Repair of truncus arteriosus in the neonatal period can be performed routinely with excellent survival, even in patients with major associated abnormalities.     

Severe truncal valve stenosis: diagnosis and management

Severe truncal valve stenosis, due to dysplastic valve, may be found in a small number of patients with truncus arteriosus. Its presence complicates the surgical management of this anomaly due to lack of optimal therapeutic options and associated ventricular dysfunction. We report a case of truncal root replacement using a homograft for the management of such condition, and review the literature related to its management and outcome.     

Rastelli and Norwood combination for the treatment of type I truncus arteriosus and hypoplastic aortic arch: a case report

Although truncus arteriosus is often treated with low mortality and morbidity rates, truncal valve patency and aortic arch and coronary artery anomalies are factors that can contribute to a worse outcome. In this report, we present our experience with the combination of Rastelli and Norwood procedures for the treatment of Type I truncus arteriosus that was complicated by a hypoplastic aortic arch.     

Staged biventricular repair for persistent truncus arteriosus with aortic arch obstruction following bilateral pulmonary artery banding

Persistent truncus arteriosus associated with aortic arch obstruction (Van Praagh type A4) is a rare combination with an extremely high rate of mortality. Our current approach is staged biventricular repair following bilateral pulmonary artery banding (bPAB). The patient in case 1 underwent bPAB at five days old, then biventricular repair was performed 10?days later because duct became restrictive despite prostaglandin administration. In case 2, the patient underwent bPAB via a left side thoracotomy at eight days old concomitant with an aortic arch repair using an extended direct anastomosis. Complete biventricular repair was delayed until eight months after the bPAB due to respiratory issues, then both banded branch pulmonary arteries were augmented by auto-pericardial patches at that time. A catheter examination three years after the complete biventricular repair showed that both branch pulmonary arteries were widely open. The patient in case 3 underwent emergency bPAB and truncal valve repair with a cardiopulmonary bypass at three days old for severe truncal regurgitation. Banding tapes on both sides were tightened two days after the operation. Although the hemodynamic condition was stable and truncal regurgitation was reduced, the patient died one month later from sudden septic shock.     

Ⅰ型和Ⅱ型永存动脉干的外科治疗结果及随访分析

目的分析单中心过去5年内Ⅰ型和Ⅱ型永存动脉干患者外科治疗及随访结果,以提高其临床治疗效果。方法回顾性研究分析2006年5月至2010年10月阜外心血管病医院共收治17例共同动脉干患者的临床资料,其中Ⅰ型共同动脉干13例,Ⅱ型4例;平均年龄4.7(0.7～19.0)岁。合并其它心脏畸形分别为共同瓣关闭不全、肺静脉异位引流、二尖瓣关闭不全和房间隔缺损等。术前心导管检查测得肺血管阻力(4.4±2.2)Wood.U。所有患者均行一期根治术,其中右心室到肺动脉的连接材料分别为同种主动脉(3例)、同种肺动脉(2例)和牛颈静脉管道(12例)。对所有生存患者进行随访。结果术后死亡1例,早期死亡率5.8%(1/17),生存16例;体外循环时间(165±52)min,主动脉阻断时间(114±29)min,术后呼吸机使用时间(106±148)h;有2例发生胸腔积液,2例气管切开,其余患者无相关并发症。随访16例,随访0.6～5.0年,生存质量良好,无再次手术患者。结论对于年龄错过了最佳治疗期的Ⅰ型和Ⅱ型永存动脉干患者,术前应常规进行心导管检查,对肺血管阻力在可接受范围内的患者应积极治疗,可以取得较好的近期生存率,但其远期生存率还有待进一步随访。     

Left atrioventricular valve repair technique in partial atrioventricular septal defects

BACKGROUND: The aim of our study was to evaluate the effect of chordal transfer around the cleft on left atrioventricular valve competence in the late postoperative period. METHODS: Forty-four adult patients underwent surgical correction of partial atrioventricular septal defect between 1983 and 1997. Fenestration was found in 8 patients (18.2%) and cleft, in 35 (79.5%). There was no chordal support of the free edges of the left superior and left inferior leaflets around the cleft in 18 patients. Two chordae were mobilized from the left lateral leaflet and reimplanted into the tip of the left superior and left inferior leaflets around the cleft. RESULTS: At 5 years postoperatively, left atrioventricular valve insufficiency was severe in 5 patients and moderate in 11 patients who had had cleft closure alone. In contrast, severe valvular insufficiency was present in only 1 patient in the group with chordal transfer (p < 0.05). Reoperation was done in 5 patients with isolated cleft closure. Left AV valve replacement was performed in 1 patient. CONCLUSIONS: Chordal transfer plus cleft closure with interrupted sutures significantly reduces early and late left atrioventricular valve incompetence and also decreases the rate of reoperation.     

Fifteen-year experience with surgical repair of truncus arteriosus

From 1965 until March, 1982, 167 patients underwent surgical repair of truncus arteriosus. The age at operation ranged from 18 days to 33 years (mean 6 years). There were 48 hospital deaths (28.7%). The following factors had a positive correlation with the possibility of a surgical death: age at operation less than 2 years (p less than 0.001), a postrepair pulmonary arterial/left ventricular pressure ratio greater than 0.5 for patients with two pulmonary arteries (p less than 0.001) and greater than 0.6 for patients with unilateral absence of a pulmonary artery (p less than 0.02), and a postrepair right ventricular/left ventricular pressure ratio greater than 0.8 (p less than 0.008). The 119 hospital survivors were followed up for a total of 829 person-years. Late survival rate at 5 years was 84.4% and at 10 years, 68.8%. Preoperative factors that correlated with a reduced long-term survival rate were as follows: increasing age at operation (p = 0.004), the presence of moderate or severe truncal valve insufficiency (p = 0.008), lower pulmonary/systemic flow ratio (p = 0.04), and unilateral absence of a pulmonary artery (p less than 0.001). Thirty-six patients required reoperation during the follow-up period (30%) primarily for replacement of the right ventricular-pulmonary arterial conduit and/or for truncal valve replacement. The long-term results obtained in these patients support the need for early repair of the anomaly, improvement in the methods for control or repair of the truncal valve insufficiency, and the continued search for better extracardiac valved conduits.