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.     

The Cryopreserved Homograft Valve in the Pulmonary Position: Early Results and Technical Considerations

Since September, 1985, 20 patients have undergone implantation of a homograft valve in the pulmonary position (16 pulmonary, 4 aortic). There were 11 primary operations and 9 reoperations. In 7 of 11 primary operations the homograft valve was utilized as a composite conduit with a short Dacron extension. In four of five reoperations for a failed porcine valved conduit, a composite homograft conduit was used. Four patients underwent implantation of a free homograft in a previously repaired right ventricular outflow tract (RVOT). Age ranged from 15 days to 22 years. There was one operative death (5%), a seven-week-old infant with truncus arteriosus. Long-term follow-up ranges from 1 to 30 months. Clinical performance has been satisfactory in 18 of 19 patients. One patient undergoing free implantation of a pulmonary valve in the RVOT required replacement at 18 months with a porcine valve. In this patient, pulmonary insufficiency was caused by distortion of the annulus secondary to dilatation and pulmonary hypertension. Nine of 18 survivors do not require medication. Eleven of 18 have trivial to mild pulmonary insufficiency murmurs without symptomatology. The homograft valve is extremely useful in reconstruction of the right heart, however, early insufficiency murmurs have been noted. Distortion of the valve annulus may contribute to the early onset of a benign insufficiency murmur. Residual distal obstruction or pulmonary hypertension may be a contraindication to the use of a free homograft in the orthotopic position.     

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.     

Repair of Truncus Arteriosus and Interrupted Aortic Arch

A total of seven patients with truncus arteriosus and interrupted aortic arch (IAA) comprises our surgical experience in this condition. All underwent primary complete repair via median sternotomy between June 1985 and December 1989. Median age at repair was 8 days and median weight, 3.2 kg. Anatomy of these seven patients was truncus arteriosus type "1 1/2" in five patients and type II in two patients, IAA type B in six patients and type A in one patient. Aortic arch was reconstructed by direct anastomosis of ascending aorta and descending aorta. Right ventricle to pulmonary artery continuity was established with a porcine valved conduit in four patients, aortic homograft in two, and aortic homograft monocusp patch in one. Three patients have required five reoperations (three in one patient). One reoperation was due to compression of the left main bronchus from the reconstructed aorta, one was due to obstruction of the aorta at the site of IAA repair, and one was due to compression of the left main bronchus, right pulmonary artery, and residual stenosis across the hypoplastic ascending aorta. There were no early or late deaths and all seven survivors are currently well with a mean follow-up of 29 months from initial repair.     

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。     

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     

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.     

Repair of truncus arteriosus in infancy

Improvements in myocardial protection, surgical technique, and postoperative care have decreased operative mortality for neonatal repair of truncus arteriosus. Primary repair of truncus arteriosus in infancy without prior pulmonary artery banding is currently the preferred approach. During the period from 1982 to December 1990, 32 patients under the age of 12 months underwent surgical correction of truncus arteriosus at UCLA. The average age was 3.5 months (range, 12 days to 12 months). Three patients had interrupted aortic arch. Early mortality for the entire group was 15.6% (5/32); for those older than 1 month early mortality was 7% (2/28). In the past 4 years, early mortality has decreased to 8.3% (2/24); both of these patients had interrupted aortic arch. Excluding patients with interrupted aortic arch, there were no early deaths in the last 22 patients (1986 to 1990). Late mortality overall was 7.4% (2/27). In a mean follow-up of 73 months (range, 40 to 110 months), 71% (5/7) of the survivors with Dacron porcine-valved conduits required conduit replacement secondary to obstruction. In a mean follow-up of 36 months (range, 1 to 89 months), only 14% (3/21) of the patients with homografts required replacement secondary to obstruction.     

肺动脉右心室直接吻合纠治复杂先天性心脏病

目的 探讨肺动脉右心室直接吻合纠治复杂先天性心脏病的效果。方法 2001年3月至2004年6月，对17例复杂先天性心脏病病儿采用肺动脉右心室直接吻合方法予以纠治，其中3周岁以下者11例。肺动脉闭锁合并室间隔缺损6例，完全性大动脉错位合并室间隔缺损和肺动脉狭窄4例，永存动脉干4例，法洛四联症合并冠状动脉横跨右心室流出道3例。12例一期根治，5例分期纠治。结果 手术死亡1例。5例分期手术中的3例已成功二期根治。随访1～40个月，所有病儿生长发育良好，心功能0～Ⅰ级。结论 肺动脉右心室直接吻合应用于纠治某些复杂先天性心脏病可取得满意效果。对无同种带瓣管道可选用的婴幼儿病例，此术式尤为适用。     

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.     

Repair of truncus arteriosus: a considered approach to right ventricular outflow tract reconstruction.

OBJECTIVE: In repair of truncus arteriosus the accepted methods of establishing right ventricle (RV) to pulmonary artery (PA) continuity utilize an allograft or xenograft valved conduit. Alternatively, the PA confluence may be directly anastomosed to the RV with anterior patch augmentation, which may allow growth and delay or avoid subsequent RVOT obstruction. These methods of RVOT reconstruction were evaluated in infants undergoing truncus arteriosus repair. METHODS: A retrospective analysis of 61 infants undergoing repair of truncus arteriosus between November 1988 and June 2000 was performed. Median age was 34 days (range 1 day to 6.4 months). The patient cohort was subdivided into two groups (1) Valved conduit group: RV to PA continuity performed with a conduit in 38 patients using allograft (28) or xenograft (10). (2) Direct anastomosis group: direct RV-PA anastomosis performed in 23 patients, augmented anteriorly with monocusp (15) or simple pericardial patch (eight). RESULTS: There were eight hospital deaths (13%, 95% confidence limits 5--21%). Hospital mortality did not differ significantly between group 1 and 2 (three patients (8%) versus five patients (22%) respectively, P=0.23). By multivariate analysis, low operative weight (P=0.023), severe truncal regurgitation (P=0.022) and major coronary abnormalities (P=0.018), were independent risk factors for hospital death. Hospital survivors were followed-up from 1.3 months to 11.8 years (mean 4.2+/-3.4 years). There were eight late deaths with survival of 73+/-6% at 2 years and beyond. Survival was not influenced by method of RVOT reconstruction (Conduit versus direct RV-PA anastomosis, 2.76+/-7%, 63+/-10%, respectively, P=0.23). Freedom from surgical RVOT reintervention was 56+/-10% in group 1 and 89+/-10% in group 2 at 10 years (P=0.023). The use of a xenograft conduit was an independent risk factor for reintervention (P<0.001). CONCLUSIONS: In truncus arteriosus repair, RV to PA continuity established by a direct anastomosis was associated with a low incidence of surgical RVOT re-intervention. This technique has the potential for RVOT growth and may be a useful alternative when an appropriate allograft is unavailable, particularly in the neonate where the risk of pulmonary hypertension are lower.     

Pulmonary homograft implantation for ventricular outflow tract reconstruction: early phase results

The pulmonary valve homograft (PH) has been reported to have potential advantages over the aortic valve homograft, including a larger diameter, a thinner wall, and decreased intrinsic calcification. From January 16, 1986, to July 14, 1987, eight consecutive patients underwent repair of congenital cardiac anomalies using a cryopreserved PH. Patients ranged in age from 18 months to 32 years. Diagnoses included tetralogy of Fallot with pulmonary atresia (3 patients); tetralogy with absent pulmonary valve (1 patient); corrected transposition with pulmonic stenosis (1 patient); transposition of the great arteries, ventricular septal defect, and pulmonic stenosis (2 patients); and double-outlet right ventricle with pulmonic stenosis (1 patient). The PH was implanted orthotopically in the patient with absent pulmonary valve, and in the other 7 it was placed as a valved extracardiac conduit. Two of the tetralogy patients with severe bifurcational pulmonary stenosis and another with nonconfluent pulmonary arteries and origin of the left pulmonary artery from a patent ductus arteriosus had their repairs facilitated using the branching pulmonary arterial portion of the PH. There were no hospital or posthospital deaths. Postrepair right ventricular to left ventricular systolic pressure ratios were a mean of 0.35 at 18 hours postoperatively (range, 0.21-0.61). All patients were studied with Doppler and echocardiography after repair. The mean gradient across the PH was 9 mm Hg (range, 2-27 mm Hg), and no pulmonary valve incompetence was present. One patient (12.5%) required reoperation seven months after repair for conduit revision due to compression by the sternum and is now well.(ABSTRACT TRUNCATED AT 250 WORDS)     

永存动脉干纠治术中右心室流出道重建方式的选择

目的比较采用自体肺动脉和外管道连接右心室的两种不同手术方法，以选择更佳的手术方案。方法2000年2月至2006年9月，我院共收治23例永存动脉干患者，手术年龄为1．5～63．3月龄。根据右心室切口与肺动脉连接的方法不同分为两组，组Ⅰ：18例，采用自体肺动脉连接右心室；组Ⅱ：5例，采用外管道连接右心室至肺动脉。3例合并主动脉弓中断。采用Kaplan—Meier法分析两组患者的早期生存情况、术后生存时间、再手术情况，采用配对t检验或团体t检验分析患者远期肺动脉生长情况以及心功能的变化。结果术后早期死亡2例。术后随访17例，随访时间2．14±1．97年（32．00d～6．95年），随访中无死亡。总生存率为91．30％（21／Z3），生存时间的95％可信区间为5．55～7．15年。1例因术后右室流出道梗阻（RVOTO）再次手术。组Ⅰ生存率为94．44％（17／18），组Ⅱ为80．00％（4／5）。术后组Ⅰ主肺动脉与右心室吻合口直径早期为1．01±0．26cm，远期为1．32±0．45cm（P=0．019）；组Ⅱ术后主肺动脉与右心室吻合口直径早期为1．46±0．77cm，远期1．26±0．21cm（P=0．581）；两组吻合口、左右肺动脉开口处血流速度变化差异无统计学意义（P〉0．05）。组Ⅱ近远期左、右肺动脉开口直径均大于组Ⅰ。组Ⅰ早期左心室射血分数（LVEF）为62.82％，远期为69．87％（P=0．026）；组Ⅱ早期LVEF为58．17％，远期为64．00％（P=0．029），两组患者术后远期心功能均较术后近期明显改善。无因动脉干瓣膜反流而再手术者。结论永存动脉干手术成功率高，术后生存率及随访结果满意。采用自体肺动脉重建右心室流出道，使吻合口具有生长性，术后肺动脉及分叉处梗阻率低，术后心功能良好。术后早期死亡与合并主动脉弓中断及动脉干瓣膜反流有关。     

Homograft replacement of the pulmonary artery bifurcation

Severe deformity or nonconfluence of the pulmonary arteries was repaired during correction of congenital heart defects by implantation of a complete, fresh, antibiotic-sterilized homograft pulmonary artery bifurcation in 17 patients, aged 15 months to 17 years. Six patients had Fallot's tetralogy; five, pulmonary atresia and ventricular septal defect; three, truncus arteriosus; two, ventricular septal defect; and one, pulmonary atresia with intact ventricular septum. All but one had previously undergone one or more palliative or corrective operations. Thirteen patients survived the operation (76%; 70% confidence interval = 61% to 88%) and were followed up from 2 to 56 months postoperatively (mean = 16 months). All had symmetrical pulmonary perfusion on chest x-ray films and satisfactory homograft function on Doppler echocardiograms. There were no late deaths or reoperations. Four patients (24%; 70% confidence interval = 12% to 39%) died in the hospital of pulmonary or cardiac dysfunction despite a technically satisfactory reconstruction. Comparison of predicted and observed ventricular pressure ratios in nine patients showed that the factor contributed by a right pulmonary artery branch stenois was cancelled in most. The use of a complete, adult-size homograft has thus facilitated reconstruction of the pulmonary artery bifurcation and given encouraging early results.     

Homograft valve insertion for pulmonary regurgitation late after valveless repair of right ventricular outflow tract obstruction.

OBJECTIVE: Pulmonary regurgitation after valveless repair of right ventricular outflow tract obstruction (RVOTO) results in progressive right ventricular (RV) dilatation and dysfunction in an increasing number of patients. Since 1989, we have exclusively used cryopreserved homografts to restore pulmonary valve competence in these patients. Our 9-year-experience with pulmonary valve insertion (PVI) in such cases has been reviewed to evaluate the indications for this procedure and its benefits. METHODS: From 1989 to 1998, 49 patients (original diagnosis: tetralogy of Fallot in 42 patients and pulmonary stenosis in seven) aged from 3 to 42 years (mean 18 +/- 9 years) underwent PVI with homografts late (mean 13 +/- 7 years) after valveless repair of RVOTO (transannular patch, n = 38; pulmonary valvulotomy therefore tau chi infundibular patch, n = 11). Preoperatively, all patients had severe pulmonary regurgitation, cardiomegaly, significant to severe RV dilatation and dysfunction, fatigue, reduced exercise tolerance, and were in NYHA class II (n = 43) or III (n = 6). Ten patients had ventricular arrhythmia. RESULTS: There was one early death, due to air embolism, and one late death, due to ventricular arrhythmia. All survivors but one, who subsequently underwent heart transplant, had symptomatic improvement after homograft insertion. The mean RV end-diastolic diameter decreased from 38 +/- 9 to 26 +/- 8 mm (P < 0.01), and cardiothoracic ratio decreased from 0.62 +/- 0.07 to 0.54 +/- 0.04 (P < 0.01). Good late homograft function was the rule, with all the survivors being free of reoperation for valve failure. At a mean follow-up of 42 +/- 28 months, 41 patients (87% of the survivors) were in New York Heart Association (NYHA) class I and six in class II. Within this group three patients are still in treatment for RV failure and five for ventricular arrhythmias. In these patients, the average interval between RVOTO repair and PVI was significantly longer than in the others (18 +/- 7 vs. 12 +/- 6 years, P < 0.01). CONCLUSION: Homograft PVI is safe and provides clinical improvement with a significant reduction in RV volume overload and excellent mid-term results in most patients with severe PR late after RVOTO repair. This procedure should be undertaken early in symptomatic patients, before severe RV failure and ventricular arrhythmias ensue.     

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.     

Surgical treatment of complex cardiac anomalies: the 'one and one half ventricle repair'.

OBJECTIVE: One and one half ventricle repair is a surgical option for congenital cardiac anomalies characterised by right ventricle (RV) hypoplasia and/or dysplasia. METHODS: From March 1994 to March 2001, eight patients (mean age 9.1 years, range 7 months to 35 years) with hypoplastic and/or dysplastic RV underwent correction of their intracardiac anomaly in association with a BCPS (one and one half ventricle repair). Preoperative diagnoses included: Ebstein's anomaly of tricuspid valve (TV) in two, inlet ventricular septal defect (VSD) in association with straddling/overriding TV in two patients, pulmonary atresia-intact ventricular septum in one, tertralogy of Fallot in association with complete atrioventricular canal defect in one, truncus arteriosus in one and heterotaxy syndrome with VSD and anomalous systemic venous return in one. Four patients underwent previous surgery which included: main pulmonary artery (MPA) banding in two patients, pulmonary valvotomy, central shunt and right ventricular outflow tract reconstruction in one, pulmonary artery separation from truncus arteriosus and modified Blalock-Taussig shunt in one, and MPA closure in one. Two patients underwent a bidirectional cavo-pulmonary shunt before the one and a one half ventricle repair. Associated cardiac lesions were treated simultaneously. RESULTS: There were no hospital deaths. All the patients were discharged home in good clinical conditions. There were no late deaths or reoperations. At mean follow-up of 29.8 months (range 8 months to 7.3 years) all the patients are alive and in good general conditions. MPA percutaneous balloon dilation was performed in two patients at 33 and 4 months, respectively, both after MPA reconstruction (which was previously ligated) and dilation of the left pulmonary artery branch in one patient, repeated twice at 10 and 14 months from repair, for a hypoplastic left pulmonary artery after truncus arteriosus repair. CONCLUSIONS: Surgical treatment of congenital cardiac anomalies in the presence of a hypoplastic and or a dysplastic RV by means of one and one half ventricle repair has the advantage of reducing the surgical risk for biventricular repair, and compared to the Fontan circulation, it maintains a low right atrium pressure, a pulsatile pulmonary blood flow and improves the systemic oxygen saturation. Short and medium-term results are promising. Longer follow-up is needed, to prove the efficacy of such a repair, in the long term.     

One hundred pulmonary valve replacements in children after relief of right ventricular outflow tract obstruction

Background. Surgical repair of obstructive lesions of the right ventricular outflow tract (RVOT) in children commonly creates pulmonary valve incompetence that may eventually require pulmonary valve replacement (PVR). We reviewed our experience with PVR late after RVOT reconstruction.

Methods. We performed 100 PVRs in 93 children 1.1 months to 22.4 years (median 8) after RVOT reconstruction. Children with right ventricular to pulmonary artery conduits and primary PVRs were excluded. Age at PVR was 4.5 months to 27.9 years (median 9.5 years). Initial diagnosis was tetralogy of Fallot and variants, 62; critical pulmonary stenosis, 15; pulmonary atresia with intact ventricular septum, 7; and others, 9. Eleven patients had a redo PVR. A total of 62 PVRs were homografts; 38 were porcine valves.

Results. There was one early death. On follow-up of 5 months to 12.4 years (mean 4.9 years) there were no late deaths although 1 child underwent cardiac transplantation. Actuarial freedom from redo PVR at 8 years was 100% for porcine valves but 70% for homograft valves (p = 0.17). For children younger than 3 years at PVR, freedom from reoperation was 76% at 1 year and 39% at 8 years compared with freedom from redo PVR at 8 years of 100% for children older than 3 years. On latest echocardiogram 97% of porcine valves had mild or no pulmonary regurgitation compared with 72% of homograft valves.

Conclusions. PVR after RVOT reconstruction can be performed with low risk. Porcine valves may be superior to homograft valves although this advantage may be due to older age at time of PVR.     

Surgical treatment of complex cardiac anomalies: the ‘one and one half ventricle repair’

Objective: One and one half ventricle repair is a surgical option for congenital cardiac anomalies characterised by right ventricle (RV) hypoplasia and/or dysplasia. Methods: From March 1994 to March 2001, eight patients (mean age 9.1 years, range 7 months to 35 years) with hypoplastic and/or dysplastic RV underwent correction of their intracardiac anomaly in association with a BCPS (one and one half ventricle repair). Preoperative diagnoses included: Ebstein's anomaly of tricuspid valve (TV) in two, inlet ventricular septal defect (VSD) in association with straddling/overriding TV in two patients, pulmonary atresia-intact ventricular septum in one, tertralogy of Fallot in association with complete atrioventricular canal defect in one, truncus arteriosus in one and heterotaxy syndrome with VSD and anomalous systemic venous return in one. Four patients underwent previous surgery which included: main pulmonary artery (MPA) banding in two patients, pulmonary valvotomy, central shunt and right ventricular outflow tract reconstruction in one, pulmonary artery separation from truncus arteriosus and modified Blalock–Taussig shunt in one, and MPA closure in one. Two patients underwent a bidirectional cavo-pulmonary shunt before the one and a one half ventricle repair. Associated cardiac lesions were treated simultaneously. Results: There were no hospital deaths. All the patients were discharged home in good clinical conditions. There were no late deaths or reoperations. At mean follow-up of 29.8 months (range 8 months to 7.3 years) all the patients are alive and in good general conditions. MPA percutaneous balloon dilation was performed in two patients at 33 and 4 months, respectively, both after MPA reconstruction (which was previously ligated) and dilation of the left pulmonary artery branch in one patient, repeated twice at 10 and 14 months from repair, for a hypoplastic left pulmonary artery after truncus arteriosus repair. Conclusions: Surgical treatment of congenital cardiac anomalies in the presence of a hypoplastic and or a dysplastic RV by means of one and one half ventricle repair has the advantage of reducing the surgical risk for biventricular repair, and compared to the Fontan circulation, it maintains a low right atrium pressure, a pulsatile pulmonary blood flow and improves the systemic oxygen saturation. Short and medium-term results are promising. Longer follow-up is needed, to prove the efficacy of such a repair, in the long term.     

Current Method of Repair of Truncus Arteriosus

Surgical treatment of truncus arteriosus has been demonstrated to be optimized when approached in early infancy. In the past, the materials available, namely the 12-mm porcine valve Dacron conduit, restricted the size of the patient in whom early repair could be performed with increasing risk associated with decreasing age and size. The availability of homografts for reconstruction of right ventricular pulmonary artery continuity has allowed repair even in the neonatal period. Two hundred forty-four patients with truncus arteriosus have been operated on at the University of California San Francisco since 1975. In the past 5 years, 49 have been repaired using homograft reconstruction. The method of repair differs markedly from that of Dacron conduits, and specific points concerning homograft insertion including orientation, method of proximal anastomosis, and sizing are discussed in detail and the difference in insertion from the Dacron bovine heterograft conduit are discussed.