Absent pulmonary valve syndrome: operation in infants with airway obstruction.

From 1979 through 1991, 19 infants with absent pulmonary valve syndrome and airway obstruction were seen for surgical treatment. All patients underwent extensive pulmonary artery aneurysmorrhaphy using cardiopulmonary bypass. Fourteen patients had simultaneous transatrial ventricular septal defect (VSD) closure, infundibular resection, and placement of a short transannular patch; 2 had transventricular VSD closure and infundibular resection without a transannular patch; 1 underwent transventricular VSD closure and transannular patching; and 2 underwent pulmonary artery aneurysmorrhaphy alone with the VSD left open. All 19 infants had good hemodynamics when taken from the operating theater, but 3 died postoperatively of severe airway obstruction, despite further tracheobronchopexy procedures in 2 (hospital mortality rate, 16%; confidence limits, 7% to 29%). Among the 16 patients discharged from the hospital, there was one late death. Five other patients have required reoperation for branch pulmonary artery stenosis (n = 2), residual airway obstruction resulting from persistent pulmonary artery dilatation (n = 1), closure of VSD (n = 1), and homograft valve insertion for pulmonary incompetence and right ventricular dysfunction (n = 1). There are 15 long-term survivors. Eight of them have episodic bronchospasm of mild to moderate severity, and all are responsive to sympathomimetic bronchodilator aerosols. The remaining 7 are asymptomatic.     

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)     

Discontinuity between the heart and the pulmonary circulation. Its management with the use of a valved external conduit.

The Hancock conduit that contains a porcine xenograft valve has been used in part of the cardiac repair of 22 patients with complex congenital heart disease. Five patients had a severe form of tetralogy of Fallot; six had pulmonary atresia; five had transposition of the great vessels, ventricular septal defect (VSD), and pulmonic stenosis; five had truncus arteriosus; and one had "corrected" transposition, VSD, and pulmonic stenosis. The hospital mortality was 2/22. This conduit has proved a satisfactory method to establish right ventricular-pulmonary artery continuity.     

Arterial switch with internal pulmonary artery banding. A new palliation for TGA and VSD in complex cases.

In most cases, one stage repair by arterial switch operation (ASO) is the optimal treatment for neonates with transposition of the great arteries (TGA). Nevertheless, a ventricular septal defect (VSD) associated with TGA remains a major risk factor for early death and reoperation after complete repair in neonates with complex anatomy. A new palliative approach for such specific cases is proposed. An internal pulmonary artery banding (IPAB), as that already used to palliate other cardiac malformations, is performed in association with ASO instead of VSD closure. At the end of ASO, a circular polytetrafluorethylene (PTFE) patch with a 4-mm central hole is oversewn into the neo-pulmonary trunk. We adopted this method in a 17-day-old boy with TGA, VSD, hypoplastic tricuspid valve and diminutive right ventricle. After the operation the child thrived and was doing well without medication. Satisfactory growth of the right ventricle and tricuspid valve was observed by echocardiography during the following months. The patient successfully underwent VSD closure and IPAB removal 2 years after the first procedure. ASO with IPAB could be appropriate in all forms of TGA and VSD in which VSD closure appears too challenging in the neonatal period and in patients with uncertain suitability for biventricular repair. We preferred to use IPAB instead of classic PAB in order to reduce the risk of pulmonary valve damage, pulmonary artery distortion, and above all pulmonary artery dilatation and related coronary compression. In the presented case the strategy as well as IPAB worked according to our expectations.     

What is necessary for improvement of arterial switch operation?

We report long-term results of the arterial switch operation (ASO) and postoperative complications related to operative procedure. Between 1998 and 2007, ASO were performed in 42 patients [transposition of the great arteries (TGA) with intact ventricular septum:21, TGA with ventricular septal defect (VSD):13, Taussig-Bing anomary (TBA):7, and double outlet right ventricle with noncommitted VSD:1]. Hospital death occurred in 1 patient (2.4% mortality rate) due to low cardiac output syndrome (LOS) caused by prolonged aortic clamp, who had TGA with VSD, coactation of aortic arch and right ventricular outflow obstruction. Four patients required re-operation (freedom from re-operation rate was 84.7%). Two had pulmonary stenosis and 1 needed right ventricular outflow tract (RVOT) reconstruction with transannular patch. His pulmonary valve used for previous VSD closure was diminished. The other required muscle resection of RVOT. Five patients presented grade II or more neo-aortic insufficiency and 2 of them were TBA. Both these TBA, rerouting of left ventricular outflow tract (LVOT) to the aorta was challenging, they showed LVOT obstruction postoperatively. One underwent aortic valve plasty and the other had residual VSD closure. None had coronary event or abnormality in coronary arteriography. Selection of surgical procedure should be based on morphologic features in order to improve surgical outcome.     

Repair of complete atrioventricular septal defect with tetralogy of Fallot

Repair of complete atrioventricular canal with tetralogy of Fallot was performed in 9 patients. Ventricular septal defect was closed through the right atrium using a single polytetrafluoroethylene patch with ample anterior extension to avoid subaortic obstruction. The atrial septal defect was closed with a separate patch. Undivided atrioventricular valve leaflets were sandwiched between the two patches. Right ventricular outflow tract stenosis was relieved by pulmonary valvotomy and an infundibular patch in 7, a supravalvar patch (none transannular) in 6, and right ventricle-to-pulmonary artery conduit in 2. There was one hospital death (1/9, 11%) in a patient with persistent clinically significant postoperative pulmonary stenosis and low cardiac output requiring reoperation and right ventricle-to-pulmonary artery conduit insertion. There was no late mortality. All patients are asymptomatic 0.3 to 5.6 years after operation. Follow-up right ventricular outflow tract gradient ranged from 11 to 43 mm Hg and was 70 mm Hg in 1 patient who later had successful relief of obstruction. Three patients had mitral valve insufficiency; 1 needed reoperation. Aggressive relief of right ventricular outflow tract stenosis with maintenance of pulmonary valve competence and use of two separate patches for closure of the septal defects contribute to optimum immediate and long-term results after repair of this lesion.     

Tetralogy of Fallot with subpulmonary ventricular septal defect

Among a total of 608 patients with tetralogy of Fallot, 35 with tetralogy and subpulmonary ventricular septal defect (VSD) have undergone intracardiac repair at the Children's Hospital, Buenos Aires. The crista supraventricular was absent in 62.9% of these patients; it was hypoplastic in 37.1%. Only 3 patients (8.6%) were severely cyanotic and required operation in infancy. The main and left and right pulmonary arteries were relatively large in all but 1 patient. Nevertheless, patch-graft enlargement across the pulmonary valve ring was necessary in 20 (57.1%). We believe that closure of the subpulmonary VSD increase the apparent severity of the pulmonary stenosis and necessitates patch-graft enlargement in patients in whom the appearance of the right ventricular outflow tract before repair would not have suggested its need.     

Autologous Pericardium Patch Aneurysm after Ventricular Septal Defect Closure and Arterial Switch Operation

Abstract   A neonate with L-transposition of the great arteries with ventricular septal defect underwent complete repair using fresh autologous pericardium to close the ventricular septal defect as well as to reconstruct the neo pulmonary artery sinuses. Four months later, the child came back with right ventricular inflow obstruction related to aneurysmal pericardial patch, severe tricuspid regurgitation, and severe supra-valvular pulmonic stenosis. At reoperation, there was a redundant, aneurysmal pericardial patch densely adherent to the septal and posterior leaflets of the tricuspid valve, which was damaged. The pericardial patch was replaced, the pulmonary artery enlarged, and tricuspid valve repaired. Postoperative course was uneventful, but residual moderate tricuspid regurgitation required intensive medical treatment.     

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     

Repair of ventricular septal defect after pulmonary artery banding.

Since 1964, 90 patients have undergone two-stage surgical repair of ventricular septal defect (VSD) with pulmonary artery banding (PAB) in early infancy and total repair at an average age of 4 years. Reconstruction of the pulmonary artery was accomplished with a pericardial patch, woven Dacron patch, or transverse angioplasty. The VSD was closed with a knitted Dacron patch in 75 patients and by primary suture technique in 13 patients. The VSD closed spontaneously in 2 patients. The mortality rate for patients who had repair and debanding was 9 per cent (8 patients), including 4 deaths due to severe pulmonary hypertensive disease, 3 from congestive heart failure, and one from atrioventricular block. Twenty patients underwent repeat cardiac catheterization several months to 7 years after total repair. This study revealed no shunt in 16 patients and a minimal shunt not requiring operation in the other 4 children. Slight residual stenosis of the pulmonary artery was found in 2 patients and a residual infundibular stenosis in another 2 patients. We believe two-stage surgical treatment of VSD in severely ill infants under one year of age is safe and reliable.     

Arterial repair for simple and complex forms of transposition of the great arteries

Transposition of the great arteries (TGA) has traditionally been repaired by redirection of atrial flow. Concern over the late development of right ventricular and sinus node dysfunction has prompted increased use of the arterial repair. This approach was utilized in 11 patients ranging in age from 7 days to 22 months (mean 5.3 months) and in weight from 2.6 to 12.2 kg (mean 5.1 kg). Five patients had intact ventricular septum and underwent primary repair electively or after poor response to balloon atrial septostomy. A large ventricular septal defect (VSD) was present in 6 patients, one of whom had coarctation of the aorta and one with type B interrupted aortic arch. Pulmonary artery banding was done in 3 of these patients in addition to subclavian artery aortoplasty and primary arch repair. Spontaneous VSD closure occurred in 2 banded patients. All patients had normal coronary artery distribution. Conduits were not used in any repair. There were 2 operative deaths caused by injury to the right coronary artery prior to establishing bypass in one patient and to left ventricular (LV) failure in a patient operated on at 5 months of age with an LV pressure of only one-half systemic level. The 9 surviving patients are well from 4 to 38 months after operation (mean 15 months). No patient has congestive heart failure and 5 receive no medications. Catheterization in 4 patients revealed widely patent coronaries in each. Trivial aortic insufficiency was noted in 1 patient. Each had mild to moderate supravalvar pulmonary stenosis (12 to 51 mmHg). LV function was normal in each case.(ABSTRACT TRUNCATED AT 250 WORDS)     

Innovative technique for repair of Tetralogy of Fallot with absent pulmonary,valve syndrome using autologous pericardial patch with monocusp valve

Background  Conventional surgery for absent pulmonary valve is repair with valve conduit between right ventricle (RV) and Pulmonary artery (PA).We describe a technique of repairing absent pulmonary valve with autologous pericardial patch with monocusp valve, without using valve conduit and its results. Methods  From February 2004 to September 2006, 14 consecutive patients with absent pulmonary valve syndrome (APVS) were repaired using this technique. Age range was 5–168 months (median- 24 months) and weight range was 6–31 kgs (median-10 kgs), 5 were infants. Varying degree of respiratory symptoms were present in all patients including tachypnoea, difficulty in feeding, wheezing, recurrent pneumonia and cyanosis. Repair consisted of ventricular septal defect closure, relief of right ventricular outflow obstruction with autologous pericardial patch with monocusp valve. PA aneurysmorrhaphy and PA reduction arterioplasty were done in selected cases. Result  There was one mortality. The follow up ranged from 12 months to 36 months, respiratory symptoms disappeared or were significantly improved in all patients. Two dimensional (2D) Echo showed trivial to mild pulmonary regurgitation (PR) in 11 patients & moderate in two patients. One patient had minimal right ventricular outflow tract (RVOT) obstruction. None of them required reintervention. Conclusion  New innovative technique of using autologous pericardial patch with monocusp valve in absent pulmonary valve syndrome can be safely performed in infants and children. It avoids conduit related problems like reintervention and is economical. Our early and midterm results are encouraging, however long term results are awaited.     

Growth of the hypoplastic ascending aorta after radical palliation

Surgical treatment of hypoplasia of the ascending aorta is a difficult problem. An approach using proximal pulmonary artery to distal aorta bypass with distal pulmonary artery banding was employed in 19 patients with an ascending aorta less than 6 mm in diameter but a patent aortic outflow. There were 11 male and 8 female neonates and infants, and weight ranged from 2 to 4.5 kg (median weight, 3.5 kg). Four patients had transposition of the great arteries, 12 had subaortic stenosis, and 5 had interrupted aortic arch. There was one operative death. Cerebral cyanosis developed in 3 of the 4 patients with transposition of the great arteries and necessitated additional operations within the first year postoperatively. Therefore, palliation with a pulmonary artery to descending aorta conduit and banding was abandoned in such patients. All 14 surviving patients with normally related great arteries had successful palliation and growth of left heart structures. Subsequent procedures included a Fontan/Damus procedure in 1 patient, patch aortoplasty and pulmonary arterioplasty in 5 patients, arch reconstruction with aortic valvulotomy and pulmonary arterioplasty in 2, double arch reconstruction in 2, and simple patch aortoplasty with plans for subsequent pulmonary arterioplasty and ventricular septal defect closure in 1. Results demonstrate that in patients with a hypoplastic ascending aorta, this radical method of palliation can result in growth of right and left heart structures and thus provides the possibility of biventricular repair.     

Fontan type operation for complex lesions. Surgical considerations to improve survival

Twenty-five of 49 patients who underwent a Fontan type operation had complex lesions other than tricuspid atresia with ventriculoarterial concordance. Three patients had significant subaortic stenosis. Thirty-four palliative operations, including nine Glenn shunts, were performed before the Fontan operation. Direct atriopulmonary anastomosis was performed in 21 patients. In four, valved conduits were used. Twelve patients had right atrioventricular valve patch closure (three had running and nine had interrupted suture technique). On the basis of the presence of increased or decreased pulmonary blood flow before any surgical intervention, patients were divided into Group 1 (previous pulmonary artery banding, N = 8) and Group II (pulmonic stenosis, N = 17). Postoperatively, in Group I, 87% had significant effusions, mean right atrial pressure was higher (20.6 +/- 6.5 torr), and hospital stay longer (31 days). In Group II, 40% had significant effusions, mean right atrial pressure was lower (16.5 +/- 4.3 torr), and hospital stay shorter (15 days). Significant atrioventricular valve patch disruption occurred in three patients (two had running suture technique), and conduit occlusion occurred in two. Four patients (three with subaortic stenosis and pulmonary artery banding) without an established Glenn shunt required Fontan takedown for persistent low cardiac output, two of whom died (2/25 or 8%). There were three late deaths (3/23 or 13%). Nineteen of 20 surviving patients observed from 2 months to 6 years are doing well. We believe that early Fontan takedown in patients with persistent low cardiac output, interrupted suture technique for atrioventricular valve closure, avoidance of valved conduits, and a preliminary Glenn shunt in patients with pulmonary artery banding and/or subaortic stenosis can further improve the results with the Fontan operation for complex lesions.     

Unilateral absence of a pulmonary artery in absent pulmonary valve syndrome: a case report and review of literature.

A six-year-old boy presented to the Cardiology clinic with history of mild cyanosis and dyspnea on exertion from age 1. He had a to-and-fro murmur at the middle left sternal border. Chest examination was normal but chest x-ray showed a small left lung. Echocardiography established the diagnosis of tetralogy of Fallot (TOF) and absent pulmonary valve with severe pulmonary regurgitation and moderate stenosis at the pulmonary valve site. There was severe dilatation of the main and right pulmonary arteries. The left pulmonary artery (LPA) could not be seen. Angiography failed to show a LPA. This case of an absent LPA associated with absent pulmonary valve syndrome is discussed and the literature is reviewed.     

Repair of aortic coarctation in the first year of life

Twenty-five infants under 1 year of age (mean, 10.3 weeks and 4.0 kg) underwent coarctation repair. Eight had ventricular septal defect (VSD), 3 had transposition of the great arteries with VSD, and 5 had severe tubular hypoplasia. One infant required mitral valve replacement, and 1 required repair of total anomalous pulmonary venous return. Fifteen had repair by primary anastomosis. Seven underwent Dacron or subclavian aortoplasty; the advantages and technique of angioplasty are reviewed. Three patients required bypass grafts. Seventeen patients survived operation. All 5 patients who had severe tubular hypoplasia died postoperatively. The mortality for repair of coarctation with VSD by simultaneous pulmonary artery banding was high; for coarctation with VSD we currently recommend repair without banding, followed by VSD closure if indicated. Three infants have been treated successfully in this manner, with early VSD closure in 1 and regression of the VSD during follow-up in 2. The 17 survivors have been followed for a mean of 41 months with 3 late deaths. Of the 17 survivors, all of whom had a primary anastomosis, 3 have residual gradients. Of the 11 survivors who had preoperative hypertension, 6 are still hypertensive; 3 of these have a gradient between the upper and lower extremities. It is striking that 3 have persistent hypertension despite repair under the age of 1 year.     

Closure of Isolated Ventricular Septal Defect with Detachment of the Tricuspid Valve

Detachment of the septal leaflet of the tricuspid valve is an alternative technique for obtaining complete visualization of a perimembranous ventricular septal defect (VSD) in cases where the VSD is obscured by the chordae tendineae or a pouch formation of the septal leaflet. This method presents theoretical concerns because it has the potential for causing postoperative valvular insufficiency. We therefore evaluated valvular function in patients who underwent VSD closure with detachment of the tricuspid valve. In a consecutive series of 153 patients who underwent VSD closure using a transatrial approach, 13 had incision of the tricuspid valve. Follow-up echocardiographic studies were performed on these patients at least 1 year following operation. There were no operative deaths. Color Doppler echocardiography revealed no residual shunt in any of these patients. Ten patients had no evidence of tricuspid stenosis or regurgitation. One patient had trivial tricuspid regurgitation. Moderate tricuspid regurgitation was observed in two patients of these, one patient was a small infant who had a VSD complicated by pulmonary hypertension. The other patient had a VSD with a mitral cleft, pulmonary hypertension, and Down's syndrome. The incised tricuspid valve was resus-pended by solely running sutures. In conclusion, detachment of the tricuspid valve is a safe and useful method for adequate exposure of a VSD. However, this method should be avoided in patients with Down's syndrome and in small infants. Furthermore, repair of the incised valve should not be performed using only running sutures.     

Biventricular repair of double outlet right ventricle with non-committed ventricular septal defect (VSD) by VSD rerouting to the pulmonary artery and arterial switch

Objectives: Biventricular repair of double outlet right ventricle non-committed ventricular septal defect (DORVncVSD) is usually achieved by a VSD rerouting to the aorta. This technique can be limited by the presence of tricuspid chordae and by the pulmonary artery to tricuspid valve distance. Furthermore, there is an important risk of late subaortic obstruction related to the long patch required that creates a potential akinetic septal area. Presented here is another technique; by VSD rerouting to the pulmonary infundibulum and arterial switch. Methods: Ten patients, with DORVncVSD, underwent a VSD rerouting to the pulmonary infundibulum followed by arterial switch. Seven had a previous pulmonary artery banding and one a moderate infundibular stenosis. The median age at surgery was 16 months (range 3 weeks to 4.5 years). All patients had a bilateral infundibulum, with a large persistent subaortic conus, D malposition of the aorta, side-by-side vessels and double loop coronary patterns. The VSD was perimembranous with inlet or trabecular extension. Subaortic obstruction was constant. The VSD was severely distant from both the aortic and the pulmonary annulus. The operation was conducted through a combined approach. The VSD was constantly enlarged superiorly. The almost permanent subaortic obstruction was released. The VSD was always found quite close to the pulmonary infundibular ostium. The arterial switch technique was adapted to the complex coronary anatomy. Results: There was one non-cardiac death. At a mean follow-up of 20 months, all nine survivors are in NYHA class I, in sinus rhythm, and have no subaortic gradient greater than 15 mm. Conclusion: This technique of VSD rerouting to the pulmonary artery and arterial switch limits greatly the size of the rerouting patch, respects the tricuspid chordae and is independent of the pulmonary artery–tricuspid valve distance. In this early series of biventricular repair of DORVncVSD, the VSDs were always found close to the pulmonary artery, allowing this new type of repair.     

Arterial switch operation with ventricular septal defect repair and aortic arch reconstruction

The arterial switch operation for transposition of the great arteries or double outlet right ventricle with ventricular septal defect (VSD) and aortic arch obstruction is a challenging procedure. One-stage neonatal repair is preferred; however, palliation may be indicated in the newborn who presents with Swiss cheese septum or the patient with extracardiac complications such as necrotizing enterocolitis or subarachnoid hemorrhage. The aortic arch repair is performed with continuous cerebral perfusion and includes patch enlargement of the transverse aortic arch and ascending aorta to control for the important diameter mismatch between the aorta and the pulmonary root. The VSD is no longer closed through the pulmonary valve. Instead, the VSD is approached through the aortic valve after harvesting of the coronary buttons and/or through the tricuspid valve and, if necessary, through an infundibulotomy. The coronary artery transfer is often complex because of the presence of unusual coronary artery patterns and requires precise technique. Subaortic obstruction is often present and requires correction. The right ventricle can be mildly hypoplastic but is not a contraindication to repair unless there is important tricuspid stenosis.     

Valved homograft replacement of aneurysmal pulmonary arteries for severely symptomatic absent pulmonary valve syndrome

Background. Patients with absent pulmonary valve syndrome (APVS) with respiratory distress (RD) have previously had a high mortality. In 1990 we adopted a strategy of primary repair including total replacement of the aneurysmal central pulmonary arteries (PAs) for patients with RD.

Methods. Retrospective review was made of 54 consecutive patients with APVS between 1960 and 1998. Median age and weight were 4 months and 4.8 kg. RD was present in 23 patients (10 neonates, 16 required ventilation). Fifteen patients had repair with homograft replacement of the PAs and VSD closure (group 1). Twenty-seven patients had transannular patch with VSD closure with PA-plasty (group 2, n = 21) or without PA plasty (group 3, n = 6). Twelve had miscellaneous procedures (group 4); in 6 the VSD was left open.

Results. Operative, 1-, 5-, and 10-year survivals were 83%, 80%, 78%, and 78%, respectively. Risk factors for operative mortality in multivariate analysis were RD (p = 0.04), neonates (p = 0.02), weight less than 3 kg (p = 0.02), open VSD (p = 0.02) and surgery before 1990 (p = 0.04). Since 1990 operative mortality has decreased to 11% (p = 0.04). RD was the only time-related predictor of survival in multivariate analysis (p = 0.004). In patients with RD, survival with homograft was 73% versus 41% with other techniques (p = 0.2). Mean follow-up was 72 ± 50 months. There were no significant differences in freedom from reintervention rates among the surgical groups (p = 0.08).

Conclusions. Aggressive homograft replacement of the central pulmonary arteries has been associated with improved survival in patients with APVS especially in neonates with severe RD.