Surgical outcomes in the treatment of patients with tetralogy of Fallot and absent pulmonary valve.

OBJECTIVE: Tetralogy of Fallot and absent pulmonary valve (TOF/APV) is associated with significant pulmonary artery dilatation and airway compression. Treatment of infants presenting with respiratory symptoms early in life is associated with high mortality (20-60%). We aim to report our results and identify factors associated with survival and prolonged ventilation. METHODS: We performed a retrospective review of 62 consecutive patients following repair of TOF/APV (1982-2006). Median age at repair was 1.4 years (1 day-35 years). Twenty patients required preoperative intubation. RESULTS: Sixty-one patients underwent complete repair. Thirty-three patients underwent pulmonary artery plication (n=15) or reduction (n=18). The right ventricular outflow tract (RVOT) was reconstructed with valved conduit (n=31), bioprosthetic valve (n=18), monocusp (n=8), or transannular patch (n=4). There were three perioperative and five late deaths. All perioperative deaths were in neonates and before 1995. Five- and ten-year survival was 93+/-4% and 87+/-5%. Mean ventilatory requirements for neonates, infants, and children > or =1 year were 36+/-35, 8+/-8, and 2.6+/-2.4 days (p<0.0001). On multivariable analysis, significant factors associated with prolonged ventilation were neonates (p<0.0001) and preoperative mechanical ventilation (p=0.088). Eight airway reinterventions were needed in seven infants with persistent postoperative airway compromise, pulmonary artery suspension (n=4), innominate artery suspension (n=2), and lobectomy (n=2). Freedom from RVOT reoperation was 89+/-5% and 59+/-9% at 5 and 10 years. There were no significant risk factors for time-related survival or RVOT reoperation on multivariable analysis. CONCLUSIONS: In contrast to children and adults with TOF/APV, neonates and small infants presenting with respiratory symptoms require prolonged ventilation and additional reinterventions for airway compression. Our current surgical approach which includes reduction and suspension of pulmonary arteries, reconstruction of a competent RVOT, and aggressive postoperative ventilatory management to relieve airway obstruction offers satisfactory outcomes.     

Early results of right ventricular-pulmonary artery conduits in patients under 1 year of age.

OBJECTIVES: Management strategies for the repair of many complex heart defects require the implantation of a valved conduit between the right ventricle (RV) and the pulmonary artery (PA), often using aortic or pulmonary homograft valves. Their limited availability, however, has led to the development and use of new conduits. We retrospectively compared our experience with small homografts in patients of less than 1 year of age with the TissueMed bioprosthetic valved conduit. METHODS: From March 1994 to November 1997 29 patients in their first year of life underwent conduit implantation for complex heart defects. These were retrospectively reviewed in order to determine the incidence of death or conduit stenosis. Seventeen patients received homografts and 12 TissueMed conduits. RESULTS: Diagnoses and operative details including conduit size were similar in the two groups and in all cases complete repair of the underlying defect was carried out. Early post-operative mortality was 4/17 (23.5%) in the homograft group and 3/12 (25%) in the TissueMed group. Echo Doppler evaluation within 1 month of operation showed no right ventricular outflow tract (RVOT) obstruction in any of the survivors. In the TissueMed group 8/9 (77%) survivors have gone on to develop significant RVOT obstruction within 12 months of operation. There have been three late deaths in this group all related to severe RVOT obstruction. Two patients died during an attempt at balloon dilatation and one patient died of progressive right heart failure. Five patients had successful replacement of the TissueMed conduit. One child remains well with no evidence of RVOT obstruction. At operation to replace conduit, or at autopsy, the stenoses were related to the deposition of fibrous tissue at the anastomotic suture lines. In the homograft group none of the survivors developed RVOT obstruction during the first 12 months post-operatively. There was one late death (non-cardiac in origin) and one child is awaiting conduit replacement 40 months after initial implantation for obstruction. CONCLUSIONS: The homograft is a satisfactory conduit for re-establishment of RV-PA continuity in infancy. Further work needs to be undertaken in order to elucidate the mechanisms of early graft failure in bioprosthetic conduits if these are to be a suitable alternative for RV outflow reconstruction in infants.     

Results with the freestyle porcine aortic root for right ventricular outflow tract reconstruction in children

BACKGROUND: The ideal choice for valved reconstruction of the right ventricular outflow tract (RVOT) in children is undetermined. This study explores the Freestyle porcine aortic root for these patients. METHODS: From January 1998 to December 2002, 56 patients ages 1.6 to 29.9 years old (mean 11.8 years old) underwent RVOT reconstruction using a Freestyle porcine aortic root. The patients averaged 1.9 prior operations (range 0 to 5) for tetralogy of Fallot +/- pulmonary atresia (28 patients), critical pulmonary stenosis (10 patients), Ross procedure (5 patients), pulmonary atresia/intact ventricular septum (4 patients), complete atrioventricular septal defect +/- tetralogy of Fallot (4 patients), and others (5 patients). At time of RVOT reconstruction, 42 patients (75%) had additional procedures including the following: tricuspid or mitral repair (24 patients), pulmonary arterioplasty +/- Glenn (12 patients), ventricular septal defect closure (5 patients), aortic valve replacement (3 patients), placement of a cardioverter/defibrillator or pacemaker (3 patients), and others (8 patients). RESULTS: One patient developed mediastinitis; another was treated for Candida endocarditis (his excised homograft unexpectedly grew Candida). All patients are well on follow-up from 2 to 60 months (mean 30 +/- 20 months) with no deaths. The patient with endocarditis underwent conduit replacement for recurrent pulmonary stenosis 3.5 years postoperatively. Echocardiography revealed mild or no pulmonary insufficiency in 93%. The calculated mean peak systolic RVOT gradient by echocardiography was 19.7 +/- 15.4 mm Hg. CONCLUSIONS: These data demonstrate excellent results with the Freestyle bioprosthesis for RVOT reconstruction in children. This valve may serve as a readily available alternative to homograft valves in RVOT reconstruction, particularly since early insufficiency seems to be less problematic. Questions of long-term durability and significance of echocardiographic stenosis remain unanswered.     

Outcomes following surgical repair of absent pulmonary valve syndrome: 30 years of experience from a Swedish tertiary referral centre

Open in a separate windowSurgical approach with reduction pulmonary artery plasty and valved conduit in patients with respiratory compromise prior to repair is associated with excellent long-term survival at the cost of a higher reintervention rate. OBJECTIVESAbsent pulmonary valve syndrome is a rare congenital heart defect with pulmonary artery dilatation and secondary airway compression. Although preoperative respiratory support and early surgical repair with pulmonary arterioplasty are often required in patients with airway compromise, the need for extensive plasty in these patients and for plasty in general in those with no or mild respiratory issues remains debatable.METHODSWe performed a retrospective survey of patients with this diagnosis and repair from 1988 to 2018.RESULTSTwenty patients were identified. The median age and weight at repair were 0.8 (0.1–2.4) years and 7.0 (2.5–13.8) kg and included a valved conduit in 17 (85%) patients and a transannular patch in 3 patients. Five (29%) patients were ventilator-dependent prior to repair at the age of 0.3 (0.1–0.4) years. Pulmonary arterioplasty was performed in 7 patients (35%), including all 5 with ventilator dependency and 2 with respiratory symptoms due to recurrent infections. Two patients (10%) with preoperative ventilator dependency underwent extensive intrahilar arterioplasty. Preoperative ventilator dependency was associated with earlier repair and reinterventions (P < 0.05). There were 3 late deaths among cases with repair after 2000 (n = 14), none with preoperative ventilator dependency.CONCLUSIONSThe long-term outcomes of patients with this rare defect are good, comparable to those of other previous studies. Reduction pulmonary arterioplasty, which in this study was used only in patients with respiratory distress and ventilator dependency, is associated with excellent survival. Reinterventions are common in these patients.     

164例法洛四联症的外科治疗

目的总结164例法洛四联症(tetralogyofFallot,TOF)患者手术治疗的经验,探讨手术时机、危险因素和围术期处理等。方法对164例TOF患者行根治手术,其中单纯右心室流出道漏斗部狭窄37例,漏斗部及肺动脉瓣狭窄14例,主肺动脉及左右肺动脉狭窄113例。合并肺动脉闭锁5例。分别给予自体心包片或自体心包卷成心外管道加宽右心室流出道、同种带瓣血管行右心室-肺动脉连接等处理;合并的心脏畸形作相应的矫正。结果全组手术死亡6例,手术死亡率为3.66%(6/164)。死亡原因严重低心排血量综合征2例,冠状动脉移植后不能脱离体外循环机1例,发生室性心律失常1例,术后发生急性呼吸窘迫综合征2例。术后发生心肺并发症55例,均经治疗后恢复。结论行TOF根治术患者年龄应减小,低心排血量综合征已不是导致术后并发症和死亡的主要原因,术中、术后处理的重点应为预防肺动脉残余梗阻和肺部并发症。     

Polytetrafluoroethylene conduits versus homografts for right ventricular outflow tract reconstruction in infants and young children: An institutional experience

Objective

Our institution uses a valved polytetrafluoroethylene conduit as an alternative to homografts. The objective of this study was to investigate the performance of bicuspid valved polytetrafluoroethylene conduits used for right ventricular outflow tract reconstruction in children aged less than 2 years and to evaluate risk factors for earlier conduit explant.

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     

Complete repair of tetralogy of Fallot in the neonate: results in the modern era

OBJECTIVE: To review more than a decade of experience with complete repair of tetralogy of Fallot (TOF) in neonates at the University of Michigan; to assess early and late survival, perioperative complications, and the incidence of reoperation; and to analyze patient, procedural, and morphologic risk factors to determine their effects on outcome. SUMMARY BACKGROUND DATA: Palliation of TOF with systemic-to-pulmonary artery shunts has been the accepted standard for symptomatic neonates and infants. Complete repair has traditionally been reserved for infants older than 6 months of age because of the perception that younger and smaller infants face an unacceptably high surgical risk. RESULTS: A retrospective review from August 1988 to November 1999 consisted of 61 consecutive symptomatic neonates with TOF who underwent complete repair. Thirty-one patients had TOF with pulmonary stenosis, 24 had TOF with pulmonary atresia, and 6 had TOF with nonconfluent pulmonary arteries. The mean age at repair was 16 +/- 13 days, and the mean weight was 3.2 +/- 0.7 kg. Before surgery, 36 patients were receiving an infusion of prostaglandin, 26 were mechanically ventilated, and 11 required inotropic support. Right ventricular outflow tract obstruction was managed with a transannular patch in 49 patients and a right ventricle-to-pulmonary artery conduit in 12. Cardiopulmonary bypass time averaged 71 +/- 26 minutes. Hypothermic circulatory arrest was used in 52 patients (mean 38 +/- 12 minutes). After cardiopulmonary bypass, the average intraoperative right/left ventricular pressure ratio was 55% +/- 13%. There were no new clinically apparent neurologic sequelae after repair. The postoperative intensive care unit stay was 9.1 +/- 8 days, with 6.8 +/- 7 days of mechanical ventilation. There was one hospital death from postoperative necrotizing enterocolitis on postoperative day 71 and four late deaths, only one of which was cardiac-related. Actuarial survival was 93% at 5 years. Follow-up was available for all 60 hospital survivors and averaged 62 months (range 1-141 months). Twenty-two patients required a total of 24 reoperations at an average interval of 26 months after repair. Indications for reoperation included right ventricular outflow tract obstruction (19), branch pulmonary artery stenosis (11), severe pulmonary insufficiency (4), and residual ventricular septal defect (1). The 1-month, 1-year, and 5-year freedom from reoperation rates were 100%, 89%, and 58%, respectively. CONCLUSIONS: Complete repair of TOF in the neonate is associated with excellent intermediate-term survival. Although the reoperation rate is significant, this is to be expected with the complex right ventricular outflow tract and pulmonary artery anatomy seen in symptomatic neonates and the need for conduit replacement in patients with TOF with pulmonary atresia.     

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.     

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)     

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.     

Contegra bovine jugular vein right ventricle to pulmonary artery conduit in Ross procedure

BACKGROUND: In the Ross procedure a valved conduit, most commonly pulmonary or aortic homograft, is used in place of autotransplanted pulmonary valve. Increasing demand and diminishing supply of homografts has resulted in a search for alternatives. A biological conduit from the valved segment of bovine jugular vein (Contegra) has been used successfully as an alternative. METHODS: Early clinical and echocardiographic results were analyzed retrospectively for 20 patients (median age 14.4 years) who underwent a Ross procedure with Contegra as right ventricle to pulmonary artery conduit between November and June 2003 (during the last 31 months). RESULTS: There was no operative mortality and late mortality or morbidity during the mean follow-up of 13.8 +/- 9.1 months (range 1 to 31 months). No patient required reoperation. The median gradient at discharge was 16 +/- 4.5 mm Hg, which remained unchanged at last follow-up. No deterioration in conduit or conduit valve function was noted. CONCLUSIONS: This new bovine jugular vein conduit can be a viable alternative to a homograft in the Ross procedure. The early clinical and hemodynamic results are encouraging. Ease of availability and favorable handling and technical characteristics make it more attractive than a homograft. Xenograft origin of this conduit necessitates close follow-up for assessment of durability and longer-term results.     

A valved conduit replacement for a calcified homograft 12 years following a Rastelli operation: a case report

A 25 year-old male with transposition of great arteries, ventricular septal defect and pulmonary stenosis successfully underwent Rastelli procedure with aortic homograft in 1969. After the procedure he grew up and became an engineer without any complaints. However, gradually his homograft degenerated with calcification and the pressure gradient between pulmonary artery and right ventricle increased to 77 mmHg in systolic phase. Twelve years after the conduit repair, we operated upon and enlarged the ventricular septal defect, repaired the residual interventricular shunt, removed his homograft and, replaced it with the Hancock valved conduit from the right ventricular outflow tract through cardiopulmonary bypass. On postoperative cardiac catheterization, the pressure gradient decreased, and the patient returned to his work.     

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.     

A case of coronary artery-pulmonary artery fistula in tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (MAPCA)]

A case of four year old girl with tetralogy of Fallot, pulmonary atresia, major aortopulmonary collateral arteries and coronary artery to pulmonary artery fistula is reported. The patient was misdiagnosed as truncus arteriosus, pulmonary stenosis and major aortopulmonary collateral arteries (MAPCA) preoperatively. The ligation of MAPCAs was carried out through left thoracotomy as an initial procedure, because we believed that the area fed by MAPCAs had dual blood supply. However, the patient demonstrated pulmonary infarction post operatively. This complication forced us to postpone an intracardiac repair. At the time of intracardiac repair, after mentioned diagnosis was established and a Rastelli type operation and ligation of coronary-pulmonary artery fistula were carried out. A 18 mm Carpentier-Edwards valved conduit was used as an extracardiac conduit. In conclusion: 1) We experienced a rare association of coronary artery fistula in TOF with PA. 2) The MAPCAs functioning as a sole source of blood-supply to the lung should be precisely diagnosed preoperatively and uniforcalized before an intracardiac repair.     

Persistent truncus arteriosus repaired beyond infancy

Introduction

Patients with untreated Persistent Truncus Arteriosus (PTA) usually do not survive or develop irreversible pulmonary vascular obstructive disease beyond infancy. The present study reports the anatomic and hemodynamic data, and results of surgery in patients undergoing surgical repair of PTA beyond 1?year of age.

Patients and Methods

Between January 2000 and March 2012, 9 patients aged 1?year or more underwent complete surgical repair of PTA. The median age was 3?years (range, 1?year to 12?years). Median weight was 9?kg (range, 4.7?kg to 30?kg). Seven patients had type I PTA and two patients had type II PTA. Five patients had mild and two patients had moderate truncal valve regurgitation. The mean pre-operative oxygen saturation was 87?% (SD?±?5.07); mean indexed pulmonary vascular resistance was 9.1 units.m² (range, 4.5 units.m² to 12.3 units.m²). The right ventricular to pulmonary artery continuity was created by aortic homograft (n?=?3), pulmonary homograft (n?=?2) or valved bovine xenograft (n?=?4). In one patient, the ventricular septal defect was closed with unidirectional valved patch. Follow up was complete for all patients.

Results

There were two in-hospital deaths: one each due to sepsis and intractable pulmonary hypertension. Mean follow up duration was 39?months (range, 3?months to 138?months). There were no late deaths. One patient underwent conduit replacement secondary to aneurysmal dilatation of the conduit with clots leading to conduit obstruction 1?month after the initial operation. None of the patients underwent an additional procedure for truncal regurgitation, which at follow up was trivial in 4 and mild in 3 patients. All patients had good biventricular function and pulmonary hypertension subsided in all. All survivors have systemic saturation in excess of 95?%.

Conclusion

A few naturally selected patients with PTA are still suitable candidates for surgical repair after infancy and the early and mid-term outcomes are satisfactory.     

Long-Term Results After Extracardiac Valved Conduits Implanted for Complex Congenital Heart Disease

Between August 1982 and December 1986, 56 patients survived implantation of an extracardiac valved conduit for complex congenital heart disease. The mean age at operation was 4.2 years (16 days to 24 yrs) and the mean weight was 15.9 kg (2.4 to 93.0 kg). The diagnosis was pulmonary atresia (PA) with ventricular septal defect (VSD) in 13 patients, tetralogy of Fallot in 11, transposition of the great arteries (TGA) with VSD in 8, truncus arteriosus, in 7, complex left ventricular outflow tract obstruction (LVOTO) in 6, complex left atrioventricular valve obstruction in 4, double outlet right ventricle with VSD and subaortic obstruction in 3, univentricular heart with pulmonary stenosis in 2, TGA with LVOTO in 1, and PA with intact ventricular septum in 1. In 35 patients, a preclotted conventional Dacron conduit (CDC) with bioprosthetic valve was used, in 19 patients a collagen-sealed Tascon valved conduit (TC) was implanted, and in 1 patient an aortic homograft was used. In a mean follow-up of 32.5 months (9 to 64 mo), there were two deaths (2/56, 3.6%) that were not related to the conduit. All survivors have been evaluated by two-dimensional and Doppler echocardiography, and 29/56 (51.8%) underwent cardiac catheterization. Nine patients (9/56, 16.1%) underwent successful valved conduit replacement, in seven cases with a nonvalved conduit. There was a significant difference (P = .011) with regard to the incidence of conduit replacement between the group with CDC (2/36, 5.5%) and the group with TC (7/19, 36.8%). Five patients underwent percutaneous transluminal balloon dilatation of the prosthetic conduit, with adequate relief of the gradient in four patients.(ABSTRACT TRUNCATED AT 250 WORDS)     

Extracardiac valved conduits in the pulmonary circuit

Extracardiac valved conduits represent one of the weakest facets of reconstructive surgery for congenital heart disease in that they invariably need to be replaced because of growth of the patient or because of valve or conduit failure. Between 1979 and 1989, 141 patients had 169 valved conduits placed between the heart and the pulmonary artery circuit. There were 81 male and 60 female patients, aged 2 days to 35 years (mean age, 5.9 years), with 46 patients less than 1 year of age. We performed primary repair in 117 patients; in this group, there have been 28 conduit replacements in 27 patients. In 17 patients initial repair with a conduit was performed elsewhere and we replaced these conduits in 15 and removed them in 2. A further group of 9 patients were seen after repair of tetralogy of Fallot or double-outlet right ventricle, with severe pulmonary incompetence or right ventricular outflow tract aneurysm. All had valved conduits inserted as secondary procedures. The types of valved conduits used were xenograft (n = 126) and homograft (n = 43). There were six hospital deaths (3.6%; 70% confidence limits [CL], 2% to 6%) and seven late deaths (4.1%; CL, 2.5% to 6.5%) in a total of 169 conduit insertions. Forty-five conduits have been removed and 43 reinserted without early or late mortality (0%; CL, 0% to 4%). Actuarial survival after conduit insertion was 87% at 5 years (CL, 80% to 92%), including operative mortality. Actuarial freedom from conduit replacement was 37% at 5 years (CL, 20% to 56%). Conduit insertion in infants and small children ensures subsequent replacement, but this can be done at low risk.(ABSTRACT TRUNCATED AT 250 WORDS)     

Current strategy of repair of tetralogy of Fallot in children and adults: emphasis on a new technique to create a monocusp-patch for reconstruction of the right ventricular outflow tract

Abstract Objectives: In older children (>four years) and adults the strategy of repair of tetralogy of Fallot (TOF) should have a low rate of transannular patch to avoid pulmonary insufficiency (PI), and in addition, we developed a new method of reconstruction of the right ventricular outflow tract (RVOT) to reduce PI. Methods: From 2001 through 2005, 74 patients (50 male, 24 female; mean age of 13.6 ± 0.8 years, ranging from four to 34 years) with TOF (67) or double outlet RV (DORV)‐type‐TOF (seven) underwent complete repair. The resection of RVOT stenosis ± pulmonary valvotomy was principally through RA. A new two‐patch technique to create a folded mono‐cusp valve by using autologous pericardium was applied since August 2004. Results: The repair was through RA in 52 patients and through RA + RVOT/PA in 22 patients (18/4). TAPR was performed in 17 patients (23%, eight non‐valved and nine valved). The aortic cross‐clamp time was 130.1 ± 6.8 min in TAPR group and 85.8 ± 4.6 min in non‐TAPR group (p < 0.0001). The operative mortality was 4.0% (3/74) in all and 2.9% (2/67) in TOF patients (due to low output, uncontrollable bleeding, and repeated bleeding from the pulmonary collateral vessels late) and was similar in TAPR or non‐TAPR. The valved patch‐repaired patients had mild PI and good RV function postoperatively up to eight to 12 months. Conclusions: Repair of TOF in older children/adults should include low rate of TAPR of RVOT. If indicated, the new folded monocusp‐patch technique is recommended. It is an effective and simple way to markedly reduce postoperative PI with no additional cost.     

Surgery for right ventricle to pulmonary artery conduit obstruction: risk factors for further reoperation.

OBJECTIVE: To identify the surgical approaches and risk factors which influence longevity of right ventricle to pulmonary artery (RV-PA) conduits following first reoperation for obstruction. METHODS: Between January 1993 and August 2003, 114 patients underwent 141 reoperations for RV-PA conduit obstruction. Diagnoses included 'Truncus Arteriosus' (n=52), 'Pulmonary atresia/Tetralogy of fallot' (n=39), 'Double outlet right ventricle' (n=10), 'Transposition of great arteries, VSD, and pulmonary atresia' (n=9), and the 'Ross operation' (n=4). All patients had undergone a previous biventricular repair. The first reoperation for conduit obstruction was performed in 112 hospital survivors by: total conduit replacement (Group A, n=73) with valved (homograft=10 and xenograft=54) or non-valved (n=9) conduit, and patch enlargement of the obstructed RV outflow tract with preservation of the posterior and sides of the conduit wall after removing of the fibrocalcific peel and degenerated valve (Group B, n=39). Mean age at first reoperation was 8.8+/-6.7 and 7.5+/-5.3 years in patients of groups A and B, respectively. Seven patients in Group A and 18 in Group B required a second reoperation and two patients in Group B a third reoperation. RESULTS: There were two hospital deaths and no late deaths. Mean follow-up was 5.8+/-3.2 years. Risk factors for second reoperation by univariate analysis were: homograft conduit use (P=0.004), Group B surgical approach (P=0.0001), higher RV-PA systolic pressure gradient at discharge (P=0.02), and age <5-years-old (P=0.01). Multivariate analysis showed that inclusion in Group B and younger age (<5-years-old) at repair were independent risk factors for second reoperation. Group B surgical approaches had higher RV-PA systolic pressure gradient at discharge (P=0.02) and required more PA bifurcation repair at the time of second reoperation (P=0.05). Freedom from second reoperation for conduit obstruction was significantly higher in Group A patients at 5 and 8 years (P<0.04) and those with xenografts rather than homograft (P=0.04). CONCLUSIONS: Our results support the optimal surgical approach for RV-PA conduit obstruction is total replacement with a xenograft. RV outflow reconstruction by other techniques without complete dissection of PA bifurcation does not completely relieve the stenosis and could cause early restenosis. Higher systolic gradients at discharge and younger age at first reoperation are predictors of earlier reoperation.