心脏外科手术与介入性心导管术镶嵌治疗小儿先天性心脏病

目的 报道23例介入性心导管术与心脏外科手术镶嵌治疗小儿先天性心脏病(CHD)的经验。方法外科术后镶嵌治疗：12例动脉导管未闭结扎术后残余分流及2例心脏术后留置室间隔缺损(VSD)的复杂CHD行经导管封堵术。2例法洛四联症(TOF)根治术后分支肺动脉狭窄分别行球囊血管成形术及支架置入术，1例肺动脉闭锁合并VSD根治术后出现体肺侧支血管(APCAs)破裂行介入栓塞止血术。外科术前介入治疗：5例室间隔完整的大血管转位(TCA)病例行大动脉调转术前行球囊房隔造口术(BAS)，1例有巨大APCAs的重症。TOF在根治术前予以侧支血管堵塞术。结果动脉导管未闭术后残余分流及复杂CHD残留VSD病例均封堵成功，分支肺动脉狭窄病例介入治疗后狭窄减轻或治愈；侧支血管破裂病例栓塞止血后出血停止。BAS病例介入术后低氧及酸中毒改善，大动脉调转术后3例治愈，2例死亡。TOF病例APCAs堵塞后成功行根治术。结论心脏外科手术与介入性心导管术镶嵌治疗是治疗小儿CHD术后残余分流及部分复杂疑难CHD的有效方法。     

Intraoperative stents to rehabilitate severely stenotic pulmonary vessels

BACKGROUND: Patch enlargement of severe branch pulmonary artery stenosis (PAS) or pulmonary vein ostial stenosis (PVS) can be technically challenging. Recurrences are common and exposure may require long periods of cardiopulmonary bypass (CPB). METHODS: Since 1993, we performed 31 procedures on 27 patients with endovascular stents placed intraoperatively under direct surgical vision: 22 patients with tight PAS and 5 patients with PVS. Selection for intraoperative (vs catheterization laboratory) stent placement was prompted by: (1) the need for a concomitant cardiac surgical procedure (16 cases); (2) limited vascular access for catheterization laboratory stent placement (11 cases); or (3) "rescue" of patients with complications after attempted placement of stents (4 cases). RESULTS: In this group of very complex and challenging patients there were 5 hospital deaths (hospital survival, 81%). Follow-up of survivors has ranged from 1 month to 7 years (mean 2.8 +/- 1.7 years). There have been 3 late deaths (late "series" survival, 70%). No complication or death was related to stent placement. Surviving patients have had significant clinical improvement. Mean pulmonary gradient (postoperative vs preoperative echo) has fallen in all survivors and has decreased from a mean of 66 mm Hg preoperatively to 28 mm Hg postoperatively (p = 0.01). All pulmonary arteries are appreciably enlarged and will be easier to deal with at a later date if necessary. One patient (DORV, HLHS ) with pulmonary vein stents has gone on to a successful Glenn procedure. The other two surviving patients with PV stents have occlusion of their proximal PVs on follow-up catheterization; thus only 1 of 5 patients with stents for PVS has had a successful outcome. Four patients have had repeat surgery. Stents have produced no impediment to subsequent surgical procedures, and the pulmonary arteries were easy to work with. CONCLUSIONS: Intraoperative stenting provides an attractive option for "rehabilitation" of pulmonary vessels. Direct vision insertion on CPB is extremely quick and immediately effective, limiting the CPB exposure required to treat this problem. Once stented, vessels remain open and are amenable to future surgical intervention as necessary. Outcome is better for patients with PAS versus those with PVS.     

Bronchial compression due to stent placement in pulmonary artery in a child with congenital heart disease

Congenital heart disease, such as transposition of the great vessels (TGV), requires surgical procedures which can lead to important complications. We report on a case of bronchial obstruction following placement of a pulmonary artery stent in a 4-year-old boy who had undergone a Rastelli procedure to correct TGV, ventricular septal defect and pulmonary stenosis. There are many complications that can arise as a consequence of intravascular stents in heart surgery, as well as many causes of bronchial compression. However we have not found any report which describes bronchial compression as a direct consequence of endovascular stent.     

Hybrid intra-operative pulmonary artery stenting in congenital heart disease

Objective

Percutaneous branch Pulmonary Artery (PA) stenting can be challenging, especially when associated with stenosis of the Right Ventricular Outflow Tract (RVOT), after previous angioplasty or with complexity of PAs branches. Surgical patch enlargement of Pas alone can be disappointing in these situations. A hybrid-approach, with PA stenting during surgery, may be an alternative to optimize surgical management in complex pulmonary artery stenosis.

Methods

In a 2 year period, 14 consecutive patients with PA stenosis associated with congenital heart disease underwent intraoperative PA stenting. We retrospectively analyzed clinical data, procedural details and outcomes of all patients.

Results

14 patients (1 female), underwent hybrid stenting of the PA. The median age was 6.9 years (range 6 days to 28 years). Primary cardiac diagnoses were pulmonary atresia (n?=?7), tetralogy of Fallot (n?=?5) and other (n?=?2). The left PA was stented in 4 patients, the right in 1 patient and both in 8 patients, for a total of 21 stents. The median maximal balloon diameter was 12 mm (range 6 to 25 mm). Concomitant surgical procedures were RVOT reconstruction with PA patch angioplasty (n?=?8), pulmonary valve replacement (n?=?3) and other (n?=?3). Active suture fixation of the proximal stent was performed in each case. No complications occurred during stent implantation, and no significant cardiac complications occurred perioperatively. The median length of stay in the intensive care unit was 2 days (range 2 to 7 days). The mean follow-up was 9.8 months (range 1 to 19 months). Echocardiography at discharge showed good results in PA branches: the mean peak gradient was 3.8 mmHg (range 0 to 14 mmHg). Most patients showed significant fall in right ventricular systolic pressure after the procedure. No stent needed repeat dilatation.

Conclusion

Hybrid PA stenting during surgery for congenital heart disease in cases of complex branch PA stenosis is an alternative to traditional angioplasty. The procedure is safe and effective, and ameliorates right-ventricular dysfunction.

     

杂交(hybrid)手术在复杂性先天性心脏病治疗中的初步应用

目的探讨结合介入器械和实时影像学的术中"杂交"(hybrid)手术对复杂性先天性心脏病的治疗价值.方法2005年3月～10月,我院行hybrid手术治疗7例复杂性先天性心脏病.球囊扩张组3例均为室间隔完整型肺动脉闭锁(PAIVS)的新生儿.缺损封堵组4例,其中1例右冠状动脉异常起源于肺动脉合并房间隔缺损,1例右侧肺静脉异位引流合并房间隔缺损(ASD),2例多发室间隔缺损(VSD).正中进胸,在超声引导下经右室流出道置入球囊扩张管扩张肺动脉瓣或经右心房植入封堵器.多发室间隔缺损于体外循环下经三尖瓣植入封堵器.合并的其他心脏病变同期常规外科矫正.术后心脏超声随访.结果7例均顺利出院,无一例手术死亡.3例PAIVS术后中位住院时间10 d,余4例均在术后7 d出院.随访1～6个月,7例生长发育良好,人工体肺分流管通畅,未发现中度以上的肺动脉瓣再狭窄,ASD和多发VSD术后均未发现明显残余分流及封堵器移位,均无影响瓣膜功能等并发症发生.结论hybrid手术可以不采用体外循环,减少手术创伤,对于提高复杂性先天性心脏病的疗效具有重要的意义.     

Aortic translocation for the management of transposition of the great arteries with a ventricular septal defect, pulmonary stenosis, and hypoplasia of the right ventricle.

A 2-month-old patient with transposition of the great arteries, a ventricular septal defect, pulmonary stenosis, and severe hypoplasia of the right ventricle successfully underwent a biventricular repair utilizing the aortic translocation technique. Advantages of this surgical repair over the Rastelli procedure in the management of this complex congenital heart lesion are discussed.     

Surgical approach to critical pulmonary valve stenosis in infants less than six months of age

During the past 7 1/2 years, 36 infants (less than or equal to 6 months of age) underwent surgical relief of critical pulmonary valve stenosis. The results of closed and open (cardiopulmonary bypass, CPB) procedures were compared. Closed procedures included Brock valvotomy (two patients) and normothermic inflow stasis with transpulmonary valvectomy (10 patients). Twenty-two patients had open procedures with valvectomy alone (five patients) and right ventricular outflow tract (RVOT) reconstruction (17 patients). Two additional patients had staged operations (shunt followed by open procedure at 3 and 5 months postoperatively). Patients who had the closed procedures, as compared with CPB, were significantly younger (10 versus 61 days, p less than 0.01) and more critically ill with congestive heart failure and acidosis. In addition, the mean peak systolic right ventricular-left ventricular pressure ratios (RVP/LVP) were higher (1.6 versus 1.4, p less than 0.02) in the closed group. There were no deaths in the closed group in contrast to six deaths among those having RVOT reconstruction (0/12 versus 6/17 or 35%, p less than 0.04). Critical obstruction of the pulmonary valve, tricuspid valve regurgitation, and hypoplastia of the right ventricle occurred more frequently in the closed group. All operative deaths resulted from right heart failure. There were no late deaths in the entire series. Three patients in the closed group (mean follow-up 2.8 years) required operations for recurrent pulmonary valve stenosis approximately 2 years postoperatively, and none in the open group (mean follow-up 2.0 years). In conclusion, despite current advances in infant CPB and care, the results from our experience suggests that closed procedures, especially inflow stasis and pulmonary valvectomy, are superior to the use of CPB and RVOT reconstruction, even in the presence of pulmonary valve dysplasia.     

Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum

BACKGROUND: Since 1991 we have performed a multistage palliative approach to biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum in infants with a detectable right ventricular infundibulum. METHODS: A total of 25 patients (19 pulmonary atresia and 6 critical pulmonary stenosis) underwent initial palliation consisting of a transarterial pulmonary valvotomy and a polytetrafluoroethylene shunt between the left subclavian artery and pulmonary trunk. Among the 23 survivors, 15 underwent balloon valvotomy. Six of these patients later required additional palliative surgery that consisted of repeat pulmonary valvotomy, adjustment of an atrial communication, and resection of the hypertrophied muscles in the right ventricle. RESULTS: Of the 25 patients, 23 (92%) survived. In all, 20 patients underwent definitive operations: 18 (90%) biventricular repair (12 pulmonary atresia, and 6 critical pulmonary stenosis), one bidirectional Glenn, and one Fontan procedure. The actuarial probability of achieving a biventricular repair at 36 months of age was 69%. In 18 patients right ventricular end-diastolic volume significantly increased but tricuspid valve diameter did not change. CONCLUSIONS: The multistage palliation procedure to promote right ventricular growth makes a definitive biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum possible in the majority of infants with a patent infundibulum.     

Management of neonatal critical pulmonic stenosis in the balloon valvotomy era

We evaluated our recent experience with management of neonatal critical pulmonic stenosis and intact ventricular septum between 1982 and 1988. Thirty-nine patients (aged less than 3 months) were treated initially by operation (group A, n = 19) or with balloon pulmonary valvotomy (group B, n = 20). Patients in group A were younger (5 +/- 1.3 versus 18 +/- 4 days in group B) (mean +/- standard error of the mean) and had a greater degree of hypoxia (oxygen tension, 55 +/- 4 versus 80 +/- 6 mm Hg) (p less than 0.05 for all variables). Ten patients in group A and 8 patients in group B had right ventricular hypoplasia, based on an angiographically determined index. Balloon pulmonary valvotomy was attempted in 20 patients at the time of the initial catheterization but was unsuccessful in 9 owing to inability to catheterize the hypoplastic right ventricular outflow tract (n = 8) and to recurrent infundibular stenosis (n = 1). Patients with failed balloon valvotomy were subsequently operated on within 24 hours. The early operative mortality (less than 30 days) was 25% (7 of 28); one death (9%) occurred after successful balloon valvotomy owing to associated critical aortic stenosis. The early postoperative gradient was 20 +/- 2 mm Hg; the post-balloon valvotomy gradient was 18 +/- 3 mm Hg. We conclude that balloon pulmonary valvotomy yields good results in patients with critical pulmonary stenosis with essentially normal-sized right ventricle, whereas surgical pulmonary valvotomy is required for patients with right ventricular hypoplasia.     

分期全腔静脉-肺动脉连接术治疗复杂先天性心脏病

目的 总结分期全腔静脉.肺动脉连接术(TCPC)治疗复杂先天性心脏病的经验.方法 1998年6月至2008年3月,22例先天性心脏病复杂畸形患者接受分期TCPC.本组中单心室合并肺动脉狄窄9例,合并肺动脉闭锁3例;完全性大动脉转位,十字交叉心,肺动脉瓣狭窄1例;完全性房室通道,左心窒发育不良,肺动脉闭锁,房室瓣少-中量反流1例;完全性房室通道,动脉导管未闭,肺动脉狭窄,双向Glenn术后伴吻合口狭窄房室瓣大量反流1例;镜面右位心,功能性单心室,肺动脉闭锁,动脉导管未闭,体肺侧支,双侧双向Glenn术后2年,右下肺动-静脉瘘1例;三尖瓣闭锁并肺动脉狭窄4例;三尖瓣闭锁伴肺动脉闭锁,动脉导管未闭1例;镜面右位心,右心室双出口,窒间隔缺损,左心室发育小良,功能性单心房,肺动脉瓣狭窄,三尖瓣少量反流,粗大体肺侧支1例.第一期体肺分流术后,二期双向Glenn术5例,第一期单侧或双侧双向Glenn手术17例.行双向Glenn手术的年龄为(5.9±4.4)岁,Glenn术前肺动脉压为17～20 mm Hg(1 mm Hg=0.133 kPa);房室瓣膜关闭不全3例;一期手术前Nakata指数小于200 mm2/m2者4例.接受TCPC术平均年龄为(9.6±4.9)岁,与双向Glenn术间隔时间(3.7 ±1.2)年,术前经上腔静脉置管测得肺动脉压力均小于15 mm Hg.全组22例均采用心外管道TCPC,房窜瓣成形1例.结果 全组住院死亡1例,病死率为4.5%.死亡病例为单心室,肺动脉闭锁,第一期体肺分流术后,二期左肺动脉成形双向Glenn术后5年第三期行TCPC,术后左肺反复多次大出血死亡.余患者均顺利出院,术后中心静脉压12～18 mm Hg.出院前经皮测血氧饱和度为90%～96%,活动量明显增加,发绀及气促症状消失,心功能均为NYHA分级Ⅰ～Ⅱ级.结论 对于高危患者根据病情选择分期TCPC,可以使更多有高危因素不能行Fontan手术的患者得到救治的机会,扩大手术适应证,并取得满意疗效.     

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.     

Options for surgical repair in hearts with univentricular atrioventricular connection and subaortic stenosis.

Thirteen patients have undergone surgical treatment because of subaortic obstruction in hearts with a univentricular atrioventricular connection. Nine patients underwent surgical enlargement of the ventricular septal defect and four patients had construction of an aortopulmonary anastomosis and closure of the pulmonary trunk (the Damus-Kaye-Stansel procedure). Two patients undergoing enlargement of the septal defect and two having the Damus-Kaye-Stansel procedure also had a modified Fontan procedure. One patient had complete atrioventricular dissociation after direct enlargement of the ventricular septal defect, which necessitated insertion of an epicardial pacemaker. One patient died within 30 days of the operation after enlargement of the defect and two patients after the Damus-Kaye-Stansel procedure. There was one late death, occurring in a patient who underwent enlargement of the ventricular septal defect. Ten patients have subsequently undergone conventional cardiac catheterization and angiography or transcutaneous Doppler flow studies to assess the relief of the subaortic obstruction. The result has been satisfactory in all. Because of this experience, we now recommend direct surgical enlargement of the restrictive ventricular septal defect for direct relief of subaortic stenosis occurring with a univentricular atrioventricular connection to a dominant left ventricle, inasmuch as it appears to be hemodynamically effective with a low operative mortality and morbidity. The Damus-Kaye-Stansel procedure can also have a role in relieving subaortic stenosis when the atria are connected to a dominant right ventricle.     

Pure pulmonary atresia complicated by major sinusoidal-coronary artery communication--a case report

The current treatment of pure pulmonary atresia consists of early initiation of prostaglandins followed by a combination of surgical procedures according to the right ventricular morphology. Major right ventricular sinusoidal-coronary artery communications (SCAC) occasionally present in patients with hypoplastic right ventricle may remain as coronary artery-right ventricular fistulae after right ventricular decompression, which may in turn be the cause for poor surgical results after pulmonary valvotomy in neonates with the SCAC. Herein, a case of this anomaly is reported with a review of literature. A 2-month-old infant underwent an aorto-pulmonary artery shunt operation using Gore-Tex graft for an emergency treatment of pure pulmonary atresia with major SCAC. The catheterization and ventriculography performed 6 months later disclosed suprasystemic right ventricular pressure and more extended SCAC. Then, right ventricular outflow tract was constructed utilizing Gore-Tex patch across the pulmonary valve under cardiopulmonary bypass. The aortico-pulmonary artery shunt was left functioning. After another 6 months, cardiac catheterization and ventriculography were repeated. Right ventricular pressure decreased to the systemic level, right ventricular ejection fraction improved, and the SCAC was not visualized by ventriculography. The decreased inter-ventricular pressure difference and improved right ventricular emptying after the second operation may have played a role in the disappearance of the SCAC. For the neonates with pure pulmonary atresia and sinusoidal-coronary artery communications, we still advocate systemic-pulmonary shunt procedure with concomitant or slightly delayed pulmonary valvotomy as the initial procedure. Unless the tricuspid valve is critically small or stenotic, there may still be a chance for the growth of the right ventricle even in this type of case.     

Supravalvular pulmonary stenosis 9 years after surgically created pleuro‐pericardial window

Supravalvular pulmonary stenosis acquired as a postoperative cardiac procedure complication is relatively common. However, that occurring after a surgically created pleuro‐pericardial window has not been described until now, to the best of our knowledge. We present a case of acquired supravalvular pulmonary stenosis that developed 9 years after the pleuro‐pericardial window creation for pyopericardium due to a constricting pericardial band. The child underwent successful surgical relief of the stenosis along with repair of the atrial and ventricular septal defects.     

Perioperative complications in children with pulmonary hypertension undergoing general anesthesia with ketamine

Background: Pulmonary arterial hypertension (PAH) is associated with significant perioperative risk for major complications in children, including pulmonary hypertensive crisis and cardiac arrest. Uncertainty remains about the safety of ketamine anesthesia in this patient population. Aim: Retrospectively review the medical records of children with PAH to ascertain the nature and frequency of peri‐procedural complications and to determine whether ketamine administration was associated with peri‐procedural complications. Methods: Children with PAH (mean pulmonary artery pressure ≥25 mmHg and pulmonary vascular resistance index ≥3 Wood units) who underwent general anesthesia for procedures during a 6‐year period (2002–2008) were enrolled. Details about the patient, PAH, procedure, anesthetic and postprocedural course were noted, including adverse events during or within 48 h of the procedure. Complication rates were reported per procedure. Association between ketamine and peri‐procedural complications was tested. Results: Sixty‐eight children (median age 7.3 year, median weight 22 kg) underwent 192 procedures. Severity of PAH was mild (23%), moderate (37%), and severe (40%). Procedures undertaken were major surgery (n = 20), minor surgery (n = 27), cardiac catheterization (n = 128) and nonsurgical procedures (n = 17). Ketamine was administered during 149 procedures. Twenty minor and nine major complications were noted. Incidence of cardiac arrest was 0.78% for cardiac catheterization procedures, 10% for major surgical procedures and 1.6% for all procedures. There was no procedure‐related mortality. Ketamine administration was not associated with increased complications. Conclusions: Ketamine appears to be a safe anesthetic option for children with PAH. We report rates for cardiopulmonary resuscitation and mortality that are more favorable than those previously reported.     

The recognition, identification of morphologic substrate, and treatment of subaortic stenosis after a Fontan operation. An analysis of twelve patients.

Twelve children were identified with subaortic stenosis after Fontan's operation. All had absent resting and isoproterenol-provoked pressure gradient before the Fontan procedure. Six had a univentricular heart of left ventricular morphology, three had a single ventricle of right ventricular morphology, one had tricuspid atresia with transposition of the great arteries, one had pulmonary atresia, intact ventricular septum, and hypoplastic right ventricle, and one had corrected transposition with hypoplastic systemic ventricle. The median interval between the Fontan operation and the recognition of subaortic stenosis was 2.5 years. Ten patients underwent surgical treatment after a prior Fontan operation: Five had myectomy and enlargement of ventricular septal defect with two operative deaths; two had placement of a valved conduit from the ventricular apex to the descending aorta, and both died postoperatively; two with single ventricle had subaortic myectomy, and one had enlargement of ventricular septal defect and pulmonary aortic connection. Complete heart block developed in only one patient. Postoperative testing with Doppler echocardiography with color flow imaging demonstrated good relief of subaortic stenosis. All six children who survived the operation are well 4 months to 4 years later. Subaortic stenosis is a progressive lesion that may develop after a Fontan operation. Its surgical treatment continues to carry a significant mortality. Myectomy and enlargement of ventricular septal defect achieve direct relief of the obstruction with minimal risk of heart block.     

Assessment of residual right ventricular outflow tract obstruction following surgery using the response to intravenous propranolol

The response to intravenous administration of propranolol hydrochloride was studied in 24 children undergoing operation for isolated or complex right ventricular outflow tract obstruction. Ten had pulmonary valve stenosis, 4 had pulmonary valve stenosis plus ventricular septal defect, 8 had tetralogy of Fallot, and 2 had complex lesions. Propranolol (0.01 to 0.2 mg per kilogram of body weight) was given to patients with a residual right ventricular to left ventricular systolic pressure ratio greater than 0.75. Four children did not respond to propranolol and required further surgical intervention. Twenty patients responded, 2 of whom died. Fifteen were restudied by cardiac catheterization 3 weeks to 27 months postoperatively. The right ventricle-pulmonary artery gradient was 25 mm Hg or less in 13 patients. We conclude that a small dose of propranolol given intraoperatively can identify patients in whom a reduction in the right ventricle-pulmonary artery gradient to acceptable levels will occur in the months following operation. This may reduce the need for placement of a subannular or transannular patch in some patients.     

A rare case of quadruple valve surgery with bioprosthetic pulmonary valve replacement in an octogenarian for degenerative valvular disease: Technical aspects

Quadruple valve surgery represents a complex and rare surgical procedure, requiring a systematic and standardized approach. In this intervention, pulmonary valve replacement (PVR) in patients without pre‐existing congenital heart disease represents a challenge and no large data have been reported yet. We hereby report a case of bioprosthetic PVR in an octogenarian within a quadruple valve procedure, illustrating our preferred technique of PVR with bioprosthetic implantation and right ventricular outflow tract reconstruction.     

The management of pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries by definitive single stage repair in early infancy.

OBJECTIVE: The management of infants and children with pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries (PA/VSD/MAPCA) has proven to be challenging. Therapeutic approaches have included staged unifocalization, shunting, coiling of collateral vessels, and heart/lung transplantation. Results have been variable and frustrating. Hoping to take advantage of growth potential in pulmonary segments supplied by MAPCA, a more radical approach was adopted in March of 1997. This consists of single stage complete unifocalization with closure of the ventricular septal defect and establishment of right ventricular to pulmonary arterial continuity with a cryopreserved pulmonary allograft (Rastelli type correction) through a midline sternal incision. METHODS: During an 18-month period, eleven consecutive infants with PA/VSD/MAPCA underwent complete surgical correction. The ages ranged from 5 days to 5 months. Weights ranged from 2.2 to 5.6 kg. Through a standard median sternotomy incision, the pericardium and both pleural spaces were opened. Normothermic cardiopulmonary bypass was instituted. Section of all collaterals was accomplished without hypoxemia, and all collaterals were ligated at their origin from the aorta. They were then brought through posterior mediastinum to construct a pulmonary artery confluence. The ventricular septal defect was closed, and continuity was established between the right ventricle and the newly created pulmonary artery confluence with cryopreserved allografts. RESULTS: Ten of 11 patients survived operation, with postoperative courses that were uncomplicated. Length of stay ranged from 7-16 days, with a median length of stay of 11 days. One perioperative death occurred in a patient with preoperative co-morbidities of necrotizing enterocolitis, with no functioning gastrointestinal tract, intraventricular hemorrhage, and ventilator dependency since birth. At angiography, this patient has no demonstrable central pulmonary arteries and multiple diminutive aorta pulmonary collaterals. Autopsy revealed no demonstrable pulmonary arteries within the pulmonary parenchyma. All patients have been followed closely, and have grown normally. Two patients undergone repeat cardiac catheterization because of the echocardiographic demonstration of right ventricle pressures that had exceeded 50% of systemic. Both patients were treated with balloon angioplasty and one of these patients has had stenting of stenotic pulmonary arterial segments. No other patients have required additional hospitalization. Right ventricular pressures have remained less than fifty percent of systemic by echocardiographic assessment in all other patients. CONCLUSIONS: We feel that a single stage correction of pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries can be accomplished in early infancy with acceptable morbidity and mortality. The initiation of normothermic cardio-pulmonary bypass greatly facilitates dissection of collaterals and prevents hypoxemia. Interventional cardiology with balloon angioplasty and stenting of abnormal pulmonary arterial segments is both an important essential adjunct in the management of these patients. We remain optimistic that a single stage approach to this complex lesion coupled with cardiac catheterization, balloon angioplasty, and stenting, will provide long-term results superior to staged approaches.