Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries

OBJECTIVE: To assess the results of a staged surgical approach for tetralogy of Fallot with pulmonary atresia, hypoplastic or absent pulmonary arteries, and major aortopulmonary collateral arteries. METHODS: We retrospectively reviewed a consecutive series of these patients from a single institution. RESULTS: From July 1993 to April 2001, 46 consecutive patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries were treated with staged surgical repair. The operative sequence usually began with a central aortopulmonary shunt followed by unifocalization of aortopulmonary collateral arteries depending on the source and distribution of pulmonary blood flow. Twenty-eight patients (61%) subsequently underwent complete repair with ventricular septal defect closure and right ventricle to pulmonary artery connection. Those patients who underwent complete repair had a median of 3 total operations (range 1-6). The ratio of the mean pulmonary artery pressure to the mean systemic blood pressure at the time of complete repair was 0.36 (range 0.19-0.58). Two of the 28 repaired patients (7.1%) required subsequent fenestration of the ventricular septal defect closure due to later development of supersystemic right ventricular pressure and right ventricular failure. Eighteen patients (39%) have undergone 1 or more staging operations and are considered good candidates for eventual complete repair. There were no hospital deaths. There was 1 late death (2.2%; 95% CI 0.4-11.3%) in a patient born prematurely who developed severe bronchopulmonary dysplasia precluding complete repair. CONCLUSIONS: For tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries, a staged surgical approach yields low overall mortality and acceptable hemodynamics after complete repair.     

Prediction of severe obstruction to right ventricular outflow after repair of tetralogy of Fallot and pulmonary atresia

Cineangiograms were available for a quantitative retrospective study along with complete clinical information in 96 patients who underwent intracardiac repair of tetralogy of Fallot with pulmonary atresia. Multivariate analysis determined that the risk factors for too high a ratio (greater than or equal to 1) between the peak pressure in the right ventricle and that in the left, in the operating room about 30 minutes after repair, were as follows: size of the patient, small size of the right and left pulmonary arteries, and a larger number of large aortopulmonary collateral arteries. When, according to the multivariate equation, the predicted probability of this ratio being equal to or greater than 1 is 50% or more, consideration may be given to preliminary operations before repair; when the predicted probability is 70% or more, complete repair at that stage may be unwise.     

Early and long-term results of the surgical treatment of tetralogy of Fallot with pulmonary atresia,with or without major aortopulmonary collateral arteries

OBJECTIVE: We sought to determine the results of surgical treatment of patients with tetralogy of Fallot and pulmonary atresia with or without major aortopulmonary collateral arteries, to clarify variables affecting early and late mortality, and to expose late, nonfatal events affecting surgical patients. METHODS: The records of 495 patients operated on from 1977 to 1999 were reviewed. Patients were separated into those who did not undergo complete repair (group A) and those who did (group B). RESULTS: Group A consisted of 160 patients. Eighty-one (51%) had palliative procedures, 45 (28%) had preliminary surgical stages (unifocalization and right ventricular outflow tract reconstruction) as initial operations, and 34 (21%) had all surgical stages but were rejected for complete repair. Early and late mortality were 16.3% (n = 26) and 23.1% (n = 31), respectively. Mean follow-up was 72.3 months. The presence of major aortopulmonary collateral arteries was a risk factor for late mortality (P =.0182). Group B consisted of 335 patients. Mean age at complete repair was 11.3 years (SD, 9.2). One hundred three (30%) patients had single-stage complete repair, whereas 232 (69%) had staged reconstruction. Twenty-two (6.6%) patients underwent reopening of the ventricular septal defect for high right ventricular pressure. Early and late mortality were 4.5% (n = 15). Risk factors were a peak right ventricular/left ventricular pressure ratio of greater than 0.7 and reopening of the ventricular septal defect (P < or = .05). Late mortality was 16% (n = 51). Mean follow-up was 11.4 years (SD, 7.5). Risk factors included male sex, nonconfluent central pulmonary arteries, reopening of the ventricular septal defect, and postrepair conduit exchange (n = 137). Ten- and 20-year results were an actuarial survival of 86% and 75% and freedom from reoperation of 55% and 29%, respectively. CONCLUSIONS: Surgical repair of patients with simple or complex forms of tetralogy of Fallot with pulmonary atresia can be achieved with low early mortality. Late mortality and need for reoperation, especially conduit replacement, continue to affect the long-term well-being of these patients.     

Management of large aortopulmonary collateral arteries in patients with ventricular septal defect and pulmonary atresia: simultaneous ligation through median sternotomy during intracardiac repair

Nine consecutive patients with ventricular septal defect (VSD) and pulmonary atresia associated with large aortopulmonary collateral arteries underwent primary repair with simultaneous ligation of these collateral arteries. The patients ranged from 1 year to 20 years old (average, 8.0 years). The average number of large aortopulmonary collateral arteries was 1.9 per patient. Arborization abnormality was found in 5 patients. The aortopulmonary collateral arteries were reached solely through a median sternotomy, with dissection of the posterior pericardium or anterior mediastinal pleura before or after the initiation of cardiopulmonary bypass. The immediate postoperative peak pressure ratio between the right and left ventricles was higher in patients with an arborization abnormality, but all ratios ranged from 0.60 to 0.87. There was 1 operative death. Postoperative transcatheter embolization for a residual large aortopulmonary collateral artery was required in 1 patient and pulmonary infarction of mild degree developed with spontaneous recovery in another. These results indicate the usefulness and safety of simultaneous ligation of large aortopulmonary collateral arteries through median sternotomy, even with an associated arborization abnormality.     

Primary repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a useful approach

BACKGROUND: The ultimate goal of surgical therapy for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is to create unobstructed and separate in series pulmonary and systemic circuits. Our preference has been a 1-stage complete unifocalization technique, avoiding collateral anastomosis with either the native pulmonary arteries or other aortopulmonary collateral vessels. METHODS AND RESULTS: Since 1998, 5 patients (median age 29.6 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone surgical correction, consisting of (1) exclusion of a descending thoracic aortic segment from which all major aortopulmonary collateral arteries originate, and (2) connection of this aortic segment to the native pulmonary artery using an interposition polytetrafluoroethylene conduit. The ventricular septal defect was closed in all patients, and the right ventricle was connected to the unifocalized pulmonary artery with a valved conduit. All patients survived the operation. Two patients required reexploration for postoperative bleeding. One patient remained on mechanical ventilation for 17 days due to a pulmonary infection. During follow-up (12-21 months), no patient required additional interventions. The postoperative right ventricular/left ventricular pressure ratio was 0.55 median. No significant stenosis within the reconstructed pulmonary circuit was identified. All patients remain free of symptoms, requiring no medications. CONCLUSION: Intracardiac repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries can be accomplished by a midline 1-stage repair including complete unifocalization of all pulmonary blood supply without individual collateral anastomosis in selected patients. This approach offers a convenient and satisfactory surgical option.     

Preoperative prediction from cineangiograms of postrepair right ventricular pressure in tetralogy of Fallot.

To aid preoperative decision-making, we have related the ratio of postrepair peak pressure in the right and left ventricles (PRV/LV) to preoperative cineangiographic measurements in a retrospective study of 135 patients undergoing complete repair of tetralogy of Fallot or tetralogy of Fallot with pulmonary atresia. Postrepair PRV/LV was related to the preoperative diameter of right (DRPA) and left (DLPA) pulmonary arteries normalized to the descending thoracic aorta (DescThAo) in patients undergoing repair with transannular patching or a valved external conduit by the dquation: PRV/LV = 0.4840/(DRPA/DescThAO + DLPA/DesThAo) + 0.2007. Stenosis of the right pulmonary artery orifice and pulmonary artery arborization abnormalities incrementally increased postrepair PRV/LV. When a transannular patch was not used in classical tetralogy of Fallot, an increment of postrepair PRV/LV usually resulted, depending upon the size of the "anulus" measured intraoperatively: Incremental PRV/LV = 0.09437 . exp(-0.6344 . Z) where Z is a normalized expression in circumference terms of the diameter of the pulmonary arterial outflow tract (DPAOT) measured intraoperatively after infundibular dissection and valvotomy. DPAOT is itself related to the cineangiographically measured pulmonary valve anulus diameter (DPVA): DPAOT = 3.357 . DPVA0.5789 . BSA0.1551. In toto, these relations allow postrepair PRV/LV without transannular patching to be estimated from preoperative cineangiographic measurements. This allows preoperative predictiom in classical tetralogy of Fallot of the need for transannular patching, and in infants this can determine the choice between primary one-stage repair and two-stage repair. Prediction of postrepair PRV/LV when transannular patching or an external conduit is planned allows identification of patients in whom right and left pulmonary arteries are too small for safe complete repair, and in them an initial palliative operation should be done to enlarge the arteries.     

Staged surgical repair of pulmonary atresia, ventricular septal defect, and hypoplastic, confluent pulmonary arteries

In patients with pulmonary atresia and ventricular septal defect, hypoplasia of the central pulmonary arteries prevents single-stage complete repair. Over an interval of 8 1/2 years, 105 patients underwent establishment of continuity between the right ventricle and a hypoplastic central pulmonary arterial confluence (first stage). There were 12 hospital deaths (11%) and 11 late deaths before second-stage (complete) repair. Twenty-five patients await late evaluation. The remaining 57 individuals have had follow-up cardiac catheterization a mean of 33 months postoperatively. In 31 of these, final repair was deferred because of insufficient pulmonary arterial enlargement (14), restricted peripheral arborization (nine), or both (eight). The final 26 patients were accepted for second-stage repair, which has been performed in 24. Complete repair included ventricular septal defect closure (24), right ventricular outflow tract reconstruction (18), relief of central pulmonary arterial stenosis (14), and ligation of systemic-pulmonary collateral arteries (10). The mean postrepair peak systolic right ventricular-left ventricular pressure ratio was 0.67 (range 0.32 to 1.0). One of these patients (4%) died in the hospital and there was one late death (4%) from sepsis after tricuspid valve replacement. Three patients were lost to follow-up; the remaining 19 patients are in functional Class I or II. A two-stage surgical approach is highly successful in those patients whose pulmonary arteries are too hypoplastic to allow a single-stage repair.     

The structure of the pulmonary circulation in tetralogy of Fallot with pulmonary atresia. A quantitative cineangiographic study

Cineangiograms of 172 patients with tetralogy of Fallot and pulmonary atresia were qualitatively and quantitatively analyzed retrospectively. Findings: Twenty-eight (16%) patients had congenital absence of the central and unbranched hilar portions of the left or right pulmonary artery or both, and thus had congenitally nonconfluent pulmonary arteries. Five additional patients (3%) with originally confluent pulmonary arteries had nonconfluent ones because of an end-to-end surgical shunt, as did seven others (4%) because of thrombosis of one pulmonary artery. Among the 132 patients with confluent right and left pulmonary arteries, 70 (53% of 132) had incomplete arborization (distribution) of one or both, as did 23 (82% of 28) of those with congenitally nonconfluent pulmonary arteries (P for difference = 0.005). Ten (36% of 28) of the latter group had fewer than 10 pulmonary vascular segments (normal is 20) in continuity with central pulmonary arteries, compared with four (3%) of the former group. The proximal left and right pulmonary arteries were small but variable in size in all groups (median value 1.05 McGoon ratio), but were largest in the patients with confluent pulmonary arteries. Seventy-nine (60% of 132) patients with confluent left and right pulmonary arteries had large aortopulmonary collateral arteries, as did 100% of those with congenitally nonconfluent pulmonary arteries (P less than 0.001). The number of large aortopulmonary collateral arteries correlated inversely with the completeness of arborization of the left and right pulmonary arteries (P less than 0.0001). Inferences: Most patients with tetralogy and pulmonary atresia have gross morphologic abnormalities in the pulmonary vasculature and differ quantitatively in this regard from those with tetralogy and pulmonary stenosis. The prevalences of the various types of these abnormalities are considerably interrelated.     

Current trends in heart surgery for the treatment of congenital anomalies: tetralogy of Fallot and its variants

The surgical repair of tetralogy of Fallot is one of the most common procedures to correct congenital cardiac anomalies. However, controversy remains regarding the benefits and drawbacks of early primary repair. The management of patients with pulmonary atresia has become more aggressive in recent years, i.e., earlier primary repair with complete unifocalization is performed even if they have associated major aortopulmonary collateral arteries. On the other hand, the durability of small-sized conduits is unsatisfactory, and the rate of freedom from reoperation for those who received conduits in infancy is still low. Ventricular arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot are disastrous complications in adult survivors, but it remains difficult to predict which patients will experience such complications. Sustained ventricular tachycardia with pulmonary regurgitation is the predominant hemodynamic lesion. Achieving complete relief of the pressure gradient in the right ventricular outflow tract with no pulmonary regurgitation is the ultimate goal of surgical treatment of tetralogy of Fallot and its variants.     

Morphologic and surgical determinants of outcome events after repair of tetralogy of Fallot and pulmonary stenosis. A two-institution study.

Survival after entry and survival after repair (94%, 91%, and 91% at 1 month, 1 year, and 5 years, respectively) were similar in two institutions treating 196 consecutive patients, and there was no advantage (and a possible disadvantage) of a protocol of preliminary shunting and later repair in very young patients. Size and configuration of the right and left pulmonary arteries had no demonstrable effect on survival, prevalence of transannular patching, or postrepair right ventricular-left ventricular pressure ratio. Small size of the pulmonary "anulus" and trunk were risk factors for death, transannular patching, and high postrepair pressure ratio. High postrepair pressure ratio was a risk factor for death after repair. Very young age (less than about 3 months) was a risk factor for death after repair, particularly when other risk factors coexisted. The prevalence of transannular patching in patients with mild infundibular and pulmonary anulus and trunk hypoplasia decreased across the time of the study, without ill effect. The usefulness of measuring postrepair right ventricular-left ventricular pressure ratio is emphasized by the data.     

Staged repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 104 patients

OBJECTIVE: To determine the early and intermediate-term outcome of the staged repair used to treat children with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. METHODS: We reviewed a retrospective case series of 104 patients with this complex lesion. Information was obtained from medical records and referring physicians. RESULTS: Of the 104 patients treated with the staged repair, 58 achieved completion of anatomic repair. The 10-year mortality was 16.5%. In the patients with complete repair, the median right-to-left ventricle pressure ratio was 0.5. The overall surgical reoperation rate was 17%, and 15.5% of patients required postoperative interventional cardiac catheterization. In the multivariate analysis, the number of collateral vessels incorporated in the repair was found to be an independent risk factor for postoperative mortality and an elevated right-to-left ventricle pressure ratio after complete repair. CONCLUSION: The staged repair can be successfully used to treat patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. This method yields a relatively low mortality with good functional results.     

Surgical strategies for pulmonary atresia with ventricular septal defect associated with major aortopulmonary collateral arteries

Treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is challenging and controversial. Basically, the collateral arteries are treated surgically by unifocalization to integrate them and unify the blood flow in the pulmonary circulation. These major collateral arteries are anatomically similar to the bronchial arteries; however, they develop into different vessels because of different environmental exposures. Currently, treatment plans involving surgical intervention in early infancy to address the multiple variations of major aortopulmonary collateral arteries are being established to achieve definitive intracardiac repair. Historically, several surgical approaches have been proposed, including multi-stage unifocalization performed by lateral thoracotomy, rehabilitation of the central pulmonary artery followed by intracardiac repair, and single-stage unifocalization by a midline incision. Recently, single-stage unifocalization performed during infancy has become the preferred method for achieving low right ventricular pressure after closing the ventricular septal defect. Furthermore, for the maintenance of the lowest possible right ventricular pressure after definitive repair, combination therapy or hybrid therapy with catheter and surgical intervention is essential. The surgical treatment strategy for a single ventricle with major aortopulmonary collateral arteries is more difficult than that involving two ventricles.     

法洛四联症合并肺动脉闭锁的手术治疗

目的 探讨法洛四联症合并肺动脉闭锁(TOF-PA)的手术适应证、手术方法和效果.方法 1984年6月至2009年6月对66例TOF-PA患者进行了外科手术69次.其中男34例,女32例;年龄6个月～29岁.包括Ⅰ型31例,Ⅱ型14例,Ⅲ型12例,Ⅳ型9例.11例行姑息性分流术;2例行一期单源化和室间隔缺损开放;40例行一期修复术,13例行一期单源化和心内修复术.3例分流术后再次心内修复术.结果术后早期死亡6例,其中修复手术后死亡5例,分别为严重低心排血量综合征3例,灌注肺1例,多脏器功能衰竭1例;分流术后死亡1例,为切口感染、心内膜炎.47例随访3个月～15.5年,NYHA心功能Ⅰ-Ⅱ级44例,Ⅲ-Ⅳ级3例.结论 TOF-PA肺血管的解剖学和形态学特点是决定手术适应证和方法的重要指标,对于存在自体肺动脉并且周围肺动脉发育较好的TOF-PA,尽可能早期进行一期矫治手术.     

Transatrial-transpulmonary repair of tetralogy of Fallot

Classical tetralogy of Fallot was repaired by a transatrial-transpulmonary approach in 61 of 70 patients treated surgically on one surgical service between July 1981 and December 1985. Their ages ranged from 2.5 months to 57 years (mean 52.9 +/- 104.2 months). The ventricular septal defect was closed through the right atrium and tricuspid valve in 60 patients and via the pulmonary artery in one patient with a subpulmonary defect. A pulmonary arteriotomy was made in 38 patients to expose the stenotic pulmonary valve for valvotomy and to begin the infundibular mobilization and resection. It was extended through a small pulmonary anulus primarily in 15 patients and secondarily in one, a minimal distance upon the right ventricular infundibulum to enlarge the outflow tract to 2 standard deviations larger than the mean normal pulmonary valve anulus diameter. There were no hospital or late deaths or reoperations among the entire group. The mean postrepair peak right ventricular/left ventricular pressure measured in the operating room was 0.52 +/- 0.165 for the entire group of 61 patients which indicates similar relief of pulmonary stenosis to that obtained by the classical transventricular repair. At follow-up between 2.3 and 53.8 months (mean 22.6) each patient was in New York Heart Association Class I and without cardiac medication. Postoperative cardiac catheterization studies performed in nine patients between 4 and 28 months after operation showed no residual ventricular septal defects and a mean peak right/left ventricular pressure of 0.42 +/- 0.13. This series indicates that successful repair of tetralogy of Fallot can be accomplished in most patients by a transatrial-transpulmonary approach, which may provide improved right ventricular function over the long term.     

Increasing experience with integrated approach to pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

OBJECTIVES: To validate the preliminary surgical results of 'integrated approach' to pulmonary atresia, ventricular septal defect (VSD), and multiple aortopulmonary collateral arteries by retrospective analysis of our center experience. METHODS: Between 01/94 and 03/02, 37 patients aged 22 days to 13 years underwent surgery for pulmonary atresia, VSD, and multiple aortopulmonary collaterals. Case selection was based on preoperative calculation of total neopulmonary arterial index (TNPAI), pulmonary arterial index (PAI), and pulmonary arteries-to-collateral arteries lung segment perfusion ratio (S(pa):S(ca)). The decision for a possible VSD closure during one-stage procedures was based on an intraoperative pulmonary flow study. Twenty-five patients with a TNPAI equal to or greater than 150 mm(2)/m(2) underwent primary unifocalization, irrespective of PAI and S(pa):S(ca). Conversely, 12 patients with a TNPAI less than 150 mm(2)/m(2) and hypoplastic (PAI less than 100 mm(2)/m(2)) dominant (S(pa):S(ca) greater than 1) pulmonary arteries received a first-stage right ventricular outflow tract reconstruction, followed by unifocalization and repair (i.e. VSD closure) in nine cases. RESULTS: Among 34 patients who received total unifocalization, the overall repairability rate was 85% (first instance repairs: n=27; delayed VSD closure: n=2; 95% confidence interval, CI: 73-97%), with a survival rate at 7 years of 81%. Repaired survivors (n=26) are asymptomatic (n=22) or mildly symptomatic (n=4) at a follow-up interval of 43+/-28 months, with a 0.48+/-0.2 mean haemodynamic right ventricular/left ventricular pressure ratio, whereas palliated ones are waiting for either repair (n=3) or catheter study (n=2). Analysis of results has shown the following: (1) 100% (34/34 cases) feasibility of one-stage unifocalization in patients with a preoperative TNPAI equal to or greater than 150 mm(2)/m(2), whereas combined repairability rate was 79% only (95% CI: 65-93%); (2) 100% (12/12 cases) fulfillment of criteria for second-stage repairability (acquired TNPAI greater than 150 mm(2)/m(2)) in all patients treated with right ventricular outflow tract reconstruction; and (3) 93% (95% CI: 83-100%) overall accuracy of intraoperative flow study in predicting either postrepair mean pulmonary arterial pressure (VSD closed: n=23) or balanced pulmonary to systemic blood flow ratio (VSD left open: n=4). CONCLUSIONS: Increasing experience with 'integrated approach' to pulmonary atresia, VSD, and multiple aortopulmonaty collaterals has confirmed the preliminary results of our surgical series. The pulmonary flow study remains the most accurate intraoperative test for successful management of VSD during unifocalization procedures     

Decision-making in repair of tetralogy of Fallot based on intraoperative measurements of pulmonary arterial outflow tract.

The equation that we proposed in this JOURNAL (1977), relating the postrepair ratio between peak right ventricular pressure and peak left ventricular pressure (PRV/LV) to the intraoperatively measured internal diameter of the narrowest point in the pulmonary arterial outflow tract (formerly called pulmonary "anulus"), performed well when prospectively tested in 25 patients undergoing repair of the tetralogy of Fallot. The observed PRV/LV in all 21 patients in whom no transannular patch was used fell within the 70 percent confidence limits (1 standard deviation) of that predicted from the measured diameter. A trivial difference (-0.03 +/- 0.069 PRV/LV units, P = 0.08) was found between predicted PRV/LV and that observed after repair, with body surface area (BSA) used for normalization. When the four additional patients who had secondary transannular patching are included, or where the normalization was with weight, the differences were not significant (p greater than 0.2). A slightly revised equation has been devised using the combined data from our original group and the patients used for prospective testing. This equation predicts with reasonable accuracy (r = -0.69, p less than 0.0001) postrepair PRV/LV without transannular patching from the intraoperatively measured diameter of the pulmonary arterial outflow tract. We believe it is therefore helpful in the operating room in making the important decision for or against using transannular patching.     

Primary repair minimizing the use of conduits in neonates and infants with tetralogy or double-outlet right ventricle and anomalous coronary arteries

OBJECTIVE: The purpose of this study was to review our results with an approach of early primary repair for tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries, using several techniques to minimize the use of a conduit. METHODS: Twenty consecutive neonates and infants with anomalous coronary arteries crossing an obstructed right ventricular outflow tract underwent primary repair. Median age was 5.5 months and mean weight 6.22 kg. The anomalous coronary arteries included the left anterior descending from the right coronary artery (n = 10), the right coronary artery from the left anterior descending (n = 1), the left anterior descending from the right sinus (n = 1), and a significant conal branch from the right coronary artery (n = 7) or left anterior descending (n = 1). Two neonates had pulmonary atresia. The right ventricular outflow tract was reconstructed without a conduit in 18 patients, including those with pulmonary atresia. Surgical techniques included main pulmonary artery translocation in 4 patients, transannular repair under a mobilized left anterior descending coronary artery in 2 patients, and displaced ventriculotomy with subcoronary suture lines in 8 patients. In 4 patients the right ventricular outflow tract was repaired via the ventriculotomy and/or pulmonary arteriotomy. A homograft was used as the sole right ventricle-pulmonary artery connection in 1 patient and in another a homograft was added to a hypoplastic native pathway. RESULTS: There have been no early or late deaths. The right ventricular/left ventricular pressure ratio within 48 hours of the operation was 0.47 +/- 0.10. There were 2 reoperations at 8 and 11 years after the operation, during a mean follow-up of 5.2 years (1-11.3 years). CONCLUSIONS: Primary repair of tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries can be done in neonates and infants with excellent results. Alternative surgical techniques for right ventricular outflow tract reconstruction, such as main pulmonary artery translocation, can avoid the use of a conduit in most patients.     

One-stage unifocalization and palliative right ventricular outflow tract reconstruction

We report a successful case of one-stage unifocalization concomitant with palliative right ventricular outflow tract reconstruction for pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries and central pulmonary arterial absence. Through a median sternotomy, one-stage unifocalization was accomplished using autologous pericardial conduit and no prosthetic material. To achieve an adequate pulmonary blood flow, the right ventricular outflow tract was reconstructed so that it would be 70% of the normal pulmonary annular diameter, while the ventricular septal defect was left open. One-stage unifocalization concomitant with palliative right ventricular outflow tract reconstruction is considered to be a good surgical option for selected patients with these diseases.     

伴室间隔缺损的肺动脉闭锁的外科治疗

目的探讨伴室间隔缺损的肺动脉闭锁（VSD-PA）的外科治疗的手术时机、适应证和方法。方法1984年6月至2005年3月对32例VSD-PA进行外科手术治疗33例次,32例中男15例、女17例,年龄6个月～9岁,其中合并主动脉到肺的侧支循环动脉（APCA）的9例。3例周围肺动脉发育差、肺动脉指数（PAI）〈150mm^2／m^2,行升主动脉-肺动脉分流术;2例全部新的肺动脉指数（TNPAI）〈150mm^2／m^2或肺循环完全由分散、细小的MAPCA供应者,行一期单源化手术和室间隔缺损开放;27例（28例次,包括1例分流术后13个月）肺动脉发育达到PAI〉150mm^2／m^2或TNPAI〉200mm^2／m^2,行中度低温体外循环下一期矫治手术。结果术后早期死亡5例,其中一期矫治手术后死亡4例,分别为严重低心排血量综合征2例,灌注肺1例,多脏器功能衰竭1例;升主动脉肺动脉分流术后死亡1例,为切口感染、心内膜炎。21例随访3个月-15．5年,NYHA心功能Ⅰ和Ⅱ级19例,Ⅲ和Ⅳ级各1例。结论VSD-PA的外科治疗包括姑息或矫治手术,对于肺动脉发育较好的VSD—PA,尽可能早期进行一期矫治手术可以得到满意的效果。     

Anesthetic management of a hemophilia A carrier for palliative operation and radical repair of a cardiac anomaly]

We experienced the anesthetic management of a hemophilia A carrier for two palliative operations (left and right unifocalization) and radical repair of cardiac anomaly. The patient was a ten-year-old girl and her father had hemophilia A. She was diagnosed as tetralogy of Fallot, major aortopulmonary collateral arteries and pulmonary atresia. Laboratory findings showed prolonged bleeding time and activated partial thromboplastin time, and decreased levels of factor VIII activity of 26%. When she was eight and nine years old, she underwent unifocalization. At both occasions, excessive bleeding tendency continued into postoperative period. This time she underwent radical repair in which her bleeding tendency was successfully controlled by use of a usual dose of concentrated factor VIII for twice.