Repair of double-outlet right ventricle. An analysis of 62 cases.

Sixty-two patients with double-outlet right ventricle (DORV) underwent complete intracardiac repair between 1967 and July, 1978. Five patients (three deaths) with DORV and complete atrioventricular (AV) canal are the subject of a separate report and are not discussed further here. Twenty-eight patients had relatively uncomplicated DORV (subaortic ventricular septal defect [VSD], doubly committed VSD, or 1-malposition of the aorta with subaortic VSD) with or without pulmonary stenosis. Two (14%) of 14 died after a completely intraventricular repair, and one (12%) of eight after repair including use of a valved external conduit. Use of a transannular patch in this group was an incremental risk factor, five (83%) dying among six treated in this way. Anterior enlargement of a restrictive or unfavorably located VSD did not increase risk. A noncommitted VSD was an incremental risk factor, two (50%) of four dying after repair. No instances of complete heart block occurred in these groups. Two late deaths occurred in the uncomplicated group, from persisting servere pulmonary hypertension. The late results were good in the remainder and in the two surviving patients with noncommitted VSD. After repair of the Taussig-Bing type of DORV, eight deaths (32%) occurred among 25 patients. Complete heart block developed in two patients. In the Taussig-Bing type of DORV, the 6 year actuarial survival rate was only 38%. Most late deaths were related to improtant pulmonary vascular disease. The surgical technique that has evolved for the basic tunnel repair in the various types of DORV is described.     

Biventricular repair of double outlet right ventricle with noncommitted ventricular septal defect

Double outlet right ventricle (DORV) with noncommitted ventricular septal defect (VSD) (DORVncVSD) represents the most extreme form of DORV, raising challenging surgical difficulties for biventricular repair. The considerable distance between the VSD and the aorta is primarily because of the very abnormal location of the aorta. The definition of DORVncVSD includes: (1) a VSD distant (greater than aortic diameter) from both arterial valves; (2) both great vessels arising fully from the right ventricle; and (3) a double conus. Double outlet right ventricle with noncommitted ventricular septal defect is a primitive right ventricle, as seen during embryologic development, characterized by the presence of the entire conotruncus from the right ventricle. One surgical technique for repair of DORVncVSD is rerouting of the VSD to the aorta by a long intraventricular tunnel. This technique is limited by the presence of conal tricuspid chordae and by the distance between the tricuspid and pulmonary valves, and is associated with an important risk of subaortic obstruction. Rerouting through the pulmonary artery followed by arterial switch seems a more satisfactory surgical solution. When the VSD is distant from the aorta, it is almost always quite close to the pulmonary artery. Rerouting to the pulmonary artery creates a smaller channel, and its application is not limited by the presence of tricuspid chordae or the tricuspid-to-pulmonary valve distance. However, the arterial switch frequently involves relocating complex coronary arteries. Copyright © 2002 by W.B. Saunders Company     

Surgical management of double-outlet right ventricle with subaortic ventricular septal defect

Double-outlet right ventricle (DORV) and subaortic ventricular septal defect (VSD) is defined anatomically as a defect where the entire pulmonary trunk and at least half of the aorta arises from the right ventricle (RV) and the VSD is most closely aligned with the aorta. The surgical management of DORV and subaortic VSD usually results in a 2 ventricle repair where the left ventricular outflow is diverted via the VSD to the aorta. Pulmonary and/or subpulmonary obstruction is found in a large portion of these patients and requires relief at the time of repair. Resection of subpulmonary muscular obstruction with right ventricular outflow augmentation with a patch is required in most to eliminate the obstruction. Important anatomic features to consider in constructing the left ventricular outflow tunnel include the prominence of the conal septum, tricuspid valve attachments to conal septum, and the distance between the pulmonary and tricuspid valves. Operative mortality is low and long-term complications and need for reoperation is uncommon. This report describes the surgically relevant anatomic features and operative techniques. Copyright © 2000 by W.B. Saunders Company     

Double-outlet right ventricle with non-committed ventricular septal defect

Objective: The term non-committed was used to define hearts in which the VSD was anatomically related to, or was close to, neither great vessel, being separated from both by considerable muscle. We report our experience of the surgical management of this subset, considered being of particular surgical relevance. Methods: Between January 1987 and December 1997, 23 patients having double-outlet right ventricle (DORV) with non-committed VSD underwent biventricular repair. Nine (39%) had undergone previous palliation. The median age was 20 months and the median weight was 8.5 kg. Two main types of repair were used: intraventricular baffle repair (n=21) and arterial switch operation with VSD to pulmonary artery baffle (n=2). At repair, 12 (52%) patients required concomitant VSD enlargement. In two other patients presenting with restrictive inlet VSD associated with tricuspid attachments, crossing the subaortic pathway biventricular repair was abandoned at operation. Results: There were two hospital deaths (9%, 70% CL: 3–19%). Eight patients (35%, 70% CL: 23–48%) underwent nine reoperations, six for subaortic stenosis. No late death occurred. At last visit, all patients were asymptomatic and only two had cardiac medication. Conclusions: The biventricular repair of DORV with non-committed VSD is feasible in the vast majority of cases with comparable results to other subsets of DORV. After repair, the subaortic region is at risk for development of subaortic stenosis.     

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

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

A case report: Rastelli operation for double outlet right ventricle associated with a chordae insertion to the infundibular septum]

A successful Rastelli operation for double outlet right ventricle (DORV) with a chordae insertion of the tricuspid valve to the infundibular septum was reported. A patient was a 6-year-old boy and the diagnosis was DORV, d-malposition of the aorta and pulmonary stenosis. The infundibular septum was not resected but mobilized with two incision, one anterior and vertical, and other one subaortic. This procedure allowed the construction of the tunnel similar to the closure of a large, subarterial VSD and of the straight unobstractive left outflow tract. The postoperative echocardiographic and angiographic examination revealed neither the pressure gradient in the left ventricular outflow tract nor the tricuspid regurgitation.     

Transatrial repair of double-outlet right ventricle in infants.

In three infant cases of double outlet right ventricle (DORV), two with normally related great arteries (NGA) and one with side-by-side great arteries, a transatrial repair was carried out. In all three cases, the results were excellent. It is concluded that in the small baby with DORV with NGA and in DORV with side-by-side great arteries with a hypoplastic crista, a transatrial repair should be successful. This is dependent on the VSD being in the perimembranous (and, therefore, subaortic) location and on the absence of infundibular pulmonary stenosis. In all other varieties of DORV the repair should probably be done through the ventricle.     

303例右室双出口手术后早期转归

目的　总结右室双出口 (DORV)病例的术后处理及不同外科术式的早期转归。方法　 1988年 1月至 2 0 0 3年 12月期间收治 30 3例DORV病儿 ,按室间隔缺损 (VSD)的部位分为主动脉下VSD ,肺动脉下VSD ,双动脉下VSD和远离大动脉下VSD 4组。总结比较不同外科术式术后的并发症和死亡率及其原因。结果　再手术 18例 ,死亡 2 9例 ,死亡率为 9 6 % ,其中肺动脉下VSD死亡率最高 ,为 2 4 . 4 % (χ2 =14 . 12 ,P <0 . 0 1) ;根治术和姑息术的死亡率分别为 10 . 6 %和 6 . 6 % (χ2 =1. 0 3,P >0 .0 5 )。死亡原因主要是低心排血量综合征 (LCOS) ,占 82 . 6 % ;其也是术后主要并发症 ,占 31 .0 %。结论　LCOS是术后主要并发症和死亡的高危因素。有效预防和控制LCOS是取得治疗成功的关键。     

Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification.

BACKGROUND: The STS-EACTS International Nomenclature for Congenital Heart Surgery (CHS) defines four anatomic subtypes of double outlet right ventricle (DORV) based on the relationship of the ventricular septal defect (VSD) with the great vessels and the presence of right ventricular outflow tract obstruction (RVOTO). We reviewed our experience with DORV patients and two ventricles that underwent repair, applying this nomenclature. METHODS: Between January 2000 and January 2005, 50 patients with DORV and two viable ventricles underwent surgical intervention: 44 patients had biventricular repair, 3 had 1.5 ventricular repair, 2 underwent a Fontan, and 1 died prior to corrective surgery. Median age at repair was 9.1 months (range: 4D-4Y). Eighteen patients (36%) were DORV-Fallot (including 5 with AVSD and heterotaxy), 9 (18%) were DORV-TGA (Taussig-Bing), 12 (24%) were DORV-VSD, and 11 (22%) were DORV non-committed VSD. Corrective surgery included 35 repairs with a VSD-aorta baffle+/-RVOTO procedure and 12 arterial switches with a VSD-PA baffle (9 Taussig-Bing and 3 DORV-ncVSD). Associated procedures included 13 VSD enlargements, 8 subaortic resections, 9 arch repairs, 5 AVSD repairs, and 7 others. RESULTS: There were three deaths in the 50 patients studied (overall mortality of 6%). Excluding one patient that died prior to corrective surgery and the two patients palliated with a Fontan procedure, the actual surgical mortality for a corrective repair was 4.3% (2/47 patients). Two surgical deaths occurred following, respectively, one repair of a Taussig-Bing with an interrupted arch and a Swiss cheese VSD and one repair of ncVSD-type with pulmonary atresia that had undergone a previous cavo-pulmonary anastamosis. No late deaths occurred. Two late reoperations included a heart transplant in a DORV-Fallot patient with Swiss cheese VSD and subaortic resection in a DORV-ncVSD patient. Angioplasties were needed for PA stenosis (n=2) and aortic arch obstruction (n=2). Four patients had LV to aorta baffle gradients between 10 and 20 mmHg. All patients were of NYHA class I/II. CONCLUSIONS: The STS-EACTS International Nomenclature provides more uniform analysis of outcomes with respect to acceptable surgical risk and mortality. Biventricular repair can be safely achieved on selected DORV, including DORV-ncVSD and DORV with AVSD and heterotaxy lesions traditionally indicated for a single ventricle palliative approach.     

Current risks and protocols for operations for double-outlet right ventricle. Derivation from an 18 year experience

Recent follow-up of 127 patients having repair of double-outlet right ventricle during an 18 year experience (1967 to July, 1984) indicated an overall actuarial survival rate at 12 years of 38%. However, multivariate analysis supported by contingency tables indicated that currently the early (2 week) survival rate after the intraventricular tunnel repair for double-outlet right ventricle with subaortic ventricular septal defect in 6-month-old infants is 99% and the 10 year survival rate 97%. Reoperation of the tunnel repair was rarely required (one of 56 patients), and the functional results were excellent. Results were similar in patients with doubly committed ventricular septal defect, except that two of 10 patients developed typical discrete localized subaortic stenosis late postoperatively. Early and late results in patients with double-outlet right ventricle and subpulmonary ventricular septal defect were poor when an atrial switch operation was part of the repair; when an intraventricular tunnel repair between the left ventricle and aorta was combined with a right ventricular-pulmonary arterial conduit, no early deaths occurred, but three patients died late postoperatively. Several techniques of repair of double-outlet right ventricle with noncommitted ventricular septal defect have provided only a 22% overall 10 year survival rate. These data are used to derive inferences as to optimal treatment protocols.     

Single ventricle (single- or double-inlet) complicated by subaortic stenosis: surgical options in infancy

Intracardiac obstruction to the systemic circulation can develop in patients with many forms of congenital heart disease. When transposition of the great arteries accompanies tricuspid atresia, narrowing of the ventricular septal defect (VSD) leads to subaortic stenosis. In a similar fashion, a restrictive bulboventricular foramen compromises systemic arterial outflow in patients who have single left ventricle with subaortic outflow chamber. The same effect can be seen in VSD closure in mitral atresia with normally related great vessels. Although some forms of single ventricle can be treated by ventricular septation, the modified Fontan procedure can be more generally applied to this entity. Pulmonary vascular resistance must be low, however, which excludes the application of the Fontan principle in infants. While pulmonary artery banding may protect the lungs from the development of excessive pulmonary vascular resistance, it may also accelerate the development of subaortic obstruction. We have successfully applied the Norwood operation, as described for hypoplastic left heart syndrome, to palliate subaortic stenosis due to restrictive VSD in 3 infants with single ventricle or atrioventricular valve atresia. Consideration should be given to a primary Norwood procedure in infants with single ventricle in whom subaortic stenosis is likely to develop. Patients who do receive pulmonary artery bands should be followed closely for the development of subaortic stenosis and should undergo an early, physiologically corrective operation if it occurs.     

The optimal procedure for the great arteries and left ventricular outflow tract obstruction. An anatomical study.

OBJECTIVE: To describe the optimal surgical strategy in heart specimens with transposition of the great arteries (TGA) and left ventricular outflow tract obstruction (LVOTO). METHODS: Thirty-three specimens with LVOTO were selected: TGA with intact ventricular septum (TGA/IVS) (10), TGA/VSD (21), and Taussig-Bing (2). RESULTS: LVOTO in TGA/IVS consisted of combinations of bicuspid pulmonary valve (four), subpulmonary fibrous ridge (four), obstructive muscular conus (two) and bulging muscular septum (four). Arterial switch operation (ASO) with LVOTO resection/valvotomy was feasible in nine hearts. Obstructive anterior papillary muscle prohibited LVOTO relief in one specimen. In TGA/VSD and Taussig-Bing LVOTO consisted of combinations of bicuspid (nine) or unicommissural (one) pulmonary valve, fibrous ridge (three), obstructive muscular conus (five), malaligned outlet septum (six), accessory mitral valve tissue (two), straddling mitral valve (two) and anterior mitral valve rotation (four). VSDs were subpulmonary in 13 (9 perimembranous, 4 muscular), subaortic in 3 (2 perimembranous, 1 anterior muscular), doubly committed in 2, inlet in 3 (2 perimembranous, 1 muscular), non-committed and anterior in 1, and finally 1 VSD extended both into inlet and subpulmonary outlet septum. LVOTO resection and ASO with VSD closure was possible in 10. In six specimens, both a Rastelli and a Nikaidoh operation were feasible. For two hearts, a Nikaidoh procedure was the only option, while Rastelli was considered optimal in another specimen. Mitral valve anomalies prevented LVOTO relief in four, only permitting for Senning/VSD closure (one) or univentricular palliation (three). CONCLUSIONS: LVOTO resection and pulmonary valvotomy frequently permits an ASO. Inlet VSD, impossibility of VSD enlargement, straddling mitral valve, distant aorta and small right ventricle make the Nikaidoh procedure the best option. Mitral anomalies preventing LVOTO relief can make biventricular repair impossible.     

Primary biventricular repair for interrupted aortic arch with left ventricular outflow tract obstruction and tricuspid valve regurgitation; report of a case

An interrupted aortic arch was diagnosed in a 10-day-old girl weighing 3.3 kg, as was perimembranous ventricular septal defect (VSD) and severe tricuspid valve regurgitation (TR). The subaortic diameter was 3.6 mm and the aortic valve (3.7 mm in diameter) was bicuspid. We chose definitive repair, modified Yasui procedure, because of severe TR and no straddling of mitral valve. In primary biventricular repair, we undertook extended aortic arch anastomosis. Left ventricular outflow tract reconstruction consisted of intracardiac rerouting from the VSD to the pulmonary artery by using expanded-polytetrafluoroethylene (ePTFE) and Damus-Kaye-Stansel (DKS) anastomosis. Right ventricular outflow tract reconstruction was performed by the Rastelli procedure with an ePTFE valved conduit. Moreover, we carried out semicircular annuloplasty for severe TR.     

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

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

Morphosurgical correlation of outcomes in complete double outlet right ventricle

Even though surgical experience with wide spectrum of double outlet right ventricle (DORV) is available, the experience with extreme form of complete DORV where both great arteries completely arise from the right ventricle is limited. We present our experience with this unique subset where the systemic outflow is far removed from the interventricular foramen and hence, the systemic ventricle. In this situation, biventricular repair can be technically demanding and challenging. Between June 2002 and February 2006, 12 patients underwent biventricular repair of this subset. The VSD was subaortic in all; eight patients had infundibular and valvar obstruction. Aorta was anterior and to the right, with the pulmonary artery far posterior and to the left. The interventricular foramen was patched with a long Gore-Tex patch to route the LV flow to the aorta. Eight patients had infundibular resection and right ventricular outflow tract (RVOT) enlargement with an autologous monocusp pericardial patch. No patient required a valved conduit. There was no operative mortality. In one patient, there was a small residual VSD that was not of haemodynamic significance. There was no RVOT and left ventricular outflow tract obstruction and no RV inflow obstruction. The early and mid-term results are good.     

右心室双出口的手术治疗

目的　总结右心室双出口手术治疗的早期和远期结果及经验。　方法　 1985年 5月～ 2 0 0 1年 12月 4 1例右心室双出口患者接受了外科治疗 ,其中男 2 6例、女 15例 ,年龄 10个月～ 2 1岁 ,平均 (10± 6 )岁 ,体重 6 5～ 6 5 0kg ,平均 (2 6± 16 )kg。 4 1例中 ,14例行经典的Rastelli手术 (内隧道外管道手术 ) ;11例行经内隧道成形左心室流出道、右心室流出道直接疏通或用心包片加宽 ;11例行改良Rastelli手术 ;2例行全腔静脉肺动脉吻合术 ;2例行双向格林手术 ;1例行大动脉调转术。结果　早期 (术后 1个月 )死亡 1例 ,出院 4 0例。随访 36例 ,随访 5个月～ 17年 ,中位数为 7年 ,无死亡 ,二次手术 3例。所有患者紫绀消失 ,症状明显改善 ,心功能Ⅰ级 34例 ,心功能Ⅱ级 2例 ,能够进行正常的生活、学习和工作。　结论　绝大多数右心室双出口患者经双心室矫治可以取得很好的早期及远期结果 ;对于合并其他严重畸形的患者可行右心室旁路手术。     

Accessory tricuspid valve--a report of two cases]

Accessory tricuspid valve is a rare cardiac anomaly. We describe two cases with accessory tricuspid valve. Case 1: A 7-year-old boy underwent repair of tetralogy of Fallot. Under deep hypothermic cardiopulmonary bypass, right ventricle was opened. An accessory tricuspid leaflet was noted to the right side of the VSD attaching to the infundibular septum from medial papillary muscle as a chordal origin. After excision of the accessory tricuspid leaflet, the VSD was closed with a Dacron patch followed by relieve of the right ventricular outflow obstruction and pulmonary valvotomy. Case 2: A 8-year-old boy had Rastelli operation to correct transposition of the great arteries. An accessory leaflet originated from the medial papillary muscle and was floating like a parachute in the way between VSD and aorta. There was no additional apparatus in the pathway between the VSD and aorta after excision of the accessory tricuspid leaflet. Intraventricular tunnel was created with a Dacron patch. Extracardiac conduit between the right ventricle and the distal pulmonary artery was constructed using a valved pericardial roll.     

Ross procedure for aortic insufficiency due to doubly committed subarterial ventricular septal defect in adults

A 52-year-old female, with aortic insufficiency due to doubly committed subarterial ventricular septal defect (VSD) underwent a successful surgical repair by the Ross procedure. Preoperatively, she developed congestive heart failure because of less compliance to oral medication, raising concerns regarding life-threatening thromboembolism if she undergoes mechanical valve replacement. Despite the pulmonary autograft being defective, there were no difficulties in completing the surgery. The defect of pulmonary autograft and the VSD was closed by an expanded polytetrafluoroethylene patch. She tolerated the procedure well and now enjoys improved quality of life. We present a discussion of the indication of Ross procedure in the rare presentation of congenital heart disease, as well as several issues raised in this case.     

Results of the probe technique for transatrial repair of tetralogy of Fallot

OBJECTIVE: Total correction of classical tetralogy of Fallot (TOF) by transatrial approach has become a standard procedure in the goal to minimize structural damage to the pulmonary pump. The most critical point in transatrial repair of TOF is infundibular dissection. Right atrial approach provides better surgical exposure for parietal extension of the infundibular septum when compared to the right ventricular approach. However it is not always easy to determine the localization and amount of muscle bundles to be resected and this surgical maneuver requires experience. METHODS: Nineteen patients who had repair of isolated TOF using this technique from 1993 to 2001 were reviewed. The mean age of patients were 5 +/- 2 years. Transatrial-transpulmonary approach were performed for all patients. To make easier the infundibular muscle bundles resection and to determine the localization and amount of muscle bundle to be resected, we placed a Hegar dilator into the right ventricle through pulmonary arteriotomy. The muscle bundles between the dilator and the anterior leaflet annulus of the tricuspid valve were totally excised until the intraventricular part of the dilator and pulmonary annulus became completely visible. The area between the Hegar dilator and the margins of the ventricular septal defect (VSD) was left untouched. None of the patients had transannular patch. To improve exposure, tricuspid valve detachment was performed in 11 patients. All patients were followed-up in our clinic every 6 months using echocardiography. RESULTS: There were no early or late deaths, and no reoperation for residual VSD or residual right ventricle (RV) outflow obstruction. All patients were in NYHA Class I. RV on the echocardiography was spared late dilatation and had a good late functional status. Eighteen patients had no or mild pulmonary regurgitation. One patient who had undergone tricuspid anterior leaflet detachment showed mild tricuspid insufficiency. CONCLUSION: On the basis of hemodynamic outcomes, this procedure for elective repair of TOF in selected cases shows excellent early and mid-term results.     

The results of probe technique for transatrial repair of tetralogy of Fallot

OBJECTIVE: Total correction of classic tetralogy of Fallot (TOF) by transatrial approach has become a standard procedure with a principal theoretical aim of minimizing structural damage to the pulmonary pump. The most critical point in transatrial repair of TOF is infundibular dissection. Right atrial approach provides better surgical exposure for parietal extension of the infundibular septum when compared to a right ventricular approach. However, it is not always easy to determine the localization and amount of muscle bundles to be resected and this surgical maneuver requires experience. METHODS: Nineteen patients were reviewed who had repair of isolated TOF by this technique from 1993 to 2001. The mean age of patients was 5 +/- 2 years. Transatrial-transpulmonary approach was performed for all patients. To make the infundibular muscle-bundle resection easier and to determine the localization and amount of muscle bundle to be resected, we placed a Hegar dilator into the right ventricle through pulmonary arteriotomy. The muscle bundles between the dilator and the anterior leaflet annulus of the tricuspid valve were totally excised until the intraventricular part of the dilator and pulmonary annulus became completely visible. The area between the Hegar dilator and the margins of the ventricular septal defect (VSD) was left untouched. None of the patients had transannular patch. Tricuspid valve detachment in order to improve the exposure was done in 11 patients. All patients were followed up in our clinic at regular six-month intervals by echocardiography. RESULTS: There was no early or late mortality nor reoperation for residual VSD or residual right ventricle (RV) outflow obstruction. All patients were in NYHA class I. RV on the echocardiography was spared late dilatation and had a good late functional status. Eighteen patients had no or mild pulmonary regurgitation. One patient who had undergone tricuspid anterior leaflet detachment showed mild tricuspid insufficiency. CONCLUSIONS: On the basis of hemodynamic outcomes, this procedure for elective repair of TOF in selected cases gives excellent early and mid-term results.