A case report of definitive repair for pulmonary atresia and intact ventricular septum associated with aortic valve regurgitation

A successful definitive repair for a 10-year-old girl with pulmonary atresia and intact ventricular septum (PA.IVS) associated with aortic valve regurgitation is described. The Fontan type repair was not indicated in this case because of the left ventricular dysfunction due to aortic valve regurgitation and inadequate size of the pulmonary artery. Therefore, right ventricular outflow tract reconstruction, Glen shunt and aortic valve replacement were performed despite severe hypoplastic right ventricle (RVEDVI; 33% of normal) and restrictive tricuspid valve (TVD; 48% of normal). Postoperatively, good result was obtained. There is general agreement that biventricular repair could be safely performed using Glenn shunt, when RVEDVI is above 40% of normal and TVD is above 50% of normal in a patient with PA.IVS. Moreover recently including our case, several successful repairs for PA.IVS with more hypoplastic right ventricle and tricuspid valve have been reported. So it is suggested that the right ventricular outflow tract reconstruction and Glenn shunt can be reliably applied for PA.IVS with more hypoplastic right ventricle and more restrictive tricuspid valve. To our knowledge, this is the first successful report of definitive repair (right ventricular outflow tract reconstruction, Glenn shunt and AVR) for PA.IVS associated with AR.     

Improved results with selective management in pulmonary atresia with intact ventricular septum

OBJECTIVE: Late outcome of neonatal pulmonary atresia with intact ventricular septum remains poor in most reported series. We have followed a selective approach toward either single ventricle repair versus complete or partial biventricular repair based on the presence of right ventricle-dependent coronary circulation and growth of the right ventricle. METHODS: A retrospective chart review was conducted of 47 patients who underwent surgery between January 1991 and September 1998. RESULTS: Sixteen (34%) patients had a right ventricle-dependent coronary circulation, with a tricuspid valve Z-score of -3.0 +/- 0.66 versus -2.0 +/- 0.95 (P =.002) for those without a right ventricle-dependent coronary circulation. A systemic-pulmonary artery shunt only was performed in all patients with a right ventricle-dependent coronary circulation, with 1 death. Fourteen of 16 patients with a right ventricle-dependent coronary circulation underwent a bidirectional Glenn shunt at a median of 9 months after their first operation, 9 of whom have had a Fontan procedure (no deaths). In the 31 (66%) patients without a right ventricle-dependent coronary circulation, 6 patients underwent only a systemic-pulmonary artery shunt, 23 had a shunt and right ventricular decompression, and 2 had only a transannular patch. In this group, 10 patients received a 2-ventricle repair, 6 a 1. 5-ventricle repair, and 8 patients had a Fontan procedure. There was 1 early death and the overall survival was 98% at 1 year, 5 years, and 7 years. CONCLUSIONS: If patients are stratified well, excellent survival can be achieved in the treatment of pulmonary atresia with intact ventricular septum. This result may be at the price of achieving a 1-ventricle as opposed to a 2-ventricle repair.     

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.     

Partial biventricular repair for double-outlet right ventricle with left ventricular hypoplasia

The presence of left ventricular hypoplasia in double-outlet right ventricle may increase the risk of biventricular repair and Fontan procedures. The hypoplastic left ventricle of an 11-year-old girl with complex double-outlet right ventricle was successfully incorporated in a modified biventricular repair by Dacron patch closure of the ventricular septal defect, diversion of venous return of the inferior vena cava to the mitral valve with an intraatrial baffle, insertion of a left ventricular apex to pulmonary artery valved aortic homograft, and diversion of residual systemic venous return directly to the lungs with a bidirectional superior vena cava to pulmonary artery shunt. Postoperatively, the systemic venous atrial pressure was 6 mm Hg and the pulmonary artery pressure was 14 mm Hg. This method is proposed as a partial biventricular repair hemodynamically superior to the Fontan procedure, although aortic homograft revision may be required in the future.     

Early and late results in pulmonary atresia and intact ventricular septum.

We examined the early and late results of operations in 29 consecutive neonates with pulmonary atresia and intact ventricular septum treated from 1980 to 1988. Transventricular pulmonary valvotomy and central aorta-pulmonary artery shunting were performed in 19 of 22 infants who had a patent infundibulum. Pulmonary valvotomy alone was performed in 3 of the 22 infants with a patent infundibulum, but 2 of these required subsequent systemic-pulmonary artery shunts. Primary shunting was used to palliate 7 infants who had absent infundibular portions of the right ventricle and a very diminutive right ventricular cavity. Tricuspid valve excision and atrial septectomy were also performed in 5 of these 7 infants to decompress large fistulous communications between the right ventricule and coronary artery. Two early deaths (2/29, 6.9%) occurred overall. Both were in infants who had a very small right ventricle. Definitive operation has been accomplished in 16 patients; 13 have had closure of residual interatrial communications and shunt ligation with no deaths, and 3 have undergone modified Fontan repair with 1 death. Actuarial survival rate for the entire group, including operative deaths, is 86% at 5 years. The technique of transventricular pulmonary valvotomy and systemic-pulmonary artery shunting offers a reliable means of palliating neonates with pulmonary atresia and intact ventricular septum and obtains good late right ventricular growth. Systemic-pulmonary shunting, tricuspid valvectomy, and atrial septectomy may offer a means of reducing or obliterating right ventricular-coronary artery fistulas.     

A rare case of congenital tricuspid stenosis treated with modified Fontan operation

A rare case of congenital tricuspid stenosis treated with modified Fontan operation was reported. The patient was 13 years old boy and his chief complaint was cyanosis. Cyanosis appeared in his neonatal period and squatting developed in his childhood. He underwent Blalock's shunt operation at 5 years of age. His exercise tolerance was limited at the admission for the radical operation. Angiocardiography showed the annular stenosis of tricuspid valve and the moderate hypoplasia of right ventricle. The right ventricular volume was 56 ml (equivalent to 35% of the normally expected right ventricular volume) and the diameter of tricuspid annulus was 13 mm (equivalent to 58% of the normally expected diameter). Atrial septal defect was associated with but pulmonary stenosis did not exist. His left ventricular function was good and pulmonary vascular resistance was not raised. A diagnosis of tricuspid stenosis was made and Fontan's repair was considered to be possible for his correction. Operative findings showed normal architecture of tricuspid valve with the three leaflets but tricuspid annulus was narrow. Tricuspid valve stenosis was considered to be caused by the hypoplasia of tricuspid ring and surgical dilatation was found to be difficult. Therefore, modified Fontan operation (RA-RV connection) was performed. His cyanosis disappeared and exercise tolerance was improved after the operation. Cardiac catheterization and angiocardiography were performed 8 months and 28 months after the operation. His righ ventricular volume was increased to 123 ml (equivalent to 57% of the normally expected volume) followed by RA-RV connection.     

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.     

Surgical treatment of complex cardiac anomalies: the 'one and one half ventricle repair'.

OBJECTIVE: One and one half ventricle repair is a surgical option for congenital cardiac anomalies characterised by right ventricle (RV) hypoplasia and/or dysplasia. METHODS: From March 1994 to March 2001, eight patients (mean age 9.1 years, range 7 months to 35 years) with hypoplastic and/or dysplastic RV underwent correction of their intracardiac anomaly in association with a BCPS (one and one half ventricle repair). Preoperative diagnoses included: Ebstein's anomaly of tricuspid valve (TV) in two, inlet ventricular septal defect (VSD) in association with straddling/overriding TV in two patients, pulmonary atresia-intact ventricular septum in one, tertralogy of Fallot in association with complete atrioventricular canal defect in one, truncus arteriosus in one and heterotaxy syndrome with VSD and anomalous systemic venous return in one. Four patients underwent previous surgery which included: main pulmonary artery (MPA) banding in two patients, pulmonary valvotomy, central shunt and right ventricular outflow tract reconstruction in one, pulmonary artery separation from truncus arteriosus and modified Blalock-Taussig shunt in one, and MPA closure in one. Two patients underwent a bidirectional cavo-pulmonary shunt before the one and a one half ventricle repair. Associated cardiac lesions were treated simultaneously. RESULTS: There were no hospital deaths. All the patients were discharged home in good clinical conditions. There were no late deaths or reoperations. At mean follow-up of 29.8 months (range 8 months to 7.3 years) all the patients are alive and in good general conditions. MPA percutaneous balloon dilation was performed in two patients at 33 and 4 months, respectively, both after MPA reconstruction (which was previously ligated) and dilation of the left pulmonary artery branch in one patient, repeated twice at 10 and 14 months from repair, for a hypoplastic left pulmonary artery after truncus arteriosus repair. CONCLUSIONS: Surgical treatment of congenital cardiac anomalies in the presence of a hypoplastic and or a dysplastic RV by means of one and one half ventricle repair has the advantage of reducing the surgical risk for biventricular repair, and compared to the Fontan circulation, it maintains a low right atrium pressure, a pulsatile pulmonary blood flow and improves the systemic oxygen saturation. Short and medium-term results are promising. Longer follow-up is needed, to prove the efficacy of such a repair, in the long term.     

Surgical treatment of complex cardiac anomalies: the ‘one and one half ventricle repair’

Objective: One and one half ventricle repair is a surgical option for congenital cardiac anomalies characterised by right ventricle (RV) hypoplasia and/or dysplasia. Methods: From March 1994 to March 2001, eight patients (mean age 9.1 years, range 7 months to 35 years) with hypoplastic and/or dysplastic RV underwent correction of their intracardiac anomaly in association with a BCPS (one and one half ventricle repair). Preoperative diagnoses included: Ebstein's anomaly of tricuspid valve (TV) in two, inlet ventricular septal defect (VSD) in association with straddling/overriding TV in two patients, pulmonary atresia-intact ventricular septum in one, tertralogy of Fallot in association with complete atrioventricular canal defect in one, truncus arteriosus in one and heterotaxy syndrome with VSD and anomalous systemic venous return in one. Four patients underwent previous surgery which included: main pulmonary artery (MPA) banding in two patients, pulmonary valvotomy, central shunt and right ventricular outflow tract reconstruction in one, pulmonary artery separation from truncus arteriosus and modified Blalock–Taussig shunt in one, and MPA closure in one. Two patients underwent a bidirectional cavo-pulmonary shunt before the one and a one half ventricle repair. Associated cardiac lesions were treated simultaneously. Results: There were no hospital deaths. All the patients were discharged home in good clinical conditions. There were no late deaths or reoperations. At mean follow-up of 29.8 months (range 8 months to 7.3 years) all the patients are alive and in good general conditions. MPA percutaneous balloon dilation was performed in two patients at 33 and 4 months, respectively, both after MPA reconstruction (which was previously ligated) and dilation of the left pulmonary artery branch in one patient, repeated twice at 10 and 14 months from repair, for a hypoplastic left pulmonary artery after truncus arteriosus repair. Conclusions: Surgical treatment of congenital cardiac anomalies in the presence of a hypoplastic and or a dysplastic RV by means of one and one half ventricle repair has the advantage of reducing the surgical risk for biventricular repair, and compared to the Fontan circulation, it maintains a low right atrium pressure, a pulsatile pulmonary blood flow and improves the systemic oxygen saturation. Short and medium-term results are promising. Longer follow-up is needed, to prove the efficacy of such a repair, in the long term.     

Pulmonary atresia with intact ventricular septum

Neonates with pulmonary atresia with intact ventricular septum are ductus dependent from birth. The initial approach in these patients is to ensure continued adequate pulmonary blood flow in anticipation of imminent spontaneous closure of the ductus. Our experience and evolving approach in the management of 16 consecutive neonates with pulmonary atresia with intact ventricular septum from 1978 to 1984 is presented. On the basis of the revised classification by Bull and associates, we divided the patients into four different groups according to right ventricular morphology. Basically, three types of palliative surgical procedures were performed: systemic-pulmonary artery shunt; systemic-pulmonary artery shunt with pulmonary valvotomy; and pulmonary valvotomy alone. Systemic-pulmonary artery shunt is the most important part of neonatal palliation. Our present routine is to perform left subclavian-main pulmonary artery shunt with a polytetrafluoroethylene tube in all patients and, in addition, to perform an open transpulmonary valvotomy without cardiopulmonary bypass in patients with a patent infundibular portion of the right ventricle. Our technique and the advantages of this type of shunt procedure are discussed. Patients with right ventricular sinusoidal-coronary artery communications are a subgroup with an additional problem. In our series, patients with this anomaly are categorized into groups with major and minor connections and their surgical significance is discussed. Overall, there was one death among 16 patients after neonatal palliative procedures and one death among five patients after hemodynamic repair.     

Strategy for pulmonary atresia and intact ventricular septum

Pulmonary atresia with an intact ventricular septum is characterized by varying degrees of right ventricular cavity hypoplasia. This factor is critical in determining the most appropriate surgical approach for each patient. We describe a patient who underwent definitive biventricular surgical repair in early infancy. We used an atrial septal defect patch with a one-way valve and performed a right ventricular overhaul after a balloon valvotomy.     

Creation of a One-Way Interatrial Communication in the Treatment of Critical Pulmonary Stenosis with Intact Ventricular Septum: A Case Report

A bstract Background : In biventricular repair of pulmonary outflow tract obstruction with intact ventricular septum, the right ventricle is loaded with total pulmonary blood flow acutely as the right-to-left shunt is abolished by closure of the atrial septal defect (ASD). Methods : We designed a one-way interatrial communication by creation of an atrial septal flap to reduce the excessive volume load of the right ventricle. Results : This procedure was successfully performed in a 3-year-old girl undergoing definitive biventricular repair for critical pulmonary stenosis associated with tricuspid stenosis and a small right ventricle. Conclusions : We believe that creation of a one-way interatrial communication might be a good alternative to adjustable ASD and/or bidirectional Glenn shunt in biventricular repair of critical pulmonary stenosis or pulmonary atresia with intact ventricular septum.     

Definitive repair in patients with pulmonary atresia and intact ventricular septum

Between 1977 and 1988, 22 patients underwent definitive repair for pulmonary atresia with intact ventricular septum. Fifteen underwent biventricular repair (mean age 24 months). All had mild to moderate right ventricular hypoplasia at the time of definitive repair. Repair consisted of closure of the atrial septal defect with enlargement of the right ventricular cavity and outflow tract with a patch in eight, insertion of a valved homograft in three, and superior vena cava-pulmonary artery connection in four. There was one operative death (7%). Seven patients had severe right ventricular hypoplasia at the time of definitive repair and underwent a Fontan procedure (mean age 46 months). In two a valved connection was made to the right ventricle and in five a right atrial-pulmonary artery nonvalved connection. There were two operative deaths (29%). Three patients had right ventricular-coronary sinusoids: Two of them underwent a Fontan procedure and one a biventricular repair. We conclude that with adequate early palliation a biventricular repair may be successfully performed for patients with mild or moderate right ventricular hypoplasia, and the Fontan procedure may be used for those with severe right ventricular hypoplasia.     

Pulmonary atresia with intact ventricular septum: is neonatal repair advisable?

The optimal management of pulmonary atresia with an intact ventricular septum in the neonate remains controversial. The introduction of balloon septostomy and prostaglandin has significantly reduced early mortality but early surgical intervention is necessary to obtain a more adequate pulmonary blood flow. Fourteen neonates with pulmonary atresia and an intact ventricular septum were admitted to the Wessex Cardiothoracic Unit, Southampton from 1979 to 1986. Thirteen patients underwent cardiac catheterization. Cardiac catheterization data and right ventricular angiograms were reviewed retrospectively. Four patients with tripartite ventricles underwent total repair. The others received various palliative operations (valvotomy + modified Blalock-Taussig shunt or modified Blalock-Taussig shunt alone). Retrospective analysis of the angiograms indicated that right ventricular morphology alone is not a satisfactory criterion for surgical management. We have been able to demonstrate that there is a good correlation between the diameter of the tricuspid valve and the diameter of the infundibulum and that successful neonatal repair is possible when the tricuspid valve diameter is above 80% of the normal value for weight and when the tricuspid valve diameter to infundibular diameter ratio (TV/Inf ratio) is 2.2 or less. In patients with a tripartite ventricle but inadequate TV diameter and TV/Inf ratio, a closed pulmonary valvotomy with a modified Blalock-Taussig shunt remains the treatment of choice.     

Biventricular repair for pulmonary atresia with intact ventricular septum associated with sinusoidal communications

Pulmonary atresia with intact ventricular septum (PA-IVS) is very difficult to treat due to the variety of right ventricular hypoplasia and coronary artery anomalies. Biventricular repair is viewed as possible in patients with mild right ventricular hypoplasia but not in those with severe right ventricular hypoplasia or with sinusoidal communication. We report a case of a 17-day-old boy with PA-IVS and severe right ventricular hypoplasia and large sinusoidal communications. We successfully conducted biventricular repair, ligated coronary artery and right ventricular fistulas, and implemented right ventricular outflow tract reconstruction. He now enjoys good health and attends elementary school. Sinusoidal communications are not always a limiting factor in biventricular repair for PA-IVS if the right ventricle can be decompressed without inviting ventricular dysfunction by ligating fistulas.     

Pulmonary atresia with intact ventricular septum: strategy based on right ventricular morphology

OBJECTIVES: Management strategy for pulmonary atresia with intact ventricular septum is controversial. We treated patients with this anomaly according to a management protocol based on our quantitative assessment of right ventricular morphology (index of right ventricular development and right ventricle-tricuspid valve index). In the present study, we reviewed a 20-year experience of the surgical treatment at our institution to define guidelines for selecting the appropriate type of surgical procedure. METHODS: Between April 1981 and June 2002, 45 consecutive patients with pulmonary atresia with intact ventricular septum underwent surgical treatment. Open transpulmonary valvotomy was performed in 27 patients and Blalock-Taussig shunt in 18 patients as the initial palliative procedure. Three patients who underwent a successful transpulmonary valvotomy alone in the neonatal period required no further operation. Definitive repair was performed in 32 patients. Biventricular repair was performed on 19 patients, one and a half ventricular repair in 3, and Fontan-type operation in 10. RESULTS: There were 1 early and 2 late deaths before the definitive operation in patients who underwent transpulmonary valvotomy. Two patients who had coronary artery interruption died 3 months and 13 years after the initial Blalock-Taussig shunt. There were 1 early and 2 late deaths after the definitive operation. Actuarial survival, including noncardiac death, was 91.1% at 5 years after the initial procedure and 81.5% at 10 years. CONCLUSIONS: We treated patients with pulmonary atresia with intact ventricular septum according to a management protocol based on our quantitative assessment of right ventricular morphology with good results.     

Right and left isomerism: the cardiac surgeon's view

We have treated by palliative or corrective surgery 12 patients with the complex cardiovascular anomalies associated with right isomerism (asplenia syndrome) or left isomerism (polysplenia syndrome). A systemic-pulmonary artery shunt was performed in 4 patients, with 2 early deaths. Anatomical intracardiac repair was successfully done in 2 patients, while an orthoterminal repair using a modified Fontan procedure was attempted in 5 patients, only 1 of whom survived. A 1-year-old girl with diffuse pulmonary arteriovenous fistulas associated with left isomerism was not considered a good candidate for operation after an exploratory sternotomy. The surgical options and technical problems related to the complex intracardiac morphology of these developmental syndromes are presented.     

Surgical palliation of cardiac malformations associated with right isomerism

Between 1985 and 1993, palliative surgery was performed on 13 pediatric patients who had complex cardiovascular anomalies associated with right isomerism. The patients included two neonates, ten infants, and one child who were divided into two groups according to whether or not a total anomalous pulmonary venous connection (TAPVC) was present. Group 1 consisted of six patients with TAPVC and group 2 consisted of seven patients without TAPVC. In group 1, the surgical procedures involved TAPVC repair alone in two patients, combined TAPVC repair with a modified Blalock-Taussig shunt in two, combined TAPVC repair with pulmonary artery banding in one, and a modified Blalock-Taussig shunt alone in one. There were five hospital deaths and one late death in this group: pulmonary venous obstruction in two patients, perioperative myocardial failure in the two neonates, and congestive heart failure caused by increased pulmonary blood flow in two patients. In group 2, all the patients underwent systemic-pulmonary artery shunts, and there was one hospital death and three late deaths, the causes of which were unknown in two patients, and shunt failure and pneumonia in one patient each. These results suggest that surgical palliation for right isomerism produces poor results in young infants with obstructed TAPVC. Thus, we conclude that TAPVC repair should be performed without delay if pulmonary venous obstruction has been diagnosed clinically. Resolving pulmonary venous obstruction without cardiopulmonary bypass (CPB) may be preferable for infants, considering their difficult management. The systemic-pulmonary artery shunt should be of the low-calibrated type, especially if common atrioventricular valve regurgitation exists. If infants survive the surgery, they must be carefully followed up for a long period due to the risk of sudden death or infection.     

The spectrum of double-outlet right atrium including hearts with three atrioventricular valves

Double-outlet right atrium (DORA) is characterized by simultaneous right atrial emptying into both ventricles. Ventriculoatrial septal malalignment is the cardinal morphological feature. Three cases are presented to depict two major types of DORA-DORA with a malaligned atrial septum and DORA with a malaligned ventricular septum. We describe two subtypes of each form of DORA: DORA with a malaligned atrial septum presents with either a common atrioventricular (AV) junction (guarded by a common AV valve) or with a single AV junction (due to the absence of the left AV junction). DORA with a malaligned ventricular septum may be associated with a right ventricle (RV) that is adequate for biventricular repair or a severely hypoplastic RV not compatible with biventricular repair. DORA with a malaligned ventricular septum is closely related to typical straddling of the tricuspid valve. Peculiarly, DORA with a malaligned ventricular septum presents three AV valves at the AV junction and is associated with an abnormal disposition of the AV conduction axis. Clear understanding of the morphology of these lesions is important in preventing a surgical misadventure at the crux of the heart.     

A successful case report of one and one half ventricle repair for pure pulmonary stenosis in a 4-year-old girl

A 4-year-old girl with pure pulmonary stenosis, hypoplastic right ventricle and atrial septal defect, underwent left modified Blalock-Taussig shunt at the age of 2 year. Her RVEDV was 62.7% of normal and TVD was 64.2% of normal at the age of 3 year. We observed development of right ventricle and performed simultaneously Glenn shunt and right ventricular outflow reconstruction (one and one half ventricle repair). Her general condition after operation became better. The size of tricuspid valve and right ventricle grew on Cardiac ultrasonography and catheterization examined after one year operation. In futur,If the size of RV and TV might grow further, we should recommend her biventricular repair.