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.     

Results of the Probe Technique for Transatrial Repair of Tetralogy of Fallot

Abstract 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.     

A new pulmonary valve cusp plasty technique markedly decreases transannular patch rate and improves midterm outcomes of tetralogy of Fallot repair

Objectives: To assess the operative and mid-term outcome of a very aggressive pulmonary annulus preservation strategy and pulmonary valve leaflet plasty technique which achieved 95% freedom of transannular patch (TAP) in complete tetralogy repair. Methods: From August 2006 through May 2010, 139 consecutive patients underwent repair of tetralogy of Fallot at a median age of 6.5 months, with median weight 7.2 kg. None of the patients had a prior shunt. A total of 132 patients (95%) had pulmonary valve annulus-sparing procedures, predominantly through a transatrial and transpulmonary approach (n = 120%, 86.3%). Special techniques included generous pulmonary supra-valve patch, bicuspid pulmonary leaflets plasty, or augmentation to maximally preserve pulmonary annulus. All the patients who survived were followed up closely. Results: Only seven (5.0%) patients had a TAP. Among them, five (71.4%) had doubly committed subarterial defect. None of the tricuspid pulmonary valves need TAP. Preoperative size of pulmonary annulus < –6 is associated with TAP (p < 0.001). A total of 12 (13.7%) patients needed repump to do either a ventriculotomy and patch the incision or a TAP to relieve residual stenosis. One patient needed a third pump run to do a TAP. The operative mortality was 0.7%, and there was one late death due to hemoptysis. The pulmonary regurgitation of non-TAP patients was less than mild in 105 (79.5%) and mild to moderate in 27 (20.5%) patients. Three patients had peak gradient greater than 50 mmHg across the right ventricle and pulmonary artery at 1-month follow-up, but the gradient dropped to less than 50 mmHg in two patients at 6-month follow-up. One patient had a persistent 50–55 mmHg gradient across pulmonary valve and he is under close follow-up. Conclusions: Excellent outcome can be achieved even with 95% freedom of TAP in complete repair of tetralogy. The function of most patients’ pulmonary valve was well preserved.     

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.     

Absent pulmonary valve syndrome: operation in infants with airway obstruction.

From 1979 through 1991, 19 infants with absent pulmonary valve syndrome and airway obstruction were seen for surgical treatment. All patients underwent extensive pulmonary artery aneurysmorrhaphy using cardiopulmonary bypass. Fourteen patients had simultaneous transatrial ventricular septal defect (VSD) closure, infundibular resection, and placement of a short transannular patch; 2 had transventricular VSD closure and infundibular resection without a transannular patch; 1 underwent transventricular VSD closure and transannular patching; and 2 underwent pulmonary artery aneurysmorrhaphy alone with the VSD left open. All 19 infants had good hemodynamics when taken from the operating theater, but 3 died postoperatively of severe airway obstruction, despite further tracheobronchopexy procedures in 2 (hospital mortality rate, 16%; confidence limits, 7% to 29%). Among the 16 patients discharged from the hospital, there was one late death. Five other patients have required reoperation for branch pulmonary artery stenosis (n = 2), residual airway obstruction resulting from persistent pulmonary artery dilatation (n = 1), closure of VSD (n = 1), and homograft valve insertion for pulmonary incompetence and right ventricular dysfunction (n = 1). There are 15 long-term survivors. Eight of them have episodic bronchospasm of mild to moderate severity, and all are responsive to sympathomimetic bronchodilator aerosols. The remaining 7 are asymptomatic.     

Simultaneous enlargement of the pulmonary annulus and the pulmonary cusp with autologous pericardium in right ventricular outflow tract reconstruction

BACKGROUND: Surgical repair of obstructive lesions of the right ventricular outflow tract (RVOT) commonly creates pulmonary valve incompetence, which continues to stimulate research for the optimal materials and surgical techniques to reconstruct RVOT. In this study, we present the early results with simultaneous enlargement of the pulmonary annulus and the pulmonary cusp with a transannular patch of autologous pericardium in RVOT reconstruction. PATIENTS AND METHODS: From January 2003 to December 2005, the surgical technique of simultaneous enlargement of the pulmonary annulus and the pulmonary cusp was used in 32 patients who had complex congenital heart anomalies with pulmonary artery hypoplasia. The functional status of the patients was followed up in the cardiologic clinic of our institute. The motion of the newly constructed valve and the degree of pulmonary insufficiency were evaluated by echocardiography before discharge and at 2-6 months, 12 months, and 36 months postoperatively. RESULTS: Early death occurred in one patient (3.1%). Postoperative complications occurred in six patients but they recovered uneventfully. During the follow-up, 28 of 31 operative survivors were in New York Heart Association functional class I without medication and the other three were in class II. Seventeen patients had no or trivial pulmonary regurgitation; mild regurgitation was present in 12 patients, and moderate regurgitation was seen in 2 patients. None of these patients needed reoperation and echocardiography showed good motion of the reconstructed valve. CONCLUSIONS: The surgical technique of simultaneous enlargement of the pulmonary annulus and the pulmonary cusp with a transannular patch of autologous pericardium is a safe, reliable, and effective way for RVOT reconstruction. Satisfactory early results have been achieved; however, long-term follow-up is necessary to determine the true value of this technique.     

Tricuspid valvectomy to facilitate repair of postinfarction ventricular septal defect

Abstract   Repair of postinfarction ventricular septal defect (VSD) located posteriorly can be a challenging procedure both in regard to operative mortality and recurrence of the defect. Avoidance of ventriculotomy by transatrial repair may decrease risks of bleeding and impairment of ventricular function secondary to suture placement; however, adequate exposure of the defect through the tricuspid valve is not always possible. We present a case of successful transatrial repair of posterior postinfarction VSD with concurrent tricuspid valvectomy and coronary artery grafting.     

Right atrial approach for surgical correction of tetralogy of Fallot

Total correction of tetralogy of Fallot was performed without a ventriculotomy in 39 patients aged 8 months to 39 years (mean age, 9.1 years) between May 1984 and July 1988. A transatrial approach was used to resect the obstructed infundibulum and to close the ventricular septal defect. In 14 patients, the pulmonary annulus was not enlarged (group 1). Twenty-five patients required a transannular patch (group 2), placed by extending the pulmonary artery incision 1 cm into the right ventricular infundibulum. Eleven patients had repair of pulmonary artery branch stenosis, and associated intracardiac anomalies were simultaneously corrected in 10 patients. After repair, the right ventricular to left ventricular systolic pressure ratios ranged from 0.36 to 0.59 (mean ratio, 0.45) in group 1 and 0.33 to 0.70 (mean ratio, 0.51) in group 2. There were no hospital or late deaths in group 1. Two patients in group 2 with a small left ventricle died shortly after operation. The 37 survivors were followed for 2 to 51 months. Postoperative catheterization in 7 patients detected no residual ventricular septal defects, mild pulmonary regurgitation in 2 patients (group 2), and right ventricular to left ventricular pressure ratios ranging from 0.25 to 0.42 (mean ratio, 0.34). Only 1 patient with a previous total repair by ventriculotomy is symptomatic and requires antiarrhythmic agents and diuretics. The other 36 patients are asymptomatic. In conclusion, tetralogy of Fallot can be safely repaired at any age without a ventriculotomy. The results indicate a minimal incidence of postoperative arrhythmias and pulmonary regurgitation, as well as improved right ventricular function.     

Repair of ventricular septal defect in adults

To evaluate the outcome of ventricular septal defect (VSD) with long duration of haemodynamic derangement, a retrospective study was made of 42 consecutive patients who underwent closure of VSD as adults (age range 15-48, mean 27 years). The mean systolic pulmonary arterial pressure was 53 mmHg, mean pulmonary vascular resistance 2.5 Wood units and mean pulmonary/systemic flow ratio 2.4. VSD was complicated by aortic regurgitation in 12 cases, mitral regurgitation in 4, and sinus of Valsalva fistula in 6 cases. There were 15 supracristal, 24 infracristal and 3 muscular VSDs. In addition to VSD closure, surgery included aortic valve replacement (7 cases), mitral valve replacement (2), valve repair by suture (7) and repair of Valsalva sinus fistula (6 cases). Two patients died in the early postoperative period and two during follow-up (1-10, mean 4.5 years). The early and the late mortality were related to large infracristal VSD, pulmonary hypertension and irreversible pulmonary vascular changes which could not be anticipated on the basis of high calculated shunt flow at preoperative catheterization. No patient with supracristal VSD died. Recurrent VSD was diagnosed in five patients, three of whom needed reoperation and recovered uneventfully. Reduction of heart size and improved exercise tolerance were the most pertinent follow-up findings. The results suggest that large supracristal VSD with aortic valve involvement can be successfully closed in adults, but that the prospect for large infracristal VSD is less favourable if correction is postponed until adulthood.     

Late repair of the native pulmonary valve in patients with pulmonary insufficiency after surgery for tetralogy of Fallot

Pulmonary regurgitation developing late after tetralogy of Fallot repair is now recognized as a serious threat to the long-term welfare of these patients. This article summarizes our experience with 5 patients who underwent reoperations for treatment of severe pulmonary regurgitation after transannular patch repair of tetralogy of Fallot. In each case, the intraoperative findings revealed anatomy favorable for valve repair and enabled preservation of the native pulmonary valves.     

Early results after transatrial/transpulmonary repair of tetralogy of Fallot.

OBJECTIVES: Right ventricular (RV) dysfunction is a significant cause of morbidity and mortality after surgical correction of tetralogy of Fallot (TOF). Transatrial/transpulmonary repair avoids a ventriculotomy (in contrast to the transventricular approach) emphasizing maximal preservation of RV structure and function. We have adopted this technique as less traumatic for the right ventricle. This study evaluates the early surgical results of our approach. METHODS: Between September 1997 and July 2001, 110 consecutive patients with TOF were referred to our unit for surgical therapy. Of these, 14 were unsuitable for repair and underwent aortopulmonary shunting+/-pulmonary artery patching. In the remaining 96 patients (median age 1.4 years), complete transatrial/transpulmonary repair was performed. Previously placed shunts (ten patients) were taken down and any secondary stenoses or branch pulmonary artery distortion repaired. In all cases, subpulmonary resection and ventricular septal defect (VSD) closure were accomplished transatrially. Whenever pulmonary valvotomy and valve ring widening were necessary, it was achieved through a pulmonary arteriotomy. In 84 patients the main pulmonary artery was augmented with an autologous pericardial patch, and in 23 the patch was extended to pulmonary artery branch(es). A limited (<1cm ) or extended (>1cm, but 相似文献   

Right ventricular dysfunction and pulmonary valve replacement after correction of tetralogy of Fallot

Background. Correction of tetralogy of Fallot often leads to pulmonary regurgitation, sometimes warranting pulmonary valve replacement, for which indications and timing to achieve optimal results are not yet clear. This retrospective study describes follow-up and reinterventions in our tetralogy of Fallot population.

Methods. Review of all consecutive patients operated on for tetralogy of Fallot between 1977 and 2000 was conducted. Included are date and type of repair, Doppler echocardiography (two-dimensional echocardiography), electrocardiographs, reoperations, and physical condition.

Results. Total repair was performed in 171 patients at a mean age 1.9 ± 2.5 years, follow-up time counted 9.6 ± 7.0 years. Right ventriculotomy was used in 92%, and transatrial ventricular septal defect closure was used in 8%; 74% received a transannular outflow patch. Twenty-year survival was 91%. Last follow-up electrocardiographs showed right bundle branch block in 67% and serious arrhythmias in 11%. Two-dimensional echocardiography demonstrated severe pulmonary insufficiency and dilated right ventricle in 31% and 38%, respectively, increasing with postrepair age (p < 0.001). Poor clinical condition (New York Heart Association class II+) and echocardiographic proof of right atrial dilatation (p = 0.012) and arrhythmias (p = 0.03) were significantly associated. Furthermore, the influence of residual hemodynamic lesions, such as a remaining ventricular septal defect or pulmonary stenosis, or right ventricular dilatation was important (p = 0.04). Reintervention was necessary in 32 patients (19%; 10-year freedom, 83%), including angioplasty for residual stenosis and pulmonary valve replacement. At a mean age of 9.2 years after correction, 14 patients received a homograft, and 2 patients received a heterograft. In 7 patients the right ventricle returned to normal dimensions and symptoms disappeared. The incidence of right ventricular dilatation was considerably higher (p = 0.020) in patients with a transannular patch; the transatrial approach showed the opposite (p = 0.03), and patients presented with lower QRS duration (p = 0.007), although no difference could be found between survival after both surgical techniques. Effects of early timing (correction < 6 months) on right ventricular dysfunction could not be established.

Conclusions. Severe right ventricular dilatation and pulmonary regurgitation secondary to outflow tract repair in tetralogy of Fallot are frequently occurring sequelae developing slowly over time. Indications for pulmonary valve replacement remain controversial because echocardiographic findings or arrhythmias are not always accompanied by deterioration of clinical condition. However, right atrial dilatation and additional hemodynamic lesions demand increased vigilance. Transatrial repair is associated with a favorable outcome.     

Double-barrel right ventricular outflow: tetralogy of fallot annulus preservation technique

In patients with tetralogy of Fallot (TOF) repair and a borderline pulmonary valve annulus (PVA) size, surgical repair often necessitates a transannular incision and subsequent placement of a patch with or without a monocusp or, alternatively, a right ventricle-to-pulmonary artery conduit. We discuss here a technique in which the pulmonary valve annulus can be safely preserved, with infrequent postoperative issues as well as the potential for less incidence of right ventricular outflow intervention in the long term.     

Congenital quadricuspid aortic valve with tetralogy of Fallot and pulmonary atresia

We report the case of a 4-year-old girl who had quadricuspid aortic valve regurgitation with tetralogy of Fallot and pulmonary atresia. This combination is very uncommon. Aortic valve replacement was performed successfully due to aortic regurgitation which had progressed one year after the total repair. The dilated aortic annulus plus quadricuspid aortic valve may result in progressive aortic regurgitation for a short period.     

Fan-shaped expanded polytetrafluoroethylene valve in the pulmonary position

BACKGROUND: Residual pulmonary insufficiency remains a problem after transannular repair in pediatric patients. We have developed fan-shaped monocuspid and bicuspid valves made of expanded polytetrafluoroethylene (ePTFE) membrane for repair of right ventricular outflow tract obstruction in patients with tetralogy of Fallot. METHODS: We studied 20 patients (mean age, 39.6 months) with tetralogy of Fallot. The right ventricular outflow tract was reconstructed with use of a short, wide transannular patch and a fan-shaped valve made of 0.1-mm-thick expanded ePTFE membrane (PRECLUDE Pericardial Membrane, W.L. Gore & Associates, Inc, Flagstaff, AZ). The width of the valve and the outflow patch were constructed so that the neopulmonary annulus was approximately 130% the normal size. RESULTS: A monocuspid valve was used in 17 patients and a bicuspid valve in 3. The mean follow-up time was 32.3 months (range, 14.7 to 56.7 months). Right ventricular function was well maintained in all patients. Valvular motion remained competent in 13 patients (65%). In the remaining 7 (35%), the valve became fixed in the open position. Trivial and mild pulmonary insufficiency was detected in 10 patients with a competent valve and 7 with an incompetent valve. No pressure gradient across the neopulmonary annulus or calcification of the expanded ePTFE membrane was detected in any patient. CONCLUSIONS: The fan-shaped expanded ePTFE valve is a useful substitute with adequate function in transannular right ventricular outflow reconstruction.     

Half-turned truncal switch operation for transposition of great arteries with ventricular septal defect and pulmonary regurgitation

A three-month-old girl weighing 4.2 kg, diagnosed with transposition of the great arteries (TGA) and ventricular septal defect (VSD) was referred to us. She had normal-sized pulmonary annulus and moderate pulmonary regurgitation. Because her pulmonary valve was not suitable for systemic circulation due to valvular incompetence, the half-turned truncal switch operation was selected. The postoperative course was uneventful without left or right ventricular outflow obstructions over a year of follow-up. Our report demonstrated that the TGA and VSD with normal pulmonary annulus is not contraindicated for half-turned truncal switch operation.     

Pulmonary valve preservation in Tetralogy of Fallot with a mildly hypoplastic annulus-should we do it?

Background  

The decision to preserve the pulmonary valve during intracardiac repair of Tetralogy of Fallot [TOF] is traditionally based on the intra-operative measurement of pulmonary annulus by a Hegar dilator as per Rowlatt’s table. We sought to evaluate if there can be flexibility in not using a transannular patch repair in Indian population with mildly hypoplastic pulmonary annulus.     

Tricuspidisation of the aortic valve with creation of a crown-like annulus is able to restore a normal valve function in bicuspid aortic valves

Objective: To evaluate the early results of a new method to repair malfunctioning bicuspid aortic valves by creating a tricuspid valve with a crown-like (i.e. anatomic) annulus. Material and methods: Twelve patients (ages from 10 to 27 years) with chronic regurgitation (and flow-dependent stenosis) of a bicuspid aortic valve underwent repair with the principle of creating a tricuspid valve and a crown-like annulus. The fused leaflets were trimmed and reinserted underneath the existing aortic annulus to create one new native cusp. The third leaflet was fashioned out of a xenopericard patch and was inserted underneath the existing annulus as well to restore the crown-like anatomy of a normal aortic annulus. A tricuspid aortic valve with a morphologically normal annulus was thus created, which resulted in improved coaptation of the leaflets. The repair was immediately assessed by transesophageal echocardiography (TEE) with the heart loaded at 50%. In two patients, a second run helped fine-tune the repair. Median cross-clamping time was 82 min. Follow-up ranged from 3 to 46 months (median 13 months). Results: No significant complication occurred. The function of the aortic valve was excellent with trivial or mild regurgitation in 11 patients and moderate regurgitation in 1 patient. There was no stenosis across the valve. The repair remained stable over time. Remodelling of the left ventricle occurred as expected. Conclusions: Aortic valve repair is feasible in some dysfunctioning bicuspid aortic valves. Tricuspidisation of the valve can result in excellent systolic and diastolic functions. The creation of a crown-like annulus results in improved coaptation of the cusps and could lead to more reliable outcome. Although long-term results are needed, this anatomic correction seems to be a good alternative to valvular replacement in certain sub-groups of patients.     

Surgical management of patients with pulmonary valve dysplasia

Pulmonary valve dysplasia is a distinct pathological entity consisting of markedly thickened, deformed, and largely immobile pulmonary valve leaflets. The clinical features and surgical management of 21 consecutive patients operated upon between 1975 and 1985 were reviewed. Mean age at operation was 26.7 months (range: 1 month to 9.5 years). Initial surgical intervention in 3 patients consisted of closed valvotomy or systemic pulmonary artery shunt. One of these patients subsequently had a partial pulmonary valvectomy; the other 2 had total valvectomy and pulmonary annuloplasty. Two patients had partial valvectomy as a primary procedure, and 1 has subsequently required total valvectomy and annuloplasty. Sixteen patients initially had total pulmonary valvectomy, 4 with a simultaneous transannular outflow tract patch. One patient subsequently required a transannular outflow tract patch. Therefore, definitive repair consisted of partial valvectomy in 2 patients, total valvectomy in 19, and a transannular outflow tract patch in 9 patients with a hypoplastic pulmonary annulus. There were no operative deaths, and all patients are asymptomatic at a mean follow-up of 37.9 months. Five patients who underwent total valvectomy were catheterized postoperatively. The mean residual gradient was 25 mm Hg. Pulmonary valvectomy appears to provide both effective relief of stenosis and long-term asymptomatic status in patients with pulmonary valve dysplasia. Total excision of all valve leaflets is recommended. Careful consideration should be given to a simultaneous transannular outflow tract patch if the annulus is hypoplastic.