Clinical features in a family with an R460H mutation in transforming growth factor beta receptor 2 gene.

OBJECTIVES: To describe the clinical findings and natural history in 22 carriers of an R460H mutation in the transforming growth factor beta receptor 2 gene (TGFbetaR2) from a five-generation kindred ascertained by familial aortic dissection. METHODS: 13 of the confirmed carriers were interviewed and examined, and information about the remaining carrier was obtained from medical records. Clinical information about deceased individuals was obtained, when possible, from postmortem reports, death certificates and medical records. RESULTS: There have been eight sudden deaths; the cause of death was aortic dissection in all six cases in which a postmortem examination was performed. Three individuals had undergone aortic replacement surgery. Dissection had occurred throughout the aorta, and in one case in the absence of aortic root dilatation. Subarachnoid haemorrhage, due to a ruptured berry aneurysm, had occurred in two individuals. Four gene carriers and one deceased family member who were investigated had tortuous cerebral blood vessels. One had tortuous vertebral arteries, two had tortuous carotid arteries and one a tortuous abdominal aorta. Two individuals were found to have a brachiocephalic artery aneurysm and a subclavian artery aneurysm, respectively. CONCLUSIONS: Despite the predisposition to aortic dilatation and dissection, individuals did not frequently manifest the skeletal features of Marfan syndrome, with the exception of joint hypermobility. No one individual had ocular lens dislocation. Striae and herniae were common. There was some overlap with Ehlers-Danlos syndrome type 4, OMIM 130050, with soft translucent skin, which is easily bruised. Other features were arthralgia, migraine and a tendency to fatigue easily, varicose veins and prominent skin striae. This family provides further evidence that mutations in TGFbetaR2 cause a distinct syndrome that needs to be distinguished from Marfan syndrome to direct investigation and management of patients and shows the natural history, spectrum of clinical features and variable penetrance of this newly recognised condition.

Electrophysiological mapping and ablation of intra-atrial reentry tachycardia after Fontan surgery with the use of a noncontact mapping system.

BACKGROUND: Atrial tachyarrhythmias are a complication of Fontan surgery. Conventional electrophysiological mapping and ablation techniques are limited by the complex anatomic and surgical substrate and a high arrhythmia recurrence rate. This study investigates the use of noncontact mapping to identify arrhythmia circuits and guide ablation in Fontan patients. METHODS AND RESULTS: Eleven arrhythmias were recorded in 6 patients. Noncontact mapping improved recognition of the anatomic and surgical substrate and identified exit sites from zones of slow conduction in all clinical arrhythmias. Radiofrequency linear lesions were targeted across these critical zones in 5 patients. One patient underwent surgical cryotherapy. Although immediate success was achieved in 3 of 5 patients with radiofrequency ablation, 2 patients had a recurrence after a mean of 6.4 months of follow-up. The patient who underwent cryoablation remains free of arrhythmias. CONCLUSIONS: Noncontact mapping can identify arrhythmia circuits in the Fontan atrium and guide placement of ablation lesions. Arrhythmia recurrence is high, possibly because of inadequate lesion creation rather than inaccurate mapping and lesion targeting.

The value of Doppler color flow mapping in determining pulmonary blood supply in infants with pulmonary atresia with ventricular septal defect.

Thirty-two neonates and infants with pulmonary atresia with ventricular septal defect were initially investigated with cross-sectional and spectral Doppler echocardiography and Doppler color flow mapping. All 32 had subsequent correlative angiography. This demonstrated that 24 infants had adequate-sized right and left pulmonary arteries (19 confluent, 5 nonconfluent). Of the five infants with nonconfluent pulmonary arteries, four had bilateral ductus arteriosus and one had a single left-sided ductus with anomalous origin of the right pulmonary artery from the ascending aorta. Nineteen infants had confluent pulmonary arteries, all of which were supplied by a single ductus. Eight infants had complete absence of or inadequate pulmonary arteries; all had multiple aortopulmonary collateral vessels arising from the descending aorta. The presence of adequate-sized right and left pulmonary arteries was correctly predicted in 21 of 24 infants by cross-sectional echocardiography alone and in all 24 by Doppler color flow mapping. Confluence of the right and left pulmonary arteries was predicted by cross-sectional imaging in 14 of the 19 infants in whom it occurred, and by Doppler color flow mapping in all 19 infants. The precise definition of the pulmonary blood supply was correctly predicted by Doppler color flow mapping in 16 of the 19 infants with confluent pulmonary arteries and a single ductus. However, in three infants in this group, Doppler color flow mapping made a false diagnosis of multiple aortopulmonary collateral vessels.(ABSTRACT TRUNCATED AT 250 WORDS)

Late noninvasive evaluation of cardiac performance in mildly symptomatic older patients with Ebstein's anomaly of tricuspid valve: role of radionuclide imaging.

Ten patients 8 to 54 years of age with isolated Ebstein's anomaly of the tricuspid valve were evaluated by electrocardiography, maximal exercise treadmill testing, 24 h electrocardiographic (ECG) monitoring, echocardiography and rest radionuclide imaging of the left ventricle. The patients presented after the 1st year of life and had not undergone surgical intervention. All except one were in functional class II. No patient had preexcitation on the surface ECG, but abnormal tachyarrhythmias or bradyarrhythmias were seen in five patients on 24 h ECG monitoring. Subnormal exercise performance was observed in five patients. Echocardiography demonstrated typical variable tricuspid valve displacement and paradoxic interventricular septal motion. Left ventricular end-diastolic dimensions were normal in all patients, but posterior wall motion was reduced in two. Moderate to severe tricuspid regurgitation with a Doppler jet velocity less than 2.5 m/s was demonstrated in eight patients. Left ventricular radionuclide scintigraphy revealed a subnormal ejection fraction (less than 50%) in 5 of 10 patients; these 5 had previously shown suboptimal exercise performance. The two youngest patients (less than 15 years) had no arrhythmia, normal exercise performance and normal left ventricular ejection fraction. There was no correlation between the degree of tricuspid valve displacement or regurgitation and the presence of rhythm disturbance, exercise performance or radionuclide left ventricular function. Late evaluation of patients with Ebstein's anomaly may demonstrate significant unsuspected abnormalities in cardiac rhythm, exercise performance and left ventricular function. Radionuclide scintigraphy is a useful noninvasive technique for assessing left ventricular dysfunction in these patients.

Influence of bidirectional superior cavopulmonary anastomosis on pulmonary arterial growth.

Right-sided BSCA provides for satisfactory pulmonary arterial growth in infants and children with complex congenital heart defects, and it could enhance the growth of a small right pulmonary artery. The growth of the left pulmonary artery, particularly in younger patients, needs close attention to confirm the safe role of BSCA in long-term palliation.

Right ventricular function and exercise performance late after primary repair of tetralogy of Fallot with the transannular patch in infancy.

To evaluate the late effects of chronic pulmonary regurgitation against the putative benefits from the current surgical trend of primary repair of tetralogy of Fallot with a transannular patch in infancy, 10 patients > 10 years after early primary repair and 7 matched normal controls underwent exercise stress test and cine magnetic resonance imaging assessment of ventricular functions. Right ventricular impaired diastolic function and decreased exercise capacity, both significantly associated with pulmonary regurgitation in patients, indicated that early primary repair of tetralogy may not prevent late ventricular dysfunction and diminished exercise performance if chronic regurgitation results from right ventricular outflow tract reconstruction.

Anatomy of coronary arteries in univentricular hearts and its surgical implications.

The coronary arterial anatomy in 26 univentricular hearts, its relation to the morphologic characteristics of the ventricles and rudimentary chambers, and its surgical implications were analyzed. All of the hearts except two had been operated on; 18 had septation with or without an extracardiac conduit and 6 had had palliative procedures. Twenty-one univentricular hearts with a left ventricular type main chamber had an anterior outlet chamber (17 left-sided subaortic, 3 right-sided subaortic and 1 right-sided subpulmonary). Right and left delimiting arteries outlined the outlet chamber in 16 hearts (76 percent). In 20 of the 21 hearts, large delimiting parallel branches of the right coronary artery course over the anterior wall of the heart; 13 of these vessels had been injured surgically with resultant ischemic myocardial necrosis. Five univentricular hearts did not have an outlet chamber; two had a left ventricular type main chamber and three had a morphologically right ventricular main chamber. Three of the five hearts had rudimentary pouches, located anteriorly in one and posteriorly in two. The two rudimentary pouches lying posteriorly were not outlined by delimiting arteries. Two of the five univentricular hearts without an outlet chamber also had injured coronary arteries. Thus, the identification of outlet chambers and rudimentary pouches in univentricular hearts is facilitated by the determination of coronary anatomy. The presence of major delimiting parallel branches over the usually favored ventriculotomy sites renders them vulnerable to surgical injury; such mishaps occurred in 15 of the 24 hearts that had either corrective or palliative operations.

Correction of interrupted aortic arch.

Twelve consecutive infants with interrupted aortic arch, ventricular septal defect, and persistent ductus arteriosus have had the anomaly repaired without the use of synthetic grafts. In two infants (5 and 9 months) the ductus arteriosus was used for the arch repair. In three patients (mean age 14 days) the left carotid artery was turned down to form the new arch. In the remaining seven (mean age 12 days) a direct anastomosis was achieved, but one of these patients died at operation. Two others in this group also had persistent truncus arteriosus. Five patients have required another operation (two for stenosis of the anastomosis with one death). The 10 survivors (mean follow-up 5 years) are well and support our belief that complete repair without the use of synthetic grafts is the treatment of choice in this rare and difficult group.

The failing Fontan circulation: successful conversion of atriopulmonary connections.

OBJECTIVES: Symptoms from low cardiac output or refractory atrial arrhythmias are complicating atriopulmonary (classical) Fontan connections. We present our experience of converting such patients to total cavopulmonary connections with and without arrhythmia surgery. METHODS: Between 1997 and 2002, 15 patients (mean age, 19.7 +/- 7.0 years) underwent conversion operations 12.7 +/- 3.5 years after atriopulmonary Fontan operations. Preoperative New York Heart Association functional class was I in 2 patients, II in 2 patients, III in 6 patients, and IV in 5 patients. Four patients underwent intracardiac lateral tunnel conversion alone, and 11 received extracardiac total cavopulmonary connection, right atrial reduction, and cryoablation. RESULTS: No mortality occurred. One patient had conduit obstruction in the immediate postoperative period requiring replacement, and another required a redo operation for endocarditis. Average hospitalization was 17.9 +/- 9.38 days; chest drains were removed on median day 4 (range, 1-29; mean, 7.4 +/- 7.58 days). At follow-up (mean, 42.6 +/- 22.1 months), late atrial arrhythmias had recurred in 3 of 4 patients with intracardiac total cavopulmonary connections (without ablation) and 1 of 11 patients with extracardiac total cavopulmonary connections with ablation. All patients are in New York Heart Association class I or II. Exercise ability (Bruce protocol) improved 69% from a mean of 6.18 +/- 4.01 minutes to 10.45 +/- 2.11 minutes (P <.05). Need for antiarrhythmic agents decreased postoperatively (patients receiving < or =1 antiarrhythmic: 9 preoperatively vs 15 at long-term follow-up, P <.05). No patient has required transplantation. Protein-losing enteropathy, which was present in 1 patient, improved transiently with conversion. There was 1 late death from gastrointestinal hemorrhage. CONCLUSIONS: Fontan conversion can be achieved with low mortality and improvement in New York Heart Association class and exercise ability. Concomitant arrhythmia surgery reduces the incidence of late arrhythmias.

Histological changes in the left and right ventricle in hearts with Ebstein's malformation and tricuspid valvar dysplasia: A morphometric study of patients dying in the fetal and perinatal periods.

Both Ebstein's malformation and the related tricuspid valvar dysplasia are often associated with tricuspid regurgitation, and impaired right ventricular function may develop. Impaired function of the left ventricle in Ebstein's malformation has also been described. Interstitial fibrosis has been shown in the right and left ventricles of hearts with Ebstein's malformation from neonates, children, and adults. The objective of this study was to determine whether interstitial fibrosis seen in Ebstein's malformation is an intrinsic part of the congenital malformation or is acquired. From the fetal and perinatal periods, we compared 13 hearts with Ebstein's malformation (6 isolated and 7 with additional abnormalities) and 11 with tricuspid valvar dysplasia (3 isolated and 8 with additional abnormalities) with 16 controls. Three adult cases of isolated Ebstein's malformation in patients aged 17 to 20 years, were compared with 5 controls. The percentage of interstitial fibrous tissue and the thickness of the endocardium in the right and left ventricles were measured using histomorphometry. There were similar findings in Ebstein's malformation and tricuspid valvar dysplasia. Of 24 fetal and perinatal cases, 23 had normal interstitial fibrous tissue. Interstitial fibrosis was found in the right ventricle of only 1 perinatal heart with Ebstein's malformation and pulmonary stenosis. Of the 9 fetal cases, 4 had minimal right ventricular endocardial thickening (up to 10 µm). The left ventricular endocardium was normal in this group. Of the 6 perinatal cases with isolated Ebstein's malformation or tricuspid valvar dysplasia, 4 had right and 2 had left ventricular endocardial thickening (up to 345 µm). Of the 3 adult hearts with Ebstein's malformation, 2 had right ventricular endocardial thickening (47 and 225 µm) and 2 had right and 1 had left ventricular interstitial fibrosis. These results indicate that in both Ebstein's malformation and tricuspid valvar dysplasia the endocardial thickening develops in perinatal life, and in Ebstein's malformation the interstitial fibrosis develops in later life.

Repair of truncus arteriosus in early infancy with antibiotic sterilized aortic homografts.

BACKGROUND: To evaluate the fate of the truncal valve, the antibiotic sterilized aortic homografts, and the survival after repair of truncus arteriosus in the first 6 months of life. METHODS: Between 1974 and 1994, 23 infants (mean age 1.7 months, range 5 days to 6 months) underwent primary repair of truncus arteriosus by one surgeon (J.L.M). Sixteen were neonates (age range 5 to 30 days). Continuity between the right ventricle and the pulmonary artery was established with an aortic antibiotic sterilized homograft (mean diameter 14.9 mm, range 11 to 17 mm). Follow-up was 100% complete. RESULTS: Four neonates with severe truncal regurgitation died early (17.4%). Fourteen patients underwent reoperations. Five had a truncal valve replacement (mean time 7 years, range 6 months to 17 years). Ten-year freedom from truncal valve replacement was 78.2%. Eleven patients had homograft replacement (mean time 12.7 years, range 2 to 26.2 years). Ten-year freedom from homograft replacement for any cause was 77.1% (for homograft-related problems it was 86.7%). Seven patients retained the original homografts (mean time 14.3 years, range 6 to 18.7 years). There was one late death. Overall 10-year survival was 79% and for the hospital survivors it was 95%. All survivors are in New York Heart Association functional class I. CONCLUSIONS: Abnormal truncal valves pose serious early and late problems but the patients with normal truncal valves do well and seem unlikely to need replacement of these valves. The durability of the antibiotic sterilized aortic homograft even in sizes less than 14 mm is remarkably good. Late survival is excellent.

Growth potential in the new aortic arch after non-end-to-end repair of aortic arch interruption in infancy.

BACKGROUND: Complete repair of infants with interrupted arch and ventricular septal defect through a midline incision has been the preferred method for more than 20 years. End-to-end anastomosis can result in restenosis if there is excess tension. Two methods of reducing this tension have been described, and the subsequent growth of the new aortic arch is demonstrated. METHODS: In 2 infants (5 and 9 months old) the duct was used to create a new aortic arch. In 3 other younger infants the left carotid artery was divided, turned down, and anastomosed to the descending aorta to form the new arch. These operations were performed through the midline at the same time as the ventricular septal defect was closed. RESULTS: All 5 patients are well now 8 to 19 years postoperatively. One patient required reoperation for stenosis at the anastomotic site, but all have subsequently shown good growth on follow-up angiographic and magnetic resonance imaging studies. CONCLUSIONS: Although end-to-end repair is best, these alternative methods have shown very satisfactory aortic growth into adult life.

Open commissurotomy for critical isolated aortic stenosis in neonates.

BACKGROUND: The optimal management of critical aortic stenosis in early infancy remains controversial. The aim of this study was to assess the early and late outcomes following open surgical valvotomy for critical aortic stenosis in neonates and to provide a framework of data against which current results of other treatment approaches can be evaluated. METHODS: Eighteen consecutive neonates (mean age 9.2 days, range 1 to 26 days) undergoing an open valvotomy for critical isolated aortic stenosis (the standard treatment for this condition in our unit) between 1984 and 2000 were studied. The mean aortic valve gradient was 79.4 mm Hg. Twelve neonates received prostaglandins and 10 received inotropic agents preoperatively. Follow-up was complete (mean 8.1 years, range 1 month to 15 years). RESULTS: There was no operative mortality. At discharge, the mean aortic valve gradient was 37.2 mm Hg, with 6 patients having mild and 2 having moderate aortic regurgitation. Six patients required a reoperation; 3 of these had an aortic valve replacement at 9 to 11 years of age. Kaplan-Meier 5- and 10-year freedoms from any aortic reoperation or reintervention were 85 and 55%, respectively; 5- and 10-year freedoms from aortic valve replacement were 100 and 79%, respectively. A 14-year-old boy died from endocarditis 4 years following an aortic valve replacement in another unit. Kaplan-Meier 10-year survival was 100%. All survivors are in New York Heart Association I class and are leading normal lives. Their mean aortic valve gradient is 34.5 mm Hg, and none has significant aortic regurgitation. CONCLUSIONS: Open valvotomy for critical aortic stenosis in neonates carries a low operative risk and provides lengthy freedom from recurrent stenosis or regurgitation. Reoperations are inevitable, but aortic valve replacement can be delayed until the implantation of an adult-sized prosthesis is possible. Late survival is excellent. We consider open surgical valvotomy to be the treatment of choice for critical neonatal aortic stenosis.

Outcome after repair of tetralogy of Fallot in the first year of life.

BACKGROUND: The purpose of this study was to evaluate the early and late outcome after repair of tetralogy of Fallot in the first year of life. METHODS: Between 1974 and 2000, 89 consecutive infants with a mean age of 6.3 +/- 2.6 months (range, 15 days to 12 months) underwent repair of tetralogy of Fallot (ventricular septal defect and pulmonary stenosis) by one surgeon (J.L.M.). Three infants had previous palliative operations. Sixty-seven procedures were urgent or emergency. A transannular patch was inserted in 69 patients (77.5%). Follow-up was complete, averaging 13.4 +/- 5.6 years (range, 0 to 25.4 years). RESULTS: There was one operative death (1.1%). Mean right ventricular to left ventricular pressure ratio postoperatively was 0.4 +/- 1.1 (in 79 patients, < 0.5). Fourteen patients underwent reoperations or reinterventions. There were no reoperations for residual or recurrent ventricular septal defect. Kaplan-Meier freedom from reoperation or reintervention for any cause at 20 years was 85% +/- 4.4%, for relief of right ventricular outflow tract obstruction it was 94% +/- 3.1%, and for pulmonary valve replacement this was 95.4% +/- 2.6%. Use of a transannular patch did not significantly affect the need for reoperation or reintervention. There was one late death (leukemia). Kaplan-Meier 20-year survival was 97.8% +/- 1.9%. On latest echocardiography, 42 patients had moderate pulmonary regurgitation, 4 had a right ventricular outflow tract gradient more than 40 mm Hg, and 86 had good biventricular function. Twelve-lead electrocardiography was performed in all and 24-hour electrocardiography in 61 patients. One patient (1.1%) exhibited late recurrent ventricular tachycardia requiring implantation of a defibrillator. The remaining 86 patients are in New York Heart Association class I with none of them receiving antiarrhythmic medications. CONCLUSIONS: These data strongly support the concept of early repair of tetralogy of Fallot. It is associated with an acceptable operative risk and a low incidence of significant arrhythmias, and provides long-term survival similar to that observed in the general population. Late complications may, however, develop, and long-term follow-up for their early recognition is essential.

Aortic valve repair of congenital stenosis with bovine pericardium.

BACKGROUND: Conservative surgical options in the treatment of congenital aortic stenosis are limited. To relieve the obstruction necessitates full incision of the raphe of the larger valve leaflet, but this inevitably causes prolapse. METHODS: We performed aortic valve repair in 6 children, aged 14 months to 17 years, with congenital aortic stenosis, 2 having had aortic valvotomy as infants. The repair consisted of suturing the base of a triangular piece of bovine pericardium, with a simple vertical fold, to the free edges of the incised raphe. The pericardial fold was then sutured vertically to the aortic wall. RESULTS: At follow-up of 2 to 60 months, the mean peak systolic Doppler gradients had decreased from 80 +/- 15 mm Hg to 26 +/- 9 mm Hg. The effective valvular orifice area increased from 33% +/- 6% to 64% +/- 3%, allowing blood flow to increase by a factor of 3.76. Two patients have mild and 2 have mild-to-moderate aortic regurgitation. CONCLUSIONS: The described conservative repair renders the valve tricuspid and trisinusoidal, and the deficient interleaflet triangle is recreated, preventing cusp prolapse. Longer follow-up is required to assess the durability of unstented pericardium in the aortic position, but the early results are encouraging.

Bidirectional superior cavopulmonary anastomosis: how young is too young?

OBJECTIVE: To define the lowest age at which the bidirectional superior cavopulmonary anastomosis can safely be used in infants with complex congenital heart defects. DESIGN: A retrospective analysis of clinical, echocardiographic, haemodynamic, and angiographic data in four consecutive patients undergoing bidirectional superior cavopulmonary anastomosis below the age of 2 months. PATIENTS: Between November 1990 and September 1993, four infants less than 8 weeks of age (3, 4, 6, and 7 weeks) underwent bidirectional superior cavopulmonary anastomosis as a primary palliation for complex heart disease. The indication for early surgical intervention was progression of cyanosis (n = 3) and high pulmonary blood flow causing heart failure (n = 1). In two infants with tricuspid atresia, surgery was performed through a right thoracotomy without the use of cardiopulmonary bypass. In one infant with double inlet left ventricle and discordant ventriculoarterial connection, cavopulmonary anastomosis was combined with an arterial switch procedure. The final infant had double inlet left ventricle with pulmonary atresia; the central pulmonary arteries were virtually discontinuous and each branch was supplied by a separate arterial duct. The central pulmonary arteries were reconstructed using the subaortic innominate vein. Temporary prostacyclin infusion was used in three patients in the immediate postoperative period. RESULTS: Early postoperative extubation (5, 7, and 48 h) was successful in three patients. The youngest child required ligation of the ductus arteriosus four days later because of severe upper body oedema. The postoperative course was complicated by prolonged effusions in two patients. All were alive and well 14-48 months postoperatively, with satisfactory systemic saturations (80-87%) and haemodynamic indices. CONCLUSIONS: This limited experience challenges the widely held belief that the bidirectional superior cavopulmonary anastomosis cannot be used as a primary palliation for complex heart disease in early infancy. A wider experience is required to determine the safety and indications for this approach.

Atrioventricular septal defect with a well developed primary component of the atrial septum ("septum primum").

A heart is described with an atrioventricular septal defect in which the lower part of the atrial septum ("septum primum") was well developed and in which superior and inferior bridging leaflets were attached neither to the crest of the ventricular septum nor the inferior rim of the atrial septum. Diagnostic pre-operative angiographic and echocardiographic features and the method of surgical correction are outlined.

Complete transposition with posteriorly located aorta and multiple ventricular septal defects.

A case of complete transposition with posterior aorta and multiple ventricular septal defects is reported. One perimembranous defect, diagnosed preoperatively, was closed while performing a Mustard operation. Three coexisting muscular ventricular septal defects, however, were missed and posed considerable problems at surgery. Although not previously reported, it should be possible to diagnose this condition preoperatively with a view to correction.

Limitations of Doppler ultrasound in the diagnosis of neonatal coarctation of the aorta.

Blood flow velocities were recorded using Doppler ultrasound in 40 babies aged between 1 day and 4 weeks who had coarctation of the aorta or interruption of the aortic arch. High velocity jets of 3 m/sec or more in the descending aorta were detected in only 3 patients, two of whom had interruption of the aortic arch. There was very poor agreement between upper and lower limb blood pressure difference and Doppler derived peak pressure drop. Doppler ultrasound may provide useful information on coexisting intracardiac abnormalities, but in the presence of ductal patency is of little value in the diagnosis of coarctation itself.

Use of larger sized aortic homograft conduits in right ventricular outflow tract reconstruction.

Between 1973 and 1993 sixty aortic homograft valved conduits in fifty-six patients were used to establish continuity between the right ventricle and the pulmonary artery in congenital heart disease. Age range was one day to 23.5 years (median 3.6 years) which included twenty-six patients less than one-year-old. Conduit size ranged from 11 to 23 mm (median 17.6 mm). there were nine hospital deaths and eight late deaths. The 45 survivors have been followed for a median of 8.6 years (range 6 months to 20 years). All patients have had serial echocardiographic assessments and 35 have had post repair cardiac catheterization. Almost all patients had mild-to-moderate degrees of homograft regurgitation. There were eleven with severe homograft regurgitation and two are being considered for reoperation. The follow up homograft gradient ranged from 0 to 64 mmHg (mean 24 mmHg). Freedom from reoperation for conduit obstruction was 98.2% at five years falling to 91% (C.L. 82%-100%) at 10 years. Of the 23 homografts inserted more than 10 years ago, only one (4.3%) has been replaced because it was causing important obstruction. None have been replaced for regurgitation. Our results indicate that larger sized aortic homografts used in reconstruction of the right ventricular outflow tract give satisfactory results and there is a low incidence of reoperation for replacement at medium term follow up.