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.     

Options for surgical repair in hearts with univentricular atrioventricular connection and subaortic stenosis.

Thirteen patients have undergone surgical treatment because of subaortic obstruction in hearts with a univentricular atrioventricular connection. Nine patients underwent surgical enlargement of the ventricular septal defect and four patients had construction of an aortopulmonary anastomosis and closure of the pulmonary trunk (the Damus-Kaye-Stansel procedure). Two patients undergoing enlargement of the septal defect and two having the Damus-Kaye-Stansel procedure also had a modified Fontan procedure. One patient had complete atrioventricular dissociation after direct enlargement of the ventricular septal defect, which necessitated insertion of an epicardial pacemaker. One patient died within 30 days of the operation after enlargement of the defect and two patients after the Damus-Kaye-Stansel procedure. There was one late death, occurring in a patient who underwent enlargement of the ventricular septal defect. Ten patients have subsequently undergone conventional cardiac catheterization and angiography or transcutaneous Doppler flow studies to assess the relief of the subaortic obstruction. The result has been satisfactory in all. Because of this experience, we now recommend direct surgical enlargement of the restrictive ventricular septal defect for direct relief of subaortic stenosis occurring with a univentricular atrioventricular connection to a dominant left ventricle, inasmuch as it appears to be hemodynamically effective with a low operative mortality and morbidity. The Damus-Kaye-Stansel procedure can also have a role in relieving subaortic stenosis when the atria are connected to a dominant right ventricle.     

Long-term results of relief of subaortic stenosis in univentricular atrioventricular connection with discordant ventriculoarterial connections

BACKGROUND: We set out to examine the long-term results of relief of subaortic stenosis by enlargement of ventricular septal defect in patients with univentricular atrioventricular connection to a dominant left ventricle and discordant ventriculoarterial connections. METHODS: Twenty-four patients underwent enlargement of ventricular septal defect between 1985 and 1998 at a median age of 3.2 years (range, 3 weeks to 14 years). Ten patients were younger than 1 year of age. Eighteen had undergone previous banding of the pulmonary trunk, 9 of whom also required repair of coarctation of the aorta. The median subaortic gradient before enlargement was 46 mm Hg. Twenty-three patients had a patch to enlarge the rudimentary right ventricle. RESULTS: Five patients (21%) died in the early postoperative period. The overall survival at 1 and 3 years was 73%, and at 5 and 10 years was 68% and 60%, respectively. Complete heart block requiring insertion of a pacemaker occurred in 2 patients (8%). A Fontan operation was performed in 10 patients, 5 underwent a bidirectional Glenn procedure, and 2 required cardiac transplantation. Follow-up was complete in all survivors at a median time of 6.7 years (range, 8 months to 13 years). From the earlier part of the series, 3 patients experienced aortic insufficiency and 2 had recurrent obstruction. Factors adversely affecting survival were age younger than 1 year at operation and presence of obstruction within the aortic arch. CONCLUSIONS: Our experience shows that, in patients with univentricular atrioventricular connection to a dominant left ventricle and subaortic stenosis, enlargement of the ventricular septal defect provides satisfactory relief of obstruction except in those younger than 1 year of age, and those who have associated obstruction in the aortic arch.     

A reconsideration of risk factors for the Fontan operation.

We reviewed our experience in 38 patients who underwent a Fontan operation. In the first five patients ages 7.5 to 23 years (mean, 15 years), a conduit was placed from the right atrium to the small right ventricle or the pulmonary artery (PA). The remaining 33 patients, ages 7 months to 14 years (mean, 4.8 years), had a modified Fontan operation with direct systemic venous or right atrial to PA anastomosis. The diagnoses were tricuspid atresia (n = 14), single ventricle (n = 10), hypoplastic right or left ventricle (n = 9), double-outlet right ventricle with inlet ventricular septal defect and pulmonary atresia or stenosis (n = 3), criss-cross ventricles and transposition of the great arteries (n = 1), and atrioventricular canal and anomalous pulmonary venous connection (n = 1). Thirty-two patients had previous surgery. Other procedures included PA banding (n = 7), systemic to PA shunts (n = 25), Norwood operation (n = 3), and a Damus-Kaye-Stansel anastomosis (n = 1), repair of total anomolous pulmonary venous connection (n = 1), a Blalock-Hanlon atrial septectomy (n = 1), and enlargement of a restrictive ventricular septal defect (n = 1). There were four operative deaths (10.5%), three from low cardiac output and one from subaortic obstruction. There were no deaths in patients younger than 3 years of age (n = 13). Subaortic obstruction developed in six of the seven patients who had pulmonary artery banding and resulted in three deaths. In our experience, diagnosis, previous surgery, type of previous operation, PA pressure, and younger age are not risk factors for early or late death. Subaortic obstruction is a major risk factor for late death. Accordingly we now perform a Damus-Kaye-Stansel anastomosis combined with a systemic to PA shunt in those children with excessive pulmonary blood flow who anatomically are likely to develop subaortic obstruction. A modified Fontan operation can be performed any time after 1 year of age and in some patients after 6 months of age, providing the anatomy and physiology of the patient are acceptable.     

Surgical outcome of double-outlet right ventricle with subpulmonary VSD

BACKGROUND: Optimal management of double-outlet right ventricle with subpulmonary ventricular septal defect remains controversial. We reviewed our 7-year experience with patients who had this anatomic configuration. METHODS: Between January 1992 and January 1999, 20 patients underwent an arterial switch operation (ASO group), and 12 underwent a bidirectional Glenn procedure followed by a modified Fontan in 10 (Glenn/Fontan). Mean follow-up was 23 +/- 18 months. RESULTS: An initial palliative operation was done in 19 patients (9 in the ASO group, 10 in the Glenn/Fontan group). There were no deaths in the Glenn/Fontan group. Four patients in the ASO group died within 33 days postoperatively. Two of them had a single coronary artery, 1 had a straddling mitral valve, 1 had a hypoplastic aortic arch, and 1 had multiple ventricular septal defects. Three patients had reoperation for subaortic stenosis (n = 2) or pulmonary stenosis (n = 1) after the ASO. Four patients (3 in the ASO group, 1 in the Glenn/Fontan) required a pacemaker for postoperative complete atrioventricular block. Actuarial survival at 5 years for the entire group was 87% (70% confidence interval, 81% to 93%). CONCLUSIONS: The ASO remains our preferred treatment for infants with double-outlet right ventricle and subpulmonary ventricular septal defect. However, associated anatomic defects are important risk factors.     

Surgical Treatment of Double-Outlet Left Ventricle in Situs Inversus {I,D, D}

A case of double-outlet left ventricle {I, D, D} with a subaortic ventricular septal defect and pulmonary stenosis is reported. The diagnosis was made at operation and was reconfirmed postoperatively by two-dimensional echocardiography. Repair was achieved by patch closure of the ventricular septal defect and enlargement of the functional right ventricular outflow tract using a valved patch. The postoperative course was uneventful except for a transient complete heart block. To our knowledge, this represents the second instance of surgical treatment of double-outlet left ventricle {I, D, D} reported in the literature.     

Reverse subclavian flap repair of hypoplastic transverse aorta in infancy

BACKGROUND: Management of hypoplastic aortic arch associated with coarctation in infancy can be challenging. Reverse subclavian flap aortoplasty plus coarctation resection offers simplicity without needing foreign material or cardiopulmonary bypass. METHODS: Since 1988, 46 of 162 infants less than 3 months undergoing coarctation repair had hypoplastic arch enlargement with reverse subclavian flap aortoplasty. Median age was 11 days; mean weight was 3.2 kg. Thirty-seven patients (80%) had associated cardiac defects including single or multiple ventricular septal defects (14 infants), transposition of the great arteries (7), aortic or mitral stenosis (5), and complete atrioventricular septal defect (5 infants). Twenty-eight patients had pulmonary artery banding; 2 had an arterial switch operation through a separate median sternotomy. RESULTS: There were two hospital deaths: one 4 months postoperatively in a patient requiring a Norwood procedure the next day for underestimated left ventricular hypoplasia; the other of sepsis more than 1 month postoperatively. On follow-up from 1 to 129 months (mean, 38 months), there were five recurrent obstructions: three at the coarctation site treated with balloon dilatation and two at the arch site. Twenty-six children had their heart defects corrected with 29 subsequent operations including an arterial switch operation for transposition of the great arteries/ ventricular septal defect (3 infants), relief of aortic or mitral stenosis +/- ventricular septal defect closure (5), multiple ventricular septal defect closure (3), a bidirectional Glenn (2), complete atrioventricular septal defect (2), and anomalous left coronary with ventricular septal defect repair (1 infant). Four children await debanding and ventricular septal defect closure or Glenn anastomosis. There have been two late deaths (overall survival, 91%). CONCLUSIONS: Reverse subclavian flap aortoplasty is excellent for relief of arch hypoplasia and coarctation in infants with low recurrence rates and acceptable operative and intermediate survival.     

Conal enlargement for diffuse subaortic stenosis

Twelve patients underwent conal enlargement for diffuse subaortic stenosis over a 3 1/2-year period. The subaortic stenosis was due to tunnel outflow in 11 and malattached mitral valve in one. Mean age was 4.4 +/- 4 years and mean subaortic gradient was 50 +/- 21 mm Hg. Three infants had a malalignment ventricular septal defect. In eight patients significant obstruction occurred 2 to 7 years (mean 4 +/- 2) after simple resection of subaortic stenosis (n = 2), ventricular septal defect closure (n = 2), ventricular septal defect closure and subaortic stenosis resection (n = 2), and canal repair (n = 2). In three infants the tunnel outflow distal to a malalignment ventricular septal defect was enlarged and closed with the defect. In three patients with subaortic stenosis proximal to a previously repaired ventricular septal defect, transatrial conal enlargement through the ventricular septal defect was performed. Another patient without a ventricular septal defect had transatrial conal enlargement. The remaining five patients had the modified Konno procedure. Two patients had postoperative complete heart block and one infant had insertion of an apicoaortic conduit for aortic anulus hypoplasia 9 months later. One patient died of pneumonia during the follow-up period. Postoperative echographic outflow gradients up to 3 1/2 years (mean 1.2 +/- 1) ranged up to 25 mm Hg (mean 7 +/- 11) and were mainly at the aortic level. The 11 surviving patients are doing well up to 3 1/2 years of follow-up (mean 1.5 +/- 1). We conclude that conal enlargement procedures with aortic valve preservation are preferable, effective, and can be safely performed for diffuse subaortic stenosis in infants and children.     

Surgical "correction" of solitus-concordant, double-outlet left ventricle with L-malposition and tricuspid stenosis with hypoplastic right ventricle.

This report concerns a 2-year-old boy who had situs solitus and atrioventricular concordance with double-outlet left ventricle, L-malposition of the aorta, pulmonary stenosis, tricuspid stenosis, hypoplastic right ventricle, ventricular septal defect, and secundum atrial septal defect. These lesions were correctly diagnosed preoperatively, and "correction" was achieved by closing the atrial septal defect and tricuspid valve and performing a Fontan procedure using an antibiotic-sterilized aortic homograft for the conduit. The patient initially did well postoperatively but, despite vigorous diuretic therapy, developed large bilateral pleural effusions necessitating frequent aspiration. He died unexpectedly one month after the operation. Postmortem examination showed the repair to be intact. Despite his death, we are encouraged to consider this operation in such cases in the future.     

Midterm results of surgical treatment of systemic ventricular outflow obstruction in Fontan patients

BACKGROUND: Achieving unobstructed blood flow from the systemic ventricle to the aorta is important during the Fontan procedure for complex cyanotic congenital heart disease when there is systemic ventricular outflow obstruction (SVOO). Because SVOO can progress after the Fontan procedure if there is morphologic obstruction, we have adopted a policy of relieving obstructions to systemic blood flow. METHODS: Twenty-five patients were treated by the Fontan procedure with SVOO. Twenty-one patients had undergone prior pulmonary artery banding and 10 patients had undergone prior arch repair. Systemic ventricular outflow obstruction progressed in 5 patients after the Fontan procedure. Main diagnosis was single ventricle in 12, tricuspid atresia in 5, transposition of the great arteries in 4, double-outlet right ventricle in 3, and common atrioventricular canal in 1. Mean age at operation was 6.5 years (range 1 to 15 years) and the average preoperative pressure gradient across the ascending aorta and systemic ventricle was 29 mm Hg (range 0 to 100 mm Hg). The Damus-Kaye-Stansel procedure was performed in 18 patients (double-barrel anastomosis in 13, end to side anastomosis in 5), and subaortic resection or ventricular septal defect or bulboventricular foramen enlargement was performed in 7. Double-barrel anastomosis has been our first choice since 1994, if the pulmonary valve is intact. Follow-up has ranged from 4 months to 14 years (average 5.0 years). Twenty-three of the 25 patients have undergone recatheterization (average 21.4 months later). RESULTS: No early deaths were found; one late death was reported of a patient with single right ventricle (4.0%). The postoperative average pressure gradient was 1.1 mm Hg (0 to 10 mm Hg), and the average right atrial pressure was 14 mm Hg (9 to 20 mm Hg). In all patients who underwent ventricular septal defect or bulboventricular foramen enlargement, regular sinus rhythm was maintained postoperatively. Regarding the Damus-Kaye-Stansel procedure, there was minimal progression of semilunar valve insufficiency except in 1 patient who underwent end-to-side anastomosis with moderate pulmonary regurgitation postoperatively. CONCLUSIONS: The midterm results of the Fontan procedure with SVOO have been satisfactory. Because SVOO might progress after the Fontan procedure if there is morphologic obstruction, an appropriate strategy to relieve obstruction to systemic blood flow should therefore be performed concomitantly with the Fontan procedure.     

Results of Norwood stage I operation: comparison of hypoplastic left heart syndrome with other malformations

OBJECTIVE: We compared the Norwood stage I operation for hypoplastic left heart syndrome and other complex malformations with ductus-dependent systemic circulation. METHODS: A retrospective study of 194 patients who underwent a Norwood stage I palliation between 1990 and 1998 was conducted. Malformations in 131 patients were classified as hypoplastic left heart syndrome, defined as aortic and mitral atresia or severe stenosis, normal segmental anatomy, intact ventricular septum, and hypoplasia of the left ventricle. Sixty three patients had other lesions: hypoplastic left ventricle with ventricular septal defect (n = 18), unbalanced complete atrioventricular canal (n = 9), complex double-outlet right ventricle (n = 14), double-inlet left ventricle (n = 11), tricuspid atresia with transposition of the great arteries (n = 6), and others (n = 5), including heterotaxia. RESULTS: Operative (>30 days) and 1-year survivals were lower for patients with hypoplastic left heart syndrome than for those with other lesions (63.4% vs 81%, P =.008, and 51.2% vs 71.4%, P =.02, respectively). The presence of a nonhypoplastic left ventricle (n = 27) was associated with higher operative and 1-year survivals (96.3% vs 64.7%, P =.002; 88.9% vs 52. 7%, P <.001). A restrictive atrial septal defect and prematurity tended to increase mortality across both groups. Cox proportional hazards regression indicated that a single right ventricle was the most important independent predictor of death (P <.001). Operative mortality for all patients undergoing the stage I procedure decreased from 38.5% (1990-1994) to 21.4% after 1994 (P =.02). CONCLUSIONS: The survival of patients with malformations other than hypoplastic left heart syndrome after the Norwood procedure is greater than for those with hypoplastic left heart syndrome. Staged palliation is valid surgical therapy in these patients, with good results in intermediate follow-up.     

Pulmonary artery banding and ventricular septal defect enlargement in patients with univentricular atrioventricular connection and the aorta originating from an incomplete ventricle.

BACKGROUND: In patients with univentricular atrioventricular connection and the aorta originating from an incomplete ventricle, subaortic stenosis is generally due to a restrictive ventricular septal defect (RVSD), that may be present at birth or develop after palliative procedures. In particular, a primary role in the genesis of the RVSD has been ascribed to pulmonary artery banding (PAB). The aim of this paper is to analyse the possible risk factors for the development of an RVSD, including PAB, and the results of one of the proposed procedures for treatment of this condition (RVSD enlargement). METHODS: We retrospectively reviewed clinical records and outpatient records of 24 consecutive patients with univentricular atrioventricular connection and the aorta originating from the incomplete ventricle that received their first treatment at our institution from January 1991 to April 2000. The variables age, sex, weight, diagnosis, surgical procedures, associated anomalies, associated surgical procedures, were considered. RESULTS: Four patients (16.7%) had absent left atrioventricular connection, seven (29.7%) had absent right atrioventricular connection and discordant ventriculo-arterial connection, and 13 (54.7%) had double inlet left ventricle and discordant ventriculo-arterial connection. Five patients (20.8%) had associated coarctation or hypoplasia of the aorta, and eight (33.3%) had pulmonary stenosis or atresia. Median age at the first operation was 7.5 days (range: 1-376). Median weight was 3.5 kg (range: 1.9-6.3). Seventeen patients underwent pulmonary artery banding, one underwent a Damus-Kaye-Stansel connection, one received a Glenn shunt and five a modified Blalock-Taussig shunt. Early mortality was 12.5%. The only variable associated with operative mortality was the presence of coarctation or hypoplasia of the aorta (P=0.004). Ten patients (41.6%) developed subaortic stenosis. None of the tested variables, including pulmonary artery banding, was associated with the development of subaortic stenosis. Subaortic stenosis was due to a restrictive VSD in eight patients, six of whom underwent direct VSD enlargement by muscular resection and are well at last follow-up (four complete repairs). None of the procedures was complicated by complete heart block. In two cases subaortic stenosis was treated by a Damus-Kaye-Stansel connection. A single patient died during follow-up, and 11 patients have achieved a complete one-ventricle repair. CONCLUSION: In our experience, pulmonary artery banding was not associated with an increased risk of developing an RVSD. VSD enlargement proved to be safe and effective for treatment of subaortic stenosis due to an RVSD.     

Transatrial relief of diffuse subaortic stenosis after ventricular septal defect closure

Transatrial enlargement of the left ventricular outflow tract for serious obstruction was performed in 3 patients with previous ventricular septal defect closure. Two patients had recurrent subaortic stenosis as resection had already been performed at initial operation. In all patients, the obstruction was located below the ventricular septal defect patch. Patch enlargement of the left ventricular outflow tract was carried out by opening the ventricular septal defect patch through the tricuspid valve and extending the incision downward through the area of obstruction and the left ventricular body. All patients had uneventful postoperative course and effective relief of left ventricular outflow tract obstruction. We feel that the approach is simple and effective; it avoids a right ventriculotomy and provides a viable option in certain patients with left ventricular outflow tract obstruction.     

Operations for subaortic stenosis in univentricular hearts

Optimal prevention and treatment of subaortic stenosis (SAS) in the univentricular heart with subaortic outlet chamber and high pulmonary blood flow remains controversial, especially when complicated by aortic arch obstruction. Herein we analyze our surgical results. Group 1 consisted of 11 infants (mean age, 10 days) with univentricular heart and SAS. Ten required repair of interrupted aortic arch (n = 7) or coarctation with hypoplastic arch (n = 7). Four patients had relief of SAS by either Damus-Kaye-Stansel connection (n = 2) or aortopulmonary window (n = 2), with three operative deaths and one late death. Six had one-stage arterial switch and atrial septectomy with arch repair (5/6) with one operative death and one late death. Two survivors have progressed to bidirectional cavopulmonary shunt, a third has had a Fontan operation, and a fourth awaits Fontan. In group 2, 11 children required operation for acquired SAS after pulmonary artery banding. Nine have progressed to Fontan operation with either staged (n = 3) or concurrent (n = 6) relief of SAS by Damus-Kaye-Stansel connection or subaortic resection. Fontan mortality was 11% (70% confidence limits, 2% to 32%). Group 3 consisted of 3 patients without pulmonary artery banding who had SAS diagnosed at Fontan evaluation. All 3 survived Fontan operation and relief of SAS by Damus-Kaye-Stansel connection or subaortic resection. Group 4 consisted of 1 patient with previous pulmonary artery banding (no SAS) who underwent Fontan operation but required Damus-Kaye-Stansel connection 30 months later for SAS.(ABSTRACT TRUNCATED AT 250 WORDS)     

Norwood operation for univentricular heart with subaortic stenosis in the neonate.

In the setting of a single ventricle, subaortic stenosis may be enhanced by pulmonary artery banding and may later contraindicate a Fontan operation. The Norwood operation may prove a preferable alternative in some infants as a preparatory procedure. We have successfully used this procedure as the initial operation to palliate a newborn with tricuspid atresia, transposition of the great arteries, coarctation, and severe arch hypoplasia secondary to a restrictive bulboventricular foramen.     

Surgical management of double-outlet right ventricle

From 1977 to 1983, 32 consecutive patients, ranging in age from 15 days to 24 years, underwent operations for double-outlet right ventricle. Twenty patients had a palliative operation either to increase (12 cases) or to reduce (eight cases) pulmonary blood flow: Ten of them have subsequently undergone total repair, and in another six correction was delayed because of possible incremental operative risk factors, such as multiple ventricular septal defects or the need for an extracardiac conduit. Four patients with multiple, complex associated intracardiac anomalies are currently considered to have uncorrectable defects. A total of 22 patients underwent correction either primarily (12) or after palliation (10). Intraventricular tunneling was performed in 16 patients with a subaortic ventricular septal defect and in one with a doubly committed ventricular septal defect. Seven of these had pulmonary stenosis and five had reconstruction of the right ventricular outflow by means of a patch (three) or a conduit (two); among this group, five also had enlargement of the ventricular septal defect. In three patients with a subpulmonary defect and in one with a remote ventricular septal defect, all of them without pulmonary stenosis, total repair was achieved by a Senning, a Mustard, or an arterial switch operation. Finally, the only patient with atrioventricular discordance and pulmonary stenosis had insertion of a left ventricle-pulmonary artery conduit. No operative deaths were observed after palliation, but one patient died of intrapulmonary hemorrhage after total repair (4.5%). Major postoperative complications included detachment of the ventricular septal defect patch in one patient and late progression of pulmonary vascular obstructive disease in another. No late deaths have been recorded. Surgical repair of double-outlet right ventricle can be accomplished with gratifying early and late results, the risk of operative death being below 5%. The outcome in patients with subaortic ventricular septal defect appears particularly favorable, despite the extensive intracardiac procedures required for total correction. An early intervention is recommended to prevent development of pulmonary vascular obstructive disease and to avoid massive cardiac hypertrophy and fibrosis, which may cause late rhythm disturbances and impede the intracardiac repair.     

Early palliation of univentricular hearts with subaortic stenosis and ventriculoarterial discordance. The arterial switch option.

The optimal Fontan-type operation greatly depends on appropriate initial palliation. Several surgical techniques have been used in infancy to palliate patients with univentricular hearts, ventriculoarterial discordance, and subaortic stenosis. The two most common are pulmonary artery banding and Damus-Norwood procedures. Palliative arterial switch operation is another surgical option that was used in this early series of seven infants. The principle of this operation is to "switch" the subaortic obstruction into a subpulmonary obstruction; the coronary artery relocation on the large pulmonary trunk creates a harmonious aortic root and the connection of the rudimentary ventricular chamber to the pulmonary artery trunk creates a natural protection of the pulmonary vascular bed through the restrictive bulboventricular foramen. Seven infants with univentricular hearts, ventriculoarterial discordance, and subaortic stenosis underwent a palliative arterial switch operation. All infants had an associated aortic arch obstruction of various degrees, including one with interrupted aortic arch, five with coarctation with severe arch hypoplasia, and one with isolated arch hypoplasia. There were three with double-inlet left ventricle, three with tricuspid atresia, and one with transposition of the great arteries with ventricular septal defect and severe right ventricular hypoplasia. The subaortic obstruction was patent at birth in five patients who underwent a palliative switch operation in the first 2 months of life, and rapidly occurred following a previous neonatal pulmonary artery banding associated with arch repair in two patients who underwent a switch operation at 5 and 8 months of age, respectively. The operation includes aortic arch repair without prosthetic material, an atrial septectomy, and the arterial switch. An associated pulmonary shunt was required in five patients and a pulmonary artery banding in one. There was one early death in a patient with [S,L,L] anatomy and congenital atrioventricular block, leading to an early mortality of 14% (95% confidence limits: 1% to 28%). There was one late death. Four survivors are waiting for a Fontan-type procedure, and one survivor had satisfactory right ventricular growth. Early palliative arterial switch operation offers several advantages: reconstruction of a harmonious aortic root, natural protection of the pulmonary bed through the restrictive bulboventricular foramen, prevention of deleterious myocardial hypertrophy, and arch reconstruction without the introduction of a foreign material. This aggressive technique may provide a satisfactory palliation in infants with univentricular hearts and ventriculoarterial discordance, when the bulboventricular foramen/aortic anulus ratio is less than 0.8 or when the subaortic stenosis is severe enough to be associated with an arch obstruction.(ABSTRACT TRUNCATED AT 400 WORDS)     

Left ventricular outflow obstruction after Fontan procedure involving a patient with complete atrioventricular septal defects complicated by a small left ventricle

We report a 1-year-old girl who developed ventricular outflow tract obstruction early after a Fontan operation, necessitating surgical relief using the Damus-Kaye-Stansel procedure. The patient had a complete atrioventricular septal defect complicated by a muscular ventricular septal defect (VSD) and a small left ventricle, a morphology that has not previously been reported in cases of systemic outflow tract obstruction after the Fontan procedure. Postoperative systemic outflow obstruction must be considered as a possible sequela following Fontan surgery in patients with an atrioventricular septal defect and a small left ventricle.     

Evaluation of the Damus-Kaye-Stansel operation in infancy

Thirteen patients, 12 of whom younger than 2 years, underwent a Damus-Kaye-Stansel procedure for complete transposition of the great arteries, ventricular septal defect, or double-outlet right ventricle and subpulmonary ventricular septal defect. In 6 patients, associated cardiac anomalies caused systemic flow obstruction. There were six hospital deaths (mortality rate, 42%). In a mean follow-up period of 57 months, 5 of 7 survivors required relief of right ventricular hypertension through conduit replacement or enlargement (4 patients) or conduit valve balloon dilation (1 patient). The aortic valve became regurgitant in 2 patients in whom it had been left in potential connection with the right ventricle. One patient has moderate pulmonary valve regurgitation. The main advantage of the Damus-Kaye-Stansel procedure is that it avoids coronary relocation; also, the spatial relationship of the great arteries and the coronary anatomy do not affect its feasibility. One drawback is the need for a conduit in infancy. Our present indication for Damus-Kaye-Stansel procedure is confined to double-outlet right ventricle with subpulmonary ventricular septal defect; 5 of 6 patients survived repair in this series. Possible indications are for patients with associated subaortic obstruction or unusual coronary arrangements. Fresh or cryopreserved homografts as extracardiac conduits and primary closure of the subaortic area may reduce the need for reoperation after Damus-Kaye-Stansel procedure.     

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

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