The Damus-Stansel-Kaye procedure: anatomical determinants and modifications

Seven of 119 patients undergoing anatomical correction for transposition of the great arteries and Taussig-Bing anomalies without pulmonary stenosis had the Damus-Stansel-Kaye procedure and the rest, the arterial switch. The age of the patients having the Damus-Stansel-Kaye procedure ranged from 0.5 year to 5 years (mean age, 2.2 +/- 1 years). Four patients had transposition, 2 had Taussig-Bing anomaly, and 1 had corrected transposition. Indications for the Damus-Stansel-Kaye procedure were side-by-side great arteries associated with difficult coronary anatomy (5 patients), single coronary system (1 patient), and subaortic stenosis (1). A graft between the ascending and descending aorta for interrupted aortic arch made mobilization and posterior displacement of the ascending aorta for the arterial switch difficult. Subaortic stenosis (1 patient), subpulmonary ventricular septal defect (2 patients), and restrictive ventricular septal defect (4) precluded the Rastelli procedure. In 6 patients, the main pulmonary artery was transected at the band, a proximal main pulmonary artery to aorta anastomosis was complemented with a synthetic patch, and a right ventricle to distal main pulmonary artery valved conduit was inserted. Four patients had closure of the aortic outflow. Two patients had postoperative bleeding and 2, heart block. The only patient who did not have transection of the main pulmonary artery, an omission that led to an obstructed conduit at the distal anastomosis, died late. Two patients subsequently needed aortic outflow closure for critical aortic insufficiency. The Damus-Stansel-Kaye procedure has a definite role and can be safely performed in patients with transposition of the great arteries and Taussig-Bing anomalies.(ABSTRACT TRUNCATED AT 250 WORDS)     

Surgical treatment of interrupted aortic arch in infants under three months of age

From March, 1982, through March, 1988, 8 infants less than 3 months of age underwent surgical treatment of interrupted aortic arch. Five patients had IAA type A and 3 patients had type B. Seven patients with associated VSD underwent staged operations and 1 infant with aortopulmonary window underwent primary repair off cardiopulmonary bypass. Aortic arch reconstruction was by subclavian aortic anastomosis (6) or large tube graft (8 mm) interposition (2) combined with pulmonary artery banding (5). All seven patients with associated VSD survived the first-stage operation. One patient subsequently underwent pulmonary artery banding, two underwent patch aortoplasty and four patients underwent intracardiac repair and removal of a pulmonary artery band, with six survivors (86%). The operative death occurred in an infant in whom modified Damus-Kaye-Stansel operation was carried out for severe subaortic stenosis, which rerouting all left ventricular blood through VSD and the main pulmonary artery into the ascending aorta and reconstructing right ventricular-distal pulmonary artery connection by a valved external conduit. All six surviving patients are doing well at present (mean follow up of 3 years) without any significant pressure gradient between the ascending and thoracic aorta. Based on these data we conclude: (1) Aortic arch reconstruction and pulmonary artery banding can be reliably performed even in critically ill infants with IAA and isolated VSD. (2) The primary repair will provide better chance of survival in infants with IAA associated with significant LVOTO, truncus arteriosus or aortopulmonary window that do not readily lend themselves to pulmonary artery banding.     

Repair of interrupted aortic arch and associated malformations in infancy: indications for complete or partial repair

There is uncertainty regarding the best method of repair of interrupted aortic arch. The question is whether to perform primary definitive repair of this anomaly plus the associated defects versus arch repair only and palliation of the intracardiac defects, usually by pulmonary artery banding. Since 1976, 16 infants with interrupted aortic arch have been treated surgically. They were seen at 5.2 +/- 3 days of age and weighed 3.2 +/- 0.7 kg. The interruption occurred between the left carotid and left subclavian arteries (type B) in 9 and between the left subclavian artery and the descending aorta in 7 (type A). Isolated ventricular septal defect (VSD) was the only associated anomaly in 7 and aortopulmonary window, in 4. Two patients had truncus arteriosus type 1. Three had transposition of the great arteries: 1 with VSD and 2 with single ventricle. Prior to 1980, our policy was to palliate all patients. Between 1976 and 1980, 4 infants underwent left thoracotomy with arch repair plus pulmonary artery banding (3, VSD; 7, transposition of the great vessels and single ventricle) with only 1 (25%) survivor. Because of this high mortality, 8 patients with interrupted aortic arch and VSD or aortopulmonary window, seen since 1980, received complete repair with median sternotomy, end-to-end arch anastomosis, and closure of the VSD or aortopulmonary window utilizing profound hypothermia and circulatory arrest. All 8 survived.(ABSTRACT TRUNCATED AT 250 WORDS)     

Arterial switch operation with ventricular septal defect repair and aortic arch reconstruction

The arterial switch operation for transposition of the great arteries or double outlet right ventricle with ventricular septal defect (VSD) and aortic arch obstruction is a challenging procedure. One-stage neonatal repair is preferred; however, palliation may be indicated in the newborn who presents with Swiss cheese septum or the patient with extracardiac complications such as necrotizing enterocolitis or subarachnoid hemorrhage. The aortic arch repair is performed with continuous cerebral perfusion and includes patch enlargement of the transverse aortic arch and ascending aorta to control for the important diameter mismatch between the aorta and the pulmonary root. The VSD is no longer closed through the pulmonary valve. Instead, the VSD is approached through the aortic valve after harvesting of the coronary buttons and/or through the tricuspid valve and, if necessary, through an infundibulotomy. The coronary artery transfer is often complex because of the presence of unusual coronary artery patterns and requires precise technique. Subaortic obstruction is often present and requires correction. The right ventricle can be mildly hypoplastic but is not a contraindication to repair unless there is important tricuspid stenosis.     

Palliative reconstructive surgery for hypoplastic left heart syndrome

From August, 1985, through August, 1987, 104 consecutive, nonselected neonates underwent palliation of hypoplastic left heart syndrome. The technique included pulmonary artery homograft augmentation of the diminutive ascending aorta and aortic arch, atrial septectomy, transection of the main pulmonary artery with patch closure of the distal main pulmonary artery, anastomosis of the proximal main pulmonary artery to the augmented ascending aorta, and a 4-mm, modified, right Blalock-Taussig (N = 21) or central (N = 83) shunt. There were 30 early and 11 late deaths. Early mortality was most commonly associated with hypoventilation. Complications included development of aortic arch obstruction (N = 11) and progressive hypoxemia (N = 11). Alterations in surgical techniques and perioperative management should permit continued improvement in early and long-term survival.     

Left-sided lesions after anatomic repair of transposition of the great arteries, ventricular septal defect, and coarctation: surgical factors

OBJECTIVE: This study was undertaken to identify potential anatomic and surgical factors creating left-sided lesions, namely recoarctation of the aorta and neoaortic regurgitation, after anatomic repair of transposition of the great arteries with ventricular septal defect and aortic coarctation. METHODS: From 1983 to September 2002, 109 survivors out of 120 patients were studied. Two-stage repair was performed in 42 patients (group A), and single-stage repair was performed in 67 (groups B and C). Before repair, the diameters of the ascending aorta and main pulmonary artery were measured. In the patients with single-stage repair, coarctation was repaired by extended end-to-end anastomosis in 35 patients (group B) and by pulmonary homograft patch augmentation in 32 patients (group C). The ventricular septal defect was closed through the pulmonary artery in 70 patients and through the right ventricle or atrium in 39 patients. The neoaorto-aortic discrepancy was treated by V-shaped resection of the posterior sinus of Valsalva in 7 cases, pulmonary homograft patch in 32 cases, and anterior splitting of the ascending aorta in all cases. Before discharge from the hospital, neoaortic root and ascending aorta diameters and aortic regurgitation grade were recorded. Neoaortic regurgitation progression and reintervention were the end points of follow-up (97.2 +/- 61.2 months). RESULTS: Early and late survivals were significantly better in group C (P <.001). Risk factors for neoaortic regurgitation at discharge by univariate analysis were single-stage repair (P <.05) and ventricular septal defect closure through the pulmonary artery (P =.0076). On multivariate analysis, the latter was the only risk factor for neoaortic regurgitation at discharge and at last follow-up. Multivariate analysis showed that higher neoaortic root/ascending aorta ratio and ventricular septal defect closure through the pulmonary artery were risk factors for neoaortic regurgitation evolution at last follow-up. There were 29 reinterventions, 19 for recoarctation of the aorta and 10 for neoaortic regurgitation with or without aortic root dilatation. Group B (P <.05), high neoaortic root/ascending aorta ratio (P <.01), and progressive neoaortic regurgitation (P <.05) were risk factors for recoarctation of the aorta. Group A was a risk factor for aortic valve replacement at 10 years (P <.05). CONCLUSION: Neonatal single-stage repair with pulmonary homograft aortic augmentation remains the optimal approach to transposition of the great arteries with ventricular septal defect and aortic coarctation. It provides better early and late survivals and freedoms from left-sided lesions. Avoidance of late recoarctation of the aorta and progressive neoaortic regurgitation requires meticulous closure of the ventricular septal defect and evenly sized reconstruction of the aorta from root to distal arch.     

Surgical management of patients with interrupted aortic arch and severe subaortic stenosis

Ten patients underwent palliative surgery for interrupted aortic arch and severe subaortic obstruction due to posterior displacement of the conal septum. Their ages ranged between 4 and 28 days (mean, 11.0 +/- 7.7 days) and their weights, between 2.1 and 4.2 kg (mean, 2.85 +/- 0.6 kg). Preoperative echocardiography and cardiac catheterization were performed on all patients. The ratios of the left ventricular outflow tract diameters and the ascending aortic diameters to the descending aortic diameters were 0.56 +/- 0.03 and 0.56 +/- 0.06, respectively, compared with 0.81 +/- 0.12 and 0.95 +/- 0.17, respectively, in 20 patients with interrupted aortic arch but without obstruction (p less than 0.001). Four of the 10 patients underwent pulmonary artery banding and insertion of a bypass graft between the ascending and the descending aorta. All 4 died of low cardiac output soon after operation (100% operative mortality). The remaining 6 patients underwent banding and insertion of a graft between the main pulmonary artery proximal to the band, and the descending aorta. All of these patients survived, and all except 1 are doing well 3 months to 4 years postoperatively. The use of a pulmonary artery-descending aorta conduit and of distal pulmonary artery banding provides good palliation for patients with interrupted aortic arch and major subaortic stenosis.     

Stage I bilateral pulmonary artery banding maintains systemic flow by prostaglandin E1 infusion or a main pulmonary artery to the descending aorta shunt for hypoplastic left heart syndrome

Since 2002, we have performed bilateral pulmonary artery banding for stage I palliation and maintained systemic flow by prostaglandin E1 infusion or a main pulmonary artery to the descending aorta shunt, and here report our experience. Three of the 4 patients were diagnosed with aortic atresia/mitral atresia and 1 with aortic stenosis/mitral stenosis. Balloon atrial septostomy was performed in 2 before stage I. Bilateral pulmonary artery banding (right circumference: 10 or 14, left circumference: 10.5 to 14 mm) was performed from 7 to 19 days after birth. Systemic flow was maintained by prostaglandin E1 infusion in 2 patients and a Van Praagh procedure was performed in the other 2. Balloon atrial septostomy was required in 2 patients, and an atrial septal defect enlargement was in one during the interstage before stage II palliation, which was performed at ages 3 to 9 months. Bidirectional cavopulmonary shunt with aortic arch and coronary flow reconstruction was also performed. For patients younger than 4 months, we do not require pulmonary arterioplasty in stage II. All patients are alive and well and waiting for Fontan completion. Excellent early results were obtained for this surgical strategy that avoids the stage I Norwood palliation.     

Aortic valve repair after arterial switch operation

A patient with transposition of the great arteries and a ventricular septal defect underwent an arterial switch operation 15 months after pulmonary artery banding. At 12 years of age, severe neoaortic valve regurgitation, due to dilated aortic sinuses and poor leaflet coaptation, developed. Aortic valve repair involved placement of subcommissural sutures, elliptical excision and tailored reduction of two anterior aortic sinuses, with triangular patch expansion of the proximal ascending aorta. A good result was obtained.     

The modified Norwood procedure for hypoplastic left heart syndrome: early to intermediate results of 120 patients with particular reference to aortic arch repair

BACKGROUND: Classic first-stage Norwood repair of hypoplastic left heart syndrome uses a homograft patch enlargement to obtain an unobstructed aorta and coronary arteries. Because of possible disadvantages of the homograft, such as lack of growth, degeneration and calcification, and availability, we have tried to repair the aorta without patch supplementation. METHODS: Between February 1993 and September 1997, 120 patients, aged birth to 47 days (median 4 days) and weighing 1.7 to 4.4 kg (median 3.1 kg), underwent first-stage palliation for hypoplastic left heart syndrome. The diameter of the ascending aorta ranged from 1.5 to 8.0 mm (median 3.0 mm). Eight patients had an aberrant right subclavian artery arising from the descending thoracic aorta. In 95 patients (group I), all duct tissue was excised and the descending aorta was anastomosed to the aortic arch, which had been opened back into the ascending aorta. Then to this confluence was anastomosed the proximal main pulmonary artery. In the remaining 25 patients (group II), continuity of the aortic arch was maintained and the repair was performed with a Damus-Kaye-Stansel anastomosis. The size of the systemic-to-pulmonary shunt was 3 mm in 48 patients, 3.5 mm in 70, and 4.0 mm in 2. RESULTS: Circulatory arrest time ranged from 19 to 105 minutes (median 54 minutes). A homograft patch was necessary for the arch reconstruction in 18 patients (15%); 9 group I patients (10%) and 9 group II (36%) (P =.001). There were 82 hospital survivors (68%); 69 group I patients (73%) and 13 group II (52%) (P =.04), 71 patients without a patch (70%) and 11 with a patch (61%) (P >.2). By multiple logistic regression, the aberrant right subclavian artery was a significant risk factor for hospital death (P =.008). There were 6 late deaths. Sixteen of 71 patients (23%) who underwent second-stage palliation had a neoaortic arch obstruction develop, with a peak gradient greater than 10 mm Hg; 14 group I patients (23%) and 2 group II (22%) ( P >.2), 15 without a patch (23%) and 1 with a patch (17%) (P >.2). Overall survivals were 57% at 1 year and 55% at 2 years. CONCLUSION: The modified Norwood procedure for first-stage palliation of hypoplastic left heart syndrome is possible in the majority of patients without the use of exogenous materials and does not result in an increased incidence of neoaortic arch obstruction. Repair of the aorta without patch supplementation may improve the potential for long-term growth of the new aorta.     

Heart transplantation in repaired transposition of the great arteries

Cardiac transplantation was carried out in a 40-year-old man with the diagnosis of repaired transposition of the great arteries and right-sided aortic arch who had end-stage cardiac failure due to myopathy of the ventricles. Because of several previous surgical repairs and the orientation of the great vessels, the operation presented some technical problems. Therefore, modifications of operative procedures were used, including recipient hypothermia, circulatory arrest, and changes in the donor heart implantation. The removal of the donor heart was modified in such a way that the graft included the aortic arch and both pulmonary arteries. With the extra length of ascending aorta and transverse arch, the innominate, left carotid, and left subclavian vessels were excised as a button, thereby leaving the distal orifice of the aorta in the superior portion of the transverse arch. For the recipient, the operation was performed using hypothermic total circulatory arrest to dissect free the huge pulmonary artery and the short right-sided aortic arch to place the clamp. Implantation of the donor heart was modified accordingly. The technical results were confirmed one and a half months later on a control digital angiogram. Thirty-five days postoperatively the patient was discharged. Six months after operation, the patient is doing better than ever before in his life. Our findings suggest that a complicated conotruncal development does not preclude cardiac transplantation.     

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.     

Repair of coarctation with persistent fifth arterial arch and atresia of the fourth aortic arch

Coarctation of the aorta with persistent fifth arterial arch and atresia of the fourth aortic arch between the left common carotid and left subclavian arteries was treated surgically in a two-month-old boy with transposition of the great arteries and double-outlet right ventricle. The aortic arch was repaired using side-to-side anastomosis of the left common carotid and left subclavian arteries, patch repair of the coarctated segment at the origin of the left subclavian artery, and ligation of the patent ductus arteriosus. Pulmonary arterial banding and balloon atrioseptostomy were performed for associated anomalies.     

Stage I palliation of hypoplastic left heart syndrome: the importance of neoaorta construction

After Norwood's initial report of successful first-stage palliation of hypoplastic left heart syndrome in neonates, the occurrence of distal aortic obstructions, shunt problems, and late deaths have led to modifications in the surgical technique. Between January 1986 and December 1987, 12 neonates from three to 16 days old underwent stage I palliation with the same objectives. An open atrial septectomy was always performed. The pulmonary artery bifurcation was transected from the main pulmonary artery and closed with an aortic homograft patch. The aortotomy was begun 2 cm below the patent ductus arteriosus insertion and extended across the transverse arch and down the ascending aorta. The neoaorta was constructed using the hypoplastic ascending aorta-transverse aortic arch, the main pulmonary artery, and an aortic homograft augmentation patch. The homograft is hemostatic and pliable, and molds well in forming the neoaorta. A 4-mm shunt was inserted between the right innominate artery and the right pulmonary artery in 5 patients and the neoaorta and the pulmonary artery bifurcation patch in 7 patients. The early systemic oxygen saturation was optimized at 75% to 80% with hyperventilation, high concentration of inspired oxygen, sodium bicarbonate, and the frequent use of vasopressors to maintain an arterial blood pressure of 65 to 75 mm Hg. Two patients (17%) died early after operation; 1 had severe right ventricular dysfunction and both had severe tricuspid regurgitation. There were 2 late deaths at 7 and 13 months, of sepsis and hypoxia. The 8 survivors (67%) continue to do well over follow-up. The preoperative tricuspid regurgitation has remained stable in 3 survivors and disappeared in 2 survivors.(ABSTRACT TRUNCATED AT 250 WORDS)     

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)     

Two-stage repair of the transposition of great arteries with interruption/coarctation of the aorta

We report on 10 patients who underwent two-stage repair of transposition of the great arteries (TGA) with interruption (IAA) or coarctation (CoA) of the aorta. First, an operation for aortic arch reconstruction was performed: Blalock-Park with pulmonary artery banding (PAB) for IAA (5 patients), subclavian flap with PAB for CoA (4 patients) and end-to-end anastomosis without PAB (1 patient). All survived the first operation and had no significant pressure gradient with good growth of the ascending aorta, except for the 1 case without PAB. Half of the 8 patients who underwent PAB developed migration of the PAB. The arterial switch operation (ASO) was performed 0.7-12.6 (5.6+/-4.7) months after the first surgery. One patient with an abnormal coronary artery tract was lost after ASO. Five developed pulmonary artery stenosis and 1 developed supra-aortic stenosis late after ASO. Two patients need reoperation, 1 for supra-aortic stenosis, and the other for reCoA. Two-stage repair for TGA with IAA/CoA is still a useful method with a good operative result. However, strict follow-up is necessary because of the high frequency of late morbidity.     

Development of neo-coarctation in patients with transposed great arteries and hypoplastic aortic arch after Lecompte modification of anatomical correction

We report on three patients with kinking in the proximal aortic arch that developed after Lecompte modification of the arterial switch operation. Two patients had a previous subclavian patch repair of coarctation of the aorta and had an associated hypoplasia of the transverse aortic arch, and one patient had hemodynamically mild coarctation at the anatomical repair. A severe pressure gradient across the kinked area ("neo-coarctation") necessitating reoperation developed in one patient. The acute arch angulation appears to be due to an excessive posterior displacement of the ascending aorta by the anterior relocation of either the right or left main pulmonary artery branch from underneath the aortic arch. A foreshortened and frequently hypoplastic transverse aortic arch, a common association with coarctation of the aorta, appears to be especially vulnerable to the development of "neo-coarctation" after the Lecompte modification of the anatomical repair of transposed great arteries.     

A simple technique to circumvent the aorto-pulmonary size mismatch in arterial switch operation with arch repair: a report of five cases

The discrepancy in the sizes of the aorta and the pulmonary artery is a major technical concern in arterial switch operations for transposition of great arteries with ventricular septal defect, arch hypoplasia/interruption and Taussig Bing anomaly. The size mismatch increases the difficulty in neoaortic reconstruction and securing hemostasis. It alters the Sino tubular anatomy and contributes to development of Neoaortic Regurgitation (NAR). We present five cases of successful repairs for Transposition of Great Arteries (TGA) with hypoplastic transverse arch and Coarctation where the extended homologous gluteraldehyde treated pericardial patch was used to augment the isthmus, transverse arch and the ascending aorta upto the line of transaction to avoid the aorto-pulmonary size mismatch.     

A case report--combined Jatene procedure and extended aortic arch reconstruction for the original Taussig-Bing malformation associated with hypoplastic aortic arch, subaortic stenosis and coarctation of the aorta]

A successful repair with combined Jatene procedure (Lecompte modification) and extended aortic arch reconstruction in a two-month-old boy with the original Taussig-Bing malformation, associated with hypoplastic aortic arch, subaortic stenosis and coarctation of the aorta was described. The ascending aorta was measuring 8 mm and the distal aortic arch was 4 mm in outer diameter. The interventricular rerouting was not feasible, because the infundibular septum was almost aligned to the interventricular septum. So that arterial switch operation was preferred. The primary interventricular foramen was enlarged by wedge resection and the secondary interventricular foramen was closed with a patch of preserved equire pericardium. An extensive patch angioplasty of entire aortic arch down to descending aorta was performed with a composite patch of Xenomedica (preserved equire pericardium) and Dacron velour. Hypertrophied muscle bundles were resected for relief of subaortic stenosis. Jatene procedure was then performed. During neo-pulmonary reconstruction, distal pulmonary orifice was shifted towards right to avoid kinking and compression on the coronary arteries. One stage correction consisting of Jatene procedure and extended aortic arch reconstruction was considered to be a procedure of choice in this type of complex anomalies.     

Reoperation for diffuse supravalvular aortic stenosis with Williams syndrome —Extended patch aortoplasty and extra-anatomic bypass from the ascending aorta to the descending aorta in a median sternotomy—

A case of diffuse supravalvular aortic stenosis (SVAS) with Williams syndrome is reported. In this case of severe diffuse SVAS, we performed the diamond-patch aortoplasty in a child. However he has been suffering from residual SVAS. At 9-years old, the myocardial injury was noted by myocardial scintigraphy. Preoperative cardiac catheterization and angiography revealed the hypoplastic ascending aorta and arch with a pressure gradient of 89 mmHg at the distal site from the left subclavian artery. Through only a median stenotomy, an extended patch aortoplasty between the valsalva sinus and distal arch was performed and an extraanatomic bypass from the ascending aorta to the descending aorta was employed using a 10 mm tube graft. We realize this technique is available because this method can relieve the left ventriculus of the pressure load and operate via only median sternotomy.