Fibrous skeleton and ventricular outflow tracts in double-outlet right ventricle

Twenty-four hearts in which both great arteries arose from the right ventricle were studied to establish variations present within the fibrous skeleton and infundibular morphologies. Variations were also noted in the location of the ventricular septal defect and measurements were obtained of the outlet septum and the circumferences of the arterial valves. Completely muscular subarterial infundibulums were present in only 9 (37.5%) of the hearts, with varying degrees of fibrous continuity between the leaflets of the arterial and atrioventricular valves in the remainder. The aorta was rightward and posterior in 12 (50%) of the hearts, and subaortic and subpulmonary ventricular septal defects were present in equal numbers in this group. No subaortic defects were present when the aorta was side-by-side and right-sided. No subpulmonary defects were present in hearts with a posterior aorta. The mean ratio of 0.91 +/- 0.36 for the subpulmonary to subaortic length of the outlet septum was significantly less than the value of 1.54 +/- 0.41 noted previously in hearts with tetralogy of Fallot (p less than 0.001).     

A successful arterial switch operation for the transportation of the great arteries with posterior aorta--with special reference to its anatomical features and possibility of the indication of the Jatene procedure

A five months old infant with the transposition of the great arteries with posterior aorta undergoing arterial switch operation is reported. A pre-operative diagnosis of the transposition of the great arteries with posterior aorta, subarterial ventricular septal defect, anterior pulmonary artery, bilateral conus and fibrous continuity between aortic and mitral valve was made. This diagnosis was confirmed by the open heart surgery. The ventricular septal defect was subaortic from the transected aortic aspect, and the infundibular and trabecular septum was aligned. For these reasons, this subarterial ventricular septal defect was easily closed transaortically. The right coronary artery arose from the left sinus and the left coronary artery from the posterior sinus, so the coronary arterial pattern of this patient was a Shaher type 9. And in addition, another small ostium located in the left sinus gave rise to an additional small branch. Translocation of the coronary arteries was performed. The new pulmonary trunk was reconstructed to the right pulmonary artery so as not to compress the coronary artery and distort the great arteries. This patient was restudied three months after the repair. The right to left ventricular systolic pressure ratio was 0.42 and no pressure gradient was present between the right ventricle and the right pulmonary artery. But a moderate aortic regurgitation was detected. This was caused by deformity of the aortic sinus of valsalva. Only 28 patients with the transposition of the great arteries with posterior aorta were reported in the literature, but there were no reports on the successful surgical treatment for its rare anomaly, to our knowledge.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pulmonary Approach for Closure of Ventricular Septal Defect and Resection of Infundibular Obstruction in Malaligned Ventricular Septum

Presented is the technique of closure through the pulmonary artery of the perimembranous ventricular septal defects and defects with malaligned septum as an alternative method to the transatrial or transventricular approach. The technique of infundibular resection without opening the right ventricle in cases of tetralogy of Fallot is also described.     

The potential for subpulmonary obstruction in complete transposition after the arterial switch procedure. An anatomic study

Increasing experience with the arterial switch procedure has revealed that, in some instances, obstruction of the newly created sub-pulmonary outflow tract is a major problem. In this study, we examined the right ventricular outflow tract in 51 specimens of complete transposition, 18 of which were associated with a ventricular septal defect. We focussed our attention on the musculature which made up the outflow region and considered variations from that found in the normal right ventricle. Our gross observations showed differences in morphology which produced a sharper angulation between inflow and outflow when compared to the normal. Discrete outflow obstruction was found in 11 hearts (8 with ventricular septal defect and 3 with an intact ventricular septum). The obstruction was due to deviation of the outlet septum in 5, and anomalous muscle bundle in 1, hypertrophy of septomarginal and septoparietal trabeculations in 3 and circumferential infundibular hypertrophy in 2 hearts. Coexisting obstruction of the left ventricular outflow was present in 3 hearts. The anatomical substrates of discrete obstruction should be identified by preoperative cross-sectional echocardiography and/or angiography. Modifications in surgical techniques or radical resection of the obstructing musculature could then be performed to avoid the problem of subpulmonary obstruction after the arterial switch procedure.     

Infundibular septal resection: surgical anatomy of the superior approach

A technique for extensive resection of the infundibular septum through the superior transarterial approach is proposed. Infundibular septal resection facilitates intraventricular rerouting for anatomic correction of transposition of the great arteries with ventricular septal defect and double-outlet right ventricle. This technique also may be used to enlarge progressively obstructive but physiologically advantageous ventricular septal defects, for example, with tricuspid atresia. Through the superior transaortic or transpulmonary approach, the landmarks of the infundibular septum are the intercoronary commissure of the aortic valve and the septal commissure of the pulmonary valve, both of which are directly above the middle of the infundibular septum. With this exposure, the infundibular septum may then be resected easily and relatively completely and a large opening created immediately beneath the semilunar valves. The feasibility of infundibular septal resection by the superior approach was assessed post mortem in 25 cases of transposition of the great arteries with ventricular septal defect and in 20 autopsied cases of double-outlet right ventricle. This technique was subsequently applied successfully to enlarge the obstructive ventricular septal defect of a 2-year, 11-month-old girl with D-transposition of the great arteries. The advantages of the proposed technique include technical ease, relative completeness of infundibular septal resection, and the facilitation of intraventricular repair of transposition of the great arteries with ventricular septal defect and double-outlet right ventricle.     

Pulmonary atresia (with ventricular septal defect)]

The study has been carried out on eight cases of pulmonary atresia with ventricular septal defect, this abnormality involves both valvular atresia and the stenosis of the outflow tract of the right ventricle; that means a pulmonary atresia both valvular and infundibular. The atresia is always combined with a dextroposition of the aorta, overriding the ventricular septum; often with an auricular septal defect or a patency of the foramen ovale. In two cases, other associative caridac malformations exist : either a single ventricle or an atresia of the tricuspid valve. The arterial supply to the lungs may be assured by a persistent duct or by systemic arteries, or an association of both.     

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)     

Anatomic correction of transposition of the great arteries with ventricular septal defect. Experience with 118 cases

One hundred eighteen patients, 100 with transposition of the great arteries plus ventricular septal defect and 18 with double-outlet right ventricle and subpulmonary ventricular septal defect have undergone arterial switch and patch closure of the ventricular septal defect since February 1983. In transposition of the great arteries the ventricular septal defect was perimembranous in 70 cases, trabecular in 28, and infundibular in 10. Eleven patients had multiple ventricular septal defects. In addition to 18 patients with double-outlet right ventricle, malalignment of the conal septum was present in 19 cases. Coronary type A distribution was recognized in 79 cases, type C in one, type D in 24, and type E in 14. Great arteries were side by side in 19% of cases. Aortic coarctation was present in 31 cases, and subaortic obstruction in 9. Age at operation ranged from 4 days to 4 years (mean, 3.5 +/- 8.3 months), and mean weight was 4.0 +/- 1.6 kg. Thirty-seven infants were younger than age 1 month. Thirty-six patients underwent previous operations: pulmonary artery banding alone (n = 13), pulmonary artery banding and coarctation repair (n = 13), and coarctation repair alone (n = 10). Mean time between the first procedure and the switch was 2.2 months. Six patients with aortic coarctation underwent one-stage repair, through median sternotomy, aortic reconstruction, closure of the ventricular septal defect, and arterial switch. Perioperative mortality was 13.5% (70% confidence limit 10% to 17.6%, n = 16). It was directly related to coronary artery kinking in 50% of deaths and to anatomy and size discrepancy of the great arteries in the remaining deaths. Univariate analysis could not find any significant risk factor of in-hospital mortality. Mean follow-up of 30.3 +/- 23.5 months was achieved in all but 2 survivors. There was one late death. Ten patients underwent 11 reoperations for recoarctation (n = 1), pulmonary stenosis (n = 7), residual ventricular septal defect (n = 2), and stenosis of superior vena cava (n = 1). Two patients needed a permanent pacemaker. Actuarial survival and freedom from reoperation at 5 years were 84.5% +/- 3.6% and 85.7% +/- 4.6%, respectively. We conclude that anatomic correction of complex transposition is a safe method that offers good early and midterm results.     

Transatrial repair of ventricular septal rupture under preoperative localization by transesophageal echocardiography.

We report about a 71-year-old woman with postinfarction ventricular septal rupture who was successfully treated by the transatrial closure under preoperative localization by transesophageal echocardiography. In an attempt at transatrial repair of the ventricular septal rupture, the most important thing is preoperative localization of the defect in the septum, which is located high and posterior, where it is smooth with relatively few trabeculations and can be readily exposed by retraction of the tricuspid valve.     

Correction of Tetralogy of Fallot with Combined Transatrial and Pulmonary Approach: Experience with 184 Consecutive Cases

The authors present their technique and results for complete correction of Fallot's tetralogy by closure of the ventricular septal defect by the transatrial approach, and resection of the infundibular hypertrophy by a combined approach through the tricuspid and pulmonary valve orifices. Right ventricular pressures were often high at the end of operation due to a residual muscular gradient but the outcome of these postoperative gradients was similar to that of valvular pulmonary stenosis with an intact ventricular septum.     

New surgical approach to palliate tricuspid atresia in infants

An experimental surgical technique is proposed to increase pulmonary blood flow in tricuspid atresia with normally related great arteries. The method consists of the creation of ventricular septal defect in the infundibular septum by means of a closed surgical procedure. The expected advantage would be the growth of the right ventricular cavity. This would permit performance of a Fontan's procedure later, using the right atrium and the right ventricle. Microscopic sections from the hearts of 4 infants showed no damage in the conduction system. When performed in the beating hearts of dogs, the feasibility of the procedure was tested repeatedly without production of rhythm disturbances.     

The morphologic nature of noncommitted ventricular septal defects in specimens with double-outlet right ventricle

BACKGROUND: Lev's contribution to the understanding of the morphology of hearts with double-outlet right ventricle and the surgical feasibility for correction is important and remains in current use. However, the term noncommitted ventricular septal defect remains enigmatic. The aim of this study was to elucidate the morphologic nature of the noncommitted ventricular septal defect in view of its surgical implications. METHODS: We examined 67 specimens with double-outlet right ventricle, focusing on the relationship of the ventricular septal defect to the semilunar orifices. RESULTS: The defect was subaortic, subpulmonary, or doubly committed in 55 specimens. In a further 8 specimens, the defect opened into the outlet portion of the right ventricle, but the distance between the ventricular septal defect and the semilunar orifice was extensive, either because of extreme dextroposition of the aorta or a broad ventriculoinfundibular fold, which, in some cases, was associated with a long-outlet septum. A truly noncommitted ventricular septal defect was found in the inlet in the remaining 4 specimens. An atrioventricular septal defect without extension to the outlet was present in 3 cases, and a ventricular septal defect limited to the inlet was found in another case. The ventriculoinfundibular fold, part of the outlet septum and septal band or septomarginal trabeculation, had fused to form a crestlike structure. The septomarginal trabeculation is a useful landmark in the right ventricle to differentiate the inlet from the outlet in different forms of double-outlet right ventricle. CONCLUSION: We do not suggest to discard the Lev terminology but rather to differentiate the noncommitted ventricular septal defect into 2 types: the truly noncommitted defect of the inlet type and the not-directly-committed defect, which does open into the outlet portion of the right ventricle. The implication for the surgeon is 2-fold. The tricuspid valve or right part of the atrioventricular valve is interposed between the noncommitted ventricular septal defect and the semilunar orifice. The not-directly-committed defect opens into the outlet portion of the right ventricle but is not directly subaortic or subpulmonary.     

Management of ventricular septal defect: a survey of practice in the United Kingdom.

BACKGROUND: A survey of pediatric cardiac surgeons was performed to establish current opinions in the United Kingdom concerning closure of ventricular septal defect. METHODS: Questionnaires were sent to 14 pediatric cardiac centers in 1995 (16 surgeons, 100% response), and again in 1997 (20 surgeons, 100% response). RESULTS: Results are presented for 1997, with findings from 1995 shown in parentheses. Eleven (6) surgeons used bypass exclusively, 9 (10) sometimes used circulatory arrest. Operative techniques were similar, although the material used for the patch varied. Multiple defects were approached via the transatrial route by 18 (15), right ventriculotomy by 11 (7) and left ventriculotomy by 7 (6). The juxta-arterial defect was approached via the transpulmonary route by 16 (13), a combination by 9 (11), transatrial by 10 (6), and transventricular by 9 (5). The most common indications for pulmonary arterial banding were "Swiss cheese" defect for 13 (13), and functionally single ventricle for 5 (6). Ventricular septal defect associated with coarctation was repaired in two stages by 13 (10), a single stage by 5 (3), or either by 1 (3). CONCLUSIONS: Pediatric cardiac surgeons in the United Kingdom demonstrate a uniform, evidence-based approach to the management of ventricular septal defect.     

Exclusive double outlet right ventricle with atrioventricular concordance and pulmonary stenosis. Results of reconstructive surgery

Double outlet right ventricle associated with atrioventricular concordance, pulmonary stenosis and situs solitus of the atria is a subset of double outlet right ventricle related through the surgical treatment. From 1974 to 1985, 14 patients, 5 males, 9 females (mean age 8.9 years, range 13 months-22 years) were operated upon. All patients had infundibular stenosis and normal or large pulmonary arteries. The apex of the heart was to the right in 2 patients, the right and left ventricles were superior and inferior in 2 patients and 1 patient had both anomalies. The ventricular septal defect was subaortic in 11 patients (aorto-mitral discontinuity in 5) and non-committed in 3 patients. Three patients had 2 ventricular septal defects. The aorta was anterior in 3 patients and to the right of the pulmonary artery in 11 patients. All patients, through a transventricular and transatrial approach, had a reconstructive surgery. In 3 patients, an aortic homograft valved conduit was used. One patient had the ventricular septal defect enlarged. There was one early death (7.1%) from high residual right ventricle pressure and no late death. One patient had a transient atrioventricular block. One patient was reoperated upon for a residual ventricular septal defect. All survivors had a good clinical result. Re-evaluation in 8 patients confirmed excellent haemodynamics: the right ventricle to pulmonary artery pressure gradient decreased from 80 mm Hg (range 60-95) preoperatively to 24 mm Hg (range 3-32) postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)     

Arterial switch operation after left ventricular retraining in the adult

Retraining the morphological left ventricle in transposition of the great arteries has been successfully reported in infancy, while older age seems to be a contraindication. A 23-year-old woman with ?S,D,D? transposition of the great arteries and ventricular septal defect developed severe right systemic ventricular dysfunction 22 years after Mustard procedure and ventricular septal defect closure. Hemodynamic investigation revealed moderate pulmonary hypertension and preserved left ventricular function. A pulmonary artery band was applied to obtain a left-right ventricular pressure ratio of 0.91. Her postoperative course was characterized by biventricular failure, treated effectively with inotropic support. Six months later, she underwent a Mustard baffle takedown and arterial switch procedure. Her postoperative course was uneventful. She was discharged home on postoperative day 15. At 24-months follow-up, she is in excellent clinical condition; echocardiographic evaluation shows good left ventricular function (ejection fraction: 0.69) with left ventricular volume within normal limits (70 ml/m2). Our experience demonstrates that, despite adult age, a staged arterial switch operation can be performed successfully in selected patients when left ventricular function is preserved.     

Systemic right ventricular failure after atrial switch operation: midterm results of conversion into an arterial switch

BACKGROUND: Failure of the systemic right ventricle after atrial switch operation can be treated by conversion into an arterial switch operation. METHODS: Four patients, age 38 to 59 months, presented with right ventricular failure after Senning operation and ventricular septal defect closure. One patient had elevated left ventricular pressure; in the other three patients the left ventricle was retrained to a left ventricular/right ventricular pressure ratio of 0.8 or greater by pulmonary artery banding in 12 to 24 months. RESULTS: Postoperative course after arterial switch operation was prolonged, but clinical condition was good at discharge. Fractional shortening ranged from 20% to 28%. Trace-to-moderate aortic regurgitation was present; only 1 patient had preserved sinus rhythm. After a mean follow-up of 43.5 months 1 patient had died due to left ventricular dysfunction. The survivors are in New York Heart Association functional class I to II. Fractional shortening has improved (29% to 37%); aortic regurgitation has not increased. No patient has undisturbed sinus rhythm. CONCLUSIONS: Conversion of an atrial into an arterial switch is an alternative to cardiac transplantation in childhood. However, the procedure is demanding. Long-term morbidity is caused by rhythm disturbances. Aortic valve performance and left ventricular function require close observation.     

Post aortic left innominate vein in a patient with a surgically corrected double outlet right ventricle]

We report the presence of a post aortic left innominate vein (PALIV) in a patient with a surgically corrected double outlet right ventricle. A 30-year-old male was admitted to our hospital with exertional dyspnea. The patient had undergone right ventricular outflow tract reconstruction and closure of ventricular septal defect at the age of 14. Echocardiography and cardiac catheterization showed severe pulmonary regurgitation and a residual ventricular septal shunt. After resternotomy, right ventricular outflow tract reconstruction and residual shunt closure were performed. During the operation, the left innominate vein was not found in front of the aorta. Postoperative cardiac catheterization and computed tomography showed that the left innominate vein was positioned behind the ascending aorta draining to the superior caval vein.     

Jatene procedure for the intermediate type between original Taussig-Bing malformation and posterior transposition of the great arteries: a case report

A successful Jatene procedure (Lecompte modification) in a 8-month old boy with the intermediate type between the original Taussig-Bing and the posterior transposition of the great arteries (p-TGA) is described. The aorta was located posterior to the right of the pulmonary artery (PA) which overrode the interventricular septum. There was no fibrous continuity between aortic and atrioventricular valves. In the original Taussig-Bing, intraventricular rerouting is applicable because of malaligned infundibular septum. However, in our case which was similar to p-TGA, the infundibular septum extending anteriorly as far down as the anterior papillary muscle, was almost aligned to the interventricular septum, so that intraventricular rerouting is not applicable but arterial switch operation is preferable. Hence, the primary interventricular foramen was enlarged by the wedge resection and the secondary interventricular foramen was closed using pericardial patch through PA. The continuity of PA was reestablished anterior to the new aorta. The left half of distal orifice of PA was suture-closed to shift the site of anastomosis rightwards to avoid kinking and compression on the coronary arteries. Postoperative catheterization demonstrated no pressure gradient between left ventricle and aorta.     

Clinical results of arterial switch operation for double-outlet right ventricle with subpulmonary VSD.

OBJECTIVE: An arterial switch operation is considered a good alternative for the repair of double-outlet right ventricle (DORV) with atrioventricular concordance connection and subpulmonary ventricular septal defect (VSD) when intraventricular rerouting is not feasible. The clinical results of an arterial switch operation with ventricular septal defect closure for this anomaly were studied. METHODS: Between 1986 and 1997, 27 patients ranging from 10 days to 5 years of age (mean 0.4 years) underwent an arterial switch operation with ventricular septal defect closure for the correction of double outlet right ventricle with subpulmonary VSD. The 50% rule was used to define double-outlet right ventricle. Arch anomalies were associated in nine cases, and were corrected either previously or simultaneously. A subarterial muscle resection was performed in 14 without any subsequent stenosis of the ventricular outflow tract. The relationship of the great arteries was mostly anteroposterior in 15 and mostly side by side in 12. The left coronary artery (main trunk or circumflex artery) courses behind the pulmonary artery in 15/27 (six/15 in the anteroposterior relation and ten/12 in the side by side relation). The Lecompte maneuver was used to reconstruct the pulmonary artery in all but five cases with a side by side relationship of the great arteries. RESULTS: There was one operative death (3.7%) and three late deaths. The actuarial survival rate was 83 +/- 8% at 9 years. Right ventricular outflow tract obstruction including peripheral pulmonary stenosis developed in seven cases operated on in the early era. The reoperation free rate was 46 +/- 20% at 9 years. CONCLUSION: Although double-outlet right ventricle with subpulmonary VSD has complex features, including an aortic arch obstruction and coronary artery anomalies, an optimal definitive surgical repair using an arterial switch operation can be performed safely with a thorough understanding of this variable anomaly. The prevention of right ventricular outflow tract obstruction at the time of an arterial switch operation may thus help improve the rate of late morbidity.     

Pulmonary root translocation in transposition of great arteries repair

Anterior pulmonary root translocation is used as a new approach for anatomic repair of transposition complexes with ventricular septal defect and pulmonary stenosis. It is performed to construct the right ventricle outflow tract, after patch diversion of left ventricle to aorta. Since 1994, 3 infants underwent this procedure. The preliminary results indicate some growth of the pulmonary root and suggest that this technique could diminish reoperations in this group of patients.