Biventricular repair of transposition of the great arteries and unbalanced ventricles.

BACKGROUND: It is well established that the arterial switch operation is the surgical procedure of choice in patients with transposition of the great arteries and balanced ventricular anatomy. The surgical approach of choice in patients with transposition but unbalanced ventricular size is unknown. OBJECTIVES: Since the beginning of the arterial switch operation program, patients with transposition of the great arteries and unbalanced ventricles underwent biventricular repair by means of the arterial switch operation and repair of any associated lesions, either through a single or staged surgical procedure. The aim of this retrospective study is to analyze whether this approach can be proposed to such patients. METHODS: Forty-four patients with transposition of the great arteries and unbalanced ventricles underwent this surgical approach since 1984. Two groups were defined: group I had transposition with a dominant right ventricle (n = 28), and group II had transposition with a dominant left ventricle (n = 16). In group I the median age and weight at the arterial switch operation were 8.5 days (range, 5-70 days) and 3.1 kg (range, 1.5-3.7 kg), respectively. The median end-diastolic left ventricular volume, mass, and long-axis ratio were 15 mL/m2 (range, 11-16 mL/m2), 31.5 g/m2 (range, 20-66 g/m2), and 0.85 (range, 0.9-0.7), respectively. The mitral valve diameter was slightly hypoplastic, with a median z value of -1.22 (range, -0.3 to 3.7). In group 2 the median age and weight at the arterial switch operation were 42 days (range, 8 days-15 years) and 3.5 kg (range, 2.8-35 kg), respectively. Associated lesions in this group were coarctation in 9 and single (n = 12) or multiple (n = 4) ventricular septal defects. The median long-axis ratio and tricuspid z value were 0.6 (range, 0.3-0.8) and -0.9 (range, -0.5 to 3.3), respectively. In this group 9 patients had a single-stage procedure with fenestrated ventricular defect patches, atrial septal defect patches, or both; 7 patients underwent the staged approach. RESULTS: In group I there was 1 early death from sepsis after weaning from postoperative extracorporeal membrane oxygenation. Three patients had severe pulmonary hypertension, one of whom died 1 year later. All survivors demonstrated, at discharge from the hospital, equilibrated ventricular size, with a median left ventricular end-diastolic volume of 25 mL/m2 (range, 21-30 mL/m2). In group II there were 2 early and 1 late deaths. All early deaths occurred in patients without voluntary residual intracardiac shunts. Median early postoperative long-axis ratio and tricuspid z value were 0.8 (range, 0.7-1) and -0.2 (range, 0.74 to 1.2), respectively. CONCLUSION: This study demonstrates that the arterial switch operation in patients with transposition of the great arteries and unbalanced ventricles remains a good surgical option.

Late outcome after arterial switch operation for transposition of the great arteries.

BACKGROUND: Early and midterm results of the arterial switch operation (ASO) in transposition of the great arteries (TGA) are good, but late outcome data in large populations are still few. METHODS AND RESULTS: Twelve hundred patients had an ASO for TGA between 1982 and 1999, with prospective follow-up of 1095 survivors. Outcome measures included late death, reoperation, aortic insufficiency (AI), pulmonary stenosis (PS), and coronary anomaly. Median follow-up was 4.9 years (range 0.5 to 17 years). Late death occurred in 32 patients; survival was 88% at both 10 and 15 years. The hazard function for death declined rapidly, with no deaths after 5 years. Late mortality was correlated with reintervention and major events in the intensive care unit. Reoperation was performed in 103 patients, more often in complex TGA; the cause was mainly PS. Freedom from reintervention was 82% at 10 and 15 years, with a hazard function that declined rapidly but slowly increased after 3 years. At the last follow-up, PS was present in 3.9% of patients, and grade II or more AI was present in 3.2%, with a cumulative incidence of 9% at 15 years. Among the 278 patients who had a coronary arteriography, 8% had coronary lesions. Normal left ventricle and sinus rhythm were seen in 96.4% and 98.1%, respectively. CONCLUSIONS: Fifteen years after ASO, late mortality was low, with no deaths after 5 years; reoperation, mainly owing to PS, occurred throughout the follow-up. AI and coronary obstruction are rare but warrant further follow-up. Good left ventricular function and sinus rhythm are maintained.

Surgical management and indication of left ventricular retraining in arterial switch for transposition of the great arteries with intact ventricular septum.

OBJECTIVE: Arterial switch is the operation of reference for the surgical treatment of transposition of the great arteries. In cases of late referral, perinatal complications or early left ventricular (LV) dysfunction, the one stage arterial switch is contra indicated. Anatomical repair remains possible in these patients following a LV retraining. METHODS: From January 1992 to January 2000, a LV retraining was attempted in 22 patients with transposition of the great arteries with intact ventricular septum (TGA IVS), whereas 470 direct arterial switch and 2 Senning were performed. Indication for LV retraining was based on a combination of factors including: an age older than 3 weeks, a "banana shape" aspect of the inter-ventricular septum and mainly a LV mass <35G/m(2). RESULTS: The mean age at LV retraining was 3.2 months ranging from 9 days to 8 months. Usually conducted by sterntomy, it associated a loose PA banding with a LV/RV at 65% with a systemico-pulmonary shunt. The first stage was associated with frequent LV dysfunction and the LV retraining was discontinued in two patients in favor of one Senning and one early switch followed by ECMO. One patient died at first stage from a mediastinitis. Nineteen patients underwent a second stage arterial switch that was performed when the LV mass had reached 50 G/m(2) after a mean delay of 10 days, ranging from 5 days to 6 weeks. After a mean follow up of 25 months, there was one non-cardiac late death. The 17 patients followed and leaving with an arterial switch are in NYHA class I, with a mean LV shortening fraction of 39%. CONCLUSIONS: Arterial switch following LV retraining in TGA IVS is a satisfactory option. The inferior limit of 35 G/m(2) adopted, to indicate LV retraining, seems a safe landmark. The quality of the myocardium generated and the respective roles played by the LV afterload, LV wall shear stress, LV inflow and outflow to induce the LV remodeling remain under debate.

Surgical management of transposition of great arteries associated with multiple ventricular septal defects.

OBJECTIVE: The presence of associated multiple ventricular septal defects (VSDs) increases the risk of the anatomic repair for transposition of the great arteries (TGA). The aim of this study was to define the optimal management of this complex anomaly. METHODS: Between January 1988 and December 1998, 45 patients underwent anatomic repair of TGA associated with multiple VSDs. The median age was 50 days and the median weight 4 kg. Eighteen (40%) had undergone previous palliation including 17 pulmonary artery banding procedure (PAB), seven associated with coarctation repair and one isolated coarctation repair. The perimembraneous septum was involved in 24 patients, the trabecular in 43, the inlet in seven and the infundibular in two. Closure of the VSDs included Dacron or pericardial patchs and matress sutures. The initial approach was through right atriotomy which was sufficient in 15 patients. VSDs were closed through right ventriculotomy in 13 patients, through pulmonary artery in six, through the aorta in one and in the remaining (n = 10) combined approaches were used. Only one patient required left apical ventriculotomy. RESULTS: There were five hospital deaths (11%; 70% CL: 6-18%) including the one early reoperation for residual VSD closure. Five patients had successful early reoperation for secondary PAB for residual VSD. Three late deaths occurred (7%; 70% CL: 3-13%). At the last visit, 95% of survivors were asymptomatic and without any cardiac medication. CONCLUSION: Mid-term survival with good quality of life can be achieved following either one or two-stage repair of this complex anomaly. In the presence of VSD closure failure a secondary PAB may be the procedure of choice.

[Biventricular repair in neonates with hypoplasia of the left ventricle]. / Réparation biventriculaire chez les nouveau-nés ducto-dépendants avec hypoplasie relative des cavités gauches.

BACKGROUND: Whether to perform uni or biventricular repair in ducto dependent neonates with hypoplastic but morphologically normal left ventricle and multi level left ventricle obstructions (hypoplastic left heart syndrome class III) remains unanswered. Echocardiographic criteria have been proposed for surgical decision. HYPOTHESIS: Increased afterload and multi level left ventricle obstruction is constant. We assumed that restoration of normal loading conditions by relief of left ventricle obstructions promotes its growth, provided that part of the cardiac output was pre operatively supported by the left ventricle, whatever the echocardiographic indices. METHODS: Twenty one ducto dependent neonates presented with this anomaly. All had aortic coarctation associated to multi level left ventricle obstruction. Pre operative echocardiographic assessment showed: mean end diastolic left ventricular volume of 13.3 +/- 3.5 mL/m2 and mean Rhodes score of -1.43 +/- 0.9. Surgery consisted in relief of left ventricle outflow tract obstruction by coarctation repair in 21 associated to atrial septal defect closure in 2, aortic commissurotomy in 1 and ascending aorta enlargement in 1. RESULTS: There were 3 early and 3 late deaths. There was no predictive risk factor for failure. Growth of the left heart was demonstrated in most patients. At hospital discharge the end diastolic left ventricular volume was 19.4 +/- 3.12 mL/m2 (p = 0.0001) and the Rhodes score was -0.38 +/- 1.01 (p = 0.0003). Actuarial survival and freedom from reoperation rates at 5 years were: 68.5% and 40.75%, respectively. CONCLUSION: Biventricular repair can be proposed to ducto dependent neonates with hypoplastic but morphologically normal left ventricle provided that all anatomical causes of left ventricle obstruction can be relieved. Secondary growth of the left heart then occurs, however the reoperation rate is not low.

Population pharmacokinetic of nadroparin calcium (Fraxiparine) in children hospitalised for open heart surgery.

Administration of low molecular weight heparin following heart surgery in paediatric patients in order to prevent thromboembolic events results in a large variation in anti-Xa activities. A population study was undertaken to determine pharmacokinetic parameters after nadroparin calcium (Fraxiparine) administration and the effects of potential covariates; this study included 154 children divided into two groups: a model group (124 patients) and a validation group (30 patients). The 432 anti-Xa activities were analysed using NONMEM on the basis of a one-compartment model with three parameters: apparent clearance, apparent volume of distribution and absorption rate. The influence of body weight, age, sex and dose regimen (once or twice daily) were investigated. The best fit corresponds to the formula: apparent clearance (l/min)=0. 541 x weight1.51/(6.151.51 + weight1.51) and apparent volume (l)=0.355 x weight. The inter-individual variability (expressed in coefficient of variation) of these parameters are high, especially with regard to the apparent volume (92%), but no other available covariate was found to explain this variability.

Biventricular repair approach in ducto-dependent neonates with hypoplastic but morphologically normal left ventricle.

OBJECTIVES: Increased afterload and multilevel LV obstruction is constant. We assumed that restoration of normal loading conditions by relief of LV obstructions promotes its growth, provided that part of the cardiac output was preoperatively supported by the LV, whatever the echocardiographic indexes. BACKGROUND: Whether to perform uni- or biventricular repair in ducto dependent neonates with hypoplastic but morphologically normal LV (hypoplastic left heart syndrome classes II & III) remains unanswered. Echocardiographic criteria have been proposed for surgical decision. METHODS: Twenty ducto dependent neonates presented with this anomaly. All had aortic coarctation associated to multilevel LV obstruction. Preoperative echocardiographic assessment showed: mean EDLW of 12.4 +/- 3.03 ml/m2 and mean Rhodes score of -1.73 +/-0.8. Surgery consisted in relief of LV outflow tract obstruction by coarctation repair in all associated to aortic commissurotomy in one and ASD closure in 2. RESULTS: There were 3 early and 2 late deaths. Failure of biventricular repair and LV growth was obvious in patients with severe anatomic mitral stenosis. The other demonstrated growth of the left heart. At hospital discharge the EDLVV was 19.4+/-3.12 ml/m2 (p = 0.0001) and the Rhodes score was -0.38+/-1.01 (p = 0.0003). Actuarial survival and freedom from reoperation rates at 5 years were 72.5% and 46%, respectively. CONCLUSIONS: Biventricular repair can be proposed to ducto dependent neonates with hypoplastic but morphologically normal LV provided that all anatomical causes of LV obstruction can be relieved. Secondary growth of the left heart then occurs; however, the reoperation rate is high.

[Double outlet right ventricle with sub-aortic obstruction caused by hypertrophy of the parietal band. Echographic image apropos of a case]. / Ventricule droit à double issue avec obstruction sous-aortique créée par une hypertrophie de la bande pariétale. Description échocardiographique à propos d'un cas.

The authors report the case of an 18 month old infant with double-outlet right ventricle and subaortic ventricular septal defect with severe subaortic obstruction. Echocardiography showed the subaortic obstruction to be due to severe hypertrophy of the parietal band which grew upwards to the aortic annulus. A peak instantaneous Doppler gradient of 72 mmHg was recorded between the right ventricle and the aorta. The hypertrophy of the band was secondary to pulmonary artery banding performed in the neonatal period at the same time as cure of an isthmic coarctation of the aorta. Surgical exploration confirmed the echocardiographic findings a circular subaortic muscular stenosis related to hypertrophy of the parietal band. Resection of this band liberated the left ventricular outflow tract and made possible a tunnelisation procedure between the left ventricle and the aorta. Postoperative Doppler echocardiography showed the absence of subaortic stenosis.

Two-stage correction of transposition of great arteries with complete atrioventricular canal.

We report a successful two-stage anatomic correction of D-transposition of the great arteries associated with complete atrioventricular canal without pulmonary obstruction, combining an arterial switch procedure and a two-patch repair for complete atrioventricular canal, after pulmonary artery banding in the neonatal period.

[Anatomical repair in transposition of great vessels]. / La réparation anatomique de la transposition des gros vaisseaux.

Transposition of the great arteries (TGA) covers a wide range of anatomic variants involving not only the origin of the great arteries, but the intracardiac structures and the aortic arch. TGA can now benefit from complete anatomic repair, namely switch operation, performed in simple forms during the neonatal period. Accurate pre and intraoperative assessment must take into account the main anatomic features: relationship between the aorta and the pulmonary artery and coronary arteries distribution. Over the last sixteen years, 1,063 patients with TGA underwent anatomic repair in our institution. Among 728 patients with simple TGA, 92% were operated on within the first 2 weeks of life. Among 335 patients with complex TGA, all had a large VSD and 154 had additional anomalies. Among these 335 patients, 264 had one-stage complete repair and 71 had two-stage repair, at the beginning of our experience. In complex forms the main associated anomalies involved the ventricles (right ventricle hypoplasia (11), malaligned VSD (90), restrictive VSD (3), multiple VSD (43), the atrioventricular valves (straddling of the mitral or tricuspid valve (15), mitral valve anomalies (cleft or tissue tag, 4) and the aortic arch (coarctation [88], interrupted aortic arch (8)). The hospital mortality was 6.9% for simple TGA, 14.6% for complex TGA. Main causes of early death were failure in coronary artery relocation and pulmonary hypertensive crisis. Mean follow-up (95.5% of patients) was 49 months. Overall 16-year survival was 87.9%, 91.5% for simple forms and 80.4% for complex forms. Anatomic repair is nowadays the treatment of choice for TGA. Long-term results will depend on the evolution of the relocated coronary arteries and of the neo-aortic valve.

Biventricular repair of conotruncal anomalies associated with aortic arch obstruction: 103 patients.

BACKGROUND: Biventricular repair of conotruncal anomalies associated with aortic arch obstruction is a complex surgical procedure that combines a cardiac repair and a aortic arch reconstruction. METHODS AND RESULTS: From January 1984 to April 1996, such a repair was performed in 103 patients. The conotruncal anomalies included: 15 transpositions of the great arteries (TGAs) with intact ventricular septum, 44 TGAs with ventricular septal defect, 32 double outlet right ventricle with subpulmonary ventricular septal defect, 10 truncus arteriosus, one double outlet left ventricle, and one tetralogy of Fallot. The arch obstruction included 88 coarctation and 15 interrupted aortic arch. One-stage repair has been the favored technique since 1990 and was performed in 58 neonates, including 38 TGAs or double outlet right ventricle and ventricular septal defect, 10 TGAs with intact ventricular septum, and all of the 10 truncus arteriosus. The cardiac repair included 89 arterial switch operations, 2 Kawashima rerouting, 10 truncus arteriosus repairs, and one double-outlet left ventricle repair and one tetralogy of Fallot repair. The aortic arch was reconstructed by direct anastomosis in 85 patients, with a Gore-Tex conduit in three patients and more recently by an ascending aortic patch augmentation in 15 patients. The hospital mortality was 12% (7 of 58) for the one-stage repair and 20% (9 of 45) for the two-stage repair. There were six late deaths. Reoperations or angioplasties were mandatory for 12 right ventricle outflow tract obstructions after arterial switch, involving 10 patients with double outlet right ventricle (P=.02), 10 recurrent arch obstruction, and six miscellaneous lesions. CONCLUSIONS: One-stage biventricular repair of conotruncal anomalies associated with aortic arch obstruction can be achieved in selected patients with an 83% survival rate at 7 years.

[Secondary subaortic obstruction after correction of double outlet right ventricle. Echocardiographic aspects and surgical findings]. / Obstruction sous-aortique secondaire après cure de ventricule droit à double issue. Aspects échocardiographiques et confrontation chirurgicale.

The authors report echocardiographic appearances of subaortic obstruction secondary to correction of double outlet right ventricle in 8 children reoperated between January 1994 and June 1996. The initial repair of the double outlet right ventricule was performed before 3 months of age in 6 cases. Forms with subaortic ventricular septal defects (4 cases) and those with ventricular septal defects not connected to the great arteries (2 cases) were treated by tunneling between the left ventricule and the aorta, and forms with subpulmonary ventricular septal defects (2 cases) by tunneling between the left ventricle and pulmonary artery and detransposition. Reoperation for subaortic obstruction was performed after 18 to 33 months. The instantaneous maximal gradient measured by Doppler echocardiography was high, ranging from 60 to 145 mmHg. The causes of the secondary subaortic obstruction were stenosis of the tunneling patch (n = 2), subaortic fibrous ring (n = 3), muscular septal hypertrophy (n = 1), antero-lateral muscular hypertrophy (n = 1), relics of tricuspid tissue inserted on the infundibular septum (n = 1). Subaortic obstruction was diagnosed in the echocardiographic subcostal views in all cases; the nature of the obstruction was determined in 6 of the 8 cases. The mechanism of obstruction by stenosis of the tunneling patch was only detected in 1 of the 2 cases. This study show, 2D echocardiography to be the investigation of choice for follow-up of operated double outlet right ventricle for the diagnosis of secondary subaortic obstruction.

Midterm results after surgical repair of the anomalous origin of the coronary artery.

BACKGROUND: Immediate results after surgical repair of the anomalous origin of the coronary artery are well known, but few studies reported midterm results in a relatively large population. METHODS AND RESULTS: Between 1980 and 1995, 39 consecutive patients with anomalous origin of the coronaries artery from the pulmonary artery had restoration of a two-coronary-artery system. Median age at surgery was 18.5 months (range, 2.7 months to 38 years). Left ventricular (LV) function was normal in 13 patients. Direct aortic implantation was performed in 34 patients (87%), associated with mitral valvuloplasty in 3 patients. Hospital death occurred in 5 patients (13%; 70% confidence limits [CL], 8 to 20). Two patients were lost to follow-up. Mean follow-up was 40 +/- 42 months. There was no late death after 1 month, and the survival rate was 84% (70% CL, 67 to 93). One patient had a mitral valvuloplasty 18 months after reimplantation. At the last follow-up, LV shortening fraction (SF) was normal in 86% (70% CL, 61 to 96), but LV dilation persisted in 73% of patients, and 12 of the survivors (39%) had abnormal regional wall motion of the left ventricle. A perfusion defect with incomplete redistribution was observed in 8 patients on thallium-201 imaging performed in 45% of survivors. Total mortality was related only to the preoperative SF: 12% versus 24.8% (P < .05). No factor was related to SF recovery. CONCLUSIONS: Despite no late deaths and SF recovery, LV dilation and ischemic segments of the left ventricle persisted at the long-term follow-up.

Aortico-left ventricular tunnel in fetuses and infants.

BACKGROUND: Aortico-left ventricular tunnel is a rare congenital abnormal communication between the aorta and the left ventricle presenting in early childhood as aortic regurgitation and cardiac failure. This condition has rarely been reported in fetuses. Operation is the only treatment, and postoperative aortic incompetence could be related to the age or the type of repair. METHODS: We conducted a retrospective, two-institution review, from 1983 to 1995, of aortico-left ventricular tunnel diagnosed in utero and before 6 months of age. RESULTS: Three cases of aortico-left ventricular tunnel were diagnosed in utero by Doppler echocardiography between 22 and 24 weeks' gestation. Prenatal aortico-left ventricular tunnel was associated with severe left ventricular dysfunction, aortic valve anomalies, and fetal hydrops. One death occurred in utero and one immediately after birth, and in 1 case pregnancy was interrupted. In these 3 cases the diagnosis was confirmed by autopsy. Three neonates and 2 infants had the diagnosis of aortico-left ventricular tunnel made after birth and underwent successful surgical repair. At short and midterm follow-up all patients are alive and aortic valve regurgitation is absent or trivial. CONCLUSIONS: This series shows that aortico-left ventricular tunnel covers an anatomic spectrum of lesions. Cases diagnosed in utero by Doppler echocardiography are characterized by severe ventricular dysfunction, associated aortic valve lesions, and poor outcome. Postnatal cases represent the more favorable end of the spectrum, with no associated lesions, and can be repaired without mortality and with good functional results.

Truncus arteriosus repair: influence of techniques of right ventricular outflow tract reconstruction.

Fifty-six consecutive patients underwent total correction of truncus arteriosus. Median age at repair was 41 days, with a range of 2 days to 8 months. In 71% the operation was done in the first 2 months of life. Nine patients had complex forms of truncus and 11 patients had aortic insufficiency. The truncal aortic root was transected, which provides a clear exposure of the coronary ostia. The aorta was reconstructed by direct end-to-end anastomosis, and the truncal valve was preserved in every case. Several different techniques were used for pulmonary reconstruction, including three types of anatomic reconstruction of the pulmonary valve with a trisigmoid leaflet system and two types of nonanatomic reconstruction. The anatomic techniques included use of 33 Dacron valved conduits, eight homograft valved conduits, and one porcine aortic root bioprosthesis. The nonanatomic reconstructions included direct anastomosis to the right ventricle in nine patients and insertion of autologous pericardial valved conduits in five. The hospital mortality was 16% (9/56; 95% confidence limits, 2% to 30%). Multivariate analysis outlines two independent incremental risk factors for hospital death: nonanatomic pulmonary valve reconstruction techniques and age younger than 1 month. The hospital mortality was 7.1% in the group with anatomic pulmonary valve reconstruction versus 43% in the group with nonanatomic pulmonary valve reconstruction (p = 0.015). The hospital mortality was 5.7% in those older than 1 month versus 33% in those younger than 1 month of age (p = 0.04). There were two late deaths. The actuarial freedom from reoperation and angioplasty at 7 years was 100% for patients receiving pericardial conduits, 80% for those undergoing direct anastomosis, 77% for those receiving Dacron conduits, and only 43% for those receiving homografts (p = 0.02). In conclusion, anatomic reconstruction of the pulmonary valve seems important at the time of the operation, age younger than 1 month remains an incremental risk factor, and the truncal valve can be preserved.

Transposition of the great arteries complicated by tricuspid valve incompetence.

BACKGROUND: Tricuspid valve insufficiency secondary to structural anomalies of the valve itself or to an iatrogenic complication of the Rashkind procedure is very rarely associated with transposition of the the great arteries. This condition represents an interesting perioperative challenge. Rapid restoration of the tricuspid valve to a low-pressure system by arterial switch operation associated with tricuspid repair should theoretically improve the outcome in terms of myocardial and valve function. METHODS: Thirteen of 839 patients who underwent an arterial switch operation for various forms of transposition of the great arteries presented with moderate to severe tricuspid insufficiency. Three of them had a ventricular septum defect. Nine experienced severe cardiac failure with profound hypoxemia. Ventilatory support was necessary in 7, 6 had renal or hepatic dysfunction, and 5 had coagulation disorders. Inotropic support was started preoperatively in 8 patients. RESULTS: Tricuspid lesions were as follows: primary annular dilatation and lack of coaptation at the commissural level (n = 1), straddling tricuspid valve (n = 1) redundant tricuspid valve tissue leading to left ventricular outflow tract obstruction (n = 1), small cleft of the septal leaflet (n = 1), and dysplastic valve tissue with juxtacommissural regurgitation (n = 1). In 8 patients, the cause of the tricuspid valve insufficiency was most probably an iatrogenic lesion, with rupture of the papillary muscle (n = 2), rupture of the chordae (n = 1), or tear of the anterior leaflet (n = 5), whereas no clear cause could be found in 1 patient. Repair consisted of the arterial switch operation associated with tricuspid valve repair in 10 patients. In 2 patients with only discrete anomaly and in 1 without a clear cause of tricuspid regurgitation, no valve repair was performed. Three patients had their ventricular septal defect closed. There were only one early and one late death, both not related to the tricuspid lesions. Late postoperative (mean, 6.5 years) evaluation revealed normal left ventricular function in 10, with no tricuspid incompetence in 7 and trivial tricuspid insufficiency in 3. CONCLUSIONS: Restoration of an incompetent tricuspid valve in a low-pressure system by the arterial switch operation combined with valve repair provides good ventricular and valvar results. Preoperative management and appropriate timing of operation seem to be of utmost importance.

Surgical approaches for double-outlet right ventricle or transposition of the great arteries associated with straddling atrioventricular valves.

The surgical management of patients with double-outlet right ventricle or transposition of the great arteries and straddling atrioventricular valves remains a subject of controversy. Biventricular repair has theoretic advantages because it establishes normal anatomy and physiology. In some instances, however, it seems to carry too high operative risk, and a univentricular heart repair is preferred. Since 1984, we have operated on 34 patients with double-outlet right ventricle (n = 15) or transposition of the great arteries (n = 19) with isolated straddling tricuspid valve (n = 17), isolated straddling mitral valve (n = 9), both mitral and tricuspid straddling (n = 2), or abnormal insertion of tricuspid (n = 7) or mitral (n = 2) chordae in the left ventricular outlet, precluding an adequate tunnel construction. Straddling was categorized according to the location of the papillary muscle insertion in the opposite ventricular chamber: type A, on the edge of the ventricular septal defect (n = 14); type B, on the opposite side of the ventricular septum away from the edge of the defect (n = 8); type C, on the free wall of the opposite ventricular chamber (n = 8). Abnormal chordal insertions were classified according to the location of their attachments around the edges of the defect. Three types of chordal distribution were identified: on the aortic conus, on the pulmonary conus crossing the ventricular septal defect, or around the defect closing it like a curtain. All but three patients had two ventricles of adequate size. Sixteen patients underwent palliation. Median age at the definitive operation was 6.5 months (range 1 to 130 months). Thirty patients underwent a biventricular repair and four had a univentricular repair. Biventricular repair was achieved by an arterial switch operation in 18 patients and by tunnel construction from the left ventricle to the aorta in 12. In isolated straddling of types A and B, the ventricular septal defect was closed by adjusting the septal patch on the ventricular side above the straddled papillary muscle. In type C, the patch was sewn over the papillary muscle by applying it on the septum. In double straddling, the ventricular septum was incised between the two papillary muscles, and an ellipsoid patch was used to reconstruct the septal defect, directing each subvalvular apparatus into its own ventricular chamber. When the abnormal chordae in the left outflow tract inserted on the aortic or pulmonary conus, the conus was incised and tailored to make a flap, leaving an unobstructed left ventricular outflow tract. In two patients the subvalvular apparatus was resected and reattached to the patch. Curtainlike chordae were a contraindication to biventricular repair in double-outlet right ventricle but not in transposition. There were four early deaths and one late death, all occurring in the group having biventricular repair. Death was due to myocardial ischemia (n = 1), right ventricular hypoplasia (n = 1), pulmonary hypertension (n = 1), and residual subaortic stenosis (n = 1). Two patients had moderate to severe postoperative atrioventricular valve incompetence, caused by a cleft in the mitral valve in one patient. Three patients were reoperated on for subaortic stenosis (n = 1), pulmonary stenosis (n = 1), and mitral regurgitation (n = 1). Mean follow-up of 30.7 +/- 19.4 months was achieved in the survivors. All but one patient (univentricular repair) were in New York Heart Association class I, without atrioventricular valve incompetence. Actuarial survival at 4 years was 85.3% +/- 3%. We conclude that straddling or abnormal distribution of chordae tendineae of the atrioventricular valves does not preclude biventricular repair in double-outlet right ventricle or transposition of the great arteries provided that the ventricles are of adequate size. Curtainlike abnormal tricuspid chordae remain a contraindication to biventricular repair in double-outlet right ventricle.

Reoperation after the arterial switch operation for transposition of the great arteries.

Although most children after an arterial switch operation for transposition of the great arteries have normal development and cardiac function, a few require reoperation. During the last 10 years, 68 of 753 patients who underwent arterial switch operations (9.3%) underwent 75 reoperations. Thirty underwent early reoperation (< 30 days or during the same hospital stay) and 38 underwent late reoperation. Causes for reoperation included pacemaker insertion (n = 5), left diaphragm plication (n = 4), revision for hemostasis (n = 1), mediastinitis (n = 2), superior vena cava thrombosis (n = 9), subvalvular pulmonic stenosis (n = 5), supravalvular pulmonic stenosis (n = 16), residual atrial (n = 2) or ventricular (n = 8) septal defects, isolated mitral valve insufficiency (n = 2), aortic valve insufficiency (either isolated [n = 1] or in association with mitral incompetence [n = 1] or stenosis [n = 1]), left coronary artery ostial stenosis (n = 1), and recurrent aortic (n = 6) or neoaortic (n = 4) aortic coarctation. In all but 27 patients, the residual defects were already present immediately after the completion of the arterial switch operation; however, only patients with critical lesions were reoperated on early. Interventional catheterization procedures were performed when indicated; however, they only postponed inevitable reoperation. Successful relief of superior vena cava thrombosis was achieved by atriojugular bypass grafting in two patients, by early open thrombectomy in six patients, and by direct patch angioplasty of the superior vena cava once. Patch plasty for subvalvular or supravalvular pulmonic stenosis was carried out in 21 patients, septal defect closure was carried out in nine patients, and pulmonary artery banding was performed in one patient with criss-cross atrioventricular relationship and multiple ventricular septal defects. Valve repair was performed in all five patients with either isolated or combined aortic and mitral valve dysfunction. One patient with left coronary ostial stenosis underwent a patch enlargement of this ostium. Recoarctation was repaired by end-to-end anastomosis in eight patients and by a subclavian flap and a patch angioplasty in one patient each. Seven patients underwent a second reoperation for supravalvular pulmonary stenosis (n = 3), mitral valve replacement (n = 1), ventricular septal defect closure (n = 1), and recurrent coarctation (n = 2). There were six intraoperative (8.8%) and two late deaths. All early deaths occurred after early reoperations.(ABSTRACT TRUNCATED AT 400 WORDS)

[Transposition of great vessels with position anomaly of the atrioventricular valves or chordae: echocardiographic aspects and surgical correlations]. / Transposition des gros vaisseaux avec anomalie de position des valves ou des cordages auriculo-ventriculaires: aspects échocardiographiques et confrontation chirurgicale.

The authors report the echocardiographic appearances of 27 cases of transposition of the great arteries (TGA) with ventricular septal defect (VSD) (including the Taussig-Bing malformation) complicated by displacement of one or both atrioventricular valves and/or chordae tendinae diagnosed between September 1985 and September 1994. An anatomical repair was performed in 21 cases and allowed accurate correlation of the echocardiographic and peroperative findings; a cavo-bipulmonary conduit or a palliative procedure was performed in 6 cases. The cases were classified in 3 groups: group I: 16 children with isolated tricuspid valve abnormalities; group II: 7 cases of mitral valve straddling; group III: 4 cases of displacement of both atrioventricular valves and/or their chordae. The cases of group I were divided into 3 subgroups: subgroup Ia with insertion of the tricuspid chordae on the infundibular septum (7 cases); subgroup Ib with isolated overriding of the tricuspid valve (2 cases); subgroup Ic straddling with or without overriding of the tricuspid valve (7 cases). In all three groups, there were 10 cases of straddling of the tricuspid and 11 cases of straddling of the mitral valve: 9 type A, 3 type B and 9 type C of Tabry's classification. Echocardiography misdiagnosed one case of type A straddling of the tricuspid valve for a type A overriding and straddling of the mitral valve. The ventricular septal defect was situated in the admission septum in the 10 cases of straddling of the tricuspid; in the 11 cases of straddling of the mitral valve the chordae passed through the superior anterior part of the interventricular septum.(ABSTRACT TRUNCATED AT 250 WORDS)

Surgical management of neonatal coarctation.

Between 1983 and 1994, 307 consecutive neonates underwent coarctation repair by a single surgical technique: extended end-to-end anastomosis. Mean age at operation was 13 +/- 8 days. Isolated coarctation was present in 95 patients (group 1), 102 patients had associated ventricular septal defect (group 2), and 110 patients had associated complex intracardiac lesions (group 3). Aortic arch hypoplasia was present in 81% of the patients (62% in group 1 versus 85% in group 2 and 93% in group 3: p < 0.001). In 271 patients, the aortic arch reconstruction was performed via a left thoracotomy with normothermia (100% of group 1, 95% of group 2, and 72% of group 3); in the other 36 patients, undergoing one-stage repair or palliation of the associated lesion, it was performed via a midline sternotomy during a short period of deep hypothermia and circulatory arrest (5% of group 2 and 28% of group 3). Pulmonary artery banding was performed in 94 patients. Spontaneous ventricular septal defect closure was observed in 39% of the patients of group 2 operated on via thoracotomy. Early mortality rates in groups 1 (2%) and 2 (2%) were significantly lower than in group 3 (17%) (p < 0.001). There were 29 late deaths, all related to associated cardiac lesions or their subsequent repair. The overall total mortality was 16.9%. In group 3 this rate was significantly higher in patients undergoing two-stage procedures (47%) than in those undergoing one-stage repair (23%) (p < 0.05). All but 14 survivors were followed up for a mean of 61 +/- 36 months. Actuarial survivals at 10 years were 98% in group 1, 94% in group 2, and 60% in group 3. The recoarctation rate was 9.8%, leading to 21 reoperations and three angioplasties without mortality. Patients with a more extended or severe form of aortic arch hypoplasia had a significantly higher risk of recoarctation (p < 0.001). Actuarial freedom from reoperation for recoarctation at 10 years was 93%. The findings of this study suggest that extended end-to-end anastomosis provides an adequate and safe repair of neonatal coarctation. Low recoarctation rate, owing to effective relief of the obstruction created by aortic arch hypoplasia and to complete resection of ductal tissue, freedom from major morbidity, and feasibility via both lateral and anterior approaches are the main advantages of the extended end-to-end anastomosis.(ABSTRACT TRUNCATED AT 400 WORDS)