Ventricular septal defects (VSD) can be associated with aortic valve prolapse (AVP) with or without regurgitation. Aortic valve prolapse may be due to both lack of leaflet support and Venturi's phenomenon. There is a great diversity in the literature about the anatomic type of the VSDs associated with AVP. The objective of the study was then to assess the anatomy of the VSD on echocardiography. From 2009 to 2017, 51 consecutive patients with VSD complicated by AVP were admitted for surgery. We screened retrospectively all preoperative echocardiographies in double-blind test. We analyzed the anatomic type of the VSD according to ICD-11 classification and looked for outlet septum malalignment, the nature of the prolapsed aortic leaflet, the severity of aortic regurgitation and other complications (subaortic membrane, right ventricular stenosis). The VSD was central perimembranous (pm) in 23 patients (45%), outlet with malalignment in 21 (41%) and outlet juxta-arterial in 7 (13.7%). We found a significant difference between the initial diagnosis and the final one after reviewing echography in double-blind test: among the 23VSD initially described as central pm, eleven were reclassified as outlet VSD, and one outlet VSD was reclassified as central pm (P < 0.001). Aortic valve prolapse involved the right aortic cusp in 98% of patients. Aortic regurgitation was severe in 2 patients, moderate in 5, mild in 24 and trivial or absent in 20. Outlet VSDs were significantly more often associated with aortic regurgitation (P = 0.036). Subaortic membrane was found in 8 patients (15.6%), 6 with outlet and 2withcentral pm VSD (P < 0.001). Right ventricular obstruction was found in 8 patients (15.6%). Aortic valve prolapse complicates indifferently central pm and outlet VSD. However, aortic insufficiency is more frequent in outlet VSD. The accurate echocardiographic diagnosis of the anatomic type of the VSD is difficult and multiple views are necessary.

OBJECTIVES This study sought to assess procedural characteristics, early clinical outcome, and long-term complications after transcatheter closure of atrial septal defect (ASD) in children. BACKGROUND Transcatheter closure has become the preferred strategy in most cases of isolated secundum ASD. However, reported experience in the pediatric population is limited. METHODS A 1998 to 2016 retrospective multicenter study was performed in 9 French tertiary institutions. All children who had an attempt of percutaneous ASD closure with an Amplatzer Septal Occluder were included. RESULTS In 1,326 children (39% males; median age, 9 years [0.7 to 18]; weight, 29 kg [3.6 to 92]), transcatheter ASD closure was performed. Median ASD size was 15 mm (3 to 41); 254 (19.1%) patients had a large ASD (>= 20 mm/m(2)). Procedural success rate was 95.3% (95% confidence interval: 93.9% to 96.3%). No death was observed but periprocedural complications occurred in 24 patients (1.8%). After a median follow-up of 3.5 years (range 6 months to 18 years; 173 patients [13%] followed > 10 years), delayed major complications were minimal (n = 12; 1.04%) including no death and/or cardiac erosion. Periprocedural and delayed complications rates were significantly higher in children <= 15 kg (5.2% vs. 1.5%; p = 0.007 and 3.1% vs. 0.7%; p < 0.007, respectively) and those with large ASD (3.5% vs. 1.4%; p = 0.008 and 1.7% vs. 0.7%; p = 0.052, respectively). CONCLUSIONS Transcatheter ASD closure using Amplatzer Septal Occluder is safe in children with a minimal rate of periprocedural complications and a favorable long-term outcome, especially with no death or cardiac erosion despite a substantial proportion of large defects. Children # 15 kg and those with large ASDs had a greater risk of complications. (c) 2018 by the American College of Cardiology Foundation.

Background—Data are needed on the safety and efficacy of device closure of large atrial septal defects. Methods and Results—Between 1998 and 2013, 336 patients (161 children <15 years) with large, isolated, secundum atrial septal defects (balloon-stretched diameter ≥34 mm in adults or echocardiographic diameter >15 mm/m2 in children) were managed using the Amplatzer device, at the Marie Lannelongue Hospital. Transthoracic echocardiographic guidance was used starting in 2005 (n=219; 65.2%). Balloon-stretched diameter was >40 mm in 36 adults; mean values were 37.6±3.3 mm in other adults and 26.3±6.3 mm/m2 in children. Amplatzer closure was successful in 311 (92.6%; 95% confidence interval, 89%–95%) patients. Superior and posterior rim deficiencies were more common in failed than in successful procedures (superior, 24.0% versus 4.8%; P=0.002; and posterior, 32.0% versus 4.2%; P<0.001). Device migration occurred in 4 adults (2 cases each of surgical and transcatheter retrieval); in the 21 remaining failures, the device was unreleased and withdrawn. After a median follow-up of 10.0 years (2.5–17 years), all patients were alive with no history of late complications. Conclusions—Closure of large atrial septal defects using the Amplatzer device is safe and effective in both adults and children. Superior and posterior rim deficiencies are associated with procedural failure. Closure can be performed under transthoracic echocardiographic guidance in experienced centers. Early device migration is rare and can be safely managed by device extraction. Long-term follow-up showed no deaths or major late complications in our population of 311 patients.

Background— Data are needed on the safety and efficacy of device closure of large atrial septal defects. Methods and Results— Between 1998 and 2013, 336 patients (161 children <15 years) with large, isolated, secundum atrial septal defects (balloon-stretched diameter ≥34 mm in adults or echocardiographic diameter >15 mm/m 2 in children) were managed using the Amplatzer device, at the Marie Lannelongue Hospital. Transthoracic echocardiographic guidance was used starting in 2005 (n=219; 65.2%). Balloon-stretched diameter was >40 mm in 36 adults; mean values were 37.6±3.3 mm in other adults and 26.3±6.3 mm/m 2 in children. Amplatzer closure was successful in 311 (92.6%; 95% confidence interval, 89%–95%) patients. Superior and posterior rim deficiencies were more common in failed than in successful procedures (superior, 24.0% versus 4.8%; P =0.002; and posterior, 32.0% versus 4.2%; P <0.001). Device migration occurred in 4 adults (2 cases each of surgical and transcatheter retrieval); in the 21 remaining failures, the device was unreleased and withdrawn. After a median follow-up of 10.0 years (2.5–17 years), all patients were alive with no history of late complications. Conclusions— Closure of large atrial septal defects using the Amplatzer device is safe and effective in both adults and children. Superior and posterior rim deficiencies are associated with procedural failure. Closure can be performed under transthoracic echocardiographic guidance in experienced centers. Early device migration is rare and can be safely managed by device extraction. Long-term follow-up showed no deaths or major late complications in our population of 311 patients.

The arterial switch operation (ASO) is the procedure of choice for treatment of all forms of transposition of the great arteries (TGA) including complex forms associated with ventricular septal def...

Background: Congenitally corrected TGA (CC-TGA) is characterized by discordant atrioventricular and ventriculo arterial connections. In absence of right ventricular outflow tract obstruction (RVOTO), repair by atrial. and arterial switches remains a challenging procedure for which tong term follow-up is uncertain. Methods: From 1995 to 2007, 20 patients (median age: 26 months) with CC-TGA had double switch procedure. Segmental anatomy was {SLL} in all patients, dextrocardia in two patients, mesocardia in two patients. Ventricular septal. defect was present in 17 patients, aortic coarctation in 2 patients and interrupted aortic arch (IAoA) in 1 patient. Five patients had tricuspid valve regurgitation. Six patients had AV blocks, 4 patients had pacemaker implantation prior to repair. Pulmonary artery banding was performed in 17 patients, for congestive heart failure (14 patients) or left ventricular retraining (3 patients). Three patients, including one patient with IAoA had primary repair. After W retraining, repair was performed when indexed LV mass to W volume ratio was above 1.5. A median follow-up of 60 months was achieved in all. Results: There were no deaths. Postoperative pacemaker implantation was required in four patients. Reoperation for Senning obstruction was necessary in one patient, and pacemaker battery replacement in another patient. One patient had mild neoaortic insufficiency, two had mild tricuspid regurgitation and two had mild mitral regurgitation. All were in NYHA I-II. Actuarial survival at 10 years was 100% and freedom from reoperation at 5 and 10 years were 93% and 77.4%, respectively. Conclusion: Double switch for CC-TGA without RVOTO can be performed with no mortality and tow morbidity. Since these results seem to last for several years, it should be considered as the optimal procedure. (C) 2009 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Background. Subaortic obstruction is one of the risk factors for anatomic repair of double outlet right ventricles (DORV). A comprehensive approach to such lesions has been developed in our institution since 1981. This retrospective work analyzes the results of this approach. Methods and Results. Between January 1981 and September 1992, 30 patients aged 15 days to 15 years (mean, 44.8 months) underwent repair of a DORV associated with subaortic obstruction. Eighteen patients had a palliative procedure before complete repair. The ventricular septal defect (VSD) was subaortic in 15 patients, doubly committed in 1, noncommitted in 9, and subpulmonary in 5. The subaortic obstruction was a result of restrictive VSD in 29 patients and of double straddling of mitral and tricuspid valves once. The preoperative peak systolic pressure gradient between the left ventricle and the aorta (LV-Ao) was 68.7±23 mm Hg. Reconstruction of the left ventricular outflow tract comprised a ventral enlargement of the VSD in subaortic, doubly committed, and those subpulmonary VSDs scheduled for an arterial switch operation or a conal resection in noncommitted and other subpulmonary forms. Reconstruction of the right ventricular outflow tract included primary closure of the right ventricle in 12 patients, an infundibular patch in 9, a transannular patch in 4, and insertion of a right ventricular pulmonary valved conduit in 5. There were two early (6.6%) and two late (7.1%) deaths. Three patients required reoperation. A mean follow-up of 60.5 ±46.8 months was achieved in all the survivors. They were all in New York Heart Association class I or II, in sinus rhythm. At last follow-up, the mean LV-Ao gradient was 7.5±6.2 mm Hg, and LV function indices were within normal ranges. Actuarial survival and freedom from reoperation rates at 8 years were 86.6% and 87.0%o, respectively. Conclusions. Surgical relief of subaortic obstruction in DORV has to be adapted toVSD location and spatial arrangement of atrioventricular valves and great vessels. (Circulation. 1993;88[part 21:177-182.)

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 Q. 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 Q. 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. Neo-aortic 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.

OBJECTIVES Some features of the left atrioventricular valve (large mural leaflet, dystrophic tissue) represent a challenge for repair of atrioventricular septal defects without postoperative regurgitation. A retrospective study was conducted to evaluate the results of surgically creating a double-orifice left atrioventricular valve in such circumstances. Clinical results were analyzed according to valvular and subvalvular left atrioventricular valve measurements in pathologic specimens with atrioventricular septal defects. METHODS Among 157 patients operated on for atrioventricular septal defect since October 1989, 10 patients underwent primary repair (n = 8) or reoperation (n = 2) by this procedure. Median age at repair was 3.3 years (0.1-33 years). Anatomic types were complete (n = 3), intermediate (n = 5), and partial (n = 2). Preoperative moderate to severe left atrioventricular valve regurgitation was present in 6 patients. After the repair (two-patch technique in complete atrioventricular septal defect, cleft closed in each case), these 10 patients were found to have moderate to severe residual regurgitation not amenable to repair by annuloplasty. The top edge of the mural leaflet was anchored to the facing free edge of the cleft. RESULTS No hospital death or morbidity was observed. Left atrioventricular valve regurgitation was absent or trivial (8 patients) and mild (2 patients). Color-coded echocardiography did not show significant left atrioventricular valve stenosis. The mean diastolic pressure gradient across the left atrioventricular valve was 3.2 +/- 1.1 mm Hg (1.4-4.5 mm Hg). At a median follow-up of 72 months (6-91 months), there was 1 late death, unrelated to left atrioventricular valve malfunction, due to pulmonary vascular obstructive disease. Left atrioventricular valve regurgitation did not increase over time, except in 1 patient in whom regurgitation recently progressed from mild to moderate. At rest, the mean diastolic pressure gradient across the left atrioventricular valve was 3.8 +/- 2.9 mm Hg (1.5-11.2 mm Hg). One child had an early moderate stenosis without pulmonary hypertension. Studies on pathologic specimens (n = 34) indicated that long chordal lengths and large mural leaflet size are essential independent anatomic features to assess its feasibility. CONCLUSIONS Surgical creation of a double-orifice left atrioventricular valve is an effective additional procedure for repair of atypical cases of atrioventricular septal defect. The operation may decrease the need for reoperation or left atrioventricular valve replacement.

Background-Congenital mitral stenosis (CMS) remains a surgical challenge, particularly when it is associated with other heart defects. As in other groups of heart defects, there is a trend toward early single-stage complete repair, but the optimal surgical approach remains unanswered.Methods and Results-This study was designed to analyze the evolution of surgical strategies in patients with CMS and associated defects through single-stage and staged repair. Between 1980 and 1999, 72 children were operated on for congenital heart defects, including CMS. Preoperative transmitral gradient was 12.6+/-7 mm Hg. Preoperatively, all the patients were NYHA class In to IV. Thirteen patients had an isolated CMS; in 59, it was associated with other heart defects, mainly ventricular septal defect (n=28) or multilevel left ventricular obstruction (n=41). In this group of patients, 33 had a staged approach, and 16 had a single-stage approach. Early mortality was 12.5% (9 patients). There were no deaths in the isolated CMS and single-stage repair groups. Logistic regression revealed that early mortality was influenced by association with left ventricular outflow tract obstruction (P<0.001) and by use of a staged approach (P<0.01). There was no late mortality in isolated CMS; there were 2 late deaths in the group of single-stage repair and 6 late deaths in the staged approach group (P<0.01). Reoperation was required in 24 patients, mainly for residual mitral valve dysfunction or residual left ventricular outflow tract obstruction. Including the reoperations, 10 patients received a prosthetic mitral valve. At 15 years after surgery, survival was 69.6+/-7.5%, freedom from reoperation was 70.8+/-6.3%, and freedom from mitral valve replacement was 69+/-6%.Conclusions-Surgery for isolated CMS gives excellent early and long-term results. In patients with associated heart defects, a single-stage operation seems superior to a staged approach. Mitral valve replacement in this category of patients should be reserved as a salvage procedure.

BACKGROUND:The occurrence of a progressive pulmonary venous obstruction after the repair of the total anomalous pulmonary venous connection is a severe complication. OBJECTIVES:The objectives of this study were to retrospectively review the patients with this condition and to report our experience with a new surgical technique with a sutureless in situ pericardium repair. METHODS:Of 178 patients who underwent correction of total anomalous pulmonary venous connection, 16 patients (9%) experienced the development of a progressive pulmonary venous obstruction in a median interval of 4 months (5 weeks-12 years). Three patients had isolated anastomotic stenosis, 4 patients had isolated pulmonary venous ostial stenosis, and 9 patients had both. Pulmonary venous obstruction was bilateral in 7 patients. The surgical procedures used at reoperation included 8 patch enlargements, 5 ostial endarterectomies, 1 intraoperative stenting, and 7 sutureless in situ pericardium repairs. RESULTS:There were 4 deaths after reoperation (4 of 15 patients; 27%). The only significant mortality risk factor was the bilateral location of the pulmonary venous obstruction (P =.045). In patients with isolated anastomotic stenosis or with only 1 pulmonary venous ostial stenosis (n = 5), there was no death, except the patient presenting with a single ventricle. In patients with 2 or more pulmonary venous ostial stenoses (n = 10), there were 3 deaths; 5 of the 7 survivors were successfully treated with the in situ pericardial technique, with normalized pulmonary artery pressure at a mean follow-up of 26 months. CONCLUSION:Progressive pulmonary venous stenosis after repair of total anomalous pulmonary venous connection remains a severe complication when bilateral. The sutureless in situ pericardial repair offers a satisfactory solution, particularly on the right side.

Background/Aims: Early liver transplantation is crucial in children with liver disease and pulmonary artery hypertension, Some severe pulmonary vascular anomalies associated with portal hypertension disappear after isolated liver transplantation. Evolution of pulmonary artery hypertension due to plexogenic arteriopathy is controversial, as this association is still considered a contraindication to isolated liver transplantation, Outcome of pulmonary hypertension after isolated liver transplantation is reported in three patients with portal hypertension.Methods: After echocardiographic diagnosis, the patients had a complete hemodynamic exploration, and two had a lung biopsy, After liver transplantation, the survivors had echocardiographic follow up and a second hemodynamic exploration.Results: In two children, pulmonary pressures and resistances returned to near-normal values 1 and 6 years after successful isolated liver transplantation. The third patient, with the most severe arteriopathy, had to wait 1 year for a donor, and the attempted transplantation was complicated by ventricular tachycardia; death occurred 2 days after surgery.Conclusions: Liver transplantation can reverse pulmonary artery hypertension due to high pulmonary resistances complicating liver disease with portal hypertension, provided it is carried out at an early stage, Early detection of pulmonary hypertension by systematic echocardiography may thus be crucial in these children with portal hypertension.

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.

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.

BACKGROUND:The purpose of the present study was to define the optimal management and to identify the risk factors for death and repeat operation in patients with double-outlet right ventricle.METHODS AND RESULTS:From 1985 through 1996, 154 consecutive patients underwent biventricular repair for double-outlet right ventricle. The presence of bilateral infundibular structures was the major inclusion criteria (142 patients). According to the relationship of the ventricular septal defect (VSD) to the great arteries, there were 86 patients with a subaortic VSD (56%), 45 patients with a subpulmonary VSD (29%), 18 patients with a noncommitted VSD (12%), and 5 patients with a doubly committed VSD (3%). Sixty-five patients (42%) had undergone previous palliative procedures. At repair, the median age was 10 months, and the median weight was 6.5 kg. Two main types of repair were used: intraventricular baffle repair (n = 115) and arterial switch operation with VSD-to-pulmonary artery baffle (n = 39). There were 14 hospital deaths (9%; 70% confidence limit [CL], 7% to 12%). The only significant risk factor for early death was the presence of congenital mitral valve anomalies (P = 0.02). Twenty-eight patients (18%) required 39 repeat operations. The repeat operation rate was higher in patients with associated VSD enlargement at baffle construction (n = 29; 19%) (P = 0.01). There were 6 late deaths (4%; 70% CL, 2% to 7%). Patients presenting with pulmonary stenosis constituted a low-risk group for global death (P = 0.008). The median follow-up was 52 months. Ten-year actuarial survival and survival with freedom from repeat operation rates were 86% and 62% (70% CL, 83% to 89% and 54% to 70%), respectively.CONCLUSIONS:Long-term survival with good quality of life can be achieved after either 1- or 2-stage repair of this complex anomaly.

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.

Objective: Atrio ventricular septal defects (AVSD) with normal caryotype represent in average 25% of AVSD. They constitute a more complex group of patients characterized by frequent left sided heart obstructive lesions, raising the problem of the appropriate indications between biventricular and univentricular procedures. Methods: Sixty-nine consecutive patients, who had AVSD with normal caryotype underwent surgery. According to the anatomical complexity there were 22 intermediate AVSD, 36 complete AVSD and II complex AVSD. Associated lesions were present in 68% of the patients including left sided heart anomalies in 57%. According to the size of the left ventricle (LV) evaluated on the LV/RV end diastolic diameter ratio calculated at 2D echocardiogram: right ventricular (RV) dominance was found in 29%; with border line LV in 13 patients and truly hypoplastic LV in 7 patients. Biventricular repair was always favored in case of border line LV and precluded when the LV/RV ratio was less than 0.33. Results: There were 57 biventricular repairs with 10 years actuarial survival of 70 +/- 8% and respectively, 100% in the complex AVSD, 86% in the intermediate AVSD and 51% in the complete AVSD (P = 0.006). The risk factors for biventricular repair were the association to a subaortic stenosis (P = 0.01) and the severity of the mitral valve lesions (P = 0.03) that led to 38% reoperation. There were 12 univentricular palliation with 10 years survival of 66 +/- 14%. The risk factor for univentricular palliation was the association to a severe pre-operative mitral regurgitation (P = 0.005). Conclusions: biventricular repair should be precluded in patients presenting with subaortic stenosis. Severe mitral valve anomalies lead to elevated mortality and morbidity with frequent reoperations. Univentricular repair might have larger indications and cardiac transplantation might be considered in patients with truly hypoplastic LV presenting with severe pre-operative AV valve regurgitation. (C) 1997 Elsevier Science B.V.

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.