[Large atrial septal defects in adults: results of attempted systematic percutaneous closure]. / Communications interauriculaires larges de l'adulte: résultats d'une tentative systématique de fermeture percutanée.

Percutaneous closure of ostium secundum atrial septal defects became routine practice from 1998 but remained limited by the size of their diameter at balloon calibration. In March 2004, after an experience of 551 patients, the maximum admissible diameter (40 mm), the biggest size available of the Amplatzer prosthesis, could often be exceeded. The essential anatomical condition was the presence of a circumferential edge--even minimal--except adjacent to the aorta where its absence was not a problem. From 31/03/04 to 31/12/05, 17 patients, 17 to 58 years of age, were included and underwent attempted percutaneous closure by the usual protocol. The insertion of the prosthesis was difficult in every case. It required the use of several techniques to orientate the prosthesis in a satisfactory plane without risking tearing part of the residual atrial septum. The attempt was successful in 16 of the 17 patients. The one failure occurred in a patient with complete absence of a posterior edge. The postoperative course was uncomplicated without residual shunt or displacement of the occluder in the follow-up studies. The authors conclude that very big atrial septal defects with diameters of over 40 mm may be closed percutaneously with the Amplatzer device with a reasonable chance of success. These attempts do not expose the patient to any particular risk and the results, in successful attempts, are comparable to those observed in smaller atrial septal defects.

Coronary events after arterial switch operation for transposition of the great arteries.

BACKGROUND: Transfer of the coronary arteries is a crucial step during the arterial switch operation (ASO) for transposition of the great arteries. This retrospective study aims to assess the incidence and risk factors of coronary events after ASO and sensitivity of noninvasive tests in the diagnosis of the coronary obstruction. METHODS AND RESULTS: Between 1982 and 2001, 1304 newborn and infants had an ASO and the 1 198 hospital survivors had a 59-month mean follow-up. Coronary events occurred in 94 patients (7.2%; 95% CI, 6 to 9). Survival without coronary events were 92.7, 91, and 88.2% at 1, 10, and 15 years, respectively. The incidence was bimodal: high early and slow later. Multivariate analysis showed correlation with type B or C coronary pattern and major operative events (P<0.0001 and P=0.0024). In a subset of 324 patients who underwent a coronary artery angiography, lesions were observed in 22 patients (6.8%; 95% CI, 5 to 10). Multivariate analysis showed correlation with only type B or C coronary pattern (OR=20.8, P=0.0002). All of these patients had electrocardiogram and echocardiogram, 174 patients also had a treadmill test, and 115 patients had a myocardial scintigraphy. The association of these tests had the highest diagnosis sensitivity, 75%. CONCLUSIONS: After ASO, coronary events are not rare, occurring most often early and are an important cause of death. Coronary repair can be needed lately. Noninvasive tests are not sensitive enough to detect significant delayed coronary artery stenosis and coronary artery angiography should be performed.

[Prevalence and diagnosis of coronary lesions after arterial switch]. / Prévalence et dépistage des lésions coronaires après switch artériel.

In anatomical repair procedure of transposition of the great arteries (arterial switch), translocation of the coronary arteries is crucial and coronary complications remain the principal cause of death. The aim of this retrospective study was to assess the prevalence of coronary lesions and to evaluate the diagnostic methods to prevent their consequences. From 1982 to 2001, 1,304 patients were operated for transposition of the great arteries by the switch procedure at the Marie Lannelongue Surgical Centre. The average follow-up of the survivors was 59 months (3 days to 17 years) during which 324 patients underwent coronary angiography. All had an ECG and an echocardiogram (N = 324); 174 underwent exercise stress testing and 115 had myocardial scintigraphy. Of the 324 patients who underwent coronary angiography, 22 had coronary lesions (6.8%; 95% CI 5-10). In multivariate analysis a type II coronary network by the Marie Lannelongue Classification was related to the risk of coronary lesions (OR = 0.28; p < 0.0002). Each non-invasive method studied separately had a low sensitivity (< 50%) for the detection of these lesions. The association of ECG, echocardiography and myocardial scintigraphy had the best sensitivity at 75%. Therefore, after the arterial switch procedure non-invasive investigations are not sensitive enough to diagnose coronary lesions and systematic coronary angiography and aortography should be performed in all patients.

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.

Long-term results after valvotomy for congenital aortic valvar stenosis in children.

As interest increase in the Ross procedure performed as a therapeutic option for children with congenital aortic valvar stenosis, it becomes increasinly important to know the late results of aortic valvotomy in this population. We have therefore examined retrospectively the medical records of 121 consecutives survivors undergoing aortic valvotomy before 10 years of age between 1974 and 1992. The mean age at the first valvotomy was 29 months, with a range from 3 days to 10 years. The mean duration of follow up was 9.4 years, with a range from 1.6 to 22 years. Fifteen patients (12.3%; 70% CL: 10-16) died: 9 following reoperation, and 6 late after surgery. Death was related to the hearts in 86% of cases. The actuarial survival rate was 79% (70% CL: 72/84) at 10 years. Young age at the first valvotomy, and the number of procedures, emerged as risk factors of secondary mortality. Reoperations on the aortic valve, 73 in all, were required in 56 patients. The second procedure was done after a mean interval of 6 years, with a range from 1 day to 18 years. This was for restenosis in three-quarters of the cases. The aortic valve was replaced in 30 patients, at a mean of 9 years, and with a range from 9 months to 18 years, after the first procedure. The survival without replacement at 20 years was 29% (70% CL: 15-49). No factor was identified with a relationship either to reoperation or valvar replacement. Long term results after aortic valvotomy, therefore, show a high late mortality, frequent reinterventions, and an almost inescapable eventual need for valvar replacement. The ongoing use of the Ross operation is justified, even if longterm studies in children are still needed to validate its use.

[Hemolysis, a rare complication after percutaneous closure of an atrial septal defect]. / L'hémolyse, une complication exceptionnelle après fermeture percutanée de communication interauriculaire.

Haemolysis after closure of an atrial septal defect with an Amplatzer device is a rare complication. The suggested mechanism is the association of a residual shunt with flow of tricuspid regurgitation, impinging on the inferior edge of the prosthesis, factors which should be taken into consideration during the implantation of this type of device.

[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.

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.

[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.

[Late complications of percutaneous closure of atrial septal defects with the Sideris occluder]. / Complications tardives après fermeture percutanée des communications interauriculaires par prothèse boutonnée de Sideris.

Between June 1992 and January 1996, 27 patients aged 3.9 to 74 years with an ostium secundum (22 patients) or patent foramen ovale with right-to-left shunts (5 patients) underwent percutaneous closure of their atrial septal defects with the Sideris occluder. After a thromboembolic complication, transesophageal echocardiography was performed routinely after the procedure in 15 patients between 1 month and 2 years, and in 6 patients on the 15th day. Two patients died, on the 2nd day and 21st month, of non-related causes. After an average follow-up of 33 months, 59% of patients had complete occlusion of the atrial septal defects or only a minimal residual shunt. Displacement of the prosthesis was defects or only a minimal residual shunt. Displacement of the prosthesis was observed in 7 cases with no relationship to size: 4 parallel to the septum with reappearance or increase in shunt, 3 with tilting of the prosthesis. All of these patients had a large residual defect compared with 20% with a normally positioned prosthesis (p < 0.05). Tilting of the occluder was associated with left atrial thrombosis (present in 40% of these patients), complicated by systemic embolism in one case: there were no cases of left atrial thrombus in the 9 with complete occlusion and the 5 patients with an isolated residual defect (p < 0.05). Occlusion of atrial septal defect with the Sideris device is effective and a safe method in the majority of cases. However, a badly positioned prosthesis with a residual shunt should be extracted as seen as possible or within three weeks if displacement is observed at control echocardiography.

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.

Absent pulmonary valve syndrome: surgical treatment and considerations.

BACKGROUND: The operative management of absent pulmonary valve syndrome remains controversial regarding the need for pulmonary valve implantation and remodeling of pulmonary arteries. Moreover, symptomatic infants are considered to have a poor prognosis. This retrospective report summarizes the experience of a single institution. METHODS: Between May 1977 and May 1995, 37 consecutive patients underwent repair of absent pulmonary valve syndrome. Patients were divided into two groups according to age at operation: group A (10 infants less than 1 year old) and group B (27 patients older than 1 year). Mean age at operation was 5 +/- 4 months in group A and 72 +/- 42 months in group B. Initially, repair consisted of ventricular septal defect closure and relief of right ventricular outflow tract obstruction combined with pulmonary valve implantation. More recently, the concept of treatment has evolved with pulmonary arterioplasty without pulmonary valve insertion, except in patients with elevated pulmonary artery pressure. RESULTS: Of the 37 patients, 34 had successful repair. The overall in-hospital mortality rate was 8% (two deaths in group A and one in group B). No hemodynamic data were correlated with operative death. Death was associated with longer extracorporeal circulation time (p = 0.005) and longer aortic cross-clamping time (p = 0.019). In fact, these were clearly related to more complex anatomy (p = 0.001): multiple ventricular septal defects in 1, left pulmonary artery arising from the ductus in another, and left pulmonary artery arising from the aorta in the remainder. Follow-up was available in 22 of the 34 survivors. Mean follow-up time was 30 +/- 47 months in group A and 38 +/- 33 months in group B. All but 1 had no restriction of exercise, and most of them had pulmonary incompetence of Doppler echocardiography. One developed severe exercise intolerance because of pulmonary valve stenosis (xenograft), leading to uneventful reoperation 123 months after initial repair. One infant died suddenly of complete atrioventricular block 3 months after repair. The late mortality rate was 5%. CONCLUSIONS: Surgical treatment of absent pulmonary valve syndrome should include pulmonary arterioplasty to reduce bronchial obstruction, with no need for pulmonary valve insertion. This procedure is feasible and is recommended especially in markedly symptomatic infants.

[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)

[Taussig-Bing malformation with straddling of both atrioventricular valves. Echocardiographic description of a case]. / Malformation de Taussig-Bing avec straddling des 2 valves auriculo-ventriculaires. Description échocardiographique à propos d'un cas.

The authors report the case of an infant with the Taussig-Bing malformation and straddling of the two atrioventricular valves, the diagnosis of which was made by echocardiography and confirmed at surgery. Anatomic repair of the transposed great arteries was preceded by reconstruction of the interventricular septum which left the abnormal tricuspid subvalvular apparatus in the right ventricle and the abnormal mitral subvalvular apparatus in the left ventricle. Postoperative Doppler echocardiography confirmed the absence of left ventricular outflow obstruction.

[Endoluminal mitral valvuloplasty in young adults and children. Apropos of 10 cases]. / Valvuloplastie mitrale endoluminale chez l'adulte jeune et l'enfant. A propos de 10 cas.

Between April 1 and May 15, 1986, transluminal dilatation of the mitral valve was performed at the Marie-Lannelongue Hospital, near Paris, in 10 patients aged from 12 to 48 years (mean 25,3 years) suffering from rheumatic mitral stenosis with supple valve leaflets. The procedure, carried out under local anaesthesia, included trans-septal catheterization followed by installation of one, then usually two balloons opposite the mitral orifice. The total diameter of the balloons was often greater than that of the mitral annulus. No haemorrhage, embolism or arrhythmia was observed. A significant left-to-right shunt, evaluated by radioisotope exploration with technetium pyrophosphate performed on the 8th day, was present in only one patient. The LV-LA end-diastolic gradient fell from 14.4 +/- 9.8 to 4.6 +/- 3.4 mmHg (p less than 0.01). The mean pulmonary arterial pressure was reduced less significantly from 39.9 +/- 18.4 to 27.2 +/- 11.3 mmHg (p less than 0.02). The cardiac output was only moderately increased from 2.52 +/- 0.48 to 2.88 +/- 0.66 l/min/m2. The mitral valve area, measured from the smaller axis on 2-dimensional echocardiography, more than doubled, reaching a mean value of 1.99 +/- 0.56 cm2. This figure was confirmed by the reduction, at Doppler velocimetry, of the gradient half-regression time from 267 +/- 62 to 118 +/- 46 ms (p less than 0.01). Similarly, the end-systolic gradient evaluated by Doppler, fell from 17.5 +/- 8.9 to 5.3 +/- 4.7 mmHg (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

[Rheumatic mitral stenosis. The medical solution]. / Rétrécissement mitral rhumatismal. La solution médicale.

The first case of balloon catheter valvular dilatation for rheumatic mitral stenosis is reported in a 13 year-old child. The technique, under local anesthesia, required a transseptal access, then the introduction of one, then simultaneously 2 balloon catheters through the mitral valve. A perfect opening of the valve, without mitral insufficiency was obtained. This technique, even if difficult, seems to be adaptable to most children with rheumatic mitral stenosis.