[Endovascular treatment of coronary arterial fistulae in children and adults]. / Traitement endovasculaire des fistules artérielles coronaires de l'enfant et de l'adulte: à propos de 25 cas.

The authors report their experience of endovascular treatment of coronary fistulae in 25 patients aged 2 to 77 years (median 29 years) who underwent 30 interventional catheterisation procedures. The origin of the fistulae was variable: left coronary (14 cases), right coronary (6 cases) and bilateral (5 cases) as were the sites of drainage: right atrium (5 cases), right ventricle (8 cases) pulmonary artery (7 cases) and bronchial artery (5 cases). Different materials were used: releasable balloons (9 cases), coils (17 cases), microparticles (5 cases) and Amplatzer occluder and plug (2 cases). The result, judged by occlusion or sub-occlusion of the given pedicle, was a success in 92% of cases. Total or sub-total occlusion of the fistula was obtained in 22 patients (88%); 2 patients had residual fistulae due to non-embolisation of the pedicle; in one patient, it was not possible to embolise the pedicle despite two attempts. A single serious complication, ventricular fibrillation during the catheterisation, was observed but without a sequel. Follow-up ranged from 6 to 176 months; 4 patients died of other causes, 3 were lost to follow-up; one coronaro-pulmonary fistula recurred but no other recurrence was observed in the other patients. The authors conclude that embolisation is the treatment of choice for coronary fistulae providing the cases are managed by teams trained in the use of different materials. The choice of material of embolisation should be adapted to the anatomical conditions which determine the success rate and the absence of complications.

[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.

[Cardiac failure in children]. / Insuffisance cardiaque de l'enfant. Nouvelles approches physiopathologiques.

The understanding of cardiac failure pathophysiology in children has advanced greatly in recent years. Whilst cardiac failure secondary to left ventricular dysfunction with viral or metabolic cardiomyopathy is comparable in pathophysiological terms with adults, other aetiological mechanisms are also implicated, linked both to anatomical anomalies in congenital cardiopathy and their surgical repair. The role of the neuroendocrine system with adrenergic activation of the renin-angiotensin system, secretion of natriuretic peptides and the inflammatory system, before and during surgical repair procedures, have been proven both experimentally and clinically. Understanding these pathophysiological mechanisms opens new therapeutic perspectives for improving the management of children with congenital cardiopathy in cardiac failure and reducing postoperative mortality and morbidity.

[Congenital cardiopathy: indications for anticoagulant treatment]. / Cardiopathies congénitales: indication d'un traitement anticoagulant.

Congenital cardiopathy is common in children with thromboembolic disease. There are no objective clinical data for medium and long term treatment. Vitamin K antagonists could be envisaged for this. Children with a mechanical valve should be anticoagulated. This has been validated in adults, there has been no study in children which contradicts this, and the serious nature of thromboembolic complications in this group is significant. In atrial fibrillation, the most frequent indication for oral anticoagulants is after a Fontan procedure, because of the thromboembolic risk factors. Anticoagulation following Fontan or similar procedures relies on the thrombotic factors and their temporal distribution, being more pronounced in the first postoperative year; anticoagulants should be given over this period. In Eisenmenger's syndrome haemorrhage is common: these patients are not anticoagulated unless there is an indication such as recent pulmonary embolus. During interventional catheterisation thromboembolic accidents are rare, occurring early, and more commonly with certain prostheses. In adults with an intra-atrial prosthesis oral anticoagulants are used for three months, and then replaced by antiplatelet drugs. In congenital cardiopathy the stent is often pulmonary or aortic, and the incidence of thromboembolic accident is therefore very low. For a venous channel or in the context of a Fontan procedure, oral anticoagulants are justified for between three and six months. It is to be hoped that prospective studies, of which some are in progress, will provide further guidance for these indications.

Transcatheter closure of patent foramen ovale in patients with platypnea-orthodeoxia: results of a multicentric French registry.

BACKGROUND: Dyspnea and the decrease in arterial saturation in the upright position in elderly subjects is described as platypnea-orthodeoxia syndrome (POS). POS is secondary to the occurrence of an atrial right-to-left shunt through a patent foramen ovale (PFO). METHODS: This French multicentric study reports on 78 patients (mean age 67 +/- 11.3 years) with POS who had transcatheter closure of the PFO; frequently associated diseases were pneumonectomy (n = 36) and an ascending aortic aneurysm (n = 11). In all patients, the diagnosis was confirmed by transthoracic or/and transesophageal echocardiography. Five different closure devices were used: Amplatz (n = 45), Cardioseal (n = 13), Sideris (n = 11), Das Angel Wings (n = 8) and Starflex (n = 1). Closure was successful in 76 patients (97%). RESULTS: Oxygen saturation increased immediately after occlusion from 84.6 +/- 10.7% to 95.1 +/- 6.4% (p < 0.001) and dyspnea improved from grade 2.7 +/- 0.7 to grade 1 +/- 1 (p < 0.001). A small residual shunt was immediately observed in 5 patients (3 with the Cardioseal device, 1 with the Sideris and 1 with the Amplatz) leading to the implantation of a second device in one case (Cardioseal). Two early deaths occurred unrelated to the procedure (one due to sepsis probably related to pneumonectomy, another due to respiratory insufficiency). Other complications were: a small shunt between the aorta and the left atrium, two atrial fibrillations and a left-sided thrombus which disappeared with anticoagulant therapy. At a mean follow-up of 15 +/- 12 months, there were 7 late deaths related to the underlying disease. CONCLUSION: Percutaneous occlusion of the foramen ovale is safe and gives excellent results thanks to continuing improvement in available devices. This technique enables some patients in an unstable condition to avoid a surgical closure.

Is surgical intervention still indicated in recurrent aortic arch obstruction?

BACKGROUND: Introduction of balloon dilatation has become the standard treatment for recurrent aortic arch obstruction and has changed the therapeutic approach to patients with this disorder. OBJECTIVES: Whether all patients with recurrent aortic arch obstruction are candidates for balloon dilatation remains unanswered. In addition, only few reports have tried to compare the results between patients undergoing balloon dilatation or redo operations. METHODS: Since 1983, 97 patients underwent reintervention for recurrent aortic arch obstruction (42 dilations and 55 reoperations). Eight had immediate unsuccessful dilatation and were shifted to the surgical group (n = 63). The median age at reintervention was 21.7 months (10 days-45 years), and the median delay was 13.6 months (7 days-17 years). Anatomy of the aortic arch oriented the surgical approach to treat arch hypoplasia. It could be performed through a left thoracotomy in 52 patients, with extended end-to-end anastomosis in 34 patients, subclavian flap repair in 9 patients, conduit insertion in 6 patients, and patch enlargement in 3 patients. More recently, an anterior approach with cardiopulmonary bypass without circulatory arrest was applied to enlarge the patch in all the aortic arches. RESULTS: There was one early death in the surgical intervention group and 2 late deaths in the dilation group. Major complications and recurrence were higher in the dilated group (4 vs 0, P <.01, and 14 vs 5, P <.0004, respectively). At a mean follow-up of 11.8 +/- 4.1 years in the surgical intervention group and 7.5 +/- 2.5 years in the dilated group, systemic hypertension was normalized in all but 5 patients in the surgical intervention group and 6 patients in the dilated group. CONCLUSION: Reoperation for recurrent aortic arch obstruction can be performed safely, with low rates of mortality and morbidity. This approach should be considered versus balloon angioplasty, especially in patients older than 4 years and in the presence of aortic arch hypoplasia.

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.

[Acenocoumarol (Sintrom) and fluinidione (Previscan) in pediatrics after cardiac surgical procedures]. / Acénocoumarol (Sintrom) et fluindione (Previscan) en pédiatrie après intervention de chirurgie cardiaque.

PATIENTS AND METHODS: Between 1997 and 2001, 150 children (one month to 16 years of age) were treated with oral anticoagulants after cardiac surgery (Fontan's operations and congenital heart diseases without valvulopathy: 62%, valvular prosthesis: 20%, arrhythmia: 4.6%, thrombosis: 4%, other: 9.4%). They were first treated by either unfractionated heparin (49%) or nadroparin (51%), then by acenocoumarol (n1 = 114) or fluindione (n2 = 36) until steady state. RESULTS: The retrospective analysis of data (age, body weight, international normalized ratio, loading and maintenance doses, time to achieve the steady state) led to the building of a dosage nomogram usable in pediatrics. CONCLUSION: We demonstrated that the mean maintenance dose depended on age and weight. After three years, that dose (mg/kg) was getting close to adult values; it was higher before three years of age, especially before 12 months (p < 0.01), and very variable from a child to another. The recommended loading dose should be as close as possible to the effective maintenance dose: within that cohort, about 0.14 and 0.05 (acenocoumarol) or 1.1 and 0.40 mg kg-1 day-1 (fluindione), before 12 months and after three years respectively.

[Three-dimensional multislice CT scanner: value in patients with pulmonary atresia with septal defect]. / Scanner multidétecteur tridimensionnel: intérêt chez les patients atteints d'atrésie pulmonaire à septum ouvert.

The aim of this study was to evaluate the interest of multislice CT scanning using three-dimensional reconstructions for pre- or postoperative management of patients with pulmonary atresia with ventricular septal defect. Twenty-one consecutive patients (median age: 8 months, median weight: 5.6 kg) with pulmonary atresia with ventricular septal defect were explored by multislice CT. Standard protocol consisted in one helical thoracic acquisition, associated with an injection of 2 to 3 mL/kg of iodinated contrast medium at a flow rate from 0.5 to 1 mL/s. Three-dimensionnal reconstructions were systematically performed. Before operation, status and size of central pulmonary arteries, major aorto-pulmonary collateral arteries, and associated anomalies were analyzed. In 13 operated patients, the permeability of palliative anastomosis and postoperative anatomical changes were studied. The status of central pulmonary arteries was assessed in all cases and the sizes found were in agreement to surgical findings. Main aorto-pulmonary collateral arteries were depicted and their relative position to central airways was assessed. Various associated anomalies were found: right aortic arch, anomalous coronary artery, abnormal pulmonary venous return, left superior vena cava. The anastomosis and the surgical connections (unifocalization, complete repair) were clearly analyzed. The multislice CT with three-dimensional reconstructions is a complementary imaging technique, reliable, useful to pre-operative management and postoperative follow-up of patients with pulmonary atresia with ventricular septal defect.

[Paroxysmal atrial fibrillation in community medicine: management intentions and cost estimates]. / La fibrillation auriculaire paroxystique en médecine de ville: intentions de prise en charge et estimation du coût.

This study was undertaken to describe the declared management of atrial fibrillation in community medicine in France for patients between 50 and 80 years of age, and to estimate the total cost of treatment for Society in the year 2000. A questionnaire was sent to a sample of 306 private sector cardiologists, representative for their geographic location: 100 replies were received. Only 10% of cardiologists prescribed a treatment in patients without cardiovascular precedents who had a single regressive episode of atrial fibrillation and three quarters of them advised follow-up. This management cost an average between 228 and 296 Euros/year. When the episode of fibrillation was persistent at the time of consultation, 82% of practitioners prescribed antiarrhythmic reduction (amiodarone) after antithrombotic treatment (oral anticoagulants) followed by an antiarrhythmic therapy (class lc mainly) with an average cost of 659 to 783 Euros/year. In patients with previous cardiovascular disease, when a first episode of atrial fibrillation was present at the time of consultation, 92% of cardiologists prescribed pharmacological reduction with amiodarone, followed by long-term therapy when successful for an average cost of 755 to 1,092 Euros/year. All cardiologists requested systematic blood tests to search for thyroid complications of amiodarone with an average cost of 59 Euros/year. The costs were high, especially in chronic and recurrent pathology: the cost of treatment of the first two episodes of atrial fibrillation in the over 65 age group was 305 million Euros/year.

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.

Percutaneous closure with Amplatzer device is a safe and efficient alternative to surgery in adults with large atrial septal defects.

BACKGROUND: In adults with atrial septal defect (ASD) and large right-to-left shunt, closure of the defect is recommended. Percutaneous closure is still rarely used in this population. This study presents the results of transcatheter closure with the Amplatzer occluder in such patients. METHODS AND RESULTS: We studied 44 consecutive adult patients with a secundum ASD and 2 of the 3 following criteria: QP/QS >/=2 by oximetry, echocardiographic right ventricle overload, and ASD size >20 mm. Forty-two patients had a successful implantation. In 1 patient an unstable device was withdrawn; in another one, the device embolized in the pulmonary artery. At 6-month median follow-up, 95% had a complete closure; 2 patients with an additional defect had a small residual shunt. Major complications were the aforementioned embolization and a cerebrovascular accident in a patient with atrial fibrillation treated with aspirin. Others were minor and transitory: premature atrial beats in 3 patients, and paroxysmal atrial fibrillation and pulmonary edema in 1 patient each. CONCLUSIONS: Transcatheter closure of large ASDs with the Amplatzer device is efficient with less morbidity than surgical closure.

Surgically created double-orifice left atrioventricular valve: a valve-sparing repair in selected atrioventricular septal defects.

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.

[Aerosolized iloprost therapy in an infant with chronic pulmonary hypertension after a neonatal arterial switch operation]. / Traitement par iloprost en aérosol d'un nourrisson atteint d'hypertension artérielle pulmonaire chronique après cure chirurgicale néonatale d'une transposition des gros vaisseaux.

UNLABELLED: The treatment of chronic pulmonary hypertension with prostacyclin in children is prone to severe complications due to mandatory long-term venous therapy. Inhaled iloprost has been evaluated in adult patients with good preliminary results. CASE REPORT: We report our experience of the use of aerosolized iloprost in an infant treated for pulmonary hypertension associated to a right ventricular failure, which occurred after a neonatal arterial switch operation for transposition of the great arteries. For nine months, hemodynamic and functional status improved and the quality of life was satisfactory at home. CONCLUSION: If further experiences and studies support this observation, aerosolized iloprost could be an alternative to prostacyclin venous therapy in treating children with chronic pulmonary hypertension.

[Secondary displacement of an Amplatz occluder for atrial septal defect. 2 case reports]. / Déplacement secondaire d'une prothèse d'Amplatz de communication interauriculaire. A propos de 2 cas.

The authors report two cases of secondary displacement of an Amplatz occluder of isolated ostium secundum atrial septal defects. The displacement was observed at 1 month and the prosthesis removed at 3 months. To the best of the authors' knowledge, these are the first two reported cases of secondary displacement of this type of device. The main risk factor in the two cases was the absence of a subaortic septal remnant observed at surgical removal. This anatomical characteristic can be identified by transoesophageal echocardiography, but was poorly assessed by transthoracic echocardiography. Three-dimensional transoesophageal echocardiography should improve selection of atrial septal defects suitable for percutaneous treatment by better identification of their form and anatomical characteristics.

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.

Changes in venous return parameters associated with univentricular Fontan circulations.

To clarify the physiology of venous return (Q(vr)) in Fontan circulations, venous return conductance (G(vr)) and mean circulatory filling pressure (P(mcf)) were determined in pentobarbital sodium-anesthetized pigs. Relationships between Q(vr) and right (biventricular, n = 8) or left (Fontan, n = 8) filling pressures are described by straight lines with significant correlation coefficients. Estimated P(mcf) values were correlated with observed P(mcf) values in either circulations (P