Quality of Life of Children with Congenital Heart Diseases: A Multicenter Controlled Cross-Sectional Study.

To assess the health-related quality of life (QoL) in children with congenital heart diseases (CHD) with a validated questionnaire in comparison with control children. We prospectively recruited 282 children with CHD aged from 8 to 18 years in two tertiary care centers (France and Belgium) and 180 same-age controls in randomly selected French schools. Children's QoL was self-reported with the KIDSCREEN-52 questionnaire and reported by parents with the KIDSCREEN-27. QoL scores of each dimension were compared between CHD and controls and between the classes of disease severity. Both centers were comparable for most demographic and clinical data. Age- and gender-adjusted self-reported QoL scores were lower in CHD children than in controls for physical well-being (mean ± SEM 45.97 ± 0.57 vs 50.16 ± 0.71, p < 0.0001), financial resources (45.72 ± 0.70 vs 48.85 ± 0.87, p = 0.01), peers/social support (48.01 ± 0.72 vs 51.02 ± 0.88, p = 0.01), and autonomy in the multivariate analysis (47.63 ± 0.69 vs 49.28 ± 0.85, p = 0.04). Parents-reported scores were lower in CHD children for physical (p < 0.0001), psychological well-being (p = 0.04), peers/social support (p < 0.0001), and school environment (p < 0.0001) dimensions. Similarly, the disease severity had an impact on physical well-being (p < 0.001), financial resources (p = 0.05), and peers/social support (p = 0.01) for self-reported dimensions, and on physical well-being (p < 0.001), psychological well-being (p < 0.01), peers/social support (p < 0.001), and school environment (p < 0.001) for parents-reported dimensions. However, in multivariate analysis on self-reported QoL, disease severity was significantly associated with the self-perception dimension only. Self-reported QoL of CHD children was similar to that of same-age healthy children in seven of 10 dimensions, but parents-reported QoL was impaired in four of five dimensions.

Fetal heart disease and interruption of pregnancy: factors influencing the parental decision-making process.

OBJECTIVE: To identify factors influencing parental decision when a fetal cardiac disease is diagnosed. METHOD: All pregnancies with fetal cardiac abnormalities diagnosed at three academic hospitals of Marseille, France, between 2004 and 2008, were retrospectively studied. The association between maternal and fetal variables (maternal age, parity, ethnicity, gestational age at diagnosis, nuchal translucency, fetal gender, chromosomal and extra cardiac abnormalities, and severity of the cardiopathy) and parental decision was tested using univariate and multivariate statistical methods RESULTS: One hundred eighty-eight cases of fetal cardiac disease were analysed, of which 63 were interrupted pregnancies (IP) and 125 continued pregnancies (CP). Four factors were important in the parental decision-making process: the severity of cardiac malformation, the ethnic origin of the parents, the gestational age at diagnosis and the chromosomal abnormalities. CONCLUSION: Counselling of parents following the diagnosis of a congenital heart disease should take into account that, in addition of the severity of the congenital heart disease (CHD), ethnicity, gestational age at diagnosis and chromosomal abnormalities influence parental decision regarding pregnancy continuation or interruption.

[A monocenter experience of ventricular septal defects treated by catherization]. / Expérience monocentrique de la fermeture par cathétérisme interventionnel des communications interventriculaires.

INTRODUCTION: Studies on ventricular septal defects closure by catheterization confirm its feasibility without reporting clearly the indications and difficulties encountered. PATIENTS AND RESULTS: From 2001 to end-2006, 22 patients benefited from 26 ventricular septal defects closure (15 muscular and 7 membranous) at a median age and weight of 2.1 years and 12.5 kg, respectively. A perventricular catheterization was performed in 2 cases. Eighteen patients (82%) benefited from 21 prostheses with success. The closure was associated to surgery in 9 cases (41%) whereas it substituted surgery in the other 13 cases (59%). The median duration of the procedure was significantly longer in case of muscular ventricular septal defects (215 min (175-510) vs. 170 min (120-225), p=0.04). Major complications are reported in 5 cases out of 26 catheterization (19%), including one death related to conduction block, occurring after the implantation of two prostheses in a patient with aortopulmonary transposition. All other associated cardiac diseases have been corrected. A prosthetic emboli occurred in one case, 1.5 months after implantation. It had been retrieved by catheterization. Two patients died afterwards from non-procedure-related causes. After a median follow-up of 1.1 years, the 17 other patients remained asymptomatic. One child with a perimembranous prosthesis presents a paroxystic atrio-ventricular block. CONCLUSION: Even though indispensable for the curative treatment of several congenital cardiac diseases including non-operable ventricular septal defects, this procedure is related to a substantial rate of mortality and morbidity. The risk of atrio-ventricular block must be adequately considered in case of membranous ventricular septal defects.

[Long-term results of complete atrio-ventricular canal correction]. / Résultats à long terme du traitement chirurgical du canal atrioventriculaire complet.

OBJECTIVES: To assess the prognosis of surgically corrected complete atrio-ventricular canal and determine risk factors for death and redo surgery. PATIENTS: and method: From January 1984 to March 2006, 126 patients were distributed in 3 groups according to their date of intervention: group I (1984-1991), group II (1992-1999) and group III (2000-2006). They were operated at mean age of 8.35 + 13.3 months. Down syndrome was present in 99 patients (79%). A partial or total closure of the left atrio-ventricular valvular cleft was performed in 47 patients (37%). RESULTS: The in-hospital mortality was at 14.28% (18 patients), including 23.7% in group I, 16.2% in group II and 4.4% in group III (p=0.06). The predictive factors of survival were the Down syndrome (p<0.05) and surgery of the left atrio-ventricular valvular cleft (p=0.05). An early reoperation was required in 6 cases, for a severe leak of this valve (n=4) or an important residual shunt (n=2). After a mean follow-up of 9.5+6.9 years, the survival rate was at 83.6% and 10 patients (9.9%) had a redo surgery for a leak of the left atrio-ventricular valve (n=6) or sub-aortic stenosis (n=4). The rate of no-redo surgery for valve insufficiency was at 94.2% at 5 years, 91.1% at 10 years and 87.6% at 15 years. CONCLUSION: The surgical treatment of complete atrio-ventricular canal became more and more efficient with poor rates of mortality and redo surgery during these last years. The closure of the left atrio-ventricular valve cleft, mostly partial, is frequently performed. Patients with the Down syndrome have a better vital prognosis and lower rates of long-term redo surgery.

[Rehabilitation of hypoplastic pulmonary arteries in pulmonary atresia with ventricular septal defect. Medium term results]. / Réhabilitation des artères pulmonaires hypoplasiques dans l'atrésie pulmonaire avec communication interventriculaire. Résultats à moyen terme.

The treatment of pulmonary atresia with ventricular septal defect, hypoplasia of the native pulmonary arteries and major aorto-pulmonary collaterals is controversial with a wide range of options from symptomatic treatment to surgical correction, but usually unifocal surgery. However, the collateral vessels used in the reconstruction are often tortuous and stenosed, resulting in a problematic haemodynamic result. The authors report the results of medico-surgical rehabilitation of the native pulmonary arteries in three stages. Since 1990, patients with an average Nakata index of 27.5 +/- 16.7 mm2/m2 underwent elective surgical connection of the native pulmonary arteries to the right ventricle (1st stage) followed by their rehabilitation with catheter occlusion of the collaterals (2nd stage) with the aim of preparing surgical correction (3rd stage). The first surgical stage at an median age of 5 months (0.1 to 25.2) was complicated by one death. After an average of 2.1 +/- 1 catheterisations, 17 patients underwent surgical correction at an median age of 2.18 (0.6 to 10.3) years with an average Nakata index of 207 +/- 91 mm2/m2. All survived surgery with three late deaths in patients with poor haemodynamic results (right ventricular/left ventricular pressure ratio>80%). After an median follow-up of 9.2 (3.5 to 17) years, the 14 survivors are in NYHA Class I or II with a good haemodynamic result in 10 patients. The authors conclude that despite some late deaths and raised right ventricular and pulmonary artery pressures in a minority of patients, the rehabilitation of the pulmonary arteries is an effective management of this cardiopathy.

Assessment and risk modeling of airborne enteric viruses emitted from wastewater reused for irrigation.

Reclamation of wastewater (WW) for irrigation, after treatment represents a challenge that could alleviate pressure on water resources and address the increasing demand for agriculture. However, the risks to human health must be assessed, particularly those related to human enteric viruses that resist standard treatments in most wastewater treatment plants (WWTP). The risks associated with exposure to viral bioaerosols near WWTP and near agricultural plots irrigated with WW are poorly documented. The objectives of this study were to 1) better characterize human enteric viruses found in bioaerosols near a "standard WWTP" and over fields irrigated with treated WW and 2) propose a numeric model to assess the health risk to populations located close to the irrigated areas, with particular attention to norovirus, which is responsible for most viral gastroenteritis in France. Water and air samples were collected at various locations in the largest French WW-irrigated site near Clermont-Ferrand, at the WWTP entrance and after treatment, in the air above activated sludge basins, and above fields irrigated with WW. Various enteric viruses were found in the water samples collected both before and after treatment. Norovirus was the most abundant with >10e4 genome copies/l (GC/L) before treatment and ~10e3 GC/L after treatment. Low quantities (<10e3GC/m3) were detected in the air above active sludge pools and irrigated plots. Hepatitis E virus was detected in all sampled compartments. A quantitative microbial risk assessment (QMRA) approach, including a simplified atmospheric dispersion model, allowed assessment of norovirus infection risk. The Bayesian QMRA approach considered wind speed measurements over 21years, and the variability and uncertainty of all measurements throughout the chain up to the risk. The probability of infection within one year for the most exposed WWTP employees was >10e-4 for strong wind speed (≥3m/s) and a constant emission rate of 8e3 GC/m3/s. This probability decreases by 3 log when the distance to the emission source is doubled. This information can aid development of safe water reuse policies in terms of local setback distance and wind conditions for wastewater reuse.

Challenging Investigation of a Norovirus Foodborne Disease Outbreak During a Military Deployment in Central African Republic.

In January 2016, a large-scale outbreak of acute gastroenteritis was reported among French armed forces deployed in the Central African Republic. Challenging investigations, conducted from France, made it possible to identify a norovirus genogroup II in both stool and food samples, confirming a norovirus foodborne disease outbreak. Infected food handler management is discussed.

[Value of the Norwood-Sano compared with the classical Norwood procedure in hypoplastic left heart syndrome]. / Intérêt de l'intervention de Norwood-Sano par rapport à l'intervention classique de Norwood dans l'hypoplasie du coeur gauche.

Palliative treatment of the hypoplastic left heart syndrome is rarely practiced in France because of the poor prognosis of the first surgical stage of the Norwood procedure. The recent modification proposed by Sano seems to give more promising results. The authors report their experience with the Norwood-Sano procedure compared with the classical Norwood procedure in 13 neonates with hypoplasia of the left heart. From 1999 to 2005, 8 patients underwent the classical Norwood procedure (1999-2001) and 5 had the Sano modification (2002-2005). During the preoperative period, there was 1 case of a very restrictive interatrial septum and 5 patients required mechanical ventilation. The patients were operated at an average age of 8.5 +/- 12 days. The results showed that survival after the first surgical stage was significantly better with the Sano modification (12.5% versus 80%; p= 0.03). All patients who survived the first stage went on to the second stage with a bi-directional right superior cavo-pulmonary derivation at an average of 6.1 +/- 2.4 months. Despite the infundibulotomy of the Sano modification, no arrhythmia or right ventricular dysfunction was observed after an average follow-up of 21.7 +/- 7.6 months. The authors conclude that the Sano modification improved survival of patients with hypoplasia of the left heart after the first palliative surgical procedure. The long-term follow-up of patients operated by this technique should particularly look out for arrhythmias and right ventricular dysfunction.

[Medium and long-term results of anatomical correction of transposition of the great arteries in Marseille]. / Résultats à moyen et long terme de la correction anatomique de la transposition des gros vaisseaux à Marseille.

The object of this study was to evaluate the results of anatomical correction of transposition of the great arteries (TGA) in an inter-regional centre. The characteristics of 220 patients operated between 1985 and 2002 were analysed. Prenatal diagnosis of TGA was made in 38 patients (17%) including 36 of the 109 patients operated since 1995 (33%) and leading to earlier atrial septostomy in this situation (p= 0.046). Since 1995, the global early mortality of 12.3% has been significantly reduced to 5.5% (N= 6) including 4.4% (3/68 cases) of simple TGA (p= 0.002). In multivariate analysis, the only independent risk factor for early mortality was the occurrence of a major per-operative complication (p< 0.0001). The average follow-up was 4.8 +/- 4.5 years with an 86% survival at 5, 10 and 15 years and 97% survival at 5 years for patients operated after 1995. There were 22 reoperations including 14 on the right ventricular outflow tract. The non-reoperation rate at 10 years was 80% for simple TGA versus 70% in complex TGA (p= 0.0001). Survivors are asymptomatic with a normal ECG and normal LV function on echocardiography. Five patients have significant pulmonary stenosis and 4 have mild aortic regurgitation. The exercise stress tests performed between the ages of 7 and 10 were all normal. The authors conclude that that modern and reproducible management of TGA is possible in an inter-regional centre with results comparable to those of surgical centres of reference.

Echocardiographic characteristics and outcome of straddling mitral valve.

OBJECTIVES: This study sought to characterize the echocardiographic features of straddling mitral valve (SMV) and to determine its surgical implications and midterm outcome in a large clinical cohort. BACKGROUND: Despite a relatively large body of literature on the postmortem anatomy of SMV, there is a paucity of information regarding its echocardiographic features, surgical implications and preoperative predictors of outcome. METHODS: A retrospective review identified 46 patients with SMV between 1982 and 1999 who underwent echocardiography and surgery and had follow-up data. A detailed review of the echocardiograms, surgical reports and all pertinent records was undertaken. RESULTS: Review of the echocardiograms revealed a widely varying anatomy among the study patients. However, four distinct groups with relatively uniform morphologic features could be distinguished on the basis of segmental analysis. Cardiac malposition associated with right ventricular hypoplasia, superior-inferior ventricles and criss-cross atrioventricular relations were common among patients with [S,D,L] (S = visceroatrial situs solitus, D = D-ventricular loop, L = L-malposition of the great arteries) (n = 6) and [S,L,D] (n = 5) segmental combinations but were rare among patients with [S,D,D] (n = 26) and [S,L,L] (n = 9) combinations. Surgical management consisted of a functional single-ventricle palliation in 38 patients (83%) and biventricular repair in 8 patients (17%). Overall mortality was 22%, but none of the seven patients who were operated on during the cohort's last five years (1994 to 1999) has died. By multivariate analysis, noncommitted ventricular septal defect was the strongest independent predictor of death (relative risk = 10.2), followed by multiple ventricular septal defects (relative risk = 4.7). CONCLUSIONS: This study demonstrates that echocardiography provides detailed noninvasive imaging of the complex anatomic features of SMV and its associated anomalies. Anatomic classification based on segmental analysis allows the distinction of four groups with more uniform morphologic features. Although a biventricular approach is feasible in selected patients, a functional univentricular palliation is indicated in those with major straddling and markedly hypoplastic ventricles.

[Medico-surgical treatment of occlusion of the left pulmonary artery by extension of ductal tissue (pulmonary coarctation)]. / Traitement médico-chirurgical de l'occlusion de l'artère pulmonaire gauche par extension de tissu ductal (coarctation pulmonaire).

The authors report the results of surgical treatment completed by interventional catheterisation of occlusion of the left pulmonary artery by extension of ductal tissue. Since 2001, 7 patients operated for occlusive coarctation of the left pulmonary artery at an average age of 11 months (3 to 37 months) had a restenosis. The cardiac malformation was pulmonary atresia with ventricular septal defect (N=4), tetralofy of Fallot (N=2) and critical pulmonary valvular stenosis (N=1). Pulmonary artery surgery consisted of resection anastomosis in 4 cases and a plasty in 3 cases. A primary angioplasty was performed 5 to 170 months (median 12 months) later, at an average age and weight of 3.4 years (0.7 to 16.9 years) and 14 Kg (8 to 52 Kg) with implantation of 3 stents. The median diameter increased from 5 mm (1 to 9 mm) to 10 mm (6 to 16 mm). Tc-99m scintigraphy showed an increase in mean left pulmonary perfusion from 9% (6 to 28%) to 28% (18 to 42%). Secondarily, 3 repeat angioplasties were necessary with a total of 6 stents implanted in 7 patients. After an average of 2.9 years (0.8 to 6.3 years) follow-up, the patients were asymptomatic with normal right ventricular pressures and a mean left pulmonary perfusion of 33% (24 to 45%). The authors conclude that the treatment of left pulmonary occlusion by coarctation requires a medico-surgical approach in which angioplasty and stenting complete successfully the surgical revascularisation.

[Results of surgery for coarctation of the aorta in children under one year of age]. / Résultats de la chirurgie de la coarctation aortique chez l'enfant de moins de 1 an.

The aim of this study was to determine the surgical results of repair of coarcta tion of the aorta in children under 1 year of age and to assess the risk of recoarctation. Between 1984 and 2004, 206 children under 1 were operated for coarctation of the aorta. Three groups of patients were identified: Group I (isolated coarctation, N=99), Group II (associated ventricular septal defect, N=63), and Group III (complex congenital heart disease, N=44). The transverse aorta was hypoplastic in 51% of cases. Uni- and multivariate analysis of the risk factors for mortality and recoarctation was performed. An extended resection anastomosis was performed in 206 patients. Reconstruction of the transverse aorta was performed in 32 cases. The hospital mortality was 3.9% (N=8). The presence of a complex intracardiac anomaly was a risk factor for mortality on multivariate analysis (p=0.023). In univariate analysis, a two-step management of patients in Group III was a significant risk factor for mortality (p=0.036). Thirty patients (14.6%) had recoarctation (gradient > 20 mmHg, follow-up 40 +/- 44 months). On multivariate analysis the severity of the immediate postoperative gradient was the only risk factor for recoarctation. The authors conclude that surgery for coarctation of the aorta is associated with excellent results in children under 1 year of age. The prognosis is related to the presence of associated severe cardiac malformations. Survival seems to be better if treatment is carried out in a single stage in this group. The risk of recoarctation is related to the immediate postoperative transisthmic gradient which justifies extensive repair of hypoplastic aortic arches.

[Extracorporeal assistance in paediatric intensive care]. / Assistance extracorporelle en réanimation pédiatrique.

OBJECTIVE: For 3 years we have used extracorporeal assistance in intensive care frequently. This study evaluates our results. METHOD: We studied the patient records for those treated between January 2002 and January 2005. The method used, indications and morbidity/mortality were analysed. RESULTS: We performed 24 circulatory assistance procedures in 20 patients (median age: 5 months), arterio-venous with oxygenation (n=18), veino-venous with oxygenation (n=3) or biventricular (n=3). The indications were post cardiotomy cardio-respiratory failure (Group I; n=20, 16 patients), pure respiratory failure (Group II: n=1), or pre-transplant/recovery (Group III: n=3). Five procedures (4 from group I and 1 from group III) required cardiac massage (no fatalities). The average duration of assistance was 7 +/- 6 days (2 to 20 days). Treatment was successfully discontinued in sixteen patients 80%), one of them thanks to heart transplant. Four (20%) died during assistance. The morbidity essentially consisted of further surgery for haemostasis, multiple transfusions, and infections. Three patients (15%) died later (1 at 17 months after discontinuation) from complications unrelated to the assistance. No neurological sequelae were noted in the survivors. CONCLUSION: These results confirm the usefulness of circulatory assistance when medical treatment has failed, particularly in the post-operative period of paediatric cardiac surgery or while awaiting transplantation.

[The palliative care in geriatrics: a retrospective study of 40 cases]. / Les soins palliatifs en gériatrie: étude rétrospective de 40 cas.

INTRODUCTION: Owing to the very great age and the polypathology of the patients in geriatrics, we are often confronted to the palliative care decision. PURPOSE: The purposes of this retrospective study were both to define the criteria leading to palliative care and to analyse the evolution of patients. METHOD: We analysed 40 files of patients hospitalised in Geriatric internal medicine or Geriatric rehabilitation departments over 11 months. RESULTS: Mean age was 85.4 years and 62.5% of patients were females. Infections, heart failure, general weakness, orthopaedic affections, strokes and cancers were the main causes of hospitalisation. Patients had 3 medical or surgical histories of chronic or cured serious diseases and a MMSE average value of 17.7. The rate of malnutrition was 92% and 90% of patients were very dependent. Severe infections, cancers, heart failure and severe pressure ulcers were the main affections for decision of palliative care. The latter was always decided by the staff with patients or families taking part in 8 cases and being informed in other cases. The palliative care lasted 7 days on average. Morphine was used in 31 cases. No artificial nutrition was introduced. CONCLUSION: The decision of palliative care is very complex since great age, polypathology, great dependence and high prevalence of cognitive disorders are frequent in this population.

Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto-pulmonary collaterals.

OBJECTIVE: Among 63 patients with pulmonary atresia and ventricular septal defect (VSD), 10 patients with extreme hypoplasia of the pulmonary arteries (PA) (mean Nakata index 20.6 mm(2)/m(2)), but with confluent arteries and a diminutive main PA, and major aorto-pulmonary collaterals (MAPCAS), have been submitted to a 'rehabilitation' of the PA with several stages: (i) connection between RV and PAs, (ii) interventional catheterizations, (iii) complete correction with or without unifocalisation. We report here the results of this approach. METHODS: The RV-PA connection was direct (nine cases) or with an homograft conduit (one case), done under normothermic cardiopulmonary by-pass in patients aged 4.9 months (range 0.1-18 months). Subsequently, six underwent interventional catheterizations (dilations and stents in the PA, MAPCAS occlusion by coils). Complete correction was done in seven patients (mean age 30 months, range 8-49). One patient is awaiting correction. RESULTS: One patient died after the first stage. All patients having had the third stage had a satisfactory development of the PA, had a complete closure of the VSD and a satisfactory reconstruction of the PA bifurcation. There was one death of severe pulmonary infection 6 months after repair. All other patients have been followed by catheterization and/or echocardiograms. With a follow-up of 83+/-65 months, all patients are improved, 50% have no cardiac medications, none has residual shunt, RV/LV pressure ratio is 0.6 (range 0.3-1). CONCLUSIONS: The strategy of 'rehabilitation' of PA allowing: (i) antegrade flow in the PA, (ii) interventional catheterizations, (iii) growth of the PA with possible angiogenesis, (iv) complete correction, is a logical approach to be undertaken in the young patient and is a valid alternative to strategies relying more on MAPCAS for pulmonary vascular supply. The therapeutic sequences depend upon the individual anatomy.

Anatomic repair of corrected transposition or atrio-ventricular discordance: report of 8 cases.

BACKGROUND: It is well recognized that a right ventricle (RV) may eventually fail if working as systemic. This has led to arterial switch operation for transposition of the great arteries (TGA), but more recently to anatomic correction of 'corrected transposition' or atrio-ventricular discordance (AVD) combining an atrial switch and a ventricular outflow repair (arterial switch or Rastelli type). METHODS: We report here 8 cases of such anatomic correction of AVD in patients from 3 months to 10 years. Of these patients, 6 had situs solitus, and 2 situs inversus; 7 patients had a ventricular septal defect (VSD); 5 had an increased pulmonary flow, and 3 had a decreased pulmonary flow with pulmonary stenosis (PS) or pulmonary atresia (PA). In all, 6 had discordant and 1 had concordant ventriculo-arterial (VA) relations, and 1 had double-outlet RV, 2 had an associated coarctation, and 1 had a cor triatriatum. Of the 8 patients, 5 had pulmonary artery hypertension, 3 a moderate to severe tricuspid insufficiency and 6 had undergone one or more previous operations. Correction was done between 3 months and 10 years of age, with 7 having a Senning procedure (twice left-sided) and 1 a Mustard type operation. VA outflows were repaired with: 4 arterial switches, 1 left ventricle to aorta rerouting, 1 right ventricular outflow tract (RVOT) repair and 2 Rastelli. A tricuspid annuloplasty was done once. RESULTS: All patients survived the operation, but 2 patients needed a reoperation (both successful): late obstruction of pulmonary venous channel after a left-sided Senning, and residual VSD closure. Mean follow-up is 45 months (3-122). Of the 8 patients, 6 patients are in sinus rhythm, 2 have junctional rhythm. A patient with junctional rhythm and occasional tachycardia died suddenly 18 months after surgery 1 week after satisfactory evaluation. All have a normal left ventricle function as evidenced by echocardiography. CONCLUSIONS: Despite a more demanding procedure, the 'anatomic repair' of hearts with AVD is possible, even in infancy, with good early and mid-term result. Despite the potential late rhythmic problems of the Senning operation (somehow more frequent with AVD in our experience), it may become, with increasing experience, the procedure of choice in corrected transposition.

[Predictive factors of Doppler echocardiography in persistent pulmonary artery hypertension of the neonate]. / Facteurs prédictifs de l'échocardiographie-doppler dans l'hypertension artérielle pulmonaire persistante du nouveau-né.

INTRODUCTION: echocardiographic evaluation in neonates with persistent pulmonary artery hypertension is often limited to pressure measurements and analysis of pulmonary artery blood flow. The prognostic significance of a more detailed analysis, in particular of the extra-pulmonary shunt, is not known. PATIENTS AND METHOD: we analysed retrospectively the echocardiographs of neonates with persistent pulmonary artery hypertension who were also entered in a randomised therapeutic trial of treatment with inhaled nitric oxide. Our aim was to identify the predictive echographic factors for extra-corporeal circulatory assistance, death and a good response to nitric oxide. RESULTS: out of the 85 neonates studied, an extra-pulmonary right-left shunt across the foramen ovale or the ductus arteriosus was present in 80 patients (94%). Biventricular function was normal in the majority of patients while the cardiac index was reduced (< 2L/min/m2) in 61% of cases. With multivariate analysis, an exclusively right-left ductal shunt was a predictive factor for death (odds ratio 7.8; 95% confidence interval 1.2 to 52.8; p=0.04) while an exclusively left-right ductal shunt was at the limit of significance for circulatory assistance (odds ratio 0.13; 95% confidence interval 0.01 to 1.22; p=0.07). In the 40 patients randomised to receive nitric oxide, 28 responded positively with a reduction of at least 20% in the oxygenation index measured by post-ductal arterial gasometry. The existence of a left-right atrial shunt increased the risk of a poor response to nitric oxide (odds ratio 7.46; 95% confidence interval 1.23 to 45.1; p=0.028). CONCLUSION: precise echocardiographic evaluation of these patients allows identification of prognostic factors and adjustment of vasodilator treatment.

[Multicentre study of percutaneous closure of interventricular muscular defects with the aid of an Amplatzer duct occluder prosthesis]. / Etude multicentrique de la fermeture percutanée des communications interventriculaires musculaires à l'aide d'une prothèse Amplatzer duct occluder.

Percutaneous closure of interventricular defects is an alternative or adjunct to surgery with the disadvantage of a sometimes prolonged and difficult catheterisation only during which the choice of prosthesis can be determined. Despite the existence of an occlusion prosthesis, the Amplatzer Septal Defect Occluder, specifically conceived for this purpose, an arterial canal occlusion prosthesis such as the Amplatzer Duct Occluder is sometimes better suited for the occlusion of certain interventricular defects. Since 1999, 11 Amplatzer Duct Occluders were used for the closure of interventricular muscular defects, during 10 catheterisations in 9 patients with a median age of 2.5 (0.1 to 43.9) years. In 5 cases there were residual septal defects after failure of initial surgery. 10 were successful with prostheses of 6/4 mm to 12/10 mm while in one patient the interventricular communication had to be closed surgically because the 14/12 mm prosthesis could not be positioned due to a tortuous introduction route. The only complication was a pericardial effusion requiring surgical drainage in one infant. After a median follow up of 2.1 (0.3 to 4.2) years, all of the patients had a satisfactory functional status, with minimal residual shunt on echocardiography in 2 cases. The Amplatzer Duct Occluder therefore seems better adapted for the closure of post-operative residual interventricular communications, with a thickened interventricular septum or even when the tricuspid valve is very close to the right ventricular side of the orifice.

[Case history of mitochondrial cytopathy with cardiac expression]. / A propos d'une cytopathie mitochondriale d'expression cardiaque.

Childhood dilated cardiomyopathies comprise a wide aetiological spectrum for which the prognosis and treatment sometimes vary considerably. We report the case of a patient affected by a rare form of mitochondrial cardiomyopathy in whom the diagnosis of acute myocarditis had initially been made. The progression was fatal even though the patient was awaiting a cardiac transplant. Beyond the difficulties of diagnosis and treatment of this pathology, this clinical case underlines the significance of an early aetiological diagnosis based on the results of an endo-myocardial biopsy. Cardiac transplantation should be envisaged for this type of patient based not only on the clinical status but equally by taking account of the prognosis of this disease for which deterioration can sometimes be very rapid.

[Rehabilitation of hypoplastic pulmonary arteries and anatomic correction of pulmonary atresia with interventricular communication]. / Réhabilitation des artères pulmonaires hypoplasiques et correction anatomique des atrésies pulmonaires avec communication interventriculaire.

Conventional treatment of pulmonary atresia with ventricular septal defect (VSD), hypoplastic pulmonary arteries (PA) and major aorto-pulmonary collaterals (MAPCAs) is controversial: from symptomatic and palliative treatment for some authors to surgery with unifocalisation of collaterals for others. These treatments never use native pulmonary arteries as only source of pulmonary flow, but create "neo-pulmonary arteries". Nine cases of pulmonary atresia with VSD, hypoplastic PA and MAPCAs were treated by rehabilitation of native PA through a staged approach: 1) surgical neonatal connection between right ventricule (RV) and hypoplastic PA, 2) evaluation and interventionnal catheterism with angioplasty of PA stenosis and closure of collaterals, 3) complete surgical correction with reconstruction of right outflow track and PA and closure of VSD. After first surgical stage of RV-PA connection at the mean age of 4.8 months (+/- 5.6 months), 8 patients were alive and underwent 22 cardiac catheterisms (mean of 2.7 per patient), with angioplasty of PA, and occlusion of MAPCAs in 6 and 2 patients respectively. Seven patients underwent complete anatomical correction at the mean age of 28.8 months (+/- 17.7 months) with one late death. The 6 remaining patients had encouraging hemodynamic status (RV pressure/LV pressure ratio at 0.6 +/- 0.26; mean left and right distal pulmonary pressure at 15.2 mmHg (+/- 9.1 mmHg)), and good functionnal status (3 in NYHA functionnal class 1, and 3 in class 2), for a mean follow-up of 79.5 months (+/- 41.4 months). One patient had reoperation on right outflow track stenosis, 6 years after correction. This small series enhances the feasibility of a staged approach with rehabilitation of small PA, allowing complete surgical correction with the native PA with good hemodynamic and functional results in pulmonary atresia, with VSD, hypoplastic PA and MAPCAs.