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.

Assessment of team training in management of adverse acute events occurring during cardiopulmonary bypass procedure: a pilot study based on an animal simulation model (Fouilloux, Team training in cardiac surgery).

BACKGROUND: Successful cardiac surgery is highly dependent upon effective and efficient teamwork. Practical training and development will further enhance the team ability to react to a series of low-frequency occurring adverse events during cardiopulmonary bypass (CPB). One of our specialized educational programs focuses on training the whole team. This training is based on an original animal simulation model. The objective of this pilot study was to assess our method of training and learning in an attempt to optimize and improve team management and functioning. METHODS: Four members of the same cardiac surgery team joined our program. They performed a common procedure, with a cardiopulmonary bypass (CPB) circuit set up to produce several adverse incidents. Events management was analyzed and debriefed in technical and non-technical perspectives. RESULTS: Management of the adverse events was significantly improved. Discussion and debriefing time was fundamental in identifying the most appropriate management for each event. Rescue procedures were assimilated and team training was found to be effective, with the time reduced by up to 50% for 3 events and by up to 70% for air embolism. CONCLUSION: Our pilot program is an innovative, low-cost tool for the improvement of the management of adverse events occurring during CPB. It includes the different components of surgical education and training. Such an educational tool might be relevant for training. To confirm those encouraging results, it should be assessed in a larger surgical team panel. Further investigations are required for assessing efficiency in real conditions.

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.

Reform of the 3rd cycle in surgery training.

The main objectives of the reform of the 3rd cycle of medical studies in France that was instituted in 2017 after eight years of preparation, are to train future specialists in a consistent and equitable fashion and to replace the previous time-based qualification by training based on the progressive acquisition of skills. This reform was an opportunity for the 13 different French surgical specialty Colleges involved to share reflections on what a surgeon actually was and to define training in surgical sub-specialties. The current reform is well adapted to these specifications and has fostered training models that are consistent with each other. This article discusses the historical construction of this reform, what will change in the training of future surgeons, as well as some points that warrant caution. The third cycle reform has also triggered a reform of the second cycle, which is expected to come into force for the 2020 academic year. Its objective will be to eliminate the guillotine effect created by the National Classifying Examinations and to allow students to better understand and test their desire and skills for a given specialty. It will be up to these same surgical Colleges to determine how to do this for the sub-specialties of the "surgery" discipline.

[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.

[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.

[Unusual outcome of mitral valve infectious endocarditis in a premature infant]. / Evolution inhabituelle d'une endocardite infectieuse mitrale chez un prématuré.

The authors report a case of Staphylococcus Aureus infectious endocarditis in a premature baby. Echocardiography on the 8th day of life showed a large vegetation on the anterior mitral leaflet without significant regurgitation. Intravenous antibiotics and platelet antiaggregant therapy were given. At three weeks of age the vegetation split into two, one part attached to the foramen ovale and the other to the anterior mitral leaflet. At one month, the development of severe mitral regurgitation led to surgical valvuloplasty in a 2 kg neonate, the vegetation on the foramen ovale having spontaneously fragmented. The ablation of the mitral vegetation associated with commissuroplasty reduced the mitral regurgitation. At 3 months after surgery, the child was asymptomatic with a minimal mitral regurgitation.

[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.

[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.

Estrogen and progestin regulation of the progesterone receptor concentration in human endometrium.

The concentration of the progesterone receptor (PR), both cytosol and nuclear, has been measured in the endometrium of 31 normal menstruating women during the 2 phases of their menstrual cycle and compared with the plasma 17 beta-estradiol and progesterone concentrations. There was no relationship between PR concentrations and the plasma steroid levels when the 2 phases of the cycle were considered; however, a statistically significant correlation (r = 0.70; P less than 0.005) was observed between PR concentration and plasma 17 beta-estradiol when only the follicular phase was considered. PR was then measured in the endometrium of 14 postmenopausal patients treated with ethinylestradiol at increasing doses with or without association of chlormadinone acetate. Ethinylestradiol was shown to increase PR concentration (P less than 0.05), and chlormadinone acetate was found to prevent this increase. These data suggest that in humans, as in other mammalian species, the endometrial PR concentration is under estrogen and progestin control.

PIP: The respective roles of estrogens and progestins on endometrial progesterone receptors (PRs) were evaluated in 16 proliferative-phase and 15 secretory-phase biopsy samples from normally menstruating women. Plasma estradiol-17-beta and progesterone were measured in both cytosol and nuclear extracts. In addition PRs were measured in biopsied tissue taken from 14 postmenopausal women who had been treated with increasing doses of ethinylestradiol (with or without chlormadinone acetate) who presented with breast cancer. Among the normally menstruating women, there were no relationships between PR concentrations and plasma steroid levels when both phases of the cycle were considered; however, a correlation of significance (P .005) was apparent between PR concentration and plasma 17-beta-estradiol when only the follicular stage was considered. In the postmenopausal women who were under treatment, ethinylestradiol administration seemed to increase PR concentration (P ,05), whereas concomitant treatment with chlorimadinone acetate blocked this increase. Therefore, mammals seem to have endometrial PR levels controlled by both estrogens and progestins.

Measurement of the corticosteroid-binding globulin, progesterone, and progesterone "receptor" content in human endometrium.

The corticosteroid-binding globulin (CBG) content has been determined in cytosols prepared from human endometrium taken at different phases of the menstrual cycle, at 8-10 weeks gestation, and during treatment with an oral contraceptive. The data were compared to the progesterone "receptor" and the progesterone concentration in the same tissue extracts. The plasma contamination was estimated by the serum albumin content. It was observed that CBG is a constant contaminant of the endometrial cytosols. All of the CBG content can be considered to be of plasma origin. The cytoplasmic progesterone "receptor" content is between 21-33% of the CBG concentration during the menstrual cycle and is only 1% during pregnancy. The cytosol progesterone concentration is much higher in endometrium than would be expected from the plasma contamination and the level of the progesterone "receptor".

Association of mild hyperhomocysteinemia with cardiac graft vascular disease.

In non-transplant patients mild hyperhomocysteinemia is an independent risk factor for vascular disease. The aim of this study was to determine whether hyperhomocysteinemia is associated with graft vascular disease. Fasting total plasma homocysteine was assessed in 18 patients with graft vasculopathy and 18 transplanted patients without graft vasculopathy matched for age, sex and the time since transplant. All were on cyclosporin. Graft vasculopathy was defined at coronary angiography as stenoses > or = 25%, or aneurysms. We found that hyperhomocysteinemia ( > or = 15 micromol/l) is common among transplanted heart recipients and significantly more frequent in the patients with graft vasculopathy (17/18 versus 11/18). Accordingly, the mean homocysteinemia was significantly higher in the group with graft vasculopathy (23.6+/-7.8 versus 16.9+/-7.1 micromol/l, P=0.01). The elevation of homocysteine plasma levels in the heart transplant recipients has probably multiple causes. The main cause seems to be renal failure. Additional causes could be azathioprine treatment or genetic polymorphisms. These results suggest that besides the immunological factors, homocysteine can play an additional role in the pathogenesis of graft vascular disease.

Kidney function in cyclosporine-treated paediatric pulmonary transplant recipients.

BACKGROUND: Lung or heart-lung transplantation is a useful therapy in life-threatening pulmonary disorders during childhood. Cyclosporine A is a major immunosuppressive treatment but has a number of adverse effects including nephrotoxicity. There have been no reports on the long-term evolution of renal function in a large series of paediatric pulmonary transplantation recipients. METHODS: We examined 19 patients followed up for at least 3 years after pulmonary transplantation. The mean time of follow-up was 5.36 years. Kidney function was evaluated by calculation of glomerular filtration rate (GFR) according the Schwartz formula. RESULTS: The GFR was normal before transplantation in all patients. The short-term evolution of GFR was marked by a significant drop during the first and until the 6th month. Then, regardless of the level reached at the end of the 6th month, the GFR remained stable in all patients except one until the end of follow-up. At the end of follow-up, 31% had normal GFR, 57% had mild chronic renal failure, and 5% had advanced renal failure. Hypertension was frequent and associated with renal failure. CONCLUSIONS: Paediatric pulmonary recipients showed evidence of long-term cyclosporine A-associated nephrotoxicity. Most of this toxicity occurred during the first 6 months.

Fluvastatin decreases soluble thrombomodulin in cardiac transplant recipients.

We conducted a randomized, placebo controlled, double-blind, cross-over study, to assess the effects of a 4-week fluvastatin therapy on plasma markers of endothelial activation or injury in 20 transplanted heart recipients. The levels of thrombomodulin and von Willebrand factor antigen were higher at baseline in cardiac transplant recipients than in age and sex-matched healthy controls. Plasma total cholesterol showed a 21% reduction on fluvastatin therapy (p = 0.0001). Fluvastatin treatment had no significant effect on creatininemia, plasma cyclosporine, PAI-1 antigen, PAI-1 activity, tPA antigen, and Von Willebrand factor. However, fluvastatin produced a significant decrease of plasma thrombomodulin (66.7 ng/ml on placebo versus 58.8 ng/ml on fluvastatin, p <0.001), suggesting a rapid improvement of endothelial injury in these patients.

Extending the concept of the autograft for complete repair of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: a report of ten cases of a modified procedure.

BACKGROUND: In most cases of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction, a Lecompte procedure (réparation à l'étage ventriculaire) is possible without interposition of a conduit between the right ventricle and pulmonary artery. However, the anterior location of the pulmonary arteries after the Lecompte maneuver may be a potential cause for right ventricular outflow obstruction, which continues to be reported in 5% to 25% of cases. We have used a tubular segment of aortic autograft to connect the pulmonary artery, left in the orthotopic posterior position (without the Lecompte maneuver), to the right ventricle in 10 consecutive patients with transposition, ventricular septal defect, and left ventricular outflow tract obstruction. METHODS: Ten consecutive patients aged 2 months to 11 years (mean 32 months) have undergone a modified Lecompte operation. Eight had severe pulmonary stenosis, two had pulmonary atresia, and four had a restrictive ventricular septal defect at the time of the operation. Two had multiple ventricular septal defects. Seven had undergone one (n = 5) or two (n = 2) previous modified Blalock-Taussig shunts. All patients underwent a total correction with left ventricular-aortic intraventricular connection (four needed a ventricular septal defect enlargement), connection between the right ventricle and pulmonary arteries with a tubular segment of autograft aorta, without the Lecompte maneuver (anterior location of the bifurcation of the pulmonary arteries) on the right (n = 6) or the left (n = 4) of the aorta. No valvular device was used for the right ventricular outflow repair. RESULTS: No early or late deaths occurred. One patient with multiple ventricular septal defects needed an early (2 weeks) reoperation for a residual muscular ventricular septal defect. All patients are currently in New York Heart. Association class I, without medications, in sinus rhythm, at a mean follow-up of 30 months. Late results up to 3.6 years show no calcification on the chest roentgenogram, and at the most recent echocardiogram, right ventricular pressures were low (25 to 40 mm Hg, mean 33 mm Hg) and no significant gradient (over 10 mm Hg) was found between the right ventricle and pulmonary arteries. Left and right ventricular function was satisfactory. CONCLUSION: This modification of the Lecompte operation using a segment of autograft allows an excellent early and late result, with no danger of compression of anteriorly placed pulmonary arteries, no significant right ventricular outflow obstruction, and normal appearance of the tubular autograft. In view of laboratory and clinical evidence, normal growth of the autograft can be anticipated. It allows an elective correction of transposition, ventricular septal defect, and left ventricular outflow tract obstruction without a previous Blalock-Taussig shunt (three patients) and correction at a young age (three patients younger than 1 year).

Successful treatment of fungal right atrial thrombosis complicating central venous catheterization in a critically ill child.

A 9-year-old boy was admitted to our pediatric intensive care unit after multiple trauma. On the 17th day post trauma, he developed catheter-related sepsis with candidemia. After removal of the catheter and 6 days of unsuccessful intravenous antifungal therapy, conventional and transesophageal two-dimensional echocardiography was performed revealing a large right atrial thrombus. Surgical thrombectomy under cardiopulmonary bypass was performed and the patient recovered within a few days. Fungal right atrial thrombus is a rare, life-threatening complication of central venous catheterization. Two-dimensional echocardiography is a simple and effective diagnostic technique that should be performed when candidemia is detected. The proper therapeutic response depends on the findings of this examination. For a symptomatic patient with a large, mobile thrombus, we strongly recommend thrombectomy. Surgery not only allows removal of the mass and thus elimination of the mechanical complication but is also a key to management of infection.