[Orotracheal intubation difficulty with lighted stylet: correlation of body mass index and neck circumference]. / Dificultad para la intubación orotraqueal con estilete luminoso: correlación del índice de masa corporal y circunferencia cervical.

OBJECTIVES: To evaluate the difficulty of intubation with a lighted stylet by correlating the body mass index (BMI) with the neck or cervical circumference (CC), and to establish the values of BMI and CC that could help identify a possible difficult intubation with this device. PATIENTS AND METHODS: An observational and correlational study was performed on selected patients by consecutive sampling who were intubated using the lighted stylet. Variables such as age, gender, ASA physical status, BMI, CC, transillumination intensity, Cormack-Lehane grade, and Mallampati scores, were recorded. Multivariable analyses were performed. RESULTS: 103 patients were included. The mean age of the patients was 39.0 ± 16.6 years, BMI 28.6 ± 6.8 kg/m² (33% obese and 29,1% overweight), CC 37.8 ± 4.6 cm. Those patients who presented difficulty for intubation had a CC 39.9 ± 5.2 cm (P ≤ 0.05), and a BMI 33.8 ± 7.6 kg/m² (P = 0.01). CONCLUSION: A lineal correlation exists between a BMI above 33 kg/m² and/or a CC greater than 40 cm and a difficult intubation using lighted stylet. Another method other than transillumination is recommended for orotracheal intubation in a patient with both characteristics.

Perinatal outcome in prenatally diagnosed cardiac rhabdomyoma case series.

OBJECTIVE: This study aims to determine the characteristics and outcome of prenatally diagnosed cardiac rhabdomyomas. STUDY DESIGN: This retrospective descriptive study includes cases referred to our university hospital. We studied sonographic characteristics of rhabdomyoma along with the neonatal outcome. RESULTS: Eight cases were included, with a mean gestational age at diagnosis at 31 weeks of gestation and five patients diagnosed after 32 weeks. We noted a male gender in 75%, multiple rhabdomyoma in 50%, mostly situated in the interventricular septum (41%) and valvular regurgitation in 25%. Most patients delivered at term, including five cesareans (62.5%). Six babies survived (75%); three of them were later diagnosed with tuberous sclerosis (50%). CONCLUSION: Cardiac rhabdomyoma have variable ultrasound features. The usual favorable outcome can however be complicated by neonatal death (12%), valvular regurgitation and cerebral tuber.

Transcatheter implantation of a bovine valve in pulmonary position: a lamb study.

BACKGROUND: Pulmonary regurgitation can lead to severe right ventricular dysfunction, which is a delicate postoperative problem in the long-term follow-up of patients who had surgery for congenital heart diseases. Clinical conditions of patients suffering from pulmonary valve incompetence are improved by valve replacement with a prosthetic valve. To date, the surgical approach is the only option to replace a pulmonary valve. We report the first experience of percutaneous pulmonary valve implantation. METHODS AND RESULTS: A fresh bovine jugular vein containing a native valve was sutured into a vascular stent and then cross-linked with a 0.6% glutaraldehyde solution for 36 hours. After being hand-crimped onto a balloon catheter, the device was inserted percutaneously according to standard stent-placing techniques. The valved stent was finally deployed in the position of the native pulmonary valve of the lamb. Hemodynamic evaluation was carried out before and 2 months after implantation. Anatomic evaluation was finally performed. Percutaneous pulmonary valve replacement was successful in 5 lambs. No complications were noted. Early and late angiographic and hemodynamic studies confirmed a good position of the stents with a competent valve at the end of the protocol. One stent was slightly stenotic, with macroscopically visible calcifications. CONCLUSIONS: Nonsurgical implantation of pulmonary valves is possible in the lamb. This new technique is similar to standard stent implantation. Thus, it should be feasible in humans, in whom it will lead to a significant reduction of reoperations in patients in need of pulmonary valve replacement.

[Stenosis of the superior caval canal after Mustard and Senning procedures: treatment by dilatation and stent implantation. Two case reports]. / Sténoses du chenal cave supérieur après interventions de Mustard et Senning: traitement par dilatation et implantation d'endoprothèses vasculaires. A propos de deux observations.

The authors report two cases of stenosis of the superior caval canal after Mustard and Senning procedures for transposition of the great arteries in patients paced for atrial arrhythmia. During cardiac catheterisation, it was possible to treat the stenosis by perforation in one case and by balloon dilatation in the second, followed by the implantation of two stents to reestablish vascular patency. In one patient, two pacing catheters were implanted by an endovascular approach without complications three months after stenting; in the other case, epicardial pacing was required because the patient's condition could not wait for endothelialisation of the stent before implanting the pacing catheters. This technique of revascularisation may also be used in children with venous stenosis after implantation of endocavitary pacing catheters, in which the pacing system has to be changed.

[Three-dimensional reconstruction by transesophageal echocardiography of Amplatzer and CardioSEAL prosthetic devices after percutaneous closure and atrial septal defects]. / Reconstruction tridimensionnelle par échocardiographie transoesophagienne des prothèses Amplatzer et CardioSEAL après fermeture percutanée des communications interauriculaires.

OBJECTIVE: The Amplatzer and cardioSEAL devices are currently used for percutaneous closure of atrial septal defects. The in vivo morphology of these devices is unknown and this is why we sought to describe their geometric profile by three-dimensional echocardiography. METHODS: Thirteen patients, aged 6 to 18, underwent transcatheter closure of a secundum type atrial septal defect with either the CardioSEAL (n = 8) or the Amplatzer (n = 5) device. Three-dimensional reconstructions of the defect were obtained from transoesophageal bidimensional echocardiographic views. RESULTS: In the Amplatzer group, the stretched diameter of the defect was larger than in the CardioSEAL group (22 +/- 2 vs 18 +/- 1 mm, p = 0.003), but ehocardiographic surfaces were the same (3.3 +/- 1 vs 3.6 +/- 1 cm2, p = 0.3). The surface of the CardioSEAL device was measured at 4.8 +/- 2 cm2 whereas the Amplatzer's surface was 6.8 +/- 2 cm2 (p = 0.03). The Amplatzer device had a volume of 9 +/- 1 cm3 while the CardioSeal's volume was 3.1 +/- 1 cm3 (p < 0.0001). CONCLUSIONS: The CardioSEAL device occupies a small volume but it covers less of the septal defect and this may result in a higher incidence of residual shunting. The Amplatzer device almost always occludes the septum, but it tends to bulge into the atria and may cause mechanical complications.

[Treatment of obstruction of prosthetic conduct by percutaneous implantation of stents]. / Traitement des obstructions des prothèses tubulaires par implantation percutanée de stents.

Prosthetic conduits, with or without biological valves, are often inserted in surgical procedures to correct or palliate cardiac malformations. The principal problem is degeneration which causes variable degrees of obstruction requiring reoperation for their replacement. The aims of this study were to assess the feasibility, safety and efficacy of a non-surgical method of treating these obstructive prostheses by dilatation-implantation of a metallic vascular endoprosthesis (stenting). Thirteen patients were treated (age range 7.7 to 36 years; mean: 15 years). Eight had pulmonary atresia with a ventricular septal defect corrected by a valved conduit from the right ventricle to the pulmonary artery which became obstructive nearly 10 years later: the implantation of the stent reduced the transconduit pressure gradient in all cases except one who had not undergone closure of the septal defect in which the cyanosis was improved. There are two cases of obstruction of a modified Blalock anastomosis in which the stent revascularised the shunt with improvement in cyanosis. In the final 3 cases, the whole Fontan procedure was compromised by obstruction of a conduit incorporated in the system, and which dilatation with stenting considerably improved. The efficacy of the procedure was constant with no complications other than rupture of the balloon in 3 cases. The good results were maintained for an average of 7.3 months (range 1 to 25 months), but it was necessary to redilate one restenosed stent after 8 months. Dilatation followed by stenting in obstructive cardiovascular prostheses is a simple, safe and effective alternative to surgical reoperation.

[A new anatomical approach to congenital valve diseases by three-dimensional echocardiography]. / Nouvelle approche anatomique des valvulopathies congénitales par l'échocardiographie tridimensionnelle.

Congenital valvular heart disease in childhood is often complex. Conventional echocardiography provides two-dimensional views which require mental reconstruction for three-dimensional assessment. This problem may be solved by the use of three-dimensional (3D) echocardiography which obtains images of valves comparable to those seen at surgery. This was confirmed by 4 cases of congenital valvular heart disease studied by 3D echocardiography: stenotic bicuspid aortic valve disease, parachute mitral valve and two cases of mitral regurgitation in patients with atrioventricular canal. The 3D views of the aortic valve showed the commissural opening after percutaneous balloon valvuloplasty of the bicuspid valve. The surface of the aortic orifice and the surface of the two mitral leaflets were measured from 3D reconstructions. The longitudinal 3D view analysed the extension of the single obstructive mitral papillary muscle of the parachute malformation. The 3D ventricular views allowed assessment of the extension of the cleft and the surface of the three mitral leaflets of the 2 cases of atrioventricular canal. In one of these cases, the results of surgical valvuloplasty were evaluated after 3D reconstruction of the valve. 3D echocardiography is not only a diagnostic tool for congenital heart disease but also a very useful complementary investigation for accurate evaluation of congenital valvular lesions to optimise possible valve repair.

[Percutaneous closure of patent ductus arteriosus with the Amplatzer duct occluder]. / Fermeture percutanée du canal artériel par la prothèse occlusive Amplatzer.

BACKGROUND AND METHODS: Percutaneous closure of a moderate-to-large sized patent ductus arteriosus using multiple coils or a Rashkind double-umbrella may be technically demanding and raises a significant rate of embolization and residual shunting. This is why we tried a new self-expandable device called Amplatzer Duct Occluder to close transvenously large ductuses in eighteen patients at a median age of 2.8 years (range: 0.7 to 34). They had a patent ductus arteriosus with a minimal diameter > 3 mm (mean: 5 +/- 2 mm). The device had a mean diameter of 9 +/- 2 mm and was delivered through a 6F venous sheath. Aortography was done 5 to 10 minutes after the release and follow-up evaluation was performed with color Doppler echocardiography within 24 h, 1 and 3 to 6 months after the procedure. RESULTS: Placement was successful in all but the first patient (95%). Complete closure was achieved immediately in 4/17 patients (24%) and in 13/17 (76%) 24 h later. Two patients were lost for follow-up. Complete closure was confirmed in 14 of the 15 (93%) reviewed patients at 1 and 3 months. In all patients with a ductal diameter < or = 7.5 mm the closure was complete at 1 month. Embolization occurred in one patient with a 9.7 mm duct and surgery was undertaken. A mild and transient hematuria was observed in one patient. No pulmonary artery stenosis nor aortic obstruction were observed on follow-up. CONCLUSION: The Amplatzer Duct Occluder device offers a safe and effective option to treat patients with a ductus arteriosus which minimal diameter is up to 7.5 mm.

[Pseudo-inherited form of left heart obstructive defects revealing maternal phenylketonuria]. / Forme pseudo-héréditaire d'anomalies obstructives du coeur gauche révélatrice de phénylcétonurie maternelle.

If an adequate diet is not given to mothers with phenylketonuria, their offsprings often exhibit intra-uterine growth retardation with associated microcephaly and various malformations. Here, we report two families in whom we observed recurrent left heart malformations associated with microcephaly masquerading as a mendelian condition and revealing a maternal phenylketonuria. These observations suggest that when confronted to recurrent heart malformations with extra-cardiac defects that are not due either to an inherited chromosomal anomaly or to a well characterized mendelian disease, a maternal teratogen should be identified and more particularly maternal hyperphenylalaninemia if an intra-uterine growth retardation or a microcephaly is part of the syndrome.

[Baloon angioplasty with stent implantation in recoarctation of the aorta: an attractive alternative]. / Dilatation percutanée suivie d'implantation de stent dans les recoarctations de l'aorte: une alternative séduisante.

The results of transcatheter balloon angioplasty in teenagers and adults with aortic recoarctation are uncertain. Therefore, there is a current trend to prefer a more complex procedure including the implantation of a stent. This study deals with 8 patients aged 7 to 25.3 years (median: 15 years), weighing 20 to 68 kg. (median: 57) and having undergone resection of an aortic coarctation during infancy (24 days to 4 years). All had their lower limb pulses diminished or abolished, elevated blood pressure at rest (and at exercise in the 5 tested patients), and left ventricular hypertrophy. MRI documented the lesion and helped to select seven patients whose stenosis was short and remote enough from the origin of the main aortic collateral. In one case, the decision to stent was taken as an emergent measure to treat an aortic dissection which appeared shortly after balloon dilatation. The effectiveness of the procedure was immediate in all patients with a 50% increase in diameter of the dilated area, total relief of the gradient, drop to normal values of the blood pressure. These good results persisted at follow-up (3-24 months) in 6 patients, with moderate hypertensive rebounds in the last 2. There were 2 technical problems (premature burst of the balloon, asymmetrical inflation of the stent like an "Eiffel Tower") that could finally be overcome and should no longer occur with the new specially designed so-called "BIB" balloons. Would long term follow-up confirm these early results, one should conclude that this method offers an attractive, safe and effective option to surgery for adolescents and adults with late recoarctation of the aorta.

[Quality of life of adults with univentricular hearts]. / Qualité de vie des adultes atteints de coeur univentriculaire.

This study sought to evaluate the quality of life of surviving adults with univentricular heart and to determine the impact of sociodemographic and clinical characteristics on quality of life. The health records of 89 survivors with univentricular heart living in France at the time of the study were reviewed. They were 17 to 49 years old (median: 21). Sixty-seven patients answered the Duke questionnaire. Sociodemographic and clinical variables were similar in the responders and non-responders. The scores of patients who answered the questionnaire were compared with national norms. The impact of sociodemographic and clinical variables on individual Duke's measures was assessed. The Duke scores of adults with univentricular heart were similar to those of the normal population. Cyanosis predicted a worse score for physical (p = .05) and perceived health measures (p = .02). The higher educational level predicted a better score for physical (p .004), mental (p = .01), and general health measures (p = .02). Orthopaedic problems worsened social score (p = .05). Psychosocial problems worsened pain score (p = .04). Mitral atresia, in comparison with the other anatomical types, worsened perceived health score (p = .02). Finally, patients younger than 23 years scored better for almost all of health and dysfunction measures. In conclusion, despite repeated interventions and other disease-related everyday stresses, a selected group of adults with univentricular heart had a satisfying quality of life. These patients probably developed copying mechanisms, easing the psychological stress, and oriented themselves towards a different set of values in everyday life so that to accept disabilities and to recalibrate personal expectations.

New insight into left ventricular function in tricuspid atresia after total cavopulmonary connection: a three-dimensional echocardiographic study.

BACKGROUND: In patients with tricuspid atresia palliated by construction of a total cavopulmonary connection, both pulmonary and systemic circulations depend on the performance of the dominant left ventricle. When estimating the volume of such ventricles using cross-sectional echocardiography, it is necessary to make assumptions concerning the geometry of the ventricular shape. This is avoided by three-dimensional echocardiography, which provides direct volumetric data. Our purpose was to apply this new method to quantify left ventricular volumes and function in patients with tricuspid atresia after construction of a total cavopulmonary connection. METHODS: We studied ten patients (median age: 8 years) with tricuspid atresia who had undergone a total cavopulmonary connection, comparing them with 10 normal children matched for age, sex and size. The three-dimensional echocardiography was performed with electrocardiographic and respiratory gating. A new transthoracic integrated probe designed for small children was used with a rotational scanning increment of 3 degrees. The 60 slices obtained from the ventricular cavity were stored and formatted in a commercial system (TomTec). End-diastolic and end-systolic volumes, stroke volume and ejection fraction were calculated after manual tracing of the endocardial surfaces. The volumes were indexed to the body surface area. RESULTS: As seen in the reconstructions, the dominant left ventricle in tricuspid atresia had a spherical shape, whereas the normal left ventricle is oblong. The left ventricular volumes and function in tricuspid atresia were 54+/-4 ml/m2 (end-diastolic volume), 28+/-3 ml/m2 (end-systolic volume), 26+/-7 ml/m2 (stroke volume) and 48+/-6% (ejection fraction). These volumes were not different from those obtained in the controls (p = NS). The left ventricular stroke volume and ejection fraction in 10 patients with tricuspid atresia were lower than those calculated for the controls (p < 0.05). CONCLUSIONS: Three-dimensional echocardiography provides a quantitative insight into the pathophysiologic function of the dominant left ventricle in tricuspid atresia after construction of a total cavopulmonary connection.

Selective coronary angiography in patients younger than 1 year of age.

Precise assessment of coronary artery anatomy is needed in congenital coronary malformations and acquired coronary obstruction in children. The aim of the study was to describe our experience of selective coronary angiography (SCA) in newborns and infants. One hundred and three patients younger than 1 year of age underwent SCA. Indications were preoperative screening for coronary artery lesions or anomalous pattern (41 patients), signs of myocardial ischemia (43 patients), surgical difficulties during the reimplantation of the coronary arteries without signs of ischemia (15 patients), and other indications (4 patients). Success rate of selective injection was 100% with no significant complication. In the presence of clinical suspicion of myocardial ischemia, abnormalities of coronary artery pattern was found in 17 of 43 patients. Precious information for the surgical management was obtained in 13 of 41 patients. SCA can be performed safely even in newborns and small infants. Skills in catheterization and knowledge about the coronary artery anatomy are needed to perform SCA.

Influence of atrial septal defect anatomy in patient selection and assessment of closure with the Cardioseal device; a three-dimensional transoesophageal echocardiographic reconstruction.

BACKGROUND: The maximal diameter of the defect and the dimensions of the septal rims are essential parameters for the selection of optimal cases for device closure. Neither two-dimensional echocardiography nor balloon catheter sizing provide optimal data. Unique three-dimensional echocardiography might help to improve patient selection and assessment of results. Our aim was to optimize transcatheter closure of secundum type atrial septal defects using three-dimensional echocardiography. METHODS: Sixteen patients enrolled in a protocol for atrial septal defect transcatheter closure with the Cardioseal device underwent transoesophageal two- and three-dimensional echocardiography. Maximal diameter and tissue rim of the atrial septal defect were measured and compared by both methods. In the 12 patients selected for closure, the balloon stretched diameter was compared to three-dimensional echocardiography measurements. Device placement was assessed by two- and three-dimensional echocardiography. RESULTS: The shape of the atrial septal defect appeared variable on three-dimensional views: round in nine patients but complex (oval, raquet-shaped, multiple) in seven patients. The surface area of the atrial septal defect varied by 68+/-15% during the cardiac cycle. The correlation between atrial septal defect maximal diameters measured by two-dimensional transoesophageal echocardiography and three-dimensional echocardiography was better in round defects (y=1 x +1.6, r=0.99) than in complex defects (y=0.7 x -0.5, r=0.88). The antero-superior rim could only be properly assessed by three-dimensional echocardiography. In 12 patients the correlation between stretched diameter and three-dimensional echocardiography maximal diameter was poor (y=0.3 x +13, r=0.41). After placement of the device, three-dimensional echocardiography enabled the mechanism of residual shunting to be understood in three patients. CONCLUSIONS: Dynamic three-dimensional echocardiography enhances the understanding of the anatomy and physiology of atrial septal defect and should be an important process in future initiatives for device closures.

Three-dimensional echocardiographic measurement of left ventricular stroke volume in children: comparison with Doppler method.

Three-dimensional echocardiography (3DE) allows calculation of ventricular volumes without geometric assumption on the ventricular shape. Our aim was to apply 3DE in a normal pediatric population and to compare the left ventricular stroke volume measurements to the Doppler method. Twenty-four normal patients (median age 7 years) underwent Doppler echocardiography and 3DE for left ventricular stroke volume calculation. The left ventricular stroke volume by Doppler method was calculated as the product of the aortic Doppler flow mean velocity and the area of the aortic annulus. The 3DE method was performed using a transthoracic rotational probe (TomTec) and left ventricular volumes were calculated using the Simpson's rule. The mean time for 3DE acquisition was 90 seconds without any sedation. 3DE correlated well with the Doppler method for left ventricular stroke volume measurements (y = 0.8x - 0.2, r = 0.94). The mean difference between the average values of left ventricular stroke volume obtained by Doppler method and 3DE was 5 +/- 4 ml. Intraobserver and interobserver variabilities in the left ventricular stroke volume measurement by 3DE were 2.6% and 4.4%. In conclusion, 3DE compared to the Doppler method is an accurate, noninvasive, and reproducible method to measure the left ventricular stroke volume in normal children.

Assessment of the geometric profile of the Amplatzer and Cardioseal septal occluders by three dimensional echocardiography.

OBJECTIVE: To apply three dimensional echocardiography to describe the geometric profile of the Amplatzer and Cardioseal occluders after deployment for closure of atrial septal defect. METHODS: 20 patients (mean (SD) age, 14 (5) years) were enrolled for transcatheter closure of a secundum atrial septal defect with the Amplatzer occluder (10) or with the Cardioseal occluder (10). The two populations were matched for the stretched diameter of the defect (mean 18 (6) mm). The profile of the two occluders was examined. RESULTS: Transoesophageal echocardiography did not show any residual shunts after Amplatzer occluder deployment, whereas three patients had a small residual leak after Cardioseal deployment. One patient had transient atrioventricular block with the Amplatzer device. The mean surface area of the Amplatzer occluder was 6.9 (2) cm(2), and that of the Cardioseal device 5.4 (3) cm(2) (p = 0.03). The mean volume of the Amplatzer occluder was 9.2 (1) cm(3), while that of the Cardioseal occluder was 3.5 (1) cm(3) (p < 0.0001). From the three dimensional views, the Cardioseal occluder looked like a flat square after deployment whereas the Amplatzer occluder took up a ball shape in the atrial cavity. CONCLUSIONS: Three dimensional views by multiplane transoesophageal echocardiography allow a realistic in vivo description of atrial septal occluders. The Amplatzer occluder, with its high geometric profile, allows complete closure of large atrial septal defects but with some risk of mechanical complications. Use of the Cardioseal device, with its small surface coverage and high residual shunt rate, should be limited to transcatheter closure of a patent foramen ovale or small atrial septal defects.

Prenatal detection of a tetralogy of Fallot with origin of the left pulmonary artery from the ascending aorta in a familial 22q11 microdeletion.

Here we report a case of prenatal diagnosis of anomalous origin of the left pulmonary artery from the ascending aorta associated with a tetralogy of Fallot in a familial form of 22q11 deletion. The mother, who had a normal heart and a velo-cardio-facial syndrome, had a first child with a pulmonary atresia plus ventricular septal defect associated with a 22q11 deletion. Prenatal diagnosis during the second pregnancy identified the above-described cono-truncal anomaly and FISH study showed a recurrent 22q11 deletion. This case illustrates the intrafamilial variability of cardiac involvement in 22q11 deletion as well as the possibility of diagnosing complex cono-truncal malformations during fetal life.