[Justice and equity as determinants of medical decision-making]. / Justice et équité dans la décision médicale.

Every medical decision-making is to do justice to the one who suffers and who considers his pain as nonsensical. This requirement falls into 3 universal ethical principles, governing the patient-doctor relationship: the autonomy of the person even if it is a young child, beneficence and non-maleficence. Adhering to these principles gives medical decision-making its ethical dimension. It implies that the doctor makes the best use of the emotions that he feels in front of his patient and/or in front of his relatives: respect for autonomy, the beneficent compassion and the fear of maleficence in diagnosis and care. If a paediatrician combines these 3 affects harmoniously, his attitude is in keeping with the requirement of justice, which is the quintessence of Ethics. However, reality is often more complex, a source of conflicting emotions and, in fine, a source of an anguish whose benefit is yet to alert on the necessity to carry on looking for the right decision-making: the emotional revision consists of a meta-analysis of the objective and subjective data of the problem. It preciously helps to establish a compromise of justice. Eventually, in order to be just, the doctor must include the concern of equity amongst the criteria of his decision-making, in other words a fair allocation of the goods and of the care services. Unfortunately, this concern is undermined by geopolitical, socio-economic and cultural factors, which vary greatly according to the environmental conditions that might mar what should be optimal ethical decisionmaking. The doctor cannot solve these problems on his own, but he has to know them in order to deal with them.

[Intractable neonatal heart diseases: a controversy]. / Cardiopathies néonatales intraitables: une controverse.

A congenital heart disease is said intractable when the only options are either heart transplantation or a complex palliative programme including several interventions leading to a mid-term Fontan-type circulation. The latter uses the single ventricle as a systemic one while the pulmonary circuit is a non-pulsed one which by-passes the absent right ventricle through a cavo-pulmonary anastomosis. Both of these are difficult to operate and have hazardous long term results. This is why prenatal detection of such lesions usually leads to termination of pregnancy. Yet, postnatally, a serious ethical issue emerges, opposing the above-mentioned options to a third way: abstention. Information delivered to the parents plays here a crucial role in decision making and everybody agrees for the need of the greatest objectivity. But to achieve such a wish is terribly challenging since many socio-cultural factors may puzzle the way to deliver and to receive the main messages. This can easily be demonstrated by the well-known instance of hypoplastic left heart syndrome.

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.

[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.

[Interest in myocardial scintigraphy following the arterial switch procedure for transposition of the great vessels]. / Intérêt de la tomoscintigraphie myocardique au décours de la correction artérielle des transpositions des gros vaisseaux.

Coronary artery obstruction is the main late complication of the so-called arterial switch operation designed to repair transpositions of the great arteries in newborn infants by switching the great vessels and transferring the coronary ostia onto the posterior vessel. Our aim was to study the links between myocardial perfusion and coronary artery anatomy after the arterial switch operation. Forty-five patients (5.863 years) underwent a 201Tl myocardial SPECT and a selective coronary artery angiography. The latter was normal in 20 children: 13 had also a normal myocardial scan but 7 had myocardial perfusion defects including 2 with angina who had a very low coronary reserve at positron emission tomography. Twenty-five patients had severe coronary artery lesions: 5 with a normal myocardial scan and 20 with perfusion defects. Twelve out of these 20 underwent surgical revascularization and the SPECT images went back to normal in all within 6 months after surgery. Specificity and sensitivity of myocardial SPECT in detecting coronary artery lesions were 78% and 69% whereas positive and negative predictive values were 74 and 73%. We conclude that myocardial SPECT imaging is not the right way to detect late post arterial switch coronary artery lesions. It is helpful in decision making as to submit these children to surgical revascularization and in assessing its postoperative effectiveness.

[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.

[A rare cause of cardiomyopathy in childhood: propionic acidosis. Three case reports]. / Une cause rare de cardiomyopathie de l'enfant: l'acidémie propionique. A propos de 3 cas.

Dilated cardiomyopathy is a rare and unrecognised complication of propionic acidosis. It usually progresses to chronic cardiac failure with a poor prognosis. The authors report three cases of cardiomyopathy due to this condition. One of the children underwent liver transplantation which cured the cardiac complication.

Quality of life and perceived health status in surviving adults with univentricular heart.

OBJECTIVE: To evaluate the quality of life in patients with univentricular heart and to determine the impact of sociodemographic and clinical characteristics. DESIGN AND SETTING: Retrospective, cross sectional study conducted in a regional paediatric cardiology centre. PATIENTS: The health records of 89 survivors with univentricular heart (median age 21 years; range 17-49 years) were reviewed. Sixty seven answered the Duke questionnaire. Sociodemographic and clinical variables were similar in the responders and non-responders. The impact of sociodemographic and clinical variables on individual Duke's measures was assessed. RESULTS: The Duke scores of adults with univentricular heart were similar to the normal population. Cyanosis predicted a worse score for physical (p = 0.05) and perceived health measures (p = 0.02). A higher educational level predicted a better score for physical (p = 0.004), mental (p = 0.01), and general health measures (p = 0.02). Orthopaedic problems worsened the social score (p = 0.05). Psychosocial problems worsened the pain score (p = 0.04). In comparison with the other anatomical types, mitral atresia worsened the perceived health score (p = 0.02). Patients younger than 23 years scored better for almost all health and dysfunction measures. CONCLUSIONS: Despite repeated interventions and other disease related everyday stresses, a selected group of adults with univentricular heart had a satisfying quality of life.

Impaired cardiac adrenergic innervation assessed by MIBG imaging as a predictor of treatment response in childhood dilated cardiomyopathy.

OBJECTIVE: To evaluate the prognostic value of metaiodobenzylguanidine (MIBG) imaging in childhood cardiomyopathy. DESIGN: Prospective cohort study. SETTING: Tertiary referral centre. PATIENTS: 40 children (21 boys, 19 girls; mean (SD) age, 7.0 (5.6) years) with heart failure resulting from idiopathic dilated cardiomyopathy (n = 23) or various other disorders (n = 17). METHODS: At the initial examination, cardiac (123)I-MIBG uptake and release, circulating noradrenaline (norepinephrine) concentration, x ray cardiothoracic ratio, and echocardiographic variables were recorded. Cardiac MIBG uptake was obtained by measuring the heart to mediastinum activity ratio on the planar image obtained four hours after MIBG injection. MIBG washout rate was evaluated using relative decrease in cardiac activity measured at 20 minutes and four hours. Patients were treated with angiotensin converting enzyme inhibitors, diuretics, and digitalis, and were followed up for 12 (10) months. Fifteen patients did not respond to medical treatment (12 heart transplants; three deaths), and 25 did respond (improved or stable). RESULTS: Cardiac MIBG uptake was positively correlated with x ray cardiothoracic index (r = 0.55, p = 0.0008) and echocardiographic left ventricular fractional shortening (r = 0.68, p < 0.0001). Among all the clinical and laboratory variables tested, multivariate discriminant analysis showed that the only independent predictor of an unfavourable outcome was a low MIBG uptake (p < 0.001). Survival curves had a mean threshold value of 1.54 for MIBG uptake. CONCLUSIONS: Impaired cardiac adrenergic innervation is strongly related to adverse outcome in children with dilated cardiomyopathy, independently of the aetiology. MIBG imaging may help to stratify risk in such patients.

[The best in 2000 on pediatric cardiology]. / L'essentiel de 2000 en cardiologie pédiatrique.

The year 2000 was rich in events, either spectacular news or confirmed improvement of on-going advances, as far as paediatric cardiology is concerned. The selection presented by the authors includes the first percutaneous implantation in a human being of a biological (bovine) valve which was sewn on a stent, compressed into a catheter and inserted against a stenotic and leaking procine bioprosthesis in a right-ventricle to pulmonary-artery conduit. This may be a new way to further valve replacements as alternatives to surgery. Balloon dilation of late postoperative recoarctations is now also improved with the use of stents able to maintain the result and to avoid traumatic injuries, with new coaxial double balloons making the procedure easier and safer. This is probably one of the main elements in reducing this very particular form of hypertension, the anatomic cause of which is often difficult to understand. As for yesterday's daring innovations now becoming near-routine protocols, two examples are developed. First, the rehabilitation of pulmonary arteries in pulmonary atresia with ventricular septal defect and complex pulmonary blood supply, both by true pulmonary vessels and by collaterals, both being stenotic and/or hypoplastic, anastomosed or not. The anatomic and functional details of such a vascular setting should be accurately understood and treated by early and aggressive surgery and interventional procedures in order to promote antegrade flow, distal angiogenesis, and, finally, active and harmonious vascular growth compatible with complete repair. The second example is Friedreich's ataxia in which, within 3 years of the discovery of the pathogenic mechanism, the deficiency in frataxin and its intra-cellular toxic consequences have been demonstrated, leading to a logical medical therapy which proves to be effective in treating (and maybe in preventing) the severe hypertrophic cardiomyopathy associated to this disease.

[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.

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.

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.