Complete and incomplete Kawasaki disease: Clinical differences and coronary artery outcome from a national prospective surveillance study in Switzerland.

Introduction: The aim of this national prospective surveillance study was to compare the clinical presentation, laboratory findings, treatment, and coronary artery outcome in patients with incomplete and complete Kawasaki disease (KD). Methods: Between March 2013 and February 2019, children with a diagnosis of complete and incomplete KD were reported by the Swiss Paediatric Surveillance Unit and prospectively enrolled. Clinical data, laboratory values, treatment, and echocardiographic features were collected at diagnosis and 1 year of follow-up. Data were compared between children with complete or incomplete KD. Results: A total of 351 questionnaires were registered from children with a diagnosis of KD. Of them, 219 (62.4%) children had complete KD, and 132 (37.6%) children had incomplete KD. Children with incomplete KD were younger and had a longer-lasting fever; however, there were no differences in the level of C-reactive protein. All but four children received intravenous immunoglobulin treatment, whereas 14% of children were treated with corticosteroids. Children with incomplete KD were more often treated with corticosteroids than children with incomplete KD (p = 0.01). At diagnosis, 39 (11.1%) patients had only coronary artery dilation and 57 (16.2%) had at least one coronary artery aneurysm. There were no differences in coronary artery involvement between the two groups. At follow-up, 273 of 294 (92.8%) patients had no coronary artery involvement, with no difference between the two groups (p = 0.609). The overall incidence of coronary artery aneurysms at diagnosis was 16.2%. At follow-up, most coronary artery aneurysms had regressed, and coronary artery aneurysms were present in only 5.8% of the patients. Coronary artery aneurysms were slightly more frequent in patients with incomplete KD at follow-up (p = 0.039) but not at diagnosis (p = 0.208). Conclusion: Although the clinical presentation in children with incomplete and complete KD differs, the absence of coronary artery involvement does not. The use of corticosteroids appears to be preventive against the development of coronary artery aneurysms in these patients. However, the results of this study suggest a lower rate of coronary artery aneurysm regression in patients with incomplete KD. Further studies on a larger scale are needed to assess the risk of non-regression of coronary artery aneurysms in this particular group of patients.

Association of hemoglobin and spontaneous closure of the ductus arteriosus during the transitional period in very low birth weight infants.

OBJECTIVES: To determine the association between plasma hemoglobin (HB) at three time-points (birth, postnatal days 0-3 and 0-10) and spontaneous closure of the ductus arteriosus (sDAC). STUDY DESIGN: A retrospective case-control study of preterm infants born (2013-2016) between 24 and 29 weeks of gestational age (GA) was conducted in a level three perinatal center in Switzerland. We collected hemoglobin at birth, between days 0-3 and 0-10 in two distinct groups: (i) patients treated for a PDA and (ii) patients with spontaneous closure of the ductus arteriosus (sDAC). Antenatal and postnatal demographic data and neonatal morbidity were collected. Bivariate analysis was performed and a stepwise logistic regression was done to investigate factors associated with sDAC. RESULTS: We reviewed the medical chart of 184 premature infants of whom 146 (79.3%) satisfied eligibility criteria. Of these, 74 (51%) were classified as sDAC. Patients with sDAC were older (GA: 28 vs 27, p < 0.001), more stable (clinical risk index for babies score (CRIB score): 2 vs 5, p < 0.001) and had better clinical outcomes than patients who received treatment for a PDA. Infants in the sDAC group had a higher level of hemoglobin during the first ten postnatal days. Multiple logistic regression analysis revealed that lower HB level (day 0-10) were associated with failure of sDAC (p < 0.05). CONCLUSIONS: This is one of the first studies to highlight a potential association between hemoglobin during the transitional period and sDAC. The biological nature of this observation requires prospective clarification.

[Congenital heart disease: from childhood to adulthood]. / Cardiopathie congénitale: de l'enfance à l'âge adulte.

With the improvement of congenital heart surgery, most children with congenital heart disease will survive into adulthood with a good quality of life. Regular cardiac follow-up is recommended for all patients. The adolescent period coincides often with medium and long term consequences and complications and repeat surgery or catheter interventions might be needed. It is therefore of prime importance to begin the transition process early and to pursue it well into adulthood. We have elaborated a formal transition program adapted to youngsters with congenital heart disease.

[A cause of dilated cardiomyopathy in a child: primary carnitine deficiency]. / Une cause de cardiomyopathie dilatée chez l'enfant: le déficit primaire en carnitine.

AIM: The aim of this case report was to show the importance to research metabolic etiology, especially a carnitine deficiency in dilated cardiomyopathy of children. CASE REPORT: A three years old Togolese child presented muscular hypotonia, dyspnea. Examination showed left galop murmur and systolic murmur 2/6. Chest X-ray showed cardiomegaly (CTI: 0.66), electrocardiogram, a sinusal rythm, left ventricle hypertrophy and T wave abnormalities. Echocardiogram showed a markedly dilated left ventricle with reduced systolic function (EF: 0.43; reference range 0.55-0.80) and moderate mitral regurgitation. The inflammatory signs where negatives. Magnetic resonance imaging don't show signs of ischemic or myocarditis. The levels of free and total plasmatic carnitine decreased: 3µmol/L (N: 18-48µmol/L) and 5µmol/l (N: 29-70µmol/L) respectively. Mutation analysis of the gene SLC22A5 confirms the diagnosis of primary systemic carnitine deficiency. Treatment with oral carnitine was started at 200mg/kg per day. Within three weeks of treatment, we observed the decrease of all symptoms and the left ventricular size and function normalized (EF: 0.62). He has now been on oral carnitine for live. CONCLUSION: Primary carnitine deficiency is a cause of dilated cardiomyopathy in child. It must systematically be suspected when a child presents a primitive cardiomyopathy. The treatment with oral carnitine for live is simple, with excellent prognosis.

[A rare cause of arterial hypertension in children: Takayasu arteritis]. / Une cause exceptionnelle d'hypertension artérielle de l'enfant : l'artérite de Takayasu.

Takayasu arteritis, a nonspecific inflammatory arteritis, is particularly rare in children. We report the case of a 6-year-old girl presenting with severe arterial hypertension in the upper segment associated with an inflammatory syndrome. Investigations showed coarctation of the abdominal aorta at different levels, due to Takayasu arteritis. The patient was treated with percutaneous dilatation and stent implantation as well as prolonged anti-inflammatory therapy. Arterial hypertension in children needs to be investigated until its cause, which may be rare, such as Takaysu arteritis, is determined.

[Hybrid procedures in congenital heart disease]. / Interventions hybrides et malformations cardiaques congénitales.

Increasing complexity in management of congenital heart disease imposes more frequent surgeries and interventions. Each technique has its own limitations, which could impair the anticipated result. Hybrid procedures join the advantages of cardiac surgery and interventions, creating a synergy in the management of these patients with cardiac anomalies. In our experience, hybrid procedures shorten cardiopulmonary bypass, reduce morbidity of surgery and reduce duration of stay in the intensive care unit. For some complex congenital heart diseases for which there are no ideal surgical or interventional options, hybrid procedures are becoming increasingly important in their management. Finally hybrid procedures allow surgeons and cardiologist to achieve complex procedures that could not be possible in another way.

[Stents valves for percutaneous valve replacement]. / Remplacement valvulaire percutané au moyen de stent-valves--une nouvelle approche thérapeutique.

Stents have a long history in traditional valve surgery as both, porcine biological valves as well as pericardial valves are mounted on stents prior to implantation. Recently stent-mounted biological devices have been compressed up to the point, where they can be passed through a catheter. Various routes can be distinguished for implantation: open access, the trans-vascular route in antegrade or retrograde fashion, as well as direct trans-apical or trans-atrial access. Direct access has the potentialforvideo-endoscopic valve replacement. In theory, as well as in the experimental setting, valved stents have been implanted in tricuspid and caval position respectively, as well as in pulmonary, mitral and aortic locations. The largest clinical experience has been achieved in pulmonary position whereas current efforts target the aortic position.

[Pediatrics]. / Pédiatrie.

This article summarizes the medical progress achieved in 2 frequent and 2 rare pathologies: 1. Cryptorchidism should be operated around 12 months of age and hormonal treatment abandoned in order to maintain fertility and avoid development of testicular tumors. 2. For the treatment of streptococcal pharyngitis oral cephalosporins for 4 to 5 days are equivalent to a Penicillin treatment of 10 days. 3. Thanks to carvedilol (a beta-blocker agent), levosimendan (a calcium sensibiliser) and nesiritide (an analog to the natriuretic peptide) a new hormonal approach to cardiac failure is possible. 4. Corticosteroids allow to improve quality of live and life expectancy in Duchenne muscular dystrophy, provided treatment starts early and a multidisciplinary approach is assured.

[Transposition of the great arteries: long-term follow-up]. / Transposition des gros vaisseaux: suivi a long-terme.

The management of transposition of the great arteries has changed importantly over the last decades. New techniques are employed for the diagnosis and surgical intervention has improved. This has lead to an increasing number of long-term survivors, who require specialised and focussed follow-up, depending on their type of surgical repair Mustard/Senning of arterial Switch. The long-term problems vary for each type of repair and require a specific approach, pharmacotherapy, catheter intervention or surgery. These complex sequellae implicate that transposition patients are never completely cured and probably will have a limited life span.

[Treatment of congential heart disease with interventional catheterization]. / Traitement des cardiopathies congénitates par cathétérisme interventionnel.

The development in pediatric interventional cardiology has changed the therapeutic strategy for many patients with congenital heart disease. Interventional catheterization became the first choice for valvuloplasty, angioplasty and collateral vessel occlusion. Effective and safe transcatheter interventions exist for closure of atrial or ventricular septal defects and for patent ductus arteriosus. The progress in the technology used in the catheterization laboratory will permit to continue the expansion of the range of interventions performed without surgery. Pediatric cardiologists and congenital heart surgeons must understand each other's interventional techniques and how they can be used in a coordinated fashion. This interaction is essential for the optimal management of patients with both simple and complex congenital heart disease.

[Pediatrics. Advantages of prenatal diagnosis in congenital cardiopathies]. / Pédiatrie. Avantages du diagnostic prénatal dans les cardiopathies congénitales.

The diagnosis of a congenital heart disease can be made by fetal ultrasound as soon as the 12th week of pregnancy. The main indications for fetal echocardiography are maternal and fetal. Sequential approach is necessary to precise the anatomy and to elaborate a therapeutic strategy for that patient. For severe heart lesions delivery and postnatal care can be programmed, avoiding thus severe hypoxia and acidosis after birth. The prognosis of these children are thus much improved.

Surgery for aortic coarctation: a 30 years experience.

OBJECTIVE: A retrospective study to review the experience of a single center with surgery for aortic coarctation over a period of 30 years (1970-1999). METHODS: Criteria for inclusion: (a) aortic coarctation, isolated or associated with congenital heart defect; (b) surgery between 1970 and 1999. Data recorded: (1) date of surgery; (2) age at surgery; (3) associated lesions; (4) surgical technique; (5) simultaneous surgical procedures; (6) early and late surgical results in term of: (a) deaths; (b) need for reoperation because of re-coarctation or other cardiac lesion; (c) residual/recurrent pressure gradient, evaluated at cuff/Doppler at rest; (d) systemic hypertension, requiring medical treatment. RESULTS: One hundred and forty-one patients underwent surgery for aortic coarctation: 30 neonates, 29 infants, 45 children and 37 adults. Associated lesions were found in 8/37 (=21.6%) adults and in 73/104 (=70.1%) pediatric patients. There were no hospital deaths. During the follow-up there were one late death in the adults group (1/37=2.7%) and three late deaths in the pediatric group (3/104=2.9%), all unrelated with aortic coarctation. Re-operation because of re-coarctation occurred only in ten late survivors of the pediatric group (10/101=9.9%), 9/10 operated on before 1980 (P<0.00001). End-to-end anastomosis, enlarged to the aortic arch in neonates, was associated with the lowest incidence of re-coarctation (P<0.005). A significant (>20 mmHg at rest) pressure gradient was found in none of the adults, and in seven of the 91 pediatric patients (7/91=7.7%) late survivors. Three adults (3/36=8.3%) late survivors are on medical treatment to control systemic hypertension. CONCLUSIONS: The long-term results of our retrospective study confirm that surgery has to be considered the gold standard for the treatment of aortic coarctation. The interventional angioplasty techniques have to provide long-term outcome at least similar to the results obtained with surgery.

[Mitral valve prolapse and type II mucopolysaccharidosis. Report of two familial cases]. / Prolapsus mitral et mucopolysaccharidose type II. Observation de deux cas familiaux.

Type II mucopolysaccharidosis (Hunter's disease) is a hereditary condition due to a deficit of a lysosome specific hydrolase (iduronate sulfatase) inducing an accumulation of dermatane-sulphate and heparane-sulphate in certain organs. Cardiac involvement is constant in this disease and manifests itself essentially by aortic valve stenosis and insufficiency and/or mitral insufficiency which is progressive, irreversible and life-threatening. Two brothers of Portuguese nationality suffering from a so-called slight form of this disease had classical mucopolysaccharide infiltration of their aortic valves. The elder brother, aged 11, had severe aortic insufficiency associated with mild stenosis requiring treatment with vasodilator drugs. The younger, aged 8, had asymptomatic mild aortic regurgitation. Curiously, mitral valve prolapse with regurgitation was present in both children. The association of mitral valve prolapse and type II mucopolysaccharidosis, without other typical cardiac involvement, has only been reported once in the literature.

Non-invasive imaging of the ring-sling complex in children.

The Ring-Sling Complex is an uncommon, congenital vascular and tracheobronchial malformation with a persistent high death rate. We report three patients in whom computed tomography (CT) and magnetic resonance imaging (MRI) were used for the preoperative diagnosis and for staging of the morphologic tracheal and vascular anomalies.

Treatment of congenital aortic valve stenosis: impact of the Ross operation.

GOAL: To evaluate the impact of the Ross operation, recently (1997) introduced in our unit, for the treatment of patients with congenital aortic valve stenosis. METHODS: The period from January 1997 to December 2000 was compared with the previous 5 years (1992-96). Thirty-seven children (< 16 yrs) and 49 young adults (16-50 yrs) with congenital aortic valve stenosis underwent one of these treatments: percutaneous balloon dilatation (PBD), aortic valve commissurotomy, aortic valve replacement and the Ross operation. The Ross operation was performed in 16 patients, mean age 24.5 yrs (range 9-46 yrs) with a bicuspid stenotic aortic valve, 7/10 adults with calcifications, 2/10 adults with previous aortic valve commissurotomy, 4/6 children with aortic regurgitation following PBD, and 1/6 children who had had a previous aortic valve replacement with a prosthetic valve and aortic root enlargement. RESULTS: PBD was followed by death in two neonates (fibroelastosis); all other children survived PBD. Although there were no deaths, PBD in adults was recently abandoned, owing to unfavourable results. Aortic valve commissurotomy showed good results in children (no deaths). Aortic valve replacement, although associated with good results (no deaths), has been recently abandoned in children in favour of the Ross operation. Over a mean follow-up of 16 months (2-40 months) all patients are asymptomatic following Ross operation, with no echocardiographic evidence of aortic valve regurgitation in 10/16 patients and with trivial regurgitation in 6/16 patients. CONCLUSIONS: The approach now for children and young adults with congenital aortic valve stenosis should be as follows: (1) PBD is the first choice in neonates and infants; (2) Aortic valve commissurotomy is the first choice for children, neonates and infants after failed PBD; (3) The Ross operation is increasingly used in children after failed PBD and in young adults, even with a calcified aortic valve.

[Diagnosis and treatment of childhood cardiomyopathies]. / Diagnose und Therapie von Kardiomyopathien im Kindesalter.

Cardiomyopathies have either a primary (without associated anomalies) or a secondary origin. They are classified in three groups according to their anatomy and function: hypertrophic, dilated or restrictive. We review here the relevant diagnostic points of each type as well as their treatment. Restrictive cardiomyopathies, arrhythmogenic right ventricle, non compaction and Uhl's anomaly will not be dealt with in detail as they are very seldom in children.

Management of paediatric patients undergoing diagnostic and invasive cardiology procedures.

Since diagnostic cardiac catheterization in children with congenital heart disease was first reported in 1947, echocardiography has been used as a non-invasive diagnostic tool in congenital heart disease, resulting in a decrease in diagnostic cardiac catheterizations. However, the total number of cardiac catheterizations remained at a steady level until the mid-1980s and has since increased progressively. This is a result of the introduction of interventional transcatheter techniques to improve or correct congenital heart malformations. Since the first description of balloon atrial septostomy, the range of indications for such techniques has steadily increased, particularly in the past 15 years. 'Deep' sedation or general anaesthesia is essential for the conduct of cardiac catheterization in children, particularly in the younger age group.

Transposition of the great arteries, pulmonary atresia, and multiple ventricular septal defects associated with multiple cardiac rhabdomyomas in a case of tuberous sclerosis.

Multiple cardiac rhabdomyomas are frequently associated with tuberous sclerosis. However, very few cardiac malformations have been described with tuberous sclerosis. We report an unusual case of tuberous sclerosis with cerebral and renal lesions associated with dextrotransposition of the great arteries, pulmonary atresia, patent ductus arteriosus, multiple ventricular septal defects, and parachute mitral valve deformity. After reviewing other cases of congenital heart diseases associated with tuberous sclerosis, emphasis is made on the potential influence of multiple rhabdomyomas developing very early during fetal life on final cardiac structures.