Erik Jan Meijboom

Sudden cardiac death in athletes: the Lausanne Recommendations

Objectives This study reports on sudden cardiac death (SCD) in sport in the literature and aims at achieving a generally acceptable preparticipation screening protocol (PPSP) endorsed by the consensus meeting of the International Olympic Committee (IOC). Background The sudden death of athletes under 35 years engaged in competitive sports is a well-known occurrence; the incidence is higher in athletes (∼ 2/100 000 per year) than in non-athletes (2.5:1), and the cause is cardiovascular in over 90%. Methods A systematic review of the literature identified causes of SCD, sex, age, underlying cardiac disease and the type of sport and PPSP in use. Methods necessary to detect pre-existing cardiac abnormalities are discussed to formulate a PPSP for the Medical Commission of the IOC. Results SCD occurred in 1101 (1966–2004) reported cases in athletes under 35 years, 50% had congenital anatomical heart disease and cardiomyopathies and 10% had early-onset atherosclerotic heart disease. Forty percent occurred in athletes under 18 years, 33% under 16 years; the female/male ratio was 1/9. SCD was reported in almost all sports; most frequently involved were soccer (30%), basketball (25%) and running (15%). The PPSP were of varying quality and content. The IOC consensus meeting accepted the proposed Lausanne Recommendations based on this research and expert opinions (http://multimedia.olympic.org/pdf/en_report_886.pdf). Conclusion SCD occurs more frequently in young athletes, even those under the age of 18 years, than expected and is predominantly caused by pre-existing congenital cardiac abnormalities. Premature atherosclerotic disease forms another important cause in these young adults. A generally acceptable PPSP has been achieved by the IOCs acceptance of the Lausanne Recommendations.

The International Olympic Committee (IOC) consensus statement on periodic health evaluation of elite athletes, March 2009.

LJUNGQVIST Arne (Sweden) Chairman, IOC Medical Commission JENOURE Peter (Switzerland) Coordinator PHE Associate Professor of Sports Medicine Basel and Neuchâtel Member of the IOC Medical & Scientific Group Member Interfederal Commission FIMS ENGEBRETSEN Lars (Norway) IOC, Head of Scientific Activities Professor, Orthopaedic Centre, University of Oslo Co-Chair, Oslo Sports Trauma Research Center Norwegian School of Sport Sciences, Oslo Panel members:

Considerations for Prenatal Counselling of Patients with Cardiac Rhabdomyomas based on their Cardiac and Neurologic Outcomes

Cardiac rhabdomyomas are benign cardiac tumours with few cardiac complications, but with a known association to tuberous sclerosis that affects the neurologic outcome of the patients. We have analysed the long-term cardiac and neurological outcomes of patients with cardiac rhabdomyomas in order to allow comprehensive prenatal counselling, basing our findings on the records of all patients seen prenatally and postnatally with an echocardiographic diagnosis of cardiac rhabdomyoma encountered from August, 1982, to September, 2007. We analysed factors such as the number and the location of the tumours to establish their association with a diagnosis of tuberous sclerosis, predicting the cardiac and neurologic outcomes for the patients.Cardiac complications include arrhythmias, obstruction of the ventricular outflow tracts, and secondary cardiogenic shock. Arrhythmias were encountered most often during the neonatal period, with supraventricular tachycardia being the commonest rhythm disturbance identified. No specific dimension or location of the cardiac rhabdomyomas predicted the disturbances of rhythm.The importance of the diagnosis of tuberous sclerosis is exemplified by the neurodevelopmental complications, with four-fifths of the patients showing epilepsy, and two-thirds having delayed development. The presence of multiple cardiac tumours suggested a higher risk of being affected by tuberous sclerosis. The tumours generally regress after birth, and cardiac-related problems are rare after the perinatal period. Tuberous sclerosis and the associated neurodevelopmental complications dominate the clinical picture, and should form an important aspect of the prenatal counselling of parents.

Left ventricular diverticulum in a neonate with Cantrell syndrome

A newborn baby was transferred to our hospital because of a pulsating mass of the abdominal wall just above the umbilical insertion. The patient was comfortable, not cyanotic and breathing spontaneously, but with mild tachypnoea. Physical examination was unremarkable except for a pulsating purple mass in the midline just above the umbilicus (middle panel). The chest x ray showed a moderate dextroposition of the heart without any pulmonary pathology. The ECG was normal. Echocardiography revealed a normal intracardiac anatomy except for a long and narrow appendiform diverticulum at the apex of the left ventricle of almost 3 cm, contracting …

Validation of two echocardiographic indexes to improve the diagnosis of complex coarctations

OBJECTIVES Coarctation of the aorta is one of the most common congenital heart defects. Its diagnosis may be difficult in the presence of a patent ductus arteriosus, of other complex defects or of a poor echocardiographic window. We sought to demonstrate that the carotid-subclavian artery index (CSA index) and the isthmus-descending aorta ratio (I/D ratio), two recently described echocardiographic indexes, are effective in detection of isolated and complex aortic coarctations in children younger and older than 3 months of age. The CSA index is the ratio of the distal aortic arch diameter to the distance between the left carotid artery and the left subclavian artery. It is highly suggestive of a coarctation when it is <1.5. The I/D ratio defined as the diameter of the isthmus to the diameter of the descending aorta, suggests an aortic coarctation when it is less than 0.64. METHODS This is a retrospective cohort study in a tertiary care childrens hospital. Review of all echocardiograms in children aged 0-18 years with a diagnosis of coarctation seen at the authors institution between 1996 and 2006. An age- and sex-matched control group without coarctation was constituted. Offline echocardiographic measurements of the aortic arch were performed in order to calculate the CSA index and I/D ratio. RESULTS Sixty-eight patients were included in the coarctation group, 24 in the control group. Patients with coarctation had a significantly lower CSA index (0.84+/-0.39 vs 2.65+/-0.82, p<0.0001) and I/D ratio (0.58+/-0.18 vs 0.98+/-0.19, p<0.0001) than patients in the control group. Associated cardiac defects and age of the child did not significantly alter the CSA index or the I/D ratio. CONCLUSIONS A CSA index less than 1.5 is highly suggestive of coarctation independent of age and of the presence of other cardiac defects. I/D ratio alone is less specific than CSA alone at any age and for any associated cardiac lesion. The association of both indexes improves sensitivity and permits diagnosis of coarctation in all patients based solely on a bedside echocardiographic measurement.

A noninvasive diagnostic tool to differentiate myocarditis from myocardial infarction: late gadolinium enhanced cardiac magnetic resonance

Studies in adults have shown that late gadolinium enhanced cardiac magnetic resonance is a safe and noninvasive diagnostic tool which allows one to differentiate myocardial infarction from myocarditis. We believe that it may also be highly useful in the paediatric population for the same purpose.

OP04.04: Neonatal outcome following labor induction in patients with prenatal diagnosis of congenital heart disease

6–10 10 1 1 4 2 7 2 3 2 1 1 1 8 2 6 51 10–12 8 2 5 11 8 16 1 12 7 9 6 1 2 1 3 4 4 6 106 12–14 7 1 10 18 1 10 24 1 11 4 1 5 6 5 10 3 1 1 1 1 3 124 14–16 3 1 13 8 3 10 18 13 7 5 5 2 8 4 7 2 1 3 113 16–20 11 9 14 10 4 3 6 57 20–24 10 4 12 7 2 3 3 41 24–28 3 2 7 3 1 16 28–32 2 2 6 2 1 3 16 32–40 3 3 1 2 3 12 Total/Class 28 5 28 29 38 8 30 15 65 2 38 28 21 3 19 33 18 9 20 12 8 4 13 9 15 4 18 16 Total System 90 91 133 76 59 34 53 536

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.

P04.26: Maternal diabetes and hypertrophic cardiomyopathy in the fetus

Objective: Fetuses with problems, for example growth restriction, diabetic mothers or complex heart disease may change the hemodynamics of ventricular chambers come to alter chamber size, shape, contractility, filling properties, stroke volume (SV,mL) and ejection fraction (EF,%). However normal ranges have been varied, the purpose of this study is to report the mean ranges of cross-sectional and M-mode echocardiographic measurements and to compare with each methods. Methods: This was a prospective study of 62 normal fetuses (Gestational age 20 ∼ 38 weeks) referred for a routine sonographic examination. Two operators using a 2 ∼ 6 MHZ sector probe (Accuvix XQ,MEDISON,co.,LTD.Korea) performed all measurements. The cross-sectional formula employed was the method of discs, which divides the ventricles into 20 equal-thickness discs. (Simpson method). The other method was Teichholz formula. All volumetric data by M-mode echocardiography were computed. Results: We collected normal ranges for the left and right enddiastolic ventricular dimension (EDD) and ejection fraction. M-mode measurements: The mean LVEDD was 9.1 ± 0.45 mm, LV SV 1.09 ± 0.92 mL, LV EF 63.38 ± 16.07%, RVEDD 1.08 ± 1.67 mm and RV EF 60.42 ± 19.12%. Simpson method measured the LV EF was 56.62 ± 17.67%, RV EF was 58.51 ± 19.55%. As gestational age, there was no significant difference between M mode and crosssectional echocardiography. Also there was not a difference of the ventricular function which it follow gestational age. Conclusion: This is the initial research which shows ranges for fetal ventricular ejection fraction and quantify them in fetus by the noninvasive echocardiography. In the future, we apply this method in the fetuses who have a problem and reveal an interrelation between prenatal and postnatal ventricular function. It will be helpful to decide a treatment time and predict their prognosis.