Diagnostic yield and cost analysis of electrocardiographic screening in Swiss paediatric athletes.

OBJECTIVES: Athletes performing sports on high level are at increased risk for sudden cardiac death. This includes paediatric athletes, even though data on screening strategies in this age group remain scarce. This study aimed to assess electrocardiogram interpretation criteria in paediatric athletes and to evaluate the cost of screening. METHODS: National, multicentre, retrospective, observational study on 891 athletes of paediatric age (<18 years) evaluated by history, physical examination and 12-lead electrocardiogram. The primary outcome measure was abnormal electrocardiogram findings according to the International Recommendations for Electrographic Interpretation in Athletes. The secondary outcome measure was cost of screening. RESULTS: 19 athletes (2.1%) presented abnormal electrocardiogram findings requiring further investigations, mainly abnormal T-wave inversion. These 19 athletes were predominantly males, performing endurance sports with a mean volume of 10 weekly hours for a mean duration of 6 years of training. Further investigations did not identify any relevant pathology. All athletes were cleared for competition with regular follow-up. Total costs of the screening were 108,860 USD (122 USD per athlete). CONCLUSIONS: Our study using the International Recommendations for Electrographic Interpretation in Athletes identified a low count of abnormal findings in paediatric athletes, yet raising substantially the cost of screening. Hence, the utility of electrocardiogram-inclusive screening of paediatric athletes remains to be elucidated by longitudinal data.

[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.

Population-based review of tetralogy of Fallot with absent pulmonary valve: is prenatal diagnosis really associated with a poor prognosis?

OBJECTIVES: Tetralogy of Fallot with absent pulmonary valve syndrome (TETAPV) is reported in obstetric literature to have an extremely poor prognosis. We sought to determine the clinical outcome associated with TETAPV and whether prenatal diagnosis confers a poor prognosis. METHODS: All cases of TETAPV diagnosed in British Columbia between 1980 and 2009 were reviewed and grouped according to time of diagnosis, either prenatal or postnatal. The groups were compared with respect to mortality, respiratory problems, number of interventions and functional capacity at last follow-up. RESULTS: Eight and 11 patients were included in the prenatally and postnatally diagnosed groups, with overall long-term survival of 71% and 82%, respectively. There was no significant difference in mortality, frequency of preoperative intubation, number of interventions or functional capacity between groups. CONCLUSION: From a population-based retrospective analysis of TETAPV cases identified over three decades it is concluded that the prognosis for TETAPV is better than that previously reported in the obstetric literature. This information should be used to guide prenatal counseling.

[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.

Neonatal oxygen exposure leads to increased aortic wall stiffness in adult rats: a Doppler ultrasound study.

We have previously shown that neonatal high oxygen (O2) exposure in rats leads to hypertension and vascular dysfunction in adulthood. Pulse-wave velocity (PWV), an indirect measure of vascular biophysical properties (arterial stiffness or distensibility), is a sensitive marker of cardiovascular health. Its measurement in rats is mostly based on invasive hemodynamics measurements, prohibiting longitudinal studies particularly relevant in models of developmental programming of cardiovascular dysfunctions. With this study, we sought (1) to verify the feasibility and validity of measuring of aortic PWV in Sprague-Dawley rats by ultrasound; (2) to use the technique to compare aortic PWV in adult rats exposed to O2 as newborns (80% day 3-10 of life) v. controls; and (3) to develop an algorithm to calculate PWV in a non-invasive manner. We calculated aortic PWV using standard echocardiography and electrocardiogram, and validated the measures with PWV obtained by intraaortic catheters. Aortic full length was measured at sacrifice. PWV was significantly increased in O2 exposed (505 ± 18 cm/s) v. control animals (421 ± 17 cm/s, P < 0.01). With regard to weight, femur length and distance from the manubrium to the anal margin (MA length), the latter showed the best correlation (R = 0.84, P < 0.0001) with full aorta length derived from (L) = 0.339 × (MA length) + 4.281. The current data using echo-Doppler method demonstrated increased aortic stiffness in adult rats exposed to hyperoxia as newborns and suggests that non-invasive longitudinal studies of aortic PWV can be performed using the proposed algorithm for estimation of the full aorta length.

Feasibility and reliability of Doppler flow recordings in the fetal aortic isthmus: a multicenter evaluation.

OBJECTIVES: To evaluate the performance of three different centers with respect to their ability to identify the fetal aortic isthmus (AoI) adequately and place a Doppler sample volume in the AoI correctly, and to address the reproducibility of the isthmic flow index (IFI) calculated from Doppler waveforms recorded in the three centers. METHODS: The three collaborating centers sent several ultrasonographic recordings taken at random over a 6-week period to the Saint-Justine Fetal Cardiology Unit (StJ-FCU). A performance quotient ((number of total readings - number of unsatisfactory results)/number of total readings) was calculated for each center by each of three judges, who were experienced fetal cardiologists, to assess the ability of each center to identify the isthmus and to place the Doppler sample volume (DSV) adequately. Intraclass correlation coefficients (ICC) were computed to quantify the variability of IFI measurements ((systolic + diastolic)/systolic flow velocity integrals). RESULTS: Fifty-five recordings were available for this study. Concerning isthmus identification, there was 100% agreement between the three judges from StJ-FCU and the performance quotients of Centers A, B and C were: 0.90, 0.95 and 1.00, respectively. For DSV positioning, agreement between the judges varied; for Judge 1 vs. Judge 2, kappa = 0.836 (95% CI, 0.651-1.000); for Judge 1 vs. Judge 3, kappa = 0.773 (95% CI, 0.557-1.000); for Judge 2 vs. Judge 3, kappa = 0.941 (95% CI, 0.805-1.000). The performance quotients of the three centers for DSV positioning were consistently lower than were those for identification of the isthmus, being 0.85, 0.76 and 0.92, respectively. The ICC between the first and second measurements of the IFI by Rater 1 was 0.96 (95% CI, 0.93-0.98, P < 0.001) and that between Raters 1 and 2 was 0.97 (95% CI, 0.95-0.99, P < 0.001). CONCLUSION: Adequate imaging of the fetal AoI can be achieved easily by a trained sonographer, while DSV positioning is challenging. The intra- and interrater variability of the IFI are low.

[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.

[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.