The Impending Dilemma of Electrocardiogram Screening in Athletic Children.

Sudden cardiac death (SCD) affects 2/100,000 young, active athletes per year of which 40% are less than 18 years old. In 2004, the International Olympic Committee accepted the Lausanne Recommendations, including a 12-lead electrocardiogram (ECG), as a pre-participation screening tool for adult Olympic athletes. The debate on extending those recommendations to the pediatric population has recently begun. The aims of our study were to highlight the characteristics of the young athlete ECG, phenotypical manifestations of SCD-related disease in children, and challenges of implanting ECG screening in athletic children. A systematic review of the literature is performed. We searched available electronic medical databases for articles relevant to SCD, ECG, silent cardiac diseases, and athletic children. We focused on ECG screening and description in a pediatric population. We identified 2240 studies. Sixty-two relevant articles and one book were selected. In children, prepubertal ECG and the ECG phenotype of most SCD-related diseases differ notably from adults. The characteristics of the prepubertal ECG and of the phenotypical manifestation of SCD-related disease in children will result in less specific and less sensitive ECG-based screening programs. Those limitations advise against extending the adult recommendation to children, without further studies. Until then, history and physical exam should remain the cornerstone of screening for SCD-related pathologies in children.

Aortic valve dysfunction and aortic dilation in adults with coarctation of the aorta.

OBJECTIVES: To determine the prevalence of aortic valve dysfunction, aortic dilation, and aortic valve and ascending aortic intervention in adults with coarctation of the aorta (CoA). BACKGROUND: Aortic valve dysfunction and aortic dilation are rare among children and adolescents with CoA. With longer follow-up, adults may be more likely to have progressive disease. METHODS: We retrospectively reviewed all adults with CoA, repaired or unrepaired, seen at our center between 2004 and 2010. RESULTS: Two hundred sixteen adults (56.0% male) with CoA were identified. Median age at last evaluation was 28.3 (range 18.0 to 75.3) years. Bicuspid aortic valve (BAV) was present in 65.7%. At last follow-up, 3.2% had moderate or severe aortic stenosis, and 3.7% had moderate or severe aortic regurgitation. Dilation of the aortic root or ascending aorta was present in 28.0% and 41.6% of patients, respectively. Moderate or severe aortic root or ascending aortic dilation (z-score > 4) was present in 8.2% and 13.7%, respectively. Patients with BAV were more likely to have moderate or severe ascending aortic dilation compared with those without BAV (19.5% vs. 0%; P < 0.001). Age was associated with ascending aortic dilation (P = 0.04). At most recent follow-up, 5.6% had undergone aortic valve intervention, and 3.2% had aortic root or ascending aortic replacement. CONCLUSION: In adults with CoA, significant aortic valve dysfunction and interventions during early adulthood were uncommon. However, aortic dilation was prevalent, especially of the ascending aorta, in patients with BAV.

Adaptable pulmonary artery band for late arterial switch procedure in transposition of the great arteries.

BACKGROUND: In late-diagnosed transposition of the great arteries (TGA), the left ventricle (LV) involutes as it pumps against low resistance and needs retraining by applying a pulmonary artery band (PAB) in preparation for an arterial switch operation. We report our experience with a telemetrically adaptable band compared with classic banding. METHODS: Ten patients underwent retraining of the LV, 4 patients with an adaptable band and progressive weekly tightening of the band (group 1) and 6 patients with a traditional band (group 2). RESULTS: Mean weight and age at pulmonary band placement was 5.8±2.36 kg and 11.7±11.1 months for group 1 and 5.0±2.3 kg and 6.4±7.6 months for group 2. Time between palliation and switch procedure was 4.2 months in both groups. Group 1 showed an initial mean pulmonary gradient of 25.5±4.43 mm Hg with a 5% closure of the device. The mean gradient increased with progressive closure to 63.5±9.8 mm Hg at the time of the arterial switch operation. There were no reinterventions or deaths in this group. In group 2, the mean pulmonary gradient increased with growth from 49±21.4 mm Hg to 68.4±7.86 mm Hg at the time of the switch procedure. However, 4 of these patients required reoperations during retraining: 2 needed 1 reoperation and 2 needed 2 reoperations. Two patients died-1 after banding and 1 after the switch operation. CONCLUSIONS: Retraining of the LV by the adaptable device allows precise control of the tightening, avoids repetitive operations, and diminishes morbidity.

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.

Right ventricular outflow tract reconstruction with the bovine jugular vein graft: 5 years' experience with 133 patients.

BACKGROUND: We analyzed the results in two centers of using bovine jugular vein graft for right ventricular outflow tract reconstruction. METHODS: From April 1999 to July 2005, 133 children with a median age of 30.9 months (range, 4 days to 19 years) underwent graft implantation. Echocardiography was performed during follow-up and retrospectively reviewed. RESULTS: Nongraft-related early mortality occurred in 8 patients. Late mortality occurred in 11 patients, 2 late deaths were graft related (endocarditis). Median follow-up was 31.6 months (range, 1 to 73). Twelve patients received a new graft, because of endocarditis (3), distal pulmonary artery branch stenosis (4), graft obstruction caused by fibrosis (4), or thrombosis (1). Echocardiography Doppler studies showed good conduit function, with 92% of the patients having absent, trivial, or only mild valve regurgitation at last follow-up. A moderate degree of conduit stenosis due to external compression was observed in 2 patients. Twenty-five patients with otherwise intact conduits had hemodynamically significant distal stenosis. In most cases, the pulmonary branch stenosis was related to preoperative small pulmonary arteries and young age at operation. At 31.6 months, significant graft dilatation was observed in 4 grafts and was related to pulmonary artery branch obstruction or pulmonary hypertension. Calcification did not occur in 5 years time. Survival was 85.7%, freedom from conduit explantation was 91%, and freedom from intervention for pulmonary artery branch stenosis was 80% after 5 years. CONCLUSIONS: The bovine jugular vein graft is a valuable right ventricular outflow tract conduit, but younger age and small pulmonary arteries increase the risk of distal conduit stenosis.

Neurological outcome in isolated congenital heart block and hydrops fetalis.

OBJECTIVE: Isolated fetal heart block (HB), a condition associated with fetal hydrops, carries a high mortality rate and may result in neurodevelopmental sequelae. To the best of our knowledge, no data exist regarding the long-term outcome of such hydropic fetuses. We reviewed our experience with this condition to determine the neurodevelopmental outcome of prenatally diagnosed cases with isolated HB complicated by hydrops fetalis. METHODS: Neurodevelopmental assessment of 5 children presented prenatally with isolated HB associated with hydrops fetalis. RESULTS: During the last 18 years, 10 cases were detected prenatally with isolated HB and hydrops fetalis. 3 of the 10 fetuses died in utero, and 2 died postnatally, due to a dilated cardiomyopathy, at the age of 9 months and 4 years, respectively. Neurodevelopmental studies done on the 5 remaining children were normal. CONCLUSION: Long-term neurodevelopmental assessments of 5 surviving cases presented prenatally with isolated HB and hydrops fetalis are reassuring.

Pathologic ventricular hypertrophy in the offspring of diabetic mothers: a retrospective study.

AIMS: Diabetes in pregnant women is increasing and with that the complications in their offspring. We studied our population of diabetic mothers (2003-2005) for pathologic ventricular hypertrophy (PVH). METHODS AND RESULTS: In our retrospective study of all 87 diabetic pregnancies (92 neonates), 16 were type 1, 17 were type 2, and 54 were gestational diabetes (GD). Haemoglobin glycated (HbA1c) median was 5.8% (5.3-6.5): 17 with HbA1c above normal 2 with congenital heart disease (CHD) and six with PVH. A total of 75 neonates were normal, five had CHD, and 12 had PVH (1/12 died post-natally, 1/12 stillborn, 2/12 required premature delivery, 8/12 normal). The 16 type 1 pregnancies resulted in three neonates with CHD and in 50% PVH, including one death, one premature Cesarean section because of PVH. The 17 neonates of type 2 pregnancies showed in one CHD and in 25% PVH. Of the 54 GD pregnancies, one had CHD and one had PVH. CONCLUSION: Pregnancies of both type 1 and 2 diabetes carry an increased risk for foetal development of PVH compared with those with GD. The insufficient effect of preventive glycaemia controls leads to conclude that although no definite predictive parameters for malignant outcome can be presented, close monitoring of these pregnancies may prevent perinatal catastrophes.

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 (approximately 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 IOC's acceptance of the Lausanne Recommendations.

[Tetralogy of Fallot: after the successful surgical correction...]. / Tétralogie de Fallot: les suites d'un succès....

Tetralogy of Fallot is the most frequent cyanotic congenital heart disease (1/10000 births). The first complete corrections have been done in the fifties, and we now have long-term follow-up studies, revealing the most frequent arrhythmias and anatomic substrates compromising the long-term survival of these patients. The purpose of this article is to review some of the long-term complications of these patients, who, even if they have been completely corrected, will never be cured.

Transient non-autoimmune fetal heart block.

OBJECTIVES: Fetal heart block is a rare and irreversible condition associated with structural heart defects or maternal autoantibodies (SS-A/Ro and SS-B/La) resulting in permanent damage of the atrioventricular (AV) node. This is the first report of 4 cases with a transient fetal heart block in structurally normal hearts without maternal autoantibodies. METHODS: A report on 4 patients seen within a 14-year period at one center with fetal heart block without intracardiac abnormalities or maternal autoantibodies. RESULTS: Three patients were referred to our center with a fetal bradycardia (heart rate 70-85 bpm), between 20 and 33 weeks' gestational age, and 1 for a 'triple' test at 16 weeks' gestational age. Echocardiography showed a complete heart block in 2 fetuses, and a second-degree AV block in the other 2. Heart block had completely resolved at all following visits. Postnatal ECG recordings showed normal sinus rhythm in all patients. Echocardiographic evaluation at presentation and follow-up showed normal cardiac anatomy, without signs of hydrops or cardiac decompensation in all patients. All mothers tested negative on SS-A/Ro and SS-B/La autoantibodies. CONCLUSIONS: Fetal heart block can occur in the absence of structural heart defects and maternal autoantibodies to SS-A/Ro and SS-B/La. The origin of such heart block is unknown, but its course seems benign: none of the patients ever showed ventricular heart rates <55 bpm, signs of congestive heart failure or fetal hydrops. Heart block resolved spontaneously in all patients.

Doppler-guided regulation of a telemetrically operated adjustable pulmonary banding system.

OBJECTIVES: We report on the Doppler-assessed regulation of an adjustable pulmonary artery band (PAB) in an animal model and in our first group of patients. BACKGROUND: Indications for pulmonary artery banding have expanded to include patients requiring a late arterial switch. A telemetry-operated, fully implantable, adjustable PAB system (FloWatch- PAB, Endoart SA, Lausanne, Switzerland) has been developed to facilitate these operations. METHODS: The device was implanted in 13 minipigs (age one to five months, weights 3.2 to 12.0 kg). The main study was performed on nine minipigs with adjustments of the PAB at implantation and at 1, 3, 5, 8, and 12 weeks after, assessed by Doppler pressure gradients. Explanation was performed 12 weeks after surgery. A long-term histology study (6 months and 14 months after surgery) was done on the other four minipigs. After approval by the ethics committee, the device was implanted in eight patients with weights between 2.8 and 9 kg to decrease pulmonary blood flow and pressure and to retrain the left ventricle before arterial switch. The device was progressively tightened, with increasing transband Doppler gradients. Follow-up was one to three months. RESULTS: An excellent correlation between transbanding systolic pressure gradient and degree of PAB constriction was encountered in the minipig study as well as in the human setting. No early or late deaths or reoperations occurred. Malfunction of the device was noted in three of 21 implanted devices. Two were related to surgically inflicted damage at implantation and one to an electronic problem that was fixed by resetting the control device. CONCLUSIONS: The device offers a Doppler-controllable adjustment of pulmonary blood flow. It permits controlled tightening and release of the band, which improves perioperative and postoperative courses and decreases surgical interventions to adjust tightness of the band. It allows a protracted occlusion protocol, which may provide the best effect on retraining the left ventricle.

The effects of intrapartum hypoxia on the fetal QT interval.

BACKGROUND: The morphology of the fetal ECG complex provides information on the fetal condition during labour, such as the ST segment and T-wave configuration. We hypothesised that the intrapartum fetal QT interval may provide additional information on the condition of the fetus, as it is known that the QT interval reacts to situations of stress and exercise. DESIGN: Retrospective study. SETTING: Data were substracted from a European community multicentre trial. METHODS: The intrapartum QT interval was measured in 68 fetuses who were acidemic at birth (pH <7.05 and BD (ecf) >12 mmol/L) and in a control group of similar size. All of these cases were monitored by STAN S21. Measurements were performed at the start of the recording at baseline heart rate, during variable decelerations and at the end of the recording. The QTc was calculated using Bazett's formula: QT/ radical RR. The intervals were compared using the Wilcoxon signed ranks test. MAIN OUTCOME MEASURES: Fetal QT interval, and the corrected QT interval: QTc. RESULTS: In the acidemic fetuses, there was a significant shortening of the QTc interval at the end of the recording compared with the start of the recording (397 ms at the end vs 359.3 ms at start; P < 0.001), in association with a significantly lowered heart rate (136.3 vs 110.9 bpm, P < 0.001). Measurements of QT and QTc during variable decelerations at the start and end of the recording also showed a shortening of the QT interval (301.9 vs 273.3 ms, P< or = 0.001) and QTc interval (381.6 vs 340.3, P < 0.001), and this was not dependent on heart rate. In the control cases, no differences in FHR, QT and QTc intervals were present. CONCLUSIONS: In intrapartum hypoxia, resulting in metabolic acidosis, a significant shortening of the fetal QT and QTc is present, irrespective of changes in heart rate. In control cases, this shortening does not occur. The intrapartum fetal QT interval may therefore provide additional information on the condition of the fetus.

Comparison of surgical repair with balloon angioplasty for native coarctation in patients from 3 months to 16 years of age.

OBJECTIVE: Surgery and balloon angioplasty (BA) for coarctation of the aorta have shown comparable short-term results, but long-term follow-up remains unclear. Comparison of surgical repair and balloon coarctation for native coarctation of the localised membranous form is performed retrospectively. To allow a valid comparison between both techniques, identical inclusion criteria were applied. METHODS: Results of surgery (group A, 18 patients, age 0.30-14 years, median 0.63 years) and BA (group B, 28 patients, age 0.25-15 years, median 5.8 years) for isolated, native coarctation in children >3 months, performed in a 10-year-period, were compared. Kaplan-Meier analysis was performed in both groups. Mean follow-up ranged from 2.5 to 11 years (mean 7.2+/-2.4 years) in group A and from 1.4 to 10 years (mean 5.4+/-2.8 years) in group B. RESULTS: Immediate success was obtained in all patients following surgery and 27/28 patients (96%) following BA. No statistical difference between surgery and angioplasty with respect to resultant pressure gradient decreases were found. Mortality was not encountered. Hospital stay varied from 6 to 20 days in group A and was 48 h for all patients in group B. Recoarctation occurred in one patient (5.6%) in group A and in two patients (7%) in group B. Log-rank test reveals no statistical difference in freedom from reintervention probabilities between surgery and angioplasty. Aneurysm formation was not encountered. CONCLUSIONS: Both surgical repair and BA for native coarctation yield low reintervention probabilities in comparable patients. Aneurysm formation was not encountered following different treatment types.

Amiodarone therapy for drug-refractory fetal tachycardia.

BACKGROUND: Fetal tachycardia complicated by ventricular dysfunction and hydrops fetalis carries a significant risk of morbidity and mortality. Transplacental digoxin is effective therapy in a small percentage, but there is no consensus with regard to antiarrhythmic treatment if digoxin fails. This study evaluates the safety, efficacy, and outcome of amiodarone therapy for digoxin-refractory fetal tachycardia with heart failure. METHODS AND RESULTS: Fetuses with incessant tachycardia and either hydrops fetalis (n=24) or ventricular dysfunction (n=2) for whom digoxin monotherapy and secondary antiarrhythmic agents (n=13) were not effective were treated transplacentally with a loading dose of oral amiodarone for 2 to 7 days, followed by daily maintenance therapy for <1 to 15 weeks. Digoxin therapy was continued throughout gestation. Newborns were studied by transesophageal pacing or ECG monitoring to determine the mechanism of tachycardia. Three fetuses were delivered urgently in tachycardia during amiodarone loading, and 3 required additional antiarrhythmic agents for sustained cardioversion. Amiodarone or amiodarone combinations converted 14 of 15 (93%) with reentrant supraventricular tachycardia, 2 of 2 with ventricular or junctional ectopic tachycardia, and 3 of 9 (33%) with atrial flutter. Amiodarone-related adverse effects were transient in 5 infants and 8 mothers. Mean gestational age at delivery was 37 weeks, with 100% survival. CONCLUSIONS: Orally administered amiodarone is safe and effective treatment for drug-refractory fetal tachycardia, specifically reentrant supraventricular tachycardia, junctional ectopic, or ventricular tachycardia, even when accompanied by hydrops fetalis or ventricular dysfunction.

Comparative long-term results of surgery versus balloon valvuloplasty for pulmonary valve stenosis in infants and children.

BACKGROUND: We compared the long-term results of surgical valvotomy (S) versus balloon valvuloplasty (BV) for pulmonary valve stenosis in infants and children. METHODS: Results after surgical pulmonary valvotomy (with concomitant ASD/VSD closure) (n = 62, age 2.9 +/- 3.5 years) and balloon valvuloplasty (n = 108, age 3.6 +/- 3.9 years) were analyzed. Transvalvular mean pressure gradient decrease, freedom from reintervention for restenosis, pulmonary valve insufficiency, and tricuspid valve insufficiency were considered. RESULTS: Mean pressure gradient decreased significantly more in the surgical group (from 64.8 +/- 30.8 mm Hg to 12.8 +/- 9.8 mm Hg at a mean follow-up of 9.8 years) than after BV (decreasing from 66.2 +/- 21.4 mm Hg to 21.5 +/- 15.9 mm Hg after a mean of 5.4 years; p < 0.001). Moderate pulmonary valve insufficiency occurred in 44% after surgery, and in 11% after BV (p < 0.001). Tricuspid valve insufficiency occurred in 2% after surgery, and in 5% after BV. Restenosis occurred in 3 surgical patients (5.6%), 2 patients required reoperation, and 1 patient required a balloon valvotomy. Restenosis developed in 13 BV patients (14.1%): 6 patients were redilated and 7 patients required surgery. Surgical valvotomy led to significantly less reinterventions than balloon valvuloplasty (p < 0.04). CONCLUSIONS: Surgical relief of pulmonary valve stenosis produces lower long-term gradients and results in longer freedom from reintervention. Balloon valvuloplasty may remain, despite these results, the preferred therapy for isolated pulmonary valve stenosis, because it is less invasive, less expensive, and requires a shorter hospital stay. Surgery should remain the exclusive form of therapy in the presence of concomitant intracardiac defects, which need to be addressed.