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.

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.

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.

[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.

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.

Treatment of fetal tachycardia with sotalol: transplacental pharmacokinetics and pharmacodynamics.

OBJECTIVES: The aim of this study was to investigate the pharmacokinetics and pharmacodynamics of sotalol in the treatment of fetal tachycardia. BACKGROUND: Maternally administered, intrauterine therapy of fetal tachycardia is dependent on the transplacental passage of the antiarrhythmic agent. METHODS: In a prospective study of patients treated for fetal tachycardia with sotalol, concentrations of sotalol were determined in maternal and umbilical blood and in amniotic fluid, and the relationship between these concentrations and the occurrence of conversion to sinus rhythm was investigated. RESULTS: Eighteen fetal patients were studied, nine with atrial flutter and nine with supraventricular tachycardia. Fourteen were treated with sotalol; 13 converted to sinus rhythm, of whom 2 relapsed. There was one intrauterine death. Four patients were treated with sotalol and digoxin, of whom two were treated successfully. Mean birth weight was 3,266 g. The daily maternal sotalol dose was linearly related to the maternal plasma concentration. The mean fetal/maternal sotalol plasma concentration was 1.1 (range 0.67 to 2.87, SD 0.63), and the mean amniotic fluid/fetal blood ratio of sotalol was 3.2 (range 1.28 to 5.8, SD 1.4). The effectiveness of sotalol therapy could not be extrapolated from maternal blood levels. CONCLUSIONS: Sotalol is a potent antiarrhythmic agent in the treatment of fetal tachycardia. The placental transfer is excellent. Sotalol accumulates in amniotic fluid but not in the fetus itself. Therefore it seems that renal excretion in the fetus is efficient and greater than the oral absorption by fetal swallowing. The maternal blood level is not a reliable predictor of the chances of success of therapy. Sotalol is not associated with fetal growth restriction.

Angioplasty for coarctation in different aged patients.

BACKGROUND: Differences in the indication and outcome of balloon angioplasty for coarctation in children and adults have not been elucidated sufficiently. The results of balloon angioplasty for coarctation are compared between pediatric and adult age groups. METHODS: Balloon angioplasty for coarctation of the aorta was performed in 85 patients who were classified according to age and native coarctation/recoarctation. Groups A (patients aged <16 years, n = 32) and B (patients aged > or =16 years, n = 17) included patients with native coarctations. Groups rCoA A (patients aged <16 years, n = 33) and rCoA B (patients aged > or =16 years, n = 3) included patients with recoarctations. Follow-up included 2-dimensional Doppler scanning echocardiography and additional angiography or magnetic resonance imaging. Gradient reductions in groups were compared by use of the independent-samples t test. Kaplan-Meier and log-rank analyses were performed as a means of comparing long-term outcome. RESULTS: No mortality occurred. Immediate success was equal in groups A, B, and rCoA A (94%). Dilatation was unsuccessful in 2 patients in group rCoA B. Pressure gradients decreased 23 mm Hg in group A, 31 mm Hg in group B, 18 mm Hg in group rCoA A, and 11 mm Hg in group rCoA B. Pressure gradient drops, compared between groups A and B, showed a significant difference (P <.001). The length of hospital stay ranged from 12 to 48 hours. The period of follow-up ranged from 6 months to 12 years (mean, 4.9 years). Kaplan-Meier curves of groups A and B are not different, as determined by means of log-rank analysis. No aneurysm formation was encountered. CONCLUSIONS: The results of balloon angioplasty for native coarctation in both selected children and adults are excellent. In recoarctation, we recommend balloon angioplasty in the pediatric patients.

Comparison of polytetrafluoroethylene patch aortoplasty and end-to-end anastomosis for coarctation of the aorta.

OBJECTIVES: Although aneurysm formation and recoarctation after Dacron patch aortoplasty have been reported on extensively, less is known about these outcomes after polytetrafluoroethylene patch repair, which was compared with resection and end-to-end anastomosis in this study. METHODS: Two hundred sixty-two children had surgical repair of coarctation of the aorta by means of either resection and end-to-end anastomosis (n = 137; mean age, 1.85 +/- 3.1 years) or polytetrafluoroethylene patch aortoplasty (n = 118; mean age, 1.09 +/- 1.9 years) during a 28-year period. Coarctation was isolated in 109 (41.6%), associated with ventricular septal defect in 77 (29.4%), and associated with complex intracardiac anomalies in 76 (29.0%) patients. Follow-up ranged from 2 days to 29.3 years (median, 11.9 years). Seven patients were lost to follow-up. Kaplan-Meier survival curves were estimated, and multivariable Cox regression analysis was performed for several outcome variables. RESULTS: Mortality was 8.2% and was associated with intracardiac pathology in all cases. Recoarctation occurred in 53 patients, 23 after resection and anastomosis and 30 after patch repair, not differing statistically (P =.4, log-rank test). Aneurysm formation occurred in 8 patients after patch repair that included ridge resection in 7 of the 8 patients. Late hypertension occurred in less patients (n = 3) after resection and anastomosis than after patch repair (n = 8) (P <.03). Arch hypoplasia (P <.01) and age less than 1 month (P <.001) were found to be independent risk factors for recoarctation. CONCLUSIONS: Polytetrafluoroethylene patch repair including coarctation ridge resection was found to be a risk factor for aneurysm formation and late hypertension. Arch hypoplasia and young age must be considered to predispose to recoarctation.

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.

Right ventricular dysfunction and pulmonary valve replacement after correction of tetralogy of Fallot.

BACKGROUND: Correction of tetralogy of Fallot often leads to pulmonary regurgitation, sometimes warranting pulmonary valve replacement, for which indications and timing to achieve optimal results are not yet clear. This retrospective study describes follow-up and reinterventions in our tetralogy of Fallot population. METHODS: Review of all consecutive patients operated on for tetralogy of Fallot between 1977 and 2000 was conducted. Included are date and type of repair, Doppler echocardiography (two-dimensional echocardiography), electrocardiographs, reoperations, and physical condition. RESULTS: Total repair was performed in 171 patients at a mean age 1.9 +/- 2.5 years, follow-up time counted 9.6 +/- 7.0 years. Right ventriculotomy was used in 92%, and transatrial ventricular septal defect closure was used in 8%; 74% received a transannular outflow patch. Twenty-year survival was 91%. Last follow-up electrocardiographs showed right bundle branch block in 67% and serious arrhythmias in 11%. Two-dimensional echocardiography demonstrated severe pulmonary insufficiency and dilated right ventricle in 31% and 38%, respectively, increasing with postrepair age (p < 0.001). Poor clinical condition (New York Heart Association class II+) and echocardiographic proof of right atrial dilatation (p = 0.012) and arrhythmias (p = 0.03) were significantly associated. Furthermore, the influence of residual hemodynamic lesions, such as a remaining ventricular septal defect or pulmonary stenosis, or right ventricular dilatation was important (p = 0.04). Reintervention was necessary in 32 patients (19%; 10-year freedom, 83%), including angioplasty for residual stenosis and pulmonary valve replacement. At a mean age of 9.2 years after correction, 14 patients received a homograft, and 2 patients received a heterograft. In 7 patients the right ventricle returned to normal dimensions and symptoms disappeared. The incidence of right ventricular dilatation was considerably higher (p = 0.020) in patients with a transannular patch; the transatrial approach showed the opposite (p = 0.03), and patients presented with lower QRS duration (p = 0.007), although no difference could be found between survival after both surgical techniques. Effects of early timing (correction < 6 months) on right ventricular dysfunction could not be established. CONCLUSIONS: Severe right ventricular dilatation and pulmonary regurgitation secondary to outflow tract repair in tetralogy of Fallot are frequently occurring sequelae developing slowly over time. Indications for pulmonary valve replacement remain controversial because echocardiographic findings or arrhythmias are not always accompanied by deterioration of clinical condition. However, right atrial dilatation and additional hemodynamic lesions demand increased vigilance. Transatrial repair is associated with a favorable outcome.

Drug treatment of fetal tachycardias.

The pharmacological treatment of fetal tachycardia (FT) has been described in various publications. We present a study reviewing the necessity for treatment of FT, the regimens of drugs used in the last two decades and their mode of administration. The absence of reliable predictors of fetal hydrops (FH) has led most centers to initiate treatment as soon as the diagnosis of FT has been established, although a small minority advocate nonintervention. As the primary form of pharmacological intervention, oral maternal transplacental therapy is generally preferred. Digoxin is the most common drug used to treat FT; however, effectiveness remains a point of discussion. After digoxin, sotalol seems to be the most promising agent, specifically in atrial flutter and nonhydropic supraventricular tachycardia (SVT). Flecainide is a very effective drug in the treatment of fetal SVT, although concerns about possible pro-arrhythmic effects have limited its use. Amiodarone has been described favorably, but is frequently excluded due to its poor tolerability. Verapamil is contraindicated as it may increase mortality. Conclusions on other less frequently used drugs cannot be drawn. In severely hydropic fetuses and/or therapy-resistant FT, direct fetal therapy is sometimes initiated. To minimize the number of invasive procedures, fetal intramuscular or intraperitoneal injections that provide a more sustained release are preferred. Based on these data we propose a drug protocol of sotalol 160 mg twice daily orally, increased to a maximum of 480 mg daily. Whenever sinus rhythm is not achieved, the addition of digoxin 0.25 mg three times daily is recommended, increased to a maximum of 0.5 mg three times daily. Only in SVT complicated by FH, either maternal digoxin 1 to 2mg IV in 24 hours, and subsequently 0.5 to 1 mg/day IV, or flecainide 200 to 400 mg/day orally is proposed. Initiating direct fetal therapy may follow failure of transplacental therapy.

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.

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.

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.