Repetitive ventricular tachycardia in a syncopal child: Cause or incidental finding ?

A 15-year-old girl suffered recurrent syncopal episodes during 7 years. Events were precipitated by exercise or emotional stress, leading to the diagnosis of reflex syncope. Exercise testing induced recurrent salvos of nonsustained right ventricular outflow tract tachycardia. This arrhythmia is often asymptomatic, reflex syncope is very frequent and both causes are related to the same triggering situations. It was therefore essential to obtain recordings during syncopal events and to observe the clinical evolution under effective treatment in order to make the right diagnosis.

Paediatric presentation of cardiac involvement in hypereosinophilic syndrome.

Eosinophilic endomyocarditis is extremely rare in paediatrics. This case report aims to present the sequential changes from thrombotic to fibrotic stages of the cardiac involvement in a child with hypereosinophilic syndrome. Endomyocardial fibrosis progressively aggravated the severity of the mitral regurgitation. Bioprosthetic valve replacement was finally performed without recurrence of cardiac complications despite a late relapse of the underlying disease.

Managing Ventricular Septal Defect with Associated Aortic Regurgitation: Two Decades of Experience.

BACKGROUND AND AIM OF THE STUDY: Ventricular septal defect (VSD) with aortic regurgitation (AR) is a well-known association. However, there is still no agreement about its management, particularly regarding the technical details of its operative treatment. The study aim was to describe all components of the syndrome and to evaluate the various techniques used with regards to its anatomical and functional features. METHODS: A total of 31 patients (mean age 7.4 years; range: 1.0-14.3 years) who underwent repair of VSD and AR between 1990 and 2013 was reviewed. The VSD was perimembranous in 22 patients, and subarterial in nine. Trusler's valvuloplasty technique was used in 15 patients, Yacoub's technique in seven, and Carpentier's technique (triangular resection) in four. Two patients underwent aortic valve replacement (AVR), and three patients with no significant aortic valve lesions underwent a simple patch repair of the VSD. RESULTS: The aortic valvuloplasty results were generally good, with an initial aortic valvuloplasty avoiding AVR. During the immediate postoperative period, valvuloplasty failure occurred in three patients, regardless of the technique used, and all three patients were reoperated on. The mean duration of follow up was 8.5 years (range: 3.2-20.6 years). The initial result was maintained in all patients, except for four who underwent late AVR. CONCLUSION: The study findings contributed to an analysis of VSD and AR, and helped to clarify the best surgical strategy. The results obtained suggest that adequacy of the initial repair is the most important determinant of subsequent evolution.

Long-term conservative management of a giant cardiac fibroma.

A giant cardiac fibroma was discovered during evaluation for a soft systolic murmur in an asymptomatic 2-week-old girl. Echocardiography and magnetic resonance imaging showed a large intraventricular solid mass developed at the expense of the left ventricular lateral wall. Tumour progression resulted in failure to thrive and ventricular arrhythmia between 2 and 18 months of age. At that time, complete resection seemed unfeasible and conservative management with heart failure and antiarrhythmic medications was chosen. All drugs were discontinued when the patient was 5 years old. Since that time, the mass is stable and the patient is strictly asymptomatic. Conservative strategy seems to be acceptable in selected cases but close follow-up is mandatory.

Effects of flecainide therapy on inappropriate shocks and arrhythmias in catecholaminergic polymorphic ventricular tachycardia.

We report the case of a child with catecholaminergic polymorphic ventricular tachycardia, who had inappropriate electric shocks by an implantable cardioverter defibrillator and from recurrence of arrhythmias despite an appropriate ß-blocking treatment. An additional treatment by flecainide completely suppressed inappropriate shocks due to sinusal tachycardia and ventricular tachyarrhythmias. We briefly discuss the potentially interesting effect of flecainide in this specific arrhythmia.

Relation between red cell distribution width and clinical outcome after surgery for congenital heart disease in children.

Recent studies have reported a strong association between increased red cell distribution width (RDW) and the risk of adverse outcomes for adults with heart failure. This study investigated the association between preoperative RDW and postoperative clinical outcomes for children with cardiac disease. The relation between preoperative RDW and the length of postoperative stay was tested with 688 consecutive children undergoing surgery for congenital heart disease (CHD). The RDW was significantly higher in patients who died during the postoperative hospital stay (mean, 18.34 ± 4.69 vs 16.12 ± 2.84; p = 0.004). The risk of postoperative death was five times higher for patients with an RDW of 16% or more. In the general study population, RDW correlated with the intensive care unit (ICU) stay (p < 0.0001) and with the total hospital stay in the local population (p < 0.0001). The correlation between RDW and ICU stay was stronger for patients with acyanotic CHD (p < 0.0001) than for those with cyanotic CHD (p = 0.0007), and for the subpopulation of patients with acyanotic CHD and normal hemoglobin level (p < 0.0001) than for anemic patients with acyanotic CHD (p = 0.025). Preoperative RDW is a strong predictor of an adverse outcome in children undergoing surgery for CHD, especially in nonanemic patients, for whom it reflects an underlying inflammatory stress.

Long-term electrocardiographic follow-up after repair of tetralogy of Fallot.

BACKGROUND: Fallot patients with conduction disturbances are prone to sudden cardiac death. However, knowledge about long-term electrocardiographic changes after Fallot repair is limited. METHODS: Measurements were performed on electrocardiograms recorded preoperatively, postoperatively, and during annual follow-up in 35 Fallot patients included in three groups: G1 if they received no patch (n = 7), G2 if they received a transannular patch (n = 13), and G3 if they received a pulmonary homograft (n = 15). RESULTS: PR interval increased over the study period in all groups (Z-score: from 0.9 ± 1.1 to 1.3 ± 0.9 in G1, 0.9 ± 1.2 to 1.7 ± 1.6 in G2, and 0.7 ± 0.7 to 1.4 ± 1.3 in G3). The QRS duration increased during the follow-up at a rate of 1.78 msec/year in G1, 2.34 msec/year in G2 despite pulmonary valve replacement in 10 patients, and 1.81 msec/year in G3 despite conduit replacement in 9. At the later follow-up, the QRS duration was significantly increased (Z= 4.5 ± 3.6 in G1, 5.7 ± 1.4 in G2, and 4.6 ± 1.9 in G3). One patient in each group had QRS duration of 170 msec or longer and the one in G3 had a history of serious ventricular arrhythmia. Three patients had a QTc duration above 460 msec. CONCLUSIONS: Progressive conduction disorders are noted during long-term follow-up in Fallot patients who received transannular patch but also in those who received no patch or a pulmonary homograft. It suggests that volume overloading related to the transannular patch but also pressure overloading and myocardial injury related to surgery contribute to their development.

Pericarditis as a rare complication of pneumococcal pneumonia in a young infant.

Purulent pericarditis is an exceptionally rare complication of pneumococcal pneumonia in infants but a rapidly fatal disease if left untreated. A previously healthy 4-month-old boy presented at our emergency department with a 10-day history of fever and non-productive cough. No signs of heart failure or cardiac friction rub were evidenced. Chest radiography showed lobar pneumonia, right pleural effusion and cardiomegaly. Echocardiography revealed a massive pericardial effusion, and an emergency drainage was performed. Streptococcus pneumoniae grew up from purulent pericardial fluid and blood cultures. After intravenous antibiotherapy, the outcome was favourable. The introduction of the pneumococcal vaccine may favour an increase in the incidence of non-vaccine serotypes which most commonly cause empyaema and perhaps pericarditis. Therefore, pericarditis should always be considered a possible complication in patients with pneumococcal pneumonia and empyaema.

Prevalence of preoperative arrhythmias in children with delayed treatment of severe congenital heart disease.

AIM: Our aim was to determine the real importance of rhythm and conduction disorders in children with unoperated severe congenital heart disease. METHODS: Consecutive children with delayed treatment of severe congenital heart disease were prospectively studied for the occurrence of arrhythmias before any invasive investigation or surgical procedure was performed. RESULTS: All 168 children were in sinus rhythm. One hundred and fifty-eight patients (94%) had no significant preoperative findings. One child with double discordance had an intermittent complete atrioventricular block, and another one had a long QT syndrome. Children with severe ventricular dysfunction had paroxysmal atrioventricular re-entry tachycardia in 3 cases and abnormally frequent premature ventricular complexes in 3 other cases. Children with severe left atrial dilatation had periods of atrial ectopic tachycardia in one case and atrial fibrillation in another case. CONCLUSIONS: The prevalence of rhythm and conduction disorders is relatively low in children with delayed treatment of severe congenital heart disease. Only those with congenital heart disease classically combined with such disorders and those with prolonged severe ventricular dysfunction and/or atrial dilatation are at risk of developing significant arrhythmias and should undergo a preoperative assessment of arrhythmias.

Chronotropic impairment after surgical or percutaneous closure of atrial septal defect.

BACKGROUND: In previous studies, an attenuated heart rate response to exercise has been noted in patients after surgical closure of atrial septal defect. The aim of this study was to compare the prevalence of chronotropic impairment after surgical and percutaneous closure of atrial septal defect. METHODS: Thirty-eight pediatric patients who underwent a surgical (group A, n = 18) or transcatheter closure (group B, n = 20) of atrial septal defect in our institution were prospectively included in the study. Treadmill exercise testing was performed using the Bruce walking treadmill protocol to voluntary exhaustion, with continuous monitoring of heart rate and oxygen consumption. RESULTS: All the children were in sinus rhythm, and had normal values for peak oxygen uptake and endurance time. Exercise heart rate was significantly lower than normal in group A at the end of stage 2 (Z-score = -0.71 +/- SD 1.02), and in both groups at the end of stage 3 (Z-score = -2.06 +/- 1.76 in A and -1.00 +/- 0.71 in B) and at peak exercise (Z-score = -2.78 +/- 2.14 in A and -0.81 +/- 0.75 in B). However, the heart rate response to exercise was significantly less attenuated in group B than in group A. Moreover, maximal heart rate was <-2 SD in 8/18 surgical patients but in no patient of the group B. CONCLUSION: The chronotropic impairment is significantly less important after transcatheter closure of atrial septal defect than after surgical closure. It is an additional argument in favor of the interventional catheterization.

Congenital heart disease in 111 225 births in Belgium: birth prevalence, treatment and survival in the 21st century.

AIM: To investigate the birth prevalence, treatment modalities and short-term survival of children with congenital heart disease who were born in 2002. METHODS: We undertook a retrospective review of medical records of all patients who were born in 2002, and were diagnosed, treated and/or followed-up in one of the seven-paediatric cardiology programmes in Belgium. RESULTS: In 111 225 births, 921 children with congenital heart disease were detected, yielding a birth prevalence of 8.3 per 1000. The most frequently occurring conditions were ventricular septal defects (VSDs) (33%), ostium secundum atrial septal defects (18%) and pulmonary valve abnormalities (10%). Thirty-nine percent of the children either had a cardiosurgical operation or catheter intervention. In this study, 4% of the children died. The actuarial survival at 6 months and 1 year of age was 97% and 96%, respectively and remained stable after then. Compared to other heart defects, mortality was higher in univentricular physiology, pulmonary atresia with VSD, left ventricle outflow obstruction and tetralogy of Fallot. CONCLUSION: Survival of congenital heart disease is excellent and continued to improve in the early 21st century. New therapeutic options are increasingly used. This study provides baseline data for the longitudinal follow-up of this cohort.

Shoshin Beriberi and Severe Accidental Hypothermia as Causes of Heart Failure in a 6-Year-Old Child: A Case Report and Brief Review of Literature.

Severe accidental hypothermia has been demonstrated to affect ventricular systolic and diastolic functions, and rewarming might be responsible of cardiovascular collapse. Until now, there have been only a few reports on severe accidental hypothermia, none of which involved children. Herein, we describe here a rare case of heart failure in a 6-year-old boy admitted to the emergency unit owing to severe hypothermia and malnutrition. After he was warmed up (core temperature of 27.2°C at admission), he developed cardiac arrest, requiring vasoactive amines administration, and veno-arterial extracorporeal membrane oxygenation. Malnutrition and refeeding syndrome might have caused the thiamine deficiency, commonly known as beriberi, which contributed to heart failure as well. He showed remarkable improvement in heart failure symptoms after thiamine supplementation. High-dose supplementation per os (500 mg/day) after reconstitution of an adequate electrolyte balance enabled the patient to recover completely within 2 weeks, even if a mild diastolic cardiac dysfunction persisted longer. In conclusion, we describe an original pediatric case of heart failure due to overlap of severe accidental hypothermia with rewarming, malnutrition, and refeeding syndrome with thiamine deficiency, which are rare independent causes of cardiac dysfunction. The possibility of beriberi as a cause of heart failure and adequate thiamine supplementation should be considered in all high-risk patients, especially those with malnutrition. Refeeding syndrome requires careful management, including gradual electrolyte imbalance correction and administration of a thiamine loading dose to prevent or correct refeeding-induced thiamine deficiency.

Epidemiology and outcome of tachyarrhythmias in tertiary pediatric cardiac centers.

INTRODUCTION: Little is known about the real importance of pediatric arrhythmias. METHODS: We analyzed the epidemiology, presentation and outcome of all clinically relevant tachyarrhythmias followed up in our pediatric institutions from 1995 to 2006. RESULTS: A total of 250 cases were identified. The mean age +/- SD at diagnosis was 4.7 +/- 5.3 years, 45 cases were neonatal (18%). Supraventricular arrhythmias were noted in 210 children (84%), ventricular arrhythmias in 40 (16%). The most frequent symptoms were palpitations (n = 71) and syncope (n = 48) in older children, as well as monitoring of diseases (n = 62) and heart failure (n = 49) in younger patients. Recurrence was noted under or after therapy in 75 cases, mostly in cases diagnosed beyond infancy. At long-term follow-up, 169 patients have no recurrence without treatment (of whom 34 had required catheter ablation), 71 are under therapy and 10 died. CONCLUSION: Supraventricular arrhythmias in younger children are often an incidental diagnosis, respond to antiarrhythmic therapy and have a high incidence of resolution. In older children with supraventricular arrhythmias and in those with ventricular arrhythmias, delayed diagnosis or misdiagnosis is not rare, the arrhythmias are unlikely to resolve spontaneously and long-term antiarrhythmic treatment or catheter ablation is necessary.

Epidemiology of heart failure in a tertiary pediatric center.

BACKGROUND: In contrast to the adult age group, epidemiologic studies on heart failure (HF) in the pediatric population are lacking. The aim of this prospective study was to analyze the epidemiology of HF during infancy and childhood. METHODS: Of the 1,196 children with congenital and acquired heart diseases, primarily diagnosed during a 10-y period in one hospital, we identified those patients who developed HF. RESULTS: Within 10 y, 1,196 children with heart disease were indexed. Heart failure occurred in 124 of these patients (10.4%): 64 out of 1,031 children with congenital heart diseases (6.2%), 13 out of 96 children with rhythm or conduction disturbances (13.5%), 23 out of 39 children with acquired heart diseases (59.0%), and 24 out of 30 children with cardiomyopathies (80.0%). Heart failure occurred in 72 cases (58.1%) during the first year of life. The incidence in infancy was much more pronounced for congenital heart diseases than for other cardiac conditions. The mortality associated with HF and its cause was also lower for children with congenital heart disease (4.7%) than for the other cardiac conditions (8.7%, 23.0%, and 25.0%, for acquired heart diseases, rhythm disturbances, and cardiomyopathies, respectively). CONCLUSION: Clear differences exist between HF in infants and HF in children. Our study supports the observation that congenital heart disease is the most common causative factor of HF during infancy. Older children with HF are more likely to have acquired heart diseases, cardiomyopathies, and arrhythmias, and these conditions have a considerable mortality.

Cardiac syncope in pediatric patients.

OBJECTIVE: To assess the epidemiology of cardiac syncope in children and evaluate the guidelines on its management. MATERIAL AND METHODS: We analyzed the etiology to syncope and diagnostic workup in consecutive pediatric patients presenting with syncope in our emergency departments or cardiac outpatient clinics between 1997 and 2005, and who were subsequently diagnosed as having cardiac syncope. RESULTS: A primary cardiac cause was identified in 11 syncopal patients presenting to the emergency room and 14 patients to the cardiac clinic: supraventricular tachyarrhythmia in 9, ventricular tachyarrhythmia in 10, pacemaker dysfunction in 2, and isolated cases of sick sinus syndrome, hypoxic spell, hypertrophic cardiomyopathy, and primary pulmonary hypertension. Some elements suggested potential cardiac disease as a cause of syncope in all cases. The resting electrocardiogram and the echocardiogram were interpreted as positive and relevant to the diagnosis in 17 and 3 patients, respectively. Exercise electrocardiogram and Holter recording provided diagnostic information previously not seen on the resting electrocardiogram in six and three patients, respectively. Three children have died and one child has neurological sequelae following resuscitation. CONCLUSION: Our data support the premise that careful history taking with special focus on the events leading up to syncope, as well as a complete physical examination, can guide practitioners in discerning which syncopal children need further cardiac investigations.

Use of the Internet by parents of children with congenital heart disease.

OBJECTIVE: We sought to document the prevalence of Internet access and usage patterns among families with children diagnosed with congenital heart disease. MATERIAL AND METHODS: A questionnaire was administered to the parents of 509 unselected children with congenital heart disease attending the outpatient cardiac clinic. RESULTS: 389 questionnaires were completed. The most common source for acquiring medical information was via the paediatric cardiologist (83%), the family doctor (53%), the general paediatrician (40%) and the Internet (15%). Among the 238 families with Internet access (62%), the most common locations for Internet access were home (74%) and work (33%), and 84 used the Internet to obtain information related to their child's cardiac diagnosis, mostly at the time of diagnosis. Two-thirds of these families considered finding information related to the cardiac diagnosis as easy or very easy, and found this information helpful or very helpful. CONCLUSION: A quarter of the families uses the Internet to find information about their child's congenital heart disease. Internet use in this patient population is expected to increase and our vigilance in providing accurate references and in identifying inaccurate information is of paramount importance.