Left ventricular dysfunction in a child with asymptomatic pre-excitation: Cure with catheter ablation.

Wolff-Parkinson-White syndrome, is known to cause left ventricular dysfunction or dilated cardiomyopathy secondary to sustained tachycardia in infants and children. However, left ventricular dysfunction secondary to pre-excitation related abnormal ventricular activation has been reported in a limited number of cases. This condition should be recognized early, as catheter ablation of the accessory pathway can permit rapid ventricular function improvement. In this paper, we present a 2.5-year-old patient diagnosed with tachycardia-free Wolff-Parkinson-White syndrome with a right free wall accessory pathway and depressed cardiac function, whose left ventricular function is completely restored after successful catheter cryoablation.

Two decades of experience on ablation in children with Ebstein's anomaly.

INTRODUCTION: Accessory pathways are commonly seen due to delamination of tricuspid valve leaflets. In addition to accessory pathways, an enlarged right atrium due to tricuspid regurgitation and incisional scars creates substrates for atrial re-entries and ectopic tachycardia. We sought to describe our experience with catheter ablation in children with Ebstein's anomaly. METHODS AND RESULTS: During the study period, of 89 patients diagnosed with Ebstein's anomaly, 26 (30.9%) of them who underwent 33 ablation procedures were included in the study. Accessory pathways were observed in the majority of procedures (n = 27), whereas atrial flutter was observed in five, atrioventricular nodal reentrant tachycardia in five, and atrial tachycardia in two procedures. Accessory pathways were commonly localised in the right posteroseptal (n = 10 patients), right posterolateral (n = 14 patients), septal (n = two patients), and left posteroseptal (n = one patient) areas. Multiple accessory pathways and coexistent arrhythmia were observed in six procedures. All ablation attempts related to the accessory pathways were successful, but recurrence was observed in five (19%) of the ablations. Ablation for atrial flutter was performed in five patients; two of them were ablated successfully. One of the atrial tachycardia cases was ablated successfully. CONCLUSIONS: Ablation in patients with Ebstein's anomaly is challenging, and due to nature of the disease, it is not a rare occasion in this group of patients. Ablation of accessory pathways has high success, but also relatively high recurrence rates, whereas ablation of atrial arrhythmias has lower success rates, especially in operated patients.

Platypnea-orthodeoxia syndrome in a child: relief of symptoms after transcatheter closure of patent foramen ovale.

Platypnea-orthodeoxia syndrome is a rare clinical entity characterised by positional dyspnoea and arterial desaturation while in the upright position, the symptoms generally occurring in adults. We describe a 12-year-old girl diagnosed with platypnea-orthodeoxia syndrome associated with patent foramen ovale. The symptoms resolved following percutaneous patent foramen ovale closure.

Calmodulin mutations and life-threatening cardiac arrhythmias: insights from the International Calmodulinopathy Registry.

AIMS: Calmodulinopathies are rare life-threatening arrhythmia syndromes which affect mostly young individuals and are, caused by mutations in any of the three genes (CALM 1-3) that encode identical calmodulin proteins. We established the International Calmodulinopathy Registry (ICalmR) to understand the natural history, clinical features, and response to therapy of patients with a CALM-mediated arrhythmia syndrome. METHODS AND RESULTS: A dedicated Case Report File was created to collect demographic, clinical, and genetic information. ICalmR has enrolled 74 subjects, with a variant in the CALM1 (n = 36), CALM2 (n = 23), or CALM3 (n = 15) genes. Sixty-four (86.5%) were symptomatic and the 10-year cumulative mortality was 27%. The two prevalent phenotypes are long QT syndrome (LQTS; CALM-LQTS, n = 36, 49%) and catecholaminergic polymorphic ventricular tachycardia (CPVT; CALM-CPVT, n = 21, 28%). CALM-LQTS patients have extremely prolonged QTc intervals (594 ± 73 ms), high prevalence (78%) of life-threatening arrhythmias with median age at onset of 1.5 years [interquartile range (IQR) 0.1-5.5 years] and poor response to therapies. Most electrocardiograms (ECGs) show late onset peaked T waves. All CALM-CPVT patients were symptomatic with median age of onset of 6.0 years (IQR 3.0-8.5 years). Basal ECG frequently shows prominent U waves. Other CALM-related phenotypes are idiopathic ventricular fibrillation (IVF, n = 7), sudden unexplained death (SUD, n = 4), overlapping features of CPVT/LQTS (n = 3), and predominant neurological phenotype (n = 1). Cardiac structural abnormalities and neurological features were present in 18 and 13 patients, respectively. CONCLUSION: Calmodulinopathies are largely characterized by adrenergically-induced life-threatening arrhythmias. Available therapies are disquietingly insufficient, especially in CALM-LQTS. Combination therapy with drugs, sympathectomy, and devices should be considered.

Transcatheter baffle leak closure via an amplatzer septal occluder in a senning-operated child.

Senning operation is a surgical treatment for transposition of great arteries that can be complicated by post-procedural atrial or caval baffle leaks. We present a 6-year-old boy with a history of Senning repair for transposition of great arteries, who developed a pulmonary venous baffle leak. Percutaneous baffle leak closure was successfully performed using an Amplatzer septal occluder.

Midterm Results of Implantable Cardioverter Defibrillators in Children and Young Adults from a Single Center in Turkey.

BACKGROUND: Despite concerns about complications with the implantable cardioverter defibrillator (ICD), it is effective for the prevention of sudden cardiac death (SCD). We aimed to analyze our midterm experience with ICD in children and young adults. METHODS: This retrospective study included patients who were implanted with an ICD between 2001 and 2014. Demographic characteristics, clinical information, shock features, and complications for all patients with ICD were analyzed. The study population was divided into two groups: early-era patients implanted before 2008, and late-era patients implanted after 2008. RESULTS: Sixty-nine patients (median age: 12 years, median follow-up: 52 months) were implanted with an ICD. Diagnostic categories were channelopathy (56.6%), cardiomyopathy (36.2%), congenital heart disease (5.8%), and other (1.4%). We performed implantation for primary prevention in 66.6% (39.3% in early-era patients and 85.4% in late-era patients). Thirty-one (44.9%) received 139 appropriate shocks (66% of total shocks) while 14 (20.2%) received 71 inappropriate shocks. However, there was no statistically significant difference in the use of appropriate shocks in the primary (66.7%) versus the secondary (72.2%) prevention groups. The incidence of appropriate and inappropriate shock was 66.7% and 33.3% in the primary prevention group, and 72.2% and 27.8% in the secondary prevention group, respectively. Two patients died, although only one death was the result of a lead problem. CONCLUSIONS: Although lead integrity problems, inappropriate shocks, and infections are significant issues, ICD therapy appears to be a safe, effective, and necessary option for the prevention of SCD in both children and young adults.

Comparison of Transesophageal and Intracardiac Electrophysiologic Studies for the Diagnosis of Childhood Supraventricular Tachycardias.

In this study, we aimed to compare the results of transesophageal electrophysiologic studies (TEEPS) and intracardiac electrophysiologic studies (IEPS) in a cohort of pediatric patients with SVTs. The medical records of children aged between 0 and 18 years who underwent TEEPS between January 2007 and June 2012 were systematically reviewed, and those without pre-excitation and who underwent subsequent IEPS were identified. Post-procedural diagnoses were compared for compatibility. A total of 162 patients were included in the study with a mean age at diagnosis 11.6 ± 3.6 years. Tachycardia was induced in 152 patients by TEEPS and in 154 patients by IEPS. Overall, in 147 patients, tachycardia was induced by both TEEPS and IEPS. Diagnoses were compatible in 135 out of 147 patients (91.8 %). Nine out of the 12 patients with discrepant results were diagnosed with atrioventricular-reentrant tachycardia (AVRT) and three with atrioventricular nodal reentrant tachycardia (AVNRT) after TEEPS. Following IEPS, TEEPS diagnosis of AVRT was revised to typical AVNRT in 5 patients and atypical AVNRT in 4 patients. Two of the 3 patients who were diagnosed as having AVNRT following TEEPS were confirmed to have atrial tachycardia after IEPS, while the other patient was diagnosed with AVRT. Tachycardia terminated spontaneously in 3 patients, while overdrive pacing was attempted to terminate the tachycardia in 149 patients, with a success rate of 93.2 % (139/149). The diagnostic compatibility between TEEPS and IEPS is quite high. A diagnostic discrepancy mostly occurs in patients diagnosed with AVRT by TEEPS, and the possibility of atypical AVNRT should be considered in patients with a VA ≥70 ms to avoid such discrepancies.

Sensitivity of transesophageal electrophysiologic study in children with supraventricular tachycardia on electrocardiography.

INTRODUCTION: The aim of this study was to evaluate the inducibility of tachycardia by transesophageal electrophysiologic study (TEEPS) in patients with documented supraventricular tachycardia (SVT) on electrocardiography and to investigate the accuracy of TEEPS records by comparing with intracardiac electrophysiologic study (IEPS). MATERIAL AND METHODS: The TEEPS records of patients having documented electrocardiography during SVT were reviewed. The results of TEEPS in 43 of 85 patients were compared with results of IEPS for compatibility of diagnosis. RESULTS: A total 85 patients, 46 male and 39 female, mean weight 35.1 kg (36-87), aged 1 month-17 years, were included. Tachycardia was induced by TEEPS in 79 of 85 patients with documented electrocardiography (sensitivity 92.9%). IEPS for diagnosis or ablation was conducted in 40 patients having inducible tachycardia and three of six who had no inducible tachycardia by TEEPS. Tachycardia was induced by IEPS in 39 of 40 (97.5%) patients who had inducible tachycardia and two of three who had no inducible tachycardia by TEEPS. Mechanisms of tachycardias were similar in 97.5% of patients (37/39) who had inducible tachycardia in TEEPS and IEPS. One of the patients with atrioventricular reentry tachycardia by TEEPS was diagnosed as atrioventricular nodal reentry tachycardia (AVNRT) and the other one was diagnosed as atypical AVNRT and atrial tachycardia by IEPS. CONCLUSION: The rates of inducibility and mechanisms of tachycardias by TEEPS in children having documented SVT were similar with those obtained from IEPS.

The effect of 3D modeling on family quality of life, surgical success, and patient outcomes in congenital heart diseases: objectives and design of a randomized controlled trial.

BACKGROUND: Understanding the severity of the disease from the parents' perspective can lead to better patient outcomes, improving both the child's health-related quality of life and the family's quality of life. The implementation of 3-dimensional (3D) modeling technology in care is critical from a translational science perspective. AIM: The purpose of this study is to determine the effect of 3D modeling on family quality of life, surgical success, and patient outcomes in congenital heart diseases. Additionally, we aim to identify challenges and potential solutions related to this innovative technology. METHODS: The study is a two-group pretest-posttest randomized controlled trial protocol. The sample size is 15 in the experimental group and 15 in the control group. The experimental group's heart models will be made from their own computed tomography (CT) images and printed using a 3D printer. The experimental group will receive surgical simulation and preoperative parent education with their 3D heart model. The control group will receive the same parent education using the standard anatomical model. Both groups will complete the Sociodemographic Information Form, the Surgical Simulation Evaluation Form - Part I-II, and the Pediatric Quality of Life Inventory (PedsQL) Family Impacts Module. The primary outcome of the research is the average PedsQL Family Impacts Module score. Secondary outcome measurement includes surgical success and patient outcomes. Separate analyses will be conducted for each outcome and compared between the intervention and control groups. CONCLUSIONS: Anomalies that can be clearly understood by parents according to the actual size and dimensions of the child's heart will affect the preoperative preparation of the surgical procedure and the recovery rate in the postoperative period.

Use of urinalysis as a screening tool for asymptomatic infants.

AIM: The utility of screening urinalysis in asymptomatic children has been questioned based on studies done in school-age children or adolescents. The American Academy of Pediatrics (AAP) recommended to abandon this screening in 2007 but many paediatricians perform it at some point during childhood. Thus, we aimed to investigate usefulness of screening urinalysis during infancy. METHODS: We retrospectively reviewed results of screening urinalysis done in infants at 6-18 months of age who had regular care since birth at our centre. Infants with an ICD-10 (International Classification of Diseases, Tenth Revision) diagnostic code for routine child health exam (Z00.1) and a urinalysis requested with this code on the same date were included. RESULTS: A total of 683 infants met the inclusion criteria. 44 (6%) had an abnormal urinalysis. The most common abnormality (n = 39, 5,7%) was pyuria. Of these 39 babies, 5 had a repeat urinalysis only, 18 had a repeat urinalysis with urine culture, and 16 had a urine culture alone. Six patients had positive culture results and were given antibiotic treatment. All six babies who received treatment had normal ultrasound and two patients had a voiding cystourethrography, which were also normal. The other abnormalities (n = 5) detected were microscopic hematuria and proteinuria. Repeat urinalyses of these patients were normal. CONCLUSION: Screening urinalysis results were abnormal in 6% of the babies, but in 86% of those, abnormalities were transient. Only <1% had positive culture results. These data add to the evidence that screening urinalysis during infancy is unjustified supporting the AAP 2007 recommendations.

Interventional cardiac catheterization in neonates and premature infants with congenital heart disease: a single center experience.

BACKGROUND: The increased survival of patients with congenital heart disease over the last three decades has been associated with improvements in diagnosis and treatment. This study aimed to evaluate therapeutic interventional catheterization, outcomes and complications of these procedures in neonates and premature infants. METHODS: In this study, therapeutic catheterization procedures performed on neonates and premature infants with congenital heart disease at a university hospital between February 2000 and October 2019 were retrospectively evaluated. RESULTS: A total of 322 procedures were performed on 279 neonates and 26 premature infants. Of the patients, 217 (67.4%) were male. The median age of the patients was 8 days (interquartile range [IQR] 2-20) and the median body weight was 3050 g (IQR 2900-3600). The most common procedures were balloon atrial septostomy, balloon aortic angioplasty, balloon pulmonary valvuloplasty and balloon aortic valvuloplasty (35.4%, 20.8%, 18.3% and 12.4% respectively). The most common diagnoses were transposition of the great arteries, coarctation of the aorta, pulmonary stenosis and aortic stenosis (26.7%, 19.3%, 15.2% and 11.5% respectively). Most procedures, 274 (85.1%), were successful. Complications were observed in 74 procedures (23%). Of these complications, 45 (14%) were minor and 29 (9%) were major. The most common complication was transient dysrhythmia (6.9%). There was no significant relationship between body weight, age and the rate of complications. However, longer procedure time and fluoroscopy time were associated with higher complication rates (p < 0.05). Four procedurerelated deaths were observed. CONCLUSION: Procedure-related complications are higher in the neonatal period. Although the complication rate varies according to the type of procedure, longer fluoroscopy time and procedure duration are associated with an increased complication rate. Procedures performed with the right indications, appropriate equipment and by experienced teams will play a key role in reducing complication rates.

Isolated right superior vena cava draining into the left atrium.

A 10-year-old male was admitted to our institution with complaints of mild cyanosis and dyspnea. Transthoracic echocardiography and angiography revealed a right superior vena cava (SVC) draining into the left atrium. At the time of surgery, the right SVC was connected to the right atrium.

Severe tricuspid regurgitation secondary to dislodgement of the atrial loop into the right ventricle: an unusual complication of pacemaker implantation in a young adult.

Transvenous pacemaker leads may impair tricuspid valve function. Severe tricuspid regurgitation due to leaflet adhesion to the pacemaker lead has not been reported in a young adult patient in the literature. Our patient underwent a transvenous pacemaker implantation for symptoms of bradycardia. An atrial loop was created in the right atrium for future growth. After 10 years of follow-up, the patient was seen with severe tricuspid regurgitation and enlarged right heart structures due to migration of the atrial loop of the pacemaker lead into the right ventricle and adhesion of the lead to the tricuspid valve. Cardiac surgery and epicardial pacing was the chosen procedure to solve this problem. The venous system and right heart valves should be carefully observed during the follow-up of children who underwent transvenous pacing.

Catheter fragment embolization: a rare yet serious complication of catheter use in pediatric oncology.

Embolization is a rare but serious complication of venous central catheters in pediatric oncology. The reported cases in the literature are due in common to catheter ruptures. The most common cause is constant compression of the costoclavicular arch, known as "pinch-off" syndrome. We report a seven-year-old boy in whom embolization occurred as a late complication. Difficulty in the dissection of dense collagen periportal fibrosis was the main problem during the extraction session. The embolization occurred 10 months later. In an elective setting, percutaneous retrieval techniques were used successfully to extract the catheter fragments, and full recovery was achieved.

Analysis of idiopathic ventricular tachycardia in childhood.

Idiopathic ventricular tachycardia (VT) is a relatively benign and rare form of VT. It is seen in young people without demonstrable cardiac pathology. The aim of our study was to review the clinical picture of idiopathic VT, before evaluating the indications for antiarrhythmic treatment and the efficacy of radiofrequency ablation (RFA). The notes of patients diagnosed with idiopathic VT in the last 13 years (n: 22) were included in the study. The median age of onset was 11 years (1 month-16 years). We evaluated the findings regarding the diagnosis, treatment and prognosis of these patients. The most common initial symptom was palpitation, in 15 cases. Five children with idiopathic VT were symptom-free. VT was of right ventricular origin in 10 patients and left ventricular origin in 8 patients. Beta-blockers were the mainstay of medical treatment in right VT and calcium channel blockers (Ca-channel blocker) were mostly used in left VT cases. The success rate of RFA was 57% in right VT and 100% in left VT. The median follow-up was 41 months (9 months-60 months), and all patients are alive currently with no symptoms. VT without demonstrable cardiac pathology is associated with a good prognosis. Treatment is unnecessary for asymptomatic non-sustained VT. RFA is useful in patients with symptomatic drug-refractory idiopathic VT arising from the left or right ventricle.

Evaluation of the relationship between cardiopulmonary exercise test findings and clinical status in children and adolescents with congenital heart disease.

BACKGROUND: The cardiopulmonary exercise test is accepted as a helpful diagnostic tool in risk stratification, evaluation of prognosis, and guiding treatment modality in adults with congenital heart disease. In this study, we present our experience with the use of cardiopulmonary exercise test in children with congenital heart disease in different physiological and anatomical classifications. METHODS: In this retrospective study, 25 children and adolescents who applied to the pediatric cardiology outpatient clinic between 2017 and 2020 with the diagnosis of different types of congenital heart disease were included. Demographic characteristics, electrocardiogram, echocardiogram, cardiopulmonary exercise test, spirometry, pro-BNP values, and in selected 20 patients; cardiac MRI data were examined. The modified Ross classification was used for heart failure grading. RESULTS: The mean age of the patients was 14.8 ±2.39 years. Fifteen (60%) of the patients were male and 10 (40%) were female. In the modified Ross classification, patients in group I-II had significantly higher maximum exercise time, heart rate reserve %, peak VO2, and VO2/kg values compared to those in group III (p=0.026, p=0.007, p=0.043, p= 0.018, respectively). Cardiopulmonary exercise test and spirometry values obtained from the patients were evaluated in the light of clinical and other laboratory findings, and surgical/interventional treatment was decided for 4 patients with the use of these test results. CONCLUSIONS: Cardiopulmonary exercise test is a useful noninvasive diagnostic tool in guiding the treatment decision and predicting the prognosis of pediatric patients with congenital heart disease, who have borderline symptoms.

Transcatheter closure of atrial septal defects with transthoracic echocardiography.

OBJECTIVES: The aim of this study is to evaluate our clinical experience using an Amplatzer septal occluder for catheter closure of a secundum atrial septal defect under transthoracic echocardiography guidance without general anaesthesia. METHODS: Patients eligible for transcatheter atrial septal defect closure were selected using transthoracic echocardiography. The largest defect diameter measured in different views was selected as the reference diameter. All procedures were performed under conscious sedation with fluoroscopic and transthoracic echocardiographic guidance. RESULTS: Between November, 2006 and December, 2009 a secundum-type atrial septal defect was closed with the Amplatzer septal occluder in 40 patients with transthoracic echocardiographic guidance. The mean age and weight were 7.9 years and 26.9 kilograms, respectively. The mean atrial septal defect diameter was 11.4 millimetres, total septal diameter was 38.5 millimetres, and the mean device diameter and the difference between device and atrial septal defect diameter were 12.6 and 1.2 millimetres, respectively. There were no major complications. The mean follow-up time was 14.8 months. CONCLUSION: In selected cases, in which the defects are small and the rims are adequate and transthoracic echocardiography provides high image quality, transthoracic echocardiography can be substituted with transoesophageal echocardiography. The ratio of defect size to total septal diameter can be used as a guide for patient selection; those that have a value of 0.33 or greater can be considered eligible for closure with transthoracic echocardiography. However, transthoracic echocardiography should not be used when there are large or multiple defects, or the rims are thin and soft and the image resolution is inadequate.

Pulmonary arterial hypertension associated with congenital heart disease: lessons learnt from the large Turkish Nationwide Registry (THALES).

Pulmonary hypertension is a group of diseases, including pulmonary arterial hypertension associated with congenital heart disease (APAH-CHD), characterized by progressive deterioration in pulmonary hemodynamics associated with substantial morbidity and mortality risk. THALES is a national multicenter, prospective observational registry, providing data on patients with APAH-CHD. The study comprised APAH-CHD patients (>3 months of age) with confirmed diagnosis of right heart catheterization or echocardiographic findings. Initial and follow-up data were collected via regular hospital visits. Descriptive statistics are used for definitive purposes. Overall, 1034 patients aged 3 months-79 years (median 11.2 [Q1-Q3: 2.2-24.3] years) with APAH-CHD were enrolled at 61 centers, 50.3% being retrospectively enrolled. Most had either Eisenmenger's syndrome (49.2%) or systemic-to-pulmonary shunts (42.7%). Patients were mostly in functional class I-II at the time of diagnosis (46.6%). Mean 6-min walk distance (6MWD) was 369 ± 120 m. Mean pulmonary arterial pressure was 54.7 ± 22.2 mmHg for the whole group, and was highest in patients with Eisenmenger's syndrome. Targeted therapies were noted in 398 (38.5%) patients (monotherapy in 80.4%). Follow-up data were available in 506 patients. Survival at 140 months was 79% and was associated with baseline 6MWD >440 m (p = 0.009), brain natriuretic peptide level < 300 ng/L (p < 0.001). Follow-up 6MWD >165 m (p < 0.0001), brain natriuretic peptide level <300 ng/L (p = 0.031), and targeted therapies (p = 0.004) were also predictive of survival. THALES is the largest registry dedicated to APAH-CHD to date and provides important contributions on demographics, clinical characteristics, and gaps in disease management.

Midterm experience with implantable cardioverter-defibrillators in children and young adults.

AIMS: This single-centre study was undertaken to review our experience with implantable cardioverter-defibrillator (ICD) implantation in children with relatively different aetiologies. METHODS AND RESULTS: We retrospectively reviewed the records of the paediatric patients who underwent ICD implantation between October 2001 and December 2008. The data of these patients were collected by reviewing the patients' medical records and computerized departmental pacemaker databases. A total of 28 patients who underwent ICD implantation during this period were included in this study. The median age was 12 years and median weight was 32 kg. Most of the patients had ion-channel diseases (n = 13) or cardiomyopathy (n = 11). Devices were implanted for either secondary (n = 22) or primary (n = 6) prevention. The selected ICD generator type was single chamber in 22 patients, dual chamber in 5 patients, and biventricular in 1. Nineteen patients received 122 shocks. Fifteen of 22 patients (68.2%) from the secondary prevention group and 2 of 6 patients (33.3%) from the primary prevention group experienced at least one appropriate shock during a median period of 11.3 months (range: 4 days-6.5 years). Forty-two inappropriate shocks were delivered in seven (31.8%) patients from the secondary prophylaxis group during a median period of 11.3 months. The most important reason for inappropriate shocks was T-wave oversensing. In six patients, lead-related acute or chronic complications occurred. CONCLUSION: The ICD was safe and effective in interrupting malignant arrhythmias in children and adolescents with a high risk of sudden cardiac death. However, the occurrence of lead complications is significant. The incidence of therapies delivered by the device, with appropriate and inappropriate shocks, was high and interfered with the quality of life. The most important reason for inappropriate shocks was T-wave oversensing. Careful programming is mandatory to reduce the inappropriate shocks.