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.

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.

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.

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.

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.

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.

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.

Pacemaker lead-induced tricuspid stenosis treated with percutaneous valvotomy.

BACKGROUND: Tricuspid stenosis is an uncommon complication of ventricular pacemaker electrode implantation, with few cases reported in the literature. CASE: We present an 18-year-old male who developed severe tricuspid stenosis 15 years after endocardial VVI pacemaker implantation for complete AV block following a surgically repaired ventricular septal defect. CONCLUSION: In this case we have shown that percutaneous balloon valvuloplasty was performed and successful in treating this complication.

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.

Retrograde transcatheter closure of anterior mitral valve leaflet perforation.

Transcatheter closure of mitral valve leaflet perforation is a very rarely performed and a difficult procedure for repairing the defect. Herein, we are the first to report on both the safety and feasibility of percutaneous retrograde transcatheter closure of anterior mitral valve leaflet perforation with an AMPLATZER™ Duct Occluder II (6 mm × 6 mm, ADO II; Abbott Vascular, IL, USA) device in a 19-year-old patient with a severe mitral valve regurgitation following cardiac surgery.

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.

General Concepts in Adult Congenital Heart Disease.

Congenital heart disease in adults (adult congenital heart disease) is a growing burden for healthcare systems. While infant mortality due to congenital heart disease in the last four decades decreased by almost 3-fold, adult congenital heart disease prevalence increased by more than 2-fold in United States. Adult congenital heart disease prevalence is expected to increase steadily until 2050 in projections. Adult congenital heart disease is a multifaceted problem with many dimensions. This manuscript aims to provide an overview of the common adult congenital heart diseases and summarize important points in management of these diseases with possible problems and complications that the patients and the physicians face.

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.

Radiofrequency catheter ablation of left-sided accessory pathways via retrograde aortic approach in children.

BACKGROUND: We aimed to analyze the results of retrograde aortic radiofrequency catheter ablation of left-sided accessory pathways in children. METHODS: Between January 2010 and September 2014, 25 children who underwent left-sided accessory pathway ablation with a retrograde aortic approach were evaluated retrospectively. RESULTS: The mean age of the patients was 11.09±3.71 years. Seventeen patients were male (68%). The mean procedure and fluoroscopy times were 71.54±21.05 min and 31.42±19.57 min, respectively. Radiofrequency energy was delivered with 41.38±15.32 W at 52.38±5.45 °C. Sixteen patients (64%) presented with manifest preexcitation and, 9 had concealed accessory pathways. The location of accessory pathway was left lateral in 16 patients, posteroseptal in 5, left anterolateral in 2, and left posterolateral and left posterior in the remaining 2. The acute success rate was 96%. The patients were followed for a mean of 16.68±18.01 months. There were 2 recurrences. No major complications were observed in the periprocedural period. One patient had groin hematoma, another one had transient severe headache and vomiting. Trivial mitral regurgitation was noted in a patient, which remained the same throughout follow-up. None of the patients developed new aortic regurgitation, pericardial effusion, or thrombi at the site of ablation. CONCLUSIONS: The retrograde aortic approach can be safely employed with a high success rate for ablation of left-sided accessory pathways in children.

The Impact of Rapid Left Ventricular Pacing during Pediatric Aortic Valvuloplasty on Postprocedural Aortic Insufficiency.

OBJECTIVE: This study aims to determine efficacy and safety of rapid left ventricular pacing (RLVP) during balloon aortic valvuloplasty and effect on development of postprocedural aortic insufficiency (AI) in children. DESIGN: This is a retrospective comparison of 56 children (mean age 18.3 month; 1 day-15 years of age) who underwent valvuloplasty by using RLVP with standard method (without pacing) during same time period (74 children; mean age 12.6 month; 1 day-18 years of age). RESULTS: The systolic valvular gradient decreased from a mean 67 ± 20.4 mm Hg (26-120 mm Hg) before the procedure to a mean 27.6 ± 17.8 mm Hg (0-120 mm Hg) after the procedure. Procedure failed in two of the patients (3.6%) who received the RLVP; only two patients developed severe AI. Among those patients who underwent the standard method procedure failed in 6 patients (8.3%), whereas severe AI was seen in 15 (20.2%). RLVP decreased the incidence of postprocedure severe AI significantly (P < .01) but it had no effect on the gradient reduction of the procedure (P > .05). RLVP did not change the procedure duration, but decreased the duration of fluoroscopy significantly (P < .01). CONCLUSION: RLVP can be used effectively and safely for pacing during balloon aortic valvuloplasty procedures in all age groups; it decreases procedural failure rate, eases the procedure and prevents the development of AI through the stabilization of the balloon.

Is transesophageal electrophysiologic study valuable in children with successful radiofrequency ablation of supraventricular tachycardia on follow-up for recurrence?

OBJECTIVE: The aim of this study was to evaluate the efficacy of transesophageal electrophysiologic study (TEEPS) for the determination of supraventricular tachycardia (SVT) recurrences in symptomatic and asymptomatic children after successful radiofrequency ablation (RFA) for SVT. METHODS: A total of 66 patients who underwent TEEPS after successful RFA were included. The demographic features, symptoms of the patients, and the characteristics of the recurrences induced by TEEPS were evaluated. The arrhythmia types induced during RFA were compared with those induced by TEEPS in terms of the compatibility of the diagnosis. RESULTS: Forty-two (63.6%) girls and 24 (36.4%) boys with a mean age of 11.8±3.4 years were followed-up for 44.1±15.7 months. The average time between RFA and TEEPS was 5.2±5.9 months. The diagnoses during RFA were atrioventricular nodal reentrant tachycardia (AVNRT) in 47 of 66 patients, atrioventricular reentrant tachycardia (AVRT) in 18 of 66 patients, and ectopic atrial tachycardia in 1 of 66 patients. SVT was induced by TEEPS in 2 of 25 symptomatic and 5 of 41 asymptomatic patients. The SVT inducibility rate was 5.5% (1/18) and 12.7% (6/47) in patients with AVRT and AVNRT, respectively. In addition, 85.7% (6/7) of all recurrences occurred within 3.5 months. The recurrences as AVNRT in 2 of 25 symptomatic patients occurred in the first month after RFA. AVNRT in 4 of 41 and AVRT in 1 of 41 asymptomatic patients were induced within 3.5 months and 15 months, respectively. CONCLUSION: TEEPS seems to be a valuable screening and diagnostic method for the determination of recurrence in symptomatic and asymptomatic children who underwent successful RFA.