Day 2 :
Keynote Forum
Ofer Binah
Israel Institute of Technology, Israel
Keynote: Molecular characterization and functional properties of induced pluripotent stem cells-derived cardiomyocytes from healthy and diseased individuals. Models for investigating inherited cardiac diseases
Time : 09:05-09:55
Biography:
Ofer Binah works at the Technion – Israel Institute of Technology, Israel. He is a Professor in the Department of Physiology, Biophysics and Systems Biology at the Ruth and Bruce Rappaport Faculty of Medicine. He has published more than 108 articles in reputed journals.
Abstract:
In view of the therapeutic potential of cardiomyocytes derived from human induced pluripotent stem cells (iPSC-CM), our overall goal is to investigate their molecular characteristics, functional properties related to the excitation-contraction coupling (e.g., [Ca2+]i handling), pacemaker function and underlying ion currents, the effects of b-adrenergic stimulation, and responsiveness to common modifiers of cardiac function (e.g. If blocker). The iPSC clones we investigate are derived from human dermal fibroblasts or hair keratinocytes, and reprogramming is accomplished by infecting the cells with four human genes: OCT4, Sox2, Klf4 and C-Myc. Our major findings show that iPSC-CM: express cardiac specific RNA and proteins; exhibit regular pacemaker activity; exhibit key features of the excitation contraction coupling machinery; respond to ryanodine and caffeine (though less than adult cardiomyocytes), and express the SR-Ca2+ handling proteins ryanodine receptor and calsequestrin; respond to autonomic agonists and antagonists. Hence, our work demonstrates that iPSC-CM exhibit features resembling the adult myocardium, and thus constitute a potential source for cardiac regeneration. Concomitantly, in order to decipher the pathological mechanisms of inherited cardiac arrhythmias and cardiomyopathies, we are investigating iPSC-CM generated from skin biopsies/keratinocytes obtained from patient’s catecholaminergic polymorphic ventricular tachycardia (CPVT), laminopathies, WPW and Duchenne muscular dystrophy (DMD). Our research shows that the mutated iPSC-CM feature key clinical phenotype of the disease, thus establishing the foundation for developing novel drug modalities.
Keynote Forum
Christopher F Tirotta
Nicklaus Children’s Hospital, USA
Keynote: Dexmedetomidine use in patients undergoing electrophysiological study for atrial tachyarrhythmias
Time : 09:55-10:45
Biography:
Christopher F Tirotta has been an active member of Miami Children’s Hospital Medical Staff since 1991, practicing in the Department of Anesthesiology. He has served as the Director of Cardiac Anesthesia since 2002. He also works in the Department of Anesthesiology at the University of Miami. He received his BA from Cornell University in 1982 and his MD from New York University School of Medicine in 1986. He also received an MBA degree from Columbia University in 1999. He completed his Internship in Internal Medicine from Stony Brook University in 1987. He completed his Residency training in Anesthesiology from the University of Miami, Jackson Memorial Hospital in 1990. He is sub-specialized in Pediatric and Cardiovascular Anesthesia, including Heart Transplantation. He has been the Principal Investigator for a number of clinical drug/device trials.
Abstract:
Dexmedetomidine (DEX) is a selective alpha-2 adrenergic agonist with sedative, analgesic and anxiolytic properties. DEX has not been approved for use in pediatrics. DEX has been reported to depress sinus node and atrioventricular (AV) nodal function in pediatric patients; it was even suggested that the use of DEX may not be desirable during electrophysiological studies (EPS). Other studies have reported no association between DEX use and any significant EKG interval abnormalities in patients with congenital heart disease, other than a decrease in HR. We reviewed all cases presented to the CCL for diagnosis or treatment of atrial tachyarrhythmias since 2007. The patients were stratified into three different groups. Group 1 patients did not receive any DEX. Group 2 patients received a DEX infusion of 0.5-1 mcg/kg/hr. Group 3 patients received a DEX infusion of 0.5-1 mcg/kg/hr and a DEX bolus prior to the infusion of 0.5-1 mcg/kg. We then compared those patients for the following variables: Demographic data and anesthetic data like age, sex, height, weight; mask vs. IV induction, identity of induction agent, amount of sevoflurane and propofol used; amount of DEX used; presence of congenital heart disease and other co-morbidities; the need for isoproterenol (ISO) and dose, the need for adenosine and dose and the need for any other medications to effect rhythm both before and after RFA; the ability to induce the arrhythmia, the type of arrhythmia, the presence of Wolf-Parkinson-White (WPW) syndrome, the presence of an accessory pathway, the ablation rate and the recurrence rate. There were no differences in any of the demographic data between groups. There was no difference in the anesthetic data, except there was a lesser amount of propofol and sevoflurane used in the DEX groups. There was no difference in the electrophysiologic parameters between groups, except the Group 1 patients did require the use of ISO in the pre-ablation period less often compared to the DEX groups. However, there was no difference in the ability to induce the arrhythmia, the percentage of patients ablated, and the recurrence rate between groups.
- Pediatric Congenital Heart Disease
Pediatric Heart Murmurs
Tetralogy Of Fallot In Infants
Session Introduction
Muneer Amanullah
Aga Khan University, Pakistan
Title: Right ventricular outflow tract reconstruction with handmade valve conduit: A short experience from a developing country
Biography:
Muneer Amanullah has done his FRCS in the field of General Surgery at Edinburgh (UK). He was a Fellow Researcher in the Department of Congenital and Pediatric Cardiac Surgery in UK London. Currently, he is an Interim Associate Dean and also an Associate Professor for Congenital and Pediatric Cardiothoracic Surgery at Aga Khan University, Pakistan.
Abstract:
Background & Aim: Abnormalities of right ventricular outflow tract continuity are one of the most commonly encountered entities in congenital cardiac surgery. Various strategies utilize homografts, synthetic valve conduits, Contegra, or patch enlargement with valve replacement ($2500) to restore anatomical and functional continuity between right ventricle and pulmonary artery. In countries like Pakistan, these conduits may not be easily available or affordable. We report the experience of our short observational study of using a handmade tri-leaflet valve conduit to establish right ventricular outflow tract and pulmonary artery continuity ($700).
Materials & Methods: From September 2015 to December 2016, a total of 15 patients with different diagnoses of congenital heart disease in the pediatric age group underwent corrective surgery along with restoration of RV to PA continuity, by using a handmade valved conduit. The size of the conduit is determined by using an available nomogram. A 10x10 cm bovine pericardial sheet is used to construct the conduit and a 0.5 mm thin polytetrafluoroethylene (PTFE) sheet is used to construct the valve.
Results: Patients ranged from 1 year to 16 years. Seven patients had previous palliation. One patient underwent 3rd time redo procedure for RV to PA homograft stenosis. Late postoperative complications were observed in 2 patients. One patient developed aneurysm at RVOT-conduit junction requiring surgical repair and the other underwent conduit dilatation for moderate stenosis (gradient 60 mmg). No significant regurgitation was observed and the gradients were a mean of 25 mmHg.
Conclusions: This short report highlights that the handmade valve conduits are a cost effective alternative where well-established conduits have cost implications and questionable availability.
S U Kadirova
National Research Center for Cardiac Surgery, Kazakhstan
Title: Stenting of branch pulmonary artery stenosis after surgical correction in children under 14 kg
Biography:
S U Kadirova works in the Department of Pediatric Cardiology at the University National Research Center for Cardiac Surgery, Kazakhstan. She has many published papers in reputed journals. She is a Leading Expert in Structural Heart Interventions at NCRC and her research interests mainly deals with Minimally Invasive Procedures.
Abstract:
Background & Purpose: In infants and young children with congenital heart disease, post-operative branch pulmonary artery stenosis is a major cause of morbidity and mortality. While angioplasty is a standard treatment option, high rates of restenosis is reported. We report our experience of using stents to treat branch pulmonary artery stenosis in small children (<14 kg).
Materials & Methods: From November 2014 to December 2016, percutaneous stent implantation was performed in 15 small children (10 males and 5 females) with mean weight of 9.6±3.3 kg (2.2 kg-13.9 kg), mean age 2.2±1.5 years (1.5 month-5 years). Stents used included the Valeo Vascular stent, Palmaz Genesis XD, and genesis stent pre-mounted on OPTA PRO via 6 Fr-8 Fr Cook sheath. One patient had a left pulmonary artery occlusion; other 14 patients had pulmonary artery ostial and proximal stenosis after cardiac surgery.
Results: Interventional correction was successful for all 15 patients. Valeo Vascular stents were implanted in 10 patients, Palmaz Genesis XD stents in 4, and Genesis stent pre-mounted on OPTA PRO in 1. One patient required a second stent due to residual ostial stenosis. The minimum diameter post stent increased from 2.6±1.3 mm to 7.5±1.4 mm. Mean right ventricular to pulmonary artery gradient decreased from 62±10.4 mm Hg to 19±4.3 mm Hg. Five infants who required mechanical ventilation prior to stenting was successfully weaned off in 1-3 days.
Conclusion: Stent treatment of post-operative branch pulmonary artery stenosis in children under 14 kg is safe and effective, and may shorten mechanical ventilation support post operatively.
Oluwatoyin Ogunkunle
University of Ibadan, Nigeria
Title: Initial experience with interventional cardiology and definitive solutions for structural heart diseases in a resource-challenged setting
Biography:
Ogunkunle Oo is a Graduate of the University of Ibadan and a Fellow of the National Postgraduate Medical College of Nigeria, as well as of the West African College of Physicians. She is an Associate Professor in the Department of Pediatrics, College of Medicine, University of Ibadan, Nigeria, and a Consultant Pediatrician and Pediatric Cardiologist at the University College Hospital, Ibadan, Nigeria. She has published over 50 articles in reputable journals.
Abstract:
The first case of open-heart surgery in the University College Hospital, Ibadan took place on December 19, 1978. Since then, various attempts have been made to provide definitive solutions for patients with structural cardiac diseases, but up till now, no permanent regular service exists. We present our initial experience with interventional cardiology and open-heart surgery in a resource-challenged setting. The challenges encountered are discussed and the solutions we have proffered with each situation are presented as we forge ahead towards achieving a more regular service for interventional cardiology and open-heart surgery in our center. In January 2016, eight children underwent diagnostic cardiac catheterization with a view to perform possible corrective intervention. Two subsequently had device closure of patent ductus arteriosus, the first in the history of the hospital. Four patients (one with a large atrial septal defect, one severe pulmonary stenosis, and two with Fallot’s tetralogy) were deemed to be more suitable for open-heart surgery. One with muscular ventricular septal defect was thought to be too small to need intervention. The last patient, initially thought to have a coarctation of the aorta, was found to have normal cardiac anatomy. The patient with large ASD and VSD, and one of the two with Fallot’s tetralogy, subsequently underwent successful total repair of their lesions in our facility. The successful outcome in the four patients has encouraged us to be optimistic that despite various resource challenges, it will soon be possible to establish a regular service for interventional cardiology and open-heart surgery in our center.
Christian Enzensberger
Justus-Liebig University of Giessen, Germany
Title: Influence of the frame rate (DICOM vs. Acoustic Frame Rate) on strain analysis assessed by two-dimensional (2D) speckle tracking in fetal echocardiography
Biography:
Christian Enzensberger works as a Consultant Obstetrician in the Division of Prenatal Medicine at University Hospital Giessen, Germany. His research interests include the assessment of cardiac function by speckle tracking and tissue doppler in fetal echocardiography.
Abstract:
Introduction & Purpose: Frame rates (FR) used for strain analysis assessed by speckle tracking in fetal echocardiography show a huge variation. Due to higher heart rates compared to adults, much higher FR is requested in fetuses. The aim of this study was to investigate the influence of the FR on strain analysis in two-dimensional (2D) speckle tracking.
Material & Methods: A prospective cross-sectional study was performed. Based on an apical or basal four-chamber view of the fetal heart, cine loops were acquired on a Toshiba Aplio 500 system. Each loop was digitally stored twice: firstly as a DICOM (digital imaging and communications in medicine) file with a FR of 30 frames per second (fps), secondly with the original FR (acoustic FR=AFR). For each loop, fetal global longitudinal peak strain values of both, left (LV) and right ventricle (RV), were assessed by 2D wall motion tracking. Strain analysis was performed offline by two investigators. Interobserver variability was analyzed.
Results: A total of n=11 healthy fetuses with an echocardiogram performed between 19 and 34 weeks of gestation were included. The AFR was 126±16 fps. Relating to global longitudinal peak strain values of both ventricles, there was no significant difference between DICOM FR and AFR (LV: -18.22% (DICOM FR) vs. -17.77% (AFR), RV: -16.30% (DICOM FR) vs. -15.28% (AFR); p>0.05). The interobserver variability showed a strong agreement with an intraclass correlation coefficient (ICC) of >0.8.
Conclusions: The influence of the FR on global longitudinal peak strain values in 2D speckle tracking seems to be less important than expected. For strain analysis in fetal echocardiography FR of >100 fps seem to be non-essential.
Sara Ranchordas
Santa Cruz Hospital, Portugal
Title: Benign presentation of a potentially fatal disease
Biography:
Sara Ranchordás has completed her Medical degree in Porto University Medical Faculty in 2012 and is a Cardiac Surgery Resident in Santa Cruz Hospital, Lisbon, since 2014. She has been working with a team of congenital heart disease surgeons, having participated in diverse pediatric surgeries. She has presented poster and oral presentations in national conferences and published case reports in the area of Cardiology and Cardiac Surgery.
Abstract:
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiovascular defect that can range from being fatal early in life to presenting in adulthood asymptomatically. We present the case of a teenager whose diagnosis was incidental during examination for sports practice. The echocardiogram raised the suspicion of an anomalous flow in the coronary arteries and showed mitral valve prolapse with mild regurgitation. Magnetic resonance imaging revealed a very ectasia right coronary artery and low signal intensity in the usual location of the left anterior descending artery. The diagnostic hallmark of ALCAPA syndrome is the visualization of the left coronary artery originating from the main pulmonary artery, which was only possible in this case with coronary angiography, that showed an aneurismatic right coronary artery with retrograde filling from the left coronary artery, ending in the pulmonary artery and multiple vascular connections between the right and left coronary system. Surgical techniques can be broadly divided into one-coronary and two-coronary-system repairs. Surgery allows correction of chronic subendocardial ischemia, improvement of ventricular function, and minimizes the risk for malignant dysrhythmias and sudden death. Two-coronary-system repairs are preferred and include coronary button transfer, the Takeuchi procedure, or placement of a coronary artery bypass graft with ligation of the origin of the left coronary artery. This patient was submitted to coronary artery button transfer with excellent result.
Eva Marwali
National Cardiovascular Center, Indonesia
Title: To evaluate the efficacy and safety of oral triiodothyronine for infants and children undergoing cardiopulmonary bypass in an Indonesian population
Biography:
Eva Marwali has done her specialization in Pediatric Cardiac Intensive Care from the National Cardiovascular Center, Indonesia. She is a member of the Society of Critical Care Medicine. She is a member of Extracorporeal Life Support Organization.
Abstract:
Objectives: To evaluate the efficacy and safety of oral triiodothyronine for infants and children undergoing cardiopulmonary bypass in an Indonesian population.
Methods: We performed a single center, randomized, double-blind, and placebo-controlled trial in children age≤3 years undergoing congenital heart disease surgery with cardiopulmonary bypass. We administered oral triiodothyronine (T3, Tetronine®) 1 μg/kg-body weight/dose or placebo (saccharum lactis) via nasogastric tube every 6 hours for 60 hours since induction of anesthesia. The primary endpoint, time to extubation, was compared with Cox regression.
Results: The modified intention to treat group included 101 placebo and 104 treated subjects. The stratified log-rank test did not show a significant treatment difference (p=0.061) for time to extubation, but after adjustment for age, nutritional Z-score, and Aristotle surgical complexity, the hazard ratio (HR) was 1.33 (95% confidence interval (CI)=1.00, 1.76, p=0.049). The effect of T3 was stronger in the strata ≤5 months of age (HR: 1.86, 95% CI: 1.02, 3.39, p=0.043). Median intubation time for the placebo and T3 group in ≤5 months were 47.3 hours and 32.1 hours, respectively. Adverse events rates including arrhythmia were similar between groups, though sepsis was more frequent with placebo.
Conclusions: Oral T3 supplementation may shorten time to extubation in children undergoing congenital heart disease surgery, particularly infants ≤5 months. Administration is relatively safe, simple and inexpensive.
Cheng Wang
Central South University, China
Title: The ventricular late potentials in children with vasodepressor response of vasovagal syncope
Biography:
Cheng Wang works in the Department of Pediatric Cardiovasology, Children's Medical Center, Second Xiangya Hospital, Central South University, China. He is a Professor, Chief Physician, Doctoral Tutor, and the Deputy Director for Children's Medical Center and Pediatric Teaching and Research Section at the Second Xiangya Hospital, Central South University and Institute of Pediatrics, Central South University.
Abstract:
Objectives: The objective is to discuss ventricular late potentials (VLPs) in children with vasodepressor response of vasovagal syncope (VVS-V).
Methods: 184 children diagnosed as VVS-V by head-up tilt test (HUTT) were enrolled as VVS-V group, 105 age and gender matched healthy individuals without syncope were used as control group, then signal-averaged electrocardiogram were measured.
Results: Heart rate was decreased [(83.98±12.27) vs. (87.28±13.75) bpm, P<0.05] in VVS-V group compared with control group. The prevalence of positive VLPs was not significantly different between the two groups. However, the absolute value of TQRS [(84.89±12.05) vs. (81.21±8.23) ms, P<0.01], RMS40 [(28.73±7.23) vs. (26.89±7.36) μV, P<0.05] and LAS40 [(62.43±19.17) vs. (56.79±1.83) ms, P<0.05] were significantly prolonged in VVS-V group compared with control group, and more patients in VVS-V group had abnormal prolonged LAS40 (94.57% vs. 83.80%, P<0.01).
Conclusions: The prevalence of positive VLPs was not significantly different, TQRS, RMS40, LAS40 were longer in children with VVS-V in comparison with healthy individuals, and the abnormal LAS40 occurred in a higher proportion of VVS-V group.
Veronique Diane
University of Douala, Cameroon
Title: Congenital heart disease and impact on child development
Biography:
Veronique Diane has completed her Doctor of Medicine degree from the Faculty of Medicine and Pharmaceutical Sciences, University of Douala. She is currently working as a General Medical Practitioner at the Limber Regional Hospital in Limbe, Cameroon. She intends to be a Cardiac Specialist in the future and has been researching on Cardiac Medicines both at the University of Douala and at the Cardiac Centre of St. Elizabeth Catholic General Hospital Shisong, Cameroon.
Abstract:
Objectives: To evaluate the child development and evaluate a possible association with the commitment by bio-psychosocial factors of children with congenital heart disease and those without the infection.
Methods: This research is an observational case-control study comparing the development of children with and without heart disease, categorized in three groups. Group 1(G1): children 0-10years of age both male and female genders with congenital heart disease awaiting surgery; Group 2 (G2): children of age 0-10years of both genders who have undergone at least one surgical procedure for congenital heart disease correction between 6 months and 1 year before the application of the instruments of this study; Group 3 (G3): Children 0-10 years, healthy and both genders. As instruments, we used a bio- psychosocial data questionnaire that includes social, demographic, psychological and clinical factors, and a development screen test divided into four fields of functions i.e. personal-social, fine-motor-adaptive, language and gross motor.
Results: Ninety six children were evaluated, 19 (22.66%) belonging to the G1 (preoperative children with heart disease), 32 (33.59%) in Group 2 (postoperative children with heart disease) and 45 (43.75%) in G3 (healthy children). Of the total, 55.56% are girls and age ranged from 2 months to 10 years. The children with heart disease had more suspicious and abnormal ratings and in the group of healthy children 53.6% were considered with normal development (P≤0.0001). The biopsychosocial variables that were related to a possible developmental delay were gender (P=0.042), child's age (P=0.001) and income per capita (P=0.019).
Conclusion: The results suggest that children with congenital heart disease are likely to have a developmental delay with significant difference between children who have undergone surgery and those awaiting surgery under clinical follow-up.