Artificial intelligence in pediatric cardiology and cardiac surgery: Irrational hype or paradigm shift? Anthony C Chang Annals of Pediatric Cardiology 2019 12(3):191-194

Comparison of antistreptolysin O and anti-deoxyribonucleic B titers in healthy children to those with acute pharyngitis, acute rheumatic fever, and rheumatic heart disease aged 5–15 years Navjot Saini, Dinesh Kumar, Swarnim Swarnim, Dheeraj Bhatt, Sunil Kishore Annals of Pediatric Cardiology 2019 12(3):195-200 Background: Acute rheumatic fever (ARF) affects millions of children in the third world countries like India. The diagnosis of rheumatic fever is based on the Jones criteria with serological titers, antistreptolysin O titer (ASO), and anti-deoxyribonucleic B (ADB), taken as evidence of recent streptococci infection. There is a lack of recent data available on ASO and ADB titers in children from the Delhi/NCR and thus adequate geographical area-specific cutoffs for the region are not available. Aims and Objectives: The aim of this study is to determine and compare the ASO and ADB antibody titers in children with acute pharyngitis, ARF, rheumatic heart disease (RHD), and in healthy children of the Delhi/NCR region. Materials and Methods: Twenty-six cases of ARF, 51 cases of RHD, 50 cases of acute pharyngitis, and 84 healthy normal children were included in the study. A single ASO and ADB titer measurement was done in these children. Results: The ASO titers was raised in acute pharyngitis – 303 IU/ml (interquartile range [IQR], 142–520 IU/ml) and ARF – 347.5 IU/ml (IQR, 125–686 IU/ml) children in comparison to healthy controls – 163.5 IU/ml (IQR, 133–246.5 IU/ml) and RHD patients – 163 IU/ml (IQR, 98.250–324.500). The ADB titers were highest in ARF patients – 570.5 IU/ml (IQR, 276–922 IU/ml) followed with RHD – 205 IU/ml (IQR, 113.6–456.5), healthy controls – 78.25 IU/ml (IQR, 53.39–128.15 IU/ml), and acute pharyngitis – 75.12 IU/ml (IQR, 64.5–136 IU/ml). The upper limit of normal (ULN) values of ASO and ADB computed from normal healthy children were 262.4 IU/ml and 134.44 IU/ml, respectively, and these can be used as cutoff values for recent streptococcal infection in this geographical area. Conclusions: The median ASO titers in acute pharyngitis group and ARF were significantly raised compared to that of the control group. The ADB titers were raised in ARF and RHD patients albeit the levels were higher in ARF patients. The derived ULN values can be used as cutoff reference.

Different habitus but similar electrocardiogram: Cardiac repolarization parameters in children – Comparison of elite athletes to obese children Christian Paech, Janina Moser, Ingo Dähnert, Franziska Wagner, Roman Antonin Gebauer, Toralf Kirsten, Mandy Vogel, Wieland Kiess, Antje Körner, Bernd Wolfarth, Jan Wüstenfeld Annals of Pediatric Cardiology 2019 12(3):201-205 Introduction: The standard 12-lead electrocardiogram (ECG) remains a widely used tool in the basic cardiac evaluation of children and adolescents. With the emergence of inherited arrhythmia syndromes, the period of cardiac repolarization has been the focus of attention. So far, data on cardiac repolarization and its normal variants in healthy children are scarce. This may cause uncertainties in the differentiation between pathologies and normal variants. As abnormal autonomic regulation seems to be a major influencing factor on cardiac repolarization, this study aimed to evaluate the parameters of cardiac repolarization of children in extremely good physical shape to obese children to improve knowledge about cardiac repolarization in these subgroups of pediatric patients that are vastly affected by the alterations of autonomic regulation. Methods: A total of 426 pediatric volunteers (84 lean, healthy controls; 130 obese healthy pediatric volunteers; and 212 elite athletes) were enrolled in the study, and the parameters of cardiac repolarization were determined in 12-lead ECG. Results: Most importantly, there were no pathological findings, neither in the healthy controls nor in the obese or athletes. Athletes showed overall shorter corrected QT intervals than children from the other groups. This is also true if a correction of the QT interval is performed using the Hodges formula to avoid bias due to a tendency to lower heart rates in athletes. Athletes showed the shortest Tpeak-to-end ratios between the groups. The comparison of athletes from primarily strength and power sports versus those from endurance sports showed endurance-trained athletes to have significantly longer QT intervals. Conclusions: This study suggests that neither obesity nor extensive sports seems to result in pathological cardiac repolarization parameters in healthy children. Therefore, pathology has to be assumed if abnormal repolarization parameters are seen and might not be simply attributed to the child's habitus or an excellent level of fitness.

Percutaneous closure of patent ductus arteriosus with the Nit-Occlud® patent ductus arteriosus device in 268 consecutive cases Andrii V Maksymenko, Yulia L Kuzmenko, Arkadii A Dovhaliuk, Oleksandra O Motrechko, Florian E Herrmann, Nikolaus A Haas, Anja Lehner Annals of Pediatric Cardiology 2019 12(3):206-211 Background: The pfm Nit-Occlud® patent ductus arteriosus (PDA) device is well established for interventional closure of PDA. However, there are still limited data concerning its efficacy and follow-up in larger patient groups. Aims: This study aimed to evaluate the safety and efficacy of the Nit-Occlud® PDA device, implanted both through transpulmonary and transaortic approach, in a large cohort. Methods: From July 2008 to December 2015, 268 consecutive patients were admitted for transcatheter closure of a PDA and were treated with the Nit-Occlud® coil. Clinical, echocardiographic, and angiographic data were evaluated. Results: The median age was 5.2 years (range, 5 months to 62 years), and the median weight was 19.3 kg (range: 5.5–97 kg). Ten (3.7%) patients had weight <10 kg. The most common ductus types treated were Krichenko Type E and A (44.0% and 33.2%, respectively). Twelve (4.5%) patients were treated for residual shunting after surgical PDA closure. The median diameter at the narrowest point was 1.5 mm (range: 0.4–4 mm), the median size of the ampulla was 5 mm (range: 1–15 mm), and the median length was 9 mm (range: 2–25 mm). Device implantation could be successfully achieved in all cases. Closure rates documented immediately after the procedure, at 3–10 days, 1 month, and 6 months after intervention were 62%, 95.1%, 97.8%, and 98.5%, respectively. With the exception of one minor thromboembolic event, there were no procedure-related complications. Conclusion: Closure of PDA with various anatomic variations and sizes can be performed effectively and safely using the Nit-Occlud® coil.

Long-term follow-up and outcomes of discrete subaortic stenosis resection in children Lulu Abushaban, Babu Uthaman, John Puthur Selvan, Mustafa Al Qbandi, Prem N Sharma, Thinakar Vel Mariappa Annals of Pediatric Cardiology 2019 12(3):212-219 Background: Studies of long-term outcomes of discrete subaortic stenosis (DSS) are rare. Therefore, we reviewed the long-term outcomes of subaortic membrane resection in children with isolated DSS over 16 years from a single institution. Materials and Methods: We retrospectively reviewed the records of patients (n = 27) who underwent resection of DSS between 2000 and 2017. Patients with major concomitant intracardiac anomalies were excluded. Indications for surgery were mean left ventricular outflow tract (LVOT), Doppler gradient >30 mmHg, and/or progressive aortic insufficiency. Results: The mean age at diagnosis was 3.77 ± 3.49 years (range, 0.25–13 years) and the mean age at surgery was 6.36 ± 3.69 years (range, 1–13 years). All patients underwent resection of subaortic membrane. The mean LVOT Doppler gradient decreased from 40.52 ± 11.41 mmHg preoperatively to 8.48 ± 5.06 mmHg postoperatively (P < 0.001). The peak instantaneous LVOT Doppler gradient decreased from 75.41 ± 15.22 mmHg preoperatively to 18.11 ± 11.44 mmHg postoperatively (P < 0.001). At the latest follow-up, the peak gradient was 17.63 ± 8.93 mmHg. The mean follow-up was 7.47 ± 3.53 years (median 6.33 years; range 2.67–16 years). There was no operative mortality or late mortality. Recurrence of subaortic membrane occurred in 7 (25.92%, 7/27) patients who underwent primary DSS operation. Four (14.81%, 4/27) patients required reoperation for DSS recurrence at a median time of 4.8 years (3.1–9.1 years) after the initial repair. Risk factors for reoperation were age <6 years at initial repair. Eighteen (66.66%, 18/27) patients had AI preoperatively and progression of AI occurred in 70.37% (19/27). This included 4 (22.22%, 4/18) patients who had worsening of their preoperative AI. Short valve-to-membrane distance was found to be prognostically unfavorable. One (3.7%, 1/27) patient had an iatrogenic ventricular septal defect, and 2 (7.4%, 2/27) patients had complete AV block following membrane resection. Conclusions: Resection of subaortic membrane in children is associated with low mortality. Higher LVOT gradient, younger age at initial repair, and shorter valve-to-membrane distance were found to be associated with adverse outcome. Recurrence and reoperation rates are high, and progression of aortic insufficiency following subaortic membrane resection is common. Therefore, these patients warrant close follow-up into adult life.

Atrial and ventricular ejection force of the fetal heart: Which of the four chambers is the dominant? Elaheh Malakan Rad, Toktam Sheykhian, Ali Akbar Zeinaloo Annals of Pediatric Cardiology 2019 12(3):220-227 Background/Aim: This study aimed to measure and compare the ejection force of the cardiac chambers in healthy singleton fetuses and to investigate the relationship of ejection force of cardiac chambers with gestational age, fetal sex, and fetal heart rate. Patients and Methods: A prospective study was performed on 68 singleton fetuses with a gestational age of 17–34 weeks. Atrial and ventricular ejection force was measured. Measurements were repeated in 18 of the fetuses to assess intraobserver reliability. Results: The right atrium had the highest ejection force of all the cardiac chambers. Ejection force of both atria and ventricles increased with gestational age. Conclusion: The right atrium is the dominant chamber of the fetal heart in 17–34 weeks of gestation. Comparison of our values with previous studies indicates that left atrial ejection force almost doubles in the 1st month after birth. This study highlights the crucial role of the right atrium in the fetal cardiac function during 17–34 weeks of gestation.

Options for coronary translocation and other considerations in aortic root translocation (Bex-Nikaidoh procedure) Swaminathan Vaidyanathan, Marathe Supreet, Marathe Shilpa, Alphonso Nelson, Agarwal Vijay Annals of Pediatric Cardiology 2019 12(3):228-232 Introduction: The surgical options for patients with transposition of the great arteries (TGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction include intracardiac baffling with the right ventricle to pulmonary artery (PA) conduit (Rastelli procedure), “reparation a l'etage ventriculaire” or aortic root translocation (Bex-Nikaidoh procedure). The Bex-Nikaidoh procedure allows a more normal, anatomically aligned left ventricular outflow tract. However, the operation is technically demanding, and coronary translocation remains one of the major challenges for successful root translocation. Methods: All patients who underwent aortic root translocation in a single institute over a period of 2 years from January 2015 to December 2017 were included in the study. Surgical technique and early outcomes are described with specific focus on the different observed coronary artery patterns and surgical strategies for translocation. Results: Fourteen patients underwent aortic root translocation. The coronary artery patterns observed could be categorized into four different patterns based on the size of the pulmonary annulus and the relative position of the PA relative to the aorta. Successful translocation of the coronary arteries was achieved in every patient. Mean follow-up was 18.42 ± 9.22 months. There was no mortality and no reoperation during the follow-up period. Conclusion: The Bex-Nikaidoh procedure is a promising surgical option for TGA, VSD, and pulmonary stenosis. Good outcomes are achievable despite wide variations in anatomy using a tailored approach for coronary translocation.

Functional cardiac measurements performed by two-dimensional Doppler echocardiography in normal fetuses: Determination of Z-scores and future prospects Luciane Alves Rocha, Nathalie Jeanne Bravo-Valenzuela, Liliam Cristine Rolo, Edward Araujo Junior Annals of Pediatric Cardiology 2019 12(3):233-239 Two-dimensional (2D) echocardiogram with the aid of color Doppler and pulsed Doppler allows one to record blood flow waveforms in several structures of the heart. The determination of normal values of these flows in the fetus can help understand cardiac hemodynamics. Given this importance, numerous surveys have been conducted with various existing echocardiographic techniques in order to improve the functional evaluation and consequently, planning of delivery. The aim of this review was to discuss the findings of the reference values of blood flows obtained by 2D echocardiography with Doppler, the current trend of the determination of Z-scores in the functional measurements, and their future prospects.

Abernethy malformation: Our experience from a tertiary cardiac care center and review of literature Sushil Azad, Adhi Arya, Radhakrishnan Sitaraman, Amit Garg Annals of Pediatric Cardiology 2019 12(3):240-247 Abernethy malformation, also called as congenital extrahepatic portosystemic venous shunt, is a rare anomaly involving the portal venous system. Although rare, it is increasingly being reported and is important to diagnose given the adverse clinical consequences in untreated patients. It has myriad of clinical presentations, from being completely asymptomatic to causing hepatic carcinoma, hepatic encephalopathy, severe pulmonary hypertension, and diffuse pulmonary arteriovenous malformation. We describe our experience with five cases in a tertiary pediatric cardiac care center with Abernethy malformation, with review of literature and also discuss possible therapeutic implications.

Anomalous aortic origin of the pulmonary arteries: Case series and literature review Salvatore Agati, Carlos Guerra Sousa, Felice Davide Calvaruso, Rosanna Zanai, Ivana Campanella, Daniela Poli, Alfredo Di Pino, Luca Borro, Fiore Salvatore Iorio, Massimiliano Raponi, Robert H Anderson, Simone Reali, Andrea De Zorzi, Aurelio Secinaro Annals of Pediatric Cardiology 2019 12(3):248-253 Anomalous origin of the pulmonary arteries from the ascending aorta is a rare, but severe clinical entity necessitating a scrupulous evaluation. Either the right or the left pulmonary arteries can arise directly from the ascending aorta while the other pulmonary artery retains its origin from the right ventricular outflow tract. Such a finding can be isolated or can coexist with several congenital heart lesions. Direct intrapericardial aortic origin, however, must be distinguished with origin through a persistently patent arterial duct. In the current era, clinical manifestations usually become evident in the newborn rather than during infancy, as used to be the case. They include respiratory distress or congestive heart failure due to increased pulmonary flow and poor feeding. The rate of survival has now increased due to early diagnosis and prompt surgical repair, should now be expected to be at least 95%. We have treated four neonates with this lesion over the past 7 years, all of whom survived surgical repair. Right ventricular systolic pressure was significantly decreased at follow-up. Our choice of treatment was to translocate the anomalous pulmonary artery in end-to-side fashion to the pulmonary trunk. Our aim in this report is to update an Italian experience in the diagnosis and treatment of anomalous direct origin of one pulmonary artery from the aorta, adding considerations on the lessons learned from our most recent review of the salient literature.