Pediatric Cardiology

Use of strain, strain rate, tissue velocity imaging, and endothelial function for early detection of cardiovascular involvement in young diabetics Atul Kaushik, Aditya Kapoor, Preeti Dabadghao, Roopali Khanna, Sudeep Kumar, Naveen Garg, Satyendra Tewari, Pravin K Goel, Archana Sinha Annals of Pediatric Cardiology 2021 14(1):1-9 Background: Subtle structural and functional changes may precede the onset of overt global left ventricular (LV) dysfunction. Data pertaining to tissue velocity imaging (TVI)and strain imaging to assess regional myocardial function and flow mediated vasodilatation are limited in young patients with diabetes. Materials: Conventional echocardiography, TVI parameters along with strain (S), and strain rate (SR) were measured in 50 young diabetics (15.16 ± 2.95 years, mean HBA1c 8.15 ± 1.37 g %) and 25 controls (15.60 ± 2.51 years). Flow-mediated dilation (FMD), nitrate--mediated dilatation (NMD), and carotid intima–media thickness were also assessed. Results: Conventional echocardiography parameters were similar in patients and controls; however, deceleration time of the mitral inflow velocity (early deceleration time) was significantly shorter in patients when compared with controls (149.06 ± 31.66 vs. 184.56 ± 19.27 ms, P =0.001). Patients had lower strain values at the basal lateral LV (21.39 ± 4.12 vs. 23.78 ± 2.02; P =0.001), mid-lateral LV (21.43 ± 4.27 vs. 23.17 ± 1.92 P =0.02), basal septum (20.59 ± 5.28 vs. 22.91 ± 2.00; P = 0.01), and midseptum (22.06 ± 4.75 vs. 24.10 ± 1.99; P = 0.01) as compared to controls. SR at the basal and midsegments of the lateral LV wall and at the basal septum was also significantly lower in diabetic patients. Diabetic children also had endothelial dysfunction with significantly lower FMD (8.36 ± 4.27 vs. 10.57 ± 4.12, P = 0.04). Conclusions: LV strain indices and flow--mediated dilatation are impaired in asymptomatic children and adolescents with type 1 diabetes mellitus despite absence of overt heart failure and normal ejection fraction. Early detection of subclinical regional myocardial dysfunction by deformation analysis including strain and strain rate may be useful in the asymptomatic diabetic population.

Pulmonary arteriovenous malformations in children after the Kawashima procedure: Risk factors and midterm outcome Ibrahim J Alibrahim, Mohammed H A Mohammed, Mohamad S Kabbani, Abdulraouf M Z. Jijeh, Omar R Tamimi, Abdullah A Alghamdi, Fahad Alhabshan Annals of Pediatric Cardiology 2021 14(1):10-17 Background : Pulmonary arteriovenous malformations (PAVMs) are the major cause of progressive cyanosis in patients palliated with bidirectional cavopulmonary connection (BCPC). The aim of our study is to analyze the occurrence of PAVMs in patients after Kawashima procedure, to study the effect of total cavopulmonary connection (TCPC) on PAVMs, to evaluate the effect of axillary arteriovenous fistula (AAVF) creation on PAVMs, and to study the risk factors for PAVMs. Methods : In this retrospective cohort study, all patients with left isomerism and azygous continuation of an interrupted inferior vena cava who underwent Kawashima procedure from July 2001 to December 2017 were included. Results : Twenty.six patients after Kawashima procedure were included in our study. PAVMs were diagnosed in 12 patients (46%). Five of these 12 patients underwent TCPC with complete resolution of hypoxemia. Three patients underwent AAVF creation, 2 had complete resolution, while 1 had partial resolution of hypoxemia. Fourteen patients (54%) did not develop PAVMs. Nakata index below 267 mm2/m2 and McGoon ratio below 1.9 predicted the development of PAVMs with high sensitivity and specificity. Conclusions : PAVMs represent a serious complication in patients who undergo Kawashima procedure. Small size of pulmonary arteries is an important risk factor for the development of PAVMs. Resolution of hypoxemia after TCPC completion supports the hepatic factor hypothesis. Early TCPC completion in these patients may help to avoid the development of PAVMs by restoring the hepatic factor. Resolution of hypoxemia after AAVF creation may support the lack of pulsatile flow hypothesis.

Comparison of right ventricular outflow tract gradient under anesthesia with post-operative gradient in patients undergoing tetralogy of Fallot repair Dheemta Toshkhani, Virendra Kumar Arya, Kamal Kajal, Shyam K S Thingnam, Sandeep Singh Rana Annals of Pediatric Cardiology 2021 14(1):18-25 Background : Intra-cardiac repair for tetralogy of Fallot has some degree of residual right ventricular outflow tract (RVOT) obstruction. However, the measurement of this gradient intra-operatively might get affected by the depth of anesthesia which is important for the long-term outcome. Aims : The primary aim was to compare intraoperative RVOT gradient post repair under two different anesthetic depths of 1% and 2% end-tidal sevoflurane. The secondary objective was to follow up the changes in RVOT gradient till 1 month postoperatively. Design : Observational study. Setting : Advanced Cardiac Centre of PGIMER, Chandigarh. Methods : Following intracardiac repair, RVOT gradient was measured directly by placing needle into the right ventricle and pulmonary artery at sevoflurane 1%, and subsequently, at 2% end.tidal concentration while maintaining hemodynamic stability. These gradients were also measured using transesophageal echocardiography (TEE) (ClinicalTrials.gov NCT03234582). Results : Twenty-one patients were included in this study that had intra-cardiac repair, of which pulmonary annulus was preserved for 15 cases. Mean RVOT gradients measured invasively and by TEE at end-tidal sevoflurane concentration of 1% and 2% were not significantly different (6.67 ± 4.16 mmHg vs. 6.76 ± 3.82 mmHg, P > 0.05 invasively and 13.01 ± 7.40 mmHg vs. 12.53 ± 7.11 mmHg, P > 0.05 by TEE, respectively). RVOT gradient measured by trans-thoracic echocardiography (TTE) postoperatively at the time of extubation and during follow-up at 1 month showed significant reduction (11.37 ± 6.00 mmHg, P < 0.05 and 9.23 ± 4.92 mmHg, P < 0.01 respectively). Six patients who underwent repair with transannular patch had significant pulmonary regurgitation (PR) following surgery, with no significant change in PR severity or RVOT gradient on increasing anesthetic depth. Conclusions : Postoperative RVOT gradient was not altered by changing depth of anesthesia provided systemic blood pressure was maintained. One month postrepair RVOT gradients were significantly reduced as compared to the intraoperative values.

Utility of late pulmonary artery banding in single-ventricle physiology: A mid-term follow-up Aritra Mukherji, Sanjiban Ghosh, Nihar Pathak, Jayita Nandi Das, Nilanjan Dutta, Debasis Das, Amitabha Chattopadhyay Annals of Pediatric Cardiology 2021 14(1):26-34 Background: The standard first stage palliation for univentricular heart with unrestricted pulmonary blood flow (PBF) is surgical pulmonary artery (PA) banding for which the ideal age is within the first 8 weeks of life. This study aimed to look for the utility of PA band done beyond 3 months of age for patients presenting beyond the stipulated period. Materials and Methods: This is a retrospective analysis of the outcome of twenty patients with single ventricle (SV) physiology with unrestricted PBF who presented late and were selected on the basis of clinical, radiological, and echocardiographic parameters for PA banding. Results: The median age of the patients was 5.5 months (3.5–96 months), and the median body weight was 4.7 kg (3.2–22.0 kg). The patients were divided into three groups as follows: ten patients between 3 and 6 months of age (Group A), seven patients between 6 months to 1 year of age (Group B), and three patients > 1 year of age with additional features of pulmonary venous hypertension (Group C). The mean reduction of PA pressures following PA band was 60.9%, 48.8%, and 58.3% and the mean fall in oxygen saturation was 10.4%, 8.0%, and 6.6% in the three groups, respectively. The postoperative mortality rate was 10%. The mean follow up duration was 13.5 months (7–23 months). There was a statistically significant improvement in weight for age Z scores following PA band (P = 0.0001). On follow up cardiac catheterization, the mean PA pressures were 16.6 (±3.6), 22.7 (±5.7), and 33.3 (±12.4) mmHg, respectively, in the three groups, and the mean pulmonary vascular resistance index was 1.86 (±0.5), 2.45 (±0.7), and 3.5 (±1.6) WU.m2, respectively. Subsequently, seven patients in Group A, three patients in Group B, and one patient from Group C underwent successful bidirectional Glenn (BDG) surgery. Conclusions: Late PA band in selected patients with SV physiology can have definite benefit in terms of correction of heart failure symptoms and subsequent conversion to BDG and can potentially change the natural history of disease both in terms of survival and quality of life.

Melody valve to replace the mitral valve in small children: Lessons learned Vilius Dranseika, Rene Pretre, Oliver Kretschmar, Hitendu Dave Annals of Pediatric Cardiology 2021 14(1):35-41 Objective: Infants requiring mitral valve replacement have few viable options. Recently, stented bovine jugular vein graft (Melody) has been surgically implanted in such cases. Herein, we report our experience, elaborating on evolution of implantation technique, pitfalls, as well as long-term outcome (including late dilatability). Methods: Seven Melody valves were implanted (2013–2019). The median patient age and weight were 6.7 (1.8–30.5) months and 5.8 (4.6–9.5) kg, respectively. The indications for implantation were mitral stenosis and/or regurgitation postatrioventricular septal defect (AVSD) repair (5), congenital mitral valve dysplasia (1), and Shone's complex (1). Operative technique involved shortening the valve and creating a neo-sewing ring at 2/3 (atrial)–1/3 (ventricular) junction. Implantation was followed by intraoperative balloon dilatation. Results: Five out of seven patients survived the perioperative period (one death due to technical failure and the other due to acute respiratory distress syndrome postcardiopulmonary bypass). Two out of five medium-term survivors got transplanted (1) or died due to acute myeloid leukemia (1). No valves were replaced. The mean echo gradient at discharge was a median 4 (2–6) mmHg. None of the patients showed left ventricular outflow tract or pulmonary venous obstruction. Two Melody valves were dilated late (5 months and 3 years postoperatively), resulting in decreasing mean gradients from 6 to 1 and from 17 to 4 mmHg. At last follow-up, surviving Melody had a mean gradient of 4 (1–9) mmHg. Conclusions: Mitral valve replacement with a Melody valve is feasible in infants, is reproducible, shows good immediate results, and offers the possibility of later dilatation. This technique offers a better solution compared to the existing alternatives for infants requiring a prosthetic mitral valve.

Clinical profiles and risk factors for early and medium-term mortality following heart transplantation in a pediatric population: A single-center experience Komarakshi R Balakrishnan, Kemundel Genny Suresh Rao, Ganapathy Krishnan Subramaniam, Murali Krishna Tanguturu, Ajay Arvind, Veena Ramanan, Jagdish Dhushyanthan, K Ramasubramanian, K Suresh Kumaran, Gunalan Sellamuthu, Mohan Rajam, Senthil Mettur, Pradeep Gnansekharan, Ravikumar Ratnagiri Annals of Pediatric Cardiology 2021 14(1):42-52 Aims and Objectives: There is a paucity of data regarding the outcomes of Heart transplantation in children from the Indian subcontinent. The data of patients under the age of 18 undergoing an isolated heart transplantation was analyzed for patient clinical profiles and risk factors for early and medium-term mortality. Hospital mortality was defined as death within 90 days of transplantation and medium-term survival as follow up of up to 6 years. Materials and Methods: A total of 97 patients operated between March 2014 and October 2019 were included in this study .Data was collected about their INTERMACS status, pulmonary vascular resistance, donor heart ischemic times, donor age, donor to recipient weight ratio and creatinine levels. Results: The age range was from 1 to 18 with a mean of 10.6 ± 4.6 years. 67 % patients were in INTERMACS category 3 or less.12 children were on mechanical circulatory support at the time of transplant. The 90 day survival was 89 %.The risk factors for hospital mortality was lower INTERMACS category (odd’s ratio 0.2143, P = 0.026), elevated creatinine (odd’s ratio 5.42, P = 0.076) and elevated right atrial pressure ( odd’s ratio 1.19, P = 0.015).Ischemic time, pulmonary vascular resistance (PVR) and PVR index (PVRI) had no effect on 90 day survival. Kaplan Meier estimates for 5 year survival was 73 %.The medium term survival was affected by INTERMACS category (Hazard ratio 0.7, P = .078) , donor age > 25 ( Hazard ratio 1.6, P = 0.26) and raised serum creatinine values.(Hazard ratio 2.7, P = 0.012). All the survivors are in good functional class. Conclusions: Excellent outcomes are possible after heart transplantation in a pediatric population even in a resource constrained environment of a developing economy. More efforts are needed to promote pediatric organ donation and patients need to be referred in better INTERMACS category for optimal outcomes.

Tetralogy of fallot with coronary crossing the right ventricular outflow tract: A tale of a bridge and the artery Sachin Talwar, Sanjoy Sengupta, Supreet Marathe, Pradeep Vaideeswar, Balram Airan, Shiv Kumar Choudhary Annals of Pediatric Cardiology 2021 14(1):53-62 A coronary artery crossing the right ventricular outflow tract is a subset of a larger pathomorphological cohort known as an anomalous coronary artery (ACA) in the tetralogy of Fallot (TOF). The best possible outcome in a patient with TOF and ACA is decided by judicious selection of optimum preoperative investigative information, the timing of surgery, astute assessment of preoperative surgical findings, and appropriate surgical technique from a wide array of choices. In most instances, the choice of surgical technique is determined by the size of the pulmonary annulus and the anatomical relation of ACA to the pulmonary annulus. In the present era, complete, accurate preoperative diagnosis and primary repair is a routine procedure with strategies to avoid a right ventricle-to-pulmonary artery conduit.

Defining transposition: What have we learnt? Max E Roderick, Nikita Maria, Adrian Crucean, John Stickley, David J Barron, Robert H Anderson Annals of Pediatric Cardiology 2021 14(1):63-66 Understanding transposition is important for all who hope to effectively treat patients with the condition. The variants of the condition are frequently debated in the literature. We describe an unusual variant of transposition, in which despite the arterial roots being supported by morphologically inappropriate ventricles, the roots themselves were normally related, with the intrapericardial arterial trunks spiraling as they extended into the mediastinum. The specimen was identified following the re-categorization of our archive, and we subsequently conducted a detailed analysis of the underlying morphology. Using the principles of sequential segmental analysis, we compared the morphology with standard examples previously described. We show how it was the recognition of such hearts that promoted that concept that the combination of connections across the atrioventricular and ventriculo-arterial junctions was the essence of transposition. In the most common variant, the arrangements are concordant at the atrioventricular junctions, but discordant at the ventriculo-arterial junctions. We suggest that the overall arrangement of discordant ventriculo-arterial connections is best described simply as “transposition.” When the discordant ventriculo-arterial connections are combined with similarly discordant connections at the atrioventricular junctions, the transposition is congenitally corrected. We point out that the use of “d” and “l” as prefixes does not distinguish between transposition and its congenitally corrected variant. For those using segmental notations, the correct description for the rare variant found in the setting of a posteriorly located aortic root with the usual atrial arrangement is transposition (S, D, NR).

Video-assisted thoracoscopic pacemaker lead placement in children with atrioventricular block Sergey Termosesov, Ekaterina Kulbachinskaya, Ekaterina Polyakova, Dmitriy Khaspekov, Ivan Grishin, Vera Bereznitskaya, Maria Shkolnikova Annals of Pediatric Cardiology 2021 14(1):67-71 Background: The pacemaker lead placement is presented as one of the most appropriate procedures in children with a complete atrioventricular block (AVB). Despite the fact that video-assisted thoracic surgery (VATS) for epicardial lead placement has demonstrated positive results as to the feasibility, safety, and efficacy in adults, its role in pacemaker implantation in children remains unclear. Aim: This study sought to assess the intermediate-term outcomes of video-assisted thoracoscopic pacemaker lead placement in children with complete AVB Materials and Methods: From May 2017 to November 2019, five children with complete AVB underwent minimally invasive left ventricular (LV) lead placements via thoracoscopic video assistance approach. The procedure was performed under complex intratracheal anesthesia with single-lung ventilation, all pacing parameters were evaluated in perioperative and follow-up periods. Results: The median age of children at implantation was 3 years (range: 2 to 4 years), the median weight was 13 kg (range: 12–15 kg). All procedures were completed successfully, pacing thresholds for the active lead measured 0.3-1.1V, with R-wave amplitude of 8-18 mV and impedance of 560-1478 Ohm. Conclusion: Thoracoscopic pacemaker lead placement may provide a potential alternative to the transthoracic approach of epicardial lead placement in children with AVB.

Myocardial infarction due to thrombosis of native aorta late after Fontan procedure for hypoplastic left heart syndrome Bhavi Patel, Tacy Downing, Lourdes Prieto, Darline Santana-Acosta Annals of Pediatric Cardiology 2021 14(1):72-74 Patients with single ventricle physiology who have undergone the Fontan procedure are at risk for long-term complications such as thrombus formation. Thrombus formation in the native aortic root (NAR) can be life-threatening if retrograde filling of the coronary arteries (CAs) is impaired. We present three cases of NAR thrombus with embolization to the CA, resulting in myocardial infarction. Thromboembolic events are well-documented complications of Fontan physiology, and optimal prevention and treatment strategies are poorly defined.

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Medicine by Alexandros G. Sfakianakis,Anapafseos 5 Agios Nikolaos 72100 Crete Greece,00302841026182,00306932607174,alsfakia@gmail.com,

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