Showing papers in "Annals of Pediatric Cardiology in 2020"
TL;DR: It is shown that IV paracetamol is as effective as indomethacin and ibuprofen in promoting the closure of hs-PDA in premature infants with a better safety profile.
Abstract: Introduction: In this prospective study, we compared the efficacy and safety of ibuprofen, indomethacin, and paracetamol in the closure of patent ductus arteriosus (PDA) in preterm neonates. Materials and Methods: This randomized prospective study was conducted in the Division of Pediatric Cardiology, M. D. M and Umaid Hospital, Jodhpur. A total of 105 preterm neonates with gestational age Results: Our study shows that there was no significant difference observed in PDA closure among all the three treatment groups after the completion of two courses of treatment. The cumulative rate of PDA closure was 68% in the indomethacin group, 77.14% in the ibuprofen group, and 71.43% in the paracetamol group (P = 0.716). There were no significant changes found in Hb, platelet, blood urea nitrogen (BUN), creatinine, and liver enzymes after treatment in the paracetamol group (P > 0.05). BUN and serum creatinine levels were significantly increased after treatment in indomethacin and ibuprofen groups (P Conclusion: Our study shows that IV paracetamol is as effective as indomethacin and ibuprofen in promoting the closure of hs-PDA in premature infants with a better safety profile.
21 citations
TL;DR: The data indicate that percutaneous CA access, even in very small preterm infants, is safe and feasible with negligible vascular injury and no neurological adverse events.
Abstract: Background : Carotid artery (CA) access allows a more straight route for many left heart lesions. This has previously been achieved via a surgical cut-down approach in certain pediatric cardiac interventions. However, there are little data considering CA access in pediatric cases, percutaneously. Aim : We hypothesized that there would be notable improvements in efficiency as well as overall success when using the CA for access in selected cases. Methods : Between November 2016 and January 2019, records of patients undergoing attempted percutaneous CA access under ultrasound guidance for cardiac catheterization were reviewed. Results : Thirty patients underwent 36 catheterizations; median age 17 days (range, 6 days–9 months) and median weight 3.2 kg (1.2–7.8). Procedures performed were stenting or stent redilatation of the patent ductus arteriosus in 23, stenting or angioplasty of modified Blalock–Taussig shunts in four, aortic valvuloplasty in three, angioplasty for coarctation of the aorta in four, renal angioplasty in one, and diagnostic catheterization in one case. The intended intervention was unsuccessful in two patients despite successful CA access. Follow-up imaging showed a normal carotid in 28 of 30 (94%), with mild luminal narrowing with normal Doppler velocities in two instances. No patient had clinically apparent neurological sequelae attributable to CA access. Conclusions : Our data indicate that CA access should be employed when dealing with a select group of infants requiring vertical approach for left-sided cardiac lesions. Percutaneous CA access, even in very small preterm infants, is safe and feasible with negligible vascular injury and no neurological adverse events.
12 citations
TL;DR: Nonopioid medications appear to show promise for analgesia in infants undergoing cardiac surgery, with ketorolac being the most potent agent as a potential substitute for opioids.
Abstract: Objective: The purpose of this review is to present the available literature on the use of nonopioid analgesics such as nonsteroidal anti-inflammatory drugs in postcardiac surgery pediatric patients, mainly to focus on patients Materials and Methods: Published studies that address the use on nonopioid medications for postoperative sedation and analgesia in infants and children undergoing cardiac surgery were identified from online sources. Studies were reviewed by two authors independently to assess the quality of the data as well as the evidence. Due to limited availability of such studies, the review was then expanded to include use in noncardiac procedures as well as to expanded age groups. All studies that met the primary objective were included. Results/Data Synthesis: Majority of the studies in the population of interest were related to use of ketorolac. Five studies specifically addressed ketorolac use in cardiac patients. In addition, studies were reviewed for nonopioid analgesia in noncardiac patients and included as a part of the available evidence as in the case of acetaminophen use. Newer agents as well as agents with very limited information were also acknowledged. Conclusion: Nonopioid medications appear to show promise for analgesia in infants undergoing cardiac surgery, with ketorolac being the most potent agent as a potential substitute for opioids. These agents demonstrate a reasonable safety profile even in the very young. There continue to be significant gaps in knowledge before their adoption becomes routine. However, gives the awareness regarding short-term and long-term impact of opioid use in this vulnerable population, and studies of such agents are an urgent need.
11 citations
TL;DR: This study argues that the lesser-known axillary access route is the safest and most effective route of vascular access for balloon angioplasty in infants with aortic coarctation and advocates its more widespread clinical implementation in the treatment of critical newborns.
Abstract: Balloon angioplasty may be performed as the first treatment of aortic coarctation to stabilize newborns too sick for immediate surgery. The issue of vascular access is the key to the successful treatment of critical newborns. In our study, we argue that the lesser-known axillary access route is the safest and most effective route of vascular access for balloon angioplasty in infants with aortic coarctation. To support this argument, we present the case of eight unstable newborns with complex heart diseases, who were successfully treated with percutaneous intervention through the axillary artery. This case series is followed by an analysis of the greater efficacy of this technique compared to the more conventional femoral and carotid routes. We conclude by acknowledging the substantial advantages of this lesser-known vascular access and advocate its more widespread clinical implementation in the treatment of critical newborns.
9 citations
TL;DR: The late diagnosis of CCHD is high but does not affect the outcome, and the timing of diagnosis has improved over time.
Abstract: Aims : There are limited data regarding critical congenital heart disease (CCHD) from middle-income countries (MIC). This study aims to determine the birth prevalence, rate of late diagnosis, and influence of timing of diagnosis on the outcome of CCHD. Setting and Design : Retrospective observational cohort study in the State of Johor, Malaysia. Subjects and Methods : All infants born between January 2006 and December 2015 with a diagnosis of CCHD, defined as infants with duct-dependent lesions or cyanotic heart disease who may die without early intervention. The late diagnosis was defined as a diagnosis of CCHD after 3 days of age. Results : Congenital heart disease was diagnosed in 3557 of 531,904 live-born infants and were critical in 668 (18.7%). Of 668, 347 (52%) had duct-dependent pulmonary circulation. The birth prevalence of CCHD was 1.26 (95% confidence interval: 1.16–1.35) per 1000 live births, with no significant increase over time. The median age of diagnosis was 4 days (Q1 1, Q3 26), with 61 (9.1%) detected prenatally, and 342 (51.2%) detected late. The highest rate of late diagnosis was observed in coarctation of the aorta with a rate of 74%. Trend analysis shows a statistically significant reduction of late diagnosis and a significant increase in prenatal detection. However, Cox regression analysis shows the timing of diagnosis does not affect the outcome of CCHD. Conclusions : Due to limited resources in the MIC, the late diagnosis of CCHD is high but does not affect the outcome. Nevertheless, the timing of diagnosis has improved over time.
8 citations
TL;DR: Defect size as large as three times the weight in kg can be closed in small children provided the surrounding rims are adequate and IAS length is not a limiting factor for deciding the size of the device used.
Abstract: Background and Objectives: Device closure of secundum atrial septal defect is shown to be feasible and effective in children weighing ≤10 kg. Issues such as how large is too large, how to choose device size, does the length of the interatrial septum (IAS) matter, and need for technical modifications for successful device delivery have not been systematically addressed. Materials and Methods: This is a retrospective study, comprising 45 patients weighing ≤10 kg, who were chosen for device closure between January 2010 and June 2018. Patient selection was done on basis of transthoracic echocardiography. Device closure was done using Amplatzer septal occluder. The device size was selected primarily based on transesophageal echocardiography (TEE)-measured defect diameter. Although IAS length was taken into consideration, adequate rim size was the key factor in deciding device closure of the defect. Results: Forty-three out of 45 patients had successful device closure. The mean age and weight were 25.71 ± 8.62 months and 8.99 ± 1.24 kg, respectively. The defect measuring as large as 27 mm (14.89 ± 3.89) on TEE was closed and device as big as 28 mm was successfully deployed (16.7 ± 4.31). Regular technique of device deployment was successful in only 15 cases. In the remaining 28, one of the modified techniques was used. There was no mortality, failure of the procedure, device embolization, thromboembolism, or pericardial effusion. One patient developed moderate mitral regurgitation and two patients had transient atrioventricular block. At follow-up, all patients showed significant improvement in symptoms and growth without any complications. Conclusions: Defect size as large as three times the weight in kg can be closed in small children. Devices as large as 28 mm can be deployed in these hearts provided the surrounding rims are adequate. In majority of cases, one of the modified techniques is essential for successful deployment. IAS length is not a limiting factor for deciding the size of the device used.
8 citations
TL;DR: BPV using the transjugular approach is safe and effective to relieve critical PS in neonates compared to the transfemoral approach.
Abstract: Background: Critical pulmonary stenosis (PS) is one of the life-threatening congenital heart diseases which present during the neonatal period with cyanosis. Surgical valvotomy was once the procedure of choice for critical PS; however, balloon pulmonary valvuloplasty (BPV) has now become the standard treatment. Although the procedure is usually simple, crossing the pulmonary valve from the femoral vein can be difficult, especially when severe tricuspid regurgitation and right atrium dilatation are present. In such patients, the maneuver can be simplified by using the right internal jugular vein approach. However, many operators are reluctant to use this approach because of unfamiliarity with the technique, potential complications, and paucity of reports. Until now, there is no literature describing BPV using the transjugular approach in neonates, also none directly comparing the transfemoral and transjugular approaches. Objective: We compared transjugular with the transfemoral approach in terms of procedure time and complications. Materials and Methods: This was a retrospective cohort study. Participants were neonates with critical PS undergoing BPV in the National Cardiovascular Center Harapan Kita from 2013 to 2018. Results: Of 15 neonates undergoing BPV, eight were done using the transjugular approach and seven using the femoral approach. Mean age and weight in both groups was similar. In all eight patients using transjugular approach, crossing the pulmonary valve was consistently quick and easy. The total procedural time, pulmonary crossing time, and fluoro time was significantly shorter using the transjugular approach (65 ± 8 vs. 108 ± 17.8 min, P Conclusion: BPV using the transjugular approach is safe and effective to relieve critical PS in neonates compared to the transfemoral approach.
7 citations
TL;DR: Early experience with COVID-19 in the pediatric population reports that patients display exceptionally high levels of acute-phase reactants and the clinical syndrome in these patients is somewhat similar to Kawasaki disease with or without myocardial involvement.
Abstract: There is limited information about coronavirus disease 2019 (COVID-19) in the pediatric population. Preliminary data suggest a not insignificant prevalence of cardiac involvement. Here, we report our early experience with COVID-19 in the pediatric population. These patients display exceptionally high levels of acute-phase reactants. The clinical syndrome in these patients is somewhat similar to Kawasaki disease with or without myocardial involvement. In some cases, the presentation mimics typical myocarditis. Severe myocardial involvement is associated with transient electrocardiographic and echocardiographic abnormalities. These findings may be due to the cardiotropic nature of the virus or may be the result of an immunologic response to the infection.
7 citations
TL;DR: Perioperative outcomes in patients with GA/S suggested an increased residual cardiovascular disease and increased resource usage, and this is the first study demonstrating the effect of race and ethnicity on PLOS in CTHD patients.
Abstract: Objectives : The main objective of the study is to characterize the effects of genetic abnormalities/syndromes (GA/S) on perioperative outcomes of cardiac surgeries involving repair of conotruncal heart defects (CTHD). Design : The study involves a single-center retrospective analysis of patients who underwent complete repair of CTHDs (tetralogy of Fallot [TOF], truncus arteriosus, interrupted aortic arch, and ventricular septal defect with coarctation) between January 2000 and December 2015. The primary outcome was the post operative length of stay (PLOS). The secondary outcomes were mortality, cardiac complications, hematologic complications, infections, and number of medications-at-discharge. Setting : Cardiac intensive care unit in a tertiary pediatric hospital in South Florida that performs around 300 open-heart surgeries a year. Subjects : A total of 177 patients with CTHDs who underwent cardiac surgeries in the stated time period were included in the final study cohort. Measurements and Main Results :Majority of patients had TOF (72.5%) and 46 (26%) had GA/S. The most common GA/S was 22q11 deletion (37%). PLOS was significantly increased in patients with GA/S (P Conclusions : Perioperative outcomes in patients with GA/S suggested an increased residual cardiovascular disease and increased resource usage. Notably, this is the first study demonstrating the effect of race and ethnicity on PLOS in CTHD patients.
7 citations
TL;DR: Preoperative weight and IVC diameter appeared to have no influence on immediate postoperative outcomes in HLHS patients undergoing Fontan, and performing the Fontan off CPB and with a fenestration also conferred no added clinical benefit.
Abstract: Objective: To investigate patient-related factors, echocardiographic, and anatomic variables associated with immediate and long-term clinical outcomes after extracardiac Fontan procedure at our institution. Materials and Methods: Retrospective review of preoperative cardiac catheterizations and echocardiograms as well as medical records of all children with hypoplastic left heart syndrome (HLHS) who underwent Fontan between June 2002 and December 2018. Results: Seventy-seven patients with HLHS were included (age 4 years [1.5–11.7]). Seventy patients (91%) received a nonfenestrated Fontan and 57 patients (74%) underwent Fontan without cardiopulmonary bypass (CPB). Presence of a Fontan fenestration (P = 0.69) and use of CPB (P = 0.79) did not differ between those with Conclusion: In HLHS patients undergoing Fontan, preoperative weight and IVC diameter appeared to have no influence on immediate postoperative outcomes. Performing the Fontan off CPB and with a fenestration also conferred no added clinical benefit. These observations should be considered when deciding optimal timing for Fontan completion.
7 citations
TL;DR: All children with supraventricular tachycardia (SVT) should have close monitoring with serial ECG and plasma levels of flecainide during the 48–72 h after initiation of treatment, and consider hospitalization for patients <1 year of age.
Abstract: Flecainide is a class IC antiarrhythmic indicated for ventricular and supraventricular arrhythmias in pediatric patients without structural heart disease. Flecainide has a narrow therapeutic window and proarrhythmic effect even in therapeutic doses and could lead to a life-threatening intoxication. Dosage errors, accidental intakes, and drug or food interactions, especially with dairy products, can be the cause of the intoxication. We report three consecutive cases of flecainide intoxication in children with supraventricular tachycardia (SVT) in our hospital from 2017 to 2019. Two cases had complete and spontaneous normalization of electrocardiogram (ECG) after flecainide removal. However, admission to the intensive care was required due to a sustained ventricular tachycardia in one case. Flecainide intoxication can be a life-threatening complication in patients with SVT. We believe all children should have close monitoring with serial ECG and plasma levels of flecainide during the 48-72 h after initiation of treatment, and consider hospitalization for patients <1 year of age.
TL;DR: The sensitivity and specificity of a clinical screening tool for the identification of congenital heart defects at the first visit of an infant after birth for immunization is established and can easily be used by peripheral workers working in remote places with poor resources enabling prompt referral.
Abstract: Introduction: Neonatal screening for congenital heart defects at birth can miss some heart defects, sometimes few critical ones, and the scenario is even worse in those neonates who had never undergone a neonatal checkup (home deliveries). Immunization clinic can serve as a unique opportunity as the second checkpoint for the screening of the children. A history- and examination-based test can serve as an effective tool to screen out children with heart defects. Aims and Objectives: The aim of this study was to establish the sensitivity and specificity of a clinical screening tool for the identification of congenital heart defects at the first visit of an infant after birth for immunization. Materials and Methods: This is a cross-sectional observational study which the consecutive children presenting at 6 weeks of age for immunization or any child presenting for the first time (outborn delivery) till 6 months of age in the immunization clinic were subjected to detailed history and examination and findings were recorded on a predesigned pro forma and a clinical score was calculated. All these children were then subjected to echocardiography for confirmation of the diagnosis of congenital heart disease (CHD), and the sensitivity and specificity of the test were recorded Observations and Results: A total of 970 babies were screened, out of them 31 were diagnosed with CHD and 18 had undergone neonatal screening at birth. A clinical score of 3 or more had more chances of detecting CHD. The sensitivity of the cutoff score as 3 was 96.77% and specificity was 98.72, with a positive predictive value of 71.43%, a negative predictive value of 99.89%, and an accuracy of 98.66%. Conclusions: The history- and examination-based tool is an effective method for early identification of CHD and can easily be used by peripheral workers working in remote places with poor resources enabling prompt referral.
TL;DR: There has been improvement in surgical results for APVS over the years, however, it still remains a challenge to manage infants and patients with persistent respiratory problems.
Abstract: Background: Absent pulmonary valve syndrome (APVS) is a variant of tetralogy of Fallot characterized by aneurysmal pulmonary arteries, which compresses the tracheobronchial tree, leading to respiratory symptoms We report the mid-term outcomes of surgical correction of patients with APVS Subject and Methods: A total of 27 patients underwent surgery between 2001 and 2015, and they were followed up for a mean period of 64 ± 41 years Out of the 27 patients, 14 (519%) were infants The median age at repair was 98 months Preoperative intubation was required in six patients (222%), and 11 patients (407%) had symptoms of respiratory distress The pulmonary valve was replaced with a valved conduit in 15 patients (556%), monocusp valve in 6 patients (222%), and a transannular patch in 6 patients (222%) Reduction pulmonary arterioplasty was done in all patients Results: The overall 10-year survival was 821% There was 811% overall freedom from re-intervention at 10 years No statistically significant difference was found in 10-year survival (P = 0464) and reoperation rates (P = 0129) between valved conduit, monocusp, or transannular patch techniques Older children had statistically significantly longer survival (P = 0039) and freedom from re-intervention (P = 0016) compared to infants Patients without respiratory complications had 100% 10-year survival and 933% freedom from reoperation at 10 years compared to 556% and 601%, respectively, for patients with respiratory complications Conclusion: There has been improvement in surgical results for APVS over the years However, it still remains a challenge to manage infants and patients with persistent respiratory problems
TL;DR: CTA and catheterization are equivalent in their ability to delineate pulmonary artery anatomy and major aortopulmonary collateral arteries.
Abstract: Introduction: Pulmonary atresia with the ventricular septal defect is a rare congenital heart defect with high anatomic variability. The most important management question relates to the sources of pulmonary blood flow. The ability to differentiate between ductal dependence and major aortopulmonary collateral arteries is critical to achieving good outcomes and avoiding life-threatening hypoxia in the postneonatal period. Having accurate information about pulmonary arteries, major aortopulmonary collateral arteries, and sources of blood supply to each pulmonary segment is crucial for choosing the optimal surgical strategy. The purpose of this study is to compare computed tomography angiography (CTA) with cardiac catheterization for anatomic delineation of surgically relevant anatomy in pulmonary atresia with ventricular septal defect with major aortopulmonary collateral arteries. Materials and Methods: Retrospective review of all children with pulmonary atresia with ventricular septal defect with major aortopulmonary collateral arteries cared for at a large tertiary children's hospital who underwent cardiac catheterization with angiography and CTA close to each other without interval therapy. All studies were performed between 2007 and 2011. Results: There were 9 patients who met the inclusion criteria. Pulmonary artery anatomy (confluent vs. nonconfluent) was correctly identified in 9 patients by CTA and 8 patients by catheterization. There were no significant differences between CTA and catheterization in the identification of major aortopulmonary collateral arteries (mean = 3.4 collaterals/study via catheterization; mean = 3.1 collaterals/study via CTA; P = 0.67). CTA was superior to catheterization in the delineation of segmental pulmonary blood flow (P = 0.006). Conclusion: CTA and catheterization are equivalent in their ability to delineate pulmonary artery anatomy and major aortopulmonary collateral arteries.
TL;DR: A decrease in FMD value, which is a marker of endothelial dysfunction, was found in children with rheumatic carditis, and this study found it to be similar in the patient and the control groups.
Abstract: Background: Acute rheumatic fever (ARF) is an important cause of valvular heart disease in children Endothelial dysfunction plays an important role in the pathogenesis of valvular heart diseases The role of endothelial dysfunction in valvular heart diseases due to ARF is not exactly known In ARF, autoimmune injury, inflammation, oxidative stress, and impairment of nitric oxide in valvular endothelium may be the causes of endothelial dysfunction The purpose of this study is to evaluate endothelial dysfunction and arterial stiffness in children with ARF Materials and Methods: Thirty-six patients diagnosed with ARF (the mean age was 1180 ± 282 years) and 36 volunteered individuals with similar age, sex, and body mass index were included in the study The study groups were compared in terms of M-mode echocardiography parameters, carotid arterial strain (CAS), beta-stiffness index (βSI), and flow-mediated dilation (FMD) Results: In patients with ARF, there was a decrease in FMD% (1036 ± 726 and 1276 ± 459; P Conclusion: In our study, a decrease in FMD value, which is a marker of endothelial dysfunction, was found in children with rheumatic carditis
TL;DR: In the majority of patients with repaired TOF and severe PR, RV dilatation is unchanged during a follow-up of 3 years, and shorter time interval between repair and first CMR was the only risk factor predictive for progressive RV Dilatation.
Abstract: Objective: Pulmonary valve regurgitation (PR) and right ventricular (RV) dilatation are important residual findings after surgical repair of tetralogy of Fallot (TOF). We sought to describe the natural course of RV dilatation over time in patients with severe PR after TOF repair and to determine risk factors for quick progression of RV dilatation and dysfunction. Methods: Data of 85 consecutive TOF patients with PR and RV dilatation, undergoing serial cardiovascular magnetic resonance (CMR) scans between July 2002 and December 2016 in two institutions, were retrospectively reviewed. The dataset was analyzed regarding right and left ventricular (LV) volume and function and potential risk factors of progressive RV dilatation Results: There was no significant increase in RV end-diastolic volumes (RVEDVi) indexed body surface area (BSA) (median 150 [81–249] vs. 150 [82–260] mL/m2) and end-systolic volumes indexed for BSA (RVESVi) (75 [20–186] vs. 76 [39–189] mL/m2) between the first and last CMR in the overall group. Similarly, there were no significant changes in LV volumes indexed for BSA (LVEDVi 78 [56–137] vs. 81 [57–128] mL/m2 and LV end-systolic volume index 34 [23–68] vs. 35 [18–61] mL/m2). Global function remained also unchanged for both ventricles. RVEDVi increased statistically significantly (≥ 20 mL/m2) in twenty patients (24%) from 154 mL/m2 (87–237) to 184 mL/m2 (128–260, P Conclusion: In the majority of patients with repaired TOF and severe PR, RV dilatation is unchanged during a follow-up of 3 years. RV dilatation seems to progress early after surgery and subsequently stabilize. RV dilatation significantly progresses in a subgroup of 24% of patients, with a shorter time interval since surgical repair.
TL;DR: The study suggests the persistence of PVB19 and HHV-6 in the host can lead to subsequent viral reactivation in the transplanted heart, even in those recipients who do not have active myocarditis.
Abstract: Objectives: The aim of this study is to evaluate HHV-6 and PVB19 infection using polymerase chain reaction (PCR) and immunofluorescent assay (IFA) in the myocardium of pediatric patients with dilated cardiomyopathy (DCM) and the impact of viral persistence in the cardiac allograft after heart transplantation (HT). Methods: Multiplex droplet digital PCR was used to analyze the prevalence of viral sequences in myocardial samples from 48 pediatric DCM patients and 10 control subjects. Of the 48 DCM patients, 44 underwent HT. After HT, consecutive endomyocardial biopsy (EMB) samples were analyzed for the presence of PVB19 and HHV-6 antigens using IFA and the patients were evaluated for rejections, coronary vasculopathy, and graft loss. Results: Of the 48 DCM patients, 14 had positive viral PCR results in explanted/autopsy hearts. Among them, PVB19 was found in 8/48, HHV6 in 4/48, both PVB19 and HHV6 in 1/48, and enterovirus in one, but no adenovirus was found. The EMB samples obtained after HT were positive for PVB19 and HHV-6 in 7/44 and 3/44 cases, respectively. Viral presence in both the explanted heart and the cardiac allograft was demonstrated in 4 patients, 3 of whom were positive for PVB19, and one of whom was positive for HHV-6 pretransplant. Coronary vasculopathy and graft loss were more common in patients with PVB19-positive myocardial tissues versus those who were PVB19-negative. Conclusions: There is an association between PVB19 and HHV-6 infection and DCM in children. The study suggests the persistence of PVB19 and HHV-6 in the host can lead to subsequent viral reactivation in the transplanted heart, even in those recipients who do not have active myocarditis. PVB19 in the cardiac allograft tended toward higher adverse post-HT events.
TL;DR: Two infants with a double drainage pattern of TAPVC are reported, where the confluence has connections at two different levels.
Abstract: Total Anomalous Pulmonary Venous Connection (TAPVC) is frequently classified based on the system proposed by Craig, Darling and Rothney. Occasionally variants are reported which do not fit into these classic forms. One such variant is the double drainage of TAPVC where the confluence has connections at two different levels. We report two infants with a double drainage pattern of TAPVC.
TL;DR: The authors are congratulated for using surface-rendered virtual three-dimensional (3D) heart model for surgical planning without physically printing the model.
Abstract: © 2019 Annals of Pediatric Cardiology | Published by Wolters Kluwer Medknow Sir, We read with interest the report by Garekar et al.[1] We congratulate the authors for using surface-rendered virtual three-dimensional (3D) heart model for surgical planning without physically printing the model. Any effort to save cost is commendable. The expenditure, however, is not only from the printing of the 3D model but also from the need for specialized software. As highlighted by the authors, the conversion of a volumetric dataset to a virtual 3D model requires advanced software and coordination among various experts. The requirement of such expensive software limits the use of 3D printing in clinical practice. A similar virtual 3D heart model can also be created using open-source software but mandates navigation through a nondedicated cumbersome workflow.
TL;DR: A case of “complete bridging type DOMV” occurring as an isolated entity and presenting as severe congenital MR requiring surgery is reported.
Abstract: Double orifice mitral valve (DOMV) is a rare congenital anomaly of the mitral valve apparatus consisting of an accessory bridge of fibrous tissue, which partially or completely divides the mitral valve into two orifices. It usually occurs as an associated lesion. Encountering a DOMV as an isolated finding meriting intervention is indeed rare. We, here, report a case of "complete bridging type DOMV" occurring as an isolated entity and presenting as severe congenital MR requiring surgery.
TL;DR: A case of a 2-year-old child with complete sternal cleft and tetralogy of Fallot who presented with cyanotic spells and underwent total correction, followed by chest wall reconstruction on the next day.
Abstract: Complete sternal cleft is a very rare congenital midline defect of the sternum. It is not uncommonly associated with intracardiac defects. We report a case of a 2-year-old child with complete sternal cleft and tetralogy of Fallot who presented with cyanotic spells. The child underwent total correction, followed by chest wall reconstruction on the next day.
TL;DR: The patient was treated with an epicardial pacemaker followed by complete and sustained resolution of his hypertension with no further need for the previously prescribed antihypertensive medication.
Abstract: We herein report a case of a 6-year-old boy with hypertension and complete heart block with a unique outcome. The patient was treated with an epicardial pacemaker followed by complete and sustained resolution of his hypertension with no further need for the previously prescribed antihypertensive medication.
TL;DR: This review aims to present and compare different surgical techniques of root translocation of the great arteries except the Ross procedure.
Abstract: This review aims to present and compare different surgical techniques of root translocation of the great arteries except the Ross procedure. The historical aspects, technical considerations, and results are briefly elucidated.
TL;DR: The case of a 16-year-old girl diagnosed with myocarditis, although initial echocardiographic imaging was consistent with hypertrophic cardiomyopathy (HCM), must be vigilant for such rare presentations of myCarditis masquerading as HCM.
Abstract: We report the case of a 16-year-old girl diagnosed with myocarditis, although initial echocardiographic imaging was consistent with hypertrophic cardiomyopathy (HCM). The diagnosis of myocarditis was made with the findings of troponin elevation, presence of influenza A, and a more characteristic electrocardiogram. She eventually made a full recovery. Clinicians must be vigilant for such rare presentations of myocarditis masquerading as HCM.
TL;DR: Lipid apheresis is an inevitable alternative treatment for HFH and despite all of its application problems, DALI system is an effective therapy for decreasing atherogenic lipids from circulation.
Abstract: Introduction Familial hypercholesterolemia (FH) is characterized by severe hypercholesterolemia that can result in coronary artery disease occurring at an early age. If patients are not cured with lipid-lowering drugs and diets, lipid apheresis may be an effective treatment option in these cases. Here, we evaluate the efficacy, selectivity and safety of the DALI apheresis technique. Materials and Methods Seven pediatric patients (2 girls; 5 boys) with ages between 7 and 14 years (mean age: 6.5±2.1 years) with HFH were included in this study. We restrospectively evaluated clinical and laboratory findings. We used the DALI system for lipid apheresis concomitant with medical treatment and diet for hyperlipidemia. Results The cohort's mean T.cholesterol level prior to apheresis was 700.57±136.36 mg/dl,the mean LDL-C value was 526.86±131.56 mg and the mean HDL-C level was 36.57±4.58 mg/dl.The mean cholesterol levels after apheresis were consecutively 317.57±93.70 /257.29±90.38 / 33.36±4.78 mg/dl.We noted a 51.1% reduction in LDL-C level and an 8.7% reduction in HDL-C level in our apheresis sessions.The reduction in LDL-C was statistically significant (p<0.05). During 1025 apheresis therapy, the most frequent mild and moderate adverse events were deviceaccess problems and hypotension (in all patients);severe adverse events were mainly due to cardiac problems(myocardial infarct and arrhythmia) and hypotension. Conclusion Lipid apheresis is an inevitable alternative treatment for HFH. Despite all of its application problems, DALI system is an effective therapy for decreasing atherogenic lipids from circulation.
TL;DR: Ketamine along with low-dose fentanyl, when used for anesthetic induction, in comparison to high-doseentanyl, reduces postoperative extubation time and ICU stay, in pediatric patients undergoing corrective/palliative surgery under CPB and epidural analgesia for congenital cardiac defects.
Abstract: Background: The past two decades have seen rapid development of new surgical techniques for repair as well as palliation of complex congenital heart diseases. For a better patient outcome, minimal postoperative ventilation remains one of the most important endpoints of an effectual perioperative management. Aims and Objectives: The aim of this randomized open-label trial was to compare postoperative extubation time and intensive care unit (ICU) stay when two different anesthetic regimens, comprising of induction with ketamine and low-dose fentanyl versus high-dose fentanyl, are used, in pediatric patients undergoing corrective/palliative surgery. Materials and Methods: Patients with congenital cardiac defects, under 14 years of age undergoing cardiac surgery under cardiopulmonary bypass (CPB) and epidural analgesia, were enrolled into two groups – Group K (ketamine with low-dose fentanyl) and Group F (high-dose fentanyl) – over a period of 10 months, starting from January 2018. The effect of both these drugs on postoperative extubation time and ICU stay was compared using Mann–Whitney U-test. Results: A total of 70 patients were assessed with equal distribution in both the groups. In Group K, 32 of 35 patients were extubated in the operation room, whereas extubation time in Group F was18.1 ± 11 h. Total ICU stay in Group K and Group F was 45.2 ± 30.1 and 60.1 ± 24.5 h, respectively (P = 0.02). Systolic blood pressure was significantly higher in Group K. Conclusion: Ketamine along with low-dose fentanyl, when used for anesthetic induction, in comparison to high-dose fentanyl, reduces postoperative extubation time and ICU stay, in pediatric patients undergoing corrective/palliative surgery under CPB and epidural analgesia for congenital cardiac defects.
TL;DR: Sinus rhythm was restored by radio frequency ablation and this resulted in improvement in clinical status and cardiac function.
Abstract: We report an eight month old infant who presented with incessant Fascicular tachycardia and heart failure which was refractory to drugs and cardioversion. Sinus rhythm was restored by radio frequency ablation and this resulted in improvement in clinical status and cardiac function. Role of catheter ablation in this situation is discussed.
TL;DR: An extremely rare case of d-TGA presenting at 40 years of age, with moderately elevated pulmonary artery pressures and relatively stable symptoms is reported.
Abstract: The modern-day surgical techniques and strategies have changed the outlook of patients with dextro-transposition of great arteries (d-TGA). The survival of an unrepaired d-TGA into late adulthood is difficult to explain. Even when large intracardiac shunts are present, it still remains a lethal cyanotic congenital heart disease if it is not surgically corrected soon after birth. Here, we report an extremely rare case of d-TGA presenting at 40 years of age, with moderately elevated pulmonary artery pressures and relatively stable symptoms.
TL;DR: This work is describing a hybrid approach for aortic embolization of PDA device when the percutaneous retrieval fails, where device retrieval and PDA ligation can be done through thoracotomy incision, thus decreasing the complications.
Abstract: Embolization of the patent ductus arteriosus (PDA) device is a known adverse event of percutaneous PDA closure, which can lead to complications. Embolization can occur into the pulmonary artery or into the aorta. Device embolization can be moderate adverse event (when retrieved percutaneously) or major adverse event (when retrieved surgically). We are describing a hybrid approach for aortic embolization of PDA device when the percutaneous retrieval fails, where device retrieval and PDA ligation can be done through thoracotomy incision, thus decreasing the complications.