Showing papers in "Annals of Pediatric Cardiology in 2021"
TL;DR: Fetal echocardiography enables prenatal diagnosis and planning of postnatal repair of VRs and shows feasibility of accurate diagnosis of VR during antenatal ultrasound examination.
Abstract: Background : Vascular rings (VRs) present with varied symptoms and may result in significant morbidity before an accurate diagnosis is made. Prenatal diagnosis may be useful to plan surgery after birth. Objectives : The purpose of the study was to see the feasibility of accurate diagnosis of VR during antenatal ultrasound examination and describe their outcome. Methods : This is a retrospective observational study between January 2014 and December 2019. Vascular rings were diagnosed on the basis of three vessel tracheal view and neck vessels arrangements on fetal echocardiogram. Postnatal evaluation by transthoracic echocardiography and computerized tomography angiogram was performed. Surgical repair was done as per standard indications. Results : A total of 35 cases of fetal VRs (median gestational age: 24 weeks [range: 19–35]) were diagnosed during the study period. There were four dichorionic diamniotic twin gestation pregnancies. The right aortic arch (RAA) with anomalous left subclavian artery (ALSA) was suspected in 31 fetuses, double aortic arch (DAA) in 3, and circumflex aorta in 1. Twenty-six (74%) patients had successful deliveries. One patient had a spontaneous miscarriage, 2 underwent termination, and 6 were lost to follow-up. Postnatal assessment showed RAA with ALSA in 18, DAA in 5, circumflex aorta in 2, and no abnormality in 1. Twenty-two (86%) were operated (RAA with ALSA: 17, DAA: 4, and circumflex aorta: 1) and four were waiting for surgery. Two patients died due to prematurity-related complications. All survivors are symptom free during follow-up (median: 2.24; range: 0.2–5.6 years). Conclusions : Fetal echocardiography enables prenatal diagnosis and planning of postnatal repair of VRs.
6 citations
TL;DR: The straight and flared versions of the pericardial Venus P-valve as discussed by the authors were used in the Indian Venus Pvalve registry for efficacy, complications, and midterm outcomes, and the straight version was successfully implanted in 20 out of 22 native outflow tracts.
Abstract: Background : Balloon-expandable pulmonary valves are usually not suitable for dilated native outflow tracts. Methods : Indian Venus P-valve registry was retrospectively analyzed for efficacy, complications, and midterm outcomes. Straight valve was used in prestented conduits in patients with right ventricular pressure above two-thirds systemic pressure and/or right ventricular dysfunction. Flared valve 1–4 mm larger than balloon waist was used in native outflow in symptomatic patients, large ventricular volumes, and ventricular dysfunction. Objectives : A self-expanding porcine pericardial Venus P-valve is available in straight and flared designs.. Results : Twenty-nine patients were included. Straight valve was successful in all seven conduits, reducing gradients significantly, including one patient with left pulmonary artery (LPA) stent. Flared valve was successfully implanted in 20 out of 22 native outflow tracts. Sharp edges of the older design contributed to two failures. Complications included two migrations with one needing surgery, endocarditis in one, insignificant wire-frame fractures in three, and groin vascular complication in one patient. There were no deaths or valve-related reinterventions at a mean follow-up of 47.8 ± 24.5 months (1–85 months). Modifications of technique succeeded in three patients with narrow LPA. There was significant improvement in symptoms, right ventricular volume, and pulmonary regurgitant fraction. Conclusion : Straight and flared Venus P-valves are safe and effective in appropriate outflow tracts. Straight valve is an alternative to balloon-expandable valves in stenosed conduits. Flared valve is suitable for large outflows up to 34 mm, including patients with LPA stenosis. Recent design modifications may correct previous technical failures. Studies should focus on durability and late complications.
6 citations
TL;DR: Thoracoscopic pacemaker lead placement may provide a potential alternative to the transthoracic approach of epicardial lead placement in children with AVB.
Abstract: 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.
5 citations
TL;DR: In this article, a multipronged management approach with general measures, pharmacotherapy, and pacing has decreased morbidity and mortality in postoperative ectopic tachycardia.
Abstract: Junctional ectopic tachycardia (JET) is more common in its postoperative form. A thorough understanding of its etiology, pathophysiology, and management strategies is essential. Classically, postoperative JET is considered to arise from surgical trauma. Genetic susceptibility and an intrinsic morphologic/functional defect in the conduction system inherent in congenital heart diseases likely play a significant role. The devastating effects on postoperative hemodynamics warrant prompt attention. A multipronged management approach with general measures, pharmacotherapy, and pacing has decreased morbidity and mortality. Amiodarone and procainamide remain the preferred drugs, while ivabradine appears promising. Carefully planned randomized trials can go a long way in developing a systematic management protocol for postoperative JET.
4 citations
TL;DR: A case of 1-year 5-month-old child who developed VT with low cardiac output syndrome with deranged hepatic function, who was managed efficiently using suppressive atrial pacing to ameliorate the effects of sustained VT.
Abstract: Sustained ventricular tachycardia (VT) in the early postoperative period following intracardiac repair for tetralogy of Fallot is rare. In stable VT, amiodarone forms the mainstay of management. However, where amiodarone and other antiarrhythmic drugs are contraindicated, suppressive overdrive atrial pacing can be used as a safe and efficient alternative to maintain cardiac output. We present a case of 1-year 5-month-old child who developed VT with low cardiac output syndrome with deranged hepatic function, who was managed efficiently using suppressive atrial pacing to ameliorate the effects of sustained VT.
4 citations
TL;DR: In this article, Stented bovine jugular vein graft (Melody) has been surgically implanted in infants with mitral stenosis and/or regurgitation postatrioventricular septal defect (AVSD) repair, congenital mitral valve dysplasia (1), and Shone's complex (1).
Abstract: 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.
4 citations
TL;DR: In this article, the deceleration time of the mitral inflow velocity was significantly shorter in patients with Type 1 diabetes compared with controls when compared with the absence of overt heart failure and normal ejection fraction.
Abstract: 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.
3 citations
TL;DR: In this paper, the authors report two cases of children presenting with typical diagnosis of pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 who suffered from complete heart block requiring pacemaker placement which is an unusual presentation of PIMS-TS.
Abstract: In the midst of the COVID-19 pandemic, we report two cases of children presenting with typical diagnosis of pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) who suffered from complete heart block requiring pacemaker placement which is an unusual presentation of PIMS-TS. We also compared it with the already existing data with similar manifestations. PIMS-TS is reported to occur in children with predominantly gastrointestinal symptoms, hemodynamic instability, and myocardial dysfunction. The implications of development of atrioventricular block during critical illness in PIMS-TS are yet unknown. Both patients had an otherwise normal cardiac structure and had no gastrointestinal symptoms but suffered complete heart block without any other identifiable etiology, both requiring temporary pacemaker placement. While one child recovered completely with medical management, the other child required permanent pacemaker placement. While we cannot be certain that COVID-19 was the cause, complete heart block appeared to be temporally related to COVID-19 infection in both patients, and hence, it is important for pediatricians to be aware of the potential manifestation of this disease.
3 citations
TL;DR: It is concluded that high dose Aspirin does not have a significant role in preventing coronary arteries abnormalities in Kawasaki disease and giving standard 2 gr/kg/day Intravenous immunoglobulin without high-dose AspirIn in acute-phases therapy does not increase the risk of coronary arteries abnormality.
Abstract: Background: Standard first-step therapy for Kawasaki disease consists of Intravenous immunoglobulin and high dose Aspirin (80-100 mg/kg/day). The standard dose of Intravenous immunoglobulin (2gr/kg) is strongly effective in reducing the risk of coronary arteries abnormalities. So, the proper dose and efficacy of Aspirin to decrease the risk of coronary arteries abnormalities is a controversial issue. In this study, it is tried to assess the result of eliminating high-dose Aspirin in the treatment of the acute phase of Kawasaki and observe the incidence rate of coronary arteries abnormalities when only Intravenous immunoglobulin was administered. Methods: This study is a prospective randomized, open-label, blinded end-points clinical trial performed in Afzalipour hospital in Kerman University of Medical Sciences from September 2017 to September 2018 in 62 patients with typical and atypical Kawasaki disease. The study group received Intravenous immunoglobulin (2 g/kg) and the control group get the same dose of Intravenous immunoglobulin plus Aspirin with the dose of 80-100 mg/Kg/day until they were afebrile for 48 hours. Afterward, both groups received a daily single dose (3-5 mg/kg) of Aspirin for six weeks. Echocardiography was done after two weeks, six weeks, and six months. Internal the diameter of the left and right main coronary arteries was measured and then the corresponding Z-score was calculated. Results: In the study group, coronary arteries abnormalities decreased from 38.7% in the 2nd week to 16.1% in the 6th month. In the control group, it declined from 54.8% to 22.6%. There was no statistically significant difference between the groups in term of frequency abnormal coronary arteries abnormality at the study period (P=0.151). Conclusions: We concluded that high dose Aspirin does not have a significant role in preventing coronary arteries abnormalities in Kawasaki disease and giving standard 2 gr/kg/day Intravenous immunoglobulin without high-dose Aspirin in acute-phases therapy does not increase the risk of coronary arteries abnormality.
3 citations
TL;DR: The experience with the case of a 7-month-old infant with a giant unresectable cardiac fibroma who was successfully managed through OHT is reported, which shows Orthotopic heart transplantation (OHT) can be an exceptional but adequate alternative for some giant unre sectable CF in children.
Abstract: Cardiac fibromas (CF) are the second most common cardiac tumors in children. They can be aggressive tumors despite their benign histopathologic nature, accounting for the highest mortality rate among primary cardiac tumors. CF usually show a progressive growth and spontaneous regression is rare. Therefore, a complete surgical excision is the preferred therapeutic approach when patients become symptomatic or if mass-related life-threatening complications are anticipated, even in asymptomatic patients. However, some cases are not good candidates for surgical excision due to the impossibility of preserving a normal cardiac anatomy or function after the tumor resection. Orthotopic heart transplantation (OHT) can be an exceptional but adequate alternative for some giant unresectable CF in children. In this article, we report our experience with the case of a 7-month-old infant with a giant unresectable cardiac fibroma who was successfully managed through OHT.
3 citations
TL;DR: In this article, the authors modified surgical strategies to preserve the valve and annulus if the pulmonary valve leaflets are pliable and not significantly dysplastic during tetralogy of fallot (TOF) repair.
Abstract: Background : During repair of tetralogy of fallot (TOF) we modified surgical strategies to preserve the valve and annulus if the pulmonary valve leaflets are pliable and not significantly dysplastic. Methods : Initially, the repair was done from the main pulmonary artery (Group-1, 215 patients) and later through an additional incision in the infundibulum of the right ventricle (Group-2, 73 patients). Recently, we changed the approach to commissurotomy of the fused leaflets by releasing the supra valvar tethering and delamination of the cuspal apparatus till the base to improve the mobility of the cusps and do a controlled commissurotomy (Group-3, 14 patients). With delamination, we could extend the limit of the repair to a z-score of -3.5. Results : There was no hospital mortality; two patients died at home after discharge. A mean follow-up of 42.01 months ± 19.25 is available for 198 patients (92%) for group 1, 16.03 ± 7.45 for group 2, and 4.07 ± 2.09 for group 3. The re-intervention-free survival is 94.4% in group 1. The z value improved from -3 (-3–-2) to -1.2 (-3 – 0), P = 0.001 in Group 1, from -2.8 (-3–-2.4) to -1 (-1.1–-0.7), P = 0.001 in Group 2 and from –3 (-4–-3) to -1, P = 0.001 in Group 3. In all the groups, there was trivial or mild pulmonary regurgitation. Conclusions : During repair of TOF, adequate valve/annulus sparing is possible if the repair is done from both the main pulmonary artery and infundibular incisions using the delamination technique.
TL;DR: In this paper, the authors present an early multicenter experience of percutaneous pulmonary valve implantation (PPVI) using Melody valve (Medtronic Inc., Minneapolis, MN).
Abstract: Background Transcatheter valves provide a safe and effective alternative to surgery for treating dysfunctional right ventricular outflow tracts (RVOTs). We present our early multicenter experience of percutaneous pulmonary valve implantation (PPVI) using Melody valve (Medtronic Inc., Minneapolis, MN). Methods Patients with stenosed conduits or degenerated bioprosthetic valves in RVOT with combined stenosis and regurgitation were evaluated for suitability of Melody valve implantation. After undergoing an initial structured training, PPVI using Melody transcatheter pulmonary valve (TPV) was guided by an approved proctor. Conduits were serially dilated and prestented with careful coronary interrogation, and bioprosthetic valves were dilated with high-pressure balloons. Clinical and echocardiographic follow-up was performed at 6 monthly intervals. Results Fifteen patients (three females) aged 23.1 ± 9.5 years in NYHA Class II-III underwent Melody TPV implantation in four Indian centers. The underlying anatomy comprised surgically implanted bioprosthetic valves for pulmonary regurgitation (n= 5), conduit repair for pulmonary atresia (n = 4), Rastelli repair (n = 3), truncus (n = 1), and Ross procedure (n = 2). Twelve patients had more than one previous surgery. Doppler gradient decreased from 74.2 ± 21.5 mmHg to 10.2 ± 4.5 mmHg after the PPVI. At a median follow-up of 14 months (1-39 months), all the patients were in NYHA Class I with echocardiographic gradients of 8 ± 5.7 mmHg with no evidence of pulmonary regurgitation. There were no major procedural adverse events or deaths. Conclusions Our early experience shows encouraging results of the PPVI program in India with proctored case selection and meticulous planning. It also confirms the safety and efficacy of Melody TPV for treating dysfunctional RVOT in postoperative patients.
TL;DR: In this paper, the authors describe two cases of tachycardia-induced cardiomyopathy secondary to incessant ectopic atrial tachia (EAT) in an infant presenting with severe left ventricular dysfunction and hemodynamic instability.
Abstract: This report describes two cases of tachycardia-induced cardiomyopathy secondary to incessant ectopic atrial tachycardia (EAT) in an infant presenting with severe left ventricular dysfunction and hemodynamic instability. The two cases were managed differently. The first required mechanical ventilation and was resistant to conventional antiarrhythmic drugs. After the initiation of enteral ivabradine (0.15mg/kg) the heart rate slowed with significant improvement in hemodynamics, peripheral perfusion and sinus rhythm was restored after 12 hours. Ivabradine was continued and the patient was discharged home after 10 days of hospitalization. The second case was managed by early initiation of ivabradine and resulted in restoration of sinus rhythm within 4 hours, thus avoiding trials of conventional anti-arrhythmic drugs with unstable hemodynamic profile. The infant was discharged after 5 days of hospitalization on ivabradine..
TL;DR: An asymptomatic 3-year-old with Loeys-Dietz Syndrome (LDS) followed for a small patent ductus arteriosus and dilated aorta was found to have a massive ductal aneurysm on routine surveillance cardiac magnetic resonance.
Abstract: An asymptomatic 3-year-old with Loeys-Dietz Syndrome (LDS) followed for a small patent ductus arteriosus and dilated aorta was found to have a massive ductal aneurysm on routine surveillance cardiac magnetic resonance. The aneurysm was successfully resected. Serial advanced imaging tools are useful in surveillance, diagnosis, and management in patients with LDS.
TL;DR: Hoover's sign and raised hemidiaphragm on chest X-ray are the most specific clinical signs to suspect unilateral DP and a good diagnostic investigation comparable to fluoroscopy.
Abstract: Objective : The aims of our prospective observational study were to evaluate the (1) reliability of clinical signs in the early detection of diaphragm palsy (DP); (2) reliability of ultrasonography using echo machine as a bedside tool for the diagnosis of DP; and (3) does early diaphragm plication result in the improved outcome? We also sought to determine the incidence and predominant risk factors for DP and diaphragm plication at our center. Materials and Methods : This prospective observational study included patients with suspected DP from January 2015 to December 2018. Patients with suspected DP were initially evaluated by bedside ultrasonography using echo machine and confirmed by fluoroscopy. Diaphragm plication was considered for patients having respiratory distress, difficult weaning, or failed extubation attempt without any obvious cardiac or pulmonary etiology. Patients were followed for 3 months after discharge to assess diaphragm function. Results : A total of 87 patients were suspected of DP based on clinical signs. DP was diagnosed in 61 patients on fluoroscopy. The median time from index operation to diagnosis was 10 (1–59) days. Diaphragm plication was done among 52 patients and not done in nine patients. Bedside ultrasonography using echo machine was 96.7% sensitive and 96.15% specific in diagnosing DP. Early plication ( Conclusion : Hoover's sign and raised hemidiaphragm on chest X-ray are the most specific clinical signs to suspect unilateral DP. Bedside ultrasonography using an echo machine is a good diagnostic investigation comparable to fluoroscopy. Early plication facilitates weaning from the ventilator and thereby decreases the ICU stay and hospital stay.
TL;DR: A case of persistent atypical atrial flutter following device closure of ASD in a young man is described and the management strategy given the various underlying dilemmas are discussed.
Abstract: Atrial flutter is uncommon in young patients with uncorrected atrial septal defect (ASD). Although rare, it has been reported in the younger population following device closure of ASD/patent foramen ovale. We describe a case of persistent atypical atrial flutter following device closure of ASD in a young man and discuss the management strategy given the various underlying dilemmas.
TL;DR: Postoperative RVOT gradient was not altered by changing depth of anesthesia provided systemic blood pressure was maintained and one month postrepair RVOT gradients were significantly reduced as compared to the intraoperative values.
Abstract: 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 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.
TL;DR: The case of an iatrogenic pericardial defect in an asymptomatic 20-year-old female is presented and it is shown that the defect may present with cardiac strangulation.
Abstract: Pericardial defects are uncommon, usually congenital, and frequently involve a complete absence of the pericardium. Partial pericardial defects are more likely to result in complications. Iatrogenic pericardial defects are usually partial defects and may present with cardiac strangulation. We present the case of an iatrogenic pericardial defect in an asymptomatic 20-year-old female.
TL;DR: Being a planar structure, fetal diagnosis of aorto-pulmonary window poses great challenge but a few echocardiographic signs can help to clinch the diagnosis.
Abstract: Being a planar structure, fetal diagnosis of aorto-pulmonary window poses great challenge. A few echocardiographic signs can help to clinch the diagnosis.
TL;DR: An unusual variant of transposition is described, 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.
Abstract: 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).
TL;DR: In situ pulmonary artery thrombosis (ISPAT) is a unique form of pulmonary embolism characterized by local formation of thrombus in the pulmonary arteries as discussed by the authors.
Abstract: In situ pulmonary artery thrombosis (ISPAT) is a unique form of pulmonary embolism characterized by local formation of thrombus in the pulmonary arteries. We present here a baby with hypoplastic left heart syndrome who developed ISPAT after Glenn surgery. The patient underwent catheter-directed thrombolysis, followed by systemic anticoagulation with excellent results.
TL;DR: In this article, the authors used a PDA device delivery sheath to keep the balloon positioned at the desired location in order to dilate a dysplastic pulmonary valve (PV).
Abstract: Balloon pulmonary valvuloplasty (BPV) is an interventional procedure that has practically replaced the need of open-heart surgery in patients with severe valvular pulmonary stenosis. However, BPV in dysplastic pulmonary valve (PV) still remains a challenge in the current milieu of advanced technologies. We encountered a unique situation of severe restenosis of PV wherein the attempt to balloon dilate the valve was futile because no balloon remained stable at the level of the valve. This situation was overcome using a PDA device delivery sheath to keep the balloon positioned at the desired location.
TL;DR: The peak left atrial longitudinal strain obtained by atrial deformation analysis could potentially be diagnostic of this condition in children who do not have abnormalities identified on conventional assessment as mentioned in this paper, which is a poorly understood febrile illness potentially linked to an immune response to COVID-19 infection.
Abstract: The multi-inflammatory syndrome in children is a poorly understood febrile illness potentially linked to an immune response to COVID-19 infection. The disease is characterized by fever and elevated acute-phase reactants. A number of children with clinical and laboratory evidence of cardiovascular involvement have normal echocardiograms by conventional assessment. The peak left atrial longitudinal strain obtained by atrial deformation analysis could potentially be diagnostic of this condition in children who do not have abnormalities identified on conventional assessment.
TL;DR: A rare form of a cervical aortic arch is described and the latest methods of improved visualization using 3D virtual reality displays and smartphones are discussed.
Abstract: A cervical aortic arch is a rare vascular malformation that is characterized as a high positioned aortic arch, above the clavicle. The knowledge of its branching pattern is essential to characterize the entity further accurately. Noninvasive cross-sectional imaging, including computed tomography angiography or magnetic resonance angiography, is the imaging methods of choice. Due to highly complex anatomy, three-dimensional (3D) images help in providing improved anatomical visualization. Virtual reality is a relatively new computer-generated simulation technique that allows the interactive display of high-resolution models using a wearable headset and interactive controllers. We describe a rare form of a cervical arch and briefly discuss the latest methods of improved visualization using 3D virtual reality displays and smartphones.
TL;DR: A child presenting initially with atypical KD and coronary artery aneurysms is described, demonstrating unusual cardiac manifestations of KD and reoriented the protocol for long-term surveillance in infantile KD.
Abstract: Kawasaki disease (KD) is the most common pediatric vasculitis with coronary involvement feared as the most serious complication. The reported case describes a child presenting initially with atypical KD and coronary artery aneurysms. He was treated with intravenous immunoglobulin and aspirin. In spite of adequate compliance and no clinical recurrence, serial echocardiography revealed nonregression of aneurysm and new-onset moderate aortic regurgitation (AR) in the subacute phase produced by prolapse of noncoronary cusp of the aortic valve. AR without aortic root dilatation from persistent inflammation of the valve leaflets in KD is a rare phenomenon. This case demonstrates unusual cardiac manifestations of KD and reoriented our protocol for long-term surveillance in infantile KD.
TL;DR: This is the first case of aortic stenosis (AS) associated with DeSanto-Shinawi syndrome, and the syndrome may be the reason for the rapid worsening of AS in this case.
Abstract: A 3-month-old female was diagnosed at 1 month of age with DeSanto-Shinawi syndrome (DSS) and bicuspid aortic valve with trivial stenosis. The aortic valve stenosis progressed to severe within 2 months and required balloon aortic valvuloplasty. This is the first case of aortic stenosis (AS) associated with DSS, and the syndrome may be the reason for the rapid worsening of AS in this case.
TL;DR: The use of selexipag in the current teenage patient improved her quality of life and exercise capacity and the lack of childhood data regarding the use of this drug in pediatric patients with PAH was disappointing.
Abstract: Pulmonary arterial hypertension (PAH) is a chronic, progressive, multifactorial disease. Currently, combination therapy is an attractive option for PAH management because three pathways (endothelin, nitric oxide, and prostacyclin) are involved in this disease. Selexipag is a novel oral prostacyclin pathway drug and is a highly selective IP prostacyclin receptor agonist with vasodilatory and antiproliferative effects. We report the case of a teenage patient with idiopathic PAH who presented in World Health Organization functional Class IV and showed no clinical improvement with dual therapy. We added oral selexipag to the treatment regimen and observed substantial improvement in her quality of life at the short-time follow-up. Despite the lack of childhood data regarding the use of selexipag in pediatric patients with PAH, the use of this drug in the current teenage patient improved her quality of life and exercise capacity.
TL;DR: In this paper, the authors describe the technique of epicardial echocardiography and the various views that have been found useful for a complete postsurgical evaluation, and the limitations of the technique are also discussed.
Abstract: Intraoperative echocardiography is an integral component of the peri-operative management of pediatric heart disease. It confirms the adequacy of surgery, identifies residual lesions, and can provide useful hemodynamic data. It, therefore, helps to decide on the need for revision of repair and guides the postoperative management strategy. Intraoperative echocardiography is done with the use of either an epicardial probe or a transesophageal probe. Epicardial echocardiography is a simple, useful modality and has the ability to perform imaging in cases where transesophageal echocardiography cannot be easily performed, for example, in low birth weight babies. We attempt to describe in detail the technique of epicardial echocardiography and the various views that we have found useful for a complete postsurgical evaluation. The limitations of the technique are also discussed in detail.
TL;DR: The electrophysiology study confirmed the tachycardia as left posterior fascicular VT and it was successfully ablated guided by Purkinje potential on three-dimensional mappings and showed improvement in ventricular functions before discharge.
Abstract: A 10-years-old boy presented with a history of effort intolerance and palpitations for 4 months. His electrocardiogram showed wide complex tachycardia suggestive of fascicular ventricular tachycardia (VT). The echocardiogram showed moderate-to-severe left ventricular systolic dysfunction without any structural lesion. The tachycardia was unresponsive to adenosine and direct current cardioversion. It responded to oral verapamil. The electrophysiology study confirmed the tachycardia as left posterior fascicular VT. The tachycardia was successfully ablated guided by Purkinje potential on three-dimensional mappings. He showed improvement in ventricular functions before discharge. He is doing well on short-term follow-up.