{"title":"Early and midterm results of Cook Formula stent in children with right heart disease: A single-center experience.","authors":"Haritha Girija, Chinnasamy Sivaprakasam Muthukumaran, Rajaguru Ganesan, Sankarakuttalan Ramkishore, Vijay Arun, Vellusamy Mahitha, Neville Solomon, Swaminathan Vaidyanathan","doi":"10.4103/apc.apc_176_24","DOIUrl":"https://doi.org/10.4103/apc.apc_176_24","url":null,"abstract":"<p><strong>Background: </strong>Branch pulmonary artery (PA) stenosis must be addressed early to prevent right ventricular scarring and establish lung blood flow. Balloon-mounted stents are more useful in managing right ventricular outflow tract (RVOT) obstruction and PA stenosis.</p><p><strong>Materials and methods: </strong>We studied the clinical and angiographic data of children with congenital heart disease who underwent stenting for RVOT obstruction and branch PA stenosis using the Formula stent (Cook Medical, Limerick, Ireland) between 2018 and 2024 in a tertiary pediatric cardiac center in southern India.</p><p><strong>Results: </strong>Our patient cohort included 65 patients with a median age of 3 years (range: 3 months to 17 years). The median weight of patients was 11.5 kg (4.8-60.4 kg), with 40% weighing <10 kg. Out of the 87 stents implanted, four stents were implanted in RVOT, while the rest were branch PA stenting. The median stent length was 20 mm (12-60 mm), and the median stent diameter was 10 mm (5-10 mm). All stents were deployed using 5Fr to 8Fr sheaths or guiding catheters. The median procedure time was 62 min (20-170 min), with a median fluoroscopy time of 15 min (4-110 min). A total of 17 (19%) stents in 14 patients required further dilatation at a mean interval of 25 ± 4 months from the time of stenting. Ballooning resulted in an increase in diameter from 7.8 ± 2.2 mm to 10.2 ± 1.7 mm. Right ventricular systolic pressure had reduced from 70.3 ± 8 to 40.1 ± 1 mmHg after balloon dilatation in patients with a biventricular heart.</p><p><strong>Conclusion: </strong>The Formula stent provides a comprehensive stent size option, requiring smaller sheaths, reduced procedural time, minimum complications, and an acceptable rate of reinterventions.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 5","pages":"347-355"},"PeriodicalIF":0.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11737630/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142998979","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Systemic artery aneurysms - A marker of high-risk Kawasaki disease.","authors":"Lamk Kadiyani, Shivaprasad P Mohankumar, Praveen Arumugam, Saurabh Kumar Gupta, Narendra Kumar Bagri, Niraj Pandey, Sanjeev Kumar","doi":"10.4103/apc.apc_156_24","DOIUrl":"https://doi.org/10.4103/apc.apc_156_24","url":null,"abstract":"<p><p>Kawasaki disease, traditionally classified as medium vessel vasculitis, is known for the preferential involvement of coronary arteries. Infrequently, large systemic arteries might be involved and may affect the prognosis. Here, we present an infant with Kawasaki disease who had extensive involvement of large vessels such as the abdominal aorta and medium vessels of the extremities, along with giant coronary artery aneurysms.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 5","pages":"377-380"},"PeriodicalIF":0.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11737632/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142997839","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nikita Panigrahi, Harohalli A Venkatesh, Manas Ranjan Mishra, Rajath Pejaver, Karthik N Nagesh
{"title":"Comparing the tissue Doppler-derived left ventricular myocardial performance index before and after recovery from respiratory distress in neonates: A prospective observational study.","authors":"Nikita Panigrahi, Harohalli A Venkatesh, Manas Ranjan Mishra, Rajath Pejaver, Karthik N Nagesh","doi":"10.4103/apc.apc_167_24","DOIUrl":"10.4103/apc.apc_167_24","url":null,"abstract":"<p><strong>Background and objective: </strong>Respiratory distress is the most common cause requiring neonatal intensive care unit admission. As respiratory and cardiac functions are closely interrelated, some cardiac dysfunction is expected in respiratory distress. The myocardial performance index (MPI) is an index to assess global myocardial function, easily measurable by bedside echocardiography and reliable. Here, we conducted this study to determine the change in cardiac function in neonates with respiratory distress before initiating respiratory support and after weaning from the support.</p><p><strong>Methodology: </strong>The study was carried out in 92 neonates with a gestational age of more than 32 weeks who required invasive or noninvasive respiratory support. The tissue Doppler left ventricular MPI (LV MPI) was calculated before the initiation of respiratory support and after weaning from respiratory support. The data were analyzed using a paired <i>t</i>-test and a Wilcoxon signed-rank test.</p><p><strong>Results: </strong>This study comprised 92 neonates with a median (interquartile range) LV MPI value of 0.56 (0.10) before initiation of respiratory support and 0.47 (0.04) after weaning from respiratory support with <i>P</i> < 0.001. The isovolumetric contraction time, isovolumetric relaxation time, and ejection time increased after weaning from respiratory support (all <i>P</i> < 0.0001). The severity of respiratory distress determined by invasive mode of ventilation and longer duration of respiratory support caused higher initial LV MPI before initiation of respiratory support compared with recovery and <i>P</i> < 0.001, suggesting subclinical ventricular dysfunction with respiratory distress in neonates.</p><p><strong>Conclusion: </strong>LV MPI was higher in neonates with respiratory distress and normalized after weaning from respiratory support, which indicates that neonates requiring respiratory support may have subclinical ventricular dysfunction and should be followed up carefully.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 5","pages":"331-338"},"PeriodicalIF":0.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11737619/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142998969","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lamk Kadiyani, P M Shivaprasad, Sivasubramanian Ramakrishnan, Anubhuti Rana, Niraj Pandey
{"title":"Generalized arterial calcification of infancy - Fetal diagnosis to postnatal management.","authors":"Lamk Kadiyani, P M Shivaprasad, Sivasubramanian Ramakrishnan, Anubhuti Rana, Niraj Pandey","doi":"10.4103/apc.apc_158_24","DOIUrl":"https://doi.org/10.4103/apc.apc_158_24","url":null,"abstract":"<p><p>Generalized arterial calcification of infancy is a rare entity with poor fetal and postnatal outcomes and high morbidity in survivors. Half of the cases are diagnosed intrauterine due to hemodynamic compromise, and the associated manifestations pose unique management challenges. We hereby report an account of a fetal diagnosis in a referral for hydrops with postnatal evaluation and management.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 5","pages":"369-371"},"PeriodicalIF":0.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11737626/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142998987","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Isolated absent pulmonary valve with intact ventricular septum in a young child: A rare case report.","authors":"Damandeep Singh, Aprateem Mukherjee, Sanjeev Kumar, Sivasubramanian Ramakrishnan","doi":"10.4103/apc.apc_135_24","DOIUrl":"https://doi.org/10.4103/apc.apc_135_24","url":null,"abstract":"<p><p>Absent pulmonary valve syndrome, commonly linked with tetralogy of Fallot and ventricular septal defect, is a rare congenital condition. It is exceedingly rare to have an isolated absent pulmonary valve with an intact ventricular septum without cardiovascular shunt lesions, such as an atrial/ventricular septal defect or patent ductus arteriosus. This report presents a case of such rarity involving a young child with recurrent lower respiratory tract infections.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 5","pages":"381-382"},"PeriodicalIF":0.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11737629/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142998991","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Satyavan Sharma, Akash Bhupesh Shah, Shankar Y Machigar, Rajendra Umbarkar
{"title":"Diagnosis and management of residual atrial septal defect after surgical failure: A case series.","authors":"Satyavan Sharma, Akash Bhupesh Shah, Shankar Y Machigar, Rajendra Umbarkar","doi":"10.4103/apc.apc_151_24","DOIUrl":"https://doi.org/10.4103/apc.apc_151_24","url":null,"abstract":"<p><p>Residual defect after surgical closure of atrial septal defect is extremely uncommon. This communication reports four cases encountered in a tertiary care center during the last three decades. Clinical diagnosis was challenging, and the diverse presentations included acute ischemic stroke, cyanosis, and right ventricular volume overload. The morphology of the residual defects was complex, and multimodality imaging (transesophageal echocardiography, peripheral venous contrast studies, computed tomography, and balloon occlusion) enabled accurate recognition. Percutaneous device closure was feasible in one but required repeat surgery owing to unfavorable anatomy in the others. The communication focuses on difficulties in diagnosis and management.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 5","pages":"356-360"},"PeriodicalIF":0.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11737621/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142998974","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Prevalence of immunological aberrations and 22q11.2 deletion in children with conotruncal anomalies: A cross-sectional study.","authors":"Souvik Das, Arun Kumar Baranwal, Amit Rawat, Ashwini Nair, Sanjeev Hanumantacharya Naganur, Anupriya Kaur, Anand Kumar Mishra, Ankur Jindal, Anit Kaur","doi":"10.4103/apc.apc_168_24","DOIUrl":"https://doi.org/10.4103/apc.apc_168_24","url":null,"abstract":"<p><strong>Introduction: </strong>22q11.2 deletion is associated with conotruncal anomalies and immunological aberrations. Given the common embryonic origin of conotruncus and thymus, conotruncal anomalies may be associated with immunological aberrations irrespective of 22q11.2 deletion. We planned to study the prevalence of immunological aberrations and 22q11.2 deletion among patients with conotruncal anomaly to understand the impact of their interplay.</p><p><strong>Patients and methods: </strong>Preoperative children (age <12 years) with conotruncal anomalies were evaluated for clinical dysmorphism, lymphocyte subsets by flowcytometry, immunoglobulin levels by nephelometry, and 22q11.2 deletion by multiplex ligand-dependent probe amplification (January 2021-June 2022). Patients with asplenia and polysplenia were excluded from immunological studies.</p><p><strong>Results: </strong>Major cardiac defects ([<i>n</i> = 101], [median age, 32 days]) included dextro-transposition of great arteries (d-TGA) - 41.6%, tetralogy of Fallot - 37.6%, double outlet right ventricle (DORV) - 13.9%, and truncus arteriosus - 4.9%. Four patients had polysplenia with situs inversus, while 17 had clinical dysmorphism. Flow cytometry (<i>n</i> = 82) revealed low absolute counts of lymphocytes (33%), T-cells (51.2%), CD4+ cells (50%), and CD8+ cells (51.2%), while only 14.1% had low IgG levels. Eight patients (8/95, 8.4%) had 22q11.2 deletion, with universal deletion of <i>TBX1-2</i> and <i>TBX1-7 genes</i>; the other 19 genes were deleted in various combinations. Two patients with 22q11.2 deletion had normal T-cell subsets, while none had a complete absence of T-cells.</p><p><strong>Conclusion: </strong>Immunological aberrations, especially T-cell abnormalities, were present in almost half of the patients, irrespective of 22q11.2 deletion. Only 8.4% of patients had 22q11.2 deletion. The high incidence of d-TGA among 22q11.2 deletion patients needs further exploration.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 5","pages":"339-346"},"PeriodicalIF":0.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11737628/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142999008","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The case of the missing KONAR MF™ Occluder - Can we make some policy changes to minimize harmful radiation?","authors":"Avinash Anantharaj, Karan Kalani, Gulbadin Mufti, Sataroopa Mishra, Bhagwati Prasad Pant","doi":"10.4103/apc.apc_137_24","DOIUrl":"10.4103/apc.apc_137_24","url":null,"abstract":"","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 5","pages":"386-387"},"PeriodicalIF":0.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11737622/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142997965","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Successful heart transplantation after 4 years of bridging with HeartWare HVAD for left ventricular noncompaction in an 11-year-old boy with prohibitively high pulmonary vascular resistance.","authors":"Komarakshi R Balakrishnan, Sureshkumaran Kandasami, Suresh Rao Kemundel Genny, Rajesh Kumar Ramaswamy, Ratnagiri Ravikumar","doi":"10.4103/apc.apc_95_24","DOIUrl":"https://doi.org/10.4103/apc.apc_95_24","url":null,"abstract":"<p><p>End-stage heart failure due to left ventricular noncompaction (LVNC) poses unique challenges for ventricular assist device implantation, like inflow cannula obstruction due to trabeculations. We report a case of an 11-year-old boy with LVNC who underwent successful HeartWare implantation as a bridge to transplant for high pulmonary vascular resistance and had successful heart transplantation after 4 years of HeartWare support.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 5","pages":"361-363"},"PeriodicalIF":0.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11737625/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142997743","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Transcatheter salvage of dying Glenn - An innovative strategy for postGlenn thrombosis.","authors":"Shantanu Jain, Debasree Gangopadhyay, Sabharisundaravel Paulraj, Mahua Roy, Jayitri Mazumdar, Joyeeta Dutta","doi":"10.4103/apc.apc_187_24","DOIUrl":"https://doi.org/10.4103/apc.apc_187_24","url":null,"abstract":"<p><p>PostGlenn thrombosis is an infrequent, serious complication requiring immediate attention, which is not uncommon in a resource-limited setting where patients are often lost to follow-up and have poor compliance with medications. This rare complication has infrequently been reported in the past. We describe a case of a massive thromboembolic event in a patient presenting acutely in a decompensated state 14 years after initial palliation by Glenn shunt. We report an innovative strategy of using transcatheter mechanical thrombectomy using a rotational thrombectomy device and chemical thrombolysis for the successful salvation of the Glenn circuit.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 5","pages":"372-376"},"PeriodicalIF":0.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11737623/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142998376","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}