{"title":"Berry syndrome: First Successful neonatal single-stage repair in India.","authors":"Shefali Yadav, Anil Bhan, Rajesh Sharma, Amit Misri, Akriti Gera, Pankaj Bajpai","doi":"10.4103/apc.apc_81_24","DOIUrl":"10.4103/apc.apc_81_24","url":null,"abstract":"<p><p>Berry <i>et al</i>. (1982) described a rare syndrome associated with distal aortopulmonary window, aortic origin of the right pulmonary artery, intact ventricular septum, and interruption or coarctation of the aorta. Here, we present the first neonatal case of single-stage repair of Berry syndrome in India. Timely surgery and skilled postoperative care define the short-term and long-term outcomes. Single-staged repair is preferred wherever feasible.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 3","pages":"224-226"},"PeriodicalIF":0.9,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11573198/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142674976","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":"Percutaneous closure of aortopulmonary window in a preterm infant.","authors":"Aishwarya Prakash Gurav, Shine Kumar, Raman Krishna Kumar","doi":"10.4103/apc.apc_57_24","DOIUrl":"10.4103/apc.apc_57_24","url":null,"abstract":"<p><p>Aorto-pulmonary window (APW) is a rare congenital heart disease. The non-restrictive types of APWs present at an early age and require early surgical correction. The transcatheter device closure in young infants pose significant challenges that include hemodynamic instability during arteriovenous loop formation and due to potential encroachment of the occlusive device on vital adjacent structures, and sizing of introducer sheaths and delivery systems. We report a successful transcatheter closure of an AP window in a 1.35 Kg preterm infant with heart failure requiring mechanical ventilation. To the best of our knowledge this is the smallest and youngest baby , wherein transcatheter device closure has been attempted for a non-restrictive aorto-pulmonary window. The procedure was done through a 4 French right femoral venous access, thus avoiding an arterial access. A 0.025 Terumo wire was maneuvered through the pulmonary artery across the APW without formation of an arteriovenous loop. The APW was successfully occluded with a Konar multi-functional device delivered via a 4-French delivery system. The baby could be successfully weaned off the ventilator and could be discharge. On 1 month follow up, baby was in good health and gaining adequate weight. Thus we conclude that careful selection of patients allows successful closure of APW, even in the smallest baby.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 3","pages":"204-206"},"PeriodicalIF":0.9,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11573187/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142674751","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}
Sophia Livas de Morais Almeida, Andreza Dos Santos Laranjeira da Cunha, Renata de Castro Silva, Raillon Keven Dos Santos, Ana Luiza Menezes Teles Novelleto, Thayla Lais Lima Estevam, Luana Izabela Azevedo de Carvalho, Luísa Tiemi Souza Tuda, Celsa da Silva Moura Souza, Edward Araujo Júnior, Luciane Alves da Rocha Amorim
{"title":"Challenges in congenital heart disease in the Amazon region countries: A scoping review.","authors":"Sophia Livas de Morais Almeida, Andreza Dos Santos Laranjeira da Cunha, Renata de Castro Silva, Raillon Keven Dos Santos, Ana Luiza Menezes Teles Novelleto, Thayla Lais Lima Estevam, Luana Izabela Azevedo de Carvalho, Luísa Tiemi Souza Tuda, Celsa da Silva Moura Souza, Edward Araujo Júnior, Luciane Alves da Rocha Amorim","doi":"10.4103/apc.apc_73_24","DOIUrl":"10.4103/apc.apc_73_24","url":null,"abstract":"<p><strong>Introduction: </strong>This study aimed to systematically analyze and describe the main challenges of congenital heart diseases (CHDs) in the countries in the Amazon region.</p><p><strong>Methods: </strong>The methodology followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews checklist utilizing the Medline, Embase, Lilacs, and Google Scholar databases. The inclusion criteria were articles addressing any topic involving CHD in the Amazon region. Incomplete articles, book chapters, lectures, conference abstracts, and editorials were excluded.</p><p><strong>Results: </strong>Nine studies were identified, 7 of which were published in the last decade and were of Colombian and Brazilian origin. The methodology of the studies was cross-sectional and ecological, evaluating the regional and epidemiological factors, challenges to diagnosis and treatment, multidisciplinary team challenges, and the impact of the COVID-19 pandemic. Studies carried out by surgeons demonstrate more existing data regarding the challenges of the health-care system.</p><p><strong>Conclusions: </strong>There is growing interest in analyzing the situation of CHD in the region. However, only a few studies are available, mostly on ecological and cross-sectional analysis. These records show the lack of hospital infrastructure and multidisciplinary teams for the diagnosis and treatment of CHD in the Amazon region; we see an initiative by specialists from two countries (Colombia and Brazil) to demonstrate the difficulties by seeking international training programs and government aid to improve the health system situation.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 3","pages":"188-195"},"PeriodicalIF":0.9,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11573190/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142674979","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":"Jammed joints and constricted heart: The science of tribology and missing lubricin. A case report on camptodactyly-arthropathy-coxa vara-pericarditis syndrome.","authors":"Krishnan Ganapathy Subramaniam, Satish Mohanty, Dhruva Sharma, Komal Tamildasan, Narahari Srinath Reddy","doi":"10.4103/apc.apc_18_24","DOIUrl":"10.4103/apc.apc_18_24","url":null,"abstract":"<p><p>An autosomal recessively inherited noninflammatory arthropathy known as camptodactyly, arthropathy, coxa vara, and pericarditis (CACP) syndrome was discovered in 1999. It is distinguished by synoviocyte hyperplasia and subcapsular fibrosis of the synovial capsule, which results in a shortage of lubricin production. The resulting lack of joint lubrication induces increased mechanical stress, causing progressive deformities that become evident with weight-bearing and heightened joint activity. Animal models with a lubricin gene knock-out display similar traits, underscoring the impact of mechanical stress on disrupting type II collagen on the articular surface. The gradual development of pericarditis and constriction often results in misdiagnosis as juvenile rheumatoid arthritis with cardiac involvement, but the defining feature remains the noninflammatory nature of the disease. Early recognition is pivotal, as interventions such as pericardiectomy and recombinant human lubricin hold promise for altering the disease's natural course. In our familial case of CACP, two siblings exhibited distinct phenotypic variations - one with fibrosis-dominant features and pericardial constriction and the other displaying synovial hyperplasia without pericardial involvement.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 3","pages":"221-223"},"PeriodicalIF":0.9,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11573197/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142674481","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":"Long-term clinical course of patients with catecholaminergic polymorphic ventricular tachycardia: A more than 10-year follow-up cohort study.","authors":"Ekaterina Kulbachinskaya, Vera Bereznitskaya","doi":"10.4103/apc.apc_101_24","DOIUrl":"10.4103/apc.apc_101_24","url":null,"abstract":"<p><strong>Background: </strong>Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited disorder characterized by ventricular arrhythmias induced by physical or emotional stress. Currently, there are limited data available on the long-term prognosis of CPVT.</p><p><strong>Methods and results: </strong>In this study, which included both retrospective and prospective components, 12 patients with CPVT (7 males and 5 females) under 18 years old were enrolled to gather and evaluate demographic, clinical, and genetic data. The mean age at diagnosis onset was 7.0 ± 3.1 years. All patients experienced syncope. The mean follow-up duration was 20.1 years. During the follow-up period, all patients experienced at least one episode of supraventricular tachycardia (SVT). Despite beta-blocker therapy, nine patients experienced syncope (75%), and four patients were noncompliant with their treatment. An implantable cardiac defibrillator (ICD) implantation was performed in 10 patients (83%), and among those 5 (50%) experienced appropriate shocks. Inappropriate shocks were observed in all patients with an ICD. The left cardiac sympathetic denervation was performed in 6 patients (50%). One patient died during the follow-up period. Genetic testing was performed in eight patients, five of whom had <i>RYR2</i> mutations, one patient had mutations in <i>CASQ2</i>, one in <i>TECRL</i>, and one was gene-elusive.</p><p><strong>Conclusions: </strong>The prevalence of cardiac events, even after the initiation of beta-blocker therapy, was found to be distressingly high during long-term follow-up. SVT, such as atrial fibrillation, were found to be more common than previously thought. Combination therapy with a beta-blocker and an IC antiarrhythmic drug shows promise. An individualized approach to the selection of treatment strategies is essential.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 3","pages":"196-203"},"PeriodicalIF":0.9,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11573199/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142674407","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, Mani Kalaivani, Krishna S Iyer, Sivasubramanian Ramakrishnan
{"title":"The outcome of surgery for congenital heart disease in India: A systematic review and metanalysis.","authors":"Lamk Kadiyani, Mani Kalaivani, Krishna S Iyer, Sivasubramanian Ramakrishnan","doi":"10.4103/apc.apc_71_24","DOIUrl":"10.4103/apc.apc_71_24","url":null,"abstract":"<p><strong>Background: </strong>The mortality risks of children undergoing various cardiac surgeries for congenital heart disease (CHD) in India are not well defined. We conducted a systematic review and meta-analysis to estimate the inhospital mortality of various common CHD surgeries reported in India and compared it to representative data from established Western databases.</p><p><strong>Methods and results: </strong>We searched four bibliographic databases for studies published in India over the last 25 years. In total, 135 studies met the inclusion criteria and included 30,587 patients aged from 1 day to 65 years. The pooled mortality rate of 43 Indian studies reporting multiple CHD surgical outcomes is 5.63% (95% confidence interval [CI]: 4.26-7.16; <i>I</i> <sup>2</sup> = 93.9%), whereas the Western data showed a pooled mortality rate of 2.65% (<i>P</i> value for comparison <0.0001). The pooled mortality risk for ventricular septal defect closure and tetralogy of Fallot repair in Indian studies was 2.87% (95% CI: 0.76-5.91; <i>I</i> <sup>2</sup> = 62.4%) and 4.61% (95% CI: 2.0-8.02; <i>I</i> <sup>2</sup> = 87.4%), respectively. The estimated mortality risk was higher than the Western databases for all subcategories studied except for surgeries in the grown-ups with CHD population and coarctation repair.</p><p><strong>Conclusions: </strong>The estimated mortality risks are higher among Indian patients undergoing cardiac surgery for CHD as compared to Western data. We need prospective multicentric data to document whether the observed excess mortality exists after adjusting for various high-risk features and comorbidities in Indian patients. We need systemic measures to improve the outcomes of CHD surgeries in India.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 3","pages":"164-179"},"PeriodicalIF":0.9,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11573196/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142674758","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":"Interventional management of an unusual cause of cyanosis in repaired tetralogy of Fallot.","authors":"Lamk Kadiyani, Saurabh Kumar Gupta, Sivasubramaniam Ramakrishnan","doi":"10.4103/apc.apc_80_24","DOIUrl":"10.4103/apc.apc_80_24","url":null,"abstract":"<p><p>Arterial desaturation following surgical repair of tetralogy of Fallot (TOF) is rare. In most instances, it results from residual right ventricular outflow tract obstruction, causing right-to-left shunt across residual interatrial or interventricular communication. In this report, we present an unusual scenario of arterial desaturation due to a recanalized left cardinal vein in a child with repaired TOF. We also discuss stepwise evaluation that led to successful identification and occlusion of the abnormal venous channel by percutaneous device closure.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 3","pages":"217-221"},"PeriodicalIF":0.9,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11573195/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142675011","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}
Diego R Ruiz-Avila, Subhrajit Lahiri, Syed Javed Zaidi, Harma Khachig Turbendian
{"title":"Deferred Norwood in the setting of airway compression in double-inlet left ventricle with dextro-transposition of the great arteries.","authors":"Diego R Ruiz-Avila, Subhrajit Lahiri, Syed Javed Zaidi, Harma Khachig Turbendian","doi":"10.4103/apc.apc_30_24","DOIUrl":"10.4103/apc.apc_30_24","url":null,"abstract":"<p><p>A 4.1 kg male neonate with a diagnosis of double-inlet left ventricle with dextro-transposition of the great arteries was intubated shortly after birth due to respiratory insufficiency. The initial management consisted of a successful Stage I hybrid procedure. Persistent respiratory insufficiency led to cross-sectional imaging and bronchoscopy that demonstrated severe airway compression from a dilated main pulmonary artery. A Norwood procedure with Blalock-Thomas-Taussig shunt was performed at 1 month of age to relieve the airway obstruction. The patient was discharged home on room air at 2 months of age. This case highlights a unique single-ventricle anatomic variant with airway compression, which was successfully managed with deferred Norwood palliation.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 2","pages":"149-151"},"PeriodicalIF":0.9,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11343387/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142054737","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":"Artificial intelligence in pediatric cardiology: Where do we stand in 2024?","authors":"Supratim Sen, Sivasubramanian Ramakrishnan","doi":"10.4103/apc.apc_72_24","DOIUrl":"10.4103/apc.apc_72_24","url":null,"abstract":"","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 2","pages":"93-96"},"PeriodicalIF":0.9,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11343386/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142054734","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}