A neonate born of third-degree consanguineous marriage presented on day 12 of life with congestive cardiac failure. A male sibling died at 3 months of age, cause of which was not known. He was treated with decongestive measures and multiple inotropes. 2D Echocardiogram revealed severe Left ventricular dysfunction with prominent trabeculations and deep recesses in the left ventricle suggestive of Left ventricular non-compaction. He was also found to have horse-shoe kidney. Considering the presence of cardiac left ventricular non compaction, horse-shoe kidney and family history of neonatal death and pregnancy loss clinical exome sequencing was done. It detected a homozygous missense variant in exon 6 of the AGK gene suggestive of Senger's syndrome. Baby was on regular follow-up and was thriving well on diuretics, sacubitril-valsartan and weekly levosimendan infusions. At 8 months of age, cardiac transplantation was successfully done and baby has been doing well post-transplantation. LVNC in children is rare with an estimated incidence of 0.11 per 100,000, the highest incidence being during infancy. Senger's syndrome is autosomal recessive in inheritance. Senger's syndrome associated with Left ventricular non compaction has been reported only once in literature so far. Renal manifestations in the form of horse shoe kidney like in our index baby has not been reported previously with Senger's syndrome.
{"title":"A neonate with a spongy failing heart - What could it be?","authors":"Arumugom Archana, Chandra Kumar Natarajan, Vaanathi Hementha Kumar, Gnanasambandam Subramaniyam, Bala Ramachandran, Komarakshi Balakrishnan, K G Suresh Rao, Abhishek Berwal, Vishwanath Nandyala, Swati Iyer","doi":"10.4103/apc.apc_193_23","DOIUrl":"10.4103/apc.apc_193_23","url":null,"abstract":"<p><p>A neonate born of third-degree consanguineous marriage presented on day 12 of life with congestive cardiac failure. A male sibling died at 3 months of age, cause of which was not known. He was treated with decongestive measures and multiple inotropes. 2D Echocardiogram revealed severe Left ventricular dysfunction with prominent trabeculations and deep recesses in the left ventricle suggestive of Left ventricular non-compaction. He was also found to have horse-shoe kidney. Considering the presence of cardiac left ventricular non compaction, horse-shoe kidney and family history of neonatal death and pregnancy loss clinical exome sequencing was done. It detected a homozygous missense variant in exon 6 of the AGK gene suggestive of Senger's syndrome. Baby was on regular follow-up and was thriving well on diuretics, sacubitril-valsartan and weekly levosimendan infusions. At 8 months of age, cardiac transplantation was successfully done and baby has been doing well post-transplantation. LVNC in children is rare with an estimated incidence of 0.11 per 100,000, the highest incidence being during infancy. Senger's syndrome is autosomal recessive in inheritance. Senger's syndrome associated with Left ventricular non compaction has been reported only once in literature so far. Renal manifestations in the form of horse shoe kidney like in our index baby has not been reported previously with Senger's syndrome.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 1","pages":"81-84"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11198940/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141454777","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}
Background: Open-heart surgery is challenging in preterm neonates and infants, and its feasibility in low-resource settings has not been defined. We describe our institutional experience with open-heart surgeries performed on consecutive preterm infants.
Materials methods and results: This was a single-center retrospective cohort from a tertiary hospital in Southern India and included consecutive preterm neonates (<37 weeks) admitted for open-heart surgery. This report is limited to babies who were <3 months at the surgery. The salient features of the 15 preterm included twin gestation: 7 (46.7%); median gestational age at birth: 35 weeks (28-36 weeks); median corrected gestational age at surgery: 37 weeks (33-40 weeks); birth weight: 1.75 kg (1.0-2.6 kg); weight at surgery: 1.8 kg (1.2-2.9 kg); and small for gestational age: 12 (80%). The heart defects included transposition of the great arteries (7), total anomalous pulmonary venous return (3), large ventricular septal defect (VSD) (1), and VSD with coarctation of the aorta (4). Eleven (73%) were mechanically ventilated preoperatively and five had preoperative sepsis. The mean cardiopulmonary bypass time was 169.7 ± 61.5 min, and cross-clamp time was 99.7 ± 43.8 min. There was no inhospital mortality; one baby expired during follow-up at 1 month. Postoperative mechanical ventilation duration was 126.50 h (84.25-231.50 h), and intensive care unit stay was 13.5 days (9-20.8). The total hospital stay was 39 days (11-95 days). Two children (13.3%) had postoperative sepsis.
Conclusion: Through collaborative multidisciplinary management, excellent outcomes are feasible in low-resource environments for selected preterm neonates undergoing corrective open-heart operations.
{"title":"Open-heart surgery in preterm infants: A single-center experience.","authors":"Danish Memon, Praveen Reddy Bayya, Perraju Bendapudi, Jessin Puliparambil Jayashankar, Brijesh Parayaru Kottayil, Balaji Srimurugan, Raman Krishna Kumar","doi":"10.4103/apc.apc_3_24","DOIUrl":"10.4103/apc.apc_3_24","url":null,"abstract":"<p><strong>Background: </strong>Open-heart surgery is challenging in preterm neonates and infants, and its feasibility in low-resource settings has not been defined. We describe our institutional experience with open-heart surgeries performed on consecutive preterm infants.</p><p><strong>Materials methods and results: </strong>This was a single-center retrospective cohort from a tertiary hospital in Southern India and included consecutive preterm neonates (<37 weeks) admitted for open-heart surgery. This report is limited to babies who were <3 months at the surgery. The salient features of the 15 preterm included twin gestation: 7 (46.7%); median gestational age at birth: 35 weeks (28-36 weeks); median corrected gestational age at surgery: 37 weeks (33-40 weeks); birth weight: 1.75 kg (1.0-2.6 kg); weight at surgery: 1.8 kg (1.2-2.9 kg); and small for gestational age: 12 (80%). The heart defects included transposition of the great arteries (7), total anomalous pulmonary venous return (3), large ventricular septal defect (VSD) (1), and VSD with coarctation of the aorta (4). Eleven (73%) were mechanically ventilated preoperatively and five had preoperative sepsis. The mean cardiopulmonary bypass time was 169.7 ± 61.5 min, and cross-clamp time was 99.7 ± 43.8 min. There was no inhospital mortality; one baby expired during follow-up at 1 month. Postoperative mechanical ventilation duration was 126.50 h (84.25-231.50 h), and intensive care unit stay was 13.5 days (9-20.8). The total hospital stay was 39 days (11-95 days). Two children (13.3%) had postoperative sepsis.</p><p><strong>Conclusion: </strong>Through collaborative multidisciplinary management, excellent outcomes are feasible in low-resource environments for selected preterm neonates undergoing corrective open-heart operations.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 1","pages":"13-18"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11198942/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141454788","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}
We describe a unique report of percutaneous closure of multiple secundum atrial septal defects in a child utilizing three Occlutech Figulla septal occluders deployed sequentially. The procedure was performed under live three-dimensional transesophageal echocardiography guidance.
{"title":"Percutaneous closure of multiple atrial septal defects in a child utilizing three Occlutech Figulla septal occluders: A bailout.","authors":"Ziad Bulbul, Haytham Bou Hussein, Fadi Sawaya, Fadi Bitar","doi":"10.4103/apc.apc_202_23","DOIUrl":"10.4103/apc.apc_202_23","url":null,"abstract":"<p><p>We describe a unique report of percutaneous closure of multiple secundum atrial septal defects in a child utilizing three Occlutech Figulla septal occluders deployed sequentially. The procedure was performed under live three-dimensional transesophageal echocardiography guidance.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 1","pages":"67-69"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11198926/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141454790","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}
Pub Date : 2024-01-01Epub Date: 2024-05-24DOI: 10.4103/apc.apc_146_23
Hamid Mohammadi, Hossein Hosseini, Mohammadreza Bordbar, Nima Mehdizadegan, Hamid Amoozgar, Mohammad Reza Edraki, Amir Naghshzan, Nima Naderi, Elham Abedi, Kambiz Keshavarz
Objective: Anthracycline administration in children is associated with cardiac dysfunction. Speckle-tracking echocardiography (STE) can detect subclinical cardiac damage that may go undetected by conventional two-dimensional (2D) echocardiography. This study aims to investigate medium-term anthracycline cardiotoxicity using STE and determine a safer administrable level of anthracyclines (ACs).
Methods: This observational case-control study enrolled 37 healthy controls and 78 pediatric cancer survivors who received chemotherapy. The patients were divided into two groups: cardiotoxic received (CR) and cardiotoxic free (CF). Data on segmental longitudinal strain (LS), global LS (GLS), and 2D echocardiographic parameters were collected after a drug-free period of at least one year.
Results: A total of 115 children with a mean age of 108 ± 55 months, of whom 66% were males, were included in the study. Both the groups of cancer survivors exhibited significantly reduced GLS compared to healthy controls (CR vs. controls, P = 0.001; CF vs. controls, P = 0.013), but no significant difference in left ventricular ejection fraction (LVEF) was observed (P = 0.75). Overall, cancer survivors treated with ACs demonstrated a significant reduction in strain in 10 left ventricular segments, particularly in the basal segments (P < 0.05). Among CR patients, those with impaired GLS (n = 43, GLS worse than -21.9) had significantly higher mean age and cumulative anthracycline dose compared to CR patients with normal GLS (age, P = 0.024; anthracycline dosage, P = 0.036). Using an anthracycline cutoff of 223 mg/m2 resulted in a higher detection rate (49% vs. 25%) and fewer missed cases (51% vs. 74%) compared to the 360 mg/m2 anthracycline cutoff.
Conclusion: Childhood cancer survivors demonstrate significantly reduced GLS while preserving a normal LVEF, which does not differ significantly from reference values of healthy children. The reduction in strain appears to be associated with higher anthracycline doses and older age. Lowering the anthracycline threshold to 223 mg/m2 may improve the predictability of a decline in cardiac function using strain imaging at medium-term follow-up.
{"title":"Permanent longitudinal strain damage of cardiotoxic drugs in childhood cancer: What is the safe level?","authors":"Hamid Mohammadi, Hossein Hosseini, Mohammadreza Bordbar, Nima Mehdizadegan, Hamid Amoozgar, Mohammad Reza Edraki, Amir Naghshzan, Nima Naderi, Elham Abedi, Kambiz Keshavarz","doi":"10.4103/apc.apc_146_23","DOIUrl":"10.4103/apc.apc_146_23","url":null,"abstract":"<p><strong>Objective: </strong>Anthracycline administration in children is associated with cardiac dysfunction. Speckle-tracking echocardiography (STE) can detect subclinical cardiac damage that may go undetected by conventional two-dimensional (2D) echocardiography. This study aims to investigate medium-term anthracycline cardiotoxicity using STE and determine a safer administrable level of anthracyclines (ACs).</p><p><strong>Methods: </strong>This observational case-control study enrolled 37 healthy controls and 78 pediatric cancer survivors who received chemotherapy. The patients were divided into two groups: cardiotoxic received (CR) and cardiotoxic free (CF). Data on segmental longitudinal strain (LS), global LS (GLS), and 2D echocardiographic parameters were collected after a drug-free period of at least one year.</p><p><strong>Results: </strong>A total of 115 children with a mean age of 108 ± 55 months, of whom 66% were males, were included in the study. Both the groups of cancer survivors exhibited significantly reduced GLS compared to healthy controls (CR vs. controls, <i>P</i> = 0.001; CF vs. controls, <i>P</i> = 0.013), but no significant difference in left ventricular ejection fraction (LVEF) was observed (<i>P</i> = 0.75). Overall, cancer survivors treated with ACs demonstrated a significant reduction in strain in 10 left ventricular segments, particularly in the basal segments (<i>P</i> < 0.05). Among CR patients, those with impaired GLS (<i>n</i> = 43, GLS worse than -21.9) had significantly higher mean age and cumulative anthracycline dose compared to CR patients with normal GLS (age, <i>P</i> = 0.024; anthracycline dosage, <i>P</i> = 0.036). Using an anthracycline cutoff of 223 mg/m<sup>2</sup> resulted in a higher detection rate (49% vs. 25%) and fewer missed cases (51% vs. 74%) compared to the 360 mg/m<sup>2</sup> anthracycline cutoff.</p><p><strong>Conclusion: </strong>Childhood cancer survivors demonstrate significantly reduced GLS while preserving a normal LVEF, which does not differ significantly from reference values of healthy children. The reduction in strain appears to be associated with higher anthracycline doses and older age. Lowering the anthracycline threshold to 223 mg/m<sup>2</sup> may improve the predictability of a decline in cardiac function using strain imaging at medium-term follow-up.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 1","pages":"36-44"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11198932/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141454791","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}
Pub Date : 2024-01-01Epub Date: 2024-05-24DOI: 10.4103/apc.apc_177_23
Biswa Ranjan Panda, Jayashree Mishra
Retrieval of embolized ductal stents from the pulmonary or systemic circulation can be challenging. Most children benefit from surgical shunts in such scenarios. Although early retrieval is advised, stents lodged in the peripheral pulmonary tree can be inaccessible, making the removal complicated. In such patients, stents can be "parked" in the segmental pulmonary arterial branches for retrieval later. In the low-pressure single ventricle pulmonary circulation, partially expanded embolized stents, if left in situ, can precipitate pulmonary arterial thrombosis. This subset of patients may benefit from meticulous anticoagulation and antiplatelet agents. In our case report, we describe the successful extraction of an embolized ductal stent without damage to the right lower lobe pulmonary artery (PA). In the follow-up evaluation, the growth of the right PA is good.
{"title":"Delayed retrieval of embolized ductal stent: A surgical challenge.","authors":"Biswa Ranjan Panda, Jayashree Mishra","doi":"10.4103/apc.apc_177_23","DOIUrl":"10.4103/apc.apc_177_23","url":null,"abstract":"<p><p>Retrieval of embolized ductal stents from the pulmonary or systemic circulation can be challenging. Most children benefit from surgical shunts in such scenarios. Although early retrieval is advised, stents lodged in the peripheral pulmonary tree can be inaccessible, making the removal complicated. In such patients, stents can be \"parked\" in the segmental pulmonary arterial branches for retrieval later. In the low-pressure single ventricle pulmonary circulation, partially expanded embolized stents, if left <i>in situ</i>, can precipitate pulmonary arterial thrombosis. This subset of patients may benefit from meticulous anticoagulation and antiplatelet agents. In our case report, we describe the successful extraction of an embolized ductal stent without damage to the right lower lobe pulmonary artery (PA). In the follow-up evaluation, the growth of the right PA is good.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 1","pages":"64-66"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11198929/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141454782","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}
Pub Date : 2024-01-01Epub Date: 2024-05-24DOI: 10.4103/apc.apc_181_23
Nicola McCay, Keviin Walsh
We report an alternative technique for femoral access in neonates <3.5 kg undergoing cardiac catheterization. By modifying a 0.014" Balance middleweight Elite wire and using a 24-gauge Galt introducer needle, we have noted increased ease and success in obtaining vascular access in this challenging cohort.
我们报告了一种新生儿股骨入路的替代技术
{"title":"Alternative technique for femoral access in neonates undergoing cardiac catheterization.","authors":"Nicola McCay, Keviin Walsh","doi":"10.4103/apc.apc_181_23","DOIUrl":"10.4103/apc.apc_181_23","url":null,"abstract":"<p><p>We report an alternative technique for femoral access in neonates <3.5 kg undergoing cardiac catheterization. By modifying a 0.014\" Balance middleweight Elite wire and using a 24-gauge Galt introducer needle, we have noted increased ease and success in obtaining vascular access in this challenging cohort.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 1","pages":"52-54"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11198943/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141454778","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}
Pub Date : 2024-01-01Epub Date: 2024-05-24DOI: 10.4103/apc.apc_190_23
Pramod Sagar, Kothandam Sivakumar
Transcatheter closure of superior vena cava (SVC) form of sinus venosus defects (SVDs) using covered stents is emerging as an alternative to surgery in the current decade. A covered stent placed in the cavoatrial junction creates a roof for the right upper pulmonary vein (RUPV) that stops the left-to-right shunt and redirects the vein to the left atrium. While surgical literature has clearly documented the incidence of stenosis of SVC and RUPV, sinus nodal dysfunction, and persistent residual shunts following surgical correction, it is imperative to have similar data after this new transcatheter intervention on the incidence of complications and follow-up outcomes. Since patients with pretricuspid shunts are often clinically asymptomatic, correction is primarily performed to prevent a persistent right heart volume overload and allow remodeling of the heart chambers. Any residual left-to-right shunt after a correction will result in persistent right heart dilatation. Residual flows can result from various mechanisms, including lack of apposition of the covered stent to the free edge of the SVD, fabric breach, and persistent anomalous drainage of additional right-sided pulmonary veins that drain very high in the SVC or can be due to a coexistent defect in the oval fossa. This review analyzes the different mechanisms, explains the transesophageal and angiographic images for each one, and offers solutions tailored for various reasons. Different mechanisms warrant different treatment principles. A solution for residual shunt from one mechanism may not be appropriate for residual flow through another mechanism. A thorough understanding would aid the operator in effective interventions for these SVDs.
{"title":"Different mechanisms for persistent and residual left-to-right shunt after transcatheter sinus venosus defect closure and their management.","authors":"Pramod Sagar, Kothandam Sivakumar","doi":"10.4103/apc.apc_190_23","DOIUrl":"10.4103/apc.apc_190_23","url":null,"abstract":"<p><p>Transcatheter closure of superior vena cava (SVC) form of sinus venosus defects (SVDs) using covered stents is emerging as an alternative to surgery in the current decade. A covered stent placed in the cavoatrial junction creates a roof for the right upper pulmonary vein (RUPV) that stops the left-to-right shunt and redirects the vein to the left atrium. While surgical literature has clearly documented the incidence of stenosis of SVC and RUPV, sinus nodal dysfunction, and persistent residual shunts following surgical correction, it is imperative to have similar data after this new transcatheter intervention on the incidence of complications and follow-up outcomes. Since patients with pretricuspid shunts are often clinically asymptomatic, correction is primarily performed to prevent a persistent right heart volume overload and allow remodeling of the heart chambers. Any residual left-to-right shunt after a correction will result in persistent right heart dilatation. Residual flows can result from various mechanisms, including lack of apposition of the covered stent to the free edge of the SVD, fabric breach, and persistent anomalous drainage of additional right-sided pulmonary veins that drain very high in the SVC or can be due to a coexistent defect in the oval fossa. This review analyzes the different mechanisms, explains the transesophageal and angiographic images for each one, and offers solutions tailored for various reasons. Different mechanisms warrant different treatment principles. A solution for residual shunt from one mechanism may not be appropriate for residual flow through another mechanism. A thorough understanding would aid the operator in effective interventions for these SVDs.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 1","pages":"45-51"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11198930/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141454783","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}
Transcatheter sinus venosus defect (SVD) closure with covered stents is emerging as an alternative to surgery. An adequate anchor zone in the superior vena cava is mandatory for the stability of the covered stent to prevent caudal embolization. There is a potential risk of innominate vein occlusion by the fabric of the covered stent in patients with a very short superior caval vein. Three among a total of 105 patients who underwent SVD closure at our institution developed innominate vein occlusion. Predisposing anatomical factors, identification and management of occluded innominate vein, and follow-up outcomes are discussed.
{"title":"Innominate vein occlusion by the fabric of covered stent during transcatheter closure of sinus venosus defects - Causes, management, and outcome.","authors":"Kothandam Sivakumar, Pramod Sagar, Puthiyedath Thejaswi, Rajeshkumar Ramaswamy, Ramyashri Chandrasekaran","doi":"10.4103/apc.apc_186_23","DOIUrl":"10.4103/apc.apc_186_23","url":null,"abstract":"<p><p>Transcatheter sinus venosus defect (SVD) closure with covered stents is emerging as an alternative to surgery. An adequate anchor zone in the superior vena cava is mandatory for the stability of the covered stent to prevent caudal embolization. There is a potential risk of innominate vein occlusion by the fabric of the covered stent in patients with a very short superior caval vein. Three among a total of 105 patients who underwent SVD closure at our institution developed innominate vein occlusion. Predisposing anatomical factors, identification and management of occluded innominate vein, and follow-up outcomes are discussed.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 1","pages":"59-63"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11198928/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141454785","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}
Pub Date : 2024-01-01Epub Date: 2024-05-24DOI: 10.4103/apc.apc_170_23
Shamsa Al Kaabi, Madan Mohan Maddali, Salim Nasser Al Maskari, Halima Adam Al-Hashm, Abdullah Mohammed Al Farqani, Robert Henry Anderson, Justin T Tretter
We describe our findings in a child with a vein of Galen malformation, in whom the right superior caval and the azygos veins drained into the roof of the morphologically left atrium. A persistent left superior caval vein drained into the morphologically right atrium through the coronary sinus. The additional presence of dual brachiocephalic veins permitted the deployment of a multifunctional ventricular septal defect occluder device to occlude the right superior caval vein, correcting the right-to-left shunt. This also prevented azygos venous drainage into the left atrium.
{"title":"Systemic venous anomalies in a child with a vein of Galen.","authors":"Shamsa Al Kaabi, Madan Mohan Maddali, Salim Nasser Al Maskari, Halima Adam Al-Hashm, Abdullah Mohammed Al Farqani, Robert Henry Anderson, Justin T Tretter","doi":"10.4103/apc.apc_170_23","DOIUrl":"10.4103/apc.apc_170_23","url":null,"abstract":"<p><p>We describe our findings in a child with a vein of Galen malformation, in whom the right superior caval and the azygos veins drained into the roof of the morphologically left atrium. A persistent left superior caval vein drained into the morphologically right atrium through the coronary sinus. The additional presence of dual brachiocephalic veins permitted the deployment of a multifunctional ventricular septal defect occluder device to occlude the right superior caval vein, correcting the right-to-left shunt. This also prevented azygos venous drainage into the left atrium.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 1","pages":"77-80"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11198935/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141454793","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}
Pub Date : 2024-01-01Epub Date: 2024-05-24DOI: 10.4103/apc.apc_191_23
Samuel J H Parsons, Harry Nuttall, Alexander Jones
A right aortic arch with an isolated left innominate artery from the pulmonary artery is an exceedingly rare congenital cardiac malformation. We describe the management and complex surgical timing considerations in two such cases, successfully operated on day 4 and 7 months of age, including the use of cranial ultrasound as a helpful tool to guide decision-making. We also describe the first reported association of this defect with a 4q25 deletion encompassing the LEF1 gene.
{"title":"Isolation of the left innominate artery: When to operate?","authors":"Samuel J H Parsons, Harry Nuttall, Alexander Jones","doi":"10.4103/apc.apc_191_23","DOIUrl":"10.4103/apc.apc_191_23","url":null,"abstract":"<p><p>A right aortic arch with an isolated left innominate artery from the pulmonary artery is an exceedingly rare congenital cardiac malformation. We describe the management and complex surgical timing considerations in two such cases, successfully operated on day 4 and 7 months of age, including the use of cranial ultrasound as a helpful tool to guide decision-making. We also describe the first reported association of this defect with a 4q25 deletion encompassing the <i>LEF1</i> gene.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"17 1","pages":"74-76"},"PeriodicalIF":0.9,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11198939/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141454786","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}