Pub Date : 2023-07-01Epub Date: 2023-06-06DOI: 10.1177/21501351231174824
Alwaleed Al-Dairy, Bairak Salameh, Hadeel Badran, Hasan Hasan, Lujain Alkyakhii
The Konno-Rastan operation is performed for relief of complex left ventricular outflow obstruction with a small aortic annulus. When associated with situs inversus and dextrocardia, important aspects should be kept in mind due to the mirror-image anatomy. In this report, we present a case of a 10-year-old child with the diagnosis of recurrent diffuse subaortic stenosis and situs inversus and dextrocardia who underwent the Konno-Rastan operation successfully and was asymptomatic with normal physical activity after a follow-up period of one year.
{"title":"Konno-Rastan Operation in a Patient With Dextrocardia and Situs Inversus.","authors":"Alwaleed Al-Dairy, Bairak Salameh, Hadeel Badran, Hasan Hasan, Lujain Alkyakhii","doi":"10.1177/21501351231174824","DOIUrl":"10.1177/21501351231174824","url":null,"abstract":"<p><p>The Konno-Rastan operation is performed for relief of complex left ventricular outflow obstruction with a small aortic annulus. When associated with situs inversus and dextrocardia, important aspects should be kept in mind due to the mirror-image anatomy. In this report, we present a case of a 10-year-old child with the diagnosis of recurrent diffuse subaortic stenosis and situs inversus and dextrocardia who underwent the Konno-Rastan operation successfully and was asymptomatic with normal physical activity after a follow-up period of one year.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9796036","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background: To determine the prevalence and pattern of congenital heart disease (CHD) among elementary school children in Dongguan, China.
Methods: Between November 2011 and November 2012, 540,574 school children from 449 elementary schools were screened for CHD by trained doctors in Dongguan. The school children who were suspected to have CHD were referred to a pediatric cardiologist and/or an echocardiographer for a complete evaluation.
Results: The total prevalence of CHD was 2.14 per 1,000 school children (1,157/540,574). The most common form of CHD was isolated ventricular septal defect (37.77%; 437/1,157), followed by isolated atrial septal defect (20.22%; 234/1,157) and patent ductus arteriosus (9.94%; 115/1,157). With respect to sex, CHD was equally distributed between males and females.
Conclusion: Our data show that the prevalence of CHD in Dongguan is not as high as expected and that isolated ventricular septal defect is the predominant pathology. Echocardiography plays a crucial role in the diagnosis of CHD.
{"title":"Prevalence and Pattern of Congenital Heart Diseases in School Children in Dongguan, China.","authors":"GuanYang Kang, JianMin Xiao, Jieying Wang, HuiQing Zhang","doi":"10.1177/21501351231163133","DOIUrl":"10.1177/21501351231163133","url":null,"abstract":"<p><strong>Background: </strong>To determine the prevalence and pattern of congenital heart disease (CHD) among elementary school children in Dongguan, China.</p><p><strong>Methods: </strong>Between November 2011 and November 2012, 540,574 school children from 449 elementary schools were screened for CHD by trained doctors in Dongguan. The school children who were suspected to have CHD were referred to a pediatric cardiologist and/or an echocardiographer for a complete evaluation.</p><p><strong>Results: </strong>The total prevalence of CHD was 2.14 per 1,000 school children (1,157/540,574). The most common form of CHD was isolated ventricular septal defect (37.77%; 437/1,157), followed by isolated atrial septal defect (20.22%; 234/1,157) and patent ductus arteriosus (9.94%; 115/1,157). With respect to sex, CHD was equally distributed between males and females.</p><p><strong>Conclusion: </strong>Our data show that the prevalence of CHD in Dongguan is not as high as expected and that isolated ventricular septal defect is the predominant pathology. Echocardiography plays a crucial role in the diagnosis of CHD.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9788624","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hemophilia A is the most common severe innate bleeding disorder. It is an X-linked recessive inherited bleeding disorder characterized by a qualitative and/or quantitative deficiency of factor VIII. The clinical manifestation of this disease is hemorrhaging that can affect every organ, in particular joints (hemarthrosis) and muscles (hematoma). Some serious but rare hemorrhages can be life-threatening, in particular hemorrhage of the central nervous system and hemopericardium. We report a rare case of spontaneous hemopericardium complicated by tamponade in a child with moderate hemophilia A treated with Factor VIII replacement infusion and pericardial drainage, with a favorable outcome. To our knowledge, this is the second case described in the literature of spontaneous hemopericardium occurring in a child with hemophilia A. Our case suggests that a dose of 50 IU/kg/8 h of factor VIII maintained for up to one day after removal of the pericardial drain seems to be sufficient to ensure correct hemostasis, though further evidence is needed to confirm this impression.
血友病 A 是最常见的严重先天性出血性疾病。它是一种 X 连锁隐性遗传出血性疾病,其特征是第八因子定性和/或定量缺乏。该病的临床表现为出血,可累及各个器官,尤其是关节(血肿)和肌肉(血肿)。一些严重但罕见的出血可危及生命,尤其是中枢神经系统出血和血心包。我们报告了一例罕见的中度 A 型血友病患儿自发性血心包并发心包填塞的病例,患儿接受了因子 VIII 替代输注和心包引流治疗,结果良好。据我们所知,这是文献中描述的第二例发生在 A 型血友病患儿身上的自发性血心包积血病例。我们的病例表明,在拔除心包引流管后维持 50 IU/kg/8 h 的 VIII 因子剂量长达一天似乎就足以确保正确止血,但这一印象还需要进一步的证据来证实。
{"title":"Spontaneous Hemopericardium Complicated by Tamponade in a Child With Moderate Hemophilia A: Case-Based Review.","authors":"Seyfeddine Zayani, Farah Thabet, Majdi Ben Massoud, Chokri Chouchane, Habib Gamra, Slaheddine Chouchane","doi":"10.1177/21501351231168868","DOIUrl":"10.1177/21501351231168868","url":null,"abstract":"<p><p>Hemophilia A is the most common severe innate bleeding disorder. It is an X-linked recessive inherited bleeding disorder characterized by a qualitative and/or quantitative deficiency of factor VIII. The clinical manifestation of this disease is hemorrhaging that can affect every organ, in particular joints (hemarthrosis) and muscles (hematoma). Some serious but rare hemorrhages can be life-threatening, in particular hemorrhage of the central nervous system and hemopericardium. We report a rare case of spontaneous hemopericardium complicated by tamponade in a child with moderate hemophilia A treated with Factor VIII replacement infusion and pericardial drainage, with a favorable outcome. To our knowledge, this is the second case described in the literature of spontaneous hemopericardium occurring in a child with hemophilia A. Our case suggests that a dose of 50 IU/kg/8 h of factor VIII maintained for up to one day after removal of the pericardial drain seems to be sufficient to ensure correct hemostasis, though further evidence is needed to confirm this impression.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9791572","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-07-01Epub Date: 2023-04-02DOI: 10.1177/21501351231162894
Reena Khantwal Joshi, Neeraj Aggarwal, Mridul Agarwal, Siddartha C Rudrappa, Raja Joshi
Early and long-term outcomes in patients with pulmonary atresia-intact ventricular septum undergoing staged univentricular palliations have been known to be adversely affected by the presence of right ventricle-dependent coronary circulation. We describe a surgical technique to circumvent the coronary insufficiency caused by acute decompression of the right heart.
{"title":"Perfusion Strategy to Prevent Right Ventricular Decompression on Cardiopulmonary Bypass During Extracardiac Fontan for Right Ventricle-Dependent Coronary Circulation.","authors":"Reena Khantwal Joshi, Neeraj Aggarwal, Mridul Agarwal, Siddartha C Rudrappa, Raja Joshi","doi":"10.1177/21501351231162894","DOIUrl":"10.1177/21501351231162894","url":null,"abstract":"<p><p>Early and long-term outcomes in patients with pulmonary atresia-intact ventricular septum undergoing staged univentricular palliations have been known to be adversely affected by the presence of right ventricle-dependent coronary circulation. We describe a surgical technique to circumvent the coronary insufficiency caused by acute decompression of the right heart.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9780463","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-07-01Epub Date: 2023-05-15DOI: 10.1177/21501351231168830
Mark H D Danton, Emeka B Kesieme
Warden procedure is a popular technique for repairing partial anomalous pulmonary venous connection. We describe a modification of this technique for surgical repair of this condition by raising both a superior vena cava (SVC) flap and right atrial appendage flap to create a tension-free SVC-RA continuity (neo-SVC). The anomalous pulmonary veins are routed via the remanent of proximal SVC and baffled to the left atrium across a surgically created or enlarged atrial septal defect using autologous pericardium.
{"title":"Modification of the Warden Procedure for Surgical Repair of Partial Anomalous Pulmonary Venous Connection.","authors":"Mark H D Danton, Emeka B Kesieme","doi":"10.1177/21501351231168830","DOIUrl":"10.1177/21501351231168830","url":null,"abstract":"<p><p>Warden procedure is a popular technique for repairing partial anomalous pulmonary venous connection. We describe a modification of this technique for surgical repair of this condition by raising both a superior vena cava (SVC) flap and right atrial appendage flap to create a tension-free SVC-RA continuity (neo-SVC). The anomalous pulmonary veins are routed via the remanent of proximal SVC and baffled to the left atrium across a surgically created or enlarged atrial septal defect using autologous pericardium.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10169310","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-07-01Epub Date: 2023-03-15DOI: 10.1177/21501351231158539
Madan M Maddali, Hamood N Al Kindi, Pranav Subbaraya Kandachar, Abdulla Al Farqani, Khalid S Al Alawi, Faiza Al Kindi, Salim Nasser Al-Maskari, Diane E Spicer, Robert Henry Anderson
So as to produce totally anomalous systemic venous connection, all of the systemic venous tributaries, along with the coronary sinus, should be connected with the morphologically left atrium. Previous descriptions of this rare constellation of anomalous connections of the systemic venous tributaries of the heart have been compromised by the inclusion of individuals having isomeric atrial appendages. In these settings, most frequently, the totally, or almost totally, anomalous systemic venous connections are associated with a sinus venosus defect. It is the anomalous pulmonary venous connections that then create a venovenous bridge, which permits the systemic venous tributaries to drain into the morphologically left atrium, even though they may be predominantly connected to the right atrium. More rarely, it is feasible for the primary atrial septum to develop so as to leave the systemic venous sinus in direct connection with the body of the morphologically left, rather than the morphologically right, atrium. We report a series of patients potentially falling into the category of anomalous systemic venous connections. The findings show a spectrum from partially to totally anomalous connections, with some better interpreted on the basis of anomalous drainage. Included in our cases, nonetheless, is an autopsied example of totally anomalous systemic venous connection produced by an abnormal location of the primary atrial septum. We discuss the potential morphogenesis for this finding. We emphasize the distinction that needs to be made between anomalous systemic venous connections and anomalous systemic venous drainage.
{"title":"Identifying Anomalies of Systemic Venous Drainage: Systemic Venous Anomalies; Atrial Morphology.","authors":"Madan M Maddali, Hamood N Al Kindi, Pranav Subbaraya Kandachar, Abdulla Al Farqani, Khalid S Al Alawi, Faiza Al Kindi, Salim Nasser Al-Maskari, Diane E Spicer, Robert Henry Anderson","doi":"10.1177/21501351231158539","DOIUrl":"10.1177/21501351231158539","url":null,"abstract":"<p><p>So as to produce totally anomalous systemic venous connection, all of the systemic venous tributaries, along with the coronary sinus, should be connected with the morphologically left atrium. Previous descriptions of this rare constellation of anomalous connections of the systemic venous tributaries of the heart have been compromised by the inclusion of individuals having isomeric atrial appendages. In these settings, most frequently, the totally, or almost totally, anomalous systemic venous connections are associated with a sinus venosus defect. It is the anomalous pulmonary venous connections that then create a venovenous bridge, which permits the systemic venous tributaries to drain into the morphologically left atrium, even though they may be predominantly connected to the right atrium. More rarely, it is feasible for the primary atrial septum to develop so as to leave the systemic venous sinus in direct connection with the body of the morphologically left, rather than the morphologically right, atrium. We report a series of patients potentially falling into the category of anomalous systemic venous connections. The findings show a spectrum from partially to totally anomalous connections, with some better interpreted on the basis of anomalous drainage. Included in our cases, nonetheless, is an autopsied example of totally anomalous systemic venous connection produced by an abnormal location of the primary atrial septum. We discuss the potential morphogenesis for this finding. We emphasize the distinction that needs to be made between anomalous systemic venous connections and anomalous systemic venous drainage.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10164695","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-07-01Epub Date: 2023-03-27DOI: 10.1177/21501351231162958
Sarosh P Batlivala, Amr Matoq, Shabana Shahanavaz
Microvascular pulmonary arteriovenous malformations (pAVMs) can lead to profound hypoxemia. "Hepatic factor" is postulated to play a role in their development. Certain patients with congenital heart disease are at particular risk to develop pAVMs, including those with heterotaxy syndromes and complex Fontan palliation. Ideally, an underlying cause is identified and corrected, although pAVMs may persist despite those interventions. We report a patient with heterotaxy syndrome s/p Fontan who had pAVMs that persisted despite Fontan revision with equal hepatic flow to both lungs. We employed a novel method to produce a diabolo configuration of a large covered stent to restrict lung flow while maintaining the potential for future dilation.
{"title":"Novel Diabolo Configuration of a Large Covered Stent to Treat Cyanosis Related to Pulmonary Arteriovenous Malformations.","authors":"Sarosh P Batlivala, Amr Matoq, Shabana Shahanavaz","doi":"10.1177/21501351231162958","DOIUrl":"10.1177/21501351231162958","url":null,"abstract":"<p><p>Microvascular pulmonary arteriovenous malformations (pAVMs) can lead to profound hypoxemia. \"Hepatic factor\" is postulated to play a role in their development. Certain patients with congenital heart disease are at particular risk to develop pAVMs, including those with heterotaxy syndromes and complex Fontan palliation. Ideally, an underlying cause is identified and corrected, although pAVMs may persist despite those interventions. We report a patient with heterotaxy syndrome s/p Fontan who had pAVMs that persisted despite Fontan revision with equal hepatic flow to both lungs. We employed a novel method to produce a diabolo configuration of a large covered stent to restrict lung flow while maintaining the potential for future dilation.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9792317","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
We present a five-year-old female diagnosed with Larsen syndrome at birth with severe aortic root dilatation, failure to thrive, and developmental delay. This report highlights the specific physical findings, imaging, genetics, and surgical treatment, along with a brief overview of Larsen syndrome.
{"title":"Severe Aortic Root Dilatation in a Patient With Larsen Syndrome.","authors":"Ruth Eletta, Abinash Pandey, Tarathya Dharmasaputra, Marcial Moreno, Yonna Oparaugo, Tk Susheel Kumar, Susan Beker","doi":"10.1177/21501351231155868","DOIUrl":"10.1177/21501351231155868","url":null,"abstract":"<p><p>We present a five-year-old female diagnosed with Larsen syndrome at birth with severe aortic root dilatation, failure to thrive, and developmental delay. This report highlights the specific physical findings, imaging, genetics, and surgical treatment, along with a brief overview of Larsen syndrome.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9795378","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-07-01DOI: 10.1177/21501351231174826
Susan Nicholson
Young children requiring bypass often develop coagulopathy resulting in major postoperative blood loss. Increased post-bypass bleeding and donor exposures are independently associated with adverse outcomes. When transfusion of hemostatic blood products fails to reduce bleeding to an acceptable level, rescue therapies including prothrombin complex concentrates (PCCs), and/or recombinant activated factor VII are being given "off-label" with increasing frequency. A number of studies attempting to determine the safety and efficacy of PCCs in neonates and young children are being published. These studies are most commonly retrospective, observational, performed in a single center with varying doses, indications for, and timing of administration in a small number of patients with varying results. The results of these individual studies are questionable and are not to be generalized to other center's patients. Because factor VIII inhibitor bypassing activity (FEIBA) contains the activated form of factor VII and factor X there are concerns regarding the potential for thrombotic events in a population with a known risk of postoperative thromboembolism. Currently, there is no validated assay with which to measure the efficacy of FEIBA in vivo to determine dose titration. Well-designed multicenter randomized control trials are needed to determine the optimal dose and risk-benefit of PCCs after pediatric cardiac surgery. Until such data are available the decision to give a procoagulant to neonates and young children after bypass needs to be made when the consequences of blood loss and replacement pose more risk than the risk of thrombotic complications from the drug.
需要搭桥的幼儿往往会出现凝血障碍,导致术后大量失血。搭桥术后出血量和供体暴露量的增加与不良预后密切相关。当输注止血血制品无法将出血量降低到可接受的水平时,包括凝血酶原复合物浓缩物(PCC)和/或重组活化因子 VII 在内的抢救性疗法越来越多地在 "标签外 "使用。目前已发表了大量研究报告,试图确定凝血酶原复合物浓缩物对新生儿和幼儿的安全性和有效性。这些研究多为回顾性、观察性研究,在单一中心进行,对少数患者的用药剂量、适应症和用药时间各不相同,结果也不尽相同。这些个别研究的结果值得商榷,不能推广到其他中心的患者身上。由于因子 VIII 抑制剂旁路活性(FEIBA)含有活化形式的因子 VII 和因子 X,因此人们担心在已知有术后血栓栓塞风险的人群中发生血栓事件的可能性。目前,还没有有效的检测方法来测量 FEIBA 的体内疗效,以确定剂量滴定。需要进行精心设计的多中心随机对照试验,以确定小儿心脏手术后 PCCs 的最佳剂量和风险效益。在获得此类数据之前,需要在失血和换血的后果比药物引起血栓并发症的风险更大的情况下,决定是否在搭桥术后给新生儿和幼儿使用促凝血剂。
{"title":"Invited Commentary: Achieving Post-Bypass Hemostasis in Young Children: At What Cost?","authors":"Susan Nicholson","doi":"10.1177/21501351231174826","DOIUrl":"10.1177/21501351231174826","url":null,"abstract":"<p><p>Young children requiring bypass often develop coagulopathy resulting in major postoperative blood loss. Increased post-bypass bleeding and donor exposures are independently associated with adverse outcomes. When transfusion of hemostatic blood products fails to reduce bleeding to an acceptable level, rescue therapies including prothrombin complex concentrates (PCCs), and/or recombinant activated factor VII are being given \"off-label\" with increasing frequency. A number of studies attempting to determine the safety and efficacy of PCCs in neonates and young children are being published. These studies are most commonly retrospective, observational, performed in a single center with varying doses, indications for, and timing of administration in a small number of patients with varying results. The results of these individual studies are questionable and are not to be generalized to other center's patients. Because factor VIII inhibitor bypassing activity (FEIBA) contains the activated form of factor VII and factor X there are concerns regarding the potential for thrombotic events in a population with a known risk of postoperative thromboembolism. Currently, there is no validated assay with which to measure the efficacy of FEIBA in vivo to determine dose titration. Well-designed multicenter randomized control trials are needed to determine the optimal dose and risk-benefit of PCCs after pediatric cardiac surgery. Until such data are available the decision to give a procoagulant to neonates and young children after bypass needs to be made when the consequences of blood loss and replacement pose more risk than the risk of thrombotic complications from the drug.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9795383","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-05-01DOI: 10.1177/21501351221151050
Kirsten Graff, Anthony M Hlavacek, Nicholas Aizcorbe, Scott M Bradley, Shahryar M Chowdhury
Background: Neonates with ductal-dependent pulmonary blood flow (DD-PBF) are at risk for pulmonary artery (PA) stenosis. The objective of this study was to identify preoperative cardiovascular computed tomography angiography (CTA) measures that are associated with the need for branch PA intervention.
Methods: We identified neonates with DD-PBF who underwent preoperative CTA at our center and were followed for 24 months. The primary outcome was requiring intervention for branch PA stenosis at the initial or subsequent procedure. Patients were divided into three groups: 1) No PA intervention, 2) Initial PA intervention, and 3) Remote PA intervention. Measurements of the branch PAs and patent ductus arteriosus (PDA) were made prospectively.
Results: Forty patients were included, 7 (18%) did not receive a PA intervention, 23 (58%) were in the initial PA intervention group, and 10 (25%) were in the remote PA intervention group. The distance from PA bifurcation to the largest diameter of the PA that receives the PDA showed a difference between the no-intervention group versus the initial and remote intervention groups (0.8 mm [IQR 0.7, 2.0], 8.2 mm [IQR 1.9, 13.7], 8.5 mm [IQR 6.5, 11.1], respectively, P = .02). The receiver operating characteristic curve showed a distance >2.2 mm had a sensitivity = 91% and specificity = 86% in predicting the need for PA intervention.
Conclusion: The distance from the PA bifurcation to the largest diameter of the branch PA that accepts the PDA on preoperative CTA is highly predictive of the need for initial or remote PA intervention in this group. Preoperative CTA should be considered for risk stratification in neonates undergoing intervention for DD-PBF.
背景:导管依赖性肺血流(DD-PBF)的新生儿有肺动脉(PA)狭窄的危险。本研究的目的是确定术前心血管计算机断层血管造影(CTA)措施与需要分支PA干预相关。方法:我们确定了在我中心术前接受CTA的DD-PBF新生儿,并随访了24个月。主要结果是在初始或后续手术中需要对支动脉狭窄进行干预。患者分为3组:1)无PA干预,2)初始PA干预,3)远程PA干预。前瞻性测量动脉导管未闭分支PAs和动脉导管未闭(PDA)。结果:纳入40例患者,7例(18%)未接受PA干预,23例(58%)为初始PA干预组,10例(25%)为远程PA干预组。无干预组与初始干预组和远程干预组相比,前列腺癌分叉至接受前列腺癌最大直径的距离分别为0.8 mm [IQR 0.7, 2.0]、8.2 mm [IQR 1.9, 13.7]、8.5 mm [IQR 6.5, 11.1], P = 0.02)。受试者工作特征曲线显示,距离>2.2 mm预测PA干预的敏感性为91%,特异性为86%。结论:在本组患者中,术前CTA显示的PA分叉到接受PDA的PA分支最大直径的距离可高度预测是否需要进行初始或远程PA干预。对于接受DD-PBF干预的新生儿,术前CTA应考虑风险分层。
{"title":"Preoperative Computed Tomographic Angiography Can Predict Need for Branch Pulmonary Artery Intervention in Patients With Ductal-Dependent Pulmonary Blood Flow.","authors":"Kirsten Graff, Anthony M Hlavacek, Nicholas Aizcorbe, Scott M Bradley, Shahryar M Chowdhury","doi":"10.1177/21501351221151050","DOIUrl":"https://doi.org/10.1177/21501351221151050","url":null,"abstract":"<p><strong>Background: </strong>Neonates with ductal-dependent pulmonary blood flow (DD-PBF) are at risk for pulmonary artery (PA) stenosis. The objective of this study was to identify preoperative cardiovascular computed tomography angiography (CTA) measures that are associated with the need for branch PA intervention.</p><p><strong>Methods: </strong>We identified neonates with DD-PBF who underwent preoperative CTA at our center and were followed for 24 months. The primary outcome was requiring intervention for branch PA stenosis at the initial or subsequent procedure. Patients were divided into three groups: 1) No PA intervention, 2) Initial PA intervention, and 3) Remote PA intervention. Measurements of the branch PAs and patent ductus arteriosus (PDA) were made prospectively.</p><p><strong>Results: </strong>Forty patients were included, 7 (18%) did not receive a PA intervention, 23 (58%) were in the initial PA intervention group, and 10 (25%) were in the remote PA intervention group. The distance from PA bifurcation to the largest diameter of the PA that receives the PDA showed a difference between the no-intervention group versus the initial and remote intervention groups (0.8 mm [IQR 0.7, 2.0], 8.2 mm [IQR 1.9, 13.7], 8.5 mm [IQR 6.5, 11.1], respectively, <i>P</i> = .02). The receiver operating characteristic curve showed a distance >2.2 mm had a sensitivity = 91% and specificity = 86% in predicting the need for PA intervention.</p><p><strong>Conclusion: </strong>The distance from the PA bifurcation to the largest diameter of the branch PA that accepts the PDA on preoperative CTA is highly predictive of the need for initial or remote PA intervention in this group. Preoperative CTA should be considered for risk stratification in neonates undergoing intervention for DD-PBF.</p>","PeriodicalId":23974,"journal":{"name":"World Journal for Pediatric and Congenital Heart Surgery","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9414153","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}