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CJC Pediatric and Congenital Heart Disease

Pub Date : 2024-12-01 DOI: 10.1016/S2772-8129(24)00116-7

引用次数: 0

Retrograde Flow to Aortic Root Predicts Inferior Cardiopulmonary Performance and Restrictive Lung Physiology in Fontan Circulation

CJC Pediatric and Congenital Heart Disease

Pub Date : 2024-12-01 DOI: 10.1016/j.cjcpc.2024.08.002

Henri Juhani Pyykkönen MSc , Otto Rahkonen MD, PhD , Heikki Tikkanen MD, PhD , Karim Khanji MSc , Päivi Piirilä MD, PhD , Olli Pitkänen-Argillander MD, PhD

Background

Cardiac output in Fontan circulation depends on systemic venous pressure, pulmonary vascular resistance, and ventricular function. Because myocardial function is dependent on coronary perfusion, we studied whether retrograde flow to aortic root in the mitral/aortic atresia subgroup of hypoplastic left heart syndrome (HLHS) affects cardiopulmonary performance.

Methods

We studied 26 stable Fontan patients (14.4 ± 2.4 years) with right (RV, n = 17) and left (LV, n = 9) systemic ventricle morphology. All RV patients had HLHS and were subdivided according to postnatal flow to the hypoplastic ascending aorta being antegrade (HLHS-A) or retrograde (HLHS-R) due to valve atresia. Physical activity was assessed by questionnaire (LASERI, a questionnaire for Finnish children regarding physical activity), cardiopulmonary exercise test (1-minute ramp protocol), body composition (Biacorpus RX 4000), and muscle fitness (EUROFIT). These data were correlated with the postnatal aorta size and current branch pulmonary artery size index (McGoon index).

Results

Patients with HLHS-R seldom self-reported engagement in vigorous physical activity and had significantly lower cardiopulmonary performance (peak oxygen uptake [VO₂peak]) than patients with LV morphology (P = 0.037), but not compared with patients with HLHS-A. Branch pulmonary artery size did not correlate with VO₂peak. Patients with HLHS-R had most severe lung restrictions (forced vital capacity z-score –3 ± 0.9, P = 0.0073; forced expiratory volume in 1 second z-score –3.3 ± 1.1, P = 0.001).

Conclusions

Young Fontan patients with LV had better cardiopulmonary performance than patients with HLHS. Patients with HLHS-R were the least active and had the lowest VO₂peak and most restrictive lungs. It is important to recognize postnatally single ventricle patients at high risk for inactivity to promote an active and healthy lifestyle.

{"title":"Retrograde Flow to Aortic Root Predicts Inferior Cardiopulmonary Performance and Restrictive Lung Physiology in Fontan Circulation","authors":"Henri Juhani Pyykkönen MSc , Otto Rahkonen MD, PhD , Heikki Tikkanen MD, PhD , Karim Khanji MSc , Päivi Piirilä MD, PhD , Olli Pitkänen-Argillander MD, PhD","doi":"10.1016/j.cjcpc.2024.08.002","DOIUrl":"10.1016/j.cjcpc.2024.08.002","url":null,"abstract":"<div><h3>Background</h3><div>Cardiac output in Fontan circulation depends on systemic venous pressure, pulmonary vascular resistance, and ventricular function. Because myocardial function is dependent on coronary perfusion, we studied whether retrograde flow to aortic root in the mitral/aortic atresia subgroup of hypoplastic left heart syndrome (HLHS) affects cardiopulmonary performance.</div></div><div><h3>Methods</h3><div>We studied 26 stable Fontan patients (14.4 ± 2.4 years) with right (RV, n = 17) and left (LV, n = 9) systemic ventricle morphology. All RV patients had HLHS and were subdivided according to postnatal flow to the hypoplastic ascending aorta being antegrade (HLHS-A) or retrograde (HLHS-R) due to valve atresia. Physical activity was assessed by questionnaire (LASERI, a questionnaire for Finnish children regarding physical activity), cardiopulmonary exercise test (1-minute ramp protocol), body composition (Biacorpus RX 4000), and muscle fitness (EUROFIT). These data were correlated with the postnatal aorta size and current branch pulmonary artery size index (McGoon index).</div></div><div><h3>Results</h3><div>Patients with HLHS-R seldom self-reported engagement in vigorous physical activity and had significantly lower cardiopulmonary performance (peak oxygen uptake [VO<sub>2</sub>peak]) than patients with LV morphology (<em>P</em> = 0.037), but not compared with patients with HLHS-A. Branch pulmonary artery size did not correlate with VO<sub>2</sub>peak. Patients with HLHS-R had most severe lung restrictions (forced vital capacity <em>z</em>-score –3 ± 0.9, <em>P</em> = 0.0073; forced expiratory volume in 1 second <em>z</em>-score –3.3 ± 1.1, <em>P</em> = 0.001).</div></div><div><h3>Conclusions</h3><div>Young Fontan patients with LV had better cardiopulmonary performance than patients with HLHS. Patients with HLHS-R were the least active and had the lowest VO<sub>2</sub>peak and most restrictive lungs. It is important to recognize postnatally single ventricle patients at high risk for inactivity to promote an active and healthy lifestyle.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"3 6","pages":"Pages 265-271"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143176085","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}

引用次数: 0

Fourteenth International Kawasaki Disease Symposium: Learning From the Past, Looking to the Future

CJC Pediatric and Congenital Heart Disease

Pub Date : 2024-12-01 DOI: 10.1016/j.cjcpc.2024.10.002

Matthew D. Elias MD , Federica Anselmi MD , Luisa B. Gámez-González MD , Fujito Numano MD, PhD , Rakesh Kumar Pilania MD, DM , Alan P. Wang MD , Nagib Dahdah MD, MBA , Adriana H. Tremoulet MD, MAS , Audrey Dionne MD

引用次数: 0

Incidence, Correlates, and Prognostic Implications of New-Onset Atrial Fibrillation in Adults With Repaired Coarctation of Aorta

CJC Pediatric and Congenital Heart Disease

Pub Date : 2024-12-01 DOI: 10.1016/j.cjcpc.2024.07.004

Alexander C. Egbe MD, MPH, MS, Malini Madhavan MBBS, Heidi M. Connolly MD, Ahmed E. Ali MD, Ahmed Younis MD, Abhishek Deshmukh MBBS

Background

There are limited data about the incidence and outcomes of atrial fibrillation (AF) in adults with coarctation of aorta (COA). The purpose of this study was to determine the incidence, correlates, and prognostic implications of new-onset AF in adults with repaired COA.

Methods

A retrospective cohort study of adults with repaired COA without a prior history of atrial arrhythmias was performed. We reviewed rhythm data (electrocardiogram, Holter, and rhythm strip) obtained from baseline to the last clinical encounter. The correlates of AF and the relationship between AF and cardiovascular adverse events (heart failure hospitalization and/or all-cause mortality) were assessed using Cox regression.

Results

Of 782 patients (aged 32 [interquartile range: 21-43] years; 462 [59%] men), 42 (5.4%) developed new-onset AF. The incidence of new-onset AF was 9 per 1000 patient-years (0.9% per year), and the median age at onset of AF was 36 (interquartile range: 24-49) years. The correlates of new-onset AF were older age, hypertension, left atrial dysfunction, and left ventricular hypertrophy. Of 782 patients, 92 (12%) had cardiovascular adverse events. On multivariable analysis, new-onset AF was associated with cardiovascular adverse events (hazard ratio: 1.09, 95% confidence interval: 1.03-1.15), after adjustment for age, hypertension, and right and left ventricular structure and function.

Conclusions

Patients with COA were at risk for developing AF at a relatively young age (median age: 36 years), and AF was associated with cardiovascular adverse outcomes. There is a need to target the modifiable risk factors for AF to reduce the adverse outcomes associated with AF.

{"title":"Incidence, Correlates, and Prognostic Implications of New-Onset Atrial Fibrillation in Adults With Repaired Coarctation of Aorta","authors":"Alexander C. Egbe MD, MPH, MS, Malini Madhavan MBBS, Heidi M. Connolly MD, Ahmed E. Ali MD, Ahmed Younis MD, Abhishek Deshmukh MBBS","doi":"10.1016/j.cjcpc.2024.07.004","DOIUrl":"10.1016/j.cjcpc.2024.07.004","url":null,"abstract":"<div><h3>Background</h3><div>There are limited data about the incidence and outcomes of atrial fibrillation (AF) in adults with coarctation of aorta (COA). The purpose of this study was to determine the incidence, correlates, and prognostic implications of new-onset AF in adults with repaired COA.</div></div><div><h3>Methods</h3><div>A retrospective cohort study of adults with repaired COA without a prior history of atrial arrhythmias was performed. We reviewed rhythm data (electrocardiogram, Holter, and rhythm strip) obtained from baseline to the last clinical encounter. The correlates of AF and the relationship between AF and cardiovascular adverse events (heart failure hospitalization and/or all-cause mortality) were assessed using Cox regression.</div></div><div><h3>Results</h3><div>Of 782 patients (aged 32 [interquartile range: 21-43] years; 462 [59%] men), 42 (5.4%) developed new-onset AF. The incidence of new-onset AF was 9 per 1000 patient-years (0.9% per year), and the median age at onset of AF was 36 (interquartile range: 24-49) years. The correlates of new-onset AF were older age, hypertension, left atrial dysfunction, and left ventricular hypertrophy. Of 782 patients, 92 (12%) had cardiovascular adverse events. On multivariable analysis, new-onset AF was associated with cardiovascular adverse events (hazard ratio: 1.09, 95% confidence interval: 1.03-1.15), after adjustment for age, hypertension, and right and left ventricular structure and function.</div></div><div><h3>Conclusions</h3><div>Patients with COA were at risk for developing AF at a relatively young age (median age: 36 years), and AF was associated with cardiovascular adverse outcomes. There is a need to target the modifiable risk factors for AF to reduce the adverse outcomes associated with AF.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"3 6","pages":"Pages 247-252"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143176079","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}

引用次数: 0

Gender Distribution in Paediatric Cardiology Training Programs in Canada

CJC Pediatric and Congenital Heart Disease

Pub Date : 2024-12-01 DOI: 10.1016/j.cjcpc.2024.09.002

Michael N. Gritti MD , Megan Werger MD (c) , Alison J. Howell MD , Conall T. Morgan MD

Background

Despite female medical students being the majority, there are certain medical specialities that continue to have a prevalence of male trainees, such as adult cardiology. The purpose of this study is to examine gender distribution within Canadian paediatric cardiology training programs.

Methods

Both application and successful matches to core paediatric residency programs and paediatric cardiology programs were obtained through the Canadian Resident Matching Service. Analysis was performed to determine if the association between gender of paediatric residents and paediatric cardiology applicants/fellows was significant.

Results

Between 2016 and 2024, 12% (n = 3/26) of individuals who successfully matched into a Canadian paediatric cardiology training program identified as female and 88% (n = 23/26) identified as male. Between 2013 and 2023, 78% (n = 947/1220) of individuals who successfully matched into a Canadian paediatrics residency identified as female and 22% (n = 273/1220) identified as male. There was a statistically significant difference between the gender of paediatric residents and the gender of paediatric cardiology residents (P < 0.0001). There was no significant association between applicant gender and match outcome within paediatric cardiology training programs (P < 0.23).

Conclusions

There is a gender discrepancy within Canadian paediatric cardiology training programs with a predominance of male trainees in the recent era. It appears that female under-representation in the field is due to the low number of female applicants. Although limited by sample size, there was no clear association between applicant gender and success of admission to a paediatric cardiology training program.

{"title":"Gender Distribution in Paediatric Cardiology Training Programs in Canada","authors":"Michael N. Gritti MD , Megan Werger MD (c) , Alison J. Howell MD , Conall T. Morgan MD","doi":"10.1016/j.cjcpc.2024.09.002","DOIUrl":"10.1016/j.cjcpc.2024.09.002","url":null,"abstract":"<div><h3>Background</h3><div>Despite female medical students being the majority, there are certain medical specialities that continue to have a prevalence of male trainees, such as adult cardiology. The purpose of this study is to examine gender distribution within Canadian paediatric cardiology training programs.</div></div><div><h3>Methods</h3><div>Both application and successful matches to core paediatric residency programs and paediatric cardiology programs were obtained through the Canadian Resident Matching Service. Analysis was performed to determine if the association between gender of paediatric residents and paediatric cardiology applicants/fellows was significant.</div></div><div><h3>Results</h3><div>Between 2016 and 2024, 12% (n = 3/26) of individuals who successfully matched into a Canadian paediatric cardiology training program identified as female and 88% (n = 23/26) identified as male. Between 2013 and 2023, 78% (n = 947/1220) of individuals who successfully matched into a Canadian paediatrics residency identified as female and 22% (n = 273/1220) identified as male. There was a statistically significant difference between the gender of paediatric residents and the gender of paediatric cardiology residents (<em>P</em> < 0.0001). There was no significant association between applicant gender and match outcome within paediatric cardiology training programs (<em>P</em> < 0.23).</div></div><div><h3>Conclusions</h3><div>There is a gender discrepancy within Canadian paediatric cardiology training programs with a predominance of male trainees in the recent era. It appears that female under-representation in the field is due to the low number of female applicants. Although limited by sample size, there was no clear association between applicant gender and success of admission to a paediatric cardiology training program.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"3 6","pages":"Pages 241-246"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143176080","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}

引用次数: 0

Tachycardia-Induced Cardiomyopathy: A Case Series and a Literature Review

CJC Pediatric and Congenital Heart Disease

Pub Date : 2024-12-01 DOI: 10.1016/j.cjcpc.2024.10.007

Wisam Abozaid MD , Samantha Wong , Marc W. Deyell MD, FRCPC , Shubhayan Sanatani MD, FRCPC, FHRS , Sakethram Saravu Vijayashankar MD, MRCPCH

Tachycardia-induced cardiomyopathy (TIC), also known as arrhythmia-induced cardiomyopathy or tachycardiomyopathy, is a reversible form of heart failure characterized by persistent tachyarrhythmias and associated ventricular dysfunction. TIC is characterized by the reversal of myocardial damage with resolution of the arrhythmia. Early diagnosis of TIC is imperative, as the treatment course is distinct from cardiomyopathy of other or unknown causes. However, distinguishing TIC from tachycardia secondary to increased catecholamines due to congestive heart failure can be very challenging. There are relatively few paediatric reports, and herein we present a case series of 48 paediatric patients with TIC from literature (2014-2024). We also present 4 illustrative cases with TIC seen at our site (BC Children’s Hospital, Vancouver, Canada). The mean age in this case series was 6.98 ± 4.9 years. The majority of patients had ectopic atrial tachycardia (41.7%), followed by permanent junctional reciprocating tachycardia (20.8%), ventricular tachycardia (16.7%), and atrioventricular re-entrant tachycardia or atrioventricular nodal re-entrant tachycardia (10.4%). Pharmacologic treatment was the predominant therapy, but 70.8% of patients needed at least 1 ablation procedure. All patients demonstrated significant improvement in left ventricular ejection fraction after treatment, with most achieving at least near-normal ejection fractions in 80 days on average since presentation. In conclusion, TIC is overall a treatable condition with challenging diagnosis but generally has a favourable prognosis when diagnosed and treated appropriately. This article emphasizes the importance of considering TIC in the differential diagnosis of tachycardia in the context of reduced ventricular function, to recognize it and to enable targeted treatment initiation as soon as possible.

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引用次数: 0

Beyond the Narrowing: Atrial Fibrillation in Aortic Coarctation

CJC Pediatric and Congenital Heart Disease

Pub Date : 2024-12-01 DOI: 10.1016/j.cjcpc.2024.09.004

Marie-Hélène Gagnon MD , Paul Khairy MD, PhD , Martin Aguilar MD, PhD

引用次数: 0

Isolated Left Subclavian Artery, Multiple Ventricular Septal Defects, and Pulmonary Hypertension in a Child: A Case Report 一名儿童的孤立左锁骨下动脉、多发室间隔缺损和肺动脉高压：病例报告

CJC Pediatric and Congenital Heart Disease

Pub Date : 2024-10-01 DOI: 10.1016/j.cjcpc.2024.07.001

Mohammad Reza Khalilian MD , Manouchehr Hekmat MD , Saeed Sadr MD , Abdolhossein Tavallai-Zavvareh MD , Tahmineh Tahouri MD

引用次数: 0

The Need for Preoperative Prostaglandin E1 for d-Transposition After Balloon Atrial Septostomy 球囊心房隔膜切除术后 d 型移位术前使用前列腺素 E1 的必要性

CJC Pediatric and Congenital Heart Disease

Pub Date : 2024-10-01 DOI: 10.1016/j.cjcpc.2024.08.004

Thita Pacharapakornpong MD , Linda Edwards MD , Steven Rathgeber MD

Background

The necessity of prostaglandin E1 (PGE) usage before arterial switch operation (ASO) in infants with d-transposition of the great arteries (dTGA) after balloon atrial septostomy (BAS) remains controversial.

Methods

This study is a single-centre, retrospective review of infants with dTGA who underwent ASO from January 2014 to December 2021. Parameters analysed included post-BAS oxygen saturation, time from BAS to PGE discontinuation, necessity of reinitiation, interval before PGE restart, and lowest saturation before PGE reintroduction.

Results

Among the 35 cases of dTGA who underwent ASO, 31 (88%) required BAS, with 23 (65%) requiring PGE infusion. Of those 23 infants, 14 (60%) necessitated PGE reinitiation after discontinuation. A significant difference in post-BAS oxygen saturation was observed between the groups requiring PGE reinitiation (79.2% ± 4.7%) and those not needing reinitiation (89.0% ± 2.0%) (P < 0.001). The relative risk for the reinitiation group with BAS oxygen saturation levels ≤80% was 2.5 (95% confidence interval: 1.3-4.6). No disparity was observed in postoperative outcomes or PGE adverse effects such as fever, apnoea, bradycardia, and congestive heart failure requiring diuretic between the groups.

Conclusions

Given no significant differences in PGE adverse effects and a 2.5 times higher risk of reinitiation with post-BAS saturation below 80%, maintaining PGE until saturation reaches 80% for a few days before discontinuation may help reduce the risk of rebound hypoxaemia.

背景对于球囊心房隔成形术（BAS）后大动脉d型横位（dTGA）婴儿在动脉转换手术（ASO）前使用前列腺素E1（PGE）的必要性仍存在争议。分析的参数包括BAS后血氧饱和度、从BAS到PGE停用的时间、重新启用的必要性、PGE重新启用前的间隔时间以及PGE重新启用前的最低血氧饱和度。结果在35例接受ASO的dTGA病例中，31例（88%）需要进行BAS，23例（65%）需要输注PGE。在这 23 名婴儿中，14 名（60%）在停用 PGE 后需要重新使用。在需要重新启动 PGE 的组别（79.2% ± 4.7%）和不需要重新启动的组别（89.0% ± 2.0%）之间，观察到 BAS 后血氧饱和度存在明显差异（P < 0.001）。BAS 血氧饱和度≤80%的重启组相对风险为 2.5（95% 置信区间：1.3-4.6）。结论鉴于 PGE 不良反应无显著差异，且 BAS 后血氧饱和度低于 80% 时重新启动的风险高出 2.5 倍，因此在停用前几天维持 PGE 直到饱和度达到 80%，可能有助于降低反跳性低氧血症的风险。

{"title":"The Need for Preoperative Prostaglandin E1 for d-Transposition After Balloon Atrial Septostomy","authors":"Thita Pacharapakornpong MD , Linda Edwards MD , Steven Rathgeber MD","doi":"10.1016/j.cjcpc.2024.08.004","DOIUrl":"10.1016/j.cjcpc.2024.08.004","url":null,"abstract":"<div><h3>Background</h3><div>The necessity of prostaglandin E1 (PGE) usage before arterial switch operation (ASO) in infants with d-transposition of the great arteries (dTGA) after balloon atrial septostomy (BAS) remains controversial.</div></div><div><h3>Methods</h3><div>This study is a single-centre, retrospective review of infants with dTGA who underwent ASO from January 2014 to December 2021. Parameters analysed included post-BAS oxygen saturation, time from BAS to PGE discontinuation, necessity of reinitiation, interval before PGE restart, and lowest saturation before PGE reintroduction.</div></div><div><h3>Results</h3><div>Among the 35 cases of dTGA who underwent ASO, 31 (88%) required BAS, with 23 (65%) requiring PGE infusion. Of those 23 infants, 14 (60%) necessitated PGE reinitiation after discontinuation. A significant difference in post-BAS oxygen saturation was observed between the groups requiring PGE reinitiation (79.2% ± 4.7%) and those not needing reinitiation (89.0% ± 2.0%) (<em>P</em> < 0.001). The relative risk for the reinitiation group with BAS oxygen saturation levels ≤80% was 2.5 (95% confidence interval: 1.3-4.6). No disparity was observed in postoperative outcomes or PGE adverse effects such as fever, apnoea, bradycardia, and congestive heart failure requiring diuretic between the groups.</div></div><div><h3>Conclusions</h3><div>Given no significant differences in PGE adverse effects and a 2.5 times higher risk of reinitiation with post-BAS saturation below 80%, maintaining PGE until saturation reaches 80% for a few days before discontinuation may help reduce the risk of rebound hypoxaemia.</div></div>","PeriodicalId":100249,"journal":{"name":"CJC Pediatric and Congenital Heart Disease","volume":"3 5","pages":"Pages 214-220"},"PeriodicalIF":0.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142533451","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}

引用次数: 0

Towards Understanding Deterioration in Systemic Right Ventricular Function in the Setting of Tricuspid Regurgitation: Is There a Point of No Return? 了解三尖瓣反流导致的系统性右心室功能恶化：是否存在不归点？

CJC Pediatric and Congenital Heart Disease

Pub Date : 2024-10-01 DOI: 10.1016/j.cjcpc.2024.08.003

William A. McEachern MD, MPH, MSCI, Timothy C. Slesnick MD

引用次数: 0

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