International journal of cardiology. Congenital heart disease最新文献

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Corrigendum to ‘The role of sensitization in post-transplant outcomes in adults with congenital heart disease sensitization in adults with congenital heart disease’, Int. J. Cardiol. Congenit. Heart Dis., 8, June 2022, 100384 《致敏在成人先天性心脏病患者移植后转归中的作用》的更正。j .心功能杂志。Congenit。心脏疾病,2022年6月8日,100384
International journal of cardiology. Congenital heart disease Pub Date : 2023-11-18 DOI: 10.1016/j.ijcchd.2023.100474
Laith Alshawabkeh , Nicole L. Herrick , Alexander R. Opotowsky , Tajinder P. Singh , Michael Landzberg , Marcus A. Urey , Wida Cherikh , Joseph W. Rossano , Michael M. Givertz
{"title":"Corrigendum to ‘The role of sensitization in post-transplant outcomes in adults with congenital heart disease sensitization in adults with congenital heart disease’, Int. J. Cardiol. Congenit. Heart Dis., 8, June 2022, 100384","authors":"Laith Alshawabkeh , Nicole L. Herrick , Alexander R. Opotowsky , Tajinder P. Singh , Michael Landzberg , Marcus A. Urey , Wida Cherikh , Joseph W. Rossano , Michael M. Givertz","doi":"10.1016/j.ijcchd.2023.100474","DOIUrl":"https://doi.org/10.1016/j.ijcchd.2023.100474","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668523000368/pdfft?md5=020420f113a8d01edeed5eef586fb5cc&pid=1-s2.0-S2666668523000368-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136697051","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
Blood biomarkers to detect functional impairment in adult patients with repaired tetralogy of Fallot 血液生物标志物检测成人法洛四联症修复患者的功能损害
International journal of cardiology. Congenital heart disease Pub Date : 2023-11-03 DOI: 10.1016/j.ijcchd.2023.100483
S. Alborikan , A. Althunayyan , B. Pandya , K. Von Klemperer , F. Walker , S. Cullen , A. Bhan , S. Badiani , D. Encarnacion , R. Monteiro , S.E. Petersen , S. Bhattacharyya , G. Lloyd
{"title":"Blood biomarkers to detect functional impairment in adult patients with repaired tetralogy of Fallot","authors":"S. Alborikan ,&nbsp;A. Althunayyan ,&nbsp;B. Pandya ,&nbsp;K. Von Klemperer ,&nbsp;F. Walker ,&nbsp;S. Cullen ,&nbsp;A. Bhan ,&nbsp;S. Badiani ,&nbsp;D. Encarnacion ,&nbsp;R. Monteiro ,&nbsp;S.E. Petersen ,&nbsp;S. Bhattacharyya ,&nbsp;G. Lloyd","doi":"10.1016/j.ijcchd.2023.100483","DOIUrl":"10.1016/j.ijcchd.2023.100483","url":null,"abstract":"<div><h3>Background</h3><p>The relationship between plasma brain natriuretic peptide (NT-proBNP) and soluble suppression of tumorigenicity-2 (sST2) with structural adaptions and exercise capacity remains incompletely described in patients with repaired Tetralogy of Fallot (rTOF).</p></div><div><h3>Methods</h3><p>Peripheral venous blood samples were drawn for 99 patients with repaired TOF, 59 patients with severe pulmonary regurgitation (PR) and 40 patients with no or mild PR. NT-proBNP was measured using enzyme-linked immunosorbent assays (Roche Diagnostics, Indianapolis, IN). Soluble ST2 levels were assessed on Aspect-plus ST2 quantitative rapid test.</p></div><div><h3>Results</h3><p>The mean value of NT-proBNP was 160 ± 137 pg/ml, and sST2 was 29 ± 13, ng/ml in the entire population. 58 % had an elevated NT-proBNP, while sST2 was abnormal in 40 %. Mean NT-proBNP was significantly higher in patients with severe PR (169 ± 150 vs145 ± 118, pg/ml, p &lt; 0.001), while similar sST2 levels were observed in both groups (29 ± 14 vs30 ± 12, ng/ml, p &gt; 0.05). NT-proBNP and sST2 levels were higher in patients with transannular patch when compared to other RVOT intervention (174 ± 145 vs 107 ± 100, pg/ml, p &lt; 0.001); (31 ± 13 vs 29 ± 15, ng/ml, p &lt; 0.05). Both biomarkers were significantly associated with exercise capacity, but NT-proBNP (r = −0.60, p &lt; 0.001) was stronger. The optimal cut-off of 90 pg/ml for NT-proBNP had a sensitivity of 74 % and specificity of 63 % for detection of impaired exercise capacity.</p></div><div><h3>Conclusions</h3><p>Serum levels of sST2 and NT-proBNP are elevated in patients with repaired TOF, with higher values observed in those with severe PR, but also in patients undergoing transannular patch repair. Incorporating both markers in these patients increased the ability to detect impairment in exercise capacity.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668523000459/pdfft?md5=83336cba43d942746cdabdd4f93b9636&pid=1-s2.0-S2666668523000459-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135411337","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
A change of heart: Empowering adults with congenital heart disease for a healthy change 心灵的改变:帮助患有先天性心脏病的成年人做出健康的改变
International journal of cardiology. Congenital heart disease Pub Date : 2023-10-31 DOI: 10.1016/j.ijcchd.2023.100484
Salvatore De Rosa, Margarita Brida, Michael A. Gatzoulis
{"title":"A change of heart: Empowering adults with congenital heart disease for a healthy change","authors":"Salvatore De Rosa,&nbsp;Margarita Brida,&nbsp;Michael A. Gatzoulis","doi":"10.1016/j.ijcchd.2023.100484","DOIUrl":"https://doi.org/10.1016/j.ijcchd.2023.100484","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668523000460/pdfft?md5=55cb9eff97ca9af29f9c6d17d6ffb655&pid=1-s2.0-S2666668523000460-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134657758","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
Late follow-up of neo-aortic dimensions and coronary arteries in adult patients after the arterial switch operation 成人动脉转换手术后新主动脉尺寸及冠状动脉的后期随访
International journal of cardiology. Congenital heart disease Pub Date : 2023-10-14 DOI: 10.1016/j.ijcchd.2023.100481
Diederick B.H. Verheijen , Leo J. Engele , Anastasia D. Egorova , J. Lauran Stöger , Bart J.A. Mertens , Roel L.F. van der Palen , Dave R. Koolbergen , Mark G. Hazekamp , J. Wouter Jukema , Hubert W. Vliegen , Berto J. Bouma , Monique R.M. Jongbloed , Philippine Kiès
{"title":"Late follow-up of neo-aortic dimensions and coronary arteries in adult patients after the arterial switch operation","authors":"Diederick B.H. Verheijen ,&nbsp;Leo J. Engele ,&nbsp;Anastasia D. Egorova ,&nbsp;J. Lauran Stöger ,&nbsp;Bart J.A. Mertens ,&nbsp;Roel L.F. van der Palen ,&nbsp;Dave R. Koolbergen ,&nbsp;Mark G. Hazekamp ,&nbsp;J. Wouter Jukema ,&nbsp;Hubert W. Vliegen ,&nbsp;Berto J. Bouma ,&nbsp;Monique R.M. Jongbloed ,&nbsp;Philippine Kiès","doi":"10.1016/j.ijcchd.2023.100481","DOIUrl":"10.1016/j.ijcchd.2023.100481","url":null,"abstract":"<div><h3>Background</h3><p>After the arterial switch operation (ASO) for transposition of the great arteries (TGA), neo-aortic dilatation and coronary arterial anomalies, especially an interarterial course and acute coronary artery take-off angle, are commonly found. Long-term follow-up data after ASO is scarce. Aim of this study was to determine the prevalence of neo-aortic dilatation and coronary abnormalities, with special emphasis on acute coronary take-off angle, in adult TGA-ASO patients.</p></div><div><h3>Methods</h3><p>In this retrospective cohort study, all adult TGA-ASO patients with ≥1 CT-angiography (CTA) at the age of ≥16 years were included.</p></div><div><h3>Results</h3><p>Eighty-one patients, 69 % male and median age 21.0 [18.5–22.8] years, were included. At baseline, maximum neo-aortic diameter was 39.2 ± 5.3 mm; 35 (43 %) patients had neo-aortic dilatation (neo-aortic diameter of &gt;40 mm), 22 (27 %) patients had an acute coronary take-off angle (&lt;30°), and 5 (6 %) patients had an interarterial course of the RCA (2 %) or LCA (4 %). Neo-aortic or coronary artery re-intervention occurred in 10 (12 %) patients. All 10 patients had neo-aortic dilatation or coronary take-off angle of &lt;30° on baseline CTA.</p></div><div><h3>Conclusion</h3><p>This study reports a prevalence of 43 % of neo-aortic dilatation, 6 % of interarterial coronary course and 27 % for acute coronary take-off angle (&lt;30°) at a median term of 21.0 years post ASO. All patients with a neo-aortic re-intervention or coronary artery re-intervention during follow-up had a maximum neo-aortic diameter of &gt;40 mm or a coronary take-off angle of &lt;30° at baseline CTA. This hypothesis generating study suggests that an active surveillance in patients with neo-aortic dilation and/or an acute angulation of &lt; 30° post ASO might be considered and requires prospective evaluation.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668523000435/pdfft?md5=a2350c2e65af093919fcd981ddcf3b0d&pid=1-s2.0-S2666668523000435-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135762388","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
Screening and prevention in congenital heart disease: Whom, when, and how? 先天性心脏病的筛查和预防:谁,何时,如何?
International journal of cardiology. Congenital heart disease Pub Date : 2023-10-14 DOI: 10.1016/j.ijcchd.2023.100480
Thomas F. Lüscher , John E. Deanfield
{"title":"Screening and prevention in congenital heart disease: Whom, when, and how?","authors":"Thomas F. Lüscher ,&nbsp;John E. Deanfield","doi":"10.1016/j.ijcchd.2023.100480","DOIUrl":"10.1016/j.ijcchd.2023.100480","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668523000423/pdfft?md5=73c9662d89a36bcf216781355d6ff646&pid=1-s2.0-S2666668523000423-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136118612","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: 1
Percutaneous closure of veno-venous collaterals in adult patients with univentricular physiology after Fontan palliation: Single centre experience and systematic review Fontan姑息治疗后单心室生理的成年患者经皮静脉-静脉侧支闭合:单中心经验和系统回顾
International journal of cardiology. Congenital heart disease Pub Date : 2023-10-11 DOI: 10.1016/j.ijcchd.2023.100479
Marieke Nederend , Anastasia D. Egorova , Frank van der Kley , Philippine Kiès , Arno A.W. Roest , Martin J. Schalij , Monique R.M. Jongbloed
{"title":"Percutaneous closure of veno-venous collaterals in adult patients with univentricular physiology after Fontan palliation: Single centre experience and systematic review","authors":"Marieke Nederend ,&nbsp;Anastasia D. Egorova ,&nbsp;Frank van der Kley ,&nbsp;Philippine Kiès ,&nbsp;Arno A.W. Roest ,&nbsp;Martin J. Schalij ,&nbsp;Monique R.M. Jongbloed","doi":"10.1016/j.ijcchd.2023.100479","DOIUrl":"10.1016/j.ijcchd.2023.100479","url":null,"abstract":"<div><h3>Background</h3><p>The Fontan operation resulted in improved survival of patients with congenital heart defects not equipped to sustain biventricular circulation. Long-term complications are common, such as veno-venous collaterals (VVC). The aim of this study was to evaluate patient characteristics, percutaneous treatment strategy and (short-term) outcomes in adult Fontan patients with VVC, and review literature to date.</p></div><div><h3>Methods</h3><p>In this single-centre retrospective observational cohort study, patients who underwent percutaneous VVC closure between 2017 and 2023 were identified.</p></div><div><h3>Results</h3><p>Thirteen patients underwent percutaneous VVC closure (77 % female, age at intervention 24 ± 4 years, 77 % systemic left ventricle, 77 % extracardiac tunnel, median conduit size 16 [16–20]mm). Indications for closure were symptoms and/or significant exercise-related hypoxia. Mean Fontan pressure was 10±4 mmHg. The VVC originated from tributaries of the vena cava superior (VCS) and connected to pulmonary veins (8 VVC, 32 %), VCS to systemic atrium (3 VVC, 12 %), VCS to coronary sinus (3 VVC, 12 %) and tributaries of vena cava inferior to pulmonary veins (11 VVC, 44 %). Twenty-three VVC were occluded using coils and/or plugs. No periprocedural complications occurred. At first follow-up at least 6 months after closure (n = 11), 9 patients (82 %) reported symptom reduction. Saturation at rest and peak exercise increased significantly (96 ± 3 to 98 ± 1 %, p = 0.040; 89 ± 3 to 93 ± 5 %, p = 0.024, respectively). Exercise capacity remained unchanged.</p></div><div><h3>Conclusions</h3><p>VVC typically connect the tributaries of the vena cava inferior and/or superior with the pulmonary veins. Low Fontan pressures do not exclude the presence of VVC. Percutaneous closure of VVC is technically feasible, safe, and associated with symptom reduction and a significant rise in resting and exercise oxygen saturation.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668523000411/pdfft?md5=d412c4e75c81e5c7f972aa878e07bd1f&pid=1-s2.0-S2666668523000411-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135660789","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
Transcatheter pulmonary valve implantation in clinical practice: A nationwide survey of cardiological implanting and non-implanting physicians 经导管肺动脉瓣植入在临床实践中的应用:全国心脏植入和非植入医师的调查
International journal of cardiology. Congenital heart disease Pub Date : 2023-10-05 DOI: 10.1016/j.ijcchd.2023.100478
Biagio Castaldi , Gianfranco Butera , Massimo Chessa , Lorenzo Galletti , Alessandro Giamberti , Luca Giugno , Aurelio Secinaro , Vladimiro Vida , Giovanni Di Salvo , Mario Carminati
{"title":"Transcatheter pulmonary valve implantation in clinical practice: A nationwide survey of cardiological implanting and non-implanting physicians","authors":"Biagio Castaldi ,&nbsp;Gianfranco Butera ,&nbsp;Massimo Chessa ,&nbsp;Lorenzo Galletti ,&nbsp;Alessandro Giamberti ,&nbsp;Luca Giugno ,&nbsp;Aurelio Secinaro ,&nbsp;Vladimiro Vida ,&nbsp;Giovanni Di Salvo ,&nbsp;Mario Carminati","doi":"10.1016/j.ijcchd.2023.100478","DOIUrl":"https://doi.org/10.1016/j.ijcchd.2023.100478","url":null,"abstract":"<div><h3>Aim</h3><p>Transcatheter Pulmonary Valve Implantation (TPVI), when feasible, is the first-line approach to pulmonary valve replacement. Our aim was to obtain a picture of current TPVI practice in Italy.</p></div><div><h3>Methods</h3><p>After conducting a literature review on TPVI, online surveys were devised by an Advisory Board of 10 experts from the three Italian reference centers for congenital heart diseases and sent electronically to physicians working either in implanting center or in referral non-implanting cardiologic centers.</p></div><div><h3>Results</h3><p>Approximately 450 physicians across Italy were invited to contribute. 82 (18%) physicians answered. EchoColorDoppler, electrocardiogram and cardiac magnetic resonance were considered the first line approach to monitor these patients, before and after TPVI.</p><p>For non-implanting centers, reasons for non-referral of patients for PVR were: paucisymptomatic disease (67%) and patients’ poor adherence to disease management programs (41%), but also the lack of connections with specialized centers (33%). For implanters, the main reasons for refraining from TPVI were: high risk for coronary compression (67% first rank), the need for concomitant cardiac surgical procedures (39% first rank) and the unsuitable anatomy of the conduit (39% first rank). The availability of new larger valves of a self-expandable nature was indicated as a key technological development for expanding the cohort of patients currently eligible for TPVI.</p></div><div><h3>Conclusions</h3><p>Despite a non-invasive imaging protocol for the follow up and selection of patients candidate to TPVI is well implemented in Italy, there is still a lack in connections between non-implanting and implanting centers.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49810467","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}
引用次数: 0
Transcatheter aortic valve-in-valve implantation in right ventricle-aorta conduit in an adult patient with Fontan circulation Fontan循环成年患者右心室主动脉导管经导管主动脉瓣植入术
International journal of cardiology. Congenital heart disease Pub Date : 2023-09-17 DOI: 10.1016/j.ijcchd.2023.100476
Marieke Nederend , Frank van der Kley , Madelien V. Regeer , Regina Bökenkamp , Arend de Weger , Monique R.M. Jongbloed , Anastasia D. Egorova
{"title":"Transcatheter aortic valve-in-valve implantation in right ventricle-aorta conduit in an adult patient with Fontan circulation","authors":"Marieke Nederend ,&nbsp;Frank van der Kley ,&nbsp;Madelien V. Regeer ,&nbsp;Regina Bökenkamp ,&nbsp;Arend de Weger ,&nbsp;Monique R.M. Jongbloed ,&nbsp;Anastasia D. Egorova","doi":"10.1016/j.ijcchd.2023.100476","DOIUrl":"https://doi.org/10.1016/j.ijcchd.2023.100476","url":null,"abstract":"<div><p>Catheter interventions can offer patient tailored solutions in high-risk congenital heart disease patients. A 21-year-old male with a Fontan circulation in the setting of unbalanced atrioventricular septal defect with a hypoplastic left ventricle and an aortic homograft connecting the right ventricular outflow tract to the ascending aorta, developed failure of the heavily calcified homograft with severe regurgitation and stenosis. He underwent three sequential transcatheter aortic valve-in-valve implantations to address the homograft failure and the subsequent paravalvular regurgitation, with satisfactory result and improved hemodynamics.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49810465","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}
引用次数: 0
Clinical profile, prenatal detection and predictors of outcome of heterotaxy syndromes in Western Australia 西澳大利亚州异位综合征的临床特征、产前检测和预后预测因素
International journal of cardiology. Congenital heart disease Pub Date : 2023-09-09 DOI: 10.1016/j.ijcchd.2023.100472
Bradley MacDonald , Zoe Vetten , James Ramsay , David Andrews , Deane Yim
{"title":"Clinical profile, prenatal detection and predictors of outcome of heterotaxy syndromes in Western Australia","authors":"Bradley MacDonald ,&nbsp;Zoe Vetten ,&nbsp;James Ramsay ,&nbsp;David Andrews ,&nbsp;Deane Yim","doi":"10.1016/j.ijcchd.2023.100472","DOIUrl":"https://doi.org/10.1016/j.ijcchd.2023.100472","url":null,"abstract":"<div><h3>Background</h3><p>Heterotaxy syndromes encompass left and right atrial isomerism (LAI and RAI respectively) and are associated with variable cardiac and non-cardiac anomalies which greatly influence outcomes. RAI is usually associated with complex congenital heart disease (CHD), early surgical intervention and increased mortality. LAI is less commonly associated with complex CHD but can be associated with heart block. The objective of this study was to review the clinical features and outcomes of patients with heterotaxy syndromes in Western Australia (WA).</p></div><div><h3>Methods</h3><p>A retrospective review was performed of live born patients diagnosed with heterotaxy from 2003 to 2022 in a statewide tertiary cardiac service, representing all cases in WA with a view to compare the outcomes between LAI and RAI at our centre.</p></div><div><h3>Results</h3><p>30 patients (53% male) were diagnosed with heterotaxy; 16 (53%) with LAI and 14 (47%) with RAI. Overall incidence was 0.48 per 10,000 live births over the defined period. RAI patients were significantly more likely to have an antenatal diagnosis (81.8% versus LAI 28.6%, p = 0.03). Overall, 5-year survival was 56% for RAI and 87% for LAI. No deaths occurred after the first 12 months of life with a median follow-up of 65 months (IQR 114.8). RAI was associated with asplenia (91%), atrioventricular septal defect (91%) and a functionally univentricular circulation (71%). LAI was associated with polysplenia (100%) and complete heart block in 3 patients (19%). Surgical pathways included repair of anomalous pulmonary venous return (45%), Blalock Taussig shunt (60%), bidirectional cavopulmonary connection (50%) and Fontan completion (30%).</p></div><div><h3>Conclusions</h3><p>Patients with RAI suffer high mortality and early surgical intervention, with few making it to Fontan completion. By comparison patients with LAI have less morbidity and mortality. The management of heterotaxy continues to be challenging due to widely associated cardiac and extracardiac manifestations.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49810466","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}
引用次数: 0
Systemic hypertension in adults with congenital heart diseases 成人先天性心脏病患者的系统性高血压
International journal of cardiology. Congenital heart disease Pub Date : 2023-09-01 DOI: 10.1016/j.ijcchd.2023.100456
Jolanda Sabatino , Martina Avesani , Domenico Sirico , Elena Reffo , Biagio Castaldi , PierPaolo Bassareo , Giovanni Di Salvo
{"title":"Systemic hypertension in adults with congenital heart diseases","authors":"Jolanda Sabatino ,&nbsp;Martina Avesani ,&nbsp;Domenico Sirico ,&nbsp;Elena Reffo ,&nbsp;Biagio Castaldi ,&nbsp;PierPaolo Bassareo ,&nbsp;Giovanni Di Salvo","doi":"10.1016/j.ijcchd.2023.100456","DOIUrl":"10.1016/j.ijcchd.2023.100456","url":null,"abstract":"<div><p>Long-term effects of systemic hypertension (HTN) and HTN-mediated damages have been largely studied in non-congenital adult populations. By contrast, robust data about the predisposing factors, prevalence, consequences, and treatment of HTN in adults with congenital heart diseases (ACHD) is still scarce. Different mechanisms including the underlying cardiac disease, cardiac surgery and its consequences, the development of metabolic syndrome and secondary forms seem to play a role in HTN in ACHDs. To mitigate the potential long-term effects of HTN in this complex population, a meticulous follow-up is mandatory to identify patients who should receive treatment, and tailored strategies should be applied to obtain the best as possible result.</p><p>Thus, this review will investigate risk factors, effects, and treatments of HTN in ACHD patients.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45178589","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}
引用次数: 1
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