International journal of cardiology. Congenital heart disease最新文献

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Cancer risk in adult congenital heart disease 成人先天性心脏病癌症风险
International journal of cardiology. Congenital heart disease Pub Date : 2023-06-01 DOI: 10.1016/j.ijcchd.2023.100441
Suvasini Lakshmanan , Alessia Gimelli
{"title":"Cancer risk in adult congenital heart disease","authors":"Suvasini Lakshmanan ,&nbsp;Alessia Gimelli","doi":"10.1016/j.ijcchd.2023.100441","DOIUrl":"10.1016/j.ijcchd.2023.100441","url":null,"abstract":"<div><p>Advances in diagnostics and interventional/surgical treatment of patients with congenital heart disease (CHD) over the past several decades, allows a decline in rates of all-cause mortality, with a significant reduction in proportion of infant and childhood deaths with severe forms of CHD. However, the risk of premature death in adults with complex CHD remains elevated. A growing body of evidence has recently described the impact of non-cardiovascular comorbidities such as cancers, on morbidity, health care utilizations and mortality in adult patients with CHD. Aim of this review is to provide an appraisal of the current evidence that report associations between CHD and increased cancer risk in children and adults, and provide an overview of the specific risk factors.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49252207","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
Fifth decennium after the arterial switch operation for transposition of the great arteries 大动脉转位手术后的第五个十年
International journal of cardiology. Congenital heart disease Pub Date : 2023-06-01 DOI: 10.1016/j.ijcchd.2023.100451
Sebastiaan W.H. van Wijk , Maaike Wulfse , Mieke M.P. Driessen , Martijn G. Slieker , Pieter A. Doevendans , Paul H. Schoof , Gert Jan J. Sieswerda , Johannes M.P.J. Breur
{"title":"Fifth decennium after the arterial switch operation for transposition of the great arteries","authors":"Sebastiaan W.H. van Wijk ,&nbsp;Maaike Wulfse ,&nbsp;Mieke M.P. Driessen ,&nbsp;Martijn G. Slieker ,&nbsp;Pieter A. Doevendans ,&nbsp;Paul H. Schoof ,&nbsp;Gert Jan J. Sieswerda ,&nbsp;Johannes M.P.J. Breur","doi":"10.1016/j.ijcchd.2023.100451","DOIUrl":"10.1016/j.ijcchd.2023.100451","url":null,"abstract":"<div><h3>Background</h3><p>From 1977 onwards, patients with both simple and complex transposition of the great arteries (TGA) have been treated with the arterial switch operation (ASO) in the Wilhelmina Children's Hospital/University Medical Center Utrecht the Netherlands. In this study, we compared mortality and morbidity between two patient groups: A. operated before and B. after 1991, specifically focusing on late ventricular function and reinterventions.</p></div><div><h3>Methods</h3><p>A single institution retrospective cohort study was performed on patients who had an ASO for either simple or complex TGA. Data were collected from medical records. The entire patient cohort (n = 283) was divided in a group with more than 30 years of follow-up (A) and a group with less than 30 years of follow-up (B). Clinical and standardized echocardiographic follow-up was evaluated.</p></div><div><h3>Results</h3><p>Group A consisted of 79 patients, of whom follow-up was available in 59 patients (median follow-up 34.8 years, IQR 33.0–36.9). Group B consisted of 204 patients, of whom 195 long-term survivors (median follow-up 14.9 years, IQR 10.0–21.2). Early survival was best in group B (A: 67.8% vs. B: 96.6%, p &lt; 0.001), whereas late mortality (in total 1.8%) was similar for both groups. Reinterventions, corrected for follow-up time, were more frequent in group A (p = 0.005). In total 65 patients (25.1%) required 105 late reinterventions including 4 late aortic valve replacements. The mode of reinterventions has shifted over time, from surgical to more catheter-based (p = 0.03). The vast majority of patients functioned in NYHA class I. In contrast to the recent cohort, who have a normal average LVEF (%), the average LVEF in the oldest cohort was in the bottom percentile of normal range.</p></div><div><h3>Conclusion</h3><p>The majority of patients in their fifth decade after ASO are in functional class I. Early outcome improved showing reduced mortality and need for reoperation. However, a trend towards reduced left ventricular function and late aortic valve replacements justify further research.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47650298","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
Postpartum cardiac arrest in a woman with an uncorrected sinus venosus type of atrial septal defect: A case report 一例未矫正静脉窦型房间隔缺损妇女产后心脏骤停病例报告
International journal of cardiology. Congenital heart disease Pub Date : 2023-06-01 DOI: 10.1016/j.ijcchd.2023.100444
Joelle Régine Mekoa Mbarga , Estelle Tenisch , Aicha Saleh , Mathieu Le Bloa , David Desseauve , Raymond Pfister , Giulia Domenichini , Christina Corby-Zauner , Jean-Francois Surmely , Judith Bouchardy , Etienne Pruvot , Tobias Rutz
{"title":"Postpartum cardiac arrest in a woman with an uncorrected sinus venosus type of atrial septal defect: A case report","authors":"Joelle Régine Mekoa Mbarga ,&nbsp;Estelle Tenisch ,&nbsp;Aicha Saleh ,&nbsp;Mathieu Le Bloa ,&nbsp;David Desseauve ,&nbsp;Raymond Pfister ,&nbsp;Giulia Domenichini ,&nbsp;Christina Corby-Zauner ,&nbsp;Jean-Francois Surmely ,&nbsp;Judith Bouchardy ,&nbsp;Etienne Pruvot ,&nbsp;Tobias Rutz","doi":"10.1016/j.ijcchd.2023.100444","DOIUrl":"10.1016/j.ijcchd.2023.100444","url":null,"abstract":"<div><p>The number of women with congenital heart disease (CHD) surviving to childbearing age is continuously growing. Although most pregnancies in this patient-population are well tolerated, there is a significant risk of potentially fatal complications. We describe a case of a previously completely asymptomatic patient who was diagnosed late during pregnancy with an inferior sinus venosus type atrial septal defect (ISV-ASD) and anomalous connection of all right pulmonary veins (PAPVC) who presented a cardiac arrest with ventricular fibrillation the day after delivery. She recovered completely and underwent subsequent surgical repair and implantation of a subcutaneous defibrillator (S-ICD).</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47814184","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
Long-term outcomes of patients with single ventricle who do not undergo Fontan palliation 未接受Fontan姑息治疗的单心室患者的长期预后
International journal of cardiology. Congenital heart disease Pub Date : 2023-06-01 DOI: 10.1016/j.ijcchd.2023.100457
Wayne C. Zheng , Yves d’Udekem , Leeanne E. Grigg , Dominica Zentner , Rachael Cordina , David S. Celermajer , Edward Buratto , Igor E. Konstantinov , Melissa G.Y. Lee
{"title":"Long-term outcomes of patients with single ventricle who do not undergo Fontan palliation","authors":"Wayne C. Zheng ,&nbsp;Yves d’Udekem ,&nbsp;Leeanne E. Grigg ,&nbsp;Dominica Zentner ,&nbsp;Rachael Cordina ,&nbsp;David S. Celermajer ,&nbsp;Edward Buratto ,&nbsp;Igor E. Konstantinov ,&nbsp;Melissa G.Y. Lee","doi":"10.1016/j.ijcchd.2023.100457","DOIUrl":"https://doi.org/10.1016/j.ijcchd.2023.100457","url":null,"abstract":"<div><h3>Background</h3><p>Patients with single ventricle (SV) without Fontan palliation are uncommon, and their long-term outcomes remain unclear.</p></div><div><h3>Methods</h3><p>Retrospective study of 35 adult patients with SV without Fontan from two tertiary centers. Primary outcome was mortality.</p></div><div><h3>Results</h3><p>Median age at first follow-up was 31 years (IQR: 20–40). Most common defect was double inlet left ventricle (34%), and 69% had left ventricular morphology. Patients were unoperated (46%), had systemic-to-pulmonary artery shunt (31%) or bidirectional cavopulmonary shunt (23%) as final palliation. Most common reasons for not progressing to Fontan palliation were pulmonary vascular disease (54%), patient refusal (17%), Fontan takedown (14%), and hypoplastic pulmonary arteries (11%). Baseline mean hemoglobin was 195 ± 29 g/L, mean O<sub>2</sub> saturation 83 ± 6.9%, and 4 patients in NYHA Class III‒IV. After a mean follow-up of 10 ± 8.3 years, there were 9 deaths with heart failure being the leading cause (n = 6). Age-adjusted survival of these adult SV survivors was 73% and 53% at 40 and 50 years of age, respectively. Deceased patients more frequently had renal impairment (50% vs 0%) and QRS prolongation (75% vs 16%) at baseline (all p &lt; 0.05). During follow-up, 40% had a new arrhythmia (atrial: n = 14, ventricular: n = 3), 34% had one or more hospitalizations for heart failure, and 17% had a stroke. A greater proportion of patients with pre-existing or new atrial/ventricular arrhythmia died compared to those without (42% vs 6%, p = 0.02).</p></div><div><h3>Conclusions</h3><p>Patients with SV without Fontan have high mortality and a substantial burden of cardiovascular complications, particularly arrhythmia. QRS prolongation and renal impairment were associated with mortality.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49774923","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
Stroke and systemic embolism in adult congenital heart disease 成人先天性心脏病的中风和全身栓塞
International journal of cardiology. Congenital heart disease Pub Date : 2023-06-01 DOI: 10.1016/j.ijcchd.2023.100453
Katja Prokšelj
{"title":"Stroke and systemic embolism in adult congenital heart disease","authors":"Katja Prokšelj","doi":"10.1016/j.ijcchd.2023.100453","DOIUrl":"https://doi.org/10.1016/j.ijcchd.2023.100453","url":null,"abstract":"<div><p>Despite striking improvement in survival of patients with congenital heart disease (CHD), the risk of long-term complications remains high. Stroke and systemic embolism are common and potentially devastating complications that significantly affect morbidity and mortality in CHD. The risk of stroke in adult congenital heart disease (ACHD) is higher than in the general population, patients are affected at an earlier age, and the risk continues to increase with age. Specific types of defects are at high-risk for stroke and other systemic embolisms, particularly patients with complex congenital heart disease, cyanotic heart disease, Fontan circulation, and cardiac shunts. Associated factors such as atrial arrhythmias, heart failure, mechanical valves and intracardiac devices, and infective endocarditis increase the risk of thromboembolic events. Acquired conventional risk factors for cardiovascular disease further increase the burden of stroke in the aging ACHD population. Anticoagulation is a cornerstone for prevention of thromboembolic events. Risk stratification in ACHD remains challenging and should be individualized. General risk stratification models, such as the CHA<sub>2</sub>DS<sub>2</sub>-VASc score, are not reliable in the heterogeneous ACHD population and should only be used in mild to moderate CHD. Anticoagulation is recommended as primary prevention in high-risk patients (patients with intracardiac repair, cyanotic CHD, Fontan circulation, or systemic right ventricle) with atrial arrhythmias. In patients with other CHD, general stratification models should be used to decide when to initiate anticoagulation, taking into account specificities of underlying heart disease and potential residua. Screening and treatment of conventional risk factors for cardiovascular disease may further improve long-term outcomes.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49774934","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: 2
Structure and function of the aortic and pulmonary outflows in a patient, 12 years after Nikaidoh operation, A cautionary Note Nikaidoh手术后12年患者主动脉和肺流出物的结构和功能,一个警示
International journal of cardiology. Congenital heart disease Pub Date : 2023-06-01 DOI: 10.1016/j.ijcchd.2023.100450
Magdi H. Yacoub , Ahmed Afifi , Hatem Hosny , Ahmed Mahgoub , Mohamed Nagy , Amr El Sawy , Nairouz Shehata , Ayman M. Ibrahim , Mina Azer , Abdelrahman Elafifi , Soha Romeih
{"title":"Structure and function of the aortic and pulmonary outflows in a patient, 12 years after Nikaidoh operation, A cautionary Note","authors":"Magdi H. Yacoub ,&nbsp;Ahmed Afifi ,&nbsp;Hatem Hosny ,&nbsp;Ahmed Mahgoub ,&nbsp;Mohamed Nagy ,&nbsp;Amr El Sawy ,&nbsp;Nairouz Shehata ,&nbsp;Ayman M. Ibrahim ,&nbsp;Mina Azer ,&nbsp;Abdelrahman Elafifi ,&nbsp;Soha Romeih","doi":"10.1016/j.ijcchd.2023.100450","DOIUrl":"10.1016/j.ijcchd.2023.100450","url":null,"abstract":"<div><h3>Background</h3><p>Nikaidoh translocation operation is increasingly being used for patients with TGA, VSD and LVOTO. The early results of this operation are excellent and possibly better than the Rastelli repair. However, the long-term results remain inadequately defined.</p></div><div><h3>Methods and results</h3><p>Detailed follow up data of a patient, 12 years after Nikaidoh operation, using computerized image analysis are reported. The patient complained of chest pain on exertion. Imaging showed severe dilatation of the non-coronary sinus of the aortic root, compressing the RVOT and pulmonary artery branches resulting in RV dilatation. The dilated aortic sinus showed marked pulsatility with expansion during systole and diastole. As the child was symptomatic, with a risk of aneurysm rupture, excision was performed. This complication is thought to be due to interruption of the innervation of the root as well as cutting vasa vasorum.</p></div><div><h3>Conclusion</h3><p>Nikaidoh operation is a viable solution for the repair of TGA, VSD, PS. Dilatation of the neo-aortic root should be carefully followed and causes of the dilatation investigated.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47190266","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
Echocardiographic right ventricular remodeling after percutaneous atrial septal defect closure 经皮房间隔缺损封堵术后右心室重构的超声心动图
International journal of cardiology. Congenital heart disease Pub Date : 2023-06-01 DOI: 10.1016/j.ijcchd.2023.100459
Daan Bosshardt , Michiel Voskuil , Gregor J. Krings , Mirella M.C. Molenschot , Maarten J. Suttorp , Heleen B. van der Zwaan , Martijn C. Post
{"title":"Echocardiographic right ventricular remodeling after percutaneous atrial septal defect closure","authors":"Daan Bosshardt ,&nbsp;Michiel Voskuil ,&nbsp;Gregor J. Krings ,&nbsp;Mirella M.C. Molenschot ,&nbsp;Maarten J. Suttorp ,&nbsp;Heleen B. van der Zwaan ,&nbsp;Martijn C. Post","doi":"10.1016/j.ijcchd.2023.100459","DOIUrl":"10.1016/j.ijcchd.2023.100459","url":null,"abstract":"<div><h3>Background</h3><p>In order to prevent right ventricular (RV) dysfunction, closure of secundum type atrial septal defects (ASD) is often indicated and percutaneous closure is the preferred treatment modality to do so. The magnitude and time course of RV remodeling is still incompletely understood.</p></div><div><h3>Methods</h3><p>This retrospective cohort study included consecutive patients who underwent percutaneous secundum ASD closure in two tertiary referral centers in The Netherlands. Main study parameters were RV and right atrial dimensions measured with transthoracic echocardiography before and after percutaneous ASD closure. Secondary outcome was change in New York Heart Association (NYHA) functional class at follow-up.</p></div><div><h3>Results</h3><p>From the 454 patients who underwent secundum ASD closure, 88 patients (median age 46 [range 17–84]) were included. The majority of RV and right atrial dimensional improvement occurred within 24 h. After a median follow-up of 569 days (IQR: 280–772) a further decrease in dimensions was observed. Comparing baseline and latest follow-up, end-diastolic RV basal diameter decreased from 4.5 SEM 0.1 to 3.9 SEM 0.1 cm (<em>p</em> &lt; <em>0.001)</em> and end-systolic right atrial area from 22.9 SEM 1.0 to 17.9 SEM 0.7 cm<sup>2</sup> (<em>p</em> &lt; <em>0.001)</em>. No significant changes in RV function were observed. NYHA functional class improved from 1.5 at baseline (IQR: 1.0–2.0) to 1.0 (IQR: 1.0–1.5) at latest follow-up (<em>p</em> &lt; <em>0.001)</em>.</p></div><div><h3>Conclusion</h3><p>Remodeling of the RV heart dimensions commences within 24 h after percutaneous secundum ASD closure for the majority of patients, followed by a further gradual recovery. A concurrent improvement of NYHA functional class was observed during follow-up.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45183355","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
Not so benign- A rare cause of right ventricular outflow tract ventricular tachycardia in patient with congenital heart disease 不那么良性——先天性心脏病患者右心室流出道室性心动过速的罕见病因
International journal of cardiology. Congenital heart disease Pub Date : 2023-06-01 DOI: 10.1016/j.ijcchd.2023.100454
Becky Yi-Wen Liao , Anthony Kueh , Andrew Martin , Jignesh Shah , Ajay Iyengar , Chethan Kasargod
{"title":"Not so benign- A rare cause of right ventricular outflow tract ventricular tachycardia in patient with congenital heart disease","authors":"Becky Yi-Wen Liao ,&nbsp;Anthony Kueh ,&nbsp;Andrew Martin ,&nbsp;Jignesh Shah ,&nbsp;Ajay Iyengar ,&nbsp;Chethan Kasargod","doi":"10.1016/j.ijcchd.2023.100454","DOIUrl":"10.1016/j.ijcchd.2023.100454","url":null,"abstract":"<div><p>Right ventricular outflow tract ventricular tachycardia (RVOT VT) is a well-recognised subtype of idiopathic ventricular tachyarrhythmia and often described in young healthy individuals. This arrhythmia typically occurs in patients with a structurally normal heart, and in this context is generally considered benign. In this case we present a rare association between RVOT VT and a ruptured sinus of Valsalva aneurysm in a patient known to have a restrictive ventricular septal defect. Prompt surgical intervention was necessary to prevent potentially life threatening consequences.</p><p>The diagnostic and surgical evaluation learnt from this case would be useful to remind clinicians to explore rarer causes of RVOT VT especially when patients have underlying congenital heart disease. This case also highlights the importance of utilizing multi-modality imaging for the anatomical assessment of this pathology, which aids definitive management.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42335129","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
Outcome of Down patients with repaired versus unrepaired atrioventricular septal defect 房室间隔缺损修复与未修复下行患者的疗效
International journal of cardiology. Congenital heart disease Pub Date : 2023-06-01 DOI: 10.1016/j.ijcchd.2023.100452
Mathies Daene , Lore De Pauw , Pieter De Meester , Els Troost , Philip Moons , Marc Gewillig , Filip Rega , Alexander Van De Bruaene , Werner Budts
{"title":"Outcome of Down patients with repaired versus unrepaired atrioventricular septal defect","authors":"Mathies Daene ,&nbsp;Lore De Pauw ,&nbsp;Pieter De Meester ,&nbsp;Els Troost ,&nbsp;Philip Moons ,&nbsp;Marc Gewillig ,&nbsp;Filip Rega ,&nbsp;Alexander Van De Bruaene ,&nbsp;Werner Budts","doi":"10.1016/j.ijcchd.2023.100452","DOIUrl":"10.1016/j.ijcchd.2023.100452","url":null,"abstract":"<div><h3>Background and aims</h3><p>Patients with Down Syndrome (DS) are frequently born with an atrioventricular septal defect (AVSD). Surgical repair of the defect aims to minimize mortality and morbidity. However, a surgical intervention, specifically in DS patients, is not without risk and a subgroup of patients underwent only conservative non-surgical treatment. Outcome data of these different approaches are scarce. The aim of this retrospective study was to compare the long-term outcome of DS patients with and without surgery for AVSD.</p></div><div><h3>Methods</h3><p>DS patients registered with AVSD in the hospital's database from January 1980 till December 2020 were selected. Patient characteristics, peri-operative if appropriate, and follow-up data were obtained from the medical files.</p></div><div><h3>Results</h3><p>In total, 72 unrepaired (36 male, 50%) and 134 repaired patients (61 male, 46%) were included. After a maximum of 60 years of follow-up, the all-cause mortality was 45.8% and 17.1%, respectively. Thirty-six percent and 13%, respectively, were labeled as non-cardiovascular death. Mean survival time for unrepaired AVSD was 40.7 years (95% CI 36.1–45.2) and for repaired AVSD 38.5 years (95% CI 35.3–41.6) (Log rank p = 0.465). However, the survival rate 35 years after birth was 62.1% for unrepaired patients versus 81.7% for repaired patients. Mortality rates were the highest the first months after surgical repair.</p></div><div><h3>Conclusions</h3><p>The mean survival rate of Down patients, born with an AVSD, did not differ between repair or not. However, long-term survival rate was higher in patients who underwent surgical repair. Mortality was highest the first months after surgery.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49414544","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
Definitions of resilience and resilience resource use as described by adults with congenital heart disease 成人先天性心脏病患者对弹性和弹性资源使用的定义
International journal of cardiology. Congenital heart disease Pub Date : 2023-06-01 DOI: 10.1016/j.ijcchd.2023.100447
Jill M. Steiner , Erin Abu-Rish Blakeney , Andrea Corage Baden , Vea Freeman , Joyce Yi-Frazier , J. Randall Curtis , Ruth A. Engelberg , Abby R. Rosenberg
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