International journal of cardiology. Congenital heart disease最新文献

{"title":"A new strategy for monitoring of direct oral anticoagulants in patients with cyanotic and complex congenital heart disease","authors":"Fabienne Dirbach ,&nbsp;Eleni Goulouti ,&nbsp;Judith Bouchardy ,&nbsp;Magalie Ladouceur ,&nbsp;Lorenzo Alberio ,&nbsp;Tobias Rutz","doi":"10.1016/j.ijcchd.2024.100545","DOIUrl":"10.1016/j.ijcchd.2024.100545","url":null,"abstract":"<div><h3>Background</h3><div>Patients with congenital heart disease (CHD) often require an oral anticoagulation. Vitamin K antagonists (VKA) are the standard treatment, however, an increased hematocrit in patients with secondary erythrocytosis due to cyanosis complicates the correct measurement of the international normalized ratio. Direct oral anticoagulants (DOAC) could be an alternative, but data on their efficacy and safety in complex and cyanotic CHD patients are scarce. This study proposes a new strategy of DOAC monitoring in these patients using D-dimers and DOAC trough levels.</div></div><div><h3>Methods</h3><div>This is a retrospective study including cyanotic and complex CHD patients requiring oral anticoagulation. Clinical, cardiac imaging and laboratory data were collected before and after start of DOAC. The new monitoring strategy consists of determination of D-dimers and DOAC trough levels at 1–4 weeks, 1–6 months, 6–12 months, &gt;1 year after start of DOAC.</div></div><div><h3>Results</h3><div>Eleven patients were included. For 10 patients D-dimers and DOAC trough levels were in target range. In one patient, D-dimers increased continuously after start of DOAC despite dose escalation, suggesting insufficient DOAC efficacy and finally requiring a switch to VKA. D-dimers subsequently decreased under VKA to the therapeutic range. In three patients, one thromboembolic and two minor bleeding complications occurred. No major complications were observed.</div></div><div><h3>Conclusions</h3><div>We propose a new strategy of monitoring of oral anticoagulation with DOAC and report its implementation in clinical routine. Highlighting the importance of pharmacokinetic and -dynamic monitoring, this strategy could improve safety and efficacy of DOAC in cyanotic and complex CHD which, however, requires a prospective validation.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"18 ","pages":"Article 100545"},"PeriodicalIF":0.8,"publicationDate":"2024-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142417623","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}

{"title":"Association between insurance type, clinical characteristics, and healthcare use in adults with congenital heart disease","authors":"Julia Claire Cambron ,&nbsp;Evan F. Shalen ,&nbsp;Lidija B. McGrath ,&nbsp;Katrina Ramsey ,&nbsp;Abigail Khan","doi":"10.1016/j.ijcchd.2024.100543","DOIUrl":"10.1016/j.ijcchd.2024.100543","url":null,"abstract":"<div><h3>Introduction</h3><div>Adults with congenital heart disease (CHD) represent a heterogeneous and growing population with high healthcare utilization. We sought to understand the association between insurance type, healthcare use, and outcomes among adults with CHD in Oregon.</div></div><div><h3>Methods</h3><div>The Oregon All Payers All Claims database from 2010 to 2017 was queried for adults aged 18–65 in 2014 with ICD-9 or 10 codes consistent with CHD; patient demographics, comorbidities, healthcare use, and disease severity were identified. Insurance type was categorized as either public (Medicare and Medicaid) or private (commercial). Descriptive statistics were used to compare groups. Use rates and odds ratios were calculated representing probability of at least one event per person-year using logistic regression with clustering on patients.</div></div><div><h3>Results</h3><div>Of 13,792 adults with CHD, 48 % had a form of public insurance. More publicly insured patients had moderate or severe anatomic complexity (29.5 % vs. 23.0 %; p &lt; 0.0001), treatment for drug and alcohol use (25.0 % vs. 7.2 %; p &lt; 0.0001), and mental health diagnoses (66.6 % vs. 51.0 %; p &lt; 0.0001). They were more likely to reside in a rural area (24.5 % vs. 16.1 %; p &lt; 0.0001). Adjusted for age and CHD severity, publicly insured patients were less likely to access overall ambulatory care (aOR 0.72, 99 % CI 0.66 to 0.80) but more likely to access emergency (aOR 3.86, 99 % CI 3.62 to 4.12) and inpatient (aOR 3.06, 99 % CI 2.81 to 3.33) care, as shown in Fig. 1. Length of hospital stay (5.7 vs. 4.4 days, p &lt; 0.0001) and rates of 30-day readmission (17.1 % vs. 11.0 %, p &lt; 0.001) were higher in those with public insurance. However, individuals with public insurance were significantly more likely to undergo their annual guideline-indicated echocardiogram (aOR 1.49, 99 % CI 1.23 to 1.80) and attend their annual ACHD visits (aOR 1.62, 99 % CI 1.40 to 1.87).</div></div><div><h3>Conclusions</h3><div>Our study shows that publicly insured adults with CHD in Oregon have more anatomically complex disease, more comorbidities, and higher healthcare use. While they were more likely to receive guideline-indicated ACHD care, they were also higher utilizers of emergency room and inpatient resources, implying that they may benefit from targeted interventions to improve outcomes and decrease unplanned healthcare use.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"18 ","pages":"Article 100543"},"PeriodicalIF":0.8,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142655284","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}

{"title":"Discovery of myosin light chain kinase gene variant in a patient with tetralogy of Fallot suffering aortic dissection: Implications for pathogenesis and the role of family and population screening","authors":"Radoslaw Debiec ,&nbsp;Armia Ebeid ,&nbsp;Stephen Hamby ,&nbsp;Odeta Anciunaite ,&nbsp;Anne Illsley ,&nbsp;Ali Nizam ,&nbsp;Madiha Iqbal ,&nbsp;Kassem Safwan ,&nbsp;Tariq Saifullah ,&nbsp;Frances Bu’Lock ,&nbsp;Toru Suzuki ,&nbsp;Nilesh J. Samani ,&nbsp;Tom Webb ,&nbsp;Aidan P. Bolger","doi":"10.1016/j.ijcchd.2024.100544","DOIUrl":"10.1016/j.ijcchd.2024.100544","url":null,"abstract":"<div><h3>Background</h3><div>Thoracic aortic dissection (TAD) is an uncommon complication in patients with Tetralogy of Fallot (TOF). Information concerning risk factors for TAD in patients with TOF is very limited.</div></div><div><h3>Methods</h3><div>We report a case of Stanford type A TAD in a female patient with previously repaired TOF. Whole exome sequencing (WES); Novogene UK, Agilent V6 capture kit, Illumina HiSeq 100x depth) was performed to identify genetic variants in genes known to be associated with TAD. A systematic literature review was performed in the NCBI PubMed database to identify case reports of TAD in patients with TOF.</div></div><div><h3>Results</h3><div>The patient was a 31-year-old female who developed Stanford type A aortic dissection having had TOF repair at the age of four years. The thoracic aorta was only minimally dilated (sinus of Valsalva 43 mm) on clinical review 16 months prior to TAD. Of note the patient had completed pregnancy 5 months prior to the dissection. There were no other high-risk features predisposing to TAD. WES identified rare genetic variant in a gene previously associated with TAD: <em>MYLK</em> (p.Arg1405His). The literature review identified nine other case reports of TAD in patients with TOF. The reported patients, had no clinical characteristics that distinguished them from the wider population of patients with TOF.</div></div><div><h3>Conclusions</h3><div>The presence of a rare genetic variant in <em>MYLK</em> is a plausible explanation for the clinical presentation. The variant will need further verification to confirm pathogenicity. Pathogenic <em>MYLK</em> variants have been previously reported in context of dissection with minimally dilated aortas.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"18 ","pages":"Article 100544"},"PeriodicalIF":0.8,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142655285","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}

{"title":"How does regular exercise improve cardiovascular function: Congenital heart disease and beyond","authors":"M. Drakopoulou,&nbsp;P.K. Vlachakis,&nbsp;A. Apostolos,&nbsp;K. Tsioufis,&nbsp;K. Toutouzas","doi":"10.1016/j.ijcchd.2024.100542","DOIUrl":"10.1016/j.ijcchd.2024.100542","url":null,"abstract":"<div><div>Major advances in the fields of paediatric cardiology and cardiac surgery over the past decades have dramatically improved the survival of patients with congenital heart disease (CHD). Thus, care for CHD patients has shifted from managing short-term survival to having the best possible outcome in terms of long-term physical health, development and well-being. In this article, with a special focus on adult CHD (ACHD) population, we address the question: How does regular exercise improve cardiovascular function? We aim to underscore that regular exercise not only offers clinically relevant physiological benefits for patients living with a CHD condition but has also a positive effect on reducing the risk of future cardiovascular events.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100542"},"PeriodicalIF":0.8,"publicationDate":"2024-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143163930","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}

{"title":"Percutaneous pulmonary valve implantation guided by three-dimensional rotational angiography","authors":"Gregor J. Krings ,&nbsp;Bart W. Driesen ,&nbsp;Evangeline G. Warmerdam ,&nbsp;Mirella C. Molenschot ,&nbsp;Gert-Jan T. Sieswerda ,&nbsp;Pieter A. Doevendans ,&nbsp;Arie P.J. van Dijk ,&nbsp;M. Voskuil","doi":"10.1016/j.ijcchd.2024.100541","DOIUrl":"10.1016/j.ijcchd.2024.100541","url":null,"abstract":"<div><h3>Objectives</h3><div>To describe the workflow and value of three-dimensional rotational angiography (3DRA) in percutaneous pulmonary valve implantation (PPVI).</div></div><div><h3>Background</h3><div>3DRA offers visualization of the entire topography in the chest and may enhance safety and reduce the risk for complications in PPVI through improved pre-procedural planning and per-procedural guidance.</div></div><div><h3>Methods</h3><div>All PPVI procedures with the use of 3DRA performed between August 2011 and December 2022 were reviewed. Success rate, complications and radiation dose were assessed. Radiation dose of the latest 3DRA protocol was compared to historical 3DRA data.</div></div><div><h3>Results</h3><div>PPVI was successful in 95 of 102 procedures. Seven procedures were aborted due to coronary compression after balloon testing (n = 3), main pulmonary artery (MPA) oversize (n = 3) and not passing of a Melody valve through a calcified Melody valve in situ (n = 1). PPVI was attempted in 61 homografts, 19 native right ventricular outflow tracts (including transannular patch), 4 previously implanted Melody valves, 2 in previously implanted Sapien valves and 16 in other bioprosthetic valves. A Melody valve was implanted in 43, a Sapien valve in 49 and a Pulsta valve in 1 patient. In 2 patients a Melody as well as a Sapien valve were subsequently implanted. Mean total dose area product (DAP) was 11813 mGycm² and 179 mGycm²/kg for all attempted PPVI's. For successful PPVI 9835 mGycm² and 174 mGycm²/kg. After optimizing the 3DRA protocols the mean dose reduced from 12677 mGycm² to 8551 mGycm² (200 mGycm²/kg to 163 mGycm²/kg). Four patients experienced one or more complications. There were no deaths peri-procedural or during follow-up. Complications were; need for cardiopulmonary resuscitation (n = 2), MPA paravasation (n = 1), valve dysfunction (n = 2).</div></div><div><h3>Conclusions</h3><div>The use of rotational angiography for the guidance of PPVI results in a high success rate, low number of complications with the use of a low amount of radiation.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"18 ","pages":"Article 100541"},"PeriodicalIF":0.8,"publicationDate":"2024-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142418069","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}

{"title":"Corrigendum to <Surgical pulmonary valve replacement at a tertiary adult congenital heart centre in the current era> [Int J Cardiol Congenit Heart Dis 9 September 2022 100394]","authors":"Kana Kubota ,&nbsp;Gerhard-Paul Diller ,&nbsp;Aleksander Kempny ,&nbsp;Andreas Hoschtitzky ,&nbsp;Yasushi Imai ,&nbsp;Masaaki Kawada ,&nbsp;Darryl Shore ,&nbsp;Michael A. Gatzoulis","doi":"10.1016/j.ijcchd.2024.100516","DOIUrl":"10.1016/j.ijcchd.2024.100516","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"17 ","pages":"Article 100516"},"PeriodicalIF":0.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000259/pdfft?md5=7e8c24930838ac591e8818be10f03cb9&pid=1-s2.0-S2666668524000259-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142151498","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}

{"title":"Corrigendum to “Exercise ventilatory reserve predicts survival in adult congenital heart disease associated pulmonary arterial hypertension with Eisenmenger physiology” [Int J Cardiol Congenit Heart Dis, Volume 7, March 2022, 100331]","authors":"Chinthaka B. Samaranayake ,&nbsp;Ruth McNiven ,&nbsp;Aleksander Kempny ,&nbsp;Carl Harries ,&nbsp;Laura C. Price ,&nbsp;Michael Gatzoulis ,&nbsp;Konstantinos Dimopoulos ,&nbsp;Stephen J. Wort ,&nbsp;Colm McCabe","doi":"10.1016/j.ijcchd.2024.100517","DOIUrl":"10.1016/j.ijcchd.2024.100517","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"17 ","pages":"Article 100517"},"PeriodicalIF":0.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000260/pdfft?md5=b5f51d470f3eb3a097cff16c48d3dd74&pid=1-s2.0-S2666668524000260-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142151314","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}

{"title":"Novel techniques for quantifying oxygen pulse curve characteristics during cardiopulmonary exercise testing in tetralogy of fallot","authors":"David M. Leone ,&nbsp;Matthew J. Magoon ,&nbsp;Neha Arunkumar ,&nbsp;Laurie A. Soine ,&nbsp;Elizabeth C. Bayley ,&nbsp;Patrick M. Boyle ,&nbsp;Jonathan Buber","doi":"10.1016/j.ijcchd.2024.100539","DOIUrl":"10.1016/j.ijcchd.2024.100539","url":null,"abstract":"<div><h3>Background</h3><p>Cardiopulmonary exercise testing (CPET) is used in evaluation of repaired tetralogy of Fallot (rTOF), particularly for pulmonary valve replacement need. Oxygen pulse (O₂P) is the CPET surrogate for stroke volume and peripheral oxygen extraction.</p></div><div><h3>Objectives</h3><p>This study assessed O₂P curve properties against non-invasive cardiac output monitoring (NICOM) and clinical testing.</p></div><div><h3>Methods</h3><p>This cross-sectional study included 44 rTOF patients and 10 controls. Three new evaluations for O₂P curve analysis during CPET were developed. Best fit early and late regression slopes of the O₂P curve were used to calculate: 1) the early to late ratio, or “O₂ pulse response ratio” (O₂PRR); 2) the portion of exercise until slope inflection, or “flattening fraction” (FF); 3) the area under the O₂P response curve, or “O₂P curve area”.</p></div><div><h3>Results</h3><p>rTOF patients (median age 35.2 (27.6–39.4); 61% female) had a lower VO₂ max (23.4 vs 45.6 ml/kg/min; p &lt; 0.001) and O₂P max (11.5 vs 19.1 ml/beat; p &lt; 0.001) compared to controls. Those with a FF occurring &lt;50% through exercise had a lower peak cardiac index and stroke volume, but not VO₂ max, compared to those &gt;50%. FF and O₂P curve area significantly correlated with peak cardiac index, stroke volume, left and right ventricular ejection fraction, and right ventricular systolic pressure.</p></div><div><h3>Conclusion</h3><p>CPET remains an integral part in the evaluation of rTOF. We introduce three non-invasive methods to assess exercise hemodynamics using the O₂P curve data. These evaluations demonstrated significant correlations with stroke volume, cardiac output, and right ventricular pressure.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"17 ","pages":"Article 100539"},"PeriodicalIF":0.8,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S266666852400048X/pdfft?md5=7b84ac15bd2ec21c11b5fb7d8a372d86&pid=1-s2.0-S266666852400048X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142095696","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}

{"title":"Exercise testing in clinical context: Reference ranges for interpreting anaerobic threshold as an outcome for congenital heart disease patients","authors":"Katherine Hansen ,&nbsp;Tracy Curran ,&nbsp;Lindsey Reynolds ,&nbsp;Catherine Cameron ,&nbsp;Jennifer Pymm ,&nbsp;Julie Ann O'Neill ,&nbsp;Rachel Losi ,&nbsp;Cara Sherman ,&nbsp;Elise Ackermans ,&nbsp;Suellen Yin ,&nbsp;Tajinder Singh ,&nbsp;Mark E. Alexander ,&nbsp;Kimberlee Gauvreau ,&nbsp;Naomi Gauthier","doi":"10.1016/j.ijcchd.2024.100540","DOIUrl":"10.1016/j.ijcchd.2024.100540","url":null,"abstract":"<div><h3>Background</h3><div>Change in the oxygen consumption (VO₂) at the ventilatory anaerobic threshold (VAT) is an important outcome in research studies of children with congenital heart disease (CHD). The range of values reported by different raters for any given VAT is needed to contextualize a change in VAT in intervention studies.</div></div><div><h3>Methods</h3><div>Sixty maximal cardiopulmonary exercise tests (CPET) for CHD patients 8–21 years old were independently reviewed by six exercise physiologists and four pediatric cardiologists. For each of the unique rater pairs for the 60 CPETs, the absolute difference in VAT was calculated and displayed on a histogram to demonstrate the distribution of inter-rater variability. This method was repeated for subgroups of test modality (cycle/treadmill), patient factors (diagnoses, exercise capacity), and rater factors (cardiologist/physiologist, years of experience).</div></div><div><h3>Results</h3><div>Rater agreement was good with an intraclass correlation coefficient of 0.79–0.91 but the distribution of differences was broad. The median difference was 2.7 % predicted peak VO₂ (60 mL/min, 1.0 mL/kg/min), the 75th percentile was 6.4 % (140 mL/min, 2.5 mL/kg/min), and the 95th percentile was 16.3 % (421 mL/min, 6.5 mL/kg/min). Distributions were similar for CPET modality and years of rater experience, but differed for other factors.</div></div><div><h3>Conclusions</h3><div>The baseline distribution of reported VAT is relatively broad, varied by units, and was not explained by differences in rater experience or test modality, but varies by patient factors. When evaluating clinical relevance, a change in the VO₂ at VAT in response to an intervention of &lt;6.5 % predicted falls within the majority (75th percentile) of expected variability and should be interpreted with caution.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"18 ","pages":"Article 100540"},"PeriodicalIF":0.8,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142322346","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}

{"title":"A national Australian Congenital Heart Disease registry; methods and initial results","authors":"Calum Nicholson ,&nbsp;Geoff Strange ,&nbsp;Julian Ayer ,&nbsp;Michael Cheung ,&nbsp;Leeanne Grigg ,&nbsp;Robert Justo ,&nbsp;Ryan Maxwell ,&nbsp;Gavin Wheaton ,&nbsp;Patrick Disney ,&nbsp;Deane Yim ,&nbsp;Simon Stewart ,&nbsp;Rachael Cordina ,&nbsp;David S. Celermajer","doi":"10.1016/j.ijcchd.2024.100538","DOIUrl":"10.1016/j.ijcchd.2024.100538","url":null,"abstract":"<div><h3>Background</h3><p>Although several National Data Registries for Congenital Heart Disease (CHD) exist, few are comprehensive and contemporary. A National Australian CHD Registry has been developed that aims to redress this by creating the first comprehensive data collection for CHD children and adults, initially across Australia.</p></div><div><h3>Methods</h3><p>We defined and collected a minimum dataset of demographics, diagnoses, and procedures from people with CHD presenting at participating quaternary CHD services Australia-wide. Data were collected from a range of clinical data sources. Diagnoses and procedures were standardised to the European Paediatric Congenital Code – Short List. Methodological limitations were carefully documented.</p></div><div><h3>Results</h3><p>From 8 participating institutions, an initial 359,084 patient records were assessed for eligibility and 68,234 unique individuals with structural CHD have been included in the current dataset. There were 20,395 (30 %) people with mild CHD, 25,157 (37 %) with moderate CHD, and 13,530 (20 %) with severe CHD (6 % unknown complexity). The most common diagnoses were Ventricular Septal Defect (16,781, 25 %), Atrial Septal Defect (6,607, 10 %), Aortic Valve Disorders (5516 8 %), Coarctation of the Aorta (5,321, 8 %), Tetralogy of Fallot (4,489, 7 %), Transposition of the Great Arteries (4,009, 6 %).</p></div><div><h3>Conclusion</h3><p>The data presented here represents the most comprehensive cohort collected for the Australian CHD population thus far and is comparable with the largest contemporary CHD registries around the world. This Registry represents a key resource for improved understanding of the CHD population and will drive better care and outcomes for people living with CHD.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"17 ","pages":"Article 100538"},"PeriodicalIF":0.8,"publicationDate":"2024-08-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000478/pdfft?md5=f7a86b652a555e39ec405957490f69c7&pid=1-s2.0-S2666668524000478-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142075884","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}