{"title":"Exercise testing in clinical context: Reference ranges for interpreting anaerobic threshold as an outcome for congenital heart disease patients","authors":"","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 (VO<sub>2</sub>) 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 VO<sub>2</sub> (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 VO<sub>2</sub> at VAT in response to an intervention of <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":null,"pages":null},"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":"","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":null,"pages":null},"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}
{"title":"Assessing subaortic right ventricle function after atrial switch repair through myocardial work analysis","authors":"","doi":"10.1016/j.ijcchd.2024.100537","DOIUrl":"10.1016/j.ijcchd.2024.100537","url":null,"abstract":"<div><h3>Background</h3><div>The Mustard and Senning operations for dextro-transposition of the great arteries (D-TGA) establish a biventricular physiology with a subaortic right ventricle (sRV). While prolonged QRS has been associated with worse prognosis in these patients, current echocardiographic tools fall short in adequately assessing the (mal)performance and function decline of the sRV during follow-up. The present study is the first to characterize Myocardial Work (MW) indices of the sRV in D-TGA patients after Mustard/Senning repair.</div></div><div><h3>Methods and results</h3><div>All adult D-TGA patients at follow-up in the University Hospital of Leuven between 2018 and 2022 were screened for inclusion. We retrospectively collected the most recent electrocardiogram parameters, 2D echocardiographic images and serum biomarkers’ values. Offline calculations of MW indices were performed. We established correlations between all variables and categorized patients into QRS<120 ms and QRS≥120 ms for further analysis. A total of 51 D-TGA patients were included (13 Mustard, 33 male, 39 ± 6 years). QRS duration increased with age and was correlated with sRV dimensions and serum levels of troponins (R = 0.42, p < 0.01) and NT-proBNP (R = 0.31, p = 0.03). However, no significant correlation was found between QRS duration and intraventricular desynchrony or conventional functional echocardiographic parameters. QRS prolongation was associated with a deterioration in septal, but not lateral, MW parameters. Patients with QRS≥120 ms had significantly larger ventricles and higher levels of troponins and NT-proBNP.</div></div><div><h3>Conclusion</h3><div>QRS prolongation after Mustard/Senning repair is linked to ventricular dilatation and worse performance, particularly affecting the septal wall. Global and regional MW analysis may be useful to assess the subclinical deterioration of sRV function.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142320251","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":"Imaging in chronic thromboembolic pulmonary disease: Current practice and advances","authors":"","doi":"10.1016/j.ijcchd.2024.100536","DOIUrl":"10.1016/j.ijcchd.2024.100536","url":null,"abstract":"<div><p>Chronic thromboembolic pulmonary disease (CTEPD) with or without pulmonary hypertension (PH) occurs when thromboemboli in pulmonary arteries fail to resolve completely. Pulmonary artery obstructions due to chronic thrombi and secondary microvasculopathy can increase pulmonary arterial pressure and resistance leading to chronic thromboembolic PH (CTEPH). Mechanical interventions and/or PH medications can improve cardiopulmonary haemodynamic, alleviate symptoms, and decrease mortality risk. Imaging is pivotal throughout the CTEPD management journey, spanning diagnosis, treatment planning, and assessing treatment outcome. With just computed tomography (CT) pulmonary angiogram and right heart catheterisation, an experienced multidisciplinary team can determine surgical candidacy in most cases. Dual energy CT, lung subtraction iodine mapping CT, and dynamic contrast-enhanced magnetic resonance imaging (MRI) offer comparable sensitivities with ventilation-perfusion scintigraphy in diagnosing CTEPD. Pulmonary angiogram with digital subtraction angiography although considered the gold standard for assessing thrombi extent and vasculature morphology is now mostly used to assess targets for balloon pulmonary angioplasty. Advancements in CT modalities and innovative MRI metrics offer better insight into CTEPD management but are limited by the availability of technology and expertise. Learning from current artificial intelligence application in medical imaging, there is promise in tapping the wealth of data provided by CTEPD imaging through automating cardiopulmonary and vascular morphology analysis.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000454/pdfft?md5=00d52e64d87991bb3e2bcda91db0bbb9&pid=1-s2.0-S2666668524000454-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141991426","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":"The latest definition and classification of pulmonary hypertension","authors":"","doi":"10.1016/j.ijcchd.2024.100534","DOIUrl":"10.1016/j.ijcchd.2024.100534","url":null,"abstract":"<div><p>Pulmonary hypertension (PH) is a serious potential complication of some congenital heart diseases (CHDs). PH encompasses a range of diseases which may be idiopathic or inherited, or secondary to cardiac, respiratory, systemic or thromboembolic conditions, amongst others. Our increasing understanding of the normal ranges of pulmonary haemodynamics, as well as evidence supporting the benefits of early treatment, has resulted in a number of recent revisions to the haemodynamic definition of PH. In this Review Article, we report on the recent updates to haemodynamic definitions and classification of PH, as reflected in the 2022 Pulmonary Hypertension Guidelines and particularly focus on the CHD related sub-type of PH, where the aetiology is often multi-factorial.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000430/pdfft?md5=2f474a94fabfc36da592cb9b263a8b26&pid=1-s2.0-S2666668524000430-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141962033","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":"Subpulmonary ventricular function and inflammation are related to clinical heart failure in patients with a systemic right ventricle","authors":"","doi":"10.1016/j.ijcchd.2024.100535","DOIUrl":"10.1016/j.ijcchd.2024.100535","url":null,"abstract":"<div><h3>Background</h3><p>Timely diagnosis of heart failure (HF) in patients with a systemic right ventricle (sRV) is difficult but important since clinical deterioration is fast once HF develops. We aimed to compare echocardiography and biomarker profile between sRV patients with and without HF and patients with a systemic left ventricle diagnosed with HF (sLV-HF).</p></div><div><h3>Methods and results</h3><p>Eighty-seven sRV patients and 30 sLV-HF patients underwent echocardiographic evaluation and blood sampling. Compared to sRV patients without HF, sRV-HF patients had more remodeling of the subpulmonary LV (spLV) (internal diameter 3.9 cm [3.3–5.7] vs 3.4 cm [2.9–3.9], P = 0.03, posterior wall 0.93 cm [0.76–1.20] vs 0.71 cm [0.59–0.91], P = 0.006) and lower spLV systolic function: ejection fraction (59 % ± 14 vs 70 % ± 10, P = 0.011), mitral annular plane systolic excursion (1.7 cm ± 0.5 vs 2.1 cm ± 0.4, P = 0.003), fractional area change (47 % [38–58] vs 59 % [51–70], P = 0.002) and lateral strain rate (−1.2/s ± 0.46 vs −1.5/s ± 0.39, P = 0.016). Inflammatory biomarkers were higher in sRV-HF patients compared to those without HF: red cell distribution width (13.3 fL [12.8–14.1] vs 12.6 fL [12.3–13.1], P < 0.001), neutrophil lymphocyte ratio (NLR, 3.7 [2.2–4.9] vs 2.4 [1.9–3.0], P = 0.015), C-reactive protein (CRP, 2.5 mg/dL [1.0–4.2] vs 1.2 mg/dL [0.0–2.0], P = 0.005) and compared to sLV-HF patients (NLR (3.7 [2.2–4.9] vs 2.5 [1.7–3.3], P = 0.044) and CRP (2.5 mg/dL [1.0–4.2] vs 0.85 mg/dL [0.6–2.0], P = 0.006).</p></div><div><h3>Conclusion</h3><p>Biventricular echocardiographic evaluation with a focus on the subpulmonary LV together with assessing inflammatory status in sRV patients could help in an earlier detection of HF.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000442/pdfft?md5=98391a18df1fea3bb58f2b154788fef1&pid=1-s2.0-S2666668524000442-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141991425","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":"Outcomes, mortality risk factors, and functional status post-Norwood: A single-center study","authors":"","doi":"10.1016/j.ijcchd.2024.100533","DOIUrl":"10.1016/j.ijcchd.2024.100533","url":null,"abstract":"<div><h3>Background</h3><p>The Norwood operation (NO) for infants with univentricular physiology has high interstage mortality. This study evaluated outcomes and risk factors for mortality following NO.</p></div><div><h3>Methods</h3><p>Retrospective single-center study of patients undergoing NO from 2010 to 2020. Analysis used appropriate statistics.</p></div><div><h3>Results</h3><p>Of 269 patients undergoing NO, 213 (79.2 %) survived to discharge. Non-survivors had longer bypass times, delayed sternal closure, required nitric oxide, higher vasoactive scores, required post-operative catheterization, Extracorporeal Life Support (ECLS), and longer ventilation (p < 0.05). Logistic regression showed moderate-severe atrioventricular valve regurgitation on intraoperative TEE (OR 2.6), requiring nitric oxide (OR 2.63), delayed sternal closure (OR 2.94), post-operative catheterization (OR 10.48), and ECLS (OR 14.54) increased mortality odds (p < 0.05). Multivariable analysis confirmed catheterization (aOR 10.48) and ECLS (aOR 14.54) as significant predictors. Of survivors, 26 (12.3 %) developed new morbidity, 9 (4.2 %) had unfavorable outcomes. Functional status improved from 6.0 to 8.04, mainly in feeding and respiratory domains (p < 0.0001).</p></div><div><h3>Conclusions</h3><p>Norwood survival was 79.2 %. Requiring post-operative catheterization and ECLS significantly increased mortality risk. Multicenter evaluation of these modifiable risk factors is needed to improve outcomes in this high-risk population.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000429/pdfft?md5=e08ecc7ded54dee64f3ee71a698dc086&pid=1-s2.0-S2666668524000429-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141852509","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":"Congenital heart defects and consanguinity: An analysis of the Sidra cardiac registry data in Qatar","authors":"","doi":"10.1016/j.ijcchd.2024.100529","DOIUrl":"10.1016/j.ijcchd.2024.100529","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000387/pdfft?md5=01a3dbe06445fd5b7d931a8f94fef8a3&pid=1-s2.0-S2666668524000387-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141848881","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}