International journal of cardiology. Congenital heart disease最新文献

{"title":"Novel immunologic mechanisms for Fontan-associated liver disease","authors":"Austin Angelotti ,&nbsp;Maninder Dhesi ,&nbsp;Shyam S. Bansal ,&nbsp;Elisa A. Bradley","doi":"10.1016/j.ijcchd.2024.100554","DOIUrl":"10.1016/j.ijcchd.2024.100554","url":null,"abstract":"<div><h3>Introduction</h3><div>Single ventricle congenital heart disease resulting in Fontan palliation has led to improved survival, however, Fontan-associated liver disease (FALD) is ubiquitous in this population by adulthood. While lymphopenia has been associated with the degree of FALD, potential immunologic mechanisms remain unstudied, and were the focus of this study.</div></div><div><h3>Methods</h3><div>Single-nuclei RNA-seq (snRNA-seq) data from liver samples of adolescent Fontan and control patients were analyzed with specific focus on lymphocytes and natural killer (NK) and T-cell fractions.</div></div><div><h3>Results</h3><div>Liver samples from Fontan patients demonstrated upregulation of endothelial cells (ECs: 4.2 ± 1.0 vs. 13.6 ± 3.4 %, p = 0.037) and total lymphocytes (0.7 ± 0.1 vs. 3.6 ± 0.7 %, p = 0.007), more specifically in NK and T-cells (NK: 0.29 ± 0.16 vs. 1.40 ± 0.64 %, p = 0.028 and T-cell: 0.28 ± 0.04 vs. 1.80 ± 1.01 %, p = 0.034). Enhanced genes important in T-cell activation and differentiation were demonstrated, as well as those involved in cell-to-cell adhesion and lymphocyte migration. Supporting lymphocyte trafficking, ECs demonstrated amplification of critical chemotactic and lymphocyte recruitment genes. Increased time from Fontan palliation was associated with more dramatic lymphocytic transcriptomic changes.</div></div><div><h3>Conclusions</h3><div>Hepatic changes in adolescent Fontan patients suggest that T-cells are contributing to the early development and possible progression of FALD.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100554"},"PeriodicalIF":0.8,"publicationDate":"2024-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143163929","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":"Concurrent finding of rheumatic severe mitral regurgitation and anomalous left coronary artery from the pulmonary artery (ALCAPA) in a 23-year-old patient consulting for ventricular tachycardia: A case report and a review of the literature","authors":"Josu Erquicia Peralt ,&nbsp;Larraitz Orive Melero ,&nbsp;Jagoba Larrazabal López ,&nbsp;José Félix Larrea Egurbide ,&nbsp;Eugenia García Fernández ,&nbsp;Luis Fernández González ,&nbsp;Roberto Blanco Mata ,&nbsp;Josune Arriola Meabe","doi":"10.1016/j.ijcchd.2024.100553","DOIUrl":"10.1016/j.ijcchd.2024.100553","url":null,"abstract":"<div><h3>Introduction</h3><div>Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease that in most cases manifests in the first months of life, being a well-studied entity of myocardial ischaemia in young patients. It has been reported that up to 90 % of infants die without treatment in the first year, although late diagnosis is increasingly reported in adults.</div></div><div><h3>Case summary</h3><div>We present the case of a 23-year-old woman of Moroccan origin with no medical history of interest, who was admitted to the cardiac intensive care unit after an episode of sustained ventricular tachycardia (VT) that required electrical cardioversion. On admission, the electrocardiogram and blood test were normal, but the transthoracic echocardiogram showed moderate left ventricular systolic dysfunction and severe mitral regurgitation of rheumatic aetiology which was later confirmed with a transesophageal echocardiogram. In view of the arrhythmic event presented, a complete study was performed with cardiac magnetic resonance imaging (MRI) which showed no pathological enhancements. Nevertheless, coronary artery angiography revealed an anomalous origin of the left coronary artery in the pulmonary artery which was confirmed by cardiac computed tomography (CT). Corrective surgery was performed with direct translocation of the left coronary artery to the aorta and mitral valve replacement.</div></div><div><h3>Discussion</h3><div>In ALCAPA, the coronary steal phenomenon produced from the pulmonary artery to the coronary circulation causes myocardial ischaemia and involves a left-to-right shunt. Myocardial ischaemia is cause of angina, systolic dysfunction, mitral regurgitation and sudden cardiac death in children and young adults.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100553"},"PeriodicalIF":0.8,"publicationDate":"2024-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142759308","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":"Worldwide prevalence of heart failure due to congenital heart disease: An analysis from the Global Burden of Disease Study 2021","authors":"Ahmed Kheiwa ,&nbsp;Inhae Baek ,&nbsp;Ian S. Harris ,&nbsp;Abdul Mannan Khan Minhas ,&nbsp;Dmitry Abramov","doi":"10.1016/j.ijcchd.2024.100552","DOIUrl":"10.1016/j.ijcchd.2024.100552","url":null,"abstract":"<div><h3>Background</h3><div>There are limited data on the prevalence of heart failure (HF) due to congenital heart diseases (CHD).</div></div><div><h3>Methods</h3><div>The Global Burden of Disease (GBD) dataset was used to analyze the disease burden of HF due to CHD between 1990 and 2021. Age-standardized rates (ASR) (crude prevalence rates for age groups) and total percent change for the overall worldwide burden as well as among subgroups based on age and geographic regions were determined.</div></div><div><h3>Results</h3><div>The global prevalence of HF due to CHD in 1990 and 2021 was 2,494,547 (95 % IU 2,054,729 to 3,030,909) and 3,155,991 (95 % IU 2,578,552 to 3,843,062) individuals respectively, with an increase in ASR from 41.02 (33.79–49.79) to 45.33 (37.15–55.17) per 100,000 individuals during that period. HF due to CHD in 2021 was most common in children aged 2–4, crude rate of 248.44 (195.99–302.57), followed by children &lt;1 years of age, crude rate of 142.75 (116.87–174.26), and children aged 5–14, crude rate of 89.55 (62.35–129.61). During the study period, there was an increase in the prevalence of HF due to CHD among most age groups, other than children &lt;1 year of age who had stable rates and individuals &gt;70 who had no reported prevalence. There were geographic differences in the prevalence and trends of HF due to CHD.</div></div><div><h3>Conclusions</h3><div>There are differences in prevalence and trends of HF due to CHD among age groups and worldwide regions. These results demonstrate the worldwide burden and trends of HF due to CHD.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100552"},"PeriodicalIF":0.8,"publicationDate":"2024-11-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143163928","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":"Patent foramen ovale-induced retinal artery occlusion: Evidence for in situ Thromobosis","authors":"Junfang Shi ,&nbsp;Baiqiang Wang ,&nbsp;Weida Lu ,&nbsp;Xiao Meng","doi":"10.1016/j.ijcchd.2024.100551","DOIUrl":"10.1016/j.ijcchd.2024.100551","url":null,"abstract":"<div><h3>Background</h3><div>To report one young man suffered from retinal artery occlusion (RAO) due to patent foramen ovale (PFO).</div></div><div><h3>Objective</h3><div>The aim of this study was to assess the mechanism for RAO in a patient with a PFO.</div></div><div><h3>Method</h3><div>A 35-year-old male who developed RAO was studied. He received transoesophageal echocardiography and a right-to-left shunt across of a PFO was found. Then, the patient was scheduled for percutaneous PFO closure. An optical coherence tomography (OCT) catheter was positioned into the PFO tunnel and the microstructure of PFO was observed. We found that there were several white thrombi in the tunnel; the PFO was successfully closed. At 10-month follow-up, the patient recovered well and had no new retinal artery occlusion or stroke.</div></div><div><h3>Conclusions</h3><div>PFO was the site of thrombus formation in our case of RAO as confirmed by OCT. PFO should be considered in young patients diagnosed with RAO with no other risk factors.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"19 ","pages":"Article 100551"},"PeriodicalIF":0.8,"publicationDate":"2024-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143163418","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":"Low birth weight associations with cardiac structure and function in adults after arterial switch for transposition of the great arteries","authors":"Art Schuermans ,&nbsp;Béatrice Santens ,&nbsp;Jef Van den Eynde ,&nbsp;Xander Jacquemyn ,&nbsp;Roel L.F. Van der Palen ,&nbsp;Michael C. Honigberg ,&nbsp;Alexander Van De Bruaene ,&nbsp;Piet Claus ,&nbsp;Jan Bogaert ,&nbsp;Werner Budts","doi":"10.1016/j.ijcchd.2024.100550","DOIUrl":"10.1016/j.ijcchd.2024.100550","url":null,"abstract":"<div><h3>Background and aims</h3><div>The objective of this study was to assess the associations of birth weight with cardiac structure and function in adults with dextro-transposition of the great arteries (D-TGA) who underwent the arterial switch operation (ASO).</div></div><div><h3>Methods and results</h3><div>Thirty-nine ASO patients (age 24.4 ± 3.3 years) were included during routine clinical follow-up from July 2019 to December 2021. All patients underwent cardiopulmonary exercise testing and cardiac magnetic resonance imaging at rest and during exercise. Early-life characteristics, including birth weight, were extracted from electronic medical health records. Linear regression analysis showed that lower birth weight was associated with smaller left ventricular (LV) and right ventricular (RV) end-diastolic volume index (LV: −14.5 mL/m² [95 % confidence interval, CI: −26.5 to −2.5] per 1-kg decrease in birth weight, <em>p</em> = 0.04; RV: −11.2 mL/m² [-20.7 to −1.7] per 1-kg decrease in birth weight, <em>p</em> = 0.03). Lower birth weight was associated with greater LV and RV ejection fraction at rest (LV: +8.5 % [+4.4 to +12.5] per 1-kg decrease in birth weight, <em>p</em> &lt; 0.001); RV: +8.1 % [+2.8 to +13.4] per 1-kg decrease in birth weight, <em>p</em> = 0.005). Furthermore, lower birth weight was associated with an attenuated increase in LV stroke volume index from rest to peak exercise (−5.2 mL/m² [-9.3 to −1.2] per 1-kg decrease in birth weight, <em>p</em> = 0.02).</div></div><div><h3>Conclusions</h3><div>Birth weight may be a novel risk factor for adverse cardiac remodeling in adult ASO patients. Further research is needed to delineate the mechanisms underlying the associations between birth weight and cardiac remodeling ASO patients as well as the broader adult CHD population.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"18 ","pages":"Article 100550"},"PeriodicalIF":0.8,"publicationDate":"2024-10-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142538529","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":"Segmental MRI pituitary and hypothalamus volumes post Fontan: An analysis of the Australian and New Zealand Fontan registry","authors":"Waverley Gee ,&nbsp;Joseph Yuan-Mou Yang ,&nbsp;Tom Gentles ,&nbsp;Sonja Bastin ,&nbsp;Ajay J. Iyengar ,&nbsp;Jian Chen ,&nbsp;Dug Yeo Han ,&nbsp;Rachael Cordina ,&nbsp;Charlotte Verrall ,&nbsp;Craig Jefferies ,&nbsp;The Australian and New Zealand Fontan Registry","doi":"10.1016/j.ijcchd.2024.100549","DOIUrl":"10.1016/j.ijcchd.2024.100549","url":null,"abstract":"<div><h3>Objective</h3><div>Short stature, central hypothyroidism and infertility are common in those with a Fontan circulation. Given that the Fontan circulation often results in hepatic portal venous congestion, we hypothesize that the hypothalamic-pituitary portal circulation is also affected, contributing to subsequent hypothalamic-pituitary axis dysfunction.</div></div><div><h3>Methods</h3><div>MRI data from the Australian and New Zealand Fontan Registry (86 cases) was compared to 86 age- and sex-matched normal published controls. Total pituitary volumes (both anterior and posterior glands) were measured using a manual tracing segmentation method, and hypothalamic (and subunit) volumes using an automated segmentation tool. Measured gland volume was normalized to total brain volumes. A generalized linear model was used for statistical analysis.</div></div><div><h3>Results</h3><div>Normalized total pituitary volumes (nTPV) were increased in Fontan patients compared to controls (<em>p</em> &lt; 0.0001), due to an increase in anterior pituitary volumes (nAPV) (<em>p</em> &lt; 0.0001), with no difference in normalized posterior pituitary volumes (<em>p</em> = 0.7). Furthermore, normalized anterior and tubular hypothalamic subunit groups) were increased in Fontan patients compared to the controls (<em>p</em> &lt; 0.01 and <em>p</em> &lt; 0.0001, respectively).</div><div>The time between Fontan and MRI was positively related to nTPV, nAPV and bilateral hypothalamic volumes. nTPV increased with age, and the increase in nAPV was greater in Fontan patients.</div></div><div><h3>Conclusions</h3><div>Segmental MRI Pituitary and Hypothalamus volumes post Fontan are increased and are related to the time since Fontan procedure. These findings are consistent with venous congestion of the anterior hypothalamic-pituitary portal venous system and may explain the high frequency of endocrine dysfunction in this patient group.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"18 ","pages":"Article 100549"},"PeriodicalIF":0.8,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142553351","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":"Infective endocarditis: Awareness, knowledge gaps and behaviours amongst adults with congenital heart disease","authors":"S. Haider ,&nbsp;K. Krishanthasan ,&nbsp;I. Olakorede ,&nbsp;A. Constantine ,&nbsp;I. Rafiq ,&nbsp;K. Dimopoulos","doi":"10.1016/j.ijcchd.2024.100548","DOIUrl":"10.1016/j.ijcchd.2024.100548","url":null,"abstract":"<div><h3>Background</h3><div>Adults with congenital heart disease (ACHD) have an increased risk of infective endocarditis (IE), associated with significant morbidity and mortality. This risk is compounded by patient-related factors, including lack of awareness of IE and the presence of a learning disability (LD). Our study sought to evaluate patients' understanding of the risks and symptoms of IE and to identify patients who could benefit from targeted education.</div></div><div><h3>Methods</h3><div>Patients attending the outpatient department of a tertiary ACHD referral centre completed a questionnaire that assessed their knowledge, attitudes and behaviours towards IE. Baseline demographics and clinical data were collected from electronic patient records.</div></div><div><h3>Results</h3><div>A total of 132 ACHD patients completed the questionnaires (age 41.7 ± 16.4 years, 50 % male, 11 % with an LD). Only 37.1 % of patients accurately defined IE, none of whom had an LD. Most patients chose pyrexia (47 %) and tiredness (39.4 %) as potential symptoms of IE, however, none correctly identified all symptoms. Only 19.7 % were aware of the requirement for prolonged antibiotic treatment for IE. A third of all patients reported that they would have made lifestyle changes had they been aware of the complications of IE. There was a statistically significant association between learning disability and poor level of awareness in the questionnaire.</div></div><div><h3>Conclusions</h3><div>Our study demonstrates awareness issues regarding IE among ACHD patients, highlighting the need to invest further on patient education. This should start at the time of transition from paediatric to adult services and continue lifelong, with emphasis on patients with a learning disability.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"18 ","pages":"Article 100548"},"PeriodicalIF":0.8,"publicationDate":"2024-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142527270","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":"Comparative analysis of diagnostic accuracy in adult congenital heart disease: A study of three physician groups and ChatGPT","authors":"Edward Itelman,&nbsp;Einat Birk,&nbsp;Ayelet Machtei,&nbsp;Rafael Hirsch,&nbsp;Nili Schamroth Pravda","doi":"10.1016/j.ijcchd.2024.100547","DOIUrl":"10.1016/j.ijcchd.2024.100547","url":null,"abstract":"<div><div>This research letter evaluates the diagnostic accuracy of three physician groups—pediatric cardiologists, adult congenital heart disease (ACHD) specialists, and general cardiologists—and compares it to the performance of an AI (Artificial Intelligence) LLM model. Based on eight targeted questions in adult congenital heart disease, the analysis highlights significant disparities in correct answer rates, with congenital specialists achieving the highest accuracy and the AI model – the lowest.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"18 ","pages":"Article 100547"},"PeriodicalIF":0.8,"publicationDate":"2024-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142440888","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":"Oxygen uptake efficiency slope at anaerobic threshold can predict peak VO2 in adult congenital heart disease","authors":"Thomas Simon FitzMaurice ,&nbsp;Scott Hawkes ,&nbsp;Yuen Liao ,&nbsp;Damien Cullington ,&nbsp;Angella Bryan ,&nbsp;James Redfern ,&nbsp;Reza Ashrafi","doi":"10.1016/j.ijcchd.2024.100546","DOIUrl":"10.1016/j.ijcchd.2024.100546","url":null,"abstract":"<div><h3>Introduction</h3><div>Assessment of exercise capacity by cardiopulmonary exercise testing (CPET) in adults with congenital heart disease (CHD) is important for prognostication and preoperative assessment. Peak oxygen uptake (PVO₂) is used commonly, but can be challenging due to the difficulties of undertaking maximal CPET testing in this population. We explored whether oxygen uptake efficiency slope (OUES) at ventilatory anaerobic threshold (VAT), the point during CPET at which OUES becomes strongly correlated with PVO₂, and is more reliably available from submaximal CPET, can predict PVO₂ in adults with CHD.</div></div><div><h3>Methods</h3><div>We assessed consecutive individuals who completed maximal CPET at our cardiorespiratory centre, as part of routine service review, between March 2019 and August 2021, recording data such as PVO₂, VAT and OUES at various proportions of a maximal test (75 %, 90 %, 100 %, and VAT). We employed linear regression modelling to analyse the association between PVO₂ and OUES at VAT, and subsequently create an equation to predict PVO₂ from OUES at VAT. Parametric data are presented using Pearson's correlation coefficient and non-parametric data using Spearman's rho.</div></div><div><h3>Results</h3><div>We analysed 391 individuals (177 female, age 32 ± 11 years). Mean ± SD PVO₂ was 23.3 ± 6.86 ml/min/kg or 1724 ± 540 ml/min, peak VE 86.7 ± 25.4 l/min. The point of VAT as a percentage of PVO₂ achieved was 66.5 ± 9.4 %, and VAT as a percentage of predicted PVO₂ 46.9 ± 11.4 %. PVO₂ was correlated with OUES at 100 % (R = 0.91, P &lt; .001), 90 % (R = 0.91, P &lt; .001), 75 % (R = 0.89, P &lt; .001) of maximum, and VAT (R = 0.83, P &lt; .001). PVO₂ <em>(ml/min)</em> could be predicted by: <em>(OUES at VAT)∗685.245 + (BMI [kg/m</em>^<em>2</em><em>])∗5.045 + (FEV</em>_<em>1</em> <em>[l])∗223.620 – 153.205</em>.</div></div><div><h3>Conclusions</h3><div>OUES at VAT can be used to calculate PVO₂. To our knowledge, this is the first equation using OUES at VAT to predict PVO₂ in adults with CHD. In a population who may find maximal CPET difficult, this may be a useful submaximal measurement of cardiovascular fitness, and to calculate PVO₂, which is commonly used in guideline-based decision making in CHD.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"18 ","pages":"Article 100546"},"PeriodicalIF":0.8,"publicationDate":"2024-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142418070","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 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}