Pub Date : 2024-10-23DOI: 10.1016/j.ijcchd.2024.100550
Art Schuermans , Béatrice Santens , Jef Van den Eynde , Xander Jacquemyn , Roel L.F. Van der Palen , Michael C. Honigberg , Alexander Van De Bruaene , Piet Claus , Jan Bogaert , Werner Budts
Background and aims
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).
Methods and results
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, p = 0.04; RV: −11.2 mL/m2 [-20.7 to −1.7] per 1-kg decrease in birth weight, p = 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, p < 0.001); RV: +8.1 % [+2.8 to +13.4] per 1-kg decrease in birth weight, p = 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, p = 0.02).
Conclusions
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.
{"title":"Low birth weight associations with cardiac structure and function in adults after arterial switch for transposition of the great arteries","authors":"Art Schuermans , Béatrice Santens , Jef Van den Eynde , Xander Jacquemyn , Roel L.F. Van der Palen , Michael C. Honigberg , Alexander Van De Bruaene , Piet Claus , Jan Bogaert , 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/m<sup>2</sup> [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/m<sup>2</sup> [-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> < 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/m<sup>2</sup> [-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}
Pub Date : 2024-10-22DOI: 10.1016/j.ijcchd.2024.100549
Waverley Gee , Joseph Yuan-Mou Yang , Tom Gentles , Sonja Bastin , Ajay J. Iyengar , Jian Chen , Dug Yeo Han , Rachael Cordina , Charlotte Verrall , Craig Jefferies , The Australian and New Zealand Fontan Registry
Objective
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.
Methods
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.
Results
Normalized total pituitary volumes (nTPV) were increased in Fontan patients compared to controls (p < 0.0001), due to an increase in anterior pituitary volumes (nAPV) (p < 0.0001), with no difference in normalized posterior pituitary volumes (p = 0.7). Furthermore, normalized anterior and tubular hypothalamic subunit groups) were increased in Fontan patients compared to the controls (p < 0.01 and p < 0.0001, respectively).
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.
Conclusions
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.
{"title":"Segmental MRI pituitary and hypothalamus volumes post Fontan: An analysis of the Australian and New Zealand Fontan registry","authors":"Waverley Gee , Joseph Yuan-Mou Yang , Tom Gentles , Sonja Bastin , Ajay J. Iyengar , Jian Chen , Dug Yeo Han , Rachael Cordina , Charlotte Verrall , Craig Jefferies , 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> < 0.0001), due to an increase in anterior pituitary volumes (nAPV) (<em>p</em> < 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> < 0.01 and <em>p</em> < 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}
Pub Date : 2024-10-18DOI: 10.1016/j.ijcchd.2024.100548
S. Haider , K. Krishanthasan , I. Olakorede , A. Constantine , I. Rafiq , K. Dimopoulos
Background
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.
Methods
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.
Results
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.
Conclusions
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.
{"title":"Infective endocarditis: Awareness, knowledge gaps and behaviours amongst adults with congenital heart disease","authors":"S. Haider , K. Krishanthasan , I. Olakorede , A. Constantine , I. Rafiq , 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}
Pub Date : 2024-10-05DOI: 10.1016/j.ijcchd.2024.100547
Edward Itelman, Einat Birk, Ayelet Machtei, Rafael Hirsch, Nili Schamroth Pravda
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.
{"title":"Comparative analysis of diagnostic accuracy in adult congenital heart disease: A study of three physician groups and ChatGPT","authors":"Edward Itelman, Einat Birk, Ayelet Machtei, Rafael Hirsch, 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}
Pub Date : 2024-09-29DOI: 10.1016/j.ijcchd.2024.100546
Thomas Simon FitzMaurice , Scott Hawkes , Yuen Liao , Damien Cullington , Angella Bryan , James Redfern , Reza Ashrafi
Introduction
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.
Methods
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.
Results
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 < .001), 90 % (R = 0.91, P < .001), 75 % (R = 0.89, P < .001) of maximum, and VAT (R = 0.83, P < .001). PVO2(ml/min) could be predicted by: (OUES at VAT)∗685.245 + (BMI [kg/m2])∗5.045 + (FEV1[l])∗223.620 – 153.205.
Conclusions
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.
{"title":"Oxygen uptake efficiency slope at anaerobic threshold can predict peak VO2 in adult congenital heart disease","authors":"Thomas Simon FitzMaurice , Scott Hawkes , Yuen Liao , Damien Cullington , Angella Bryan , James Redfern , 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 (PVO<sub>2</sub>) 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 PVO<sub>2</sub>, and is more reliably available from submaximal CPET, can predict PVO<sub>2</sub> 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 PVO<sub>2</sub>, 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 PVO<sub>2</sub> and OUES at VAT, and subsequently create an equation to predict PVO<sub>2</sub> 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 PVO<sub>2</sub> 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 PVO<sub>2</sub> achieved was 66.5 ± 9.4 %, and VAT as a percentage of predicted PVO<sub>2</sub> 46.9 ± 11.4 %. PVO<sub>2</sub> was correlated with OUES at 100 % (R = 0.91, P < .001), 90 % (R = 0.91, P < .001), 75 % (R = 0.89, P < .001) of maximum, and VAT (R = 0.83, P < .001). PVO<sub>2</sub> <em>(ml/min)</em> could be predicted by: <em>(OUES at VAT)∗685.245 + (BMI [kg/m</em><sup><em>2</em></sup><em>])∗5.045 + (FEV</em><sub><em>1</em></sub> <em>[l])∗223.620 – 153.205</em>.</div></div><div><h3>Conclusions</h3><div>OUES at VAT can be used to calculate PVO<sub>2</sub>. To our knowledge, this is the first equation using OUES at VAT to predict PVO<sub>2</sub> 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 PVO<sub>2</sub>, 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}
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.
Methods
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, >1 year after start of DOAC.
Results
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.
Conclusions
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.
{"title":"A new strategy for monitoring of direct oral anticoagulants in patients with cyanotic and complex congenital heart disease","authors":"Fabienne Dirbach , Eleni Goulouti , Judith Bouchardy , Magalie Ladouceur , Lorenzo Alberio , 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, >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}
Pub Date : 2024-09-19DOI: 10.1016/j.ijcchd.2024.100543
Julia Claire Cambron , Evan F. Shalen , Lidija B. McGrath , Katrina Ramsey , Abigail Khan
Introduction
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.
Methods
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.
Results
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 < 0.0001), treatment for drug and alcohol use (25.0 % vs. 7.2 %; p < 0.0001), and mental health diagnoses (66.6 % vs. 51.0 %; p < 0.0001). They were more likely to reside in a rural area (24.5 % vs. 16.1 %; p < 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 < 0.0001) and rates of 30-day readmission (17.1 % vs. 11.0 %, p < 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).
Conclusions
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.
{"title":"Association between insurance type, clinical characteristics, and healthcare use in adults with congenital heart disease","authors":"Julia Claire Cambron , Evan F. Shalen , Lidija B. McGrath , Katrina Ramsey , 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 < 0.0001), treatment for drug and alcohol use (25.0 % vs. 7.2 %; p < 0.0001), and mental health diagnoses (66.6 % vs. 51.0 %; p < 0.0001). They were more likely to reside in a rural area (24.5 % vs. 16.1 %; p < 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 < 0.0001) and rates of 30-day readmission (17.1 % vs. 11.0 %, p < 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}
Pub Date : 2024-09-18DOI: 10.1016/j.ijcchd.2024.100544
Radoslaw Debiec , Armia Ebeid , Stephen Hamby , Odeta Anciunaite , Anne Illsley , Ali Nizam , Madiha Iqbal , Kassem Safwan , Tariq Saifullah , Frances Bu’Lock , Toru Suzuki , Nilesh J. Samani , Tom Webb , Aidan P. Bolger
Background
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.
Methods
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.
Results
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: MYLK (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.
Conclusions
The presence of a rare genetic variant in MYLK is a plausible explanation for the clinical presentation. The variant will need further verification to confirm pathogenicity. Pathogenic MYLK variants have been previously reported in context of dissection with minimally dilated aortas.
背景主动脉夹层（TAD）是法洛四联症（TOF）患者中一种不常见的并发症。方法我们报告了一例斯坦福A型TAD病例，患者是一名曾接受过TOF修复手术的女性患者。我们进行了全外显子测序（WES）；英国 Novogene 公司、Agilent V6 捕获试剂盒、Illumina HiSeq 100x 深度），以确定已知与 TAD 相关的基因变异。在NCBI PubMed数据库中进行了系统的文献综述，以确定有关TOF患者TAD的病例报告。结果患者是一名31岁的女性，在4岁时接受了TOF修复手术，并出现了斯坦福A型主动脉夹层。在进行 TAD 前 16 个月的临床复查中，胸主动脉仅有轻微扩张（瓦尔萨尔瓦窦 43 毫米）。值得注意的是，患者在夹层发生前5个月已完成妊娠。患者没有其他易患 TAD 的高危特征。WES 发现了以前与 TAD 相关的基因中的罕见遗传变异：MYLK (p.Arg1405His)。文献综述还发现了其他九例关于TOF患者TAD的病例报告。结论MYLK中存在一个罕见的基因变异是临床表现的一个合理解释。该变体需要进一步验证，以确认其致病性。致病性MYLK变体以前曾在主动脉微扩张的夹层中出现过。
{"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 , Armia Ebeid , Stephen Hamby , Odeta Anciunaite , Anne Illsley , Ali Nizam , Madiha Iqbal , Kassem Safwan , Tariq Saifullah , Frances Bu’Lock , Toru Suzuki , Nilesh J. Samani , Tom Webb , 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}
Pub Date : 2024-09-12DOI: 10.1016/j.ijcchd.2024.100541
Gregor J. Krings , Bart W. Driesen , Evangeline G. Warmerdam , Mirella C. Molenschot , Gert-Jan T. Sieswerda , Pieter A. Doevendans , Arie P.J. van Dijk , M. Voskuil
Objectives
To describe the workflow and value of three-dimensional rotational angiography (3DRA) in percutaneous pulmonary valve implantation (PPVI).
Background
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.
Methods
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.
Results
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).
Conclusions
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.
{"title":"Percutaneous pulmonary valve implantation guided by three-dimensional rotational angiography","authors":"Gregor J. Krings , Bart W. Driesen , Evangeline G. Warmerdam , Mirella C. Molenschot , Gert-Jan T. Sieswerda , Pieter A. Doevendans , Arie P.J. van Dijk , 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 mGycm<sup>2</sup> and 179 mGycm<sup>2</sup>/kg for all attempted PPVI's. For successful PPVI 9835 mGycm<sup>2</sup> and 174 mGycm<sup>2</sup>/kg. After optimizing the 3DRA protocols the mean dose reduced from 12677 mGycm<sup>2</sup> to 8551 mGycm<sup>2</sup> (200 mGycm<sup>2</sup>/kg to 163 mGycm<sup>2</sup>/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}
Pub Date : 2024-09-01DOI: 10.1016/j.ijcchd.2024.100516
Kana Kubota , Gerhard-Paul Diller , Aleksander Kempny , Andreas Hoschtitzky , Yasushi Imai , Masaaki Kawada , Darryl Shore , Michael A. Gatzoulis
{"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 , Gerhard-Paul Diller , Aleksander Kempny , Andreas Hoschtitzky , Yasushi Imai , Masaaki Kawada , Darryl Shore , 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}
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