s Identifying priority areas of nursing practice for individuals with congenital heart disease (CHD) is essential to address evolving care needs and support professional development. However, standardized criteria to guide such prioritization remain limited.
Methods
A cross-sectional observational study was conducted between April and September 2024 using an online survey distributed to all nurse members of the Italian Society of Pediatric Cardiology and Congenital Heart Diseases (SICPED). Sixteen CHD-related practice areas and five prioritization criteria were defined through literature review and expert consultation. Participants rated each area on a 5-point Likert scale according to the five criteria.
Results
A total of 78 nurses completed the survey. Among the criteria, the absence of clinical guidelines (mean = 4.04 ± 0.98) and the required level of professional competence (mean = 3.95 ± 1.24) received the highest scores. Mixed ANOVA revealed significant variability in scores across practice areas and criteria (F(48) = 2.385, p < .001). Four areas emerged as priority domains across multiple criteria: psychosocial and emotional support for individuals with CHD and their families, management of neonatal opioid withdrawal, palliative and end-of-life care, and the transition of adolescents with CHD to adult services.
Conclusions
This study provides a structured foundation for identifying nursing practice priorities in the care of individuals with CHD. These findings may guide the development of interdisciplinary strategies, clinical guidelines, and professional training initiatives within communities of practice.
确定先天性心脏病（CHD）患者护理实践的优先领域对于解决不断变化的护理需求和支持专业发展至关重要。然而，指导这种优先次序的标准化标准仍然有限。方法于2024年4月至9月对意大利儿科心脏病和先天性心脏病学会（SICPED）的所有护士成员进行在线调查，进行横断面观察性研究。通过文献回顾和专家咨询，确定了16个冠心病相关实践领域和5个优先标准。参与者根据五个标准对每个区域进行5分李克特评分。结果共78名护士完成调查。其中，缺乏临床指南（平均= 4.04±0.98）和专业能力要求水平（平均= 3.95±1.24）得分最高。混合方差分析显示，不同实践领域和标准的得分存在显著差异（F(48) = 2.385, p < .001）。在多个标准中，四个领域被列为优先领域：对冠心病患者及其家人的社会心理和情感支持，新生儿阿片类药物戒断的管理，姑息治疗和临终关怀，以及冠心病青少年向成人服务的过渡。结论：本研究为确定冠心病患者护理实践的优先事项提供了结构化的基础。这些发现可以指导跨学科策略、临床指南和实践社区内的专业培训计划的发展。
{"title":"Identification of priority practice areas for evidence-based care in congenital heart Disease: An exploratory Survey of non-medical healthcare professionals affiliated with the Italian Society of Pediatric Cardiology","authors":"Arianna Magon , Serena Francesca Flocco , Samantha Scolari , Gianluca Conte , Cristina Arrigoni , Greta Ghizzardi , Giovanna Campioni , Raluca-Bianca Nedesca , Enrico Gambirasi , Floriana Di Iorio , Rossella Orlando , Mariassunta Castaldi , Chiara Gatti , Paola Claudia Fazio , Mariella Rubin , Cinzia Pirti , Stefania Baratta , Assunta Carandente , Salvatore Angileri , Rosario Caruso","doi":"10.1016/j.ijcchd.2025.100616","DOIUrl":"10.1016/j.ijcchd.2025.100616","url":null,"abstract":"<div><h3>Introduction</h3><div>s Identifying priority areas of nursing practice for individuals with congenital heart disease (CHD) is essential to address evolving care needs and support professional development. However, standardized criteria to guide such prioritization remain limited.</div></div><div><h3>Methods</h3><div>A cross-sectional observational study was conducted between April and September 2024 using an online survey distributed to all nurse members of the Italian Society of Pediatric Cardiology and Congenital Heart Diseases (SICPED). Sixteen CHD-related practice areas and five prioritization criteria were defined through literature review and expert consultation. Participants rated each area on a 5-point Likert scale according to the five criteria.</div></div><div><h3>Results</h3><div>A total of 78 nurses completed the survey. Among the criteria, the absence of clinical guidelines (mean = 4.04 ± 0.98) and the required level of professional competence (mean = 3.95 ± 1.24) received the highest scores. Mixed ANOVA revealed significant variability in scores across practice areas and criteria (F(48) = 2.385, p < .001). Four areas emerged as priority domains across multiple criteria: psychosocial and emotional support for individuals with CHD and their families, management of neonatal opioid withdrawal, palliative and end-of-life care, and the transition of adolescents with CHD to adult services.</div></div><div><h3>Conclusions</h3><div>This study provides a structured foundation for identifying nursing practice priorities in the care of individuals with CHD. These findings may guide the development of interdisciplinary strategies, clinical guidelines, and professional training initiatives within communities of practice.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100616"},"PeriodicalIF":1.2,"publicationDate":"2025-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145106096","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-08-27DOI: 10.1016/j.ijcchd.2025.100617
Muneaki Matsubara , Alessandra Poppe , Thibault Schaeffer , Jonas Palm , Teresa Lemmen , Paul Philipp Heinisch , Nicole Piber , Andrea Amici , Alfred Hager , Peter Ewert , Jürgen Hörer , Masamichi Ono
Objective
This study evaluated thrombus formation and its impact on outcomes in neonates and early infants undergoing congenital heart surgery.
Methods
Neonates and early infants (≤90 days) undergoing congenital heart surgery with cardiopulmonary bypass from 2001 to 2024 were analyzed. Thrombi were detected by transthoracic echocardiography and cardiac catheterization.
Results
Among 2331 patients, 170 (7.3 %) developed thrombi during hospitalization. Median age at surgery and time to thrombus detection in affected patients were 12 (interquartile range: 7–34) and 7 (interquartile range: 3–15) days, respectively. Among surgical procedures performed in at least 10 patients, thrombi were most frequently observed following tricuspid valve repair (28.6 %), followed by arterial switch operation, ventricular septal defect closure, and aortic arch repair (15.8 %). The most common thrombus location was the superior vena cava in 61 patients, followed by the inferior vena cava in 33, the aorta in 31, and the right atrium in 21 patients. Additional surgical interventions were required in 28 patients. The length of hospital stay was significantly longer in patients with thrombi (27 vs. 15 days, p < 0.001). Independent risk factors for thrombus formation included preoperative cardiopulmonary resuscitation (odds ratio: 2.037, p = 0.001), tricuspid valve repair (odds ratio: 6.206, p < 0.001), and Norwood procedure (odds ratio: 1.558, p = 0.027).
Conclusions
The incidence of thrombus formation was 7.3 % in neonates and early infants undergoing congenital heart surgery. Thrombus was most frequently observed in the superior vena cava and resulted in prolonged hospitalization. Preoperative cardiopulmonary resuscitation, tricuspid valve repair, and Norwood procedures carried the highest thrombotic risk.
{"title":"Thrombus formation in neonates and early infants undergoing congenital heart surgery","authors":"Muneaki Matsubara , Alessandra Poppe , Thibault Schaeffer , Jonas Palm , Teresa Lemmen , Paul Philipp Heinisch , Nicole Piber , Andrea Amici , Alfred Hager , Peter Ewert , Jürgen Hörer , Masamichi Ono","doi":"10.1016/j.ijcchd.2025.100617","DOIUrl":"10.1016/j.ijcchd.2025.100617","url":null,"abstract":"<div><h3>Objective</h3><div>This study evaluated thrombus formation and its impact on outcomes in neonates and early infants undergoing congenital heart surgery.</div></div><div><h3>Methods</h3><div>Neonates and early infants (≤90 days) undergoing congenital heart surgery with cardiopulmonary bypass from 2001 to 2024 were analyzed. Thrombi were detected by transthoracic echocardiography and cardiac catheterization.</div></div><div><h3>Results</h3><div>Among 2331 patients, 170 (7.3 %) developed thrombi during hospitalization. Median age at surgery and time to thrombus detection in affected patients were 12 (interquartile range: 7–34) and 7 (interquartile range: 3–15) days, respectively. Among surgical procedures performed in at least 10 patients, thrombi were most frequently observed following tricuspid valve repair (28.6 %), followed by arterial switch operation, ventricular septal defect closure, and aortic arch repair (15.8 %). The most common thrombus location was the superior vena cava in 61 patients, followed by the inferior vena cava in 33, the aorta in 31, and the right atrium in 21 patients. Additional surgical interventions were required in 28 patients. The length of hospital stay was significantly longer in patients with thrombi (27 vs. 15 days, p < 0.001). Independent risk factors for thrombus formation included preoperative cardiopulmonary resuscitation (odds ratio: 2.037, p = 0.001), tricuspid valve repair (odds ratio: 6.206, p < 0.001), and Norwood procedure (odds ratio: 1.558, p = 0.027).</div></div><div><h3>Conclusions</h3><div>The incidence of thrombus formation was 7.3 % in neonates and early infants undergoing congenital heart surgery. Thrombus was most frequently observed in the superior vena cava and resulted in prolonged hospitalization. Preoperative cardiopulmonary resuscitation, tricuspid valve repair, and Norwood procedures carried the highest thrombotic risk.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100617"},"PeriodicalIF":1.2,"publicationDate":"2025-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145106098","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Survival rates for patients with congenital heart disease (CHD) have improved, but complications like pulmonary regurgitation (PR) often require re-interventions. Transcatheter pulmonary valve implantation (TPVI) with self-expandable valves, such as the Venus P-Valve, has broadened treatment options. Accurate procedural planning, particularly valve sizing, remains a significant challenge. Mixed Reality (MxR) technology enables a patient-specific approach that enhances procedural planning accuracy.
Aim
To evaluate the use of MxR in planning Venus-P valve implantation.
Materials and methods
This study included patients undergoing Venus P-Valve implantation with holographic models generated from CT data using ARTICOR® software from January 2023 to June 2024. Two independent operators used these models for procedural planning. Concordance between operators was assessed. Predictions were compared with implanted valve dimensions to evaluate concordance.
Results
Of 29 eligible patients, 26 underwent successful Venus-P valve implantation. Concordance between the operators reached 60 % (n = 15/26) for valve diameter and over 88 % (n = 23/26) for valve length. Holographic models achieved 96 % (n = 25/26) concordance in predicting valve length, type of the approach (92 %) and 50 % (n = 13/26) concordance for diameter.
Discussion
Holographic models enhanced procedural planning, enabling better visualization and collaborative decision-making. While highly effective for valve length predictions, and type of the approach limitations in predicting valve diameter highlight the need for improved methods, such as computational modelling or machine learning.
Conclusion
Patient-specific holographic models are promising tools for TPVI planning. Advancements in technology and interdisciplinary collaboration are critical to overcoming current limitations and advancing procedural planning and related outcomes in interventional cardiology.
Condensed abstract
This study assesses the use of mixed reality (MxR) technology for procedural planning in transcatheter pulmonary valve implantation (TPVI) with the Venus P-Valve. Holographic models were created from CT data to aid in valve sizing and implantation strategies, with two operators comparing measurements for valve diameter and length. Among 26 patients who underwent successful implantation, concordance between operators was 60 % for valve diameter and 88 % for valve length. Predictions using holographic models showed 96 % concordance for implanted valve length, type of the approach (92 %) and 50 % concordance for valve diameter. These results highlight the potential of MxR for improving TPVI planning, though the limited accuracy for valve diameter suggests a need for further advancements, such as computational modeling or
{"title":"Patient-specific mixed reality for venus P-valve implantation: A novel approach to procedural planning","authors":"Angelo Fabio d’Aiello , Francesca Bevilacqua , Angelo Micheletti , Diana Gabriela Negura , Giulia Pasqualin , Luca Giugno , Luciane Piazza , Laura Schianchi , Sara Boveri , Alessandro Giamberti , Massimo Chessa","doi":"10.1016/j.ijcchd.2025.100614","DOIUrl":"10.1016/j.ijcchd.2025.100614","url":null,"abstract":"<div><h3>Background</h3><div>Survival rates for patients with congenital heart disease (CHD) have improved, but complications like pulmonary regurgitation (PR) often require re-interventions. Transcatheter pulmonary valve implantation (TPVI) with self-expandable valves, such as the Venus P-Valve, has broadened treatment options. Accurate procedural planning, particularly valve sizing, remains a significant challenge. Mixed Reality (MxR) technology enables a patient-specific approach that enhances procedural planning accuracy.</div></div><div><h3>Aim</h3><div>To evaluate the use of MxR in planning Venus-P valve implantation.</div></div><div><h3>Materials and methods</h3><div>This study included patients undergoing Venus P-Valve implantation with holographic models generated from CT data using ARTICOR® software from January 2023 to June 2024. Two independent operators used these models for procedural planning. Concordance between operators was assessed. Predictions were compared with implanted valve dimensions to evaluate concordance.</div></div><div><h3>Results</h3><div>Of 29 eligible patients, 26 underwent successful Venus-P valve implantation. Concordance between the operators reached 60 % (n = 15/26) for valve diameter and over 88 % (n = 23/26) for valve length. Holographic models achieved 96 % (n = 25/26) concordance in predicting valve length, type of the approach (92 %) and 50 % (n = 13/26) concordance for diameter.</div></div><div><h3>Discussion</h3><div>Holographic models enhanced procedural planning, enabling better visualization and collaborative decision-making. While highly effective for valve length predictions, and type of the approach limitations in predicting valve diameter highlight the need for improved methods, such as computational modelling or machine learning.</div></div><div><h3>Conclusion</h3><div>Patient-specific holographic models are promising tools for TPVI planning. Advancements in technology and interdisciplinary collaboration are critical to overcoming current limitations and advancing procedural planning and related outcomes in interventional cardiology.</div></div><div><h3>Condensed abstract</h3><div>This study assesses the use of mixed reality (MxR) technology for procedural planning in transcatheter pulmonary valve implantation (TPVI) with the Venus P-Valve. Holographic models were created from CT data to aid in valve sizing and implantation strategies, with two operators comparing measurements for valve diameter and length. Among 26 patients who underwent successful implantation, concordance between operators was 60 % for valve diameter and 88 % for valve length. Predictions using holographic models showed 96 % concordance for implanted valve length, type of the approach (92 %) and 50 % concordance for valve diameter. These results highlight the potential of MxR for improving TPVI planning, though the limited accuracy for valve diameter suggests a need for further advancements, such as computational modeling or ","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100614"},"PeriodicalIF":1.2,"publicationDate":"2025-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145106151","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-08-25DOI: 10.1016/j.ijcchd.2025.100615
Pieter-Jan Lewandowski , Thomas Rosseel , Peter Haemers , Els Troost , Christophe Garweg , Werner Budts , Joris Ector , Pieter De Meester , Alexander Van De Bruaene , Bert Vandenberk
Introduction
Patients with atrial septal defect (ASD) and patent foramen ovale (PFO) are prone to atrial arrhythmias due to structural and surgical alterations. While radiofrequency catheter ablation (RFCA) is a common treatment, arrhythmia recurrence remains a clinical challenge. This study aimed to assess arrhythmia patterns, procedural outcomes, and long-term recurrence following electrophysiological interventions.
Methods
A retrospective cohort study was performed at a tertiary center including patients with ASD or PFO undergoing invasive electrophysiological procedures. Clinical and procedural data were extracted from medical records. Arrhythmias were classified based on electrocardiographic and electrophysiological findings. Kaplan-Meier analysis was used to assess event-free survival for arrhythmia recurrence.
Results
Among 87 patients (57.5 % female, median age 46.4 years), 82.8 % underwent RFCA as the first intervention. The most common arrhythmias were non-cavo-tricuspid isthmus (CTI) dependent intra-atrial reentrant tachycardia (35.4 %) and atrial fibrillation (18.6 %). Acute procedural success was achieved in 97.2 % of patients. Recurrence-free survival after the first RFCA was 83.6 % at 1 year, 64.2 % at 3 years, and 60.0 % at 5 years. After the last RFCA, recurrence-free survival improved to 88.3 % at 1 year and 74.3 % at 5 years.
Conclusion
In patients with ASD and PFO, RFCA is safe and acutely effective but followed by arrhythmia recurrence in a quarter of patients. The evolving arrhythmic substrate highlights the need for early intervention strategies and emerging technologies to optimize long-term outcomes in this patient population.
{"title":"Long-term outcome of invasive electrophysiological studies and catheter ablation in patients with patent foramen ovale and atrial septal defect–A single-center retrospective cohort study","authors":"Pieter-Jan Lewandowski , Thomas Rosseel , Peter Haemers , Els Troost , Christophe Garweg , Werner Budts , Joris Ector , Pieter De Meester , Alexander Van De Bruaene , Bert Vandenberk","doi":"10.1016/j.ijcchd.2025.100615","DOIUrl":"10.1016/j.ijcchd.2025.100615","url":null,"abstract":"<div><h3>Introduction</h3><div>Patients with atrial septal defect (ASD) and patent foramen ovale (PFO) are prone to atrial arrhythmias due to structural and surgical alterations. While radiofrequency catheter ablation (RFCA) is a common treatment, arrhythmia recurrence remains a clinical challenge. This study aimed to assess arrhythmia patterns, procedural outcomes, and long-term recurrence following electrophysiological interventions.</div></div><div><h3>Methods</h3><div>A retrospective cohort study was performed at a tertiary center including patients with ASD or PFO undergoing invasive electrophysiological procedures. Clinical and procedural data were extracted from medical records. Arrhythmias were classified based on electrocardiographic and electrophysiological findings. Kaplan-Meier analysis was used to assess event-free survival for arrhythmia recurrence.</div></div><div><h3>Results</h3><div>Among 87 patients (57.5 % female, median age 46.4 years), 82.8 % underwent RFCA as the first intervention. The most common arrhythmias were non-cavo-tricuspid isthmus (CTI) dependent intra-atrial reentrant tachycardia (35.4 %) and atrial fibrillation (18.6 %). Acute procedural success was achieved in 97.2 % of patients. Recurrence-free survival after the first RFCA was 83.6 % at 1 year, 64.2 % at 3 years, and 60.0 % at 5 years. After the last RFCA, recurrence-free survival improved to 88.3 % at 1 year and 74.3 % at 5 years.</div></div><div><h3>Conclusion</h3><div>In patients with ASD and PFO, RFCA is safe and acutely effective but followed by arrhythmia recurrence in a quarter of patients. The evolving arrhythmic substrate highlights the need for early intervention strategies and emerging technologies to optimize long-term outcomes in this patient population.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100615"},"PeriodicalIF":1.2,"publicationDate":"2025-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145106095","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-08-13DOI: 10.1016/j.ijcchd.2025.100613
Ohad Houri , Noam Shasho-Peres , Eran Hadar , Sharon Orbach Zinger , Ran Kornowski , Anat Pardo , Shimon Kolker , Shahar Vig , Asnat Walfisch , Bar Narkis , Nili Schamroth Pravda
Background
Data on the characteristics and outcomes of pregnancy amongst patients with Tetralogy of Fallot (TOF) is limited. Depending on the hemodynamic status of the patient, the risk of maternal and fetal complications during pregnancy can be considerable.
Objectives
We aimed to systematically evaluate maternal, obstetric, and fetal outcomes, and assess longitudinal changes in cardiac function among women with TOF using serial imaging.
Methods
Pregnant adult patients with TOF between 2014 and 2022 were included. We examined maternal and obstetric outcomes during pregnancy. We assessed serial imaging to evaluate the temporal effects of pregnancy on cardiac function.
Results
Our cohort included 50 patients. There were 85 pregnancies overall with 79 live births (92.9 %). The median gestational age at delivery was 38.3 [37.0–39.1] gestational weeks (GW), nine deliveries (11.3 %) occurred before 37 GW, but only one before 34 GW. The median birthweight at delivery was 2850 [2450–3048] grams. The main fetal complication was small for gestational age (n = 16,22 %). There were no deaths in the peripartum period or the year following pregnancy. There were no significant valvular or ventricular functional differences when comparing pre-conception to post-conception echocardiogram data. In the subgroup with availability of MRI data, the Right ventricular end-diastolic volume index increased significantly post-pregnancy (109 ± 37.9 ml/m2 vs 117 ± 25.0 ml/m2, p < 0.01).
Conclusions
Maternal and obstetric complications are rare among patients with TOF. Our initial findings suggest that pregnancy does influence cardiac dimensions on serial long-term imaging follow-up but further, expanded long-term data is needed.
背景：关于法洛四联症（TOF）患者妊娠的特点和结局的数据是有限的。根据患者的血流动力学状况，妊娠期间发生母胎并发症的风险可能相当大。目的：我们旨在系统地评估产妇、产科和胎儿结局，并利用序列成像评估TOF妇女心功能的纵向变化。方法选取2014 ~ 2022年妊娠期成人TOF患者。我们检查了怀孕期间的孕产妇和产科结局。我们评估了一系列影像来评估妊娠对心功能的时间影响。结果我们的队列包括50例患者。总共有85例妊娠，79例活产（92.9%）。分娩时的中位胎龄为38.3[37.0-39.1]胎周（GW）， 9例（11.3%）分娩在37 GW之前，但只有1例分娩在34 GW之前。分娩时出生体重中位数为2850[2450-3048]克。主要的胎儿并发症是胎龄小（n = 16, 22%）。围产期和妊娠后一年无死亡病例。当比较孕前和孕后超声心动图数据时，没有明显的瓣膜或心室功能差异。在可获得MRI数据的亚组中，妊娠后右心室舒张末期容积指数显著升高（109±37.9 ml/m2 vs 117±25.0 ml/m2, p < 0.01）。结论TOF患者的产妇及产科并发症较少见。我们的初步研究结果表明，妊娠确实会影响连续长期影像学随访的心脏尺寸，但需要进一步扩大长期数据。
{"title":"Pregnancy outcomes and effects on cardiac function among patients with Tetralogy of Fallot: A single-center cohort study","authors":"Ohad Houri , Noam Shasho-Peres , Eran Hadar , Sharon Orbach Zinger , Ran Kornowski , Anat Pardo , Shimon Kolker , Shahar Vig , Asnat Walfisch , Bar Narkis , Nili Schamroth Pravda","doi":"10.1016/j.ijcchd.2025.100613","DOIUrl":"10.1016/j.ijcchd.2025.100613","url":null,"abstract":"<div><h3>Background</h3><div>Data on the characteristics and outcomes of pregnancy amongst patients with Tetralogy of Fallot (TOF) is limited. Depending on the hemodynamic status of the patient, the risk of maternal and fetal complications during pregnancy can be considerable.</div></div><div><h3>Objectives</h3><div>We aimed to systematically evaluate maternal, obstetric, and fetal outcomes, and assess longitudinal changes in cardiac function among women with TOF using serial imaging.</div></div><div><h3>Methods</h3><div>Pregnant adult patients with TOF between 2014 and 2022 were included. We examined maternal and obstetric outcomes during pregnancy. We assessed serial imaging to evaluate the temporal effects of pregnancy on cardiac function.</div></div><div><h3>Results</h3><div>Our cohort included 50 patients. There were 85 pregnancies overall with 79 live births (92.9 %). The median gestational age at delivery was 38.3 [37.0–39.1] gestational weeks (GW), nine deliveries (11.3 %) occurred before 37 GW, but only one before 34 GW. The median birthweight at delivery was 2850 [2450–3048] grams. The main fetal complication was small for gestational age (n = 16,22 %). There were no deaths in the peripartum period or the year following pregnancy. There were no significant valvular or ventricular functional differences when comparing pre-conception to post-conception echocardiogram data. In the subgroup with availability of MRI data, the Right ventricular end-diastolic volume index increased significantly post-pregnancy (109 ± 37.9 ml/m<sup>2</sup> vs 117 ± 25.0 ml/m<sup>2</sup>, p < 0.01).</div></div><div><h3>Conclusions</h3><div>Maternal and obstetric complications are rare among patients with TOF. Our initial findings suggest that pregnancy does influence cardiac dimensions on serial long-term imaging follow-up but further, expanded long-term data is needed.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"22 ","pages":"Article 100613"},"PeriodicalIF":1.2,"publicationDate":"2025-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144922015","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-07-26eCollection Date: 2025-09-01DOI: 10.1016/j.ijcchd.2025.100612
Amr Moustafa, Ahmed T Abdelhalim, William R Miranda, Heidi M Connolly, Elizabeth H Stephens, Joseph A Dearani, Alexander C Egbe
Background: Valve regurgitation is associated with systemic ventricular dysfunction and mortality in patients with Fontan palliation. Valve repair and valve replacement with bioprostheses have limited durability, and outcomes after valve replacement with mechanical prostheses are poorly defined. The study objectives were to assess: (1) Operative mortality, valve-related complications (stroke, mechanical valve thrombosis, major bleeding complication, prosthetic valve endocarditis, and valve reoperation), and late mortality/transplant. (2) Postoperative changes in ventricular volumes and ejection fraction; (3) Postoperative changes in the clinical indices of disease severity.
Method: Retrospective cohort study of adults (age ≥18 years) with Fontan palliation who underwent isolated aortic or atrioventricular valve replacement at Mayo Clinic (2003-2023). Preoperative and postoperative evaluations (clinical evaluation, echocardiogram, biomarkers) were performed 6 months preoperatively and 6-24 months postoperatively, respectively.
Results: Overall, 17 patients underwent isolated atrioventricular (N = 9) or aortic valve (N = 8) replacement with mechanical prosthesis (age 27 ± 8 years; 9 [53 %] males). There was no operative mortality. There was postoperative improvement in functional capacity (NYHA class), heart failure indices (NT proBNP), and hepatorenal function (MELD-XI score). There was a postoperative decrease in systemic ventricular volumes, without change in ejection fraction. The 10-year cumulative incidence of valve-related complications and death/transplant was 15 % and 18 %, respectively.
Conclusions: Atrioventricular valve and aortic valve replacement with mechanical prostheses was associated with low (zero) operative mortality, low risk of valve-related complications, and clinical improvement. This suggests that valve replacement with mechanical prostheses may be an effective palliation in selected patients and may delay the need for transplant in this population.
{"title":"Mechanical valve replacement in adults with Fontan physiology is an effective palliation in selected patients.","authors":"Amr Moustafa, Ahmed T Abdelhalim, William R Miranda, Heidi M Connolly, Elizabeth H Stephens, Joseph A Dearani, Alexander C Egbe","doi":"10.1016/j.ijcchd.2025.100612","DOIUrl":"10.1016/j.ijcchd.2025.100612","url":null,"abstract":"<p><strong>Background: </strong>Valve regurgitation is associated with systemic ventricular dysfunction and mortality in patients with Fontan palliation. Valve repair and valve replacement with bioprostheses have limited durability, and outcomes after valve replacement with mechanical prostheses are poorly defined. The study objectives were to assess: (1) Operative mortality, valve-related complications (stroke, mechanical valve thrombosis, major bleeding complication, prosthetic valve endocarditis, and valve reoperation), and late mortality/transplant. (2) Postoperative changes in ventricular volumes and ejection fraction; (3) Postoperative changes in the clinical indices of disease severity.</p><p><strong>Method: </strong>Retrospective cohort study of adults (age ≥18 years) with Fontan palliation who underwent isolated aortic or atrioventricular valve replacement at Mayo Clinic (2003-2023). Preoperative and postoperative evaluations (clinical evaluation, echocardiogram, biomarkers) were performed 6 months preoperatively and 6-24 months postoperatively, respectively.</p><p><strong>Results: </strong>Overall, 17 patients underwent isolated atrioventricular (N = 9) or aortic valve (N = 8) replacement with mechanical prosthesis (age 27 ± 8 years; 9 [53 %] males). There was no operative mortality. There was postoperative improvement in functional capacity (NYHA class), heart failure indices (NT proBNP), and hepatorenal function (MELD-XI score). There was a postoperative decrease in systemic ventricular volumes, without change in ejection fraction. The 10-year cumulative incidence of valve-related complications and death/transplant was 15 % and 18 %, respectively.</p><p><strong>Conclusions: </strong>Atrioventricular valve and aortic valve replacement with mechanical prostheses was associated with low (zero) operative mortality, low risk of valve-related complications, and clinical improvement. This suggests that valve replacement with mechanical prostheses may be an effective palliation in selected patients and may delay the need for transplant in this population.</p>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"100612"},"PeriodicalIF":1.2,"publicationDate":"2025-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12356454/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144877076","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 : 2025-07-18DOI: 10.1016/j.ijcchd.2025.100611
Lea Behrend , Thibault Schaeffer , Muneaki Matsubara , Jonas Palm , Teresa Lemmen , Nicole Piber , Paul Philipp Heinisch , Stanimir Georgiev , Alfred Hager , Peter Ewert , Jürgen Hörer , Masamichi Ono
Objective
This study evaluated the current incidence of pulmonary arteriovenous malformations (PAVMs) following stage 2 palliation (S2P).
Methods
Patients who underwent S2P, either through a bidirectional cavopulmonary shunt (BCPS) or the Kawashima procedure (KP) between 1992 and 2022, were reviewed. The cumulative incidence of PAVMs was compared between BCPS and KP. Risk factors for the development of PAVMs were identified.
Results
Among 682 patients who underwent S2P, 661 (96.9 %) underwent BCPS and 21 (3.1 %) KP. Median age at S2P was 5.1 (interquartile ranges (IQR): 3.6–9.6) months. During the median interstage follow-up of 1.6 (IQR: 1.6–2.2) years, PAVMs developed in 11 (1.6 %) patients (1.1 % (n = 7) after BCPS and 19.0 % (n = 4) after KP). Cumulative incidence of PAVMs was higher in patients after KP than those after BCPS (p < 0.001). PAVMs were observed in the right lung in 9 patients and both lungs in two. One patient with biliary atresia died of progressive PAVMs and liver cirrhosis after KP, and the remaining 10 patients underwent Fontan completion with a median interval of 1.9 (IQR: 1.5–2.4) years. PAVMs improved in all patients (9 resolutions and 1 improved). Independent risk factors for the development of PAVMs were KP (hazard ratio (HR): 16.364, p < 0.001) in all patients, anomalous pulmonary venous connection (HR: 6.772, p = 0.023) in BCPS patients, and hypoplastic left heart syndrome (HR: 18.819, p = 0.018) in KP patients.
Conclusions
The incidence of PAVMs after S2P is very low after BCPS but still relevant after KP. Resolution or improvement of PAVMs is probable after Fontan completion.
{"title":"Incidence and risk factors for the development of pulmonary arteriovenous malformations after stage 2 palliation","authors":"Lea Behrend , Thibault Schaeffer , Muneaki Matsubara , Jonas Palm , Teresa Lemmen , Nicole Piber , Paul Philipp Heinisch , Stanimir Georgiev , Alfred Hager , Peter Ewert , Jürgen Hörer , Masamichi Ono","doi":"10.1016/j.ijcchd.2025.100611","DOIUrl":"10.1016/j.ijcchd.2025.100611","url":null,"abstract":"<div><h3>Objective</h3><div>This study evaluated the current incidence of pulmonary arteriovenous malformations (PAVMs) following stage 2 palliation (S2P).</div></div><div><h3>Methods</h3><div>Patients who underwent S2P, either through a bidirectional cavopulmonary shunt (BCPS) or the Kawashima procedure (KP) between 1992 and 2022, were reviewed. The cumulative incidence of PAVMs was compared between BCPS and KP. Risk factors for the development of PAVMs were identified.</div></div><div><h3>Results</h3><div>Among 682 patients who underwent S2P, 661 (96.9 %) underwent BCPS and 21 (3.1 %) KP. Median age at S2P was 5.1 (interquartile ranges (IQR): 3.6–9.6) months. During the median interstage follow-up of 1.6 (IQR: 1.6–2.2) years, PAVMs developed in 11 (1.6 %) patients (1.1 % (n = 7) after BCPS and 19.0 % (n = 4) after KP). Cumulative incidence of PAVMs was higher in patients after KP than those after BCPS (p < 0.001). PAVMs were observed in the right lung in 9 patients and both lungs in two. One patient with biliary atresia died of progressive PAVMs and liver cirrhosis after KP, and the remaining 10 patients underwent Fontan completion with a median interval of 1.9 (IQR: 1.5–2.4) years. PAVMs improved in all patients (9 resolutions and 1 improved). Independent risk factors for the development of PAVMs were KP (hazard ratio (HR): 16.364, p < 0.001) in all patients, anomalous pulmonary venous connection (HR: 6.772, p = 0.023) in BCPS patients, and hypoplastic left heart syndrome (HR: 18.819, p = 0.018) in KP patients.</div></div><div><h3>Conclusions</h3><div>The incidence of PAVMs after S2P is very low after BCPS but still relevant after KP. Resolution or improvement of PAVMs is probable after Fontan completion.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100611"},"PeriodicalIF":1.2,"publicationDate":"2025-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144879077","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-07-16eCollection Date: 2025-09-01DOI: 10.1016/j.ijcchd.2025.100609
M Pilati, F A D'Aiello, M Chessa, G Santoro, A Donti, E Mariucci, C Marrone, G Annoni, G Gaio, M Rebonato, E Piccinelli, M Cheli, R Formigari, G Butera
Background: Self-expandable valves may have a significant impact on the feasibility of transcatheter treatments of RVOT dysfunction. Venus P valve is the first autoexpadable Valve that received CE mark in 2022. Here, we are reporting on a multicenter data collection about patients undergoing Venus P valve implantation in Italy.
Materials: Seven italian centers were included in the study. Data were collected from patients treated with a venus P valve between July 2022 and May 2024. Indication for percutaneous valve implantation were in accordance with published guidelines.
Results: During the study period, a total of 65 patients were considered eligible to percutaneous implantation of a Venus P valve. Median age at procedure was 35 years (IQR 12-63 years) median weight 63 Kg (IQR 33-103 Kg). Indication for PPVI was severe regurgitation in all subjects but one where ventricular pressure was increased because of stenosis on a homograft conduit and pulmonary arteries which needed previous bilateral stenting. Venus P valve was implanted successfully in 61/65(94 %) patients. Four procedural complications occurred(6 %): 1 artero-venous fistula, 1 pulmonary bleeding, 1 hemothorax and a pulmonary oedema. During follow up (median 13 months,1-20months), all patients experienced a significant improvement of functional class. Neither deaths or re-intervention occurred. CT Scan showed hypoattenuating leaflet thickness (HALT) in 4 patients and one of them experienced endocarditis 12 months after the procedure.
Conclusions: Our study shows that Venus P valve implantation is safe and results are stable during a short-to-medium term follow-up. Endocarditis and HALT are inducing some concern and larger studies are needed.
{"title":"Percutaneous implantation of self-expanding pulmonary valves: results from real-life experience of the Venus-P valve Registry of the Italian Society of Pediatric Cardiology (SICPED).","authors":"M Pilati, F A D'Aiello, M Chessa, G Santoro, A Donti, E Mariucci, C Marrone, G Annoni, G Gaio, M Rebonato, E Piccinelli, M Cheli, R Formigari, G Butera","doi":"10.1016/j.ijcchd.2025.100609","DOIUrl":"10.1016/j.ijcchd.2025.100609","url":null,"abstract":"<p><strong>Background: </strong>Self-expandable valves may have a significant impact on the feasibility of transcatheter treatments of RVOT dysfunction. Venus P valve is the first autoexpadable Valve that received CE mark in 2022. Here, we are reporting on a multicenter data collection about patients undergoing Venus P valve implantation in Italy.</p><p><strong>Materials: </strong>Seven italian centers were included in the study. Data were collected from patients treated with a venus P valve between July 2022 and May 2024. Indication for percutaneous valve implantation were in accordance with published guidelines.</p><p><strong>Results: </strong>During the study period, a total of 65 patients were considered eligible to percutaneous implantation of a Venus P valve. Median age at procedure was 35 years (IQR 12-63 years) median weight 63 Kg (IQR 33-103 Kg). Indication for PPVI was severe regurgitation in all subjects but one where ventricular pressure was increased because of stenosis on a homograft conduit and pulmonary arteries which needed previous bilateral stenting. Venus P valve was implanted successfully in 61/65(94 %) patients. Four procedural complications occurred(6 %): 1 artero-venous fistula, 1 pulmonary bleeding, 1 hemothorax and a pulmonary oedema. During follow up (median 13 months,1-20months), all patients experienced a significant improvement of functional class. Neither deaths or re-intervention occurred. CT Scan showed hypoattenuating leaflet thickness (HALT) in 4 patients and one of them experienced endocarditis 12 months after the procedure.</p><p><strong>Conclusions: </strong>Our study shows that Venus P valve implantation is safe and results are stable during a short-to-medium term follow-up. Endocarditis and HALT are inducing some concern and larger studies are needed.</p>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"100609"},"PeriodicalIF":1.2,"publicationDate":"2025-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12355537/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144877077","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 : 2025-07-12eCollection Date: 2025-09-01DOI: 10.1016/j.ijcchd.2025.100610
Larissa Lloyd, Sasha Ruban, Tanya Badal, Geoff Strange, David S Celermajer, Carissa Bonner
Background: The survival prospects of adults with congenital heart disease have improved considerably due to improved surgical interventions, resulting in a growing population. This study aims to explore the experiences of adults with congenital heart disease, to identify support needs.
Methods: Participants were recruited from the National Australian Congenital Heart Disease Registry using purposive sampling to ensure diversity in terms of gender, rurality and disease complexity. Semi-structured interviews were conducted with adults with congenital heart disease. The interviews were recorded digitally and transcribed verbatim. Framework Analysis was used to ensure rigour in identifying themes.
Results: 43 interviews were completed (23 males and 20 females; age 20 to 54 years). Three main themes and seven subthemes were identified to explain varying experiences of living with congenital heart disease: I. Lifestyle factors; (i) physical activity, (ii) weight management. II. Practical issues; (iii) employment, (iv) financial stress, (v) education. III. Psychosocial impact; (vi) mental health and resilience, and (vii) social engagement. Participants across a range of disease complexities reported more impact on their lives when they felt inadequately supported by their healthcare providers to address these challenges, such as insufficient education on exercise leading to difficulty performing clinically recommended physical activity.
Conclusions: The lived experiences of adults living with congenital heart disease highlight specific areas needing support, which could inform future improvements in their "whole of life" care. This could include referral to allied health professionals, education across lifestyle and practical domains, and appropriate peer support networks.
{"title":"Living with congenital heart disease: a qualitative study to explore psychosocial impacts in adulthood.","authors":"Larissa Lloyd, Sasha Ruban, Tanya Badal, Geoff Strange, David S Celermajer, Carissa Bonner","doi":"10.1016/j.ijcchd.2025.100610","DOIUrl":"10.1016/j.ijcchd.2025.100610","url":null,"abstract":"<p><strong>Background: </strong>The survival prospects of adults with congenital heart disease have improved considerably due to improved surgical interventions, resulting in a growing population. This study aims to explore the experiences of adults with congenital heart disease, to identify support needs.</p><p><strong>Methods: </strong>Participants were recruited from the National Australian Congenital Heart Disease Registry using purposive sampling to ensure diversity in terms of gender, rurality and disease complexity. Semi-structured interviews were conducted with adults with congenital heart disease. The interviews were recorded digitally and transcribed verbatim. Framework Analysis was used to ensure rigour in identifying themes.</p><p><strong>Results: </strong>43 interviews were completed (23 males and 20 females; age 20 to 54 years). Three main themes and seven subthemes were identified to explain varying experiences of living with congenital heart disease: I. Lifestyle factors; (i) physical activity, (ii) weight management. II. Practical issues; (iii) employment, (iv) financial stress, (v) education. III. Psychosocial impact; (vi) mental health and resilience, and (vii) social engagement. Participants across a range of disease complexities reported more impact on their lives when they felt inadequately supported by their healthcare providers to address these challenges, such as insufficient education on exercise leading to difficulty performing clinically recommended physical activity.</p><p><strong>Conclusions: </strong>The lived experiences of adults living with congenital heart disease highlight specific areas needing support, which could inform future improvements in their \"whole of life\" care. This could include referral to allied health professionals, education across lifestyle and practical domains, and appropriate peer support networks.</p>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"100610"},"PeriodicalIF":1.2,"publicationDate":"2025-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12355555/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144877075","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 : 2025-07-03DOI: 10.1016/j.ijcchd.2025.100608
Alexander C. Egbe, William R. Miranda, Charles Jain, Heidi M. Connolly, Luke J. Burchill, Barry A. Borlaug
Background
Right ventricular (RV) systolic dysfunction and pulmonary hypertension is present in 20 % of adults with repaired coarctation of aorta (COA). However, the prognostic value of RV to pulmonary artery (RV-PA) coupling in this population is unknown. The purpose of this study was to assess the relationship between RV-PA coupling and clinical outcomes (heart failure hospitalization and all-cause mortality) in this population.
Methods
Retrospective cohort study of adults with repaired COA and normal RV systolic function defined as RV free wall strain ≥ -24 %. RV-PA coupling was assessed using tricuspid annular plane systolic excursion/RV systolic pressure (TAPSE/RVSP) ratio.
Results
Of 509 patients (median age 32 [20–45] years; men 290 [57 %]), the average TAPSE and RVSP were 22 ± 5 mm and 33 ± 9 mmHg, respectively, and TAPSE/RVSP ratio was 0.78 (0.56–0.96) mm/mmHg. Of 509 patients, 51 (10 %) died and 43 (8 %) were hospitalized for heart failure during a median follow-up of 8.5 (4.9–10.4) years. TAPSE/RVSP was associated with all-cause mortality (hazard ratio 0.82, 95 % confidence interval 0.78–0.86, per 0.1 mm/mmHg), and heart failure hospitalization (hazard ratio 0.86, 95 % confidence interval 0.79–0.93, per 0.1 mm/mmHg) after adjustment for anatomic lesions, cardiovascular interventions, comorbidities, and echocardiographic indices. The inclusion of TAPSE/RVSP ratio in the risk models improved prognostic power of the models to predict all-cause mortality (C-statistics difference 0.046, p < 0.001), and heart failure hospitalization (C-statistics difference 0.031, p = 0.007).
Conclusions
TAPSE/RVSP ratio was associated with outcomes in COA patients with normal RV systolic function, suggesting that abnormal RV-PA coupling may be present prior to the onset of overt RV systolic function in this population.
{"title":"Prognostic value of right ventricular-pulmonary arterial coupling in adults with repaired coarctation of aorta","authors":"Alexander C. Egbe, William R. Miranda, Charles Jain, Heidi M. Connolly, Luke J. Burchill, Barry A. Borlaug","doi":"10.1016/j.ijcchd.2025.100608","DOIUrl":"10.1016/j.ijcchd.2025.100608","url":null,"abstract":"<div><h3>Background</h3><div>Right ventricular (RV) systolic dysfunction and pulmonary hypertension is present in 20 % of adults with repaired coarctation of aorta (COA). However, the prognostic value of RV to pulmonary artery (RV-PA) coupling in this population is unknown. The purpose of this study was to assess the relationship between RV-PA coupling and clinical outcomes (heart failure hospitalization and all-cause mortality) in this population.</div></div><div><h3>Methods</h3><div>Retrospective cohort study of adults with repaired COA and normal RV systolic function defined as RV free wall strain ≥ -24 %. RV-PA coupling was assessed using tricuspid annular plane systolic excursion/RV systolic pressure (TAPSE/RVSP) ratio.</div></div><div><h3>Results</h3><div>Of 509 patients (median age 32 [20–45] years; men 290 [57 %]), the average TAPSE and RVSP were 22 ± 5 mm and 33 ± 9 mmHg, respectively, and TAPSE/RVSP ratio was 0.78 (0.56–0.96) mm/mmHg. Of 509 patients, 51 (10 %) died and 43 (8 %) were hospitalized for heart failure during a median follow-up of 8.5 (4.9–10.4) years. TAPSE/RVSP was associated with all-cause mortality (hazard ratio 0.82, 95 % confidence interval 0.78–0.86, per 0.1 mm/mmHg), and heart failure hospitalization (hazard ratio 0.86, 95 % confidence interval 0.79–0.93, per 0.1 mm/mmHg) after adjustment for anatomic lesions, cardiovascular interventions, comorbidities, and echocardiographic indices. The inclusion of TAPSE/RVSP ratio in the risk models improved prognostic power of the models to predict all-cause mortality (C-statistics difference 0.046, p < 0.001), and heart failure hospitalization (C-statistics difference 0.031, p = 0.007).</div></div><div><h3>Conclusions</h3><div>TAPSE/RVSP ratio was associated with outcomes in COA patients with normal RV systolic function, suggesting that abnormal RV-PA coupling may be present prior to the onset of overt RV systolic function in this population.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100608"},"PeriodicalIF":0.8,"publicationDate":"2025-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144604627","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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