International journal of cardiology. Congenital heart disease最新文献

{"title":"Trends in congenital heart disease mortality in the United States from 1968 to 2022","authors":"Zahra Imran ,&nbsp;Taimor Mohammed Khan ,&nbsp;Saad Ahmed Waqas ,&nbsp;Raheel Ahmed ,&nbsp;Abdul Mannan Khan Minhas","doi":"10.1016/j.ijcchd.2025.100607","DOIUrl":"10.1016/j.ijcchd.2025.100607","url":null,"abstract":"<div><h3>Purpose</h3><div>Congenital heart disease (CHD) remains the leading cause of infant mortality due to birth defects in the United States. We analyzed long-term CHD mortality trends across age, sex, and racial groups from 1968 to 2022.</div></div><div><h3>Methods</h3><div>We extracted CHD mortality data from the CDC WONDER database (1968–2022), using ICD-8/9/10 codes. Age-adjusted mortality rates (AAMRs) and crude mortality rates (CMRs) were calculated per 100,000 population. Joinpoint regression was used to assess temporal trends in mortality, reporting annual percentage changes (APCs) and average APCs (AAPCs) with 95 % confidence intervals (CIs).</div></div><div><h3>Results</h3><div>From 1968 to 2019, 234,658 CHD-related deaths were recorded. Overall AAMR declined from 3.2 (1968) to 0.8 (2019) per 100,000 (AAPC: −2.7 %; 95 % CI: −2.9 to −2.5). Males consistently had higher AAMRs than females. Racial disparities persisted, with slower declines among Black or African American individuals. Infants under 1 year accounted for 56.0 % of CHD deaths and showed the steepest mortality decline (AAPC: −3.2 %). Mortality rates plateaued in recent years.</div></div><div><h3>Conclusions</h3><div>CHD mortality in the U.S. has declined markedly over the past five decades, though progress has slowed since 2009. Persistent disparities by race and sex emphasize the need for equitable access to specialized CHD care and ongoing public health efforts.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100607"},"PeriodicalIF":0.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144614325","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}

{"title":"A novel approach to determining pulmonary valve replacement timing in repaired tetralogy of Fallot patients","authors":"Laura Delaney ,&nbsp;Andrew Browne ,&nbsp;Gruschen R. Veldtman ,&nbsp;Sihong Huang","doi":"10.1016/j.ijcchd.2025.100597","DOIUrl":"10.1016/j.ijcchd.2025.100597","url":null,"abstract":"<div><h3>Background</h3><div>Here we propose a novel approach to determine a patient-specific optimal time for pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot (rTOF) based on a mathematical technique known as optimal stopping theory.</div></div><div><h3>Methods</h3><div>We outline, in a theoretical framework, how we would apply the proposed timing rule for PVR in patients with rTOF. We define each of the model input variables specific to PVR, and we describe how the model can be assessed for its suitability for use in clinical practice to guide timing of PVR in patients with rTOF.</div></div><div><h3>Results</h3><div>In this manuscript we demonstrate, for the first time, an application of a timing rule derived from a mathematical technique known as optimal stopping theory to determine the optimal time to perform PVR in patients with rTOF. Using the technique, we derive two patient-specific-thresholds on clinical status based on patient specific risk factors. PVR is recommended if at the time of assessment, a patient's clinical status lies between the thresholds. Otherwise, they are either too well or too unwell to undergo PVR.</div></div><div><h3>Conclusions</h3><div>Current clinical practice guidelines for timing of PVR in patients with rTOF remains flawed and has since been challenged. We believe the timing rule is a suitable approach to complement current medical guidelines on when to perform PVR in patients with rTOF on a patient-specific level. To determine its suitability for generalized application in clinical practice, we intent to validate it retrospectively using large clinical cohort(s) who have undergone PVR for rTOF.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100597"},"PeriodicalIF":1.2,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144810503","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}

{"title":"Trends in mortality from pulmonary hypertension amongst population with congenital heart disease in the United States from 1999 to 2020: A CDC WONDER analysis","authors":"Allahdad Khan ,&nbsp;Tehreem Asghar ,&nbsp;Kumail Mustafa Ali ,&nbsp;Muhammad Saeed ,&nbsp;Saniya Ishtiaq ,&nbsp;Fatima Ashfaq ,&nbsp;Jamil Nasrallah ,&nbsp;Laiba Yumn ,&nbsp;Usman Ali Akbar ,&nbsp;Peter Collins ,&nbsp;Raheel Ahmed","doi":"10.1016/j.ijcchd.2025.100602","DOIUrl":"10.1016/j.ijcchd.2025.100602","url":null,"abstract":"<div><h3>Background</h3><div>Congenital heart disease (CHD) is an umbrella term describing a variety of structural cardiac malformations at birth. CHD affects approximately 1 % of live births, generating a large adult population with these abnormalities. Pulmonary hypertension (PH) in patients with adult CHD is heterogeneous, based on the type of defect and associated conditions, but is a known cause of adverse outcome.</div></div><div><h3>Method</h3><div>We retrieved death certificate data from the CDC-WONDER database using ICD codes (I27.0, I27.2, I27.8, and I27.9., and Q20-26). Crude mortality rates (CMRs) and age-adjusted mortality rates (AAMRs) per 100,000 persons were calculated. Temporal trends were examined using the annual percent change (APC) and average annual percent change (AAPC) determined by Joinpoint regression<strong>.</strong></div></div><div><h3>Result</h3><div>From 1999 to 2020, an overall declining pattern was observed in the mortality rate. Men and women with congenital heart disease and pulmonary hypertension in the U.S. experienced a statistically significant decline in mortality rates. In terms of races, among White individuals, the decline was the most pronounced. All four U.S. census regions experienced statistically significant declines in mortality due to pulmonary hypertension among individuals with congenital heart disease. The rate of decline was steeper in rural areas compared to urban ones.</div></div><div><h3>Conclusion</h3><div>This study highlights that previously implemented targeted interventions significantly contributed to the reduction of mortality amongst patiemnts with congenital heart disease and pulmonary hypertension in the U.S. Still improvements are required in certain areas, including female gender, Hispanic or Latino, and the Northeast and West regions.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100602"},"PeriodicalIF":0.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144605150","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}

{"title":"Clinical outcomes of children operated for congenital heart diseases in Rwanda: a 14-year retrospective analysis","authors":"Providence Akingeneye ,&nbsp;David J. Bradley ,&nbsp;Joseph Mucumbitsi ,&nbsp;Naphtal Nyilimanzi ,&nbsp;Yves Mutabandama ,&nbsp;Emmanuel K. Rusingiza","doi":"10.1016/j.ijcchd.2025.100605","DOIUrl":"10.1016/j.ijcchd.2025.100605","url":null,"abstract":"<div><h3>Background</h3><div>Congenital heart disease surgery and catheterization for Rwandan children has historically been accomplished with the assistance of visiting external teams and referrals abroad. However, follow-up information on patient-specific clinical outcomes is lacking.</div></div><div><h3>Methods</h3><div>This is a retrospective cross-sectional study of clinical outcomes among Rwandan children under 15 years of age at the time of heart surgery operated between 2006 and 2020. Data from two Rwandan teaching hospitals were collected from paper and digital patient charts.</div></div><div><h3>Results</h3><div>Among the 406 children included in our study, 59.1 % were female; 30.5 % and 28.1 % of the children were underweight and stunted, respectively. Tetralogy of Fallot was the most common cyanotic CHD. Of procedures, VSD repair was the most performed operation and PDA closure represented the majority of therapeutic catheterizations. Postoperatively complications such as pneumonia occurred in 6 %, residual shunt in 17 % and residual mild or greater stenosis in 41 %. The overall mortality was 3.7 %, with sepsis as the leading cause of death. Intensive care unit stay longer than seven days, symptomatic heart failure before surgery, and being underweight were significantly associated with complications. Cardiopulmonary bypass over 60 min was associated with mortality among operated children.</div></div><div><h3>Conclusion</h3><div>Procedural treatment of CHD in Rwandan children was predominantly accomplished by visiting missions during the study period. Of patients with sufficient clinical follow up data, survival was 96 % and complications occurred in 35 %. Nutritional compromise is a correlate of poor outcome. A centralized cardiac registry might mitigate the observed loss of clinical follow up data in 14 % of children.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100605"},"PeriodicalIF":0.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144656881","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}

{"title":"Late diagnosis of severe aortic coarctation in a 65-year-old endurance athlete: Insights into exercise physiology and collateral compensation","authors":"Arundhati Sharma ,&nbsp;Anubhuti Sharma ,&nbsp;Kalyan Chaliki ,&nbsp;Marco Antonio Fernandes ,&nbsp;Francois Marcotte ,&nbsp;Satyajit Reddy","doi":"10.1016/j.ijcchd.2025.100606","DOIUrl":"10.1016/j.ijcchd.2025.100606","url":null,"abstract":"<div><h3>Background</h3><div>Coarctation of the aorta (CoA) is a relatively rare congenital narrowing of the distal aortic arch to proximal descending thoracic aorta, usually diagnosed in infancy or early childhood with upper extremity hypertension as a typical finding. Late detection in asymptomatic, physically active older adults is rare and can be clinically significant.</div></div><div><h3>Case presentation</h3><div>We present the case of a 65-year-old lifelong endurance athlete found to have transient severe left ventricular (LV) systolic dysfunction after completing a marathon. Stress testing revealed a hypertensive response, prompting cardiac MRI, which detected a new severe juxta-ductal CoA with collateralization. The patient underwent successful endovascular stenting with improved blood pressure response and stabilization of LV function.</div></div><div><h3>Conclusion</h3><div>This case underscores the importance of thorough cardiovascular assessment in older athletes and highlights the impact of lifelong exercise in compensating for congenital defects like CoA.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100606"},"PeriodicalIF":0.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144631708","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}

{"title":"Incidence and risk factors for the development of pulmonary arteriovenous malformations after stage 2 palliation","authors":"Lea Behrend ,&nbsp;Thibault Schaeffer ,&nbsp;Muneaki Matsubara ,&nbsp;Jonas Palm ,&nbsp;Teresa Lemmen ,&nbsp;Nicole Piber ,&nbsp;Paul Philipp Heinisch ,&nbsp;Stanimir Georgiev ,&nbsp;Alfred Hager ,&nbsp;Peter Ewert ,&nbsp;Jürgen Hörer ,&nbsp;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 &lt; 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 &lt; 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":"2025-09-01","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}

{"title":"Clinical outcomes after implantation of bioprosthetic valves in adults with congenital heart disease","authors":"Andrea R. Hsu ,&nbsp;Snigdha Karnakoti ,&nbsp;Ahmed T. Abdelhalim ,&nbsp;Jason H. Anderson ,&nbsp;C. Charles Jain ,&nbsp;William R. Miranda ,&nbsp;Elizabeth H. Stephens ,&nbsp;Joseph A. Dearani ,&nbsp;Hartzell V. Schaff ,&nbsp;Heidi M. Connolly ,&nbsp;Alexander C. Egbe","doi":"10.1016/j.ijcchd.2025.100598","DOIUrl":"10.1016/j.ijcchd.2025.100598","url":null,"abstract":"<div><h3>Background</h3><div>Bioprosthetic valves are frequently implanted in adults with congenital heart disease (CHD), but comprehensive data on bioprosthetic valve longevity and outcomes across all valve positions are lacking. The purpose of this study was to determine the incidence and risk factors for bioprosthetic valve dysfunction (BVD) across all valve positions in adults with CHD.</div></div><div><h3>Method</h3><div>BVD was defined as Doppler mean gradient &gt;40 mmHg and/or severe prosthetic/periprosthetic regurgitation for aortic or pulmonary bioprostheses, or Doppler mean gradient &gt;10 mmHg and/or severe prosthetic/periprosthetic regurgitation for mitral or tricuspid bioprostheses.</div></div><div><h3>Results</h3><div>There were 1221 adults with CHD that underwent implantation of a bioprosthetic valve (age 39 ± 16 years, males 617 [51 %]). At the time of valve implantation, 755 (62 %), 325 (27 %), 176 (14 %), and 21 (1.7 %) received a bioprosthetic valve in the pulmonary, tricuspid, aortic, and mitral positions, respectively. The 10-year cumulative incidence of BVD was 28 %, 36 %, 43 %, and 68 % for pulmonary, tricuspid, aortic, and mitral bioprosthetic valves, respectively (p &lt; 0.001). Using the pulmonary valve position as the reference group, valve position (tricuspid [HR 1.26, 95 % CI 1.09–1.58), aortic [HR 1.64, 95 % CI 1.22–1.97], mitral [HR 2.01, 95 % CI 0.83–4.05]) were associated with higher risk of BVD. Younger age was associated higher risk of BVD (HR 0.95, 95 % CI 0.86–0.98 per 5-year increment).</div></div><div><h3>Conclusions</h3><div>These data support the existing evidence that left-sided bioprosthetic valves demonstrate reduced durability in young patients and should be included in counseling and clinical decision-making.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100598"},"PeriodicalIF":0.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144306423","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}

{"title":"Utilizing optical coherence tomography to explore pulmonary arteries and pulmonary vascular disease in Fontan patients","authors":"François Côté ,&nbsp;Leonaes Brahel Tatchinda Kuete ,&nbsp;Réda Ibrahim ,&nbsp;Jean-Claude Tardif ,&nbsp;Colombe Roy ,&nbsp;François-Pierre Mongeon ,&nbsp;Annie Dore ,&nbsp;Blandine Mondésert ,&nbsp;Paul Khairy ,&nbsp;Marie-A. Chaix","doi":"10.1016/j.ijcchd.2025.100599","DOIUrl":"10.1016/j.ijcchd.2025.100599","url":null,"abstract":"<div><h3>Background</h3><div>The Fontan procedure is the palliative intervention of choice for patients with single ventricle physiology. Pulmonary vascular disease (PVD) is a major contributor to Fontan circulatory failure. Our study utilized optical coherence tomography (OCT) imagery in pulmonary arteries to better characterize PVD in Fontan patients.</div></div><div><h3>Methods</h3><div>In a prospective pilot study, OCT images were acquired during cardiac catheterization in a distal left pulmonary artery (PA) branch (A10). The PA intima-media ratio and wall-vessel ratio (i.e., difference between the external elastic membrane area and the luminal area divided by the external elastic membrane area) were measured off-line.</div></div><div><h3>Results</h3><div>A total of 9 Fontan patients (5 women), median age of 23.0 (21.0,38.0) years, were included. All patients exhibited an intima-to-media ratio above 1.0. The ratio exceeded 2.8 in patients with high pulmonary vascular resistance. No difference is observed between patients for the wall-vessel ratio. No relation between time of completion of Fontan and intima-media ratio is identified.</div></div><div><h3>Conclusion</h3><div>OCT shows promise in evaluating PVD in patients with Fontan palliation. PA remodeling is characterized by intimal hyperplasia with medial regression. Further studies are required to determine the role of OCT in guiding treatment decisions and assessing therapeutic responses.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100599"},"PeriodicalIF":0.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144306424","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}

{"title":"GORE® CARDIOFORM ASD occluder thrombus in a patient of rheumatoid arthritis occurred three days after deployment","authors":"Naomi Nakagawa,&nbsp;Yusuke Shigemitsu,&nbsp;Kengo Okamoto","doi":"10.1016/j.ijcchd.2025.100600","DOIUrl":"10.1016/j.ijcchd.2025.100600","url":null,"abstract":"<div><div>A 67-year-old female patient with rheumatoid arthritis that was well suppressed by medications except for steroids had catheter closure of the secundum atrial septal defect (ASD). After the administration of aspirin and intravenous heparin, a 32-mm of GORE® CARDIOFORM ASD occluder was successfully deployed. On postprocedural day 3, transthoracic echocardiography showed mobile echogenic mass of 9 mm on the right side of the device. Intravenous heparin 10 IU/kg/hr was administrated continuously, and clopidogrel was added. On the following day, the mobile mass was not visible. Even though the occurrence rate of device thrombus is not high, device thrombosis can become a major complication of device closure of ASD because it may cause systemic and/or pulmonary embolism. Administration of dual antiplatelet therapy or other anticoagulant therapy should be considered if the patients have a higher risk of thrombosis.</div></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":"21 ","pages":"Article 100600"},"PeriodicalIF":0.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144631709","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}

{"title":"Prognostic value of right ventricular-pulmonary arterial coupling in adults with repaired coarctation of aorta","authors":"Alexander C. Egbe,&nbsp;William R. Miranda,&nbsp;Charles Jain,&nbsp;Heidi M. Connolly,&nbsp;Luke J. Burchill,&nbsp;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 &lt; 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":"2025-09-01","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}