Marwan H. Ahmed , William R. Miranda , Heidi M. Connolly , Snigdha Karnakoti , Patrick S. Kamath , C. Charles Jain , Maan Jokhadar , Luke J. Burchill , Alexander C. Egbe
{"title":"Protein losing enteropathy in adults with congenital heart disease and biventricular circulation","authors":"Marwan H. Ahmed , William R. Miranda , Heidi M. Connolly , Snigdha Karnakoti , Patrick S. Kamath , C. Charles Jain , Maan Jokhadar , Luke J. Burchill , Alexander C. Egbe","doi":"10.1016/j.ijcchd.2024.100502","DOIUrl":"10.1016/j.ijcchd.2024.100502","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000119/pdfft?md5=01381d7edf58f23ea21ce124edc52196&pid=1-s2.0-S2666668524000119-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139966927","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}
Alexander C. Egbe, William R. Miranda, Marwan Ahmed, Snigdha Karnakoti, Sriharsha Kandlakunta, Muhammad Eltony, Marianne Meshreky, Luke J. Burchill, Heidi M. Connolly
{"title":"Incidence and correlates of mortality in adults with congenital heart disease of different age groups","authors":"Alexander C. Egbe, William R. Miranda, Marwan Ahmed, Snigdha Karnakoti, Sriharsha Kandlakunta, Muhammad Eltony, Marianne Meshreky, Luke J. Burchill, Heidi M. Connolly","doi":"10.1016/j.ijcchd.2024.100499","DOIUrl":"10.1016/j.ijcchd.2024.100499","url":null,"abstract":"<div><h3>Background</h3><p>Aging is associated with acquired comorbidities that potentially influence the natural history and outcomes of adults with congenital heart disease (CHD). The purpose of this study was to compare the clinical characteristics, as well as the incidence and correlates of all-cause mortality between different age groups.</p></div><div><h3>Method</h3><p>Adults with CHD were categorized into 3 age groups based on age at baseline encounter: Group 1 (age 18–40 years); Group 2 (age 41–65 years), and Group 3 (age >65 years).</p></div><div><h3>Results</h3><p>Of 5930 patients (age 37 ± 15 years), 3009 (51%), 2422 (41%), and 499 (8%) were in Groups 1, 2 and 3, respectively. Compared to Group 1, patients in Groups 2 and 3 were less likely to have complex CHD, but more likely to have acquired comorbidities, end-organ dysfunction, ventricular systolic dysfunction, and valvular heart disease. Compared to Group 1, Groups 2 and 3 had higher incidence of all-cause mortality (7.2 versus 15.3 versus 47.8 per 1000 patient-years, respectively, p < 0.001), and lower proportion of deaths from cardiovascular causes (87% versus 77% versus 71%, respectively, p < 0.001). Furthermore, the correlates of all-cause mortality were different between the age groups, with acquired comorbidities such as hypertension, coronary artery disease, and hepatorenal dysfunction being associated with mortality in Group 3, while indices of CHD severity such as number of prior cardiac surgery, and presence of complex CHD being associated with all-cause mortality in Group 1.</p></div><div><h3>Conclusions</h3><p>These results suggest the need for management strategies tailored to address the correlates of outcomes in each age group.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000089/pdfft?md5=0a2dc92c0b6ac7f840cefefcaab51f00&pid=1-s2.0-S2666668524000089-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139822035","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}
Magalie Ladouceur, Francisco Javier Ruperti-Repilado, Tobias Rutz
{"title":"Arterial switch operation: A surgical triumph with long-term management challenges","authors":"Magalie Ladouceur, Francisco Javier Ruperti-Repilado, Tobias Rutz","doi":"10.1016/j.ijcchd.2023.100487","DOIUrl":"https://doi.org/10.1016/j.ijcchd.2023.100487","url":null,"abstract":"<div><p>Since the late 1980s, the standard approach for treating D-transposition of the great arteries has been the arterial switch operation (ASO), replacing the Mustard/Senning procedure. Although ASO has shown impressive long-term survival rates, recent case series have revealed late complications such as neoaortic dilation and coronary artery stenosis. New findings emphasize the need for comprehensive evaluation of coronary risk and a deeper understanding of the mechanisms leading to coronary artery stenosis and myocardial ischemia over the long term. Computed tomography angiography (CTA) has unveiled a notable prevalence of abnormal coronary arteries with potential risk of stenosis and myocardial ischemia. Moreover, the progressive dilation of the neoaortic root and the potential for valve regurgitation necessitating intervention warrant serial imaging follow-up. Considering the radiation risks associated with CTA, magnetic resonance imaging emerges as a preferred modality for post-ASO patient assessment. Ongoing research in this field holds the promise of developing improved diagnostic and therapeutic strategies for these patients, thereby enhancing their long-term care</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668523000496/pdfft?md5=cb10c497e8b102c1ae5fffab0d48a679&pid=1-s2.0-S2666668523000496-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140142013","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}
Shuktika Nandkeolyar , Tripti Gupta , D. Marshall Brinkley , Sophoclis Alexopoulos , Emily Firsich , Sally Anne Fossey , Rachel Fowler , Benjamin Frischhertz , Kimberly Harrison , JoAnn Lindenfeld , Martin Montenovo , Dawn Pedrotty , Lynn Punnoose , Aniket Rali , Alexandra Shingina , Kelly Schlendorf , Hasan Siddiqi , Ashish Shah , Sandip Zalawadiya , Mark Wigger , Jonathan N. Menachem
{"title":"Rejection in the setting of combined Heart and Liver Transplantation","authors":"Shuktika Nandkeolyar , Tripti Gupta , D. Marshall Brinkley , Sophoclis Alexopoulos , Emily Firsich , Sally Anne Fossey , Rachel Fowler , Benjamin Frischhertz , Kimberly Harrison , JoAnn Lindenfeld , Martin Montenovo , Dawn Pedrotty , Lynn Punnoose , Aniket Rali , Alexandra Shingina , Kelly Schlendorf , Hasan Siddiqi , Ashish Shah , Sandip Zalawadiya , Mark Wigger , Jonathan N. Menachem","doi":"10.1016/j.ijcchd.2024.100504","DOIUrl":"https://doi.org/10.1016/j.ijcchd.2024.100504","url":null,"abstract":"<div><h3>Introduction</h3><p>Each year the number of combined heart-liver transplants (HLT) increases, with two distinct patient populations proceeding down this pathway. The first are patients with congenital heart disease (CHD), most commonly single ventricle patients palliated with Fontan. The second group are those with long standing congestive hepatopathy, amyloidosis, hemochromatosis, or alcohol induced myopathies and liver disease.</p><p>One argument for HLT has been the low rate of rejection even among sensitized patients, with reported rejection rates ranging from 0% to 31%. Historically, those with CHD have been highly sensitized which in some cases may prevent or at least delay transplantation. As such, a recent consensus statement by Emamaulee et al. suggest that “there may be an immunological benefit to proceed with HLT with significantly fewer acute cellular and humoral rejection episodes”. The aim of this study is to demonstrate that HLT patients remain at risk for rejection and have required treatment for it.</p></div><div><h3>Results</h3><p>There were 15 patients who underwent HLT from January 2017 to February 2022. Of the four patients who did not have CHD, none were considered sensitized, and all underwent induction with basiliximab per our institutional protocol. One of these had rejection. Rejection episodes were identified in four of the 11 CHD patients (36%) patients.</p></div><div><h3>Conclusions</h3><p>In our study of 15 HLT, including 11 CHD patients (73% denied transplant at ≥ 1 center) demonstrated a higher rate of rejection than previously reported. While theoretically, HLT may mitigate the likelihood of rejection, the risk still exists, and patients benefit from close monitoring commensurate with single organ transplant.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000132/pdfft?md5=5a439ae4650f044345c9785cf054bd35&pid=1-s2.0-S2666668524000132-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140062388","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}
Omar A. Abozied, Abhishek J. Deshmukh, Ahmed Younis, Marwan Ahmed, Luke Burchill, C. Charles Jain, William R. Miranda, Malini Madhavan, Heidi M. Connolly, Alexander C. Egbe
{"title":"Right atrial reverse remodeling and risk of atrial arrhythmias after surgical pulmonary valve replacement","authors":"Omar A. Abozied, Abhishek J. Deshmukh, Ahmed Younis, Marwan Ahmed, Luke Burchill, C. Charles Jain, William R. Miranda, Malini Madhavan, Heidi M. Connolly, Alexander C. Egbe","doi":"10.1016/j.ijcchd.2024.100497","DOIUrl":"10.1016/j.ijcchd.2024.100497","url":null,"abstract":"<div><h3>Background</h3><p>Right atrial (RA) dysfunction and atrial arrhythmias are relatively common in adults with repaired tetralogy of Fallot. The purpose of this study was to determine whether RA function improved after surgical pulmonary valve replacement (PVR), and the association between postoperative RA reverse remodeling and late postoperative atrial arrhythmias.</p></div><div><h3>Method</h3><p>RA reverse remodeling (ΔRA reservoir strain based speckle tracking echocardiography) was calculated as: ([postoperative RA reservoir strain – preoperative RA reservoir strain]/preoperative RA reservoir strain)x100. Optimal RA reverse remodeling was defined as ΔRA reservoir strain >15%.</p></div><div><h3>Results</h3><p>Of 411 patients (age 36 ± 13 years), preoperative RA reservoir strain was 31 ± 13%, postoperative RA reserve remodeling was 13 ± 9%, and 171 (42%) had optimal RA reserve remodeling. Preoperative RA reservoir strain (β±SE 1.12 ± 0.09, p < 0.001) was associated with postoperative RA reverse remodeling on multivariable analysis. Preoperative RA reservoir strain ≥33% predicted optimal postoperative RA reverse remodeling (area under the curve 0.792).</p><p>ΔRA reservoir strain was associated with postoperative atrial arrhythmias (HR 0.91, 95%CI 0.86–0.96, p = 0.004), on multivariable analysis. Compared to patients with preoperative RA reservoir strain <33% (n = 242, 59%), those with RA reservoir strain ≥33% (n = 169, 41%) had more robust RA reverse remodeling (ΔRA reverse remodeling 19 ± 11% versus 7 ± 10%, p < 0.001), and lower incidence of atrial arrhythmias (1.1% versus 2.9%, p = 0.003).</p></div><div><h3>Conclusions</h3><p>Preoperative RA reservoir strain was associated with RA reverse remodeling after PVR, and in turn, postoperative atrial arrhythmia. These results suggest that RA strain indices could be used to determine optimal timing for PVR in order to reduce the risk of atrial arrhythmia.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000065/pdfft?md5=601fe7d0ff8764b12cdb822ae1611075&pid=1-s2.0-S2666668524000065-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139811909","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Marching alongside our patients on the enduring journey of the Italian society of pediatric and congenital cardiology (SICP)","authors":"Massimo Chessa, Gabriele Rinelli, Silvia Favilli","doi":"10.1016/j.ijcchd.2024.100496","DOIUrl":"https://doi.org/10.1016/j.ijcchd.2024.100496","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000053/pdfft?md5=c060e8860944cabc58042682f3967f0e&pid=1-s2.0-S2666668524000053-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140103354","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Cardiac devices in the adult congenital population; A blessing and a curse","authors":"Matthew O'Connor, Tom Wong","doi":"10.1016/j.ijcchd.2024.100493","DOIUrl":"10.1016/j.ijcchd.2024.100493","url":null,"abstract":"","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000028/pdfft?md5=195264cb4913b0a5b02ca2ce6fa1b019&pid=1-s2.0-S2666668524000028-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139871649","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}
Lauren Lastinger , Marc Lee , Lauren Hassen , Omer Cavus , Saurabh Rajpal , Jeremy P. Moore , May Ling Mah , Elisa A. Bradley
{"title":"Sudden death in adults with repaired coarctation of the aorta: A case for sex-based risk factors","authors":"Lauren Lastinger , Marc Lee , Lauren Hassen , Omer Cavus , Saurabh Rajpal , Jeremy P. Moore , May Ling Mah , Elisa A. Bradley","doi":"10.1016/j.ijcchd.2024.100500","DOIUrl":"https://doi.org/10.1016/j.ijcchd.2024.100500","url":null,"abstract":"<div><h3>Background</h3><p>Sudden cardiac death (SCD) is an important risk for adults with repaired coarctation of the aorta (rCoA). We aimed determine if there are clinical risk factors for SCD in adults with rCoA.</p></div><div><h3>Methods and results</h3><p>SCD events and clinical data from all adults with rCoA at a tertiary care center (2007–2017) were evaluated. In 167 adults with rCoA (39 ± 11 years old, 75 (45%) female) SCD occurred in 8 (5%) (vs. age-matched adults 0.9%). Those with SCD demonstrated significant QTc prolongation (QTc: 479 ± 16 vs. 434 ± 30 msec, p < 0.001). Overall, adults with rCoA and a prolonged sex-normative QTc interval had a 12-fold increased risk of SCD (ᵡ<sup>2</sup> (1) = 12.3, p < 0.001), with men sustaining SCD at younger ages (42 ± 13 years vs. women 60 ± 10 years, p < 0.05). Multiple logistic regression modeling demonstrated that prolonged QTc selectively advanced risk for SCD in men only (ᵡ<sup>2</sup> QTc prolongation 8.46, p < 0.005 and ᵡ<sup>2</sup> age 0.29, p = 0.587), whereas in women, age was associated with SCD risk (ᵡ<sup>2</sup> QTc prolongation 2.84, p = 0.092 and ᵡ<sup>2</sup> age 7.81, p = 0.005). Non-sustained ventricular tachycardia, ventricular dysfunction, and myocardial fibrosis did not significantly impact SCD risk.</p></div><div><h3>Conclusions</h3><p>There is an unanticipated high burden of SCD in adults with rCoA, occurring in men at younger age than women, suspicious for primary electrophysiologic dysfunction. Future investigation of sex-specific SCD risk in rCoA is important to better understand this disease and its late phenotype.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000090/pdfft?md5=7e0c04ba5221cb10eaa83d1c6111cf01&pid=1-s2.0-S2666668524000090-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140066966","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}
Ayse-Gül Öztürk , Mikael Dellborg , Anna Damlin , Kok Wai Giang , Zacharias Mandalenakis , Peder Sörensson
{"title":"Long-term survival in patients with univentricular heart: A nationwide, register-based cohort study","authors":"Ayse-Gül Öztürk , Mikael Dellborg , Anna Damlin , Kok Wai Giang , Zacharias Mandalenakis , Peder Sörensson","doi":"10.1016/j.ijcchd.2024.100503","DOIUrl":"https://doi.org/10.1016/j.ijcchd.2024.100503","url":null,"abstract":"<div><h3>Background</h3><p>Children with univentricular heart (UVH) have a limited life expectancy without early treatment. Long-term survival in UVH, in an unselected nationwide cohort, is unclear.</p></div><div><h3>Objectives</h3><p>To determine long-term survival in patients with UVH including non-operated patients compared with a control population in Sweden.</p></div><div><h3>Methods</h3><p>Patients with UVH born between 1970 and 2017 were identified from the National Registers and were matched for birth year and sex with 10 individuals without congenital heart disease. Follow-up was from birth until death, transplantation, or the end of study. Mortality risk was estimated by Cox proportional regression models and Kaplan–Meier survival analysis.</p></div><div><h3>Results</h3><p>We included 5075 patients with UVH including 758 (14.9%) patients with hypoplastic left heart syndrome (HLHS), and 50,620 matched controls. Median follow-up time was 13.6 (IQR 0.7; 26.8) years. The hazard ratio for death in patients with UVH was 53.0 (95% confidence interval, 48.0–58.6), and for HLHS, 163.5 (95% CI, 124.3–215.2). In patients with HLHS, 84% of those who were born between 1982 and 1993 died or had transplantation during the first year of life compared with 29% born between 2006 and 2017. In patients with UVH without HLHS, death/transplantation in the first year of life declined from 36% in those born between 1970 and 1981 to 8.7% in those born between 2006 and 2017.</p></div><div><h3>Conclusions</h3><p>The risk of mortality was >50 times higher in patients with UVH than in controls. The survival rate increased with a later decade of birth but was still <75% in patients born with HLHS.</p></div>","PeriodicalId":73429,"journal":{"name":"International journal of cardiology. Congenital heart disease","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666668524000120/pdfft?md5=a9786dd56ad351d8f70b18688d3b797e&pid=1-s2.0-S2666668524000120-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139935649","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}