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Artificial intelligence (AI) is revolutionizing healthcare by offering innovative solutions for diagnosis, treatment, and patient management. Only recently has the field of pediatric cardiology begun to explore the use of deep learning methods to analyze electrocardiogram (ECG) data, aiming to enhance diagnostic accuracy, expedite workflows, and improve patient outcomes. This review examines the current state of AI-enhanced ECG interpretation in pediatric cardiology applications, drawing insights from adult AI-ECG research given the progress in this field. It describes a broad range of AI methodologies, investigates the unique challenges inherent in pediatric ECG analysis, reviews the current state of the literature in pediatric AI-ECG, and discusses potential future directions for research and clinical practice. While AI-ECG applications have demonstrated considerable promise, widespread clinical adoption necessitates further research, rigorous validation, and careful consideration of equity, ethical, legal, and practical challenges.

Congenital heart diseases are the most common congenital malformations worldwide and represent one of the leading causes of neonatal death, in addition to the significant use of human and financial resources by health systems. The purpose of this document is to support the implementation of neonatal screening for critical congenital heart diseases using pulse oximetry according to the different geographical altitudes of Peru. This technology is widely used worldwide and has high sensitivity, specificity, and cost-effectiveness. At many latitudes, it has led to better survival in this group of patients and in the neonatal population in general since its use in the early detection of sepsis, pneumonia, and other conditions that affect the oxygenation of the newborn. Neonatal screening for critical congenital heart disease is applicable at all levels of healthcare at a national level, and its implementation must be a priority to improve neonatal health., Competing Interests: Conflictos de interés: Ninguno

Social determinants of health (SDoH) are the economic, social, environmental, and psychosocial factors that influence health. Adolescents and young adults with congenital heart disease (CHD) require lifelong cardiology follow-up and therefore coordinated transition from pediatric to adult healthcare systems. However, gaps in care are common during transition, and they are driven in part by pervasive disparities in SDoH, including race, ethnicity, socioeconomic status, access to insurance, and remote location of residence. These disparities often coexist and compound the challenges faced by patients and families. For example, Black and Indigenous individuals are more likely to be subject to systemic racism and implicit bias within healthcare and other settings, to be unemployed and poor, to have limited access to insurance, and to have a lower likelihood of transfer of care to adult CHD specialists. SDoH also are associated with acquired cardiovascular disease, a comorbidity that adults with CHD face. This review summarizes existing evidence regarding the impact of SDoH on the transition to adult care and proposes strategies at the individual, institutional, and population and/or system levels. to reduce inequities faced by transition-age youth. These strategies include routinely screening for SDoH in clinical settings with referral to appropriate services, providing formal transition education for all transition-age youth, including training on navigating complex medical systems, creating satellite cardiology clinics to facilitate access to care for those who live remote from tertiary centres, advocating for lifelong insurance coverage where applicable, mandating cultural-sensitivity training for providers, and increasing the diversity of healthcare providers in pediatric and adult CHD care., (Copyright © 2024 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Cardiovascular disease affects 37% of Hispanic women and is the leading cause of death among Hispanic women in the United States. Hispanic women have a higher burden of cardiovascular risk factors, are disproportionally affected by social determinants of health, and face additional barriers related to immigration, such as discrimination, language proficiency, and acculturation. Despite this, Hispanic women show lower rates of cardiovascular disease and mortality compared with non-Hispanic White women. However, this "Hispanic paradox" is challenged by recent studies that account for the diversity in culture, race, genetic background, country of origin, and social determinants of health within Hispanic subpopulations. This review provides a comprehensive overview of the cardiovascular risk factors in Hispanic women, emphasizing the role of social determinants, and proposes a multipronged approach for equitable care., Competing Interests: Funding Support and Author Disclosures Dr Quesada has received funding from the National Institutes of Health (NIH) National Heart, Lung, and Blood Institute (K23 23HL151867). Dr Rodriguez was funded by grants from the NIH National Heart, Lung, and Blood Institute (1K01HL144607), the American Heart Association/Harold Amos Faculty Development program, and the Doris Duke Charitable Foundation (Grant #2022051). Dr Briller has received funding for the REBIRTH trial PTE Federal Award to University of Pittsburgh 1UG3HL153847-01A1 Subaward: UIC AWD00004314 (137168-1) Steering Committee. Dr Ogunniyi has received institutional research grant support from AstraZeneca, Boehringer Ingelheim, LabCorp Drug Development, and Pfizer. Dr Briller is an unpaid consultant for the Illinois Maternal Mortality Committee and is on the steering committee and site investigator for the NIH funded REBIRTH trial of bromocriptine in peripartum cardiomyopathy. Dr Mattina has been a paid consultant for Zoll Medical. Dr Rodriguez has consulting relationships with Healthpals, Novartis, Novo Nordisk, and AstraZeneca outside the submitted work. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

There remains a paucity of investigational data about disparities in hospice services in people with non-cancer diagnoses, specifically in heart failure (HF). Black patients with advanced HF have been disproportionally affected by health care services inequities but their outcomes after hospice enrollment are not well studied. We aimed to describe race-specific outcomes in patients with advanced HF who were enrolled in hospice services. We obtained the data from PubMed, Scopus, and Embase for all investigations published until January 11, 2023. All studies that reported race-specific outcomes after hospice enrollment in patients with advanced HF were included. Of the 1,151 articles identified, 5 studies (n = 24,899) were considered for analysis involving a sample size ranging from 179 to 11,754 patients. Black patients had an increased risk of readmission (odds ratio 1.55, 95% confidence interval [CI] 1.34 to 1.79, I 2 0%) and discharge (odds ratio 1.75, 95% CI 1.53 to 1.99, I 2 0%) compared with White patients. Moreover, Black patients have a nonsignificant lower risk of mortality compared with White patients (relative risk 0.67, 95% CI 0.43 to 1.05, I 2 90%). In conclusion, this study showed that Black patients with advanced HF receiving hospice care have a higher risk of readmission and discharge compared with White patients., Competing Interests: Declaration of competing interest The authors have no competing interests to declare., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Fontan pathway obstruction is a potentially serious complication characterized by an anatomical or functional narrowing anywhere in the cavo-pulmonary pathways. Here, we report the first case in the literature where an innovative Fontan conduit rehabilitation procedure with intravascular lithotripsy was used achieving a dramatic increase in the pathway size., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Background: Neonatal pulse oximetry screening (POS) algorithms for critical congenital heart disease (CCHD) have contributed towards decreasing neonatal mortality but cannot be applied at high altitudes. New POS algorithms at high altitudes are needed., Methods: This observational, prospective study included newborns born at different altitudes from 0 to 4380 meters above the sea level in Peru. Healthy newborns underwent neonatal preductal and postductal oximetry, echocardiography and telephonic follow-up up to 12 months of age. Newborns with CCHD underwent preductal and postductal oximetry at the time of telemedicine evaluation while located at the high-altitude hospital where they were born, and their diagnoses were confirmed with echocardiography locally or after arriving to the referral center. Two new algorithms were designed using clinically accepted neonatal oximetry cutoffs or the 5th and 10th percentiles for preductal and postductal oximetry values., Results: A total of 502 healthy newborns and 15 newborns with CCHD were enrolled. Echocardiography and telephonic follow-up were completed in 227 (45%) and 330 healthy newborns (65%), respectively. The algorithm based on clinically accepted cutoffs had a sensitivity of 92%, specificity of 73% and false positive rate of 27% The algorithm based on the 5th and 10th percentiles had a sensitivity of 80%, specificity of 88% and false positive rate of 12%., Conclusions: Two algorithms that detect CCHD at different altitudes had adequate performance but high false positive rates., (© 2024. The Author(s), under exclusive licence to Springer Nature America, Inc.)

Background: The molecular mechanisms underlying Fontan-associated liver disease (FALD) remain largely unknown., Objectives: This study aimed to assess intrahepatic transcriptomic differences among patients with FALD according to the degree of liver fibrosis and clinical outcomes., Methods: This retrospective cohort study included adults with the Fontan circulation. Baseline clinical, laboratory, imaging, and hemodynamic data as well as a composite clinical outcome (CCO) were extracted from medical records. Patients were classified into early or advanced fibrosis. RNA was isolated from formalin-fixed paraffin-embedded liver biopsy samples; RNA libraries were constructed with the use of an rRNA depletion method and sequenced on an Illumina Novaseq 6000. Differential gene expression and gene ontology analyses were performed with the use of DESeq2 and Metascape., Results: A total of 106 patients (48% male, median age 31 years [IQR: 11.3 years]) were included. Those with advanced fibrosis had higher B-type natriuretic peptide levels and Fontan, mean pulmonary artery, and capillary wedge pressures. The CCO was present in 23 patients (22%) and was not predicted by advanced liver fibrosis, right ventricular morphology, presence of aortopulmonary collaterals, or Fontan pressures on multivariable analysis. Samples with advanced fibrosis had 228 upregulated genes compared with early fibrosis. Samples with the CCO had 894 upregulated genes compared with those without the CCO. A total of 136 upregulated genes were identified in both comparisons and were enriched in cellular response to cytokine stimulus or oxidative stress, VEGFA-VEGFR2 signaling pathway, TGF-β signaling pathway, and vasculature development., Conclusions: Patients with FALD and advanced fibrosis or the CCO exhibited upregulated genes related to inflammation, congestion, and angiogenesis., Competing Interests: Funding Support and Author Disclosures Dr Bravo-Jaimes was supported by the Adult Congenital Heart Association Research Grant 2021. Dr Klomhaus was supported by the National Institutes of Health (NIH)/National Center for Advancing Translational Science UCLA CTSI grant no. UL1TR001881. Dr Böstrom was supported by NIH/National Heart, Lung, and Blood Institute grant nos. HL81397 and HL154548. Dr Aboulhosn was supported by the Streisand/American Heart Association Endowed Chair in Cardiology. Dr Kaldas was supported by the Kelly Lee Tarantello Endowed Chair in Integrative Liver Transplantation. The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Achieving health equity in populations with congenital heart disease (CHD) requires recognizing existing disparities throughout the lifespan that negatively and disproportionately impact specific groups of individuals. These disparities occur at individual, institutional, or system levels and often result in increased morbidity and mortality for marginalized or racially minoritized populations (population subgroups (e.g., ethnic, racial, social, religious) with differential power compared to those deemed to hold the majority power in the population). Creating actionable strategies and solutions to address these health disparities in patients with CHD requires critically examining multilevel factors and health policies that continue to drive health inequities, including varying social determinants of health (SDOH), systemic inequities, and structural racism. In this comprehensive review article, we focus on health equity solutions and health policy considerations for minoritized and marginalized populations with CHD throughout their lifespan in the United States. We review unique challenges that these populations may face and strategies for mitigating disparities in lifelong CHD care. We assess ways to deliver culturally competent CHD care and to help lower-health-literacy populations navigate CHD care. Finally, we review system-level health policies that impact reimbursement and research funding, as well as institutional policies that impact leadership diversity and representation in the workforce.

Background: Takotsubo syndrome (TS) during pregnancy and postpartum is rare but may lead to significant maternal and fetal morbidity. We compared clinical characteristics and prognosis according to [a] timing of presentation (pregnancy vs post-partum) and [b] modes of delivery (cesarean section vs vaginal delivery)., Methods: Systematic review of articles published in PubMed, Scopus, Embase, and Medline databases from inception to July 30, 2023. Patient demographics, obstetric, electrocardiographic, laboratory, echocardiographic characteristics, and prognosis were summarized descriptively., Results: An initial database search identified 2162 articles, of which 81 studies were included in this review. TS during pregnancy can have emotional, obstetric, and metabolic triggers and has a higher proportion of adverse fetal outcomes when compared with women who developed TS postpartum. Women with TS after cesarean section had an earlier onset and higher proportion of anesthesia use when compared with those who developed TS after vaginal delivery. There were no differences regarding the degree of systolic dysfunction or the need for advanced therapies, including ventilator support, intra-aortic balloon pump, and extracorporeal membrane oxygenation among groups., Conclusions: TS is associated with various triggers and adverse fetal outcomes when it develops during pregnancy than in the postpartum period. TS occurs more rapidly and with a more aggressive course after cesarean section than after vaginal delivery., Competing Interests: Declaration of Competing Interest the authors declare that they have no competing interests., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Individuals living with tetralogy of Fallot require lifelong specialized congenital heart disease care to monitor for and manage potential late complications. However, access to cardiology care remains a challenge for many patients, as does access to mental health services, dental care, obstetrical care, and other specialties required by this population. Inequities in health care access were highlighted by the COVID-19 pandemic and continue to exist. Paradoxically, many social factors influence an individual's need for care, yet inadvertently restrict access to it. These include sex and gender, being a member of a racial or ethnic historically excluded group, lower educational attainment, lower socioeconomic status, living remotely from tertiary care centres, transportation difficulties, inadequate health insurance, occupational instability, and prior experiences with discrimination in the health care setting. These factors may coexist and have compounding effects. In addition, many patients believe that they are cured and unaware of the need for specialized follow-up. For these reasons, lapses in care are common, particularly around the time of transfer from paediatric to adult care. The lack of trained health care professionals for adults with congenital heart disease presents an additional barrier, even in higher income countries. This review summarizes challenges regarding access to multiple domains of specialized care for individuals with tetralogy of Fallot, with a focus on the impact of social determinants of health. Specific recommendations to improve access to care within Canadian and American systems are offered., (© 2023 Published by Elsevier Inc. on behalf of the Canadian Cardiovascular Society.)

Background: The molecular mechanisms underlying Fontan associated liver disease (FALD) remain largely unknown. We aimed to assess intrahepatic transcriptomic differences among patients with FALD according to the degree of liver fibrosis and clinical outcomes., Methods: This retrospective cohort study included adults with the Fontan circulation at the Ahmanson/UCLA Adult Congenital Heart Disease Center. Clinical, laboratory, imaging and hemodynamic data prior to the liver biopsy were extracted from medical records. Patients were classified into early (F1-F2) or advanced fibrosis (F3-F4). RNA was isolated from formalin-fixed paraffin embedded liver biopsy samples; RNA libraries were constructed using rRNA depletion method and sequencing was performed on Illumina Novaseq 6000. Differential gene expression and gene ontology analyses were carried out using DESeq2 and Metascape. Medical records were comprehensively reviewed for a composite clinical outcome which included decompensated cirrhosis, hepatocellular carcinoma, liver transplantation, protein-losing enteropathy, chronic kidney disease stage 4 or higher, or death., Results: Patients with advanced fibrosis had higher serum BNP levels and Fontan, mean pulmonary artery and capillary wedge pressures. The composite clinical outcome was present in 23 patients (22%) and was predicted by age at Fontan, right ventricular morphology and presence of aortopulmonary collaterals on multivariable analysis. Samples with advanced fibrosis had 228 up-regulated genes compared to early fibrosis. Samples with the composite clinical outcome had 894 up-regulated genes compared to those without it. A total of 136 up-regulated genes were identified in both comparisons and these genes were enriched in cellular response to cytokine stimulus, response to oxidative stress, VEGFA-VEGFR2 signaling pathway, TGF-beta signaling pathway, and vasculature development., Conclusions: Patients with FALD and advanced liver fibrosis or the composite clinical outcome exhibit up-regulated genes including pathways related to inflammation, congestion, and angiogenesis. This adds further insight into FALD pathophysiology., Competing Interests: Disclosures: The authors declare no conflicts of interest.

Racial disparities in cardiovascular disease are unjust, systematic, and preventable. Social determinants are a primary cause of health disparities, and these include factors such as structural and overt racism. Despite a number of efforts implemented over the past several decades, disparities in cardiovascular disease care and outcomes persist, pervading more the outpatient rather than the inpatient setting, thus putting racial and ethnic minority groups at risk for hospital readmissions. In this article, we discuss differences in care and outcomes of racial and ethnic minority groups in both of these settings through a review of registries. Furthermore, we explore potential factors that connote a revolving door phenomenon for those whose adverse outpatient environment puts them at risk for hospital readmissions. Additionally, we review promising strategies, as well as actionable items at the policy, clinical, and educational levels aimed at locking this revolving door.

Background: Malignancy is known to be a major cause of death in adult congenital heart disease (ACHD). However, data regarding cardiovascular and cancer-related outcomes in ACHD are lacking., Methods: We conducted a retrospective single-center cohort study comprising patients with ACHD and malignancy. The primary outcome was all-cause mortality. Key secondary outcomes included major adverse cardiovascular and cerebrovascular events (MACCE), cardiotoxicity events and consequent cancer therapy modifications., Results: Sixty-eight patients with ACHD and a history of cancer were included in the study. 82% of patients had moderate or great ACHD anatomic complexity. Over a median follow-up of 5 years after cancer diagnosis, 16 (24%) patients died, with 69% of deaths being due to cancer. Univariate predictors of mortality were baseline arrhythmia (OR 3.82, 95% CI 1.15-12.67, p = 0.028), baseline diuretic therapy (OR 3.54, 95% CI 1.04-12.04, p = 0.044) and advanced cancer stage at diagnosis (OR 2.37, 95% CI 1.32-4.25, p = 0.004). MACCE occurred in 40 (59%) patients and was independently predicted by baseline diuretic requirement (OR 9.91, 95% CI 1.12-87.85, p = 0.039). A 14% incidence of cardiotoxicity was seen; 3 patients needed modification and 1 patient needed temporary interruption of cancer therapy for 2 weeks., Conclusions: Considerable mortality occurred in this cohort of patients with ACHD and cancer; most deaths were cancer-related. A high rate of MACCE was observed, yet rates of obligatory modification of cancer therapy due to cardiotoxicity were low., (© 2022. The Author(s).)

In the past few decades, the accelerated improvement in technology has allowed the development of new and effective coronary and structural heart disease interventions. There has been inequitable patient access to these advanced therapies and significant disparities have affected patients from low socioeconomic positions. In the US, these disparities mostly affect women, black and hispanic communities who are overrepresented in low socioeconomic. Other adverse social determinants of health influenced by structural racism have also contributed to these disparities. In this article, we review the literature on disparities in access and use of coronary and structural interventions; delineate the possible reasons underlying these disparities; and highlight potential solutions at the government, healthcare system, community and individual levels., Competing Interests: Disclosure: The authors have no conflicts of interest to declare., (Copyright © 2022, Radcliffe Cardiology.)

Critical congenital heart disease (CCHD) represents a challenging problem in global health equity due to the need for specialized surgical or transcatheter intervention within the first year of life. CCHD screening using pulse oximetry (POS) has led to significant improvements in mortality due to early referral and intervention. Andean America represents one of the few regions in the world with increasing CHD deaths and variable POS implementation. In this manuscript, we review the current state of CCHD in Andean America, the challenges and opportunities for developing new POS algorithms that account for high-altitude physiology, data on regional cost-effectiveness supporting POS implementation and outline future directions to achieve equity in CHD care in this region., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2022 The Authors.)

There is evidence for the lower use of percutaneous mechanical circulatory support (pMCS) in women. We aimed to determine (1) whether gender differences exist regarding in-hospital mortality, hospital course, and procedures; (2) whether socio-demographic and treatment-related factors were associated with these differences. Using the National Inpatient Sample, we collected the International Classification of Diseases, Ninth Revision, Clinical Modification codes for cardiogenic shock (CS) because of acute myocardial infarction AMI or acutely decompensated advanced heart failure and included intra-aortic balloon pump, Impella or Tandem Heart percutaneous ventricular assist devices (pVADs), extracorporeal membrane oxygenation. Demographics, co-morbidities, in-hospital course and procedures were recorded, and the Charlson Co-morbidity Index was calculated. Multivariable hierarchical logistic regression analysis and additional sensitivity analyses were performed. We identified 376,116 cases of CS because of acute myocardial infarction or acutely decompensated advanced heart failure, of which 113,305 required pMCS. Women were more likely to be older, non-White, insured by Medicare, and have a higher burden of co-morbidities and higher Charlson Co-morbidity Index. pMCS devices were inserted in 35,516 women (24.9%) and 77,789 men (33.3%). Women were less likely to receive pVAD or pulmonary artery (PA) catheters. Blood transfusions and acute respiratory failure were more common in women than men. Women had 15% higher in-hospital mortality and in a multivariate analysis, women, older age, having no insurance, diabetes mellitus, chronic kidney disease, cerebrovascular disease, peripheral arterial disease, longer time to pMCS insertion, receiving PA catheter, pVAD or extracorporeal membrane oxygenation and having cardiac arrest were associated with higher in-hospital mortality. In conclusion, women requiring pMCS support had a higher co-morbidity load, in-hospital mortality, acute respiratory failure, blood transfusions, and lower PA catheter use. Studies addressing early gender-specific interventions in CS are needed to reduce these differences., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Anomalous coronary origin from the left ventricular outflow tract (LVOT) is an exceedingly rare condition thought to be associated with the bicuspid aortic valve (BAV). While the malignant presentation of this entity has been described, its pathophysiology and diagnostic evaluation are poorly understood., Case Summary: A 33-year-old woman status post Ross procedure in childhood for congenital aortic stenosis due to BAV with presumed common origin of right and left coronary arteries based on single coronary ostium seen on aortic valve inspection, presented with symptomatic pulmonary regurgitation and stenosis. Invasive left coronary angiography revealed retrograde filling of the right coronary artery (RCA) with systolic washout of contrast indicating a patent RCA ostium. No RCA ostium was found on aortic root injection, but an injection into the LVOT revealed an RCA ostium below the aortic valve. Selective RCA angiography revealed pulsatile antegrade flow down the RCA occurring during systole. There was no anatomic RCA stenosis. We proceeded with valve-in-valve TcPVR. The patient had significant improvement of symptoms and RCA reimplantation was hence deferred., Discussion: This case is the first of an anomalous coronary artery arising from the LVOT diagnosed in a patient after the Ross procedure. Our angiograms shed light on the unusual physiology of coronary filling during systole and ischaemia arising from inadequate perfusion gradient between the left ventricle and the coronary during systole, leading to collateralization despite the lack of anatomic stenosis. We urge consideration of this potentially malignant entity in any symptomatic patient, especially with concomitant BAV., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Competing Interests: None declared.

As more adults survive with congenital heart disease, the need to better understand the long-term complications, and comorbid disease will become increasingly important. Improved care and survival into the early and late adult years for all patients equitably requires accurate, timely, and comprehensive data to support research and quality-based initiatives. National data collection in adult congenital heart disease will require a sound foundation emphasizing core ethical principles that acknowledge patient and clinician perspectives and promote national collaboration. In this document we examine these foundational principles and offer suggestions for developing an ethically responsible and inclusive framework for national ACHD data collection.

Adults with congenital heart disease (ACHD) experience worse waitlist outcomes and higher early posttransplant mortality compared to non-ACHD patients. On October 18, 2018; the UNOS donor heart allocation system was redesigned giving unique listing status to ACHD patients. The impact of this change on outcomes in transplant-listed patients is unstudied. Using the Scientific Registry of Transplant Recipients (SRTR) we compared ACHD patients listed for the first-time for heart transplantation from two eras of equal duration. We analyzed waitlist outcomes, posttransplant mortality and length of stay among ACHD patients in both eras and between ACHD and non-ACHD patients in the new era. Of 12 723 listed patients, 535 had ACHD (293 in the new era) and 12 188 did not (6258 in the new era). A total of 163 (56%) ACHD patients in the new era versus 150 (62%) in the prior era were transplanted; 11 (3.8%) versus 15 (6.2%) died on the waitlist; 32 (11%) versus 35 (14%) were delisted and 15 (9.2%) versus 19 (12.7%) died within 30 days of transplant, respectively. The new UNOS donor heart allocation system improved waitlist time and decreased the proportion not transplanted during the first 300 days after listing among ACHD patients without altering early posttransplant outcomes or significantly changing the gap in outcomes compared to non-ACHD patients., (© 2021 The American Society of Transplantation and the American Society of Transplant Surgeons.)

Competing Interests: Competing interests: None declared.

Introduction: Congenital heart defects (CHD) are the most frequent congenital birth defects, affecting approximately 1% of newborns and constituting the main cause of death among all congenital malformations. The prevalence of CHD in Andean America is heterogeneous and in Peru the burden of CHD is unknown., Objective: To describe the trends of CHD outpatient visits and mortality and their variation according to sex, age and altitude levels., Methods: We performed a secondary data analysis of CHD outpatient visits and mortality using publicly available information from the Peruvian Ministry of Health national registries from 2000 to 2020., Results: There was a statistically significant increase in CHD outpatient visits of 20.4 over time. Pediatric patients, those who lived at low altitude (≤2500 ​m above sea level) and those who lived in the capital of Peru had a higher average number of outpatient visits per 100 ​000 population. CHD-related mortality increased by 0.31 per million population per year. Although this trend was not statistically significant overall, pediatric patients experienced higher mortality than adults., Conclusion: In Peru, the outpatient CHD volume has increased significantly over the last two decades, however CHD-related mortality has remained constant. Children in the first decade of life are the predominant group in both, outpatient CHD volume and mortality., (© 2022 The Authors.)

Background: This study aimed to assess sex and racial differences related to high-density lipoprotein cholesterol (HDL-C) levels in those presenting with acute coronary syndromes (ACS)., Methods: Records from patients with ACS presenting to the Emergency Department of University of Florida Hospital Jacksonville from 2009 to 2012, were reviewed. Detailed medical history was obtained. HDL-C levels were measured within 72 h of presentation. Pearson chi-square and Wilcoxon rank sum tests were used to compare groups in univariate analysis. Analysis of variance was performed to determine independent predictors of higher HDL-C levels using variable selection., Results: Of 2400 patients screened, 614 (382 men and 232 women) met inclusion criteria. Hypertension, chronic kidney disease or prior CAD history was similar between sexes and races. Women were more likely to be older (62.4 vs 58.4 years), diabetic (56.5 vs 36.5%) and have higher body mass index (31.2 vs 30.1 kg/m2). Blacks were more likely to be diabetic (50.3 vs 41.3%). After adjusting for all clinical markers, women and blacks along with absence of CAD or diabetes, were significantly associated with higher HDL-C levels., Conclusions: High HDL-C levels (> 40 mg/dL), considered cardio-protective, were seen in women and blacks with ACS more often than in men and whites. Significant differences in HDL-C levels between sexes were seen in whites but not in blacks. Relevance and quality of HDL-C levels in racial groups need further study as this may have important implications in the interpretation of current guidelines., Competing Interests: Conflicts of interest The authors declare no conflicts of interest., (Copyright © 2021 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.)

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. After more than seven decades of the first palliative surgery, TOF prognosis has changed dramatically. The prevalence of TOF is approximately 3 per 10 000 births, representing 7 to 10% of congenital heart disease. With a higher survival into adulthood, the clinical cardiologist faces challenges in the management of this population, from severe pulmonary regurgitation to heart failure and ventricular arrhythmias. Its prevalence is approximately 3 per 10 000 live births, representing 7 to 10% of congenital heart disease. This review will describe the most relevant aspects of the care of adult patients with this disease., Competing Interests: Conflictos de interés: Los autores declaran no tener ningún conflicto de interés.

Patients with cancer and aortic stenosis (AS) are exposed to several factors that could accelerate the progression of AS. This study aimed to determine the cumulative incidence of AS progression and associated factors in these patients. This retrospective cohort study included patients with cancer, mild or moderate AS and at least two echocardiograms 6 months apart between 1996 and 2016 at MD Anderson Cancer Center. AS progression was defined by an increase in mean gradient of 20 mmHg or peak velocity of 2 m/s by spectral Doppler echocardiography or as requiring aortic valve replacement. Univariate and multivariable Fine-Gray models to account for the competing risk of death were used. One hundred and two patients were included and median follow-up was 7.3 years. Overall, 30 patients (29%) developed AS progression, while 48 (47%) died without it. Yearly rate of mean gradient change was 4.9 ± 3.9 mmHg and yearly rate of peak velocity change was 0.23 ± 0.29 m/s for patients who developed AS progression. In the univariate analysis, coronary artery disease (CAD), dyspnea, prevalent cyclophosphamide and beta-blocker use were associated with AS progression. In multivariable analysis, CAD and prevalent cyclophosphamide use for the time interval of more than 3 years of follow-up remained significantly associated with increased cumulative incidence of AS progression. In conclusion, patients with mild or moderate AS and cancer are more likely to die before having AS progression. AS progression is associated with CAD and prevalent cyclophosphamide use., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Bravo-Jaimes, Palaskas, Banchs, Abelhad, Altaf, Gouni, Song, Hassan, Iliescu, Deswal and Yusuf.)

Background and Aim: Specific drug therapy to target the underlying proinflammatory and prothrombotic state in patients with metabolic syndrome (MS) is lacking. We sought to study the effect of high-intensity atorvastatin on markers of lipogenesis, inflammation and thrombogenesis, in women with MS in the absence of cardiovascular disease or diabetes., Methods and Results: This randomized double-blinded controlled trial included 88 women with MS (according to National Cholesterol Education Panel Adult Treatment Panel III criteria) and low atherosclerotic cardiovascular risk. Participants were randomized to receive atorvastatin 80 mg or matching placebo. Thrombogenic, lipogenic and inflammatory markers were collected at the time of enrollment, after a 6-week dietary run-in phase (time of randomization), and at 6- and 12-weeks after randomization. At 6 weeks post-randomization, there was significant reduction in total cholesterol, low density lipoprotein cholesterol, triglycerides, apolipoprotein-B (Apo-B) and Apo-B/Apo-A1 ratio in the atorvastatin arm compared to placebo. This difference persisted at 12-weeks post randomization. There was no significant difference in fasting blood glucose, high-density lipoprotein cholesterol, high sensitivity C-reactive protein, serum leptin, Apo-A1, intercellular adhesion molecule 1 and platelet activity. A significant increase in vascular adhesion molecule 1 at 6 and 12 weeks was seen within the atorvastatin arm. No difference was observed in blood pressure and waist circumference., Conclusions: In conclusion, high-intensity atorvastatin has an early and significant impact on lipoproteins and apolipoproteins but did not lower inflammatory, thrombogenic or biomarkers of platelet activity and aggregation in women with MS. The use of statins for primary prevention in these patients should be further explored., Competing Interests: Declaration of competing interest None., (Copyright © 2020 The Italian Diabetes Society, the Italian Society for the Study of Atherosclerosis, the Italian Society of Human Nutrition and the Department of Clinical Medicine and Surgery, Federico II University. Published by Elsevier B.V. All rights reserved.)

Purpose of Review: Cancer patients often have cardiovascular risk factors at the time of cancer diagnosis, which are known to increase the risk of cardiotoxicity. Cancer survivors have significantly higher cardiovascular risk. Current cardiovascular disease prevention guidelines are based on studies that largely excluded these patients. We reviewed recent data regarding cardiovascular disease prevention in this population., Recent Findings: Nonpharmacologic therapies aiming to reduce 'lifestyle toxicity' produced by cancer treatments have demonstrated potential to decrease the incidence of adverse outcomes. Exercise before, during and after cancer treatment not only promotes higher quality of life and cardiorespiratory fitness but also reduces adverse cardiovascular outcomes. Lipid and cardiometabolic disease management is paramount but predominantly based on data that excludes these populations of cancer patients and survivors., Summary: A comprehensive approach including medical evaluation, prescriptive exercise, cardiac risk factor modification, education, counseling, pharmacologic and behavioral interventions are needed in cancer patients. These interventions constitute the core of cardio-oncology rehabilitation programs, which if implemented appropriately may help reduce cardiovascular events in this population. Knowledge gaps in these areas are starting to be addressed by ongoing clinical trials.

Bicuspid aortic valve (BAV) is the most common congenital heart defect, found in up to 2% of the population and associated with a 30% lifetime risk of complications. BAV is inherited as an autosomal dominant trait with incomplete penetrance and variable expressivity due to a complex genetic architecture that involves many interacting genes. In this review, we highlight the current state of knowledge about BAV genetics, principles and methods for BAV gene discovery, clinical applications of BAV genetics, and important future directions., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: In Latin America, the prevalence of end-stage kidney disease (ESKD) has risen tremendously during the last decade. Previous studies have suggested that receiving dialysis at high altitude confers mortality benefits; however, this effect has not been demonstrated at >2000 m above sea level (masl) or in developing countries., Methods: This historical cohort study analyzed medical records from six Peruvian hemodialysis (HD) centers located at altitudes ranging from 44 to 3827 masl. Adult ESKD patients who started maintenance HD between 2000 and 2010 were included. Patients were classified into two strata based on the elevation above sea level of their city of residence: low altitude (<2000 masl) and high altitude (≥2000 masl). Death from any cause was collected from national registries and Cox proportional hazards models were built., Results: A total of 720 patients were enrolled and 163 (22.6%) resided at high altitude. The low-altitude group was significantly younger, more likely to have diabetes or glomerulonephritis as the cause of ESKD and higher hemoglobin. The all-cause mortality rate was 84.3 per 1000 person-years. In the unadjusted Cox model, no mortality difference was found between the high- and low-altitude groups {hazard ratio [HR] 1.20 [95% confidence interval (CI) 0.89-1.62]}. After multivariable adjustment, receiving HD at high altitude was not significantly associated with higher mortality, but those with diabetes as the cause of ESKD had significantly higher mortality [HR 2.50 (95% CI 1.36-4.59)]., Conclusions: In Peru, patients receiving HD at high altitudes do not have mortality benefits., (© The Author(s) 2020. Published by Oxford University Press on behalf of ERA-EDTA.)

Anomalous origin of the left coronary artery from the non-coronary cusp (LCANCC) is extremely rare and its prognosis and management are still controversial. We present two cases of symptomatic women with LCANCC and a comprehensive review of 19 studies reporting the prevalence, presentation, and management of LCANCC among 174,262 patients. Despite case reports of LCANCC in the pediatric population suggest a much worse prognosis, the optimal risk-stratification scheme for this type of anomaly in adults is yet to be defined, and it should not necessarily be considered a benign condition solely based on its anatomic origin or lack of an interarterial course., Competing Interests: The authors have declared financial relationships, which are detailed in the next section., (Copyright © 2020, Bravo-Jaimes et al.)

The association of pulmonary atresia, ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCA) is an extreme form of tetralogy of Fallot (TOF). It carries a high mortality risk if not intervened on during infancy with only 20% of unoperated patients surviving into adulthood. We present the case of a 40-year-old man who presented for evaluation prior to retinal surgery and was found to have hypoxia and a loud murmur. Cardiac catheterization was performed in the general catheterization laboratory, demonstrating a membranous VSD, pulmonary atresia, and MAPCA. We highlight the challenges and limitations that an adult interventional cardiologist may have when encountering these patients., Competing Interests: The authors report having no conflicts of interest., (Copyright © 2020 Katia Bravo-Jaimes et al.)

Introduction: An increasing number of patients with cardiac devices require radiation therapy for treatment of a variety of cancers. This study aimed to identify the incidence and predictors of cardiac implantable electronic devices (CIED) malfunction in a real-world population that has received radiation therapy., Methods: This retrospective cohort study included 109 adult patients who received radiation therapy at the University of Rochester Medical Center, Radiation Oncology Department, between 2000 and 2015. Sixty patients had pacemakers and 49 had automatic implantable cardioverter defibrillators. Subjects received either high energy (16 MV) and/or low energy (6 MV) photon beams with or without electron beams (6-16 MeV). We included interrogations done from first day of radiation and up to 3 months' postradiation therapy. Outcomes analyzed were device-related malfunctions and device-related clinical events. Fisher's exact, Wilcoxon, and Kruskall-Wallis tests were used for bivariate analysis. Logistic regression with robust adjustment was used for multivariate analysis., Results: We identified six device-related malfunctions. All events were minor and included partial settings reset leading to loss of historical data, pacing thresholds changes, lead impedance changes, and LV output increase. Two patients had device-related clinical events, including dyspnea and diaphragmatic-stimulation. In bivariate analysis, CIED malfunction was associated with CIED duration in situ. In multivariate analysis, there was no significant statistical association between adverse events and beam energy type, CIED location, or dose of radiation delivered to the target., Conclusions: CIED malfunctions are uncommon in real-world patients and associated with minor clinical events. In our cohort, remote CIED monitoring would have identified all events., (© 2018 Wiley Periodicals, Inc.)

Left bundle branch block following inferior myocardial infarction remains uncommon and scarcely reported in the literature. We describe a rare presentation of a 58-year-old male patient who developed left bundle branch block and third degree atrioventricular block after inferior myocardial infarction requiring permanent pacemaker placement. Pathophysiology, impact on mortality, and management options are discussed., (© 2016 Wiley Periodicals, Inc.)