Impact of the new UNOS donor heart allocation system on waitlist outcomes and early posttransplant mortality among adults with congenital heart disease.

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.

Effect of atorvastatin on lipogenic, inflammatory and thrombogenic markers in women with the metabolic syndrome.

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.

Opportunities for improved cardiovascular disease prevention in oncology patients.

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.

CIED malfunction in patients receiving radiation is a rare event that could be detected by remote monitoring.

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.

Left Bundle Branch Block and Complete Heart Block Complicating Inferior Myocardial Infarction.

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.

[Hemophagocytic lymphohistiocytosis presenting as acute liver failure in a patient with Hodgkin lymphoma: case report and review of the literature]. / Falla hepática aguda como presentación de linfohistiocitosis hemofagocítica secundaria a linfoma de Hodgkin: reporte de caso y revisión de la literature.

Hemophagocytic lymphohistiocytosis is a rare cause of acute liver failure. It requires a high level of clinical suspicion to reach the diagnosis and avoid fatal outcomes. The case of a 13-year old patient with fever, anasarca, jaundice, lymphadenopathy and encephalopathy is presented. Laboratory studies showed anemia, thrombocytopenia, hyperbilirubinemia, hyperferritinemia, elevated INR, and hemophagocytic phenomenon in centrifugated ascitic fluid. Bone biopsy showed mixed cellularity Hodgkin lymphoma. This report discusses diagnostic criteria, etiology and management of hemophagocytic limphohistocytosis, as well as its association with acute liver failure.

The Impact of Social Determinants of Health on Transition From Pediatric to Adult Cardiology Care.

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.

Intravascular Lithotripsy in an Adult With Fontan Pathway Obstruction.

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.

Racial Differences in Hospice Care Outcomes in Patients With Advanced Heart Failure: Systematic Review and Meta-analysis.

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, I2 0%) and discharge (odds ratio 1.75, 95% CI 1.53 to 1.99, I2 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, I2 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.

Differences in clinical presentation and outcomes in pregnancy-associated Takotsubo Syndrome- A scoping review of the literature: Outcomes in pregnancy-associated Takotsubo.

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.

Cardiovascular Disease in Hispanic Women: JACC Review Topic of the Week.

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.

Intrahepatic Transcriptomics Differentiate Advanced Fibrosis and Clinical Outcomes in Adults With Fontan Circulation.

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-ß signaling pathway, and vasculature development. CONCLUSIONS: Patients with FALD and advanced fibrosis or the CCO exhibited upregulated genes related to inflammation, congestion, and angiogenesis.

A new algorithm DEtectS critical Congenital Heart Disease at different altitudes: ANDES-CHD study.

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.

Health Equity and Policy Considerations for Pediatric and Adult Congenital Heart Disease Care among Minoritized Populations in the United States.

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.

Access to Specialized Care Across the Lifespan in Tetralogy of Fallot.

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.

Pour les personnes qui vivent avec la tétralogie de Fallot, des soins spécialisés en cardiopathie congénitale (CC) sont nécessaires pour surveiller et prendre en charge toute complication tardive éventuelle. Toutefois, l'accès à des soins en cardiologie demeure difficile pour de nombreux patients, tout comme aux services en santé mentale, aux soins dentaires, aux soins obstétriques et à d'autres soins spécialisés dont cette population a besoin. Des inégalités dans l'accès aux soins de santé ont été mises en lumière lors de la pandémie de COVID-19 et continuent d'exister. Paradoxalement, de nombreux facteurs sociaux agissent sur les besoins d'une personne en matière de soins, et limitent en même temps son accès à ces soins. Le sexe et le genre, l'appartenance à un groupe racial ou ethnique ayant vécu une exclusion par le passé, un niveau de scolarité ou un niveau socio-économique plus faible, l'éloignement géographique des centres de soins tertiaires, les difficultés de déplacement, une protection inadéquate en matière d'assurance de soins de santé, la précarité professionnelle et des expériences antérieures de discrimination en contexte de soins de santé font partie de ces facteurs, qui peuvent coexister et avoir un effet cumulatif. De plus, de nombreux patients croient être guéris et ne sont pas conscients qu'un suivi spécialisé est nécessaire pour eux. Pour ces raisons, les interruptions des soins sont fréquentes, en particulier au moment de la transition entre les soins pédiatriques et les soins destinés aux adultes. La pénurie de professionnels de la santé formés pour intervenir auprès d'adultes présentant une CC est un obstacle supplémentaire, même dans les pays à revenus élevés. Notre article de synthèse résume les obstacles vécus par les personnes vivant avec la tétralogie de Fallot dans l'accès à plusieurs types de soins spécialisés, en portant une attention particulière aux déterminants sociaux de la santé. Des recommandations concrètes pour améliorer l'accès aux soins en contexte canadien et américain sont présentées.

Locking the Revolving Door: Racial Disparities in Cardiovascular Disease.

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.

Intrahepatic transcriptomics differentiate advanced fibrosis and clinical outcomes in adults with the Fontan circulation.

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.

Right coronary artery originating from the left ventricular outflow tract diagnosed after a Ross procedure: a case report.

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.