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BACKGROUND: Cardiovascular disease (CVD) in pregnancy is a leading cause of maternal morbidity and mortality in the United States, with an increasing prevalence. OBJECTIVES: This study aimed to examine risk factors for adverse maternal cardiac, maternal obstetric, and neonatal outcomes as well as costs for pregnant people with CVD at delivery. METHODS: Using the National Inpatient Sample 2010-2019 and the Internal Classification of Diseases diagnosis codes, all pregnant people admitted for their delivery hospitalization were included. CVD diagnoses included congenital heart disease, cardiomyopathy, ischemic heart disease, arrhythmias, and valvular disease. Multivariable regressions were used to analyze major adverse cardiovascular events (MACE), maternal and fetal complications, length of stay, and resource utilization. RESULTS: Of the 33,639,831 birth hospitalizations included, 132,532 (0.39%) had CVD. These patients experienced more frequent MACE (8.5% vs 0.4%, P

There is emerging evidence that the cardiac interatrial septum has an important role as a thromboembolic source for ischemic strokes. There is little consensus on treatment of patients with different cardiac interatrial morphologies or pathologies who have had stroke. In this paper, we summarize the important background, diagnostic, and treatment considerations for this patient population as presented during the Federation of American Societies for Experimental Biology (FASEB) Virtual Catalytic Conference on the Cardiac Interatrial Septum and Stroke Risk, held on December 7, 2022. During this conference, many aspects of the cardiac interatrial septum were discussed. Among these were the embryogenesis of the interatrial septum and development of anatomic variants such as patent foramen ovale and left atrial septal pouch. Also addressed were various mechanisms of injury such as shunting physiologies and the consequences that can result from anatomic variants, as well as imaging considerations in echocardiography, computed tomography, and magnetic resonance imaging. Treatment options including anticoagulation and closure were addressed, as well as an in-depth discussion on whether the left atrial septal pouch is a stroke risk factor. These issues were discussed and debated by multiple experts from neurology, cardiology, and radiology.

Congenital heart disease (CHD) in pregnancy is associated with an increased risk of adverse maternal, obstetric, and neonatal outcomes, plausibly through mechanisms involving abnormal placental development and function. This retrospective study aims to elucidate how maternal CHD influences placental health. Demographic and clinical information were collected via electronic medical record review, and placentas underwent histopathological evaluation. Fifty-three singleton pregnancies were included: 35 participants (66%) were classified as lower cardiovascular risk (modified World Health Organization Classification (mWHO) I, II, II-III), and 18 (34%) were classified as higher cardiovascular risk (mWHO III, IV). 12 participants (23%) had a fetus with small for gestational age (SGA). Maternal vascular malperfusion (53%) and placental abruption (11.6%) were common in this cohort, with prevalence above baseline risk. Participants at higher cardiovascular risk had higher rates of SGA (p = 0.04), subchorionic hematomas (p = 0.01) and birth weight:placental weight

BackgroundPatients with post-acute sequelae of COVID-19 (PASC) often experience the addition of new symptoms after recovery from COVID-19 illness. These may include orthostatic intolerance and autonomic dysfunction, and postural orthostatic tachycardia syndrome has been described to occur in a proportion of patients with PASC.Case summaryIn this report, we present a 32-year-old pregnant woman (G3P2) who experiences severe orthostatic symptoms as part of her PASC syndrome, which is decoupled from normal physiologic changes of pregnancy. At 25 weeks of gestation, she was evaluated for increasing episodes of dyspnoea, marked tachycardia with minimal exertion, intermittent non-exertional chest pain, and presyncope. This patient had a moderate course of COVID-19 at 12 weeks of gestation, for which she received monoclonal antibody therapy (casirivimab/imdevimab). The patient then had complete resolution of COVID-19 symptoms and felt well for 1 month prior to developing orthostatic symptoms at 25 weeks of gestation. Evaluation with a NASA Lean Test revealed marked orthostatic tachycardia, as well as delayed orthostatic hypotension. Given her COVID-19 illness 4 months prior, PASC involving autonomic dysfunction was diagnosed.DiscussionPatients with orthostatic symptoms in PASC should be carefully evaluated with dedicated active stand tests, such as the NASA Lean Test, to characterize the autonomic response to standing. In pregnant patients, an understanding of normal pregnancy physiology is crucial to correctly identify abnormal findings in such tests.

Background Current recommendations for delivery timing of pregnant persons with congenital heart disease (CHD) are based on expert opinion. Justification for early-term birth is based on the theoretical concern of increased cardiovascular stress. The objective was to evaluate whether early-term birth with maternal CHD is associated with lower adverse maternal or neonatal outcomes. Methods and Results This is a retrospective cohort study of pregnant persons with CHD who delivered a singleton after 37 0/7 weeks gestation at a quaternary care center with a multidisciplinary cardio-obstetrics care team between 2013 and 2021. Patients were categorized as early-term (37 0/7 to 38 6/7 weeks) or full-term (≥39 0/7) births and compared. Multivariable logistic regression was conducted to calculate the adjusted odds ratio for the primary outcomes. The primary outcomes were composite adverse cardiovascular, maternal obstetric, and adverse neonatal outcome. Of 110 pregnancies delivering at term, 55 delivered early-term and 55 delivered full-term. Development of adverse cardiovascular and maternal obstetric outcome was not significantly different by delivery timing. The rate of composite adverse neonatal outcomes was significantly higher in early-term births (36% versus 5%, P

BackgroundCOVID-19 and Fontan physiology have each been associated with an elevated risk of venous thromboembolism (VTE), however little is known about the risks and potential consequences of having both.Case presentationA 51 year old male with tricuspid atresia status post Fontan and extracardiac Glenn shunt, atrial flutter, and sinus sick syndrome presented with phlegmasia cerulea dolens (PCD) of the left lower extremity in spite of supratherapeutic INR in the context of symptomatic COVID-10 pneumonia. He was treated with single session, catheter directed mechanical thrombectomy that was well-tolerated.ConclusionsThis report of acute PCD despite therapeutic anticoagulation with a Vitamin K antagonist, managed with emergent mechanical thrombectomy, calls to attention the importance of altered flow dynamics in COVID positive patients with Fontan circulation that may compound these independent risk factors for developing deep venous thrombosis with the potential for even higher morbidity.

Purpose of the reviewThis is a comprehensive update on failing Fontan physiology and the role of heart and combined heart and liver transplantation in the current era.Recent findingsSingle ventricle physiology encompasses a series of rare congenital cardiac abnormalities that are characterized by absence of or hypoplasia of one ventricle. This effectively results in a single ventricular pumping chamber. These abnormalities are rarely compatible with long-term survival if left without surgical palliation in the first few years of life. Surgical treatment of single ventricle physiology has evolved over the past 60 years and is characterized by numerous creative innovations. These include the development of arteriopulmonary shunts, the evolution of partial cavopulmonary connections, and the eventual development of the "Fontan" operation. Regardless of the type of Fontan modification, the long-term consequences of the Fontan operation are predominantly related to chronic central venous hypertension and the multi-organ consequences thereof. Atrial arrhythmias can further compromise this circulation.Patients with single ventricle physiology represent a special sub-segment of congenital cardiac transplants and are arguably the most challenging patients considered for transplantation.SummaryThis review describes in detail the challenges and opportunities of heart and liver transplantation in Fontan patients, as viewed and managed by the experienced team at the Ahmanson/UCLA Adult Congenital Heart Center.

The molecular mechanisms underlying Fontan-associated liver disease (FALD) remain largely unknown. This study aimed to assess intrahepatic transcriptomic differences among patients with FALD according to the degree of liver fibrosis and clinical outcomes. 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. 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. Patients with FALD and advanced fibrosis or the CCO exhibited upregulated genes related to inflammation, congestion, and angiogenesis. [Display omitted] [ABSTRACT FROM AUTHOR]