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Background: Adult congenital heart disease (ACHD) patients have significant morbidity and rise in cardiac admissions. Their outcome with high-dose influenza vaccination is unknown in comparison to those without ACHD., Objectives: The purpose of this study was to compare all-cause mortality or cardiopulmonary hospitalizations in self-identified ACHD versus non-ACHD patients receiving high- or low-dose influenza vaccination within the INfluenza Vaccine to Effectively Stop cardioThoracic Events and Decompensated heart failure trial., Methods: We prospectively included ACHD patients in the INVESTED (INfluenza Vaccine to Effectively Stop cardioThoracic Events and Decompensated heart failure) trial. The primary endpoint was all-cause death or hospitalization for cardiovascular or pulmonary causes., Results: Of the 272 ACHD patients, 132 were randomly assigned to receive high-dose trivalent and 140 to standard-dose quadrivalent influenza vaccine. Compared to the non-ACHD cohort (n = 4,988), ACHD patients were more likely to be younger, women, smokers, have atrial fibrillation, and have a qualifying event of heart failure. The primary outcome was 49.8 events versus 42.8 events per 100 person-years (adjusted HR: 1.17; 95% CI: 0.95-1.45; P  = 0.144) in the ACHD group and non-ACHD group, respectively. The interaction between ACHD status and randomized treatment effect was not significant for the primary outcome ( P  = 0.858). Vaccine-related adverse events were similar in both groups., Conclusions: Patients who self-identify as being ACHD had similar primary outcome of all-cause death or hospitalization for cardiovascular or pulmonary causes compared to non-ACHD cohort. High-dose influenza vaccination was similar to standard-dose influenza vaccination on the primary outcome in patients who self-identify as ACHD., Competing Interests: Internal funding was provided by Prairie Vascular Research Inc. Dr Goodman has received research grant support (eg, steering committee or data and safety monitoring committee) and/or speaker/consulting honoraria (eg, advisory boards) from Amgen, Anthos Therapeutics, AstraZeneca, Bayer, Boehringer Ingelheim, Bristol Myers Squibb, CSL Behring, Daiichi-Sankyo/American Regent, Eli Lilly, Esperion, Ferring Pharmaceuticals, HLS Therapeutics, JAMP Pharma, Merck, Novartis, Novo Nordisk A/C, Pendopharm/Pharmascience, Pfizer, Regeneron, Sanofi, Servier, Tolmar Pharmaceuticals, Valeo Pharma; and salary support/honoraria from the Heart and Stroke Foundation of Ontario/University of Toronto (Polo) Chair, Canadian Heart Failure Society, Canadian Heart Research Centre and MD Primer, Canadian VIGOUR Centre, Cleveland Clinic Coordinating Centre for Clinical Research, Duke Clinical Research Institute, New York University Clinical Coordinating Centre, PERFUSE Research Institute, TIMI Study Group (Brigham Health). Dr Hegde has received fees paid to institution from Myokardia. Dr Bhatt discloses the following relationships - Advisory Board for Angiowave, Bayer, Boehringer Ingelheim, Cardax, CellProthera, Cereno Scientific, Elsevier Practice Update Cardiology, High Enroll, Janssen, Level Ex, McKinsey, Medscape Cardiology, Merck, MyoKardia, NirvaMed, Novo Nordisk, PhaseBio, PLx Pharma, Regado Biosciences, Stasys; Board of Directors; Angiowave (stock options), Boston VA Research Institute, Bristol Myers Squibb (stock), DRS.LINQ (stock options), High Enroll (stock), Society of Cardiovascular Patient Care, TobeSoft; Chair: Inaugural Chair; American Heart Association Quality Oversight Committee; Consultant: Broadview Ventures; Data Monitoring Committees: Acesion Pharma, Assistance Publique-Hôpitaux de Paris, Baim Institute for Clinical Research (formerly Harvard Clinical Research Institute, for the PORTICO trial, funded by St. Jude Medical, now Abbott), Boston Scientific (Chair, PEITHO trial), Cleveland Clinic (including for the ExCEED trial, funded by Edwards), Contego Medical (Chair, PERFORMANCE 2), Duke Clinical Research Institute, Mayo Clinic, Mount Sinai School of Medicine (for the ENVISAGE trial, funded by Daiichi Sankyo; for the ABILITY-DM trial, funded by Concept Medical), Novartis, Population Health Research Institute; Rutgers University (for the NIH-funded MINT Trial); Honoraria; American College of Cardiology (Senior Associate Editor, Clinical Trials and News, ACC.org; Chair, ACC Accreditation Oversight Committee), Arnold and Porter law firm (work related to Sanofi/Bristol-Myers Squibb clopidogrel litigation), Baim Institute for Clinical Research (formerly Harvard Clinical Research Institute; RE-DUAL PCI clinical trial steering committee funded by Boehringer Ingelheim; AEGIS-II executive committee funded by CSL Behring), Belvoir Publications (Editor in Chief, Harvard Heart Letter), Canadian Medical and Surgical Knowledge Translation Research Group (clinical trial steering committees), Cowen and Company, Duke Clinical Research Institute (clinical trial steering committees, including for the PRONOUNCE trial, funded by Ferring Pharmaceuticals), HMP Global (Editor in Chief, Journal of Invasive Cardiology), Journal of the American College of Cardiology (Guest Editor; Associate Editor), K2P (Co-Chair, interdisciplinary curriculum), Level Ex, Medtelligence/ReachMD (CME steering committees), MJH Life Sciences, Oakstone CME (Course Director, Comprehensive Review of Interventional Cardiology), Piper Sandler, Population Health Research Institute (for the COMPASS operations committee, publications committee, steering committee, and USA national co-leader, funded by Bayer), Slack Publications (Chief Medical Editor, Cardiology Today’s Intervention), Society of Cardiovascular Patient Care (Secretary/Treasurer), WebMD (CME steering committees), Wiley (steering committee); Other: Clinical Cardiology (Deputy Editor), NCDR-ACTION Registry Steering Committee (Chair), VA CART Research and Publications Committee (Chair); Patent: Sotagliflozin (named on a patent for sotagliflozin assigned to Brigham and Women's Hospital who assigned to Lexicon; neither I nor Brigham and Women's Hospital receive any income from this patent); Research Funding: Abbott, Acesion Pharma, Afimmune, Aker Biomarine, Amarin, Amgen, AstraZeneca, Bayer, Beren, Boehringer Ingelheim, Boston Scientific, Bristol-Myers Squibb, Cardax, CellProthera, Cereno Scientific, Chiesi, CinCor, CSL Behring, Eisai, Ethicon, Faraday Pharmaceuticals, Ferring Pharmaceuticals, Forest Laboratories, Fractyl, Garmin, HLS Therapeutics, Idorsia, Ironwood, Ischemix, Janssen, Javelin, Lexicon, Lilly, Medtronic, Merck, Moderna, MyoKardia, NirvaMed, Novartis, Novo Nordisk, Owkin, Pfizer, PhaseBio, PLx Pharma, Recardio, Regeneron, Reid Hoffman Foundation, Roche, Sanofi, Stasys, Synaptic, The Medicines Company, Youngene, 89Bio; Royalties: Elsevier (Editor, Braunwald’s Heart Disease); Site Co-Investigator: Abbott, Biotronik, Boston Scientific, CSI, Endotronix, St. Jude Medical (now Abbott), Philips, SpectraWAVE, Svelte, Vascular Solutions; Trustee: American College of Cardiology; Unfunded Research: FlowCo, Takeda. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Background: There is a paucity of data on long-term outcomes after Fontan palliation in patients with a dominant morphological univentricular right (uRV) vs left (uLV) ventricle., Objectives: The purpose of this study was to compare the incidence of atrial arrhythmias, thromboembolic events, cardiac transplantation, and death following Fontan palliation in patients with uRV vs uLV., Methods: The Alliance for Adult Research in Congenital Cardiology conducted a multicenter retrospective cohort study on patients with total cavopulmonary connection Fontan palliation across 12 centers in North America. All components of the composite outcome, that is, atrial arrhythmias, thromboembolic events, cardiac transplantation, and death, were reviewed and classified by a blinded adjudicating committee. Time-to-event analyses were performed that accounted for competing risks., Results: A total of 384 patients were followed for 10.5 ± 5.9 years. The composite outcome occurred in 3.7 vs 1.7 cases per 100 person-years for uRV (N = 171) vs uLV (N = 213), respectively ( P  < 0.001). In multivariable analyses, uRV conferred a >2-fold higher risk of the composite outcome (HR: 2.17, 95% CI: 1.45-3.45, P  < 0.001). In secondary analyses of components of the primary outcome, uRV was significantly associated with a greater risk of cardiac transplantation or death (HR: 9.09, 95% CI: 2.17-38.46, P  < 0.001) and atrial arrhythmias (HR: 2.17, 95% CI: 1.20-4.00, P  = 0.010) but not thromboembolic events (HR: 1.64, 95% CI: 0.86-3.16, P  = 0.131)., Conclusions: Fontan patients with uRV vs uLV morphology have a higher incidence of adverse cardiovascular events, including atrial arrhythmia, cardiac transplantation, and all-cause mortality., Competing Interests: The TACTIC study was funded by an investigator-initiated unrestricted grant from Boehringer Ingelheim. The sponsor had no role in the study design, data collection, analysis, interpretation, writing of the paper, and decision to submit the manuscript for publication. Dr Dib is supported by the 10.13039/501100003100French Federation of Cardiology (FFC) and 10.13039/501100009597ADETEC grants. Dr Khairy is supported by the André Chagnon research chair in electrophysiology and congenital heart disease. All other have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Background and Aims: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear., Methods: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death., Results: From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P < .001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome., Conclusions: Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.)

This consensus document for the performance of cardiovascular computed tomography (CCT) to guide intervention in the right ventricular outflow tract (RVOT) in patients with congenital disease (CHD) was developed collaboratively by pediatric and adult interventionalists, surgeons and cardiac imagers with expertise specific to this patient subset. The document summarizes definitions of RVOT dysfunction as assessed by multi-modality imaging techniques and reviews existing consensus statements and guideline documents pertaining to indications for intervention. In the context of this background information, recommendations for CCT scan acquisition and a standardized approach for reporting prior to surgical or transcatheter pulmonary valve replacement are proposed and presented. It is the first Imaging for Intervention collaboration for CHD patients and encompasses imaging and reporting recommendations prior to both surgical and percutaneous pulmonary valve replacement., 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. Relationships with industry for authors are outlined in Appendix A and for reviewers are outlined in Appendix B., (© 2023 The Authors.)

Ascites is a condition typically attributed to liver disease but can be cardiac in aetiology as well. We report of an atypical case of pulmonary homograft failure status-post the Ross-Yacoub procedure as an underlying cause of anasarca. A man in his mid-30s with a history of congenital heart disease presented with abdominal distension and exercise intolerance. His prior history was relevant for congenital bicuspid aortic valve with critical aortic stenosis. Abdomen and pelvis CT revealed a grossly dilated inferior vena cava while transthoracic echocardiogram showed severe homograft pulmonary stenosis and regurgitation. Further investigation with cardiac MRI demonstrated severe pulmonary valve stenosis and regurgitation with significant tricuspid regurgitation and right ventricular dilatation. The transcatheter stent in the right ventricular to pulmonary artery conduit followed by transcatheter valve replacement, which resulted in full resolution of his presenting symptoms., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: Few data exist on mortality among patients with univentricular heart (UVH) before surgery. Our aim was to explore the results of intention to perform surgery by estimating preoperative vs postoperative survival in different UVH subgroups., Design: Retrospective., Setting: Tertiary centre for congenital cardiology and congenital heart surgery., Participants: All 595 Norwegian children with UVH born alive from 1990 to 2015, followed until 31 December 2020., Results: One quarter (151/595; 25.4%) were not operated. Among these, only two survived, and 125/149 (83.9%) died within 1 month. Reasons for not operating were that surgery was not feasible in 31.1%, preoperative complications in 25.2%, general health issues in 23.2% and parental decision in 20.5%. In total, 327/595 (55.0%) died; 283/327 (86.5%) already died during the first 2 years of life. Preoperative survival varied widely among the UVH subgroups, ranging from 40/65 (61.5%) among patients with unbalanced atrioventricular septal defect to 39/42 (92.9%) among patients with double inlet left ventricle. Postoperative survival followed a similar pattern. Postoperative survival among patients with hypoplastic left heart syndrome (HLHS) improved significantly (5-year survival, 42.5% vs 75.3% among patients born in 1990-2002 vs 2003-2015; p<0.0001), but not among non-HLHS patients (65.7% vs 72.6%; p=0.22)-among whom several subgroups had a poor prognosis similar to HLHS. A total of 291/595 patients (48.9%) had Fontan surgery CONCLUSIONS: Surgery was refrained in one quarter of the patients, among whom almost all died shortly after birth. Long-term prognosis was largely determined during the first 2 years. There was a strong concordance between preoperative and postoperative survival. HLHS survival was improved, but non-HLHS survival did not change significantly. This study demonstrates the complications and outcomes encountering newborns with UVH at all major stages of preoperative and operative treatment., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: The impact of Fontan-associated liver disease (FALD) on post-transplant mortality and indications for combined heart-liver transplant (CHLT) in adult Fontan patients remains unknown., Objectives: The purpose of this study was to assess the impact of FALD on post-transplant outcomes and compare HT vs CHLT in adult Fontan patients., Methods: We performed a retrospective-cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers. Inclusion criteria were as follows: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at referral. Pretransplant FALD score was calculated using the following: 1) cirrhosis; 2) varices; 3) splenomegaly; or 4) ≥2 paracenteses., Results: A total of 131 patients (91 HT and 40 CHLT) were included. CHLT recipients were more likely to be older (P = 0.016), have a lower hemoglobin (P = 0.025), require ≥2 diuretic agents pretransplant (P = 0.051), or be transplanted in more recent decades (P = 0.001). Postmatching, CHLT demonstrated a trend toward improved survival at 1 year (93% vs 74%; P = 0.097) and improved survival at 5 years (86% vs 52%; P = 0.041) compared with HT alone. In patients with a FALD score ≥2, CHLT was associated with improved survival (1 year: 85% vs 62%; P = 0.044; 5 years: 77% vs 42%; P = 0.019). In a model with transplant decade and FALD score, CHLT was associated with improved survival (HR: 0.33; P = 0.044) and increasing FALD score was associated with worse survival (FALD score: 2 [HR: 14.6; P = 0.015], 3 [HR: 22.2; P = 0.007], and 4 [HR: 27.8; P = 0.011])., Conclusions: Higher FALD scores were associated with post-transplant mortality. Although prospective confirmation of our findings is necessary, compared with HT alone, CHLT recipients were older with higher FALD scores, but had similar survival overall and superior survival in patients with a FALD score ≥2., Competing Interests: Funding Support and Author Disclosures This work was supported by the National Institutes of Health/National Heart, Lung, and Blood Institute (K23 HL1143136). Drs Valente and Carazo were supported by the Sarah Marie Liamos Fund for Adult Congenital Heart Disease Research. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: An increasing number of adult Fontan patients require heart transplantation (HT) or combined heart-liver transplant (CHLT); however, data regarding outcomes and optimal referral time remain limited., Objectives: The purpose of this study was to define survivorship post-HT/CHLT and predictors of post-transplant mortality, including timing of referral, in the adult Fontan population., Methods: A retrospective cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers in the United States and Canada was performed. Inclusion criteria included the following: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at the time of referral. Date of "failing" Fontan was defined as the earliest of the following: worsening fluid retention, new ascites, refractory arrhythmia, "failing Fontan" diagnosis by treating cardiologist, or admission for heart failure., Results: A total of 131 patients underwent transplant, including 40 CHLT, from 1995 to 2021 with a median post-transplant follow-up time of 1.6 years (Q1 0.35 years, Q3 4.3 years). Survival was 79% at 1 year and 66% at 5 years. Survival differed by decade of transplantation and was 87% at 1 year and 76% at 5 years after 2010. Time from Fontan failure to evaluation (HR/year: 1.23 [95% CI: 1.11-1.36]; P < 0.001) and markers of failure, including NYHA functional class IV (HR: 2.29 [95% CI: 1.10-5.28]; P = 0.050), lower extremity varicosities (HR: 3.92 [95% CI: 1.68-9.14]; P = 0.002), and venovenous collaterals (HR: 2.70 [95% CI: 1.17-6.20]; P = 0.019), were associated with decreased post-transplant survival at 1 year in a bivariate model that included transplant decade., Conclusions: In our multicenter cohort, post-transplant survival improved over time. Late referral after Fontan failure and markers of failing Fontan physiology, including worse functional status, lower extremity varicosities, and venovenous collaterals, were associated with post-transplant mortality., Competing Interests: Funding Support and Author Disclosures This work was supported by the National Institutes of Health/National Heart, Lung, and Blood Institute (K23 HL1143136). Drs Valente and Carazo were supported by the Sarah Marie Liamos Fund for Adult Congenital Heart Disease Research. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: For patients with d-loop transposition of the great arteries (d-TGA) with a systemic right ventricle after an atrial switch operation, there is a need to identify risks for end-stage heart failure outcomes., Objectives: The authors aimed to determine factors associated with survival in a large cohort of such individuals., Methods: This multicenter, retrospective cohort study included adults with d-TGA and prior atrial switch surgery seen at a congenital heart center. Clinical data from initial and most recent visits were obtained. The composite primary outcome was death, transplantation, or mechanical circulatory support (MCS)., Results: From 1,168 patients (38% female, age at first visit 29 ± 7.2 years) during a median 9.2 years of follow-up, 91 (8.8% per 10 person-years) met the outcome (66 deaths, 19 transplantations, 6 MCS). Patients experiencing sudden/arrhythmic death were younger than those dying of other causes (32.6 ± 6.4 years vs 42.4 ± 6.8 years; P < 0.001). There was a long duration between sentinel clinical events and end-stage heart failure. Age, atrial arrhythmia, pacemaker, biventricular enlargement, systolic dysfunction, and tricuspid regurgitation were all associated with the primary outcome. Independent 5-year predictors of primary outcome were prior ventricular arrhythmia, heart failure admission, complex anatomy, QRS duration >120 ms, and severe right ventricle dysfunction based on echocardiography., Conclusions: For most adults with d-TGA after atrial switch, progress to end-stage heart failure or death is slow. A simplified prediction score for 5-year adverse outcome is derived to help identify those at greatest risk., Competing Interests: Funding Support and Author Disclosures This study was funded by a joint grant from the Children’s Heart Foundation and the American Heart Association (17GRNT33670334). Dr Opotowsky was supported by the Heart Institute Research Core at Cincinnati Children’s Hospital and the Dunlevie Family Fund. The authors used the Research Electronic Data Capture (REDCap) online database, which is funded by the National Institutes of Health (UL1TR002369). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Patients with congenital heart disease now live well into adulthood because of advances in surgical techniques, improvements in medical management, and the development of novel therapeutic agents. As patients grow older into adults with congenital heart disease, many require catheter-based interventions for the treatment of residual defects, sequelae of their initial repair or palliation, or acquired heart disease. The past 3 decades have witnessed an exponential growth in both the type and number of transcatheter interventions in patients with congenital heart disease. With improvements in medical technology and device design, including the use of devices designed for the treatment of acquired valve stenosis or regurgitation, patients who previously would have required open-heart surgery for various conditions can now undergo percutaneous cardiac catheter-based procedures. Many of these procedures are complex and occur in complex patients who are best served by a multidisciplinary team. This review aims to highlight some of the currently available transcatheter interventional procedures for adults with congenital heart disease, the clinical outcomes of each intervention, and any special considerations so that the reader may better understand both the procedure and patients with adult congenital heart disease., (© 2022 The Author(s).)

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 <0.01). After adjusting for confounding variables, early-term birth remained associated with a significantly increased risk of adverse neonatal outcomes (adjusted odds ratio 11.55 [95% CI, 2.59-51.58]). Conclusions Early-term birth for pregnancies with maternal CHD was associated with an increased risk of adverse neonatal outcomes, without an accompanying decreased rate in adverse cardiovascular or obstetric outcomes. In the absence of maternal or fetal indications for early birth, induction of labor before 39 weeks for pregnancies with maternal CHD should be reserved for routine obstetrical indications.

Background: COVID-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 Presentation: A 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., Conclusions: This 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., (© 2021. The Author(s).)

We are witnessing an unparalleled pandemic caused by the novel Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) associated with coronavirus disease 2019 (COVID-19). Current data show that SARS-CoV-2 results in mild flu-like symptoms in the majority of healthy and young patients affected. Nevertheless, the severity of COVID-19 respiratory syndrome and the risk of adverse or catastrophic outcomes are increased in patients with pre-existing cardiovascular disease. Patients with adult congenital heart disease (ACHD)-by definition-have underlying cardiovascular disease. Many patients with ACHD are also afflicted with residual haemodynamic lesions such as valve dysfunction, diminished ventricular function, arrhythmias or cyanosis, have extracardiac comorbidities, and face additional challenges regarding pregnancy. Currently, there are emerging data of the effect of COVID-19 on ACHD patients, but many aspects, especially risk stratification and treatment considerations, remain unclear. In this article, we aim to discuss the broad impact of COVID-19 on ACHD patients, focusing specifically on pathophysiology, risk stratification for work, self-isolation, hospitalization, impact on pregnancy, psychosocial health, and longer-term implications for the provision of ACHD care., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2020. For permissions, please email: journals.permissions@oup.com.)

Objectives: The aim of this study was to determine the prevalence of pacemaker lead-related complications following transcatheter tricuspid valve replacement (TTVR)., Background: The rate of permanent pacemaker implantation following tricuspid valve (TV) surgery is high, and many patients have transvenous leads. The feasibility, safety, and outcomes of subsequently performing TTVR in the setting of transvenous pacemaker leads have not been established., Methods: The VIVID (Valve-in-Valve International Database) registry was used to review 329 patients who underwent TTVR following TV repair or replacement. Patients were subdivided into 3 cohorts for intergroup comparisons: no lead, epicardial lead, and transvenous lead (entrapped or not entrapped during the TTVR procedure)., Results: Of 329 patients who underwent TTVR, 128 (39%) had prior pacing systems in place, 70 with epicardial and 58 with transvenous leads. A total of 31 patients had leads passing through the TV. Three patients had the right ventricular (RV) lead extracted prior to TTVR. The remaining 28 patients had the RV lead entrapped between the transcatheter TV implant and the surgical valve (n = 22) or the repaired TV (n = 6). One patient had displacement of the RV lead during the procedure, and 2 experienced lead failure during follow-up. Overall, there was no significant difference in the cumulative incidences of competing outcomes (death, TV reintervention, TV dysfunction) between patients with and those without pacing leads or entrapped RV leads., Conclusions: TTVR in the setting of trans-TV pacemaker leads without lead extraction or re-replacement can be performed safely with a low risk for complications, offering an alternative to surgical TV replacement., (Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Cardiac catheterization procedures have revolutionized the treatment of adults with congenital heart disease over the past six decades. Patients who previously would have required open heart surgery for various conditions can now undergo percutaneous cardiac catheter-based procedures to close intracardiac shunts, relieve obstructive valvular lesions, stent stenotic vessels, or even replace and repair dysfunctional valves. As the complexity of percutaneous cardiac catheterization procedures has increased, so has the use of echocardiography for interventional guidance in adults with congenital heart disease. Transthoracic, transesophageal, intracardiac, and three-dimensional echocardiography have all become part and parcel of the catheterization laboratory experience. In this review, we aim to describe the different echocardiographic techniques and their role in various cardiac catheterization interventions specific to adults with congenital heart disease., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare., (2019 Cardiovascular Diagnosis and Therapy. All rights reserved.)

Background: Thromboembolic events contribute greatly to morbidity and mortality following Fontan surgery for univentricular hearts., Objectives: This study sought to evaluate the effect of type of Fontan surgery on thromboembolic risk., Methods: A North American multicenter retrospective cohort study enrolled 522 patients with Fontan palliation consisting of an atriopulmonary connection (APC) (21.4%), lateral tunnel (LT) (41.8%), or extracardiac conduit (EC) (36.8%). Thromboembolic complications and new-onset atrial arrhythmia were reviewed and classified by a blinded adjudicating committee. Thromboembolic risk across surgical techniques was assessed by multivariable competing-risk survival regression., Results: Over a median follow-up of 11.6 years, 10- and 20-year freedom from Fontan conversion, transplantation, or death was 94.7% and 78.9%, respectively. New-onset atrial arrhythmias occurred in 4.4, 1.2, and 1.0 cases per 100 person-years with APC, LT, and EC, respectively. APC was associated with a 2.82-fold higher risk of developing atrial arrhythmias (p < 0.001), with no difference between LT and EC (p = 0.95). A total of 71 thromboembolic events, 32 systemic and 39 venous, occurred in 12.8% of subjects, for an overall incidence of 1.1%/year. In multivariable analyses, EC was independently associated with a lower risk of systemic (hazard ratio [HR]: 0.20 vs. LT; 95% confidence interval [CI]: 0.04 to 0.97) and combined (HR: 0.34 vs. LT; 95% CI: 0.13 to 0.91) thromboembolic events. A lower incidence of combined thromboembolic events was also observed with antiplatelet agents (HR: 0.54; 95% CI: 0.32 to 0.92) but not anticoagulation (p = 0.53)., Conclusions: The EC Fontan was independently associated with a lower thromboembolic risk after controlling for time-varying effects of atrial arrhythmias and thromboprophylaxis., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: In Fontan patients with atrial arrhythmias (AA), non-vitamin K antagonist oral anticoagulants(NOACs) have a class III recommendation according to the Pediatric & Congenital Electrophysiology Society (PACES)/Heart Rhythm Society (HRS) guideline in 2014, due to lack of data on outcomes as opposed to evidence of harm. To address this gap in data, we investigated the safety and efficacy of NOACs in adults with a Fontan circulation in a worldwide study., Methods: This is an international multicentre prospective cohort study, using data from the NOTE ( n on-vitamin K antagonist o ral anticoagulants for t hrombo e mbolic prevention in patients with congenital heart disease) registry. The study population comprised consecutive adults with a Fontan circulation using NOACs. Follow-up took place at 6 months and yearly thereafter. The primary endpoints were thromboembolism and major bleeding. Secondary endpoint was minor bleeding., Results: From April 2014 onward, 74 patients (mean age 32±10 years (range 18-68), 54% male) with a Fontan circulation using NOACs were included. During a median follow-up of 1.2 (IQR 0.8-2.0) years, three thromboembolic events (2.9 per 100 patient-years (95% CI 0.7 to 7.6)) and three major bleedings (2.9 per 100 patient-years (95% CI 0.7 to 7.6)) occurred in five atriopulmonary Fontan and one total cavopulmonary connection Fontan patients with AA. Fifteen patients experienced minor bleeding episodes (15.8 per 100 patient-years (95% CI 9.1 to 25.2)). In patients (n=37) using vitamin K antagonists (VKAs) prior to the initiation of NOAC, annual incidence of historical thromboembolic events and major bleeding were 2.4% (95% CI 0.4% to 7.4%) (n = 2) and 1.2% (95% CI 0.7% to 5.1%) (n = 1), respectively., Conclusions: In this review of the largest Fontan cohort using NOACs with prospective follow-up, NOACs appear to be well tolerated and their efficacy and safety during short-term follow-up seem comparable to VKAs. Longer term data are required to confirm these promising short-term results., Competing Interests: Competing interests: BJB has received restricted research grant from Bristol-Myers Squibb and Pfizer. BJMM has received restricted research grants from Boehringer Ingelheim, Bayer and Daiichi Sankyo. Authors, HY, ACE, FM, MCP, AVD, WB, MM, MS, MG, GS, JA, GRV, CB, ST, KN, RA-G and BJB declare that they have no conflict of interest.

Despite relevant residua and sequels, follow-up care of adults with congenital heart disease (ACHD) is too often not performed by/in specialized and/or certified physicians or centers although major problems in the long-term course may develop. The most relevant encompass heart failure, cardiac arrhythmias, heart valve disorders, pulmonary vascular disease, infective endocarditis (IE), aortopathy and non-cardiac comorbidities. The present publication emphasizes current data on IE, pulmonary and pulmonary arterial hypertension and aortopathy in ACHD and underlines the deep need of an experienced follow-up care by specialized and/or certified physicians or centers, as treatment regimens from acquired heart disease can not be necessarily transmitted to CHD. Moreover, the need of primary and secondary medical prevention becomes increasingly important in order to reduce the burden of disease as well as the socioeconomic burden and costs in this particular patient group., Competing Interests: Conflicts of Interest: R Neidenbach received research grants (“Unrestricted educational grant”) from Actelion Pharmaceuticals Deutschland GmbH and from the German Heart Foundation (“Deutsche Herzstiftung e.V.”) and the patient organization “Herzkind e. V.”; H Kaemmerer received fees and/or travel expenses for consulting activities and/or lectures from the following companies within the last 3 years: Actelion, Pfizer, Bayer-Healthcare, Bristol-Myers Squibb; D Pittrow has received speaker fees or honoraria for consultations from Actelion, Bayer, Genzyme, Boehringer Ingelheim, Novartis, MSD. and Dr. Erwin Oechslin currently holds the “Bitove Family Professorship for Adult Congenital Heart Disease”. Other author have no conflicts of interest to declare.

Today most patients with congenital heart defects (CHD) survive into adulthood. Unfortunately, despite relevant residua and sequels, follow-up care of adults with congenital heart disease (ACHD) is not performed in specialized and/or certified physicians or centres. Major problems in the long-term course encompass heart failure, cardiac arrhythmias, heart valve disorders, pulmonary vascular disease, infective endocarditis, aortopathy and non-cardiac comorbidities. Many of them manifest themselves differently from acquired heart disease and therapy regimens from general cardiology cannot be transferred directly to CHD. It should be noted that even simple, postoperative heart defects that were until recently considered to be harmless can lead to problems with age, a fact that had not been expected so far. The treatment of ACHD has many special features and requires special expertise. Thereby, it is important that treatment regimens from acquired heart disease are not necessarily transmitted to CHD. While primary care physicians have the important and responsible task to set the course for adequate diagnosis and treatment early and to refer patients to appropriate care in specialized ACHD-facilities, they should actively encourage ACHD to pursue follow-up care in specialized facilities who can provide responsible and advanced advice. This medical update emphasizes the current data on epidemiology, heart failure and cardiac arrhythmia in ACHD., Competing Interests: Conflicts of Interest: R Neidenbach received research grants (“Unrestricted educational grant”) from Actelion Pharmaceuticals Deutschland GmbH and from the German Heart Foundation (“Deutsche Herzstiftung e.V.) and the patient organization “Herzkind e. V.”. H Kaemmerer received fees and/or travel expenses for consulting activities and/or lectures from the following companies within the last 3 years: Actelion, Pfizer, Bayer-Healthcare, Bristol-Myers Squibb. D Pittrow has received speaker fees or honoraria for consultations from Actelion, Bayer, Genzyme, Boehringer Ingelheim, Novartis, MSD, and Pfizer. E Oechslin currently holds the “Bitove Family Professorship for Adult Congenital Heart Disease”. Other authors have no conflicts of interest to declare.

Objective: Patients with univentricular hearts (UVH) have high mortality despite modern treatment, and better methods to identify patients at highest risk are needed. We wanted to improve risk stratification in patients with UVH by focusing on the prognostic significance of single right versus single left ventricular morphology (SRV vs SLV)., Methods: All 395 patients with UVH operated at our centre were prospectively included from 1972 to 2016 (195 SRV, 166 SLV, 34 mixed or indeterminate ventricular morphology). Diagnoses, UVH morphology, types of all operations and time and causes of death or heart transplantation (HTX) were recorded. The primary endpoint was death or HTX., Results: Among the 111 non-Fontan patients, 88 died (SRV 62 vs SLV 20; p<0.0001), 32 due to heart failure (SRV 23 vs SLV 5; p=0.0012). Twenty-five  years of cumulative SRV versus SLV survival among the 284 Fontan patients (41 deaths/HTX) was 66.9% vs 87.9% (p=0.0027), partly explained by more deaths/HTX due to heart failure among patients with SRV (p=0.0006). Survival in patients with SRV with and without hypoplastic left heart syndrome (HLHS) was similar. SRV versus SLV was a strong predictor of death/HTX in multivariable proportional hazards analyses (RR 3.3, 95% CI 1.6 to 6.6)., Conclusion: SRV versus SLV is a strong short-term and long-term predictor of survival among patients with UVH, mainly explained by higher rates of death/HTX due to heart failure in the SRV group. Our findings apply to patients with SRV both with and without HLHS., Competing Interests: Competing interests: None declared.

Objectives: This study sought to determine the safety and feasibility of transcatheter pulmonary valve replacement (TPVR) using the Melody valve in native (nonconduit) right ventricular outflow tracts (nRVOT), and to identify factors associated with successful TPVR., Background: The Melody valve is Food and Drug Administration-approved for TPVR within right ventricle-to-pulmonary artery conduits and bioprosthetic pulmonary valves. However, most patients needing pulmonary valve replacement have nRVOT and TPVR has been adapted for this indication., Methods: In this multicenter retrospective study of all patients presenting for nRVOT TPVR, we collected pre-procedural magnetic resonance imaging, echocardiography, and catheterization data, and evaluated procedural and early outcomes., Results: Of 229 patients (age 21 ± 15 years from 11 centers), 132 (58%) had successful TPVR. In the remaining 97, TPVR was not performed, most often because of prohibitively large nRVOT (n = 67) or compression of the aortic root or coronary arteries (n = 18). There were no deaths and 5 (4%) serious complications, including pre-stent embolization requiring surgery in 4 patients, and arrhythmia in 1. Higher pre-catheterization echocardiographic RVOT gradient was associated with TPVR success (p = 0.001) and larger center volume approached significance (p = 0.08). Magnetic resonance imaging anterior-posterior and lateral RVOT diameters were smaller in implanted versus nonimplanted patients (18.0 ± 3.6 mm vs. 20.1 ± 3.5 mm; p = 0.005; 18.4 ± 4.3 mm vs. 21.5 ± 3.8 mm; p = 0.002)., Conclusions: TPVR in the nRVOT was feasible and safe. However, nearly half the patients presenting for catheterization did not undergo TPV implantation, mainly because of prohibitively large nRVOT size. Improved understanding of magnetic resonance imaging data and availability of larger devices may improve the success rate for nRVOT TPVR., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Over the past decade, as the majority of patients with single ventricle anatomy who have undergone the Fontan operation reach adulthood, a newly recognized disease process, Fontan-associated liver disease (FALD), has emerged. FALD is an extracardiac complication that may lead to substantial comorbid disease and premature mortality. The risk factors, pathophysiology, longitudinal consequences, and therapeutic options related to FALD remain poorly defined. Although we recognize that Fontan circulatory properties are associated with extracardiac organ dysfunction, numerous gaps in our understanding of the nature of this relationship exist. Such extracardiac manifestations, in addition to other late complications of the circulation, can significantly affect quality of life and healthcare use. Therefore, to initiate a formal evaluation of FALD, the American College of Cardiology (ACC) sponsored a stakeholders meeting on October 1 to 2, 2015, in Washington, DC. The goal of the meeting was to bring together subspecialty experts in the fields of adult and pediatric hepatology, congenital cardiology (adult congenital and pediatric cardiology), heart failure/transplant, epidemiology, and cardiothoracic surgery, as well as patient advocates, patients, parents of children and young adults who have had the Fontan procedure, and research organizations and societies to discuss the current state of FALD. Topics included gaps in knowledge, optimal care, research opportunities and barriers, and sound practices to guide providers, patients, and families. This report summarizes findings from the stakeholders meeting and seeks to establish a platform for understanding and addressing FALD., (Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Transcatheter pulmonary valve replacement (TPVR) is an established therapy for dysfunctional right ventricular (RV) outflow tract conduits. TPVR in patients with congenitally corrected transposition of the great arteries, subpulmonary left ventricle, and left ventricular outflow tract (LVOT) conduit dysfunction has not been studied. Unique anatomic and physiological aspects of this population may contribute to distinct risks and outcomes., Methods and Results: Across 10 US centers, 27 patients with a dysfunctional LVOT conduit were evaluated in the catheterization laboratory between December 2008 and August 2015 with the intent to perform TPVR. TPVR was successful in 23 patients (85%). Five serious adverse events occurred in 4 cases (15%), including pulmonary hemorrhage, hypotension requiring vasoactive support, conduit disruption requiring covered stent (n=2), and acute RV dysfunction with flash pulmonary edema. After TPVR, the LVOT peak systolic ejection gradient decreased from median of 35 to 17 mm Hg ( P <0.001); pulmonary insufficiency was trivial/none in all but 1 patient, where it was mild. Worsening of systemic RV dysfunction or tricuspid regurgitation was seen in 12 patients (57%) and was associated with a significantly lower post-TPVR LVOT peak systolic ejection gradient (median 17 versus 21 mm Hg; P =0.02) and higher post-TPVR RV sphericity index (median 0.88 versus 0.52; P =0.004). Post-TPVR, there were 2 late deaths because of RV failure and 1 cardiac transplantation because of progressive RV dysfunction and tricuspid regurgitation., Conclusions: TPVR in dysfunctional LVOT conduits is feasible but associated with an important rate of TPV nonimplantation and procedural serious adverse events. Worsening systemic RV function and tricuspid regurgitation may develop after LVOT TPVR., (© 2017 American Heart Association, Inc.)

Background: Atrial arrhythmias are the most common complication encountered in the growing and aging population with congenital heart disease., Objectives: This study sought to assess the types and patterns of atrial arrhythmias, associated factors, and age-related trends., Methods: A multicenter cohort study enrolled 482 patients with congenital heart disease and atrial arrhythmias, age 32.0 ± 18.0 years, 45.2% female, from 12 North American centers. Qualifying arrhythmias were classified by a blinded adjudicating committee., Results: The most common presenting arrhythmia was intra-atrial re-entrant tachycardia (IART) (61.6%), followed by atrial fibrillation (28.8%), and focal atrial tachycardia (9.5%). The proportion of arrhythmias due to IART increased with congenital heart disease complexity from 47.2% to 62.1% to 67.0% in patients with simple, moderate, and complex defects, respectively (p = 0.0013). Atrial fibrillation increased with age to surpass IART as the most common arrhythmia in those ≥50 years of age (51.2% vs. 44.2%; p < 0.0001). Older age (odds ratio [OR]: 1.024 per year; 95% confidence interval [CI]: 1.010 to 1.039; p = 0.001) and hypertension (OR: 2.00; 95% CI: 1.08 to 3.71; p = 0.029) were independently associated with atrial fibrillation. During a mean follow-up of 11.3 ± 9.4 years, the predominant arrhythmia pattern was paroxysmal in 62.3%, persistent in 28.2%, and permanent in 9.5%. Permanent atrial arrhythmias increased with age from 3.1% to 22.6% in patients <20 years to ≥50 years, respectively (p < 0.0001)., Conclusions: IART is the most common presenting atrial arrhythmia in patients with congenital heart disease, with a predominantly paroxysmal pattern. However, atrial fibrillation increases in prevalence and atrial arrhythmias progressively become permanent as the population ages., (Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Aims: We sought to identify factors associated with major adverse events (MAE) after cardiac catheterization in adolescents and adults with congenital heart disease (CHD), and create the first model to individualize risk discussions in this growing population., Methods and Results: Improving Pediatric and Adult Congenital Treatment (IMPACT), a National Cardiovascular Data Registry, contains congenital catheterization data from over 87 hospitals in the United States. Demographics, pre-procedure, and procedural variables were collected for patients over age 10. Multivariable logistic regression was used to identify significant predictors of MAE, a composite of death, urgent surgery or procedure due to a catheterization complication, transfusion, embolic stroke, tamponade, extracorporeal membrane oxygenation or ventricular assist device placement, and device embolization, malposition or thrombosis requiring surgical intervention. A risk score was built based on the effect sizes of each predictor and validated in a split sample. A MAE occurred in 686 (2.5%) of the 27 293 index procedures meeting inclusion criteria. The independent multivariate predictors of MAE were older age, pre-procedural anticoagulation use, renal disease, lower haemoglobin, lower oxygen saturation, non-elective procedure, higher index procedure risk and having had no prior cardiac procedures. Being underweight or overweight had borderline significance and was added to the model. The C-statistic for the model was robust at 0.787 in the derivation and 0.773 in the validation cohort., Conclusion: The factors predicting adverse events after cardiac catheterization in adolescents and adults with CHD are different than in the general population. Validation of this model in other national or multi-institutional datasets is the next step., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.)

Today, most female children born with congenital heart disease will reach childbearing age. For many women with complex congenital heart disease, carrying a pregnancy carries a moderate to high risk for both the mother and her fetus. Many such women, however, do not have access to adult congenital heart disease tertiary centers with experienced reproductive programs. Therefore, it is important that all practitioners who will be managing these women have current information not only on preconception counseling and diagnostic evaluation to determine maternal and fetal risk but also on how to manage them once they are pregnant and when to refer them to a regional center with expertise in pregnancy management., (© 2017 American Heart Association, Inc.)

Objectives: This study sought to describe the results of transcatheter tricuspid valve-in-ring (TVIR) implantation for treatment of tricuspid regurgitation (TR)., Background: Off-label use of transcatheter valves within surgically placed tricuspid annuloplasty prostheses has only been described in small reports. An international multicenter registry was developed to collect data on TVIR implantation., Methods: Data were collected from 13 sites on 22 patients (5 to 69 years of age) with TR who underwent catheterization with the intent to perform TVIR implantation., Results: TVIR implantation was performed in 20 patients (91%). Most patients were severely impaired (86% in New York Heart Association functional class III or IV); TR was severe in 86%. A Sapien valve (Edwards Lifesciences, Irvine, California) was implanted in 17 patients and a Melody valve (Medtronic, Minneapolis, Minnesota) in 3. There were no procedural deaths. There was 1 valve embolization requiring retrieval and placement of second TVIR implant and 1 valve malposition with severe paravalvular regurgitation requiring a second TVIR implantation. Over a median follow-up of 12 months, 1 patient died and 2 underwent repeat TVIR implantation, 1 of whom subsequently underwent surgical valve replacement. Significant paravalvular leak (PVL) was treated at the time of TVIR implantation in 4 patients: 3 underwent device occlusion and 1 received a second TVIR implant. On follow-up echocardiography, 15 patients had PVL (75%), the majority of which (n = 10) were trivial or mild and did not require treatment. PVL intervention was performed in 3 patients during follow-up. Functional capacity improved in most patients (70%)., Conclusions: TVIR implantation using commercially available transcatheter prostheses is technically feasible and clinically effective in reducing TR. Paravalvular regurgitation is common and may necessitate further interventions., (Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study aims to evaluate the safety and efficacy of transcatheter fenestrated ASD closure and to summarize the literature regarding the published techniques and outcomes of transcatheter partial ASD closure., Background: Patients with left ventricular diastolic dysfunction (LVDD) or right ventricular (RV) dysfunction and/or pulmonary hypertension (PHT) may suffer untoward consequences of complete closure of an ostium secundum atrial septal defect (ASD). Therefore, for patients that fall under these categories we suggest partial occlusion of the defect, which may be better tolerated than complete defect closure., Methods and Patients: After obtaining IRB approval, a search for patients that have undergone percutaneous ASD closure was performed in the Ahmanson/UCLA Adult Congenital Heart Disease Center database to identify which patients received a fenestrated ASD closure device., Results: Eight consecutive patients ranging between 22 and 83 years of age (mean 48 years) with PHT and/or LVDD or RV dysfunction who underwent fenestrated transcatheter ASD closure at UCLA were identified. None of the subjects experienced complications related to the procedure. Postprocedure clinical evaluation showed improvement in symptoms and exercise capacity. Available follow-up transthoracic echocardiography data (mean 4 months, range 0-20 months) demonstrated patent fenestrations in four of eight patients. None of the patients had thromboembolic or infectious complications and there were no device migrations, erosions or embolizations., Conclusions: Partial ASD occlusion in patients with diastolic dysfunction or RV dysfunction and/or PHT is safe and may be better tolerated than complete ASD closure in selected patients., (© 2016 Wiley Periodicals, Inc.)

Objective: Increasing numbers of lung transplant candidates have cardiac conditions that affect their survival after transplantation. Our objective was to determine if patients who undergo concomitant cardiac surgery (CCS) during the lung transplant procedure have similar outcomes, as a cohort of isolated lung transplant recipients., Methods: This was a retrospective, observational, matched-cohort analysis. The records of lung transplant recipients who underwent CCS from August 2000 to August 2013 were reviewed. A cohort of isolated lung transplant recipients, matched on the basis of age, lung allocation score, diagnosis, type of procedure, and era, was identified. The primary endpoint of this trial was 5-year survival. The secondary endpoints were primary graft dysfunction, grade III, at 72 hours, intensive care unit and hospital length of stay, and 5-year major adverse cardiac event rates., Results: A total of 120 patients underwent lung transplantation and CCS. Compared with the isolated lung transplant group, the donor, recipient, and operation characteristics were similar. No difference was found in the survival of the 2 groups for up to 5 years, or in the incidence of primary graft dysfunction Grade III at 72 hours, intensive care unit length of stay, invasive ventilation, hospital length of stay, or incidence of 5-year major adverse cardiac events., Conclusions: Lung transplant recipients undergoing CCS have early and midterm clinical outcomes similar to those of isolated lung transplant recipients. Given that this report is the largest published experience, offering cardiac surgery at the time of lung transplantation, to selected patients, remains justified., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

We report a highly unusual case of a 22-year-old woman with single-ventricle anatomy and a history of Fontan palliation who developed takotsubo cardiomyopathy. To our knowledge, takotsubo cardiomyopathy has not previously been described in a patient with single-ventricle anatomy, or more generally in any patient with congenital heart disease. The patient presented at our clinic for urgent examination after a 2-day history of chest pain that had begun upon the death of her dog. Invasive evaluation was refused by the patient; instead, she underwent electrocardiogram-gated coronary computed tomographic angiography, to rule out obstructive coronary disease. A physician who suspects takotsubo cardiomyopathy in a patient should look for new-onset chest pain and dyspnea coupled with these features: immediate onset of segmental akinesia in the midventricular or apical aspects of the heart, elevation of brain natriuretic peptide level 12 to 24 hours after onset of akinesia, elevation of troponin level 24 to 48 hours after onset, and disappearance of the segmental akinesia a few days after onset.

Pulmonary arterial hypertension (PAH) is characterized by abnormal elaboration of vasoactive peptides, endothelial cell dysfunction, vascular remodeling, and inflammation, which collectively contribute to its pathogenesis. We investigated the potential for high-density lipoprotein (HDL) dysfunction (i.e., proinflammatory effects) and abnormal plasma eicosanoid levels to contribute to the pathobiology of PAH and assessed ex vivo the effect of treatment with apolipoprotein A-I mimetic peptide 4F on the observed HDL dysfunction. We determined the "inflammatory indices" HII and LII for HDL and low-density lipoprotein (LDL), respectively, in subjects with idiopathic PAH (IPAH) and associated PAH (APAH) by an in vitro monocyte chemotaxis assay. The 4F was added ex vivo, and repeat LII and HII values were obtained versus a sham treatment. We further determined eicosanoid levels in plasma and HDL fractions from patients with IPAH and APAH relative to controls. The LIIs were significantly higher for IPAH and APAH patients than for controls. Incubation of plasma with 4F before isolation of LDL and HDL significantly reduced the LII values, compared with sham-treated LDL, for IPAH and APAH. The increased LII values reflected increased states of LDL oxidation and thereby increased proinflammatory effects in both cohorts. The HIIs for both PAH cohorts reflected a "dysfunctional HDL phenotype," that is, proinflammatory HDL effects. In contrast to "normal HDL function," the determined HIIs were significantly increased for the IPAH and APAH cohorts. Ex vivo 4F treatment significantly improved the HDL function versus the sham treatment. Although there was a significant "salutary effect" of 4F treatment, this did not entirely normalize the HII. Significantly increased levels for both IPAH and APAH versus controls were evident for the eicosanoids 9-HODE, 13-HODE, 5-HETE, 12-HETE, and 15-HETE, while no statistical differences were evident for comparisons of IPAH and APAH for the determined plasma eicosanoid levels in the HDL fractions. Our study has further implicated the putative role of "oxidant stress" and inflammation in the pathobiology of PAH. Our data suggest the influences on the "dysfunctional HDL phenotype" of increased oxidized fatty acids, which are paradoxically proinflammatory. We speculate that therapies that target either the "inflammatory milieu" or the "dysfunctional HDL phenotype," such as apoA-I mimetic peptides, may be valuable avenues of further research in pulmonary vascular diseases.