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Severe mitral valve regurgitation (MR) is a heart valve disease that progresses to end-stage congestive heart failure and death if left untreated. Surgical repair or replacement of the mitral valve (MV) remains the gold standard for treatment of severe MR, with repair techniques aiming to restore the native geometry of the MV. However, patients with extensive co-morbidities may be ineligible for surgical intervention. With the emergence of transcatheter MV repair (TMVR) treatment paradigms for MR will evolve. The longer-term outcomes of TMVR and its effectiveness compared to surgical repair remain unknown given the differing patient eligibility for either treatment at this time. Advances in computational modeling will elucidate answers to these questions, employing techniques such as finite element method and fluid structure interactions. Use of clinical imaging will permit patient-specific MV models to be created with high accuracy and replicate MV pathophysiology. It is anticipated that TMVR technology will gradually expand to treat lower-risk patient groups, thus pre-procedural computational modeling will play a crucial role guiding clinicians towards the optimal intervention. Additionally, concerted efforts to create MV models will establish atlases of pathologies and biomechanics profiles which could delineate which patient populations would best benefit from specific surgical vs. TMVR options. In this review, we describe recent literature on MV computational modeling, its relevance to MV repair techniques, and future directions for translational application of computational modeling for treatment of MR.

Background With improving survival of patients with single ventricle physiology who underwent Fontan palliation, there is also an increase in the prevalence of overweight and obesity in these patients. This tertiary care single‐center study aims to determine the association of body mass index (BMI) with the clinical characteristics and outcomes in adults with Fontan. Methods and Results Adult patients (aged ≥18 years) with Fontan who were managed at a single tertiary care center between January 1, 2000, and July 1, 2019, and had BMI data available were identified via retrospective review of medical records. Univariate and multivariable (after adjusting for age, sex, functional class, and type of Fontan) linear and logistic regression, as appropriate, were utilized to evaluate associations between BMI and diagnostic testing and clinical outcomes. A total of 163 adult patients with Fontan were included (mean age, 29.9±9.08 years), with a mean BMI of 24.2±5.21 kg/m 2 (37.4% of patients had BMI ≥25 kg/m 2 ). Echocardiography data were available for 95.7% of patients, exercise testing for 39.3% of patients, and catheterization for 53.7% of patients. Each SD increase in BMI was significantly associated with decreased peak oxygen consumption ( P =0.010) on univariate analysis and with increased Fontan pressure ( P =0.035) and pulmonary capillary wedge pressure ( P =0.037) on multivariable analysis. In addition, BMI ≥25 kg/m 2 was independently associated with heart failure hospitalization (adjusted odds ratio [AOR], 10.2; 95% CI, 2.79–37.1 [ P P =0.029]). Conclusions Elevated BMI is associated with poor hemodynamics and worse clinical outcomes in adult patients with Fontan. Whether elevated BMI is the cause or consequence of poor clinical outcomes needs to be further established.

Background There are significant risks of parenteral prostacyclin use in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), which may limit their use. Selexipag is an oral, selective prostacyclin analogue that has been shown to reduce disease progression and improve exercise capacity in patients with PAH-CHD. Administering Selexipag in patients with PAH-CHD could potentially overcome some of the risks of parenteral therapy while improving clinical outcomes. Case summary We report five cases highlighting the clinical uses of Selexipag in patients with PAH-CHD. In the first two cases, Selexipag was initiated as part of a Treat-to-close strategy. In the third case, initiation of Selexipag improved symptoms and objective exercise capacity in a patient with Eisenmenger syndrome. In the fourth and fifth cases, rapid cross-titration protocols were used to transition from parenteral prostacyclins to Selexipag. In the fourth case, Selexipag was initiated in the context of significant side effects limiting parenteral prostacyclin use. In the fifth case, Selexipag was used to down-titrate from parenteral prostacyclins following closure of a sinus venosus atrial septal defect and redirection of anomalous pulmonary veins. Discussion Selexipag is a promising oral therapy for patients with at various stages of the spectrum of PAH-CHD to improve symptoms, exercise capacity and, in some cases, haemodynamics. Our cases also highlight practical aspects of Selexipag use including targeting the individualized maximally tolerated dose for each patient, managing side effects and managing dose interruptions.

We present this case of a 22-year-old woman with congenital heart disease as the youngest reported patient, to the best of our knowledge, to successfully undergo treatment of medically refractory torrential tricuspid regurgitation with the MitraClip system as an adjunct to guideline directed therapy. (Level of Difficulty: Advanced.), Graphical abstract, We present this case of a 22-year-old woman with congenital heart disease as the youngest reported patient, to the best of our knowledge, to…

Background: In recent years, patient-reported outcomes (PROs) have received increasing prominence in cardio-vascular research and clinical care. An understanding of the variability and global experience of PROs in adults with congenital heart disease (CHD), however, is still lacking. Moreover, information on epidemiological characteristics and the frailty phenotype of older adults with CHD is minimal. The APPROACH-IS II study was established to address these knowledge gaps. This paper presents the design and methodology of APPROACH-IS II.Methods/design: APPROACH-IS II is a cross-sectional global multicentric study that includes Part 1 (assessing PROs) and Part 2 (investigating the frailty phenotype of older adults). With 53 participating centers, located in 32 countries across six continents, the aim is to enroll 8000 patients with CHD. In Part 1, self-report surveys are used to collect data on PROs (e.g., quality of life, perceived health, depressive symptoms, autonomy support), and explanatory variables (e.g., social support, stigma, illness identity, empowerment). In Part 2, the cognitive functioning and frailty phenotype of older adults are measured using validated assessments.Discussion: APPROACH-IS II will generate a rich dataset representing the international experience of individuals in adult CHD care. The results of this project will provide a global view of PROs and the frailty phenotype of adults with CHD and will thereby address important knowledge gaps. Undoubtedly, the project will contribute to the overarching aim of improving optimal living and care provision for adults with CHD., KU Leuven - University of Leuven; Research Foundation Flanders [1159522N, 12E9819N]; Swedish Heart and Lung Foundation [20190525]; Research Council of Aga Khan University in Karachi in Pakistan; Maltese Cardiac Society; Children's Heart Unit Fund; UnIC (Unidade de Investigacao Cardiovascular da FMUP); Italian Ministry of Health, This work is supported by KU Leuven - University of Leuven, Research Foundation Flanders through grants 1159522N and 12E9819N, Swedish Heart and Lung Foundation through grant 20190525, the University Research Council of the Aga Khan University in Karachi in Pakistan, Maltese Cardiac Society, UnIC (Unidade de Investigacao Cardiovascular da FMUP) supported the costs of the translations into Portuguese used in Portugal, Children's Heart Unit Fund ( www.chuf.org.uk), Newcastle upon Tyne through their funding of the CHUF Fontan nurse specialist. This study was partially supported by Ricerca Corrente funding from the Italian Ministry of Health to IRCCS Policlinico San Donato.

Severe mitral regurgitation (MR) is a cardiac disease that can lead to fatal consequences. MitraClip (MC) intervention is a percutaneous procedure whereby the mitral valve (MV) leaflets are connected along the edge using MCs. The outcomes of the MC intervention are not known in advance, i.e., the outcomes are quite variable. Artificial intelligence (AI) can be used to guide the cardiologist in selecting optimal MC scenarios. In this study, we describe an atlas of shapes as well as different scenarios for MC implantation for such an AI analysis. We generated the MV geometrical data from three different sources. First, the patients' 3-dimensional echo images were used. The pixel data from six key points were obtained from three views of the echo images. Using PyGem, an open-source morphing library in Python, these coordinates were used to create the geometry by morphing a template geometry. Second, the dimensions of the MV, from the literature were used to create data. Third, we used machine learning methods, principal component analysis, and generative adversarial networks to generate more shapes. We used the finite element (FE) software ABAQUS to simulate smoothed particle hydrodynamics in different scenarios for MC intervention. The MR and stresses in the leaflets were post-processed. Our physics-based FE models simulated the outcomes of MC intervention for different scenarios. The MR and stresses in the leaflets were computed by the FE models for a single clip at different locations as well as two and three clips. Results from FE simulations showed that the location and number of MCs affect subsequent residual MR, and that leaflet stresses do not follow a simple pattern. Furthermore, FE models need several hours to provide the results, and they are not applicable for clinical usage where the predicted outcomes of MC therapy are needed in real-time. In this study, we generated the required dataset for the AI models which can provide the results in a matter of seconds.

ObjectiveThis tertiary centre study aims to identify factors associated with adverse outcomes in adult survivors with total cavopulmonary connection (TCPC) Fontan palliation for single ventricle.MethodsThis retrospective review of medical records identified adult (≥18 years) survivors of TCPC Fontan palliation who were followed at a single tertiary centre between 1 January 2000 and 1 July 2019. Adverse outcomes were defined as arrhythmia, pacemaker/implantable cardioverter defibrillator placement, liver cirrhosis, protein losing enteropathy, hospitalisation for heart failure, thromboembolic complication and/or death.Results160 adult TCPC patients met the inclusion criteria: 117 (73.1%) extracardiac and 43 (26.9%) lateral tunnel. The median (IQR) duration of follow-up since TCPC palliation was 17.5 (11.8–21.3) years. An adverse outcome occurred in 87 (54.4%) patients. Adverse outcome-free survival rates at 10, 20 and 25 years post TCPC were 89% (95% CI 82% to 93%), 60% (95% CI 50% to 69%) and 24% (95% CI 15% to 35%), respectively. On multivariate analysis, extracardiac Fontan (HR 2.21, 95% CI 1.20 to 4.08, p=0.011) was observed to be an independent risk factor for adverse outcomes after adjusting for age, race, morphology of the systemic ventricle and history of fenestration.ConclusionsIn this single-centre retrospective study of adult survivors of TCPC palliation, extracardiac Fontan was associated with an increased hazard for adverse outcomes. This finding could guide clinicians in developing risk modification strategies and management decisions to improve long-term outcomes in these patients.

Background Clinicians vary markedly in their ability to detect murmurs during cardiac auscultation and identify the underlying pathological features. Deep learning approaches have shown promise in medicine by transforming collected data into clinically significant information. The objective of this research is to assess the performance of a deep learning algorithm to detect murmurs and clinically significant valvular heart disease using recordings from a commercial digital stethoscope platform. Methods and Results Using >34 hours of previously acquired and annotated heart sound recordings, we trained a deep neural network to detect murmurs. To test the algorithm, we enrolled 962 patients in a clinical study and collected recordings at the 4 primary auscultation locations. Ground truth was established using patient echocardiograms and annotations by 3 expert cardiologists. Algorithm performance for detecting murmurs has sensitivity and specificity of 76.3% and 91.4%, respectively. By omitting softer murmurs, those with grade 1 intensity, sensitivity increased to 90.0%. Application of the algorithm at the appropriate anatomic auscultation location detected moderate‐to‐severe or greater aortic stenosis, with sensitivity of 93.2% and specificity of 86.0%, and moderate‐to‐severe or greater mitral regurgitation, with sensitivity of 66.2% and specificity of 94.6%. Conclusions The deep learning algorithm’s ability to detect murmurs and clinically significant aortic stenosis and mitral regurgitation is comparable to expert cardiologists based on the annotated subset of our database. The findings suggest that such algorithms would have utility as front‐line clinical support tools to aid clinicians in screening for cardiac murmurs caused by valvular heart disease. Registration URL: https://clinicaltrials.gov ; Unique Identifier: NCT03458806.

Conduction disturbances following TAVR are a common occurrence given the proximity of the various conduction system tissues, including the AV node, His-bundle, and bundle branches to the left ventricular outflow tract and aortic root. Impairment of these conduction system abnormalities may necessitate permanent pacemaker implantation, which increases morbidity and mortality, as well as length of stay, for the patient. The incidence, mechanisms, and predictors of conduction abnormalities and treatment options are discussed in this up-to-date review of the topic.

A 50-year-old man presented in cardiogenic shock. Echocardiogram showed ejection fraction (EF) 22%, apical thrombus, and severe bicuspid aortic stenosis. Transcatheter aortic valve replacement (TAVR) was recommended, given his high surgical risk. Urgent TAVR was performed without complication, using transcatheter cerebral embolic protection and intracardiac echocardiography (ICE) for left-ventricular wire placement. The patient was discharged on warfarin, and follow-up echocardiogram showed no apical thrombus, EF 55%, and well-functioning bioprosthesis. This case shows a good TAVR outcome in bicuspid aortic stenosis despite apical thrombus and poor EF. Cerebral embolic protection and ICE can minimize risk of stroke.

The ongoing severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic has profoundly impacted all fields of medicine. Infection with SARS-CoV-2 and the resulting coronavirus of 2019 (COVID-19) syndrome has multiorgan effects. The pandemic has united researchers from bench to bedside in attempts to understand the pathophysiology of the disease and define optimal treatment strategies. Cardiovascular disease is highly prevalent and a leading cause of death across gender, race, and ethnic groups. As the pandemic spreads, there is increasing concern about the cardiovascular effects of the viral infection and the interaction of infection with existing cardiovascular disease. Additionally, there are concerns about the cardiac effects of the numerous treatment agents under study. It will be essential for cardiologists to understand the interplay between underlying cardiac comorbidities, acute cardiovascular effects of COVID-19 disease, and adverse effects of new treatments. Here we describe emerging evidence of the epidemiology of SARS-CoV-2 infection and underlying cardiovascular disease, the evidence for direct myocardial injury in SARS-CoV-2 infection, the specific presentations of cardiovascular involvement by SARS-CoV-2, and the cardiac effects of emerging treatments.

Introduction: Patent ductus arteriosus (PDA) is often recognized and treated with percutaneous closure in adults. However, the impact on cardiac reverse remodeling following PDA closure in adults is not clear. We performed a meta-analysis to characterize the extent of cardiac remodeling following percutaneous PDA closure in adults. Methods: MEDLINE and EMBASE were systematically searched for original studies that reported echocardiographic variables at baseline, immediately post-procedure (within 24 hours), and at follow-up (>1 month) in adults undergoing percutaneous PDA closure. Additionally, we included echocardiographic data from a cohort of patients >18 years of age that underwent percutaneous PDA closure between 01/2015 and 12/2019 at our centre. For parameters with sufficient data for pooling, weighted averages were calculated, and pooled differences were presented as weighted mean differences. Heterogeneity was assessed using the I 2 statistic. Results: After screening 278 abstracts, 5 studies were identified. When combined with our own cohort of 13 patients, our meta-analysis encompassed 244 patients. The weighted mean age of all patients was 33 years with all studies predominantly comprised of female patients and the median follow-up was 12 months (ranging from 1 month- 5 years across the studies). When compared to baseline, left ventricular ejection fraction (LVEF) decreased significantly immediately post-procedure and all parameters significantly decreased at follow-up (Table 1). Conclusions: As demonstrated by the decreases in the left ventricular and left atrial sizes, reverse remodeling was observed in adults who underwent percutaneous PDA closure. The significantly lower LVEF immediately post-procedure could reflect withdrawal of chronic volume overload or increased afterload. The clinical significance of the statistically significant lower LVEF on follow-up testing is unclear and requires further evaluation.

Background: Arguments against universal health insurance are ongoing, but insurance coverage may be beneficial in high-risk populations such as adults with congenital heart disease (ACHD). While the number of hospital visits have exponentially increased for ACHD over the last few decades, the impact of insurance on the type of hospital encounters remains unknown. Hypothesis: Uninsured ACHD patients are more likely to have emergent hospital encounters than those insured. Methods: Using California Office of Statewide Health Planning and Development Database from 01/2005 to 10/2015, we identified all hospital encounters that were emergent (which might or might not have resulted in an admission) or nonemergent among ACHD ≥18 years old. We determined the trends over time and odds of insurance status on emergent vs. nonemergent encounters. Results: Among 69,876,425 encounters, 72,142 were in patients with CHD diagnoses (mean age 49±12 years, 43% males, 52% Caucasian, 4% uninsured). 78% had severe CHD and 75% had a comorbidity. From 2005 to 2015, while all ACHD encounters increased by 108% (p for trend Conclusions: Efforts to enhance the ability to obtain and maintain insurance throughout the lifetime of ACHD patients might result in meaningful reductions in emergency encounters and a more efficient use of resources.

OBJECTIVES This study evaluates in-hospital, 30-day, and 1-year outcomes post-transcatheter aortic valve replacement (TAVR) in end stage liver disease (ESLD) and/or end stage renal disease (ESRD) compared with patients without these comorbidities. BACKGROUND TAVR is an alternative to surgical aortic valve replacement in patients with ESLD and ESRD, though current outcomes data are limited. METHODS We compared 309 patients (N = 29 ESLD and/or ESRD, N = 280 control) age > 18 who underwent transfemoral TAVR from 2014 to 2020 have been compared. RESULTS Patients with ESLD and ESRD were younger (69.9 ± 11.7 vs. 79.1 ± 9.8, p

This article highlights our current understanding of bicuspid aortic valve (BAV) syndrome and how genetics, pathology, imaging features, and clinical symptoms direct the clinical management, surveillance, and intervention of those with BAV. While randomized data and guidelines are lacking, transcatheter therapies are routinely used for BAV stenosis in medical centers throughout the world. Guidelines suggest that younger patients age less than 50 years undergo surgical aortic valve replacement (AVR) with a mechanical valve and the elderly > 70 years of age receive a bioprosthetic valve. For the age group 50–70 years, either option should be considered. Decision on type of intervention depends on specific features such as presence of stenosis or regurgitation, if there is any associated aortopathy, comorbid illnesses, contraindications, and patient preference. We have learned that the decision to intervene and mode of intervention is best achieved by a multidisciplinary valve team. Despite improvement in our understanding of BAV and the advancement of novel therapies particularly for BAV stenosis, much is still to be learned about the optimal treatment of BAV disease in the current era.

Aortic stenosis is the most common valvular heart disease in industrialized countries and the most common cause of left ventricular outflow tract (LVOT) obstruction. Transcatheter aortic valve replacement (TAVR) is an alternative to surgical aortic valve replacement for intermediate to high-risk surgical candidates with symptomatic severe aortic stenosis. Conduction system abnormalities, including atrioventricular (AV) and intraventricular (IV) block, are the most common complication of TAVR. In this review, we aim to explore the anatomical issues relevant to atrioventricular block, the relevant clinical and procedural aspects, and the management and long-term implications of AV and IV block.

Background The risk of surgical mitral valve replacement in patients with severe mitral annular calcification (MAC) is high. Several patients worldwide with severe MAC have been treated successfully with transcatheter mitral valve replacement (TMVR) using balloon-expandable aortic transcatheter valves. The TMVR in MAC Global Registry is a multicenter registry that collects data on outcomes of these procedures. Objectives The goal of this study was to evaluate 1-year outcomes in this registry. Methods This study was a multicenter retrospective review of clinical outcomes. Results A total of 116 extreme surgical risk patients with severe MAC underwent TMVR; 106 had a procedure date >1 year before data-lock and were included in the analysis. Their mean age was 73 ± 12 years, and 68% were female. The mean Society of Thoracic Surgeons score was 15.3 ± 11.6%, and 90% were in New York Heart Association functional class III or IV. Thirty-day and 1-year all-cause mortality was 25% and 53.7%, respectively. Most patients who survived 30 days were alive at 1 year (49 of 77 [63.6%]), and the majority (71.8%) were in New York Heart Association functional class I or II. Echocardiography data at 1 year were available in 34 patients. Mean left ventricular ejection fraction was 58.6 ± 11.2%, mean mitral valve area was 1.9 ± 0.5 cm2, mean mitral gradient was 5.8 ± 2.2 mm Hg, and 75% had zero or trace mitral regurgitation. Conclusions TMVR with balloon-expandable aortic valves in extreme surgical risk patients with severe MAC is feasible but associated with high 30-day and 1-year mortality. Most patients who survive the 30-day post-procedural period are alive at 1 year and have sustained improvement of symptoms and transcatheter valve performance. The role of TMVR in patients with MAC requires further evaluation in clinical trials.

Key Teaching Points • Transseptal puncture results in the formation of an iatrogenic atrial septal defect (ASD). In patients with right side–dominant cardiac pathologies (such as arrhythmogenic right ventricular cardiomyopathy), the transseptal pressure gradient from the right to left atrium promotes the formation of a right-to-left shunt across this ASD. • A right-to-left shunt across an iatrogenic ASD should be considered in these patients who present with dypsnea following their procedure, particularly if hypoxemic and cyanotic. • Potential strategies to minimize the risk of this complication in this population include avoiding “double sheathing” a single transeptal puncture, avoiding the use of larger sheaths, and assessing a right-to-left shunt at the conclusion of the case with echocardiography. • Patients who remain symptomatic of a right-to-left shunt should undergo ASD closure.

To evaluate the effect of MRI blood pool contrast agent on 4D flow image quality and ventricular volume measurements. Adult patients referred for clinical cardiac MRI (n = 22) were imaged with 4D flow. Patients with renal failure (n = 10) received ferumoxytol, and the remainder (n = 12) received gadofosveset trisodium. Image quality was assessed with (1) signal-to-noise ratio (SNR); (2) contrast-to-noise ratio (CNR); and (3) 5-point Likert scale based on endocardial border definition (1 = none; 2 = partial but unable to visualize; 3 = able to roughly estimate; 4 = visible for most of the cardiac cycle; 5 = excellent definition). A subset (n = 15) had short axis steady-state free precession (SSFP) cine imaging allowing for comparison of standard volumetric measurement technique with 4D flow derived volumetric measurements. 4D flow studies using ferumoxytol demonstrated a higher median Likert score of 5 (IQR, 5–5) versus 3 (IQR, 2–3). Median cavity SNR and CNR were higher for ferumoxytol compared to gadofosveset trisodium [65 (IQR, 50–74) versus 22 (IQR, 14–28), p

Left ventricle—right atrial communication could be congenital (Gerbode defect) or acquired as a complication of surgery or infective endocarditis and leads to volume overloading of pulmonary circulation. Two types, direct and indirect types are known depending on the involvement of septal tricuspid leaflet. Transcatheter closure of this defect is feasible and appears an attractive alternative to surgical management. Various devices like Amplatzer duct occluder I, II, Muscular ventricular septal defect device etc. have been used to close this defect. We report two patients, a preteen boy with direct left ventricle-right atrial communication as post operative complication and an adult female with indirect communication who underwent transcatheter closure with Cera duct occluder (Lifetech Scientific (Shenzhen), China).

Background As patients with congenital heart disease ( CHD ) are living longer, understanding the comorbidities they develop as they age is increasingly important. However, there are no published population‐based estimates of the comorbidity burden among the US adult patients with CHD. Methods and Results Using the IBM MarketScan commercial claims database from 2010 to 2016, we identified adults aged ≥18 years with CHD and 2 full years of continuous enrollment. These were frequency matched with adults without CHD within categories jointly defined by age, sex, and dates of enrollment in the database. A total of 40 127 patients with CHD met the inclusion criteria (mean [ SD ] age, 36.8 [14.6] years; and 48.2% were women). Adults with CHD were nearly twice as likely to have any comorbidity than those without CHD ( P CHD ” (risk ratio, 9.41; 95% CI , 7.99–11.1), “other cardiovascular” (risk ratio, 1.73; 95% CI , 1.66–1.80), and “noncardiovascular” (risk ratio, 1.47; 95% CI , 1.41–1.52) comorbidities. After adjusting for covariates and considering interaction with age, patients with severe CHD had higher risks of previously recognized to be common in CHD and lower risks of other cardiovascular comorbidities than age‐stratified patients with nonsevere CHD . For noncardiovascular comorbidities, the risk was higher among patients with severe than nonsevere CHD before, but not after, the age of 40 years. Conclusions Our data underscore the unique clinical needs of adults with CHD compared with their peers. Clinicians caring for CHD may want to use a multidisciplinary approach, including building close collaborations with internists and specialists, to help provide appropriate care for the highly prevalent noncardiovascular comorbidities.

© 2016 Informa UK Limited, trading as Taylor & Francis Group. Introduction: Patent foramen ovale (PFO) is a common congenital cardiac abnormality and that has been associated with several disease processes including transient ischemic attacks (TIA), stroke, migraine headaches with aura, decompression sickness, platypnea-orthodeoxia syndrome, and shunt induced cyanosis. Controversy exists regarding closure of PFO as a therapeutic treatment modality for these disease processes. This review addresses the contemporary clinical indications for PFO closure. Areas covered: We conducted a comprehensive literature search of contemporary research studies focusing on randomized trials and meta-analyses comparing medical therapy and device closure of PFOs for the treatment of PFO associated clinical syndromes. We synthesized this literature into a review addressing indications for PFO closure in stroke, TIA, migraine headaches with aura, decompression sickness, platypnea-orthodeoxia syndrome, and shunt induced cyanosis. Expert commentary: Because in many PFO associated conditions it can be difficult to determine the degree to which the PFO is a causative factor in the disease process, we recommend a comprehensive diagnostic evaluation to exclude other obvious etiologies of PFO associated conditions before implicating the PFO and proceeding with closure. However in the properly selected patient population there is growing clinical experience and experimental evidence suggesting that closure of PFO is a safe and effective treatment modality.

Background The most common reason for reintervention after a Bjork modification of the Fontan procedure, in which the right ventricle (RV) is incorporated into the pulmonary circulation by connecting the right atrial (RA) appendage to the RV directly or with an extra-anatomic graft, is obstruction or regurgitation of the RA–RV connection. Transcatheter implantation of a valved stent is an appealing option for the treatment of RA–RV conduit dysfunction in these patients. In the present study, we assessed early and intermediate results after transcatheter valve implantation within an obstructed or regurgitant RA–RV modified Fontan pathway. Methods Through a retrospective multicenter registry, we collected data from 16 patients with a modified Fontan circulation who were treated with percutaneous Melody or Sapien valve implantation for dysfunction of an extra-anatomic RA–RV conduit or valve. Results All patients had successful and uncomplicated implantation of a Melody (n = 15) or Sapien 3 (n = 1) valve with hemodynamic and, in most cases, clinical improvement. During a median follow-up of 3.3 years, 3 patients died of cardiovascular causes unrelated to the procedure or the valve, and no major valve dysfunction was observed. Conclusion Percutaneous transcatheter valve deployment to treat a dysfunctional RA–RV connection after a Bjork modification of the Fontan procedure is a viable alternative to surgery, with low procedural risk, and appears to offer good early and intermediate results.© 2016 Wiley Periodicals, Inc.

Objectives This study sought to evaluate the outcomes of the early experience of transcatheter mitral valve replacement (TMVR) with balloon-expandable valves in patients with severe mitral annular calcification (MAC) and reports the first large series from a multicenter global registry. Background The risk of surgical mitral valve replacement in patients with severe MAC is high. There are isolated reports of successful TMVR with balloon-expandable valves in this patient population. Methods We performed a multicenter retrospective review of clinical outcomes of patients with severe MAC undergoing TMVR. Results From September 2012 to July of 2015, 64 patients in 32 centers underwent TMVR with compassionate use of balloon-expandable valves. Mean age was 73 ± 13 years, 66% were female, and mean Society of Thoracic Surgeons score was 14.4 ± 9.5%. The mean mitral gradient was 11.45 ± 4.4 mm Hg and the mean mitral area was 1.18 ± 0.5 cm 2 . SAPIEN valves (Edwards Lifesciences, Irvine, California) were used in 7.8%, SAPIEN XT in 59.4%, SAPIEN 3 in 28.1%, and Inovare (Braile Biomedica, Brazil) in 4.7%. Access was transatrial in 15.6%, transapical in 43.8%, and transseptal in 40.6%. Technical success according to Mitral Valve Academic Research Consortium criteria was achieved in 46 (72%) patients, primarily limited by the need for a second valve in 11 (17.2%). Six (9.3%) had left ventricular tract obstruction with hemodynamic compromise. Mean mitral gradient post-procedure was 4 ± 2.2 mm Hg, paravalvular regurgitation was mild or absent in all. Thirty-day all-cause mortality was 29.7% (cardiovascular = 12.5% and noncardiac = 17.2%); 84% of the survivors with follow-up data available were in New York Heart Association functional class I or II at 30 days (n = 25). Conclusions TMVR with balloon-expandable valves in patients with severe MAC is feasible but may be associated with significant adverse events. This strategy might be an alternative for selected high-risk patients with limited treatment options.

SummaryBackgroundEndovascular stenting is a recognised treatment strategy for aortic coarctation (CoA) in adults. We assessed systemic hypertension control and the need for antihypertensive therapy after CoA stenting in adults.MethodsData were collected prospectively on 54 patients (36 men; mean age: 34±16 years) who underwent endovascular stenting for CoA over a 7-year period. Five patients were excluded as they did not attend follow-up appointments. Patients underwent clinical examination, including right arm systolic blood pressure (SBP) and 24-hour ambulatory blood pressure monitoring at baseline, 6–12 weeks and 9–12 months.ResultsThere was a significant fall in mean peak-to-peak systolic gradient (PG) across the CoA after stenting (26±11mmHg vs. 5±4mmHg; P25mmHg before stenting); baseline SBP was significantly higher in severe versus non-severe patients (160mmHg vs. 148mmHg; P=0.02). The absolute reduction in PG after stenting was significantly higher in the severe group (31±7mmHg vs. 14±5mmHg; P

ObjectiveTo evaluate the predictive value of Computed Tomography Angiography (CTA) measurements of the RVOT for transcatheter valve sizing.BackgroundTranscatheter pulmonary valve replacement (TPVR) provides an alternative to surgery in patients with right ventricular outflow tract (RVOT) dysfunction. We studied 18 patients who underwent catheterization for potential TPVR to determine whether CT imaging can be used to accurately predict implant size.MethodsCases were grouped by RVOT characteristics: native or transannular patch (n=8), conduit (n=5) or bioprosthetic valve (n=5). TPVR was undertaken in 14/18 cases, after balloon-sizing was used to confirm suitability and select implant size. Retrospective CT measurements of the RVOT (circumference-derived (Dcirc) and area-derived (Darea) diameters) were obtained at the level of the annulus, bioprosthesis or conduit. Using manufacturer sizing guidance, a valve size was generated and a predicted valve category assigned: (1) 29mm. Predicted and implanted valves were compared for inter-rater agreement using Cohen's kappa coefficient.ResultsThe median age of patients was 37years old (IQR: 30-49); 55% were male. Diagnoses included: Tetralogy of Fallot (12/18), d-Transposition repair (3/18), congenital pulmonary stenosis (2/18) and carcinoid heart disease (1/18). Measurements of Darea (κ=0.697, p&nbsp

Right ventricular outflow tract (RVOT) dysfunction is a common hemodynamic challenge for adults with congenital heart disease (ACHD), including patients with repaired tetralogy of Fallot (TOF), truncus arteriosus (TA), and those who have undergone the Ross procedure for congenital aortic stenosis and the Rastelli repair for transposition of great vessels. Pulmonary valve replacement (PVR) has become one of the most common procedures performed for ACHD patients. Areas covered: Given the advances in transcatheter technology, we conducted a detailed review of the available studies addressing the indications for PVR, historical background, evolving technology, procedural aspects, and the future direction, with an emphasis on ACHD patients. Expert commentary: Transcatheter pulmonary valve implantation (TPVI) is widely accepted as an alternative to surgery to address RVOT dysfunction. However, current technology may not be able to adequately address a subset of patients with complex RVOT morphology. As the technology continues to evolve, new percutaneous valves will allow practitioners to apply the transcatheter approach in such patients. We expect that with the advancement in transcatheter technology, novel devices will be added to the TPVI armamentarium, making the transcatheter approach a feasible alternative for the majority of patients with RVOT dysfunction in the near future.

We describe a hybrid surgical interventional technique for minimally invasive implantation of a transcatheter aortic valve in the mitral position. This technique does not require circular mitral annular calcification and minimizes the risk of left ventricular outflow tract obstruction. We believe this technique represents a useful approach for surgeons and interventional cardiologists until specialized transcatheter mitral valve devices become available.

Introduction Patients undergoing structural heart interventions often require large-sized sheath insertion into femoral arteries and veins. Clinical outcome data on the use of suture-mediated devices for large femoral arterial access in structural heart interventions is limited. We assessed the efficacy of the Perclose™ (Abbott Vascular Devices, Santa Clara, CA, USA) suture-mediated device using the pre-closure technique in achieving hemostasis in femoral arterial access sites following large sheath insertion (≥8 Fr). Methods One hundred consecutive patients underwent 101 femoral artery access sites closures with the Perclose device using the pre-closure technique. Sixty-two percent of the patients were male and their mean (SD) age was 52 (±26) years. All patients received heparin. Results Mean arterial access site sheath diameter was 13 ± 2 Fr. Immediate hemostasis was achieved in 96/101 (96%) procedures (≤2 min). Two patients (2%) had access site-related complications requiring further interventions. On clinical follow up [mean (SD) follow-up of 24 (±12) months and median follow-up of 8.5 months], no complications were seen in the arterial access sites. Conclusion Pre-closure of large-size femoral arterial access sheath sites using the suture-mediated Perclose device is efficacious in achieving rapid hemostasis in patients undergoing structural interventions. On 1-year follow-up, there were no arterial access site complications requiring further investigations or interventions. Electronic supplementary material The online version of this article (doi:10.1007/s40119-014-0034-7) contains supplementary material, which is available to authorized users.

Summary Background Liver disease (LD) is a long-term complication in patients with a single ventricle who have had the Fontan operation. A decline in cardiopulmonary exercise testing (CPET) variables is associated with increased risk of hospitalization, but its association with LD is unknown. Aim To determine the association between CPET variables and LD in adults who have had the Fontan operation. Methods We retrospectively reviewed the medical records from two tertiary institutions. Results We identified 114 adults (≥ 18 years; mean 30.9 ± 7.4 years) who had undergone the Fontan operation: 56% were women; 63% had total cavopulmonary connection; 66% had New York Heart Association (NYHA) class I status; 42% had arrhythmias; 22% had systemic right ventricle; and 35% had ventricular dysfunction. Of 81 patients with liver-imaging data, 41% had LD (i.e. imaging evidence of cirrhosis, with or without portal hypertension, splenomegaly or varices). There were no differences in clinical or echocardiographic variables between those with and without LD. Among the 58 patients with CPET data, mean peak oxygen consumption (VO2) was 18.6 ± 5.7 mL/kg/min, per-cent-predicted peak VO2 was 53.9 ± 15.5%, peak oxygen pulse was 9.3 ± 2.9 mL/beat and per-cent-predicted peak oxygen pulse was 82.6 ± 21.5%. Of the 44 patients with liver and CPET data, each standard deviation decrease in per-cent-predicted peak VO2 (16%) and per-cent-predicted peak oxygen pulse (22%) was associated with a 2.3-fold increase in the odds of LD, after adjusting for NYHA, institution and Fontan type (P = 0.04). Similarly, each standard deviation decrease in per-cent-predicted peak VO2 and oxygen pulse was associated with an estimated 5.9-year and 4.9-year earlier onset of LD, respectively (P > 0.05). Conclusions Decline in per-cent-predicted peak VO2 and oxygen pulse was associated with increased odds of LD in adults who had undergone the Fontan operation. Our study supports more rapid hepatic evaluation among patients with abnormal or worsening CPET variables.

Background Computed tomography angiography (CTA) is the test of choice for pre-procedure imaging of transcatheter aortic valve replacement (TAVR) candidates. The iodinated contrast required, however, increases the risk of renal dysfunction in patients with pre-existing renal failure. Ferumoxytol is a magnetic resonance imaging (MRI) contrast agent that can be used with renal failure. Its long vascular resonance time allows gated MRA sequences that approach CTA in image quality. We present respiratory and cardiac gated MRA enabled by ferumoxytol that can be post-processed in an analogous fashion to CTA. Methods Seven patients with renal failure presenting for TAVR were imaged with respiratory and cardiac gated MRA at 3T using ferumoxtyol for contrast. Aortic annulus, root and peripheral access dimensions were calculated in a fashion identical to that used for CTA. Of these, 6 patients underwent a TAVR procedure and 5 had intraoperative valve assessment with transesophageal echocardiograph (TEE) using standard clinical protocols that employed both two- and three-dimensional techniques. Results Good correlation between MRA aortic annulus measurements and those from TEE were shown in 5 patients with mean annulus area of 392.4mm 2 (290–470 range) versus 374.1mm 2 (285–440 range), with a pairwise correlation coefficient of 0.92, p=0.029. All patients received Sapien valve implants (one 20mm, three 23mm, and two 26mm valves). Access decisions were guided by MRA with no complications. Annulus sizing resulted in no greater than trace/mild aortic regurgitation in all patients. Conclusions Ferumoxytol MRA is a safe alternative to CTA in patients with renal failure for pre-TAVR analysis of the aortic root and peripheral access.

Transcatheter heart valve implantation is a well-established alternative to surgical valve replacement for specific types of valvular heart disease [(1–4)][1]. We report the first case of implantation of an Edwards SAPIEN XT transcatheter heart valve (Edwards Lifesciences, Irvine, California) in

A 77-year-old man with long-standing persistent atrial fibrillation, coronary artery disease, and systolic heart failure underwent left atrial appendage (LAA) ligation with a Lariat suture delivery device (SentreHEART, Redwood City, California), and subsequent pulmonary vein isolation. One month