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We present a novel method for the treatment of right-sided bioprosthetic valve endocarditis with AngioVac debulking followed by percutaneous pulmonic and tricuspid valve replacement for residual transcatheter valve degeneration and right ventricular dysfunction.

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.

Structured Abstract Background While percutaneous closure is preferred in adults with a patent ductus arteriosus (PDA), the extent of cardiac remodeling following percutaneous PDA closure in adults is not well understood. We performed a meta-analysis to characterize 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 (24 h), and at follow-up (>1 month) in adults undergoing percutaneous PDA closure. Additionally, we included data from patients >18 years of age that underwent percutaneous PDA closure between January 2015–December 2019 at our centre. Weighted averages were calculated for parameters with sufficient data. Pooled differences were presented as weighted mean differences (WMD). Results After screening 278 abstracts, 5 studies were identified. When combined with our cohort, our meta-analysis encompassed 244 patients. Immediately post-procedure, LVEF was the only parameter to change significantly (64 vs 52%, WMD -8.64 95%, CI -14.04 to −3.25). All parameters significantly decreased from baseline to follow-up: LVEF: 64 vs 60% (WMD -3.95, CI -5.80 to −2.09), LVEDD: 62 vs 53 mm (WMD -8.79, CI -12.78 to −4.81) LVEDVI 172 vs 123 mL/m2 (WMD -26.60, CI -45.4 to −7.85), LVESD 42 vs 36 mm (WMD -4.64, CI -9.10 to −0.17) and LA dimension: 42 vs 37 mm (WMD -5.87, CI -9.85 to −1.90). Conclusions As compared to the baseline values, left ventricular and left atrial sizes decreased. The LVEF decreased immediately following PDA closure and, while it recovers, it remained lower on follow-up testing.

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.

OBJECTIVEDuring the COVID-19 pandemic, quaternary-care facilities continue to provide care for patients in need of urgent and emergent invasive procedures. Perioperative protocols are needed to streamline care for these patients notwithstanding capacity and resource constraints.METHODSA multidisciplinary panel was assembled at the University of California, San Francisco, with 26 leaders across 10 academic departments, including 7 department chairpersons, the chief medical officer, the chief operating officer, infection control officers, nursing leaders, and resident house staff champions. An epidemiologist, an ethicist, and a statistician were also consulted. A modified two-round, blinded Delphi method based on 18 agree/disagree statements was used to build consensus. Significant disagreement for each statement was tested using a one-sided exact binomial test against an expected outcome of 95% consensus using a significance threshold of p < 0.05. Final triage protocols were developed with unblinded group-level discussion.RESULTSOverall, 15 of 18 statements achieved consensus in the first round of the Delphi method; the 3 statements with significant disagreement (p < 0.01) were modified and iteratively resubmitted to the expert panel to achieve consensus. Consensus-based protocols were developed using unblinded multidisciplinary panel discussions. The final algorithms 1) quantified outbreak level, 2) triaged patients based on acuity, 3) provided a checklist for urgent/emergent invasive procedures, and 4) created a novel scoring system for the allocation of personal protective equipment. In particular, the authors modified the American College of Surgeons three-tiered triage system to incorporate more urgent cases, as are often encountered in neurosurgery and spine surgery.CONCLUSIONSUrgent and emergent invasive procedures need to be performed during the COVID-19 pandemic. The consensus-based protocols in this study may assist healthcare providers to optimize perioperative care during the pandemic.

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.

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).

This study aimed to develop a cardiorespiratory-resolved 3D magnetic resonance imaging (5D MRI: x-y-z-cardiac-respiratory) approach based on 3D motion tracking for investigating the influence of respiration on cardiac ventricular function. A highly-accelerated 2.5-minute sparse MR protocol was developed for a continuous acquisition of cardiac images through multiple cardiac and respiratory cycles. The heart displacement along respiration was extracted using a 3D image deformation algorithm, and this information was used to cluster the acquired data into multiple respiratory phases. The proposed approach was tested in 15 healthy volunteers (7 females). Cardiac function parameters, including the end-systolic volume (ESV), end-diastolic volume (EDV), stroke volume (SV), and ejection fraction (EF), were measured for the left and right ventricle in both end-expiration and end-inspiration. Although with the proposed 5D cardiac MRI, there were no significant differences (p > 0.05, t-test) between end-expiration and end-inspiration measurements of the cardiac function in volunteers, incremental respiratory motion parameters that were derived from 3D motion tracking, such as the depth, expiration and inspiration distribution, correlated (p

Here we present a novel repair strategy of a rare aortic complication following transcatheter aortic valve repair (TAVR). A 73-year-old man with a history of mitral valve replacement secondary to endocarditis underwent a TAVR for severe aortic insufficiency at an outside hospital (Medtronic Evolut R

© 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