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Functional tricuspid regurgitation (TR) is a common pathophysiologic condition in adults with ostium secundum atrial septal defect (ASD). The aim of this study was to evaluate long-term outcomes following transcatheter ASD closure, which have not been well studied among patients with significant TR.We reviewed consecutive adult patients who underwent transcatheter ASD closure at Toronto General Hospital, Ontario, Canada, from 1998 to 2016. We linked our hospital registry with Ontario population-based health administrative databases to collect longitudinal data on inpatient and outpatient health care utilisation and vital status.In this cohort study of 949 patients, 199 (22%) had moderate to severe TR before transcatheter ASD closure. A significant proportion of patients (61%) showed improvement in TR severity to at least mild TR after ASD intervention. At a median follow-up of 10.9 years, patients with baseline mild or no TR, compared with those with greater than moderate TR, had significantly lower rates of all-cause mortality (6.8 vs 22.5 per 1000 person-years [PY]; P0.001), composite hospitalisation for atrial fibrillation (AF) or heart failure (HF) (22.3 vs 49.1 per 1000 PY; P0.001), and new onset of AF (10.4 vs 20.2 per 1000 PY; P = 0.002) and HF (5.0 vs 9.2 per 1000 PY; P = 0.039). Preprocedural TR was independently associated with higher all-cause mortality (adjusted hazard ratio 1.69, 95% confidence interval 1.08-2.62).TR severity was independently associated with a higher risk of mortality and morbidity. Further investigation of earlier device closure or concomitant tricuspid valve intervention may be of interest.

Background: Sarcopenia, the age-related loss of skeletal muscle mass/function, has been identified as a marker of frailty. We examined the association between sarcopenia and adverse events following transcatheter aortic valve implantation (TAVI). Methods: A retrospective cohort study was conducted at Toronto General Hospital. All patients who underwent TAVI in the time period 2007-2017 with preoperative computed tomography were included. Skeletal muscle index (SMI) was calculated radiographically using psoas muscle area at the L3 vertebral level, divided by height. Various measures of sarcopenia, including mean SMI, SMI below the sex-specific median, and SMI in the lowest sex-specific quartile were calculated. The primary outcome was postoperative adverse events, defined as a composite of in-hospital mortality and morbidity including cardiovascular, pulmonary, neurologic, access-related, and gastrointestinal complications. Univariate and multivariate logistic regression were performed to determine the association between sarcopenia and adverse events. Results: A total of 468 patients (mean age: 80.7 years) were included. Baseline comorbidity burden was high, particularly congestive heart failure (93.4%). Postoperative adverse events occurred in 62 patients (13.2%). Univariate logistic regression demonstrated that postoperative adverse events were correlated with mean SMI (odds ratio [OR] 0.81, 95% confidence interal [CI] 0.66-0.97), events were less than the SMI (OR 2.16, 95% CI 1.24-3.84), and SMI in the sex-specific lowest quartile (OR 2.34, 95% CI 1.33-4.07). On multivariate analysis, SMI in the sex-specific lowest quartile was an independent predictor of adverse events (OR 2.53, 95% CI 1.41-4.50). Conclusions: Sarcopenia defined by radiologic psoas muscle measurements was independently associated with in-hospital mortality and morbidity following TAVI. Résumé: Contexte: La sarcopénie, soit la perte de masse et de fonction des muscles squelettiques liée à l’âge, a été identifiée comme un marqueur de fragilité. Nous avons examiné l’association entre la sarcopénie et les événements indésirables suivant l’implantation valvulaire aortique par cathéter (IVAC). Méthodologie: Une étude de cohorte rétrospective a été menée au Toronto General Hospital. Tous les patients ayant subi une IVAC avec tomodensitométrie préopératoire au cours de la période 2007-2017 ont été inclus. L’indice de masse musculaire squelettique (IMMS) a été calculé par radiographie en utilisant la surface du psoas au niveau de la vertèbre L3, divisée par la taille. Diverses mesures de la sarcopénie, y compris l’IMMS moyen, l’IMMS sous la médiane selon le sexe et l’IMMS dans le quartile inférieur selon le sexe, ont été calculées. Le critère d’évaluation principal était les événements indésirables postopératoires, définis comme un critère composite comprenant la mortalité et la morbidité à l’hôpital, notamment les complications cardiovasculaires, pulmonaires, neurologiques, gastro-intestinales et liées à l’accès vasculaire. Des régressions logistiques univariée et multivariée ont été effectuées pour déterminer l’association entre la sarcopénie et les événements indésirables. Résultats: Un total de 468 patients (âge moyen : 80,7 ans) ont été inclus. Le fardeau de comorbidité au départ était élevé, en particulier pour ce qui est de l’insuffisance cardiaque congestive (93,4 %). Des événements indésirables postopératoires sont survenus chez 62 patients (13,2 %). La régression logistique univariée a montré que les événements indésirables postopératoires étaient en corrélation avec un IMMS moyen (rapport des cotes [RC] : 0,81, intervalle de confiance [IC] à 95 % : 0,66 à 0,97), un IMMS sous la médiane selon le sexe (RC : 2,16; IC à 95 % : 1,24 à 3,84) et un IMMS dans le quartile inférieur selon le sexe (RC : 2,34; IC à 95 % : 1,33 à 4,07). Lors de l’analyse multivariée, un IMMS situé dans le quartile inférieur selon le sexe était un prédicteur indépendant d’événements indésirables (RC : 2,53; IC à 95 % : 1,41 à 4,50). Conclusions: La sarcopénie définie par les mesures radiologiques du psoas était indépendamment associée à la mortalité et à la morbidité à l’hôpital à la suite d’une IVAC.

The objective of this study was to determine the effect of transcatheter mitral valve repair (TMVr) on hospitalization rates by assessing pre- and postprocedural hospitalization patterns.TMVr has emerged as the treatment of choice for selected patients with mitral regurgitation, but the impact of these procedures on hospital utilization remains unclear.All patients who underwent TMVr in Ontario, Canada, between 2011 and 2017 were included in this observational study using population-based data. Hospitalization person-year rates were assessed in the years before and after TMVr and 4 predefined intervals: 1 to 30, 31 to 90, 91 to 182, and 183 to 365 days. Main outcomes of interest were all-cause and heart failure (HF) hospitalizations. Poisson regression models were used to compare incidence rates across all time periods.The study cohort included 523 patients. In the year preceding TMVr, 66.2% of patients were hospitalized compared with 47.4% in the year following. There were stepwise increases in both all-cause and HF hospitalization rates in the periods preceding the index procedure, and all postprocedural periods had significantly lower hospitalization rates. The adjusted rate ratios for all-cause and HF-related hospitalization in the year after TMVr were 0.65 (95% CI: 0.56-0.76) and 0.38 (95% CI: 0.29-0.51), respectively. All time periods had significant reductions in all-cause and HF hospitalization in the adjusted analysis.In this population-based study, significant reductions were observed in both all-cause and HF-related hospitalizations in all time periods after TMVr compared with the year prior. This suggests that TMVr has a sustained effect on hospitalization rates despite a high-risk population.

Background Aortic stenosis (AS) is a common cardiac condition whose prevalence increases with age. The symptom burden associated with severe aortic stenosis (AS) can introduce significant lifestyle disruptions and if left untreated can lead to a poor prognosis. Quality of life (QoL) is an important consideration in these patients. The TASQ is a QoL tool that was developed for aortic stenosis patients. We evaluated the psychometric properties of this specific questionnaire in patients who underwent transcatheter aortic valve implantation (TAVI), which is a therapeutic option for patients with severe aortic stenosis (AS). Methods The properties of the TASQ in measuring QoL were evaluated in AS patients undergoing TAVI. Patients presenting for the TAVI procedure (N = 62) were evaluated pre-TAVI, at discharge, 1-month, and 3-month follow-ups. Demographic information as well as caregiver status, and daily activities were recorded. In addition to the TASQ, they completed the KCCQ (Kansas City Cardiomyopathy Questionnaire) and the IIRS (Illness Intrusiveness Rating Scale). Results The TASQ is a 16-item self-administered questionnaire that assesses AS-specific QoL across five domains: physical symptoms; physical limitations; emotional impact; social limitations, and health expectations. TASQ subscales are internally consistent (α = 0.74–0.96) and showed significant improvements from baseline across assessments (p Conclusions The TASQ is a brief measure of AS-specific QoL that is sensitive to change in patients undergoing TAVI. Items on the TASQ capture important QoL concerns reported by AS patients, suggesting this is a measure of relevant and meaningful outcomes for this patient population. Detection of early improvements in QoL by the TASQ is promising, with important implications for the evaluation of procedural outcomes in this population.

Background: Pulmonary hypertension (PH), recently redefined as mean pulmonary arterial pressure >20 mm Hg (PH 20 ), may be observed in patients with atrial septal defects (ASD). We aimed to determine the effect of preprocedural PH 20 status on outcomes among patients undergoing ASD closure. Methods: Study population was selected from a retrospective registry of adult patients who underwent percutaneous ASD closure from 1998 to 2016 from a single center and had right heart catheterizations during the procedure. The clinical registry was linked to administrative databases to capture short- and long-term outcomes. Results: We included a total of 632 ASD closure patients of whom 359 (56.8%) had PH 20 . The mean follow-up length was 7.6±4.6 years. Patients with PH 20 were older (mean age 56.5 versus 43.1 years, P P P 25 ) cutoff, a significantly higher hazard of developing major adverse cardiac and cerebrovascular events was observed in PH versus non-PH patients. Conclusions: ASD patients with PH undergoing closure suffer from more comorbidities and worse long-term major adverse cardiac and cerebrovascular events outcomes, compared with patients without PH. The use of the new PH 20 definition potentially dilutes the effect of this serious condition on outcomes in this population.

A patent foramen ovale (PFO) is found in nearly half of patients with cryptogenic stroke. Little guidance on the use or necessity of coronary angiography at the time of percutaneous PFO closure exists. We aimed to characterize the presence of coronary artery disease (CAD) in patients undergoing PFO closure following a cryptogenic stroke.A retrospective analysis of consecutive patients who underwent concurrent percutaneous PFO closure and coronary angiography was performed. Patients were ≥40 years of age and had a preceding diagnosis of cryptogenic stroke with no known CAD. Visual analysis of coronary angiograms was performed.Of 180 patients, 8 (4%) had severe CAD, 15 (8%) had moderate CAD, 32 (18%) had mild CAD, and 12 (7%) had luminal irregularities. Of those with moderate-to-severe CAD, 9 (5%) had proximal disease and 9 (5%) had multivessel disease. Of those with moderate-to-severe CAD, 8 (35%) underwent further risk stratification with stress testing, 4 (17%) were medically managed, and 1 (4%) underwent concurrent angioplasty.Coronary angiography identified a low prevalence of CAD in patients with cryptogenic stroke undergoing PFO closure, suggesting that coronary angiography is not routinely indicated in patients undergoing PFO closure.

BackgroundComplications and need for reinterventions are frequent in patients with pulmonary valve stenosis (PVS). Pulmonary regurgitation is common, but no data are available on outcome after pulmonary valve replacement (PVR).MethodsWe performed a retrospective analysis of 215 patients with PVS who underwent surgical valvotomy or balloon valvuloplasty. Incidence and predictors of reinterventions and complications were identified. Right ventricle (RV) remodelling after PVR was also assessed.ResultsAfter a median follow-up of 38.6 (30.9–49.4) years, 93% of the patients were asymptomatic. Thirty-nine patients (18%) had at least one PVR. Associated right ventricular outflow tract (RVOT) intervention and the presence of an associated defect were independent predictors of reintervention (OR: 4.1 (95% CI 1.5 to 10.8) and OR: 3.6 (95% CI 1.9 to 6.9), respectively). Cardiovascular death occurred in 2 patients, and 29 patients (14%) had supraventricular arrhythmia. Older age at the time of first intervention and the presence of an associated defect were independent predictors of complications (OR: 1.0 (95% CI 1.0 to 1.1) and OR: 2.1 (95% CI 1.1 to 4.2), respectively). In 16 patients, cardiac magnetic resonance before and after PVR was available. The optimal cut-off values for RV volume normalisation were 193 mL/m2 for RV end-diastolic volume indexed(sensitivity 80%, specificity 64%) and 100 mL/m2 for RV end-systolic volume indexed(sensitivity 80%, specificity 56%).ConclusionsPrevious RVOT intervention, presence of an associated defect and older age at the time of first repair were predictors of outcome. More data are needed to guide timing of PVR, and extrapolation of tetralogy of Fallot guidelines to this population is unlikely to be appropriate.

Objective(s) This study was designed to compare 2 different perioperative analgesia strategies with respect to the incidence of postoperative delirium after a transapical approach for transcatheter aortic valve replacement (TAVR). The authors hypothesized that perioperative thoracic paravertebral analgesia with a local anesthetic would decrease opioid consumption and in turn reduce the incidence of postoperative delirium when compared with systemic opioid-based analgesia after a transapical TAVR procedure. Design Prospective, randomized controlled clinical trial. Setting Tertiary referral center, university hospital. Participants The study comprised 44 patients undergoing a transapical TAVR procedure. Patients with a history of serious mental illness, delirium, and severe dementia and/or patients with contraindications to regional anesthesia were excluded. Interventions Patients were randomly assigned to either the paravertebral group (perioperative continuous thoracic paravertebral block with local anesthetic) or the patient-controlled analgesia group (systemically administered opioids) using a computer-generated randomization code in blocks of four patients. Measurements and Main Results Assessment of postoperative delirium was performed by trained research staff using the confusion assessment method for intensive care unit preoperatively and postoperatively every 12 hours or more often if needed according to the patient's condition during the first 7 postoperative days or until discharge. Pain was assessed with a 10 cm Visual Analog Scale pain score system during the 48 hours postoperatively. The sedation level was assessed using the Sedation Agitation Scale during the same period. Overall postoperative delirium was detected in 12/44 (27%) patients, with 7/22 (32%) in the patient-controlled analgesia and 5/22 (23%) in the paravertebral groups, respectively (p = 0.73). Both groups were similar with respect to demographic data, preoperative medications, and comorbidities. Paravertebral analgesia was associated with an opioid-sparing effect during surgery and during the 48-hour postoperative period. Sedation and pain scores were similar between the 2 groups. In addition, paravertebral analgesia was associated with earlier extubation times; however, the overall morbidity and mortality were similar between the 2 groups. Conclusions Paravertebral analgesia in patients undergoing transapical TAVR procedures appears to have an opioid-sparing effect. However, it did not translate into a statistically significant decrease in the rate of postoperative delirium.

Objectives The authors sought to prospectively determine the safety and efficacy of next-day discharge using the Vancouver 3M (Multidisciplinary, Multimodality, but Minimalist) Clinical Pathway. Background Transfemoral transcatheter aortic valve replacement (TAVR) is an alternative to surgery in high- and intermediate-risk patients; however, hospital stays average at least 6 days in most trials. The Vancouver 3M Clinical Pathway is focused on next-day discharge, made possible by the use of objective screening criteria as well as streamlined peri- and post-procedural management guidelines. Methods Patients were enrolled from 6 low-volume ( 200 TAVR/year) centers in Canada and the United States. The primary outcomes were a composite of all-cause death or stroke by 30 days and the proportion of patients successfully discharged home the day following TAVR. Results Of 1,400 screened patients, 411 were enrolled at 13 centers and received a SAPIEN XT (58.2%) or SAPIEN 3 (41.8%) valve (Edwards Lifesciences, Irvine, California). In centers enrolling exclusively in the study, 55% of screened patients were enrolled. The median age was 84 years (interquartile range: 78 to 87 years) with a median STS score of 4.9% (interquartile range: 3.3% to 6.8%). Next-day discharge home was achieved in 80.1% of patients, and within 48 h in 89.5%. The composite of all-cause mortality or stroke by 30 days occurred in 2.9% (95% confidence interval: 1.7% to 5.1%), with neither component of the primary outcome affected by hospital TAVR volume (p = 0.51). Secondary outcomes at 30 days included major vascular complication 2.4% (n = 10), readmission 9.2% (n = 36), cardiac readmission 5.7% (n = 22), new permanent pacemaker 5.7% (n = 23), and >mild paravalvular regurgitation 3.8% (n = 15). Conclusions Adherence to the Vancouver 3M Clinical Pathway at low-, medium-, and high-volume TAVR centers allows next-day discharge home with excellent safety and efficacy outcomes.

IMPORTANCE: Adding clopidogrel to aspirin for 3 months after transcatheter atrial septal defect (ASD) closure results in a lower incidence of new-onset migraine attacks. However, the outcomes at 6- to 12-month follow-up (after clopidogrel cessation at 3 months) remain largely unknown. OBJECTIVE: To assess the incidence of migraine attacks at 6- and 12-month follow-up after transcatheter ASD closure. DESIGN, SETTING, AND PARTICIPANTS: This prespecified analysis of a randomized, double-blind clinical trial included patients with no prior history of migraine undergoing ASD closure from 6 university hospitals in Canada from December 2008 to November 2014. Patients were followed up at 3, 6, and 12 months, and a migraine headache questionnaire was administered at each time. Analysis began June 2019. INTERVENTIONS: Patients were randomized (1:1) to receive dual antiplatelet therapy (aspirin plus clopidogrel; n = 84) vs single antiplatelet therapy (aspirin plus placebo; n = 87) for 3 months following transcatheter ASD closure. After 3 months, only single antiplatelet therapy (aspirin) was pursued. MAIN OUTCOMES AND MEASURES: Incidence and severity of migraine attacks at 6- and 12-month follow-up. RESULTS: The mean (SD) age of the study population was 38 (12) years, with 106 women (62%). A total of 27 patients (15.8%) had new-onset migraine attacks within the 3 months following ASD closure (8 of 84 [9.5%] vs 19 of 87 [21.8%] in the initial clopidogrel and placebo groups, respectively; P = .03). After cessation of clopidogrel and aspirin monotherapy, the percentage of patients with migraine attacks decreased over time, with 8 (4.7%) and 4 patients (2.3%) continuing to have migraine attacks at 6 and 12 months, respectively (vs 3 months: P

The long-term outcomes after transcatheter closure of atrial septal defects (ASD) in adults are reported and compared between age groups and against population control patients.ASD is the second most common lesion in congenital heart disease. Comprehensive data on long-term outcomes after ASD closure are limited.This retrospective cohort study enrolled adult patients with secundum ASD closure between 1998 and 2016. Information from a detailed clinical registry was linked to population-based administrative databases to capture outcomes. The population control cohort was matched using important prognostic characteristics.The cohort included 1,390 ASD patients of whom 32% were 40 years of age, 45% were 40 to 60 years of age, and 23% were60 years of age at closure. The median follow-up was 10.6 years (interquartile range: 6.2 to 14.0 years). New-onset atrial fibrillation (AF) was the most frequent outcome overall (14.9%). The incidence of adverse cardiac and cerebrovascular events was higher in the60 years of age group than in the younger groups. In adjusted analysis, patients60 years of age continued exhibiting higher risk of all-cause (hazard ratio [HR]: 8.54; 95% confidence interval [CI]: 93.40 to 21.43) and cardiovascular (CV)-specific mortality compared with the 40 years of age group. The risk of new-onset AF (HR: 3.73; 95% CI: 2.79 to 4.98) and any AF hospitalization (HR: 1.55; 95% CI: 1.28 to 1.89) was higher in the ASD than in the control population, whereas there was no difference in all-cause and CV-specific mortality.As expected, rates of adverse events post-ASD closure are higher in older age groups, but long-term mortality was comparable to that of a population control cohort. The high rates of AF necessitate future investigations.

Transcatheter atrial septal defect closure (TAC) is a well-established therapy for most patients with secundum atrial septal defects (ASD) based on short-term data ([1][1]). In previous surgical studies, mortality was not significantly different from age-and-sex matched control subjects ([2][2]).

Background Twenty percent of patients born with congenital heart disease present with right ventricular outflow tract abnormalities. These patients require multiple surgical procedures in their lifetime. Transcatheter pulmonary valve replacement (TPVR) has become a viable alternative to conventional pulmonary valve and right ventricular outflow tract surgery in pediatric and adult populations. In this retrospective review, we analyze the perioperative management of adult patients who underwent TPVR in our center. Methods The study consisted of a chart review of patients who underwent TPVR at Toronto General Hospital between 2006 and 2015. Information about preoperative assessment, intraoperative anesthetic management, and intra- and postprocedural complications was collected. Two types of percutaneous valves have been used for a conduit or valve size between 16 and 28 mm. These procedures are done via the femoral, jugular, or subclavian vein under general anesthesia. Results Seventy-nine adults (17-68 years of age) who underwent elective TPVR procedures were included. General anesthesia was used in all cases. Defibrillation was necessary in 1 case, and bradycardia was spontaneously resolved in another 1. Eighty-five percent were successfully extubated at the end of the procedure. Five patients required intraoperative inotropic support. Three patients presented self-resolved hemoptysis. Mechanical ventilation for >24 hours was necessary in 3 cases, 2 of which also required concomitant inotropic support. Four failed deployments and 1 case of persistent conduit stenosis were reported. Three patients required reintubation. All patients were discharged home. Conclusions Patients undergoing TPVR represent a complex and heterogeneous population. General anesthesia with endotracheal intubation is preferred. Setup for urgent lung isolation and cardiac defibrillation should be considered. Postoperative monitoring and intensive care setting are required. Anesthesiologists with cardiac anesthesia training are probably better suited to manage these patients.

Objective Although heart failure (HF) is the leading cause of premature death in adult congenital heart disease (ACHD), little population-specific data exist. This study reports early experience from a dedicated, sub-specialty adult congenital heart disease-heart failure (ACHD-HF) clinic, aiming to identify risk factors for adverse outcome. Methods Between 2012 and 2015, 126 patients (57% male) attended the ACHD-HF clinic. Baseline and follow-up data were analysed and compared across 4 anatomical/physiological subgroups: cyanotic ACHD, Fontan circulation (1V), biventricular circulation with a subaortic right ventricle (2V-RV) and biventricular circulation with a subaortic left ventricle (2V-LV). Predictors of the composite primary outcome: death, transplant or ventricular assist device (VAD) were identified using multivariable Cox proportional hazard models. Results Mean age at first visit was 38±13years. Patients were grouped as follows: cyanotic ACHD 10%, 1V 24%, 2V-RV 29% and 2V-LV 37%. During a median follow-up of 1.7 (IQR 0.8–2.9) years, 38 patients (30%) reached the primary outcome. Event-free survival was 89%, 78% and 63% at 1, 2 and 3years. Forty (31.7%) patients experienced 69 HF hospitalisations. Between-group differences were noted for systolic function, valvular regurgitation, pacing prevalence and invasive hemodynamics. Multivariable analysis revealed 2V-RV subgroup ( p =0.001), NYHA class ( p =0.002) B-type natriuretic peptide >164pg/ml ( p =0.003) and sodium p =0.036) as independently associated with death, transplant or VAD. Conclusions Our young ACHD-HF patients experienced high adverse event rates during a short period of follow-up. The prognostic markers identified will aid clinicians to stratify short-term risk and thereby guide advanced HF management decisions in ACHD.

Background Anomalous origin of a coronary artery from the pulmonary artery (PA) is a rare condition that usually presents in childhood. The aim of this study is to describe its characteristics in unrepaired adults. Methods We conducted a retrospective study of 18 patients with left and right anomalous coronary artery from the PA (ALCAPA and ARCAPA) diagnosed in adulthood. Patients ≥18 years old at diagnosis and assessed at least once in our institution were included. Results Median age at diagnosis was 29.5 years [IQR 24.0–48.8]. Presentation mode varied, with two patients (11%) diagnosed following aborted sudden cardiac death. Diagnosis was most frequently made by coronary angiography (44%) and cardiac computed tomography (28%). The mean left ventricle ejection fraction (LVEF) at presentation was 56 ±10% and moderate or severe mitral regurgitation was present in 5 patients. Surgical or interventional repair was performed in 14 patients (78%), 9 of whom (50%) underwent reimplantation. Median follow-up was 8.5 years [IQR 0.98–13.8]. One patient with severe pulmonary hypertension died during the follow-up. Most patients (79%) were in NYHA functional class 1 at last follow-up with a mean LVEF of 53±12%. Conclusions Anomalous origin of a coronary artery from the PA can present in adulthood in various ways. Multimodality imaging allows the diagnosis and assessment of functional impact. Surgical correction of ALCAPA with establishment of a dual coronary pathway was the most frequent management strategy in our cohort. The majority of patients experienced favorable long-term outcomes.

This early feasibility study was designed to obtain in vivo data to confirm assumptions on device loading conditions of the Medtronic Harmony transcatheter pulmonary valve (TPV). Secondary objectives included procedural feasibility, safety, and valve performance. The Harmony TPV was developed for nonsurgical pulmonary valve replacement in non-right ventricle-pulmonary artery conduit patients. The Native Outflow Tract TPV Research Clinical Study was the first study approved under the Food and Drug Administration Early Feasibility Study guidance. Enrollment required that patient anatomy be precisely matched to the single-size Harmony TPV implant, necessitating a rigorous selection process. The study was nonrandomized, prospective, and performed at 3 sites. All patients met standard indications for surgical pulmonary valve replacement. The goal of the screening committee was to match the candidate anatomy to predetermined engineering criteria thought to be predictive of secure Harmony TPV implantation for the single-size device under study. A majority of the screening committee was required to recommend a patient as eligible for implant. A total of 270 patients underwent prescreening cardiac magnetic resonance imaging, 66 were enrolled and received a computed tomography scan (24%), 21 met criteria for implant and were catheterized (8%), and 20 underwent implant. Nineteen of 20 met criteria for implant success. In conclusion, the Medtronic Harmony TPV represents an emerging therapeutic option for patients with complex postoperative right ventricular outflow tract failure. The initial clinical evaluation of this technology was unique, and the highly variable anatomy of this population required careful screening to ensure acceptable device fit.

Objectives This study sought to obtain in vivo data to confirm assumptions on device loading conditions and assess procedural feasibility, safety, and valve performance. Background The Harmony transcatheter pulmonary valve (Medtronic, Minneapolis, Minnesota) was designed for patients with severe pulmonary regurgitation who require pulmonary valve replacement. Methods Three sites participated in this first Food and Drug Administration–approved early feasibility study using an innovative device design to accommodate the complex anatomy of the right ventricular outflow tract. Potentially eligible patients underwent review by a screening committee to determine implant eligibility. Six-month outcomes are reported. Results Between May 2013 and May 2015, 66 subjects were enrolled, and 21 were approved for implant and underwent catheterization; 20 were implanted. Catheterized patients had a median age of 25 years, were predominantly diagnosed with tetralogy of Fallot (95%), had severe pulmonary regurgitation (95%), and had trivial or mild stenosis. The device was delivered in the desired location in 19 of 20 (95%) patients. Proximal migration occurred in 1 patient during delivery system removal. Two devices were surgically explanted. Premature ventricular contractions related to the procedure were reported in 3 patients; 2 were resolved without treatment. One patient had ventricular arrhythmias that required treatment and later were resolved. At 1 month, echocardiography revealed none or trivial pulmonary regurgitation in all and a mean right ventricular outflow tract gradient of 16 ± 8 mm Hg (range 6 to 31 mm Hg). Conclusions In this feasibility study of the Harmony transcatheter pulmonary valve device, there was high procedural success and safety, and favorable acute device performance.

Graphical abstract, Highlights • Multimodality imaging identifies cor triatriatum sinister and its consequences. • Echocardiography is useful in defining a cor triatriatum sinister membrane. • Cor triatriatum sinister can have a variable clinical presentation.

Background Left atrial (LA) size is a marker of prognosis in severe aortic stenosis (AS). The aims of this retrospective study were to assess the impact of transcatheter aortic valve implantation (TAVI) on the recovery of LA phasic function and to assess the relationship between LA function and new-onset atrial fibrillation (NAF) after TAVI. Methods In this retrospective cohort study, LA function was measured using biplane volumes and 2-dimensional (2D) speckle tracking echocardiography (STE) in 52 patients (median age, 81 years) with severe AS before TAVI and at midterm follow-up. Twenty healthy individuals ≥ 75 years were used as controls. Results Before TAVI, the 3 phasic volumetric emptying fractions and all STE-derived parameters of LA function were significantly reduced. At 5 ± 3 months after TAVI, there was an improvement in LA reservoir and contractile function. However, LA phasic volumes and emptying fractions showed minimal changes. Fourteen patients had NAF in the early postprocedural period after TAVI. These patients experienced longer hospitalization (11 days vs 6 days; P = 0.002). By bivariable logistic regression analysis, the use of a transapical approach and the LA early diastolic strain rate (SR) before TAVI were significantly associated with NAF immediately after TAVI. Conclusions Severe AS is associated with LA dysfunction. Intrinsic LA compliance and LA contractile properties by STE improved at midterm follow-up after TAVI. Preprocedural LA early diastolic SR may predict the development of NAF after TAVI pending confirmation by larger prospective evaluations.

Objectives To report the initial clinical experience with a novel atrial septal defect (ASD) closure device, the GORE® CARDIOFORM ASD Occluder (GCO). Background Transcatheter closure has become the treatment of choice for secundum ASD. A wide range of occluder devices are available, but concern has been raised about the risk of cardiac erosion associated with rigid devices and future access to the left atrium Methods Retrospective chart review of patients treated with the GCO at three Canadian centers. Primary outcomes were procedural success and residual shunting at follow-up, as well as 30-day major adverse events. Secondary outcomes included new onset atrial arrhythmias, wire frame fractures (WFF), and all cause mortality. Clinical, echocardiographic, procedural data, and follow-up outcome variables were collected in each participating hospital. Results Between February and December 2015, 26 patients (5 children and 21 adults) underwent transcatheter ASD closure with the GCO and were included in the study cohort. Procedural success was achieved in 22 of 26 patients (85%) and no major procedural complications were observed. Two patients (8%) presented new onset atrial tachyarrhythmia during early follow-up (0–30 days). Follow-up echocardiography (median of 119 days [IQR: 92–146]) demonstrated no residual shunt in all implanted patients. After a median clinical follow-up of 174 days (IQR: 135–239), one patient died of an unrelated cause, there were no documented major adverse cardiovascular events. Fluoroscopic imaging of the device was performed in 20 patients (91%), and WFF was noted in five cases. No clinical consequence or device dysfunction was observed in these patients. Conclusions In this first-in-man multicenter study, the GCO was safe and effective for ASD closure, with no major adverse events or residual shunt at follow-up. © 2017 Wiley Periodicals, Inc.

Background Recent randomized trials have confirmed the role of patent foramen ovale (PFO) closure in the secondary prevention of cryptogenic stroke. Guidelines have suggested a central role for intraprocedural imaging using intracardiac echocardiography (ICE). However, this modality may not be required to achieve safe and effective closure. We aimed to examine the periprocedural outcomes of PFO closure retrospectively, using fluoroscopic guidance in patients with cryptogenic stroke, with provisional ICE guidance driven by anatomic and procedural factors. Methods A retrospective chart review of consecutive patients who underwent PFO closure in a single centre using the Amplatzer PFO occluder (AGA Medical Corporation, Plymouth, Minnesota) for cryptogenic stroke was conducted. Outcomes analyzed included procedural data, periprocedural complications, length of stay, and factors contributing to the use of intraprocedural imaging. Results Between 2006 and 2017, 467 patients underwent PFO closure for cryptogenic stroke with the Amplatzer PFO occluder; 381 patients underwent closure with fluoroscopy alone, and 86 with ICE and fluoroscopic guidance. Periprocedural arrhythmic complications occurred in 1.3% in the fluoroscopy group and 1.2% in the ICE group (P = 1.000). Vascular complications occurred in 0.5% in the fluoroscopy group and 2.3% (P = 0.323) in the ICE group. One device embolized requiring surgical intervention. There was no in-hospital mortality or stroke. Same-day discharge occurred in 97.6% of patients. Conclusion Our single-centre experience suggests that PFO closure can be safely conducted under fluoroscopic guidance alone with provisional adjunctive ICE use limited to specific anatomic situations.

Background There is little published data regarding percutaneous patent ductus arteriosus (PDA) closure in adults. We aim to describe the outcomes of adult patients undergoing PDA closure at a single tertiary referral centre. Methods All adults who underwent device PDA closure at our centre from 2001 to 2017 were identified and enrolled in the study. Available clinical data and imaging data were reviewed. Results At total of 141 patients were identified, with a mean age of 43 ± 15 years. Left ventricular dilation was present in 27% and pulmonary hypertension in 36% of the patients. Most ducts (74%) were of type A morphology. Mean ductal diameter at the pulmonary artery end was 4.1 ± 1.9 mm and mean length was 10.0 ± 4.7 mm. Wire passage from the pulmonary artery was achieved in 79%. Procedural success rate was 100%, and an Amplatzer duct occluder was deployed in all successful cases (ADO1 device in 97%). There was a small residual shunt in 6% at the time of closure, and only 2 patients had a residual leak on echocardiography at a median 3 months’ follow-up. No major complications occurred. Conclusions Transcatheter PDA closure with the use of an Amplatzer duct occluder is very effective in adults across all duct morphologies and associated with a very low complication rate.

Coronary artery fistulae (CAF) are rare congenital cardiac abnormalities. With increasing age, patients with moderate to large fistulae are likely to become symptomatic and encounter complications. Percutaneous closure has been accepted as a safe and effective alternative to surgery; however, information regarding long-term outcomes in adult patients after transcatheter closure is limited. METHODS We describe our 20-year experience with percutaneous closure of CAF in adult patients, focusing on long-term outcomes. RESULTS From 1995 to 2015, 25 adult patients underwent 33 transcatheter interventions to close 26 CAF at our institution. Mean age was 51.4 ± 16.8 years, and 14 (56%) were female. All the patients were symptomatic with dyspnea (21/25, 84%) being the most common complaint followed by chest pain (17/25, 68%), palpitations (9/25, 36%), and heart failure (2/25, 8%). Two patients presented with acute coronary syndrome, whereas 10/25 (40%) also had inducible ischemia on stress test. These CAFs were classified as medium (12, 46.2%), large (5, 19.2%), and giant (9, 34.6%). Immediately postprocedure complete occlusion was achieved in 21 (21/26, 81%), and residual trace leak was observed in three fistulae, whereas we could not deliver coils/device in two cases. Periprocedural complications occurred in the form of coronary dissection (n = 1), myocardial infarction (n = 2), coil embolization (n = 1), transient ST elevation (n = 1); the majority of complications were observed in the early years of our experience. Patients reported marked improvement in symptoms after the intervention. During a mean follow-up of 76 ± 69 (5-214 months) (n = 22), 15 patients were investigated by either conventional (n = 6) or CT angiography (n = 8) or both (n = 1) that demonstrated thrombotic occlusion of a proximal coronary artery where a distal fistula was treated. Another patient was noted to have thrombus proximal to a vascular plug in a distal giant fistula. Three patients were lost to follow-up. None of the investigated patients had recanalization of their fistula. We did not observe remodeling of the native coronary artery that was feeding the fistula, even after successful complete closure. CONCLUSION Percutaneous device closure of CAF is a feasible and effective therapeutic intervention in adult patients; however, patients with distal fistulas represent a significant challenge and the outcomes of these procedures are in question. All patients should have long-term angiographic follow-up.

Background: Owing to the limited data on long-term outcomes following the implantation of atrial septal occluders, their permanent nature and potential for adverse outcomes remain concerning. Methods: In this retrospective cohort study, the long-term outcomes of adult patients who underwent transcatheter and surgical closure at Peter Munk Cardiac Centre, Toronto, Canada between 1991-2015 were determined using administrative databases linkages. The transcatheter group was compared to an age-sex matched population (each included 1278 participants) in 1:1 fashion and to a surgical group (240 patients) using propensity weighting. The primary endpoint was all-cause mortality. Results: Median age was 48 years and 39 years for the transcatheter and surgical groups respectively. Compared to age-sex matched group, mortality following transcatheter closure was not significantly different after a median follow-up of 9 years [7.9 vs 6.4 per 1000-patient-years, (difference-95%CI, -0.68,4.49), P=0.20], with non-significantly different rates of stroke [1.9 vs 1.1 per 1000-patient-years, (difference-95% CI, -0.2,2.69), P=0.16] but significantly higher rates of atrial fibrillation [22 vs 4.4 per 1000-patient-years, (difference-95% CI, 11.6,25.9), P

Background Percutaneous coronary intervention (PCI) procedures are increasingly performed on complex tortuous and heavily calcified coronary lesions. The GuideLiner® catheter (Vascular Solutions, Inc. Minneapolis, MN) is a rapid exchange guide catheter extension system. There is paucity of data about the efficacy of this device in complex PCI. Therefore, we aim to report the indications, success, and efficacy of GuideLiner use in treating complex lesion subset in the present study. Methods We collected data from all patients in whom the GuideLiner was used to facilitate PCI between April 1, 2011 and December 31, 2014 at a tertiary referral center in Toronto, Canada. Demographic and procedural data were obtained from an institutional prospective data registry. Indications for use of the GuideLiner, and angiographic and procedural data specific to the device were also collected. Results The GuideLiner was utilized in 307 (317 lesions) of 6105 PCI procedures performed at our institution during the study period. The mean age was 68.4 ± 11.2 years and 82% were male. The majority of lesions were types B2 or C (98%), calcified (86.3%), with proximal tortuosity (88.6%). The overall technical success rate of the GuideLiner was 88%. The procedural success rate was 98.7%. The complication rate was low (1.6%). Conclusions Use of the GuideLiner catheter facilitated successful completion of PCI procedures in a majority of patients with complex lesions. Operators should be aware of the potential complications associated with GuideLiner use.

Objectives This study reviewed a series of patients treated with transcatheter closure of septal defect to treat platypnea-orthodeoxia syndrome, with specific attention to septal characteristics and device choice. Background Platypnea–orthodeoxia syndrome is an uncommon condition characterized by positional dyspnea and hypoxemia due to intracardiac right-to-left shunting through a patent foramen ovale (PFO), an atrial septal defect, or pulmonary arteriovenous malformations. Percutaneous closure of such defects is the treatment of choice. Methods In this single-center series, 52 patients were treated with percutaneous closure of an interatrial communication after presentation between January 1997 and July 2015. Septal morphology, clinical, procedural, and outcomes data were analyzed. Results All patients had a PFO; however, nearly one-quarter required a non-PFO device (11 Amplatzer Septal Occluder and 1 post-infarct muscular VSD), as opposed to a dedicated PFO device to achieve shunt occlusion. These patients were characterized by an aneurysmal septum, shorter primum septum overlap with the secundum septum, and greater septal angulation from the midline. After closure, all demonstrated acute improvements in oxygen saturation (pre-procedure: 81 ± 8%; post-procedure: 95.1 ± 0.5% on room air). Each patient was treated with a single device and no one required re-intervention. Conclusions Patients presenting with platypnea–orthodeoxia syndrome can be treated successfully with a percutaneous intervention often requiring a variety of devices. Those requiring a non-PFO–type device had a greater prevalence of an aneurysmal septum, shorter primum septal overlap with the secundum septum, and greater septal angulation with the midline.

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 review the outcomes for the Sapien and Sapien XT valves (Edwards Lifesciences, Irvine, California) for percutaneous pulmonary valve implantation (PPVI). Background PPVI has emerged as a viable alternative to surgery in patients with right ventricular (RV) outflow tract dysfunction. Limited data are available for the Sapien and Sapien XT valves in this setting. Methods Retrospective analysis was performed for all patients to have undergone PPVI using the Edwards Sapien system at a large quaternary center. Results Twenty-five patients (70% male, mean age 34 ± 8.9 years) were identified. Primary underlying diagnosis was tetralogy of Fallot (n = 15), Ross procedure (n = 5), and other (n = 5). RV outflow tract characteristics included: biological valve (n = 16) and homograft (n = 9). Technical success was 96%. One patient required elective surgical pulmonary valve replacement for a high residual gradient. Pre-stenting was performed in all cases (52% covered stents). Valve sizes were 23 mm (n = 8), 26 mm (n = 15), and 29 mm (n = 2). Procedural hemodynamics revealed a decrease in the mean RV-to-systemic pressure ratio from 0.64 to 0.36 (p Conclusions The Edwards Sapien system is a viable and durable option for PPVI in this single-center study.

The prevalence of congenital coronary anomalies is between 0.3% and 2%, depending on the definition used and the population studied [1,2,3]. Not all congenital coronary anomalies are correlated with negative outcomes. Only anomalous coronaries with an inter-arterial or intramural course, coronary fistulae, and anomalous left coronary artery from the pulmonary artery (ALCAPA) have been associated with an increased rate of cardiac events [4] . Classical definitions of “high-risk features” do not always correlate with necropsy studies [5]. Despite the uncertainty, stakes are high, as coronary anomalies are the secondmost prevalent cause of sudden cardiac death in young athletes [6]. Surgical series report good outcomes with surgical repair, but observational cohorts also report excellent outcomes [7,8]. Guidelines regarding themanagement of coronary anomalies provide indications for surgical repair, but they are different from the management paradigm practiced by most pediatric cardiologists [9,10] .

In June 2016, the Parliament of Canada passed federal legislation that allows eligible Canadian adults to request MAID. A 42-year-old man with VACTERL syndrome, left lung hypoplasia and recurrent heart failure secondary to repaired tetralogy of Fallot (TOF) requested information about MAID. The

OBJECTIVES: The aim of this study was to assess the incidence of persistently positive results on agitated saline contrast injection after patent foramen ovale (PFO) closure, the underlying mechanism, and management. BACKGROUND: Transcatheter intervention to close a PFO is reasonable in highly selected patients younger than 60 years, after a thorough cardioneurological investigation following a cryptogenic stroke, particularly in the presence of thromboembolic disease or in patients at high risk for venous thrombosis. The U.S. Food and Drug Administration approved the Amplatzer PFO Occluder in October 2016 for such an indication. Confirmation of PFO closure is usually verified by an agitated saline contrast injection during an echocardiographic examination. The appearance of bubbles in the left atrium raises the concern of incomplete closure or other sources of shunting. METHODS: The medical records and echocardiograms of patients who were treated with transcatheter closure of a PFO for cryptogenic stroke were reviewed. RESULTS: From January 1998 through December 2015, 880 patients were taken to the catheter laboratory for PFO closure, of whom 568 patients, 320 men (56.3%), underwent transcatheter closure of a PFO using an Amplatzer PFO Occluder, at a mean age of 48.1 ± 12.9 years. The incidence of right-to-left shunting (RLS) was 19.5% at a mean of 4 months' follow-up, which reduced to 8.4% at 11 ± 2 months. Sources of RLS were identified in 10 (1.8%); pulmonary arteriovenous malformation (n = 4) was the most common etiology, followed by leak through the device (n = 3). All patients with additional sources of RLS were treated percutaneously. At 2-year follow-up, 16 patients (2.8%) persisted with only mildly positive results on agitated saline contrast injection, without an apparent additional source of shunting. CONCLUSIONS: Coexistence of a PFO and an additional lesion responsible for RLS is uncommon, but not rare; the majority are amenable to transcatheter or surgical intervention. ispartof: JACC-CARDIOVASCULAR INTERVENTIONS vol:11 issue:11 pages:1095-1104 ispartof: location:United States status: published

The authors sought to prospectively determine the safety and efficacy of next-day discharge using the Vancouver 3M (Multidisciplinary, Multimodality, but Minimalist) Clinical Pathway.Transfemoral transcatheter aortic valve replacement (TAVR) is an alternative to surgery in high- and intermediate-risk patients; however, hospital stays average at least 6 days in most trials. The Vancouver 3M Clinical Pathway is focused on next-day discharge, made possible by the use of objective screening criteria as well as streamlined peri- and post-procedural management guidelines.Patients were enrolled from 6 low-volume (100 TAVR/year), 4 medium-volume, and 3 high-volume (200 TAVR/year) centers in Canada and the United States. The primary outcomes were a composite of all-cause death or stroke by 30 days and the proportion of patients successfully discharged home the day following TAVR.Of 1,400 screened patients, 411 were enrolled at 13 centers and received a SAPIEN XT (58.2%) or SAPIEN 3 (41.8%) valve (Edwards Lifesciences, Irvine, California). In centers enrolling exclusively in the study, 55% of screened patients were enrolled. The median age was 84 years (interquartile range: 78 to 87 years) with a median STS score of 4.9% (interquartile range: 3.3% to 6.8%). Next-day discharge home was achieved in 80.1% of patients, and within 48 h in 89.5%. The composite of all-cause mortality or stroke by 30 days occurred in 2.9% (95% confidence interval: 1.7% to 5.1%), with neither component of the primary outcome affected by hospital TAVR volume (p = 0.51). Secondary outcomes at 30 days included major vascular complication 2.4% (n = 10), readmission 9.2% (n = 36), cardiac readmission 5.7% (n = 22), new permanent pacemaker 5.7% (n = 23), andmild paravalvular regurgitation 3.8% (n = 15).Adherence to the Vancouver 3M Clinical Pathway at low-, medium-, and high-volume TAVR centers allows next-day discharge home with excellent safety and efficacy outcomes.

Background Transcatheter Closure (TC) has become the main stay therapy for many secundum atrial septal defects (ASD) based on short and intermediate term outcome data. Long-term safety and efficacy of this approach among adult patients however, is not well established. Methods and results A comprehensive search of major electronic databases for studies reporting the long-term (≥5 year) outcomes post TC among adults yielded 114 studies, 9 of which had met the inclusion criteria. This included 1015 patients with a mean age of 45 years ± 5.5 years, two third were female with a mean follow up duration 6.4 years ± 2.7 years. The weighted proportions of long-term mortality and stroke with 95% confidence intervals (CI) were 2.4% (95%CI 0.9%–6.1%) and 2.1% (95%CI 0.7%–5.7%) respectively. Atrial arrhythmia occurred in 6.5% (95%CI 3.5%–11.7%) and atrial fibrillation in 4.9% (95%CI 1.9%–11.7%). ASD related re-interventions were encountered in 2.3% (95%CI 1.0%–5.4%) and residual shunt in 4.2% (95% CI 1.3%–12.4%), with 1 case of suspected device erosion 0.9% (95%CI 0.4–2.2%). Frame fractures and late migrations were observed at 4.2% (95%CI 1.5%–11.5%) and 1.2% (95%CI 0.3%–4%) respectively. No cases of occluder endocarditis or thrombosis were reported. In conclusion This is the first study that systematically analyzes the long-term outcomes after TC providing important estimates for various clinical and occluder related outcomes. The analysis suggests preserved long-term safety post TC; however, this is limited due to the variable quality of available evidence and requires further assessment by larger studies with more comprehensive follow-up data.

Vascular complications (VCs) remain a significant cause of morbidity in transcatheter aortic valve implantation (TAVI) patients and are associated with worse outcomes. This research analysed the incidence, impact, and predictors of VCs in transfemoral cases.A retrospective chart review was performed of 388 consecutive TAVI patients between January 2007 and April 2015, which included 237 transfemoral cases. Major and minor VCs were characterised according to the Valve Academic Research Consortium (VARC) guidelines. Logistic regression was completed to identify predictors of VCs.While VCs occurred in 68 (28.7%) cases, only seven (3.38%) were classified as major complications. Twenty-six (10.9%) of these complications occurred intra-operatively, with four being major (1.6%) and 22 minor (9.3%). Post-operative VCs occurred in 42 cases (17.2%), with three (1.3%) being major. Procedures to correct VCs occurred in 10 (4.2%) cases, with the majority (90%) being surgical and the remainder being treated by endovascular techniques. Nine surgical procedures, predominantly embolectomy, were performed to correct post-operative complications. Female gender was a predictor of all major VCs (B = -2.1, p .006). Further, a logistic regression analysis found that when the largest sheath was located on the left side, there were increased minor post-operative complications (B = -0.99, p = .007). Dissections and haematomas made up the majority of VCs. Thirty day mortality was six patients (n = 2.5%), and peri-operative VCs were significantly correlated with 30 day mortality (p = .001, R = 0.21). The 30 day readmission rate comprised nine patients (3.8%), with three (1.3%) due to VCs, including haematomas and groin infections.VCs contribute to operative morbidity in TAVI patients. This study demonstrated low major VC rates over an eight year period. Left sided location of largest sheath size and female gender were predictors of VC.

Valve pathology is a significant component of pediatric cardiovascular disease. Outside the pediatric age group, patients are selecting bioprosthetic valve replacements to avoid the obligate anticoagulation associated with mechanical valves either because of the inability to take anticoagulation, pregnancy considerations, or preference. Bioprosthetic valves, however, inevitably degenerate. The standard treatment is a repeat operation that entails additional risk. Transcatheter valve therapy has rapidly emerged as an appealing alternative. In this manuscript, we discuss the progress in transcatheter valve-in-valve (VinV) procedures. This is essential knowledge for the practicing pediatric cardiologist as it may promote the application of bioprosthetic valves as a treatment option and management strategy.

Background: Recommended thresholds for pulmonary valve replacement (PVR) in asymptomatic patients after tetralogy of Fallot (TOF) repair are continually decreasing. We studied the natural history versus outcomes after PVR in various categories of indexed right ventricular end-diastolic volume (RVEDVi). Hypothesis: We hypothesized that the benefit of PVR would not be outweighed by the risk at lower RVEDVi thresholds. Methods: Acquisition of all repeated measure datapoints and cross-sectional review in 2579 patients (born 1924 - 2011), including 7553 echos, 2579 MRI scans and all interventional data. Analysis was via parametric competing risks techniques and time-related regressions adjusted for repeated measures. Results: Survival was 95%, 81% and 73% at age 20, 40 and 60 years respectively. Freedom from PVR (N=516, mean age 25 years) was 87%, 66% and 45% at 20, 40 and 60 years after repair. Overall, survival after PVR was 98%, 95% and 87% at 1, 10 and 20 years. PVR-free survival in all 345 patients with MRI RVEDVi > 150 was 95% at 15 years. Comparative survival showed no survival advantage with PVR versus natural history for RVEDVi 150-160, 160-170, 170-180 (table); these categories had excellent PVR-free survival approaching 100% at 10 years. Patients with RVEDVi > 200 had late survival decrements with PVR or without. PVR resulted in a large and significant reduction in RVEDVi (mean 40 ml/m 2 , P 150 ml/m 2 . Conclusions: Lowering the RVEDVi threshold for PVR does not appear to offer a clear survival advantage and offers small differences (if any) to RV geometry, risks potential procedure-related morbidity and would mean intervening on many teenagers with repaired TOF who could otherwise anticipate intervention in later life.