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Infective endocarditis (IE) after transcatheter aortic valve implantation (TAVI) is a rare but severe complication. Among 326 patients who underwent TAVI at Grenoble Alpes University Hospital, six (1.8%) cases of IE and 11 (3.4%) cases of bacteraemia were identified. No cases of IE were linked to the intervention; one was due to Staphylococcus aureus despite a screening and targeted decolonization strategy. This underscores the need for randomized studies to evaluate the benefit and cost-effectiveness of this policy. [ABSTRACT FROM AUTHOR]

Background: Right ventricular function predicts outcome in patients with pulmonary hypertension (PH). Therefore accurate assessment of right ventricular function is essential to graduate severity, assess follow-up, and response to therapy. Purpose: To evaluate whether PH severity could be assessed using electrocardiography-gated CT (ECG-gated CT) functional parameters. A further objective was to evaluate cardiac output (CO) using two ECG-gated CT methods: the reference Simpson technique and the fully automatic technique generated by commercially available cardiac software. Material and Methods: Our institutional review board approved this study; patient consent was not required. Twenty-seven patients who had undergone ECG-gated CT and right heart catheterization (RHC) were included. Two independent observers measured pulmonary artery (PA) diameter, PA distensibility, aorta diameter, right ventricular cardiac output (CT-RVCO) and right ventricular ejection fraction (CT-RVEF) with automatic and Simpson techniques on ECG-gated CT. RHC-CO and mean pulmonary arterial pressure (mPAP) were measured on RHC. Relationship between ECG-gated CT and RHC measurements was tested with linear regression analysis. Results: Inter-observer agreement was good for all measurements (r > 0.7) except for CT-RVCO calculated with Simpson's technique (r = 0.63). Pulmonary artery (PA) distensibility was significantly correlated to mPAP (r = -0.426, P = 0.027). CT-RVEF was correlated with mPAP only when issued from Simpson technique (r = -0.417, P = 0.034). CT-RVEF was not significantly correlated to RHC-CO (P > 0.2). CT-RVCO measured with Simpson technique (r = 0.487, P = 0.010) and automatic segmentation (r = 0.549, P = 0.005) correlated equally with RHC-CO. Conclusion: CT-RVEF and CT-RVCO measured on ECG-gated CT are significantly correlated, respectively, to mPAP and RHC-CO in this population with severe reduction of the right ventricular ejection fraction and could be useful for evaluating and following patients with PH. [ABSTRACT FROM AUTHOR]

Purpose The relationship between pulmonary arterial hypertension-specific drug therapy (PAH-SDT) and mortality in Eisenmenger syndrome (ES) is controversial. We aimed to investigate outcomes of patients with ES and their relationship with PAH-SDT. Methods Retrospective, observational, nationwide, multicenter, cohort study. Results We included 340 patients with ES (genetic syndrome, n = 119, 35.3%; pre-tricuspid defect, n = 75, 22.1%). Overall, 276 patients (81.2%) received PAH-SDT (monotherapy: endothelin receptor antagonist (ERA) or phosphodiesterase 5 inhibitor (PDE5i), 46.7%; dual therapy: ERA + PDE5i, 40.9%; triple therapy: ERA + PDE5i + prostanoid, 9.1%). Median PAH-SDT duration was 5.5 years [3.0–9.1]. Events (death, lung or heart-lung transplantation) occurred in 95 (27.9%) patients at a median age of 40.5 years [29.4–47.6]. Cumulative occurrence of events was 16.7% [95% CI 12.8–21.6%] and 46.4% [95% CI 38.2–55.4%] at 40 and 60 years of age, respectively. With age at evaluation or time since PAH diagnosis as time scales, cumulative occurrence of events was lower in patients taking one or two PAH-SDTs ( P = 0.0001 and P = 0.004, respectively), with the largest differences in the post-tricuspid defect subgroup ( P < 0.001 and P < 0.02, respectively) versus patients without PAH-SDT. By multivariate Cox analysis with time since PAH diagnosis as the time scale, NYHA/WHO functional class III/IV, lower SaO 2 , and pre-tricuspid defect were associated with a higher risk of events ( P = 0.002, P = 0.01, and P = 0.04, respectively) and one or two PAH-SDTs with a lower risk of events ( P = 0.009). Conclusion In ES, outcomes are poor but seem better with PAH-SDT. ES with pre-tricuspid defects has worse outcomes despite the delayed onset of the disease. [ABSTRACT FROM AUTHOR]

Abstract A 24-year-old patient was admitted for dyspnoea and syncope. He had a previous history of complete atrio-ventricular septal defect and trisomy 21. At the age of 6 months, in 1984, cardiac catheterization revealed a quasi-systemic pulmonary arterial hypertension with a bidirectional shunt corresponding to an Eisenmenger syndrome. Corrective cardiac surgery was not performed at this time because surgical risk was considered too high. Until the age of 20 years old, he showed few symptoms while under medical treatment. But since 2006, his functional status became worse with an increased dyspnoea, syncopes, and severe cyanosis. In these conditions, haemodynamic parameters have been re-evaluated in 2006 and 2008. They highlighted a late and progressive development of a valvular and infundibular pulmonary stenosis leading to a normalisation of pulmonary arterial pressures. At the age of 24 , the patient underwent corrective cardiac surgery which was successful. Late development of both infundibular and valvular pulmonary stenosis have not been described before in non operated congenital ventricular septal defects, but development of one or the other abnormality would be found in 8% of patients. The physiopathological mechanism of this obstruction is unclear. Nevertheless, in unoperated congenital cardiac shunt lesions, reversibility of severe pulmonary arterial hypertension should be reconidered and re-assessed during follow up.

Une fuite paraprothétique est une complication parfois observée après implantation percutanée d'une valve aortique (TAVI). Peu de données sont disponibles sur l'apport de la fermeture par voie percutanée des fuites para valvulaires cardiaques (FFPP) dans cette indication. Nous rapportons dans cet article 2 cas cliniques de FFPP sur TAVI, l'un sur une valve Edwards® Sapien 3 et l'autre sur une valve Medtronic® Evolut R. Nous présentons ensuite les résultats des 7 FFPP sur TAVI incluses dans l'observatoire prospectif multicentrique FFPP sur un total de 158 inclusions toutes FFPP confondues. Les 7 procédures ont été pratiquées sur 8 fuites avec majoritairement des « plugs » vasculaires (3 Abbott ® Amplatzer Vascular Plugs 2 (AVP2), 3 AVP3, 1 AVP4 et 1 muscular Ventricular Septal Defect occluder (musc VSD)). Ces procédures ont permis d'obtenir un bon résultat procédural sans complication. À 1 mois, les 7 patients sont devenus asymptomatiques. Le recul à 1 an est disponible pour 4 patients : 3 sont asymptomatiques, et 1, qui présentait une seconde FPP non accessible par FFPP, a eu 2 mois après un « TAVI in TAVI ». Cette courte expérience prospective confirme la faisabilité, l'efficacité et la sécurité des FFPP sur TAVI. Des résultats numériquement plus importants avec un suivi plus prolongé sont attendus à la clôture de l'observatoire FFPP. Literature concerning transcutaneous symptomatic para valvular cardiac leaks closure (PVLC) after trans aortic valve implantation (TAVI) is relatively scarce. Hereby we present 2 clinical cases, one on an Edwards® Sapien 3 valve and the other one on a Medtronic® Evolut R valve. We present also the preliminary results of the 7 PVLC on TAVI included in our prospective FFPP registry during the 2 first years of enrolment (2017–2018), for a total of 158 inclusions for all valves. Seven procedures were performed on 8 leaks, using a majority of vascular plugs (3 Abbott® Amplatzer Vascular Plugs 2 (AVP2), 3 AVP3, 1 AVP4, and 1 muscular Ventricular Septal Defect (VSD) occluder). All procedures were successful without complication. At 1-month follow-up, all patients became asymptomatic. One-year follow-up was already available for 4 patients: 3 of them were symptoms free, and one–who had a second leak not suitable for PVLC-, underwent a « TAVI in TAVI » procedure 2 months after PVLC. This short experience demonstrates the feasibility, the efficacy and the safety of PVLC on TAVI. We expect to be able to offer more in depth information at the end of our prospective ongoing study. [ABSTRACT FROM AUTHOR]

Background and Aims: Based on retrospective studies, the 2022 European guidelines changed the definition of post-capillary pulmonary hypertension (pcPH) in heart failure (HF) by lowering the level of mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR). However, the impact of this definition and its prognostic value has never been evaluated prospectively., Methods: Stable left HF patients with the need for right heart catheterization were enrolled from 2010 to 2018 and prospectively followed up in this multicentre study. The impact of the successive pcPH definitions on pcPH prevalence and subgroup [i.e. isolated (IpcPH) vs. combined pcPH (CpcPH)] was evaluated. Multivariable Cox regression analysis was used to assess the prognostic value of mPAP and PVR on all-cause death or hospitalization for HF (primary outcome)., Results: Included were 662 HF patients were (median age 63 years, 60% male). Lowering mPAP from 25 to 20 mmHg resulted in +10% increase in pcPH prevalence, whereas lowering PVR from 3 to 2 resulted in +60% increase in CpcPH prevalence (with significant net reclassification improvement for the primary outcome). In multivariable analysis, both mPAP and PVR remained associated with the primary outcome [hazard ratio (HR) 1.02, 95% confidence interval (CI) 1.00-1.03, P = .01; HR 1.07, 95% CI 1.00-1.14, P = .03]. The best PVR threshold associated with the primary outcome was around 2.2 WU. Using the 2022 definition, pcPH patients had worse survival compared with HF patients without pcPH (log-rank, P = .02) as well as CpcPH compared with IpcPH (log-rank, P = .003)., Conclusions: This study is the first emphasizing the impact of the new pcPH definition on CpcPH prevalence and validating the prognostic value of mPAP > 20 mmHg and PVR > 2 WU among HF patients., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Introduction: Pulmonary arterial hypertension (PAH) is a rare and severe disease for which most of the evidence about prognostic factors, evolution and treatment efficacy comes from cohorts, registries and clinical trials. We therefore aimed to develop and validate a new PAH identification algorithm that can be used in the French healthcare database "Système National des Données de Santé (SNDS)"., Methods: We developed and validated the algorithm using the Grenoble Alpes University Hospital medical charts. We first identified PAH patients following a previously validated algorithm, using in-hospital ICD-10 (10th revision of the International Statistical Classification of Diseases) codes, right heart catheterisation procedure and PAH-specific treatment dispensing. Then, we refined the latter with the exclusion of chronic thromboembolic pulmonary hypertension procedures and treatment, the main misclassification factor. Second, we validated this algorithm using a gold standard review of in-hospital medical charts and calculated sensitivity, specificity, positive and negative predictive value (PPV and NPV) and accuracy. Finally, we applied this algorithm in the French healthcare database and described the characteristics of the identified patients., Results: In the Grenoble University Hospital, we identified 252 unique patients meeting all the algorithm's criteria between 1 January 2010 and 30 June 2022, and reviewed all medical records. The sensitivity, specificity, PPV, NPV and accuracy were 91.0%, 74.3%, 67.9%, 93.3% and 80.6%, respectively. Application of this algorithm to the SNDS yielded the identification of 9931 patients with consistent characteristics compared to PAH registries., Conclusion: Overall, we propose a new PAH identification algorithm developed and adapted to the French specificities that can be used in future studies using the French healthcare database., Competing Interests: Conflict of interest: H. Bouvaist reports payment for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Merck, outside the submitted work. Conflict of interest: M. Humbert reports grants or contracts from Acceleron, AOP Orphan, Janssen, Merck and Shou Ti, outside the submitted work; consulting fees from 35 Pharma, Aerovate, AOP Orphan, Bayer, Chiesi, Ferrer, Janssen, Keros, Merck, MorphogenIX, Shou Ti and United Therapeutics, outside the submitted work; payment for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Janssen and Merck, outside the submitted work; and participation on a data safety monitoring or advisory board for Acceleron, Altavant, Janssen, Merck and United Therapeutics, outside the submitted work. Conflict of interest: D. Montani reports grants or contracts from Acceleron, Janssen and Merck MSD, outside the submitted work; consulting fees from Acceleron, Merck MSD, Janssen and Ferrer, outside the submitted work; payment or honoraria for speakers' bureaus from Bayer, Janssen, Boerhinger, Chiesi, GSK, Ferrer and Merck MSD, outside the submitted work. Conflict of interest: The remaining authors have nothing to disclose., (Copyright ©The authors 2024.)

Background and Aims: Cardiopulmonary fitness in congenital heart disease (CHD) decreases faster than in the general population resulting in impaired health-related quality of life (HRQoL). As the standard of care seems insufficient to encourage and maintain fitness, an early hybrid cardiac rehabilitation programme could improve HRQoL in CHD., Methods: The QUALIREHAB multicentre, randomized, controlled trial evaluated and implemented a 12-week centre- and home-based hybrid cardiac rehabilitation programme, including multidisciplinary care and physical activity sessions. Adolescent and young adult CHD patients with impaired cardiopulmonary fitness were randomly assigned to either the intervention (i.e. cardiac rehabilitation) or the standard of care. The primary outcome was the change in HRQoL from baseline to 12-month follow-up in an intention-to-treat analysis. The secondary outcomes were the change in cardiovascular parameters, cardiopulmonary fitness, and mental health., Results: The expected number of 142 patients was enroled in the study (mean age 17.4 ± 3.4 years, 52% female). Patients assigned to the intervention had a significant positive change in HRQoL total score [mean difference 3.8; 95% confidence interval (CI) 0.2; 7.3; P = .038; effect size 0.34], body mass index [mean difference -0.7 kg/m2 (95% CI -1.3; -0.1); P = .022; effect size 0.41], level of physical activity [mean difference 2.5 (95% CI 0.1; 5); P = .044; effect size 0.39], and disease knowledge [mean difference 2.7 (95% CI 0.8; 4.6); P = .007; effect size 0.51]. The per-protocol analysis confirmed these results with a higher magnitude of differences. Acceptability, safety, and short-time effect of the intervention were good to excellent., Conclusions: This early hybrid cardiac rehabilitation programme improved HRQoL, body mass index, physical activity, and disease knowledge, in youth with CHD, opening up the possibility for the QUALIREHAB programme to be rolled out to the adult population of CHD and non-congenital cardiac disease., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Background: Transcatheter closure of a patent foramen ovale (PFO) is performed in cryptogenic stroke and other conditions. Information is lacking for some devices. Methods: We aimed to evaluate the Figulla Flex II PFO Occluder (FFP) and Figulla Flex UNI Occluder (FFU) through a retrospective multi-center registry. Results : 527 patients were included. Mean age was 48.9 (±13.8) years. The procedure was under transthoracic, transesophageal or intracardiac echocardiography in 185 (35.1%), 193 (36.6%) and 149 (28.3%) cases, respectively, and under general anesthesia in 191 patients (36.2%). The FFP and FFU were used in 408 (77.4%) and 119 (22.6%) cases, respectively. The success rate was 99.1%. Median follow-up was 1.1 (0.5-2.5) years. A new atrial fibrillation/flutter within six months occurred in 14 (2.7%) cases, with no difference between devices. One device embolization in the pulmonary artery was identified two years post-procedure. Residual shunts occurred in 18 (6.9%) cases at 1 year, with TIA in three (16.6%) patients. Out of 437 patients with stroke/TIA, 260 (59%) were followed more than one year after closure. Median follow-up was 2.1 (1.17-3.1) years, with four recurrent strokes/TIA. Conclusions: The FFP and FFU devices are safe and effective for PFO closure, with very few atrial fibrillation/flutter and neurologic events, except in cases with a residual shunt., Competing Interests: Alain Fraisse and Francois Godart are both consultants and proctors for Occlutech Inc and Abbott Inc. Other authors have no conflicts of interest.

In patients with very severe CTEPH eligible for BPA, it is possible to achieve major haemodynamic improvement with upfront triple PH therapy including epoprostenol and then to perform angioplasties https://bit.ly/3vZZvib., Competing Interests: Conflict of interest: The authors have nothing to disclose., (Copyright ©The authors 2024.)

Background and Aims: Transcatheter pulmonary valve implantation (TPVI) is indicated to treat right-ventricular outflow tract (RVOT) dysfunction related to congenital heart disease (CHD). Outcomes of TPVI with the SAPIEN 3 valve that are insufficiently documented were investigated in the EUROPULMS3 registry of SAPIEN 3-TPVI., Methods: Patient-related, procedural, and follow-up outcome data were retrospectively assessed in this observational cohort from 35 centres in 15 countries., Results: Data for 840 consecutive patients treated in 2014-2021 at a median age of 29.2 (19.0-41.6) years were obtained. The most common diagnosis was conotruncal defect (70.5%), with a native or patched RVOT in 50.7% of all patients. Valve sizes were 20, 23, 26, and 29 mm in 0.4%, 25.5%, 32.1%, and 42.0% of patients, respectively. Valve implantation was successful in 98.5% [95% confidence interval (CI), 97.4%-99.2%] of patients. Median follow-up was 20.3 (7.1-38.4) months. Eight patients experienced infective endocarditis; 11 required pulmonary valve replacement, with a lower incidence for larger valves (P = .009), and four experienced pulmonary valve thrombosis, including one who died and three who recovered with anticoagulation. Cumulative incidences (95%CI) 1, 3, and 6 years after TPVI were as follows: infective endocarditis, 0.5% (0.0%-1.0%), 0.9% (0.2%-1.6%), and 3.8% (0.0%-8.4%); pulmonary valve replacement, 0.4% (0.0%-0.8%), 1.3% (0.2%-2.4%), and 8.0% (1.2%-14.8%); and pulmonary valve thrombosis, 0.4% (0.0%-0.9%), 0.7% (0.0%-1.3%), and 0.7% (0.0%-1.3%), respectively., Conclusions: Outcomes of SAPIEN 3 TPVI were favourable in patients with CHD, half of whom had native or patched RVOTs., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: Right ventricular (RV) function plays a critical role in the pathophysiology and acute prognosis of pulmonary embolism (PE). We analyzed the temporal changes of RV function in the cohort of a prospective multicentre study investigating if an early switch to oral anticoagulation in patients with intermediate-risk PE is effective and safe., Methods: Echocardiographic and laboratory examinations were performed at baseline (PE diagnosis), 6 days and 6 months. Echocardiographic parameters were classified into categories representing RV size, RV free wall/tricuspid annulus motion, RV pressure overload and right atrial (RA)/central venous pressure., Results: RV dysfunction based on any abnormal echocardiographic parameter was present in 84% of patients at baseline. RV dilatation was the most frequently abnormal finding (40.6%), followed by increased RA/central venous pressure (34.6%), RV pressure overload (32.1%), and reduced RV free wall/tricuspid annulus motion (20.9%). As early as day 6, RV size remained normal or improved in 260 patients (64.7%), RV free wall/tricuspid annulus motion in 301 (74.9%), RV pressure overload in 297 (73.9%), and RA/central venous pressure in 254 (63.2%). At day 180, the frequencies slightly increased. The median NT-proBNP level decreased from 1448 pg/ml at baseline to 256.5 on day 6 and 127 on day 180., Conclusion: In the majority of patients with acute intermediate-risk PE switched early to a direct oral anticoagulant, echocardiographic parameters of RV function normalised within 6 days and remained normal throughout the first 6 months. Almost one in four patients, however, continued to have evidence of RV dysfunction over the long term., (© 2022. The Author(s).)

Purpose: Patients with pulmonary hypertension (PH) have long been advised to avoid exercise in fear of deterioration in right-sided heart function. Since the 2009 European Society of Cardiology guidelines, rehabilitation in expert centers is considered to have a specific role in care of patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). We report routine rehabilitation effects in patients with PH as a component of real-life multimodal treatment., Methods: Patients with PAH or CTEPH were recommended for either in- or outpatient rehabilitation in addition to their usual care, unless there were practical problems or the patient declined. Assessment was conducted according to New York Heart Association classes, adverse events, 6 min-walk test, hemodynamics, and risk stratification after rehabilitation., Results: Forty-one patients, 61% female, age 60 ±18 yr were included between March 2010 and May 2019. No major adverse events or deaths related to progression of right-sided heart failure were reported. Nevertheless, 22% of participants suffered adverse events in most cases not linked with physical activity. Rehabilitation as add-on to medical therapy and/or arterial deobstruction improved New York Heart Association class: mean difference, -0.39 (95% CI, -0.68 to -0.10), 6-min walk test: mean difference, 80 m (95% CI, 46-114), and was associated with improved right-sided heart hemodynamics. The risk assessment grade improved by -0.25 points (95% CI, -0.44 to -0.06) after rehabilitation., Conclusions: For patients with PAH or CTEPH, supervised rehabilitation as add-on to medical therapy and/or arterial deobstruction is safe and effective in improving patient and clinically related outcomes., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

The current treatment algorithm for chronic thromboembolic pulmonary hypertension (CTEPH) as depicted in the 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines on the diagnosis and treatment of pulmonary hypertension (PH) includes a multimodal approach of combinations of pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and medical therapies to target major vessel pulmonary vascular lesions, and microvasculopathy. Today, BPA of >1700 patients has been reported in the literature from centers in Asia, the US, and also Europe; many more patients have been treated outside literature reports. As BPA becomes part of routine care of patients with CTEPH, benchmarks for safe and effective care delivery become increasingly important. In light of this development, the ESC Working Group on Pulmonary Circulation and Right Ventricular Function has decided to publish a document that helps standardize BPA to meet the need of uniformity in patient selection, procedural planning, technical approach, materials and devices, treatment goals, complications including their management, and patient follow-up, thus complementing the guidelines. Delphi methodology was utilized for statements that were not evidence based. First, an anatomical nomenclature and a description of vascular lesions are provided. Second, treatment goals and definitions of complete BPA are outlined. Third, definitions of complications are presented which may be the basis for a standardized reporting in studies involving BPA. The document is intended to serve as a companion to the official ESC/ERS guidelines., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Pediatric patients with congenital heart disease (CHD) often undergo low dose ionizing radiation (LDIR) from cardiac catheterization (CC) for the diagnosis and/or treatment of their disease. Although radiation doses from a single CC are usually low, less is known about the long-term radiation associated cancer risks. We aimed to assess the risk of lympho-hematopoietic malignancies in pediatric CHD patients diagnosed or treated with CC. A French cohort of 17,104 children free of cancer who had undergone a first CC from 01/01/2000 to 31/12/2013, before the age of 16 was set up. The follow-up started at the date of the first recorded CC until the exit date, i.e., the date of death, the date of first cancer diagnosis, the date of the 18th birthday, or the 31/12/2015, whichever occurred first. Poisson regression was used to estimate the LDIR associated cancer risk. The median follow-up was 5.9 years, with 110,335 person-years. There were 22,227 CC procedures, yielding an individual active bone marrow (ABM) mean cumulative dose of 3.0 milligray (mGy). Thirty-eight incident lympho-hematopoietic malignancies were observed. When adjusting for attained age, gender and predisposing factors to cancer status, no increased risk was observed for lympho-hematopoietic malignancies RR /mGy = 1.00 (95% CI: 0.88; 1.10). In summary, the risk of lympho-hematopoietic malignancies and lymphoma was not associated to LDIR in pediatric patients with CHD who undergo CC. Further epidemiological studies with greater statistical power are needed to improve the assessment of the dose-risk relationship., (© 2023. Springer Nature B.V.)

Objective: To determine whether changes in pulmonary vascular resistance (PVR) and changes in pulmonary artery compliance ( C pa ) are associated with changes in exercise capacity assessed either by changes in peak oxygen consumption ( V ' O 2 ) or by changes in 6-min walk distance (6MWD) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing balloon pulmonary angioplasty (BPA)., Methods: Invasive haemodynamic parameters, peak V ' O 2 and 6MWD were measured within 24 h, before and after BPA (interval 3.1±2.4 months) in 34 CTEPH patients without significant cardiac and/or pulmonary comorbidities, of whom 24 received at least one pulmonary hypertension-specific treatment. C pa was calculated according to the pulse pressure method: C pa =((SV/PP)/1.76+0.1), where SV is the stroke volume and PP is the pulse pressure. The resistance-compliance (RC)-time of the pulmonary circulation was calculated as the PVR and C pa product., Results: After BPA, PVR decreased (562±234 versus 290±106 dyn·s·cm -5 ; p<0.001); C pa increased (0.90±0.36 versus 1.63±0.65 mL·mmHg -1 ; p<0.001); but RC-time did not change (0.325±0.069 versus 0.321±0.083 s; p=0.75). There were improvements in peak V ' O 2 (1.11±0.35 versus 1.30±0.33 L·min -1 ; p<0.001) and in 6MWD (393±119 versus 432±100 m; p<0.001). After adjustment for age, height, weight and gender, changes in exercise capacity, assessed either by peak V ' O 2 or 6MWD, were significantly associated with changes in PVR, but not with changes in C pa ., Conclusions: Contrary to what has been reported in CTEPH patients undergoing pulmonary endarterectomy, in CTEPH patients undergoing BPA, changes in exercise capacity were not associated with changes in C pa ., Competing Interests: Conflict of interest: The authors do not have any conflict of interest to declare relative to the present study. The results of the present study are presented clearly, honestly, and without fabrication, falsification or inappropriate data manipulation., (Copyright ©The authors 2023.)

Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Background: Transcatheter closure of a symptomatic prosthetic paravalvular leak (PVL) is feasible, but there is presently no conclusive evidence to show consistent efficacy. We aimed to identify predictors of clinical success after transcatheter PVL closure., Methods: Consecutive patients referred to 24 European centers for transcatheter PVL closure in 2017 to 2019 were included in a prospective registry ( Fermeture de Fuite ParaProthétique , FFPP). Clinical success was absence of any of the following within 1 month: re-admission for heart failure, blood transfusion, open-heart valvular surgery, and death., Results: We included 216 symptomatic patients, who underwent 238 percutaneous PVL closure procedures on the mitral (64.3%), aortic (34.0%), or tricuspid (1.7%) valve. Symptoms were heart failure, hemolytic anemia, or both in 48.9%, 7.8%, and 43.3% of patients, respectively. One, 2, and 3 leaks were treated during the same procedure in 69.6%, 26.6%, and 3.8% of patients, respectively. The PVL was pinpoint or involved 1/8 or 1/4 of the valve circumference in 18.6%, 52.4%, and 28.1% of cases, respectively. The most frequently used devices were the Vascular Plug 3, Ventricular Septal Defect Occluder, Vascular Plug 2, and Paravalvular Leak Device (45.0%, 16.6%, 14.2%, and 13.6% of cases, respectively). Successful device(s) implantation with leak reduction to ≤grade 2 was obtained in 85.0% of mitral and 91.4% of aortic procedures, respectively ( P =0.164); with major periprocedural adverse event rates of 3.3% and 1.2%, respectively ( P =0.371); and clinical success rates of 70.3% and 88.0%, respectively ( P =0.004). By multivariate analysis, technical failure, mechanical valve, and hemolytic anemia were independently associated with absence of clinical success (odds ratios [95% CIs], 7.7 [2.0-25.0]; P= 0.002; 3.6 [1.1-11.1]; P =0.036; and 3.7 [1.2-11.9]; P =0.025; respectively)., Conclusions: Transcatheter PVL closure is efficient and safe in symptomatic patients but is associated with a lower clinical success rate in patients with hemolysis and/or a mechanical valve., Registration: URL: https://www., Clinicaltrials: gov; Unique identifiers: NCT05089136.

Background: Riociguat and balloon pulmonary angioplasty (BPA) are treatment options for inoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, randomised controlled trials comparing these treatments are lacking. We aimed to evaluate the efficacy and safety of BPA versus riociguat in patients with inoperable CTEPH., Methods: In this phase 3, multicentre, open-label, parallel-group, randomised controlled trial done in 23 French centres of expertise for pulmonary hypertension, we enrolled treatment-naive patients aged 18-80 years with newly diagnosed, inoperable CTEPH and pulmonary vascular resistance of more than 320 dyn·s/cm 5 . Patients were randomly assigned (1:1) to BPA or riociguat via a web-based randomisation system, with block randomisation (block sizes of two or four patients) without stratification. The primary endpoint was change in pulmonary vascular resistance at week 26, expressed as percentage of baseline pulmonary vascular resistance in the intention-to-treat population. Safety analyses were done in all patients who received at least one dose of riociguat or had at least one BPA session. Patients who completed the RACE trial continued into an ancillary 26-week follow-up during which symptomatic patients with pulmonary vascular resistance of more than 320 dyn·s/cm 5 benefited from add-on riociguat after BPA or add-on BPA after riociguat. This trial is registered at ClinicalTrials.gov, NCT02634203, and is completed., Findings: Between Jan 19, 2016, and Jan 18, 2019, 105 patients were randomly assigned to riociguat (n=53) or BPA (n=52). At week 26, the geometric mean pulmonary vascular resistance decreased to 39·9% (95% CI 36·2-44·0) of baseline pulmonary vascular resistance in the BPA group and 66·7% (60·5-73·5) of baseline pulmonary vascular resistance in the riociguat group (ratio of geometric means 0·60, 95% CI 0·52-0·69; p<0·0001). Treatment-related serious adverse events occurred in 22 (42%) of 52 patients in the BPA group and five (9%) of 53 patients in the riociguat group. The most frequent treatment-related serious adverse events were lung injury (18 [35%] of 52 patients) in the BPA group and severe hypotension with syncope (two [4%] of 53 patients) in the riociguat group. There were no treatment-related deaths. At week 52, a similar reduction in pulmonary vascular resistance was observed in patients treated with first-line riociguat or first-line BPA (ratio of geometric means 0·91, 95% CI 0·79-1·04). The incidence of BPA-related serious adverse events was lower in patients who were pretreated with riociguat (five [14%] of 36 patients vs 22 [42%] of 52 patients)., Interpretation: At week 26, pulmonary vascular resistance reduction was more pronounced with BPA than with riociguat, but treatment-related serious adverse events were more common with BPA. The finding of fewer BPA-related serious adverse events among patients who were pretreated with riociguat in the follow-up study compared with those who received BPA as first-line treatment points to the potential benefits of a multimodality approach to treatment in patients with inoperable CTEPH. Further studies are needed to explore the effects of sequential treatment combining one or two medications and BPA in patients with inoperable CTEPH., Funding: Programme Hospitalier de Recherche Clinique of the French Ministry of Health and Bayer HealthCare., Translation: For the French translation of the abstract see Supplementary Materials section., Competing Interests: Declaration of interests XJ, PB, HB, MJ, LS, OS, FP, CP, LG, EF, DM, MH, and GS report grants from the French Ministry of Health and Bayer HealthCare, during the conduct of the study. XJ reports grants from Acceleron, Janssen, and Merck Sharp & Dohme (MSD); personal fees from Janssen and MSD; and non-financial support from Janssen, outside of this study. MC reports personal fees from Menarini, and non-financial support from France Oxygène and Actelion, outside of this study. AC reports grants from Actelion and GlaxoSmithKline; personal fees from MSD, Chiesi, and GlaxoSmithKline; and non-financial support from Asten Santé, Johnson & Johnson, Chiesi, and Boehringer Ingelheim, outside of this study. VC reports grants from Boehringer Ingelheim; personal fees from Boehringer Ingelheim, Roche, Galapagos, Galecto, Shionogi, Fibrogen, RedX, Promedior, Celgene, Bristol Myers Squibb, and PureTech; and non-financial support from Boehringer Ingelheim and Roche, outside of this study. PDG reports personal fees from Bayer HealthCare and MSD, outside of this study. LS reports grants from Janssen, MSD, and GlaxoSmithKline; personal fees from Janssen and MSD; and non-financial support from Janssen, outside of this study. OS reports grants from GlaxoSmithKline, Acceleron, Janssen, and MSD; and personal fees from Gossamer Bio, Janssen, AOP Orphan, Ferrer, Acceleron, and MSD, outside of this study. FP reports grants from Acceleron, MSD, and Janssen; personal fees from MSD and Bayer HealthCare; and non-financial support from Bayer HealthCare, outside of this study. CT reports personal fees from Bayer HealthCare, Janssen, and GlaxoSmithKline; and non-financial support from Orkyn and Bayer HealthCare, outside of this study. EF reports personal fees from MSD, outside of this study. DM reports grants from Acceleron, Janssen, and MSD; and personal fees from Acceleron, Bayer HealthCare, Janssen, and MSD, outside of this study. MH reports grants from the French National Research Agency, Acceleron, Janssen, and MSD; and personal fees from Acceleron, Janssen, MSD, and United Therapeutics, outside of this study. GS reports personal fees from Acceleron, Janssen, MSD, and Bayer HealthCare, outside of this study. All other authors declare no competing interests., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Rationale: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is associated with pulmonary endothelial dysfunction. There are limited data available on the outcomes of coronavirus disease (COVID-19) in patients with pulmonary hypertension (PH), a disease characterized by pulmonary endothelial dysfunction. Objectives: To describe characteristics and outcomes of patients with precapillary PH and COVID-19. Methods: We prospectively collected characteristics, management, and outcomes of adult patients with precapillary PH in the French PH network who had COVID-19 between February 1, 2020, and April 30, 2021. Clinical, functional, and hemodynamic characteristics of PH before COVID-19 were collected from the French PH registry. Measurements and Main Results: A total of 211 patients with PH (including 123 with pulmonary arterial hypertension, 47 with chronic thromboembolic PH, and 41 with other types of PH) experienced COVID-19, and 40.3% of them were outpatients, 32.2% were hospitalized in a conventional ward, and 27.5% were in an ICU. Among hospitalized patients ( n  = 126), 54.0% received corticosteroids, 37.3% high-flow oxygen, and 11.1% invasive ventilation. Right ventricular and acute renal failure occurred in 30.2% and 19.8% of patients, respectively. Fifty-two patients (all hospitalized) died from COVID-19. Overall mortality was 24.6% (95% CI [confidence interval], 18.8-30.5) and in-hospital mortality 41.3% (95% CI, 32.7-49.9). Nonsurvivors were significantly older, more frequently male and suffering comorbidities (diabetes, chronic respiratory diseases, systemic hypertension, chronic cardiac diseases, and/or chronic renal failure), and had more severe PH at their most recent evaluation preceding COVID-19 diagnosis (in terms of functional class and 6-minute-walk distance; all P  < 0.05). Use of pulmonary arterial hypertension therapy was similar between survivors and nonsurvivors. Conclusions: COVID-19 in patients with precapillary PH was associated with a high in-hospital mortality. The typical risk factors for severe COVID-19 and severity of PH were associated with mortality in this population.

Coronary microvascular dysfunction (CMVD) is common and associated with poorer outcomes in patients with ST Segment Elevation Myocardial Infarction (STEMI). The index of microcirculatory resistance (IMR) and the index of hyperemic microvascular resistance (HMR) are both invasive indexes of microvascular resistance proposed for the diagnosis of severe CMVD after primary percutaneous coronary intervention (pPCI). However, these indexes are not routinely assessed in STEMI patients. Our main objective was to clarify the association between IMR or HMR and long-term major adverse cardiovascular events (MACE), through a systematic review and meta-analysis of observational studies. We searched Medline, PubMed, and Google Scholar for studies published in English until December 2020. The primary outcome was a composite of cardiovascular death, non-cardiovascular death, non-fatal myocardial infarction, non-fatal stroke, and rehospitalization for heart failure occurring after at least 6 months following CMVD assessment. We identified 6 studies, reporting outcomes in 1094 patients (mean age 59.7 ± 11.4 years; 18.2% of patients were women) followed-up from 6 months to 7 years. Severe CMVD, defined as IMR > 40 mmHg or HMR > 3mmHg/cm/sec was associated with MACE with a pooled HR of 3.42 [2.45; 4.79]. Severe CMVD is associated with an increased risk of long-term adverse cardiovascular events in patients with STEMI. Our results suggest that IMR and HMR are useful for the early identification of severe CMVD in patients with STEMI after PCI, and represent powerful prognostic assessments as well as new therapeutic targets for clinical intervention., Competing Interests: The authors have declared that no competing interests exist.

Excessive ventilation (V̇E) during exercise, ascribed to heightened neural ventilatory drive and/or to increased "wasted" ventilation, is a feature of chronic thromboembolic pulmonary hypertension (CTEPH). In selected CTEPH patients, balloon pulmonary angioplasty (BPA) allows near-normalization of resting haemodynamic parameters but does not allow excess exercise hyperventilation to normalize. Neural ventilatory drive can be estimated by studying how arterial PCO 2 (PaCO 2 ), end-tidal PCO 2 (PETCO 2 ), V̇E and CO 2 output (V̇CO 2 ) change across the exercise-to-recovery transition during a cardiopulmonary exercise test. Increased "wasted" ventilation can be quantified by the physiological dead space fraction of tidal volume (VD/VT) calculated with the Enghoff simplification of the Bohr equation. These measurements were made before and after BPA in 22 CTEPH patients without significant cardiac and/or pulmonary comorbidities. Our observations suggest that before BPA, excessive hyperventilation was secondary to both heightened neural ventilatory drive and increased "wasted" ventilation; after BPA, measurements made across the exercise-to-recovery transition suggest that heightened neural ventilatory drive was no longer present., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Background: In patients undergoing balloon pulmonary angioplasty (BPA) for inoperable chronic thromboembolic pulmonary hypertension (CTEPH), single-centre series from expert centres have recognized a learning curve for the magnitude of haemodynamic benefits., Objective: To report our 7-year experience with BPA, focusing on haemodynamic effects, complication rates and radiation exposure over time., Methods: Patients with CTEPH who were treated with BPA between May 2013 and February 2020 were analysed during the 'initial period' versus the 'recent period' (split date: March 2017)., Results: Among 192 patients who underwent at least one BPA procedure, 156 were included in the safety/radiation analysis and 119 were included in the efficacy analysis. During the 'recent period' versus the 'initial period', the median [interquartile range] number of procedures per patient was higher (4.5 [4.0-6.0] vs. 4.0 [3.0-4.0]; P=0.03), as was the number of dilated vessels per procedure (4.0 [3.5-5.0] vs. 3.5 [3.0-4.0]; P=0.002). Changes in haemodynamic parameters were also greater (mean pulmonary artery pressure: -22% [-31% to -14%] vs. -37% [-44% to -29%]; P=0.001; pulmonary vascular resistance: -38% [-51% to -8%] vs. -53% [-69% to -33%]; P=0.002); complication rates were similar (5.7% vs. 9.3% of procedures; P=0.38); and radiation exposure was lower (effective dose per patient: 43.9 [31.6-66.5] vs. 67.8 [47.9-101.9] mSv; P<0.001)., Conclusion: Our analysis is consistent with a learning curve for the magnitude of haemodynamic improvements. The complication rate was low and did not change over time, but radiation exposure decreased., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Coronary physiological assessment is now widely used to assess epicardial coronary lesions in cath lab. Based on clinical evidence, fractional flow reserve (FFR) is the gold standard method to select whether epicardial coronary lesions need revascularization. While additional epicardial indexes, such as instantaneous wave-free ratio (iFR), are also used for revascularization decision-making, several indexes are now also available to explore the coronary microcirculation. Therefore, coronary physiological assessment now allows to explore the entire coronary tree and offer the potential of precision medicine for patients affected by coronary artery disease (CAD). This paper will provide review of the epicardial and microvascular indexes available for the assessment of coronary physiology. More specifically, the already demonstrated contributions of these indexes in the management of CAD and the role they could play in precision medicine will be reviewed with special emphasis on chronic coronary syndrome., Competing Interests: Gilles Barone-Rochette has received research grants from Merck Sharp and Dohme, and consulting fees from Bayer, Abbott vascular, Novo Nordisk, Sanofi, and AMGEN. The others authors declare no conflict of interest., (Copyright: © 2022 The Author(s). Published by IMR Press.)

Background: Transcatheter closure of a patent ductus arteriosus in children is widely performed to reduce symptoms and decrease the risk of endocarditis. Most arterial ducts are closed successfully with dedicated devices. However, in a tubular or "type C" patent ductus arteriosus with congestive heart failure, the occlusion is more challenging with these devices, with a higher risk of complications, such as aortic or left pulmonary stenosis and device embolization., Aim: To report our experience with muscular ventricular septal defect devices for patent ductus arteriosus occlusion in small children., Methods: Retrospective observational series of patients weighing<10kg, with a tubular patent ductus arteriosus (typeC) and congestive heart failure, who underwent transcatheter closure with a muscular ventricular septal defect device between 2017 and 2019., Results: Eight patients were included. The mean age and weight at closure were 6.3 months (range 1-18 months) and 5.3kg (range 2.4-8.2kg), respectively. All patent ductus arteriosus were occluded successfully using Occlutech® (N=3) or Amplatzer® (N=5) muscular ventricular septal defect devices. In four cases, the muscular ventricular septal defect device was used after failure to close the patent ductus arteriosus with a dedicated patent ductus arteriosus device. Two patients had mild left pulmonary artery stenosis, with a maximum velocity on continuous Doppler of 3m/s and 2.7m/s, respectively. After a mean follow-up of 28 months (range 14-41 months), all patients were asymptomatic with excellent results. The mild pulmonary stenosis improved, with a maximum velocity of 2.3m/s in both patients., Conclusions: Closure of tubular patent ductus arteriosus in small children with congestive heart failure using a muscular ventricular septal defect device is safe in this preliminary experience. Further studies with more patients are warranted., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Objectives: The aim of this study was to assess the diagnostic performances of 18 F-fluorodeoxyglucose ( 18 F-FDG) positron emission tomography (PET)/computed tomography (CT) in congenital heart disease (CHD) patients with pulmonary prosthetic valve or conduit endocarditis (PPVE) suspicion., Background: PPVE is a major issue in the growing CHD population. Diagnosis is challenging, and usual imaging tools are not always efficient or validated in this specific population. Particularly, the diagnostic yield of 18 F-FDG PET/CT remains poorly studied in PPVE., Methods: A retrospective multicenter study was conducted in 8 French tertiary centers. Children and adult CHD patients who underwent 18 F-FDG PET/CT in the setting of PPVE suspicion between January 2010 and May 2020 were included. The cases were initially classified as definite, possible, or rejected PPVE regarding the modified Duke criteria and finally by the Endocarditis Team consensus. The result of 18 F-FDG PET/CT had been compared with final diagnosis consensus used as gold-standard in our study., Results: A total of 66 cases of PPVE suspicion involving 59 patients (median age 23 years, 73% men) were included. Sensitivity, specificity, positive predictive value, and negative predictive value of 18 F-FDG PET/CT in PPVE suspicion were respectively: 79.1% (95% CI: 68.4%-91.4%), 72.7% (95% CI: 60.4%-85.0%), 91.9% (95% CI: 79.6%-100.0%), and 47.1% (95% CI: 34.8%-59.4%). 18 F-FDG PET/CT findings would help to correctly reclassify 57% (4 of 7) of possible PPVE to definite PPVE., Conclusions: Using 18 F-FDG PET/CT improves the diagnostic accuracy of the Duke criteria in CHD patients with suspected PPVE. Its high positive predictive value could be helpful in routine to shorten diagnosis and treatment delays and improve clinical outcomes., Competing Interests: Funding Support and Author Disclosures Drs Venet, Jalal, and Thambo were supported by the French Government as part of the “Investments of the future” program managed by the National Research Agency (grant reference ANR-10-IAHU-04). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Highly thrombotic coronary lesions continue to be a serious and clinically significant problem that is not effectively and completely addressed by current technology., Objectives: We aimed to investigate whether a micro-net mesh (MNM) technology covering stent could preserve the index of microcirculatory resistance (IMR) after percutaneous coronary intervention (PCI) in patients with high thrombus burden., Methods and Results: Fifty-two patients with non-ST elevation myocardial infarction or ST Elevation Myocardial Infarction and high thrombus burden (TIMI thrombus grade ≥ 3) were randomized into two groups, PCI with a MNM covering stent (MNM group, n = 25) and PCI with any commercially available stent (DES group, n = 27). As the primary endpoint, IMR was measured immediately after PCI using a pressure-temperature sensor-tipped coronary wire. The secondary endpoint was left ventricular ejection fraction (LVEF) at 6 months of follow-up. The IMR in the MNM group was significantly lower in comparison to the DES group (33.2 [21.3, 48.9] vs. 57.2 [39.9, 98.0], p = 0.005). No significant differences were observed in baseline LVEF (54.5 ± 10.2% vs. 53.1 ± 6.87%, p = .57), while LVEF was significantly improved at follow-up in the MNM group (61.1 ± 7.1% vs. 53.9 ± 6.35%, p = .0001)., Conclusion: MNM technology significantly improved coronary microvascular dysfunction after PCI in patient with acute coronary syndrome and appears as a useful technological option for thrombus management., (© 2021 Wiley Periodicals LLC.)