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Background: The indications for percutaneous pulmonary valve implantation (PPVI) have been extended to include large dysfunctional right ventricular outflow tracts (RVOTs). Prestenting of the RVOT is commonly performed before PPVI in order to ensure a stable landing zone. The AndraStent XXL (AndraMed GmbH, Reutlingen, Germany), a cobalt-chromium stent with semi-open cell design, has unique mechanical properties in this indication but is no longer available in France., Aims: To assess the efficiency of AndraStent XXL before PPVI., Methods: In this retrospective multicentre cohort study, 86 AndraStents XXL were implanted in 77 patients in 6 centres., Results: PPVI was indicated mainly for pulmonary regurgitation (75.3%) in native or patched RVOT (88.3%). The stents were manually mounted on balloon catheters and delivered through sheaths using a conventional femoral approach. PPVI was performed successfully in 97.4% of patients after successful prestenting, generally during the same procedure (77.9%). There were no deaths associated with stent implantation, and four patients experienced five complications, mainly stent embolization, including one requiring surgery. Neither stent fracture nor dysfunction were observed in any patient during a mean follow-up of 19.2±8.7months. Stent analysis showed an excellent maximal stent expansion (97.1%) regardless of balloon size. A 22.3%±3.4 stent shortening with a 30mm balloon was observed., Conclusions: Implantation of large cobalt-chromium AndraStent XXL stents is efficient for prestenting before PPVI., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Background: Transcatheter closure of patent ductus arteriosus (PDA) in premature infants has been shown to be feasible in small series. Outcomes in larger series are currently lacking., Material: All premature infants (< 36 weeks GA) who underwent transcatheter PDA closure were included in a multicenter French national survey. Demographic data (gestational age [GA], birth weight [BW]) and procedural data (weight [PW], age at procedure [AP], procedural success, fluoroscopy time, and type of device) were collected. Outcomes and procedural complications were reviewed., Results: Between September 2013 and June 2017, 102 patients were included. In 71 cases, PDA pharmacological closure had been attempted. Mean GA was 27 ± 2.9 weeks. Mean BW and PW were 1,040 ± 715 g and 1,543 ± 698 g, respectively. Mean AP was 39 ± 26 days. Number of premature infants <1 kg, between 1 and 2 kg, and > 2 kg was 21, 59, and 22, respectively. Mean fluoroscopic time was 6.5 min. Success rate was 99%. Device- or procedure-related complications were reported in nine patients (8.9%) including three LPA stenoses (requiring surgery in two and balloon dilatation in one), two neo-coarctations (one requiring subsequent surgery), and three instances of tricuspid valve regurgitation at follow-up. Seven deaths were reported, none being related to the procedure. Mean follow-up was 39.75 ± 13.1 months., Conclusion: In this large series of premature infants undergoing transcatheter PDA closure, it was demonstrated that this procedure can be performed successfully in the vast majority of patients with an acceptable complication rate. Future efforts should focus on minimizing complications, particularly device-related vascular stenoses., (© 2019 Wiley Periodicals, Inc.)

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., (Copyright © 2019. Published by Elsevier Masson SAS.)

Introduction: Symptoms and functional limitation are frequently reported by survivors of acute pulmonary embolism (PE). However, current guidelines provide no specific recommendations on which patients should be followed after acute PE, when follow-up should be performed, and which tests it should include. Definition and classification of late PE sequelae are evolving, and their predictors remain to be determined., Methods: In a post hoc analysis of the Pulmonary Embolism Thrombolysis (PEITHO) trial, we focused on 219 survivors of acute intermediate-risk PE with clinical and echocardiographic follow-up 6 months after randomisation as well as over the long term (median, 3 years after acute PE). The primary outcome was a composite of (1) confirmed chronic thromboembolic pulmonary hypertension (CTEPH) or (2) 'post-PE impairment' (PPEI), defined by echocardiographic findings indicating an intermediate or high probability of pulmonary hypertension along with New York Heart Association functional class II-IV., Results: Confirmed CTEPH or PPEI occurred in 29 (13.2%) patients, (6 with CTEPH and 23 with PPEI). A history of chronic heart failure at baseline and incomplete or absent recovery of echocardiographic parameters at 6 months predicted CTEPH or PPEI at long-term follow-up., Conclusions: CTEPH or PPEI occurs in almost one out of seven patients after acute intermediate-risk PE. Six-month echocardiographic follow-up may be useful for timely detection of late sequelae.

Background: Advances in congenital heart disease (CHD) have transferred the mortality from childhood to adulthood. Exercise capacity in young patients with CHD remains lower than in the general population, resulting in deconditioning and impaired quality of life. Evidence based-medicine in cardiac rehabilitation in this age group with CHD remains limited. We present the QUALI-REHAB study rationale, design and methods., Methods: The QUALI-REHAB trial is a nationwide, multicentre, randomised, controlled study, aiming to assess the impact of a combined centre and home-based cardiac rehabilitation program on the quality of life of adolescents and young adults (13 to 25 years old) with CHD. Patients with a maximum oxygen uptake (VO2 max ) < 80% and/or a ventilatory anaerobic threshold (VAT) < 55% of predicted VO2 max , will be eligible. Patients will be randomised into 2 groups (12-week cardiac rehabilitation program vs. controls). The primary outcome is the change in the PedsQL quality of life score between baseline and 12-month follow-up. A total of 130 patients are required to observe a significant increase of 7 ± 13.5 points in the PedsQL, with a power of 80% and an alpha risk of 5%. The secondary outcomes are: VO2 max , VAT, stroke volume, clinical outcomes, physical and psychological status, safety and acceptability., Conclusion: After focusing on the survival in CHD, current research is opening on secondary prevention and patient-related outcomes. The QUALI-REHAB trial intends to assess if a combined centre and home-based rehabilitation program, could improve the quality of life and the exercise capacity in youth with CHD., Trial Registration: Clinicaltrials.gov (NCT03690518)., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Atrial septal defects (ASDs) can be symptomatic in small children in cases of co-morbidities. Transcatheter closure remains controversial for large defects in small children., Objective: To describe transcatheter closure of ASDs in children with device/weight ratio ≥1.5., Methods: We retrospectively studied between January 2000 and January 2016 all cases of percutaneous ASD closure with device/weight ratio ≥1.5 in 6 European centres., Results: Forty patients were included with female/male ratio of 1.2. Median age and weight were 30.9 months (4.1-102.0) and 11.0 kg respectively (3.8-19.0). Median device size/weight ratio was 1.7 (1.5-2.3). All patients were symptomatic, with pulmonary hypertension in 13 (33%). Procedures were performed under general anesthesia or light sedation (n = 4), with transthoracic (n = 25) or transesophageal echocardiography (n = 15) guidance. Balloon stretched diameter (n = 32) was larger than the echocardiographic diameter (19 versus 15 mm, R = 0.6; p < 0.001). Deficient rims other than the anterior-superior one were found in 33% of cases. Device implantation was successful in 39 patients (97.5%). Minor complications occurred in 10% of cases, whereas severe complications rate was 5%: Complete atrioventricular block in one patient that resolved after surgical extraction of the device and thrombosis in the inferior vena cava in one patient. During a median follow-up of 52 months, there was no residual shunt. No case of erosion or embolization was reported and pulmonary hypertension resolved in all patients., Conclusion: Percutaneous closure of large ASD in small and symptomatic children is feasible and allows clinical improvement with acceptable rate of complications in high risk population., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Percutaneous pulmonary valve implantation (PPVI) is a routine treatment for dysfunctional right ventricular outflow tract. Infective endocarditis (IE) is a major concern., Aim: To report French experience with the Melody™ valve (Medtronic Inc., Minneapolis, MN, USA)., Methods: All patients who underwent PPVI were recorded in a multicentre French national survey. Demographic and procedural data were collected from patients with IE. Bacterial identification, diagnostic tools and outcome were recorded., Results: Forty-five cases of IE were diagnosed in 43 patients. The cumulative IE incidence was 11.8% (95% confidence interval [CI] 8.5-15.9). The annualized IE incidence was 3.6% (95% CI 0-4.8). Freedom from IE was 96.3% and 85.8% at 12 months and 60 months, respectively. IE incidence did not change during the study period. The mean interval between PPVI and IE was 2.6±2.1 years (range, 5 days to 7.3 years). Fifteen patients with IE required intravenous antibiotics only. Seven patients had early interventional cardiac catheterization to relieve severe right ventricular outflow tract obstruction. Twenty-four patients had surgical valve replacement (six urgently; nine semi-urgently; nine electively). Staphylococcus aureus IE required surgery in all but one patient. Three patients died before any treatment. Three additional patients died, giving a mortality rate of 14%. Global survival in the total cohort of patients who received a Melody valve was excellent (96.5% at 5 years). When comparing survival curves between the IE and non-IE groups, death and cardiovascular events were statistically significantly higher in the IE group (log-rank P<0.0001)., Conclusion: Melody valve IE is a severe complication following PPVI. The annualized IE incidence in this cohort was similar to rates reported in other studies. With rapid diagnosis and adequate treatment, outcome has improved, and unfavourable outcome is mainly associated with S. aureus., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background: Percutaneous paravalvular leak (PVL) closure has emerged as a palliative alternative to surgical management in selected high-risk patients. Percutaneous procedures are challenging, especially for mitral PVL. Accurate imaging of the morphologies of the defects is mandatory, together with precise guidance in the catheterization laboratory to enhance success rates., Aims: To describe imaging modalities used in clinical practice to guide percutaneous PVL closure and assess the potential of new imaging tools., Methods: Data from the 'Fermeture de Fuite paraprothétique' (FFPP) register were used. The FFPP register is an international multi-institutional collaborative register started in 2017 with a retrospective and a prospective part. A descriptive analysis of multimodality imaging used to guide PVL closure in clinical practice was performed., Results: Data from 173 procedures performed in 19 centres from three countries (France, Belgium and Poland) were collected, which included eight cases of PVL following transcatheter valve replacement. Transoesophageal echocardiography was used in 167 cases (96.5%) and 3D echocardiography in 87.4% of cases. In one case, 3D-echocardiography was fused with fluoroscopy images in real time using echonavigator software. Details about multimodality imaging were available from a sample of 31 patients. Cardiac computed tomography (CT) was performed before 10 of the procedures. In one case, fusion between preprocedural cardiac CT angiography data and fluoroscopy data was used. In two cases, a 3D model of the valve with PVL was printed., Conclusion: Echocardiography, particularly the 3D mode, is the cornerstone of PVL imaging. Other imaging modalities, such as cardiac CT and cardiac magnetic resonance imaging, may be of complementary interest. New techniques such as imaging fusion and printing may further facilitate the percutaneous approach of PVLs., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Objectives: Over the last few decades different strategies have been proposed to treat persistent ductal patency in premature infants. The advent of the Amplatzer Duct Occluder II Additional Size (ADOIIAS) provided the potential to close the patent ductus arteriosus (PDA). Opinions differ on the significance and treatment of PDA in premature neonates. Because surgical ligation and medical therapy both have their drawbacks, interventional catheterisation can be considered as an alternative means of closing the ductus arteriosus. Our aim was to analyse the feasibility, safety and efficacy of this device in premature infants weighing <1200 g at procedure., Methods: Eighteen premature infants underwent transcatheter closure. The procedure was performed in the catheterisation laboratory by venous cannulation without angiography. The position of the occluder was directed by X-ray and ultrasound. We looked at procedural details, device size selection, complications and short-term and mid-term outcomes., Results: Eighteen infants born at gestational ages ranging between 23.6 and 29+6 weeks (mean±SD 25+6±3 weeks) underwent transcatheter PDA closure. Their mean age and weight at the time of the procedure was 20 days (range 8-44 days) and 980 g (range 680-1200 g), respectively. The mean PDA and device waist diameters were 3.2±0.6 mm (range 2.2-4 mm) and 4.5±0.6 mm, respectively, and the mean PDA and device lengths were 4.3±1.2 mm (range 2-10 mm) and 2.5±0.9 mm, respectively. Complete closure was achieved in all but one patient. There was no device migration. One patient developed a left pulmonary artery obstruction. Three infants died. Two deaths were related to complications of prematurity and one to the procedure., Conclusions: Transcatheter closure of a PDA is feasible in very low weight infants with ADOIIAS and is an alternative to surgery. Success requires perfect selection and placement of the occluder., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Ivabradine is an original drug that has been approved in two indications (systolic heart failure and angina). The aim of this short review is to draw the attention of clinician prescribers to the evidence base of ivabradine. Three large randomized trials testing ivabradine versus placebo have been performed. The BEAUTIFUL and SIGNIFY trials were in fact negative in the treatment of angina while the SHIFT trial found a marginal benefit of ivabradine over placebo in the treatment of heart failure. These important results are put into perspective in order to improve the assessment of risk-cost/benefit balances when ivabradine is considered. Ideally, a further clinical trial investigating the use of ivabradine in heart failure should be carried out with optimal treatment of the patient population in order to identify the subgroup of patients who respond to ivabradine.

Chronic thromboembolic pulmonary hypertension (CTEPH) is uncommon. Its diagnosis should not be delayed as its prognosis is poor if not treated. In most cases, an acute pulmonary embolism is found in the medical history of the patient. Once suspected, a specific work-up should be performed in a pulmonary hypertension (PH) center. The ventilation/perfusion scan has a central role in this workup but the emergence of non invasive imaging technologies provides morphological and functional information which take part in the therapeutic decision making, such as operability. Surgical endarterectomy remains the only curative treatment. In some specific patients, percutaneous transluminal pulmonary angioplasty (AAP) is performed. This developing technique is a safe and efficient treatment on a clinical and hemodynamic standpoint. The main complication after AAP is lung reperfusion edema., Competing Interests: Les auteurs n’ont déclaré aucun conflit d’intérêts en relation avec cet article.

This study aimed to describe the long-term outcomes of pulmonary arterial hypertension (PAH) induced by dasatinib.21 incident, right heart catheterisation-confirmed cases of dasatinib-induced PAH were identified from the French Pulmonary Hypertension Registry. Clinical and haemodynamic variables were compared from baseline to last follow-up (median (range) 24 (1-81) months).Median age was 52 years and 15 patients were female (71%). 19 patients received dasatinib for chronic myelogenous leukaemia for a median (range) duration of 42 (8-74) months before PAH diagnosis. No bone morphogenic protein receptor-2 ( BMPR2 ) mutations were found in the 10 patients tested. Dasatinib was uniformly discontinued and 11 patients received PAH medications. Four patients died during follow-up. New York Heart Association functional class improved from 76% in class III/IV to 90% in class I/II (p<0.01). Median (range) 6-min walk distance improved from 306 (0-660) to 430 (165-635) m (p<0.01). Median (range) mean pulmonary arterial pressure improved from 45 (30-70) to 26 (17-50) mmHg (p<0.01) and pulmonary vascular resistance from 6.1 (3.2-27.3) to 2.6 (1.2-5.9) Wood units (p<0.01). Patients treated with PAH medications had worse baseline haemodynamics but similar long-term outcomes to untreated patients. PAH persisted in 37% of patients.Dasatinib-induced PAH frequently improves after discontinuation but persisted in over one-third of patients, therefore systematic follow-up is essential., Competing Interests: Conflict of interest: Disclosures can be found alongside this article at erj.ersjournals.com, (Copyright ©ERS 2017.)

Aims: The aim of this study was to ascertain whether a minimalist immediate mechanical intervention (MIMI) aiming to restore an optimal Thrombolysis In Myocardial Infarction (TIMI) flow in the culprit artery, followed ≥7 days later by a second percutaneous coronary intervention with intentional stenting, is safe in patients with ST-segment elevation myocardial infarction and large thrombotic burden., Methods and Results: SUPER-MIMI was a prospective, observational trial conducted between January 2014 and April 2015 in 14 French centres. A total of 155 patients were enrolled. The pharmacological therapy was left to the operator's discretion. Eighty-one patients (52.3%) had glycoprotein IIb/IIIa inhibitors (GPI) initiated before the end of the first procedure. The median (interquartile range [IQR]) delay between the two procedures was eight (seven to 12) days. Infarct-related artery reocclusion between the two procedures (primary endpoint) occurred in two patients (1.3%), neither of whom received GPI treatment. TIMI flow was maintained or improved between the end of the first procedure and the beginning of the second procedure in all patients. Thrombotic burden and stenosis severity diminished significantly between the two procedures. Stents were ultimately implanted in 97 patients (62.6%)., Conclusions: Deferred stenting (≥7 days) in patients with a high thrombus burden was safe on a background of GPI therapy.

Nowadays, guidelines are derived from the findings of randomized controlled therapeutic trials. However, an overall significant P value does not exclude that some patients may be harmed by or will not respond to the therapeutic agent being studied. Trials in patients with a low risk of events and/or a limited chance of providing significant differences in therapeutic effects require a large patient population to demonstrate a beneficial effect. Composite efficacy endpoints are often employed to obviate the need for a large patient population when low rates of events or limited therapeutic efficacy are anticipated. Results of randomized controlled therapeutic trials are commonly expressed in terms of relative risk reduction, whereas absolute risk reduction allows the calculation of the "number needed to treat" to prevent an adverse outcome. The number needed to treat is a far more clinically relevant variable than relative risk reduction. The clinician's mission is to match treatment to patient with the goal of achieving optimal therapeutic response. Drug-safety monitoring is also of major importance to avoid exposing patients to irreversible adverse effects. Unfortunately, drug-safety monitoring is often overlooked in routine clinical practice. Finally, the lack of long-term therapeutic data (>5-10 years) is an unsolved dilemma, as most trials are limited to a duration of a few months or years., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Background: The relationship between pulmonary arterial hypertension-specific drug therapy (PAH-SDT) and mortality in Eisenmenger syndrome (ES) is controversial., Aims: To investigate outcomes in patients with ES, and their relationship with PAH-SDT., Methods: Retrospective, observational, nationwide, multicentre cohort study., Results: We included 340 patients with ES: genetic syndrome (n=119; 35.3%); pretricuspid defect (n=75; 22.1%). Overall, 276 (81.2%) patients 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 years]. 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]. The cumulative occurrence of events was 16.7% [95% confidence interval 12.8-21.6%] and 46.4% [95% confidence interval 38.2-55.4%] at age 40 and 60 years, 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 multivariable Cox analysis, with time since PAH diagnosis as time scale, New York Heart Association/World Health Organization functional class III/IV, lower peripheral arterial oxygen saturation and pretricuspid 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)., Conclusions: Outcomes are poor in ES, but seem better with PAH-SDT. ES with pretricuspid defects has worse outcomes despite the delayed disease onset., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Portopulmonary hypertension (PoPH) is diagnosed in 2-6% of liver transplantation (LT) candidates. We studied outcomes of candidates for LT suffering from PoPH. Data were collected retrospectively from a prospective registry. Pulmonary hemodynamic variables were collected at the time of PoPH diagnosis, at last evaluation before LT, and within 6 months and beyond 6 months after LT. Forty-nine patients (35 males, 48 ± 8 years) were analyzed (median Model for End-Stage Liver Disease score 20). At baseline, mean pulmonary artery pressure (mPAP) was 44 ± 10 mm Hg (range 26-73 mm Hg), cardiac index was 3.5 ± 0.9 L/min/m 2 , and pulmonary vascular resistance was 5.6 ± 2.8 Wood units. Hemodynamic reassessment performed in 35 patients who were treated with pulmonary arterial hypertension-targeted therapies before LT resulted in significant decreases in both mPAP (36 ± 7 versus 47 ± 10 mm Hg, P < 0.0001) and pulmonary vascular resistance (3.0 ± 1.4 versus 6.1 ± 3.1 Wood units, P < 0.0001). Fourteen patients (29%) died without having had access to LT. Thirty-five patients underwent LT and were followed up for a median of 38 months. Eight patients (23%) died after LT including 5 due to PoPH (after 1 day to 6 months). Among survivors (n = 27), all patients treated with intravenous epoprostenol were weaned off post-LT, and endothelin receptor antagonist or phosphodiesterase type 5 inhibitors were continued in 15/27 patients (55%). At last evaluation, 20/27 patients (74%) had mPAP <35 mm Hg and 8 of them (30%) had mPAP <25 mm Hg. Overall survival estimates after LT were 80%, 77%, and 77% at 6 months, 1 year, and 3 years, respectively., Conclusion: Stabilization or reversibility of PoPH seems to be an attainable goal using the combination of pulmonary arterial hypertension-targeted therapies and LT in patients who are transplantation candidates. (Hepatology 2017;65:1683-1692)., (© 2016 by the American Association for the Study of Liver Diseases.)

Background: The long-term effect of thrombolytic treatment of pulmonary embolism (PE) is unknown., Objectives: This study investigated the long-term prognosis of patients with intermediate-risk PE and the effect of thrombolytic treatment on the persistence of symptoms or the development of late complications., Methods: The PEITHO (Pulmonary Embolism Thrombolysis) trial was a randomized (1:1) comparison of thrombolysis with tenecteplase versus placebo in normotensive patients with acute PE, right ventricular (RV) dysfunction on imaging, and a positive cardiac troponin test result. Both treatment arms received standard anticoagulation. Long-term follow-up was included in the third protocol amendment; 28 sites randomizing 709 of the 1,006 patients participated., Results: Long-term (median 37.8 months) survival was assessed in 353 of 359 (98.3%) patients in the thrombolysis arm and in 343 of 350 (98.0%) in the placebo arm. Overall mortality rates were 20.3% and 18.0%, respectively (p = 0.43). Between day 30 and long-term follow-up, 65 deaths occurred in the thrombolysis arm and 53 occurred in the placebo arm. At follow-up examination of survivors, persistent dyspnea (mostly mild) or functional limitation was reported by 36.0% versus 30.1% of the patients (p = 0.23). Echocardiography (performed in 144 and 146 patients randomized to thrombolysis and placebo, respectively) did not reveal significant differences in residual pulmonary hypertension or RV dysfunction. Chronic thromboembolic pulmonary hypertension (CTEPH) was confirmed in 4 (2.1%) versus 6 (3.2%) cases (p = 0.79)., Conclusions: Approximately 33% of patients report some degree of persistent functional limitation after intermediate-risk PE, but CTEPH is infrequent. Thrombolytic treatment did not affect long-term mortality rates, and it did not appear to reduce residual dyspnea or RV dysfunction in these patients. (Pulmonary Embolism Thrombolysis study [PEITHO]; NCT00639743)., (Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Purpose: Association estimates between baseline characteristics and outcomes are imprecise and inconsistent among extracorporeal cardiopulmonary resuscitation (ECPR) recipients following refractory out-of-hospital cardiac arrest (OHCA). This systematic review and meta-analysis aimed to investigate the prognostic significance of pre-specified characteristics for OHCA treated with ECPR., Methods: The Medline electronic database was searched via PubMed for articles published from January 2000 to September 2016. The electronic search was supplemented by scanning the reference lists of retrieved articles and contacting field experts. Eligible studies were historical and prospective cohort studies of adult patients undergoing ECPR following OHCA., Results: Fifteen primary studies were included, totaling 841 participants. The median prevalence of the primary outcome (i.e., short- or long-term survival for five studies and cerebral performance for ten studies) was 15% (range, 0-50%). The primary outcome was associated with an increased odds ratio of initial shockable cardiac rhythm (2.20; 95% confidence interval [CI], 1.30-3.72; P=0.003), shorter low-flow duration (geometric mean ratio, 0.90; 95% CI, 0.81-0.99; P=0.04), higher arterial pH value (difference, 0.12; 95% CI, 0.03-0.22; P=0.01) and lower serum lactate concentration (difference, -3.52mmol/L; 95% CI, -5.05 to -1.99; P<0.001). No significant association was found between the primary outcome and patient age (the odds of female gender and bystander CPR attempt., Conclusion: Observational evidence from published primary studies indicates that shorter low-flow duration, shockable cardiac rhythm, higher arterial pH value and lower serum lactate concentration on hospital admission are associated with better outcomes for ECPR recipients after OHCA., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

In chronic thromboembolic pulmonary hypertension (CTEPH), stenoses or obstructions of the pulmonary arteries due to organized thrombi can cause an elevation in pulmonary artery resistance, which in turn can result in pulmonary hypertension and progressive right heart failure. CTEPH can be cured surgically by pulmonary endarterectomy (PEA); however, patients deemed unsuitable for PEA due to distal lesion, advanced age, or comorbidities have a poor prognosis and limited treatment options. Recently, an alternative interventional strategy of balloon pulmonary angioplasty (BPA) reduces pulmonary artery hypertension in patients with CTEPH. Risk/benefit inbalance appear to be safe in experimented center. BPA can treat the lesions from lobar to subsegmental pulmonary arteries, improving functional and hemodynamic status for inoperable patients. This review highlights this recent progress., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Aims: Increased left ventricular wall thickness (LVWT) is a common finding in cardiology. It is not known how often hereditary transthyretin-related familial amyloid cardiomyopathy (mTTR-FAC) is responsible for LVWT. Several therapeutic modalities for mTTR-FAC are currently in clinical trials; thus, it is important to establish the prevalence of TTR mutations (mTTR) and the clinical characteristics of the patients with mTTR-FAC., Methods and Results: In a prospective multicentre, cross-sectional study, the TTR gene was sequenced in 298 consecutive patients diagnosed with increased LVWT in primary cardiology clinics in France. Among the included patients, median (25-75th percentiles) age was 62 [50;74]; 74% were men; 23% were of African origin; and 36% were in NYHA Class III-IV. Median LVWT was 18 (16-21) mm. Seventeen (5.7%; 95% confidence interval [CI]: [3.4;9.0]) patients had mTTR of whom 15 (5.0%; 95% CI [2.9;8.2]) had mTTR-FAC. The most frequent mutations were V142I (n = 8), V50M (n = 2), and I127V (n = 2). All mTTR-FAC patients were older than 63 years with a median age of 74 [69;79]. Of the 15 patients with mTTR-FAC, 8 were of African descent while 7 were of European descent. In the African descendants, mTTR-FAC median age was 74 [72;79] vs. 55 [46;65] years in non-mTTR-FAC (P < 0.001). In an adjusted multivariate model, African origin, neuropathy, carpal tunnel syndrome, electrocardiogram (ECG) low voltage, and late gadolinium enhancement (LGE) at cardiac-magnetic resonance imaging were all independently associated with mTTR-FAC., Conclusion: Five per cent of patients diagnosed with hypertrophic cardiomyopathy have mTTR-FAC. Mutated transthyretin genetic screening is warranted in elderly subjects with increased LVWT, particularly, those of African descent with neuropathy, carpal tunnel syndrome, ECG low voltage, or LGE., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.)

Treatment for pulmonary arterial hypertension (PAH) has been underpinned by single-agent therapy to which concomitant drugs are added sequentially when pre-defined treatment goals are not met.This retrospective analysis of real-world clinical data in 97 patients with newly diagnosed PAH (86% in New York Heart Association functional class III-IV) explored initial dual oral combination treatment with bosentan plus sildenafil (n=61), bosentan plus tadalafil (n=17), ambrisentan plus tadalafil (n=11) or ambrisentan plus sildenafil (n=8).All regimens were associated with significant improvements in functional class, exercise capacity, dyspnoea and haemodynamic indices after 4 months of therapy. Over a median follow-up period of 30 months, 75 (82%) patients were still alive, 53 (71%) of whom received only dual oral combination therapy. Overall survival rates were 97%, 94% and 83% at 1, 2 and 3 years, respectively, and 96%, 94% and 84%, respectively, for the patients with idiopathic PAH, heritable PAH and anorexigen-induced PAH. Expected survival rates calculated from the French equation for the latter were 86%, 75% and 66% at 1, 2 and 3 years, respectively.Initial combination of oral PAH-targeted medications may offer clinical benefits, especially in PAH patients with severe haemodynamic impairment., (Copyright ©ERS 2016.)

Recent therapeutic trials regarding the management of acute heart failure (AHF) failed to demonstrate the efficacy of newer therapeutic modalities and agents. Low- versus high-dose and continuous administration of furosemide were shown not to matter. Ultrafiltration was not found to be more efficacious than sophisticated diuretic therapy including dose-adjusted intravenous furosemide and metolazone. Dopamine and nesiritide were not shown to be superior to current therapy. Tezosentan and tovalptan had no effect on mortality. The development of rolofylline was terminated due to adverse effect (seizures). Lastly, preliminary experience with serelaxin indicates a mortality improvement at six months that remains to be confirmed. The disappointing findings of these recent trials may reflect the lack of efficacy of newer therapeutic modalities and agents. Alternatively the disappointing findings of these recent trials may be in part due to methodological issues. The AHF syndrome is complex with many clinical phenotypes. Failure to match clinical phenotypes and therapeutic modalities is likely to be partly responsible for the disappointing findings of recent AHF trials., (© The European Society of Cardiology 2014.)

Background: Because neurological failure is the most frequent extra-cardiac complication in Infectious Endocarditis (IE), a brain computerised tomography (CT) scan is usually performed. The benefits of magnetic resonance imaging (MRI) have not been clearly established. This study aims to clarify the prevalence and type of cerebral lesions in IE detected using MRI and to compare them with those detected using CT scans., Methods: In the Grenoble University Hospital, patients diagnosed with definite or possible endocarditis according to Duke's criteria were screened from 2010-2012. Brain CT and MRI were performed as soon as possible after diagnosis., Results: Of the 62 patients with IE who underwent at least one cerebral imaging within 3 weeks of diagnosis, Streptococcus (29) and Staphylococcus (14) were the main micro-organisms present. Twenty-eight (45%) patients underwent cardiac surgery. Eight (13%) died before discharge. Twenty (32%) had neurological symptoms. A brain CT-scan was performed on 53 (85%) patients and a MRI was performed on 43 (69%) patients. CT was pathological in 26 (49%) patients, whereas 32 (74%) MRI demonstrated abnormalities. The MRI lesions were classified as follows: ischaemia (48%), microbleeds (34%), haemorrhages (16%), abscesses (9%) and microbial aneurysms (4%). Of the 37 patients who underwent both MRI and CT examinations, ischaemia (48% vs 35%) and microbleeds (34%) demonstrated the difference between the two imaging methods., Conclusion: Through the early diagnosis of cerebral damage, even in asymptomatic cases, MRI may have a role in the IE management, influence any surgical decision and assist in prognosis assessment.

Background: Patients treated for pulmonary arterial hypertension (PAH) frequently receive vitamin K antagonists (VKAs) for PAH or validated indications (such as atrial fibrillation or venous thromboembolism). In these latter indications, VKAs are challenged by direct oral anticoagulants (DOAs). Decreased dosage of DOAs has been proposed in patients at risk of bioaccumulation., Objectives: We aimed to evaluate the frequency of bioaccumulation risks in patients treated with PAH-targeted therapy, particularly regarding the presence of validated indications., Methods: We conducted a retrospective study in three different PAH referral centers. All patients receiving PAH-targeted therapy were classified according to demographics, prescription and indications of VKAs, and the presence of major bioaccumulation risk factors (renal failure, low body weight, strong P-glycoprotein or cytochrome P3A4 inhibitors)., Results: Two hundred and thirty-nine of the 366 patients included received VKAs, 94 for validated indications. At least one major risk factor was found in 231 (63.1%) of the whole study population, and in 54 (57.4%) of the patients anticoagulated for a validated indication. No specific patient phenotype could be individualized., Conclusions: About 1 in 2 patients treated with PAH therapy has at least one of the three major risk factors for DOA bioaccumulation. DOAs in the PH setting could be associated with bioaccumulation and should be individualized, mainly in patients with confirmed indication., (© 2016 S. Karger AG, Basel.)

Purpose: Mild therapeutic hypothermia (TH) is recommended as soon as possible after the return of spontaneous circulation to improve outcomes after out-of-hospital cardiac arrest (OHCA). Preclinical data suggest that the benefit of TH could be increased if treatment is started during cardiac arrest. We aimed to study the impact of intra-arrest therapeutic hypothermia (IATH) on neurological injury and inflammation following OHCA., Methods: We conducted a 1:1 randomized, multicenter study in three prehospital emergency medical services and four critical care units in France. OHCA patients, irrespective of the initial rhythm, received either an infusion of cold saline and external cooling during cardiac arrest (IATH group) or TH started after hospital admission (hospital-cooling group). The primary endpoint was neuron-specific enolase (NSE) serum concentrations at 24 h. Secondary endpoints included IL-6, IL-8, and IL-10 concentrations, and clinical outcome., Results: Of the 245 patients included, 123 were analyzed in the IATH group and 122 in the hospital-cooling group. IATH decreased time to reach temperature ≤ 34 °C by 75 min (95% CI: 4; 269). The rate of patients admitted alive to hospital was not different between groups [IATH n = 41 (33%) vs. hospital cooling n = 36 (30%); p = 0.51]. Levels of NSE and inflammatory biomarkers were not different between groups [median NSE at 24 h: IATH 96.7 μg/l (IQR: 49.9-142.8) vs. hospital cooling 97.6 μg/l (IQR: 74.3-142.4), p = 0.64]. No difference in survival and cerebral performance were found at 1 month., Conclusions: IATH did not affect biological markers of inflammation or brain damage or clinical outcome.

Purpose: To determine the value of cardiac MRI for the monitoring and the prognosis of patients with acute myocarditis. Cardiac MRI plays an increasingly important role in the diagnosis of acute myocarditis. However, it is less established as a prognostic tool, which requires specific postprocessing of images., Materials and Methods: In a prospective pilot study, we assessed the prognostic value of the evolution in a simplified visual quantitative score (SQS) of late gadolinium enhancement (LGE) between initial hospitalization and 3 months later. The prognostic value was assessed at 1 year using a combination of death, heart transplant, and confirmed recurrence as main outcome., Results: Twenty-eight patients were included in this study. A significant correlation was found between LGE measured by SQS and planimetry (r = 0.95, P < 0.001). Intraobserver and interobserver reproducibilities were good for SQS (ICC = 0.95 [95% CI: 0.86 to 0.98], and 0.94 [95% CI: 0.84 to 0.98], respectively). At initial hospitalization, patient characteristics between the two groups were similar. Patients with stable or increased SQS suffered more frequently from clinical outcome events than patients with a decrease in SQS (P = 0.02)., Conclusion: Monitoring of the evolution of LGE using a simple visual score is of interest to identify patients at risk of pejorative prognosis after acute myocarditis., (© 2013 Wiley Periodicals, Inc.)

Background: The role of fibrinolytic therapy in patients with intermediate-risk pulmonary embolism is controversial., Methods: In a randomized, double-blind trial, we compared tenecteplase plus heparin with placebo plus heparin in normotensive patients with intermediate-risk pulmonary embolism. Eligible patients had right ventricular dysfunction on echocardiography or computed tomography, as well as myocardial injury as indicated by a positive test for cardiac troponin I or troponin T. The primary outcome was death or hemodynamic decompensation (or collapse) within 7 days after randomization. The main safety outcomes were major extracranial bleeding and ischemic or hemorrhagic stroke within 7 days after randomization., Results: Of 1006 patients who underwent randomization, 1005 were included in the intention-to-treat analysis. Death or hemodynamic decompensation occurred in 13 of 506 patients (2.6%) in the tenecteplase group as compared with 28 of 499 (5.6%) in the placebo group (odds ratio, 0.44; 95% confidence interval, 0.23 to 0.87; P=0.02). Between randomization and day 7, a total of 6 patients (1.2%) in the tenecteplase group and 9 (1.8%) in the placebo group died (P=0.42). Extracranial bleeding occurred in 32 patients (6.3%) in the tenecteplase group and 6 patients (1.2%) in the placebo group (P<0.001). Stroke occurred in 12 patients (2.4%) in the tenecteplase group and was hemorrhagic in 10 patients; 1 patient (0.2%) in the placebo group had a stroke, which was hemorrhagic (P=0.003). By day 30, a total of 12 patients (2.4%) in the tenecteplase group and 16 patients (3.2%) in the placebo group had died (P=0.42)., Conclusions: In patients with intermediate-risk pulmonary embolism, fibrinolytic therapy prevented hemodynamic decompensation but increased the risk of major hemorrhage and stroke. (Funded by the Programme Hospitalier de Recherche Clinique in France and others; PEITHO EudraCT number, 2006-005328-18; ClinicalTrials.gov number, NCT00639743.).

Aims: This systematic review aimed to investigate the diagnostic accuracy of combined cardiac troponin (cTn) and copeptin assessment in comparison to cTn alone for early rule-out of acute myocardial infarction (AMI)., Methods: Primary studies were eligible if they evaluated diagnostic accuracy for cTn with and without copeptin in patients with symptoms suggestive of AMI. AMI was defined according to the universal definition, using detection of cTn as a marker for myocardial necrosis. Eligible studies were identified by searching electronic databases (Medline, EMBASE, Science Citation Index Expanded, CINAHL, Pascal, and Cochrane) from inception to March 2013, reviewing conference proceedings and contacting field experts and the copeptin manufacturer., Results: In 15 studies totalling 8740 patients (prevalence of AMI 16%), adding copeptin improved the sensitivity of cTn assays (from 0.87 to 0.96, p=0.003) at the expense of lower specificity (from 0.84 to 0.56, p<0.001). In 12 studies providing data for 6988 patients without ST-segment elevation, the summary sensitivity and specificity estimates were 0.95 (95% CI 0.89 to 0.98) and 0.57 (95% CI 0.49 to 0.65) for the combined assessment of cTn and copeptin. When a high-sensitivity cTnT assay was used in combination with copeptin, the summary sensitivity and specificity estimates were 0.98 (95% CI 0.96 to 1.00) and 0.50 (95% CI 0.42 to 0.58)., Conclusion: Despite substantial between-study heterogeneity, this meta-analysis demonstrates that copeptin significantly improves baseline cTn sensitivity. Management studies are needed to establish the effectiveness and safety of measuring copeptin in combination with high-sensitivity cTnT for early rule-out of AMI without serial testing.

Objective: We propose a non-invasive method for diagnosing post-capillary pulmonary hypertension (PH group 2). We evaluated pulmonary capillary wedge pressure (PCWP) by studying the left atrium (LA) on thoracic ECG-gated CT compared with right heart catheterisation (RHC)., Methods: We retrospectively studied 54 patients with suspected PH or followed for PH who underwent thoracic ECG-gated CT and RHC within 15 days. The diagnostic accuracy of CT morphological and functional data of the LA for the detection of PCWP >15 mmHg, evaluated by two independent readers, was assessed using correlation and receiver-operating characteristic (ROC) analysis., Results: Interobserver agreement was high (r = 0.97-0.99). Correlations were found between PCWP and the morphological criteria of the LA such as anteroposterior diameter at 0 % of the R-R interval (r = 0.70, P ≤ 0.001) as well as at 40 % (r = 0.69, P ≤ 0.001). ROC curves constructed with a threshold value of PCWP > 15 mmHg showed an area under the curve between 0.88 and 0.91. Significant correlations were found between PCWP and functional criteria of the LA, including distensibility (r = -0.49, P ≤ 0.001) and ejection fraction (r = -0.58, P ≤ 0.001)., Conclusion: Thoracic ECG-gated CT in a PH workup helps distinguish between pre- and post-capillary PH., Key Points: • Computed tomography may help differentiate the various types of pulmonary hypertension (PH). • Post-capillary PH group 2 is due to left heart disease. • Right heart catheterisation is used to separate pre- and post-capillary PH. • Left atrium anteroposterior diameter measured on CT is of value. • ECG-gated CT helps clinicians to assess patients with PH non-invasively.

Peripartum cardiomyopathy (PPCMP) is a dilated and hypokinetic cardiomyopathy occurring during pregnancy or after delivery, with an estimated incidence between 1/1000 and 1/4000 births. It has been defined as a new onset of heart failure in the month preceding or following delivery, without demonstrated aetiology nor previously known heart disease, and with echocardiographic evidences of left ventricular (LV) dysfunction (LV ejection fraction<0.45). It's a multifactorial disease, immunologic, hormonal, and possibly viral mechanisms playing a determinant pathophysiological role. The classical clinical presentation is a rapid and unexpected onset of heart failure in a previously healthy woman, echocardiography being the key examination for positive and differential diagnosis, prognostication, therapeutic decision-making, and follow-up. The potential severity of PPCMP, and its unpredictable evolution in the first days following diagnosis, require that patients be referred to a tertiary care centre with a high skill in intensive cardiology care. Therapeutic management of PPCMP does not offer any specificity when compared to other causes of acute or chronic heart failure (from diuretics to extracorporeal life support), except for ACE-inhibitors, that are contraindicated before delivery. The high incidence of thrombo-embolic complications observed in the disease requires however rapid and curative anticoagulation, and immuno-suppressive treatment has been proposed in fulminant and highly inflammatory presentation, but its efficacy remains controversial. Very recently, promising results have been reported with bromocriptin-a prolactin secretion inhibitor-for reducing 6-month morbidity and mortality, but these findings have to be confirmed in larger scale randomised trials. As for the long-term evolution, approximately half of the patients will heal, while half of the women will keep some degree of LV dysfunction, 25% of them developing moderate to severe chronic heart failure., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Introduction and Objectives: To compare acute myocardial infarction patients with or without congestive heart failure in the French FAST-MI registry., Methods: The French FAST-MI registry included 374 centers and 3059 patients over a 1-month period at the end of 2005, with 1-year follow-up. Among this population, patients with at least one congestive heart failure criterion constituted group 1 (n=1149; 37.5%) and were compared to patients without congestive heart failure (group 2, n=1910; 62.5%). The congestive heart failure patients were further divided according to presence of both beta-blockers and antagonists of the renin-angiotensin-aldosterone system at hospital discharge (n=511) or not (n=498), in order to assess the real-world clinical importance of recommended medications., Results: Overall in-hospital and 1-year mortality rates were 3.4% and 13.2%, respectively. In hospital survivors, presence of congestive heart failure was associated with increased mortality (adjusted hazard ratio: 1.55; 95% confidence interval, 1.10-2.17; P=.01). Survival was higher in patients without congestive heart failure, compared with congestive heart failure patients receiving or not recommended medications (P<.001). Congestive heart failure patients receiving neither renin-angiotensin-aldosterone system blockers nor beta-blockers (adjusted hazard ratio: 1.66; 95% confidence interval, 1.08-2.55; P=.02) had a significantly higher risk of death than patients receiving both classes of medications (adjusted hazard ratio: 1.16; 95% confidence interval, 0.82-1.64; not statistically significant). Patients receiving only one of the recommended classes had an intermediate risk (adjusted hazard ratio: 1.47; 95% confidence interval, 1.04-2.07; P=.03)., Conclusions: Patients admitted for acute myocardial infarction with congestive heart failure criteria are still at very high risk of mortality. When receiving major recommended medications, they presented with significantly reduced mortality rates. Additional efforts should therefore be made to encourage the prescription of recommended medications in acute myocardial infarction patients with congestive heart failure., (Copyright © 2011 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.)

Objectives: Current guidelines recommend utilization of prehospital emergency medical services (EMSs) by patients with ST-elevation myocardial infarction (STEMI). The aims of this study were to estimate the percentage of inappropriate initial dispatcher decisions and determine their impact on delays in reperfusion therapy for EMS users with STEMI., Methods: As part of a prospective regional registry of patients with STEMI, we analyzed the original data for 245 patients who called a university hospital-affiliated EMS call center in France. The primary study outcome was time to reperfusion therapy calculated from the documented date and time of the first patient call., Results: The initial EMS dispatcher's decision was appropriate (ie, dispatching a mobile intensive care unit staffed by an emergency or critical care physician) for 171 (70%) patients and inappropriate for 74 (30%) patients. Inappropriate decisions included referring the patient to a family physician (n = 59), providing medical advice (n = 9), and dispatching an ambulance (n = 6). Inappropriate initial decisions resulted in increased median time to reperfusion for 140 patients receiving fibrinolysis (95 vs 53 minutes; P < .001) and 91 patients undergoing primary percutaneous coronary intervention (170 vs 107 minutes; P < .001). In-hospital mortality was not different between the 2 study groups (6.8% vs 9.9%; P = .42)., Conclusion: The initial dispatcher's decision is inappropriate for 30% of EMS users with STEMI and results in substantial delays in time to reperfusion therapy. Accuracy of telephone triage should be improved for patients who activate EMSs in response to symptoms suggestive of acute coronary syndrome., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: Compliance with guidelines for the management of ST-segment elevation myocardial infarction (STEMI) may be difficult in hard-to-access areas., Aims: to analyse the characteristics, management and outcome of STEMIs occurring at altitude in the French Alps and managed by mobile medical emergency units., Methods: From January 2006 to December 2008, from the prospective RESURCOR registry, 114 patients with a STEMI of less than 12 hours' duration, occurring in a ski resort or at high altitude and managed by the RESURCOR care system, were identified. Baseline characteristics, treatments and in-hospital outcomes were analysed., Results: Ninety-three per cent of patients were men; the mean age was 57 years. STEMIs occurred during or less than 1 hour after physical activity in 76.3% of cases (mainly during or after alpine/cross-country skiing). Killip class greater or equal to 2 and cardiac arrest were observed in 35% and 7.9% of cases, respectively. Fifty-two (45.6%) patients underwent thrombolysis and 62 (54.4%) had percutaneous coronary intervention (PCI). Median delays were: first call to treatment, 82 min (17-230 min); symptoms to treatment, 165 min (52-770 min). All delays were significantly longer for PCI than for thrombolysis. First call to treatment delay was less than 120 min in 98.1% of patients who underwent thrombolysis and in 51.6% who had PCI (P<0.0001). In-hospital survival was 96.5%., Conclusion: Altitude STEMIs happen mainly during sporting activities. Clinical presentation is often severe, but an emergency coronary care network allows rapid reperfusion. These findings emphasize the need for an efficient network for STEMI management in geographically difficult-to-access areas., (2010 Elsevier Masson SAS. All rights reserved.)

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 reconsidered and re-assessed during follow up.

Background: Extracorporeal life support (ECLS) is a circulatory assistance device that is increasingly used in adults undergoing cardiopulmonary arrest (CPA) or hemodynamic collapse when conventional therapies fail., Objectives: To assess the feasibility and outcomes of 100 consecutive arteriovenous percutaneous ECLS procedures at the Grenoble University Hospital between January 2002 and September 2007., Methods: Monocentric descriptive registry with one-year prospective follow-up., Results: An ECLS device was successfully used in 93% of patients. Its indication was cardiogenic shock in 50% of the cases, CPA in 38% of the cases and unsuccessful weaning of cardiopulmonary bypass (CPB) after cardiothoracic surgery in 12% of the cases. Direct complications of ECLS were observed in 56% of patients, the most frequent being hemorrhage at the intravenous puncture site requiring red blood cell transfusions (26%), and lower limb ischemia (19%). Weaning from ECLS was achieved in 33 patients (44% cardiogenic shocks, 13% CPAs, 50% CPB weaning failures) and 20 patients were discharged from the hospital (26% cardiogenic shocks, 10.5% CPAs and 25% CPB weaning failures). All are still living without any serious sequelae (mean follow-up period of 16.8 months)., Conclusion: The use of ECLS in CPA patients, especially with cardiogenic shock, is feasible with satisfactory survival rates, given the extreme severity of their initial state.

Objective: To assess the prevalence, clinical presentation, therapeutic management and medium-term prognosis of spontaneous coronary artery dissection (SCAD)., Methods: Over a 5-year period, and out of a cath-lab database of 11,605 files, 23 cases of SCAD were confirmed by re-analysis of angiograms. Therapeutic management and in-hospital outcome were obtained from medical files and prospective follow-up was performed., Results: The population consisted of 17 women and 6 men (mean age 45 years). A total of 83% of men had >/=2 cardiovascular risk factors versus only 35% of women (p=0.07). Acute coronary syndrome (ACS) was the clinical presentation in 21 cases (ST+ 61%). The prevalence of SCAD was: 0.2% in the whole population (men: 0.07%, women: 0.6%, p<0.001), and 8.7% and 10.8% in women <50 years with ACS and ST+ ACS, respectively. Left and right coronary arteries were involved in 20 (87%) and 3 (13%) cases, respectively. Coronary stenting alone was performed in 8 (35%), bypass surgery alone in 2 (9%), and both in 3 (13%) cases. Ten patients (44%) were medically managed. One patient died during hospitalization. At 1-year follow-up (100%), 77% of discharged patients were event-free. One patient died, four experienced heart failure, and none had angina or new ACS., Conclusion: SCAD is observed in as much as 1 out 10 women <50 years presenting with ACS. After immediate coronary angiography, medical therapy is the chosen strategy in half of cases. Most patients who survive the acute phase are free from coronary events at 1 year.

Objective: Patent foramen ovale (PFO) is frequent but rarely associated with platypnea-orthodeoxia syndrome and with no pulmonary hypertension., Materials and Methods: We performed a retrospective analysis of 12 consecutive patients with hypoxemia and PFO without increased pulmonary arterial pressure. The study was conducted at a single-center university hospital. We analyzed the patients' clinical records, gas exchange, contrast echocardiography studies, and survival., Results: Twelve patients, aged 73.1 +/- 9.5 years, were diagnosed with PFO between 1993 and 2005. All patients experienced dyspnea and/or hypoxemia. Six right hemidiaphragmatic elevations were observed on radiography. The shunt was apparent in three patients using transthoracic echocardiography and in all patients using transesophageal echocardiography. The mean follow-up was 27.6 +/- 32.9 months. The PFO was closed in eight patients, six via percutaneous approach and two via surgery., Conclusion: PFO may be patent and responsible for hypoxemia without pulmonary hypertension. This condition is easily recognized with transesophageal echocardiography, leading in most cases to a percutaneous closure resulting in a dramatic correction of hypoxemia.

A 48 year old man was admitted to the intensive care unit with septicaemic shock associated with febrile jaundice and anuric renal failure. Within hours, he developed cardiogenic shock with multi-organ failure due to an acute myocarditis refractory to catecholamines and requiring intra-aortic balloon pumping. The diagnosis was an ictero-haemorrhagic leptospirosis, the outcome of which was finally favourable. Myocarditis is an underestimated complication of leptospirosis because it is often symptomless. The main signs are arrhythmias, conduction defects and ST-T wave abnormalities which have little clinical expression. The disease may progress and is sometimes fatal. Leptospirosis myocarditis should therefore be carefully considered because of its potential severity and its reversibility with appropriate antibiotic therapy and also the necessity of initial management in a specific infrastructure.

Background: Total arterial and off-pump revascularization are increasingly used in coronary artery bypass grafting. This study describes our experience with the exclusive use of both left internal thoracic artery and gastroepiploic artery by means of a median sternotomy, with and without cardiopulmonary bypass, in a subgroup of patients with two-vessel disease., Methods: From January 1995 to July 2000, 171 consecutive patients were reviewed in a prospective database. Ninety-one patients underwent coronary artery bypass grafting without cardiopulmonary bypass (group A), and 80 patients were operated on under cardiopulmonary bypass with aortic cross-clamp and cardioplegia (group B)., Results: Patient data were similar in both groups except for the Euroscore (mean; 3.4+/-6.1, group A versus 2.5+/-4.5, group B; Euroscore > 6: 26.4%, group A versus 10%, group B; p < 0.05) and ejection fraction (mean, 54.6%+/-15.8%, group A versus 63.1%+/-12.7%, group B; p < 0.001). Severe aortic calcification was present in 6 group A patients, versus no patient in group B. Operative time was shorter in group A (185 versus 213 minutes, p < 0.0001), with less distal anastomoses (2.26 versus 2.5, p < 0.05). Conversion to cardiopulmonary bypass occurred in 1 patient, who was excluded from the study. Bleeding was higher in group A (852.6+/-288 mL versus 712.4+/-274 mL, p < 0.05), but transfusion was similar in both groups. Atrial fibrillation, postoperative inotropic support, and hospital stay were similar in both groups. Myocardial infarction was less frequent in group A (1 versus 4). Postoperative intraaortic balloon pump was used in 2 patients (group B). One patient died (group A) and 1 had an embolic stroke (group B). After discharge, 2 more patients died (group A, day 91; group B day 141), and 1 patient suffered an embolic stroke (group B). One patient in each group presented with dysfunction of the gastroepiploic artery graft requiring successful percutaneous transluminal angioplasty on the right posterolateral artery., Conclusions: These results suggest that off-pump coronary artery bypass grafting using the left internal thoracic artery and gastroepiploic artery is safe even in high-risk patients. This approach allows an absolute no-touch technique of the aorta.

Aneurysm of the ampulla of Galien is a rare but serious cause of cardiac failure in the neonate or child. The diagnosis is suspected on finding a continuous murmur on auscultation of the skull. It is an intracranial arterio-venous malformation which is sometimes responsible for a very important shunt between the arterial (carotid or vertebral) and venous systems. The vital and neurological prognosis of these children is classically very poor. The authors report the cases of the last three consecutive children aneurysms of the ampulla of Galien followed up at Grenoble Hospital in the last five years. The clinical presentations were very different, corresponding to the usual three forms described in the literature: a reputedly very severe form with cardiac failure at the 3rd day of life requiring multiple percutaneous embolisations; a neonatal form revealed by an isolated macrocranium; and a form diagnosed late (4.5 years) in the investigation of effort dyspnoea which regressed after two sessions of embolisation. All three children are alive and have normal psychomotor development for their age. A multidisciplinary approach involving neonatal physicians, paediatric cardiologists and neurologists, with complementary non-invasive investigations such as transfontanellar ultrasonography, electroencephalography, MRI and echocardiography are necessary to optimise the management and limit the neurological sequellae in children with this type of malformation. Cerebral arteriography and percutaneous embolisation under general anaesthesia by a trained team gave very good medium-term results in two of these cases, lightening the usual pessimism surrounding this condition.

Cardiac myxoma, a relatively common condition in adults, is exceptionally rare in childhood. It is usually observed in the left atrium. The condition may be latent and a chance echocardiographic finding or present with arrhythmias, intermittent mitral obstruction or embolic phenomena. The authors report the case of an 11-year old child with no previous medical history, admitted to hospital after sudden right hemiplegia due to an ischaemic cerebrovascular accident. The investigation of a cardiac embolic abnormality led to the finding of a very large left ventricular tumour inserted into the mitral annulus and prolapsing into the left ventricular outflow tract, the histological examination of which confirmed the diagnosis of myxoma. Cardiac myxomas in children seem to have a much more variable site of implantation than in adults, especially in the right heart in infancy. Treatment is always surgical. Echocardiography should be requested early in children presenting with a murmur of recent origin, malaises, or unexplained inflammatory syndromes, and would seem to be the only means of avoiding the severe embolic complications of these histologically benign tumours.