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Aims Outcomes in pulmonary hypertension (PH) are related to right ventricular (RV) function and remodelling. We hypothesized that changes in RV function and especially area strain (AS) could provide incremental prognostic information compared to the use of baseline data only. We therefore aimed to assess RV function changes between baseline and 6-month follow-up and evaluate their prognostic value for PH patients using 3D echocardiography. Methods and results Ninety-five PH patients underwent a prospective longitudinal study including ESC/ERS guidelines prognostic assessment and 3D RV echocardiographic imaging at baseline and 6-month follow-up. Semi-automatic software tracked the RV along the cycle, and its output was post-processed to extract 3D deformation patterns. Over a median follow-up of 24.8 (22.1–25.7) months, 21 patients died from PH or were transplanted. Improvements in RV global AS were associated with stable or improving clinical condition as well as survival free from transplant (P < 0.001). The 3D deformation patterns confirmed that the most significant regional changes occurred within the septum. RV global AS change over 6-month by +3.5% identifies patients with a 3.7-fold increased risk of death or transplant. On multivariate COX analysis, changes in WHO class, BNP, and RV global AS were independent predictors of outcomes. Besides, the combination of these three parameters was of special interest to identify high-risk patients [HR 11.5 (1.55–86.06)]. Conclusion Changes in RV function and especially changes in 3D RV AS are of prognostic importance. Our study underlines that assessing such changes from baseline to follow-up is of additional prognostic value for PH patients. Clinical Trial Registration http://clinicaltrials.gov/ct2/show/NCT02799979

Purpose: Balloon Pulmonary Angioplasty (BPA) is a constantly improving technique option for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH). To assess pulmonary artery pressures (PAP) and BNP changes immediately after Balloon Pulmonary Angioplasty (BPA) procedures.Methods: We implemented a rigorous protocol for measuring PAP just before, then immediately after, the BPA session and BNP before, then the following morning. In a sub-group of 16 procedures we also performed an endothelin-1 (ET-1) assay before and after each procedure.Results: out of 81 BPA (32 patients), sPAP before and after were 65 mmHg [59-74] versus 75 mmHg [64-80]: P=0.005; 38 mmHg [33-43] vs 43 mmHg [36-48] for mPAP: P P=0.35. In all, PAP rose after 81% of the procedures. Likewise, BNP showed a significant increase the day after BPA: 127pg/mL [80-262] versus 116 pg/mL [58-215]: P P=0.021. Conclusion: PAP and BNP are very often transiently increased immediately after BPA. These paradoxical changes which may participate in common BPA procedure complications are not predictive of the long-term treatment benefits. Assessment of the BPA result should not be made within hours of a BPA session. The reason for the paradoxical increase in PAP and BNP is not established, but ET-1 secretion does not appear to be responsible.

Association between Hydroxychloroquine (HCQ) and Azithromycin (AZT) is under evaluation for patients with lower respiratory tract infection (LRTI) caused by the Severe Acute Respiratory Syndrome (SARS‐CoV‐2). Both drugs have a known torsadogenic potential, but sparse data are available concerning QT prolongation induced by this association. Our objective was to assess for COVID‐19 LRTI variations of QT interval under HCQ/AZT in patients hospitalized, and to compare manual versus automated QT measurements. Before therapy initiation, a baseline 12 lead‐ECG was electronically sent to our cardiology department for automated and manual QT analysis (Bazett and Fridericia’s correction), repeated 2 days after initiation. According to our institutional protocol (Pasteur University Hospital), HCQ/AZT was initiated only if baseline QTc ≤ 480ms and potassium level > 4.0 mmol/L. From March 24th to April 20th 2020, 73 patients were included (mean age 62 ± 14 y, male 67 %). Two patients out of 73 (2.7 %) were not eligible for drug initiation (QTc ≥ 500ms). Baseline average automated QTc was 415 ± 29 ms and lengthened to 438 ± 40 ms after 48 hours of combined therapy. The treatment had to be stopped because of significant QTc prolongation in 2 out of 71 patients (2.8 %). No drug‐induced life‐threatening arrhythmia, nor death was observed. Automated QTc measurements revealed accurate in comparison with manual QTc measurements. In this specific population of inpatients with COVID‐19 LRTI, HCQ/AZT could not be initiated or had to be interrupted in less than 6% of the cases.

INTRODUCTION Right ventricular (RV) contractile reserve reflects the ability of the RV to adapt to elevated afterload. RV functional response to exercise is challenging but could represent an important prognostic factor, especially in pulmonary arterial hypertension (PAH) patients. We aimed, using exercise stress echocardiography (ESE), to assess different RV contractile reserve evaluation methods in a cohort of PAH patients and controls. METHODS We prospectively included 12 patients with PAH and 12 healthy volunteers. An ESE (using tilt-table ergometer) was performed in all patients to assess RV function at rest and under peak exercise. Changes in these parameters during exercise were calculated to quantify the RV contractile reserve. 3D RV function as well as peak systolic strain, pulmonary pressures, TAPSE, pulmonary TVI and pulmonary output (using the right ventricular outflow tract diameter) were assessed in all patients. RESULTS Our patient group was composed by PAH patients, 61.5 ± 14.8 years; mean age of our control group was 29.33 ± 5.5 years. PAH patients achieved an exercise with a mean workload of 69.17 ± 26.4 Watts. There was no complication after the exercise test in all patients. Change in TAPSE was not significantly different between patients and controls (p = 0.17), whereas change in pulmonary TVI, pulmonary output and RV peak systolic strain was highly discriminant (respectively p = 0.03, p = 0.009 and p = 0.0009). Regarding RV contractile reserve parameters, RV end-systolic pressure area ratio (peak/rest) was not statistically different between controls and patients (p = 0.14) whereas change in TAPSE/sPAP, RV peak strain/sPAP, 3D RV EF/sPAP were significantly different (p = 0.005, p= 0.0008, p = 0004). CONCLUSION Changes in pulmonary output, RV peak systolic strain as well as changes in TAPSE/sPAP but mainly RV peak strain/sPAP, 3D RV EF/sPAP represent consistent and feasible tools to assess RV contractile reserve. echocardiographic parameters PAH (n = 12) Healthy Controls (n = 12) p value sPAP at rest (mmHg) 40.91 ± 10.7 15.42 ± 4.1 Abstract P941 Figure. echography (rest/peak) PAH patient

Aims Survival in pulmonary arterial hypertension (PAH) and Eisenmenger syndrome (ES) relates to right ventricular (RV) function. Little is known about differences of ventricular function between ES patients and those suffering from other PAH aetiologies. In this study, we compared global ventricular function assessed by speckle-tracking in adult patients with ES, other PAH aetiologies, or healthy controls; and assessed the relationship between ventricular function and survival. Methods and results We performed a prospective cohort study recruiting 83 adult PAH patients (43 ES and 40 other PAH aetiologies patients) and 37 controls between March 2011 and June 2015. Patients with complex congenital heart disease were excluded. Fifty-three patients (63.9%) were in NYHA functional class ≥III at baseline and 60 (72.3%) were on advanced therapies. Mean RV peak longitudinal strain was −16.3 ± 7% in ES, lower compared with healthy controls ( P

Aims Right ventricular (RV) function assessment is crucial in congenital heart disease patients, especially in atrial septal defect (ASD) and repaired Tetralogy of Fallot (TOF) patients with pulmonary regurgitation (PR). In this study, we aimed to analyse both 3D RV shape and deformation to better characterize RV function in ASD and TOF-PR. Methods and results We prospectively included 110 patients (≥16 years old) into this case–control study: 27 ASD patients, 28 with TOF, and 55 sex- and age-matched healthy controls. Endocardial tracking was performed on 3D transthoracic RV echocardiographic sequences and output RV meshes were post-processed to extract local curvature and deformation. Differences in shape and deformation patterns between subgroups were quantified both globally and locally. Curvature highlights differences in RV shape between controls and patients while ASD and TOF-PR patients are similar. Conversely, strain highlights differences between controls and TOF-PR patients while ASD and controls are similar [global area strain: −31.5 ± 5.8% (controls), −34.1 ± 7.9% (ASD), −24.8 ± 5.7% (TOF-PR), P Conclusion Chronic RV volume loading results in similar RV shape remodelling in both ASD and TOF patients while strain analysis demonstrated that RV strain is only reduced in the TOF group. This suggests a fundamentally different RV remodelling process between both conditions.

Background RV function assessment is crucial in congenital heart disease (CHD) patients, especially in atrial septal defect (ASD) and tetralogy of Fallot (TOF) patients. Indeed, prognosis substantially differs between TOF and ASD and little is known about 3D deformation and shape in RV volume overload. Purpose To assess Right ventricular (RV) remodeling differences between ASD, TOF patients and controls. Methods We prospectively included 55 patients (older than 16 yo) into this case-control study: 19 patients with an ASD (and left-to-right shunt), 15 with TOF and 21 healthy controls. 3D transthoracic RV echocardiographic sequences were acquired. Myocardial tracking was performed by a semi-automatic software. Output RV meshes including spatial correspondences were post-processed to temporally align the data and extract local deformation. Global and local statistics provided deformation and shape patterns for each subject. Results No difference in RV strain was found between ASD patients and controls. There was a trend toward increased longitudinal strain in patients with small ASD. TOF patients had lower RV area and longitudinal strain, especially in the inferior, lateral wall and in the trabecular septum. TOF patients had predominant circumferential over longitudinal strain. Using curvature indices, differences in RV shape were highlighted between controls and RV volume overload patients whereas there was almost no difference between ASD and TOF patients regarding RV shape, except at the septum. RV inferior and lateral curvatures were strongly correlated to QRS duration ( Fig. 1 ). Conclusion Volume overload alters RV shape to the same extent in ASD and TOF whereas it has differential effects on strain. In ASD patients the strain is relatively preserved whereas TOF patients had particularly impaired longitudinal strain. Such detailed analyses of strain and shape could help refine RV function assessment in particular in CHD patients.

Introduction Survival in pulmonary arterial hypertension (PAH) relates to right ventricular (RV) function. Whereas prognosis differs widely between PAH associated with congenital heart disease (CHD) and other causes of PAH, only little is known about differences in RV function. Purpose We aimed at comparing RV function assessed by 3D-speckle-tracking in patients with CHD-PAH, other PAH aetiologies and healthy controls; and assess the relationship between ventricular function and prognostic parameters. Methods We performed a prospective multi-centric study between June 2015 and June 2017 recruiting 27 patients with CHD-PAH (3 had closed shunts, 24 had Eisenmenger syndrome; among these, 11 had a pre-tricuspid shunt, 13 had a post-tricuspid shunt), to compare with 27 group 1 non-CHD related-PAH patients (nPAH) and 27 controls matched on age and sex with the CHD-PAH group. Patients with complex CHD were excluded. All patients underwent 2D and 3D transthoracic echocardiography at baseline. 3D RV echocardiographic sequences were analysed by a commercial RV-specific software and output meshes were post-processed to extract deformation data. Results There was no significant age difference between the subgroups. In CHD-PAH patients, RV global area and longitudinal strain did not significantly differ as compared to nPAH but RV global circumferential strain was significantly better (P = 0.006). All strain components were impaired as compared to controls (P Fig. 1 ). Conclusion RV remodelling differs between adults with CHD-PAH and PAH from other aetiologies: 3D RV global circumferential strain is better in CHD-PAH patients and associated with survival free from transplant.

Introduction Pulmonary hypertension (PH) is a life-threatening condition. Right atrial (RA) dilatation is a prognostic parameter, however, the role of RA and LA function as assessed by speckle-tracking imaging remains unclear. The aim of our study was to assess in PH patients RA and LA function and its relationship with outcomes. Methods and results Sixty consecutive PH patients [group 1 (72%) and group 4] not eligible to either pulmonary endarterectomy or angioplasty were prospectively enrolled into this echocardiographic cohort study. In addition to clinical assessment, BNP and 6-minute walk test (6MWT), all patients underwent transthoracic echocardiography (TTE) for PH/RV and RA function assessment with 2D speckle-tracking RA longitudinal strain (LS) including reservoir (RArLS), contractile (RActLS) and conduit (RAcLS) phases analysis, LA LS as well as RA and LA volumes and areas. Patients’ mean age was 62.8 ± 17.3 years and 57% were female. After a median follow-up of 16 months, 8 patients (13.3%) died. RArLS, RAcLS and RActLS were impaired in patients who died as compared to those who survived (respectively P = 0.003, 0.06 and 0.02). On univariate analysis, BNP, RV end-diastolic diameter, pericardial effusion, mitral lateral E/e’, LVEF, TAPSE, LA volume, RA ejection fraction, RActLS, RArLS, RA volume and area were univariate predictors of death or transplant. After multivariable adjustment for age, NYHA class and univariate predictors of death, RA volume (HR = 0.87; 95% CI: 0.78–0.96) and RArLS (HR = 0.007; 95% CI: 0.78–0.96) were independent predictors of outcomes. Conclusion RA function is an important predictor of outcomes. RA volume and reservoir function appear as independent predictors of death in our cohort. The impairment of RA reservoir function likely represents an early indicator of RV failure, independent of RA dilatation.

Background Pulmonary embolism (PE) is a common life-threatening disease, with mortality related to right ventricular (RV) dysfunction. Right atrial (RA) dysfunction may be a new marker of PE severity. In this study, we aimed to assess RA function in patients with acute PE, as compared to a control population and to correlate RA function parameters to classic PE severity parameters. Methods and results We conducted a case-control study, including 27 consecutive PE patients, excluding high-risk PE patients. All patients underwent 2D transthoracic echocardiography with atrial function study within 6 hours of PE diagnosis including RA longitudinal strain (LS) with reservoir (RArLS), conduit (RAcLS) and contractile (RActLS) phases. RA function was assessed using Autostrain LA (TomTec-Philips Medical System). A control group of 18 patients with no structural heart disease in sinus rhythm was recruited in the outpatient clinic. During acute PE, RA strain was impaired in PE patients as compared to controls: P Fig. 1 ). Conclusions Our RA strain study demonstrates an impaired RA reservoir and conduit function in PE patients, which seems to be related to PE severity. Further studies should help improve our understanding of RA dysfunction in PE.

Background Right ventricular (RV) contractile reserve reflects the ability of RV to adapt to elevated afterload. RV functional response to exercise is challenging but could represent an important prognostic factor, especially in pulmonary arterial hypertension (PAH) patients. Purpose To assess different RV contractile reserve evaluation methods in a cohort of PAH patients and controls, using exercise stress echocardiography (ESE). Methods We prospectively included 12 patients with PAH and 12 healthy volunteers. An ESE (using tilt-table ergometer) was performed in all patients to assess RV function at rest and under peak exercise. Changes in these parameters during exercise were calculated to quantify the RV contractile reserve. 3D RV function as well as peak systolic strain, pulmonary pressures, TAPSE, pulmonary VTI and pulmonary output (using the right ventricular outflow tract diameter) were assessed in all patients. Results Our patient group was composed by PAH patients, 52 ± 11 years; mean age of our control group was 31 ± 6 years. PAH patients achieved an exercise with a mean workload of 70 ± 26.4 Watts. There was no complication after the exercise test in all patients. Change in TAPSE was not significantly different between patients and controls (P = 0.17), whereas change in pulmonary VTI, pulmonary output and RV peak systolic strain was highly discriminant (respectively P = 0.03, P = 0.009 and P = 0.0009). Regarding RV contractile reserve parameters, RV end-systolic pressure area ratio (peak/rest) was not statistically different between controls and patients (P = 0.14) whereas change in TAPSE/sPAP, RV peak strain/sPAP, 3D RV EF/sPAP were significantly different (P = 0.005, P = 0.0008, P = 0004). Conclusion Changes in pulmonary output, RV peak systolic strain as well as changes in TAPSE/sPAP but mainly RV peak strain/sPAP, 3D RV EF/sPAP represent consistent and feasible tools to assess RV contractile reserve.

Background Pulmonary arterial hypertension (PAH) is a life-threatening condition. Current ESC guidelines recommend exercise training and rehabilitation in clinically stable PAH patients. Purpose To assess the beneficial effect of exercise training on exercise capacity, quality of life and cardiac function as assessed by echocardiography and cardio-pulmonary exercise test. Methods We prospectively included 11 clinically stable PAH patients over a 6-months period. Exercise echocardiography (EE), cardio-pulmonary exercise test (CPET), SF-12quality of life questionnaire, 6-minute walking test (6MWT), BNP and clinical assessment were performed before and after cardio-pulmonary rehabilitation. Patients underwent 8-weeks of exercise training (3 times a week of aerobic training and at home daily prescribed exercises). Results All patients underwent EE and CPET without any complication. Five patients experienced a reduction in WHO functional class whereas 5 remained stable. Patients significantly improved their physical quality of life (P = 0.006). They also improved their exercise capacity according to the maximum workload during CPET (P = 0.008) and CPET duration (P = 0.001) whereas a trend toward an improved 6MWT was observed (+58 m, P = 0.10). Anaerobic threshold and peak VO2 (+1.7 ± 2.7 mL/kg/min) improved significantly (P = 0.01 and 0.03). Regarding imaging data, at rest, peak strain improved after rehabilitation (P = 0.05) whereas the RV became more dilated. RV contractile reserve, defined by the change in peak systolic longitudinal RV strain between rest and maximum exercise, significantly improved (−3.9 ± 4.7%, P = 0.03). Conclusion In this preliminary study, cardio-pulmonary rehabilitation led to improved quality of life and exercise capacity in PAH. The increased RV contractile reserve post-rehabilitation might explain, in association with the peripheral muscular effects of exercise training, the clinical benefits of rehabilitation in PAH.

Background Two-dimensional transesophageal echocardiography (2DTEE) is currently validated for left atrial appendage (LAA) thrombus assessment but has some limitations. Aims To evaluate the performance and interest of systematic real time three-dimensional transesophageal echocardiography (3DTEE) for LAA thrombus assessment, when performed after 2DTEE. Methods and results Consecutive patients undergoing TEE were prospectively included. LAA was first evaluated using 2DTEE, and patients were classified as "2D-NT" if no thrombus was found, "2D-T" in case of clear thrombus, or "2D-EQ" if equivocal. Then, 3DTEE of the LAA was performed and patients were similarly classified as "3D-NT," "3D-T," or "3D-EQ." Additional LAA CT scan was only performed if LAA thrombus was not clearly ruled out or confirmed by TEE. Additional value of 3DTEE after 2DTEE LAA evaluation was then assessed. We included 104 patients undergoing TEE. Agreement between 2DTEE and 3DTEE was very good for thrombus diagnosis (k = 0.936), but moderate for vacant LAA (k = 0.562) due to more frequent 2D-EQ than 3D-EQ (11.5% vs 2.9%; P = .016). 3DTEE allowed to refine the LAA status in 11 of 12 (91.7%) 2D-EQ patients: 10 3D-NT, 1 3D-T, and 1 3D-EQ. Coupling 3DTEE to 2DTEE permitted a definite LAA diagnosis in 103 of 104 (99%) vs 92 of 104 (88.5%) patients when 2DTEE was used alone (P = .002). Nine (8.7%) LAA thrombi were diagnosed, and 3 CT scan were performed. Conclusion 3DTEE of the LAA is more effective for thrombus assessment than 2DTEE. 3DTEE should be particularly considered in case of equivocal 2DTEE, as it allows to reach a definite LAA diagnosis in almost all of the patients.

Background Outcomes in pulmonary hypertension (PH) are related to right ventricular (RV) function and remodeling. We hypothesized that changes in RV function, especially area strain (AS), could provide incremental prognostic data. Purpose To assess global and regional 3D RV function changes between baseline and 6-months visit and evaluate their prognostic value in PH. Methods In total, 65 PH patients were prospectively included in this longitudinal study. All patients underwent 2D and 3D transthoracic echocardiography at baseline and 6-month follow-up. 3D RV echocardiographic sequences were analyzed by semi-automatic software and output meshes were post-processed to extract regional deformation. Results At baseline, there was no significant difference in RV AS between patient subgroups. Patients who were considered clinically stable or improving presented at 6-months a significant increase in RV EF (P = 0.04) and decrease in RV global AS (P = 0.001) ( Fig. 1 ). The most significant regional changes occurred within the septum. Over a median follow-up of 24.8 months [22.1–25.7], 16 patients died from PH or were transplanted. On multivariate COX analysis, 6-month changes in WHO class (HR 17.4[5.2–58.5]), BNP (HR 1.003[1.001–1.004]) and RV global AS (HR 1.12[1.03–1.21]) were independent predictors of survival. Conclusion Changes in WHO function class, BNP and 3D RV AS could help stratifying the PH patients’ risk. Our study demonstrates the additional prognostic value of following changes in RV deformation using 3D echocardiography.

A 28-year-old woman with a history of long-standing dysphagia (since the age of 10 years) was admitted to the emergency room with respiratory distress and marked stridor in the context of upper res...

International audience; Background: Survival in pulmonary hypertension (PH) relates to right ventricular (RV) function. However, the RV unique anatomy and structure limit 2D analysis and its regional 3D function has not been studied yet. The aim of this study was to assess the implications of global and regional 3D RV deformation on clinical condition and survival in adults with PH and healthy controls.Methods and Results: We collected a prospective longitudinal cohort of 104 consecutive PH patients and 34 healthy controls between September 2014 and December 2015. Acquired 3D transthoracic RV echocardiographic sequences were analysed by semi- automatic software (TomTec 4D RV-Function 2.0). Output meshes were post-processed to extract regional motion and deformation. Global and regional statistics provided deformation patterns for each subgroup of subjects.RV lateral and inferior regions showed the highest deformation. In PH patients, RV global and regional motion and deformation (both circumferential, longitudinal and area strain) were affected in all segments (p-18% was the most powerful RV function parameter, identifying patients with a 48%-increased risk of death (AUC 0.83 [0.74-0.90], p

International audience

Introduction Outcomes in pulmonary hypertension (PH) are related to right ventricular (RV) function and remodeling. We hypothesized that changes in RV function, especially area strain (AS), could provide incremental prognostic data as compared to baseline data. We aimed to assess RV function changes between baseline and 6-months visit and evaluate their prognostic value in PH using 3D echocardiography. Method 95 PH patients were prospectively included in this longitudinal study. All patients underwent 2D and 3D transthoracic echocardiography at baseline and 6-month follow-up. 3D RV echocardiographic sequences were analyzed by semi-automatic software and output meshes were post-processed to extract regional deformation. Results Improvements in the global area strain (lower 2nd measure of area strain) were associated with stable or improving clinical condition as well as survival free from transplant (P Conclusion Changes in RV function and especially follow-up 3D RV AS and RV end-diastolic volume are of prognostic importance. Our study underlines the importance of follow-up data in comparison with baseline data only and demonstrates the additional prognostic value of following changes in RV deformation using 3D echocardiography ( Figure 1 , Death or transplant).

PPAR Research, 2016, ISSN:1687-4757, ISSN:1687-4765

Background Adults with Eisenmenger syndrome have a survival advantage over those with idiopathic pulmonary arterial hypertension. Improved survival may result from preservation of right ventricular (RV) function. Aims To assess left ventricular (LV) and RV remodelling in patients with Eisenmenger syndrome compared to a control population, using speckle-tracking imaging. Methods Adults with Eisenmenger syndrome and healthy controls were enrolled into this prospective two-centre study. Patients with Eisenmenger syndrome with low acoustic windows, irregular heart rhythm or complex congenital heart disease were excluded. Clinical assessment, B-type natriuretic peptide (BNP), 6-minute walk test and echocardiography (including dedicated views to perform offline two-dimensional-speckle-tracking analysis) were performed on inclusion. Results Our patient population (n = 37; mean age 42.3 ± 17 years) was mostly composed of patients with ventricular septal defect (37.8%) or atrial septal defect (35.1%). Compared with the control population (n = 30), patients with Eisenmenger syndrome had reduced global LV longitudinal strain (–17.4 ± 3.5 vs. –22.4 ± 2.3; P

BackgroundSurvival is improved in pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) patients and is related to right ventricular (RV) function. In this study we aimed to investigate the prognostic role of RV mechanics in Eisenmenger Syndrome (ES) compared with other causes of PAH using speckle-tracking imaging.Methods and resultsWe performed a prospective cohort study. We recruited 80 consecutive adult PAH patients: 39 patients with ES and noncomplex CHD (mean age 41,5±15,6 years) and 41 patients with group 1 PAH (mean age 58,9±17,8). Clinical assessment, BNP and echocardiography were realised at the time of inclusion and then clinical outcomes were collected during the regular follow-up. Normal strain values were obtained from 37 control volunteers. On inclusion, 53 patients (66.3%) were in functional class III or higher. Mean BNP was lower in ES (p

Purpose Pulmonary artery (PA) dilatation is often seen in patients with pulmonary hypertension (PH). The objective of our study was to identify factors associated with PA dilatation and to assess its prognosis significance in group 1 and 4 PH patients. Methods We performed a longitudinal cohort study, including consecutive patients with group 1 and 4 PH hospitalized in our center. All patients underwent clinical and biological evaluation, transthoracic echocardiography (TTE), CT scan and right heart catheterization and were followed every 3 months. Results 70 patients were recruited (70% group 1, 30% group 4). Mean age was 67±15 years and 70% were in functional class NYHA III or IV. PA dilatation (>30mm on CT) was observed in 87% of patients. Mean PA diameter was 37.2± 8.2mm and it was significantly larger in congenital heart disease patients: 46.2±8.6mm (p= 0.01). In multiple regression analysis, duration of symptoms (p= 0.01) and myocardial performance index (p= 0.02) were correlated with PA diameter. During a mean follow up of 59±19 months, 18 patients died (26%) (16 from heart failure and 2 from sudden death). On univariate analysis, while NYHA class (HR 2.37, 1.18-4.75), low 6-minute walk distance (HR 0.99 per 50m, 0.98-0.99), low cardiac index (HR 0.25 per 1L/min/m2, 0.09-0.71), right atrial pressure (HR 1.12 per 1 mmHg, 1.02- 1.2), low TAPSE (HR 0.84 per 1mm, 0.76-0.93), right atrial area (HR 1.08 per 1cm2,1.03-0.12) and high BNP were associated with mortality, PA diameter was not (HR: 0.97 per 1mm, 0.91-1.04). On multivariate analysis low TAPSE and high BNP level were independent predictors of all-cause mortality (respectively HR 0.72, 0.52-0.98 and 3.0, 1.01-8.90). Conclusion PA dilatation is frequent in PH and is associated with the duration of symptoms and poor myocardial performance index. However, in group 1 and 4 PH patients, PA dilatation is not predictor of mortality.

Background Non-O blood group patients are at higher risk of first episode of venous thromboembolism (VTE). However, only little is known about the risk of recurrence according to the blood group. In this study, we aimed to determine the impact of ABO blood group on VTE recurrence. Methods We prospectively recruited 106 consecutive patients with a first documented episode of pulmonary embolism (PE). Patients were followed at least 12 months after anticoagulation discontinuation. The main endpoint was recurrence of symptomatic VTE. Results Data from 100 patients were analyzed. Median follow-up was 28 months [24–34.8]. PE was unprovoked in 48 patients. Mean anticoagulation duration was 5.3 ± 2.2 months. The rate of VTE recurrence was 12.7 per 100 patient-years (30 recurrences). B blood group patients had a 2.7-fold increased risk of VTE recurrence (95%CI 1.1-6.2, p = 0.03). On multivariate analysis, B blood group was the strongest independent predictor of VTE recurrence (Hazard Ratio (HR) 2.6, 95%CI 1.1-6.1, p = 0.04). In contrast, A and AB blood groups were not associated with VTE recurrence. VTE recurrences were less frequent in O blood group compared to non-O patients (HR 0.5, 95%CI 0.2-1.1, p = 0.09). O blood group women had a 5-fold decreased risk of VTE recurrence (HR 0.2, 95%CI 0.1-0.8, p = 0.01). Conclusions Non-O blood groups, beyond being involved in the occurrence of a first VTE event, also contribute to VTE recurrence. B blood group is strongly associated with VTE recurrence, thus high-risk B blood group patients could benefit from long-term anticoagulation therapy after a first VTE event.

BackgroundComputed tomography pulmonary angiography (CTPA) is routinely used to diagnose pulmonary embolism (PE). Reflux of contrast medium into the inferior vena cava or hepatic veins (IVC) on CTPA is a simple sign that could help for PE risk stratification. The purpose of this study was therefore to investigate prognosis significance of contrast reflux into IVC in acute PE.Methods and results141 consecutive patients with acute PE confirmed by CTPA were prospectively included between March 2010 and February 2013. Degree of reflux into the IVC and the hepatic veins was graded from 1 (none) to 6 (severe) by 2 independent observers, blinded to each other. The presence of reflux in IVC was compared with clinical parameters used in the ESC guidelines for PE risk stratification: electrocardiographic signs, Troponine I, BNP and right ventricular dilatation (RV/ LV>0,9) or dysfunction (TAPSE < 17mm, S’110 bpm (OR 5.6, 1.03-30), atrial fibrillation (OR 6.3, 1.05-37.7), negative anterior T waves (OR 6.1, 1.3-29.1), elevated Troponin Ic (OR 5.4, 1.1-25.8), elevated BNP (OR 11.5, 1.3-98.2), right ventricular dysfunction (OR 5.3, 1.1-25.1) were predictors of death or clinical deterioration. Contrast reflux into IVC from grade 4 to 6 was observed in 17% of patients. Interobserver agreement was excellent (Concordance correlation coefficient 0.91). Grade 4 reflux or greater was a strong predictor of events (OR 15.1, 2.8-83.7) and had a 86% specificity and 71% sensitivity to predict adverse outcomes (AUC 0.88).ConclusionA grade 4 or higher contrast reflux into the IVC is a simple and frequent CTPA sign, highly predictive of adverse outcomes in PE patients.

Introduction Speckle tracking imaging is a recent technique that can be achieved using either vendor dependent or vendor-independent software. Right ventricular (RV) strain is increasingly used as a prognostic tool in both left and right ventricular diseases. Only little is known regarding the variability of vendor-dependent and - independent speckle-tracking imaging software in the assessment of RV free wall longitudinal strain (RLS). The aim of our study was to compare a vendor-dependent (Qlab 9.0, Philips Medical System, Andover, MA, USA) and - independent (Cardiac Performance Analysis, Tomtec Imaging Systems, Germany) software for RLS analysis. Methods and results We prospectively enrolled 90 consecutive patients with pulmonary hypertension (mean age 55,8±19years) and 26 control patients (mean age 33,9±13years) who underwent a comprehensive echocardiogram including a RV focused 4-chamber view optimised for speckle-tracking analysis. DICOM data sets were stored and analysed by 2 different cardiologists using Qlab and TomTec, blindly to the context and each other. In the whole population, mean RLS was -17,3%±9 and -8,6% ±7,2 respectively using Qlab and Tomtec. Qlab and Tomtec intra-observer coefficients of variation (CV) were -13,19% and -9,56% and interobserver CVs were -22% and -15% respectively. The concordance correlation coefficient was 0,55, indicating poor agreement between the two methods. In the control population, Qlab CV was - 3,63%, whereas CV was -17,8% in RV disease patients. Conclusion Despite an acceptable level of variability for both techniques, Tomtec appears less variable. Variability of Qlab is excellent in control patients but is highly influenced by RV morphology. The agreement between the two software products is low and should lead in clinical practice to the follow-up of patients with the same software and advocates for the development of dedicated RV speckle-tracking software products.

Dnmt2/Trdmt1 is a methyltransferase, which has been shown to methylate tRNAs. Deficient mutants were reported to exhibit various, seemingly unrelated, defects in development and RNA-mediated epigenetic heredity. Here we report a role in a distinct developmental regulation effected by a noncoding RNA. We show that Dnmt2-deficiency in mice results in cardiac hypertrophy. Echocardiographic measurements revealed that cardiac function is preserved notwithstanding the increased dimensions of the organ due to cardiomyocyte enlargement. Mechanistically, activation of the P-TEFb complex, a critical step for cardiac growth, results from increased dissociation of the negatively regulating Rn7sk non-coding RNA component in Dnmt2-deficient cells. Our data suggest that Dnmt2 plays an unexpected role for regulation of cardiac growth by modulating activity of the P-TEFb complex.

Purpose: Patent foramen ovale (PFO) is associated with paradoxical embolism and mortality in pulmonary embolism (PE). The aim of this study was to assess the prevalence of PFO comparing trans-thoracic echocardiography (TTE) and trans-oesophageal echocardiography (TOE) and its association with ischaemic stroke (as assessed by magnetic resonance imaging (MRI)) in patients hospitalized for PE. Methods and results: We prospectively enrolled 40 consecutive patients hospitalized in the cardiology department for intermediate early-mortality risk PE. Patients with recent history of stroke or contra-indication to TOE were excluded from this study. Local ethics approval was obtained for the study. Contrast TTE and TOE were performed within 5 days after admission. Mean time from admission to TOE was 3.4±1.2 days, whereas mean time to MRI was 14±5.8 days. On admission, mean systolic arterial pressure was 132±40 mmHg, mean troponine Ic was 0.4±1.7 ng/ml (normal reference value < 0.06 ng/ml) and mean BNP was 585±2200 pg/ml. PFO was found in 22.5% (n=9) and 52.5% (n=21) of our patients, respectively with TTE and TOE. Compared with TTE, TOE significantly improved the sensitivity of echocardiography in detecting PFO (p = 0.002). No significant difference on admission's characteristics was found between patients without PFO (PFO-; n=19) and patients with PFO (PFO+; n=21). On MRI evidence of recent ischaemic stroke was found in 7 PFO+ patients (33.3%), versus none in PFO- patients (p=0.004). In the sub-group of stroke patients (n=7), TOE was positive for PFO in all, whereas TTE failed to identify PFO in 3 of them (recognition of PFO, TOE vs TTE: 100 vs 57%, p=0.02). No complication of TOE occurred in our cohort (including GI-bleeding). Conclusion: PFO is frequent in patients hospitalized for intermediate risk-PE, and occurred in 52.5% of our patients. In our study, PFO is strongly associated with ischaemic stroke on MRI and recognition of PFO in patients with PE could help identifying patients with high-risk of stroke and potential bleeding complications.

Purpose Prior studies have recognised non-O blood type as a risk factor for venous thromboembolism (VTE). Though, ABO blood type contribution to VTE recurrence risk is poorly studied. We sought to determine the impact of blood type on VTE recurrences. Methods Our prospective cohort included 120 subsequent patients presenting a first episode of pulmonary embolism (PE). In all cases, PE were diagnosed with a CT scan. All participants were followed for at least 12 months after anticoagulation therapy discontinuation. Results Over a mean follow-up period of 30 months, the rate of recurrence was 8.9% per patient and per year. PE was unprovoked in 63 patients (52.5%). Mean duration of oral anticoagulation was 6 months. Compared to O blood type, the unadjusted hazard ratio (HR) of VTE recurrence for non-O blood type was 1.97 (95%CI, 0.91-4.26, p=0.07). B blood type exposed to a higher risk of VTE recurrence than O blood type with a HR of 2.6 (95%CI, 1.12-6.06, p=0.03). A and AB blood types were not associated with an increased risk of VTE recurrence when compared to O blood type. Conclusion Non O blood type are involved not only in the occurrence of a first episode of VTE but tend to contribute to VTE recurrence. Patients with B blood type develop more VTE recurrence than others. The ABO blood type could be one of the characteristics to be studied for the determination of the length of anticoagulant treatment after a first episode of VTE

SummaryPatients with pulmonary hypertension must be evaluated using a multimodality approach to ensure a correct diagnosis and basal evaluation as well as a prognostic assessment. Beyond the assessment of pulmonary pressures, the echocardiographical examination allows the evaluation of right ventricular adaptation to elevated afterload. Numbers of variables are commonly used in the assessment of the pulmonary hypertension patient in order to detect changes in right heart geometry, right-to-left interaction and right ventricular dysfunction. Whereas an isolated change in one echocardiographical variable is not meaningful, multiple echocardiographical variable modifications together provide accurate information. In this review, we will link pulmonary hypertension pathophysiological changes with echocardiographical indices and describe the clinical implications of echocardiographical findings.