Abstract Funding Acknowledgements Type of funding sources: None. Background In patients with pulmonary hypertension (PH), right ventricular (RV) function has a well-known prognostic value. Recent data suggest that right ventricular-pulmonary artery (RV-PA) coupling is an independent predictor of outcome in this setting. RV-PA coupling can be assessed invasively as the endsystolic/arterial elastance ratio or noninvasively using surrogates such as CMR-derived RV stroke-volume (SV) to endsystolic volume (ESV) ratio or 2D echocardiography-derived TAPSE/PASP ratio. Data regarding the 3D echocardiography-derived RV SV/ESV as parameter of RV-PA coupling in PH patients are scarce. Purpose To assess the RV-PA coupling as 3DE-RV SV/ESV ratio and its impact on clinical outcome in patients with precapillary PH compared with the validated 2DE-TAPSE/PASP. Methods Thirty-two patients (40±13 years, 27 women) with precapillary PH receiving vasodilator therapy and 25 controls of similar age and gender (37±9 years, 18 women) were studied. Clinical data, BNP levels and RV function parameters (TAPSE, free-RV wall S- and e’-wave, RV FAC, Tei index, RV-IVA, RVEF by 3DE) were assessed. RV global longitudinal strain (RV-GLS) was measured as the average of 6 segments and RV free wall strain as the average of 3 segments by speckle-tracking 2DE. RV-PA coupling was assessed as TAPSE/PASP and 3DE-RV SV/ESV ratios. PH patients were followed for 41 months (2–77). Cardiac death was the endpoint. Results PH patients had impaired 2DE and 3DE RV function parameters and RV-PA coupling compared to controls (p<0.001). At baseline, 3DE-RV SV/ESV and TAPSE/PAPS correlated with each other (r=0.51, p = 0.003) and both correlated with RV function parameters, but only SV/ESV correlated with BNP levels (Table) and WHO functional class (0.71±0.16 in class II, 0.66±0.21 in class III, 0.34±0.14 in class IV, p = 0.01). During follow-up, 5 patients died and these patients had at baseline higher BNP levels (LnBNP 5.91±1.62 vs 4.32±1.29, p = 0.02) and lower values of TAPSE (15±4 vs 18±3 mm, p = 0.03), S-RV (9.2±2.3 vs 11.1±1.8 cm/s, p = 0.04), RV free wall strain (−11.6±6.6 vs −18.1±5.3%, p = 0.02), RVEF (32±11 vs 40±7%, p = 0.04), and SV/ESV (0.49±0.22 vs 0.68±0.19, p = 0.05), but similar values for TAPSE/PASP (0.18±0.10 vs 0.20±0.05 mm/mmHg, p = 0.75). Conclusion In PH patients, 3DE-RV SV/ESV ratio as parameter of RV-PA coupling is altered and correlates with clinical status and RV function parameters. Besides parameters of RV function and BNP levels, 3DE-RV SV/ESV ratio seems a better predictor than TAPSE/PASP in this setting.

Abstract Funding Acknowledgements Type of funding sources: None. Background. It is widely believed that right ventricular (RV) volume overload is better tolerated than RV pressure overload and this is mainly due to the preservation of or even an increase in RV longitudinal function in patients (pts) with RV volume overload. Conversely, patients with RV pressure overload experience an early decrease in radial shortening and the global RV performance is maintained due to a normal longitudinal function. The multiparametric assessment of RV function and RV-pulmonary artery (PA) coupling could offer a better understanding of the RV adaptive mechanisms in response to volume versus pressure overload. Purpose. To assess the right heart remodelling and function in different chronic loading conditions using an integrated echocardiographic and invasive approach. Methods. Thirty-nine patients with atrial septal defect (ASD) and 41 pts with pulmonary hypertension (PH) age and gender-matched were enrolled. The etiology of PH was idiopathic (14 pts), operated congenital heart disease (3), connective tissue disease (9), chronic thromboembolic PH (8) and other forms of arterial PH (7). Clinical parameters, B-type natriuretic peptide (BNP), RV function and pulmonary artery stiffness (PAS) echocardiographic parameters were assessed. RV-PA coupling was assessed using the TAPSE to systolic PA pressure ratio. A right heart catheterization was also performed and ASD pts with pulmonary vascular resistance >3 Wu and/or Qp/Qs ratio <1.5 were excluded. Results. PH pts were more symptomatic than pts with ASD (32 PH pts vs 6 ASD pts were in NYHA class III and IV, p < 0.001). ASD pts had lower BNP levels (lnBNP 4.24 ± 1.11 vs 5.49 ± 1.29, p < 0.001), similar right atrial (RA) area (26.6 ± 7.7 vs 27.8 ± 11.9 cm2, p = 0.61) and pressure (7.5 ± 4.2 vs 8.2 ± 5.9 mmHg), lower systolic (1.11 ± 0.13 vs 1.55 ± 0.50) and diastolic (1.21 ± 0.16 vs 1.50 ± 0.32) eccentricity index (p < 0.001) and better RV function parameters than PH pts (all p < 0.001): TAPSE (26 ± 5 vs 16 ± 3 mm), RV-free wall S wave (14.4 ± 2.5 vs 9.9 ± 2.1 cm/s), RV fractional area change (46 ± 8 vs 32 ± 8%), RV global longitudinal strain on 3 segments (-27.6 ± 5.0 vs -14.4 ± 6.0%) or 6 segments (-24.2 ± 4.3 vs -12.5 ± 4.9%). Also, ASD pts had less impaired PAS parameters (pulmonary compliance 4.03 ± 5.54 vs 1.53 ± 3.01 mm2/mmHg, p = 0.016; elastic modulus 167 ± 131 vs 594 ± 369 mmHg, p < 0.001; beta index 5.36 ± 3.77 vs 11.00 ± 6.39, p < 0.001) and better RV-PA coupling (0.60 ± 0.20 vs 0.19 ± 0.06 mm/mmHg, p < 0.001) than PH pts. The BNP levels significantly correlated with RA area, parameters of RV size and systolic function and RV-PA coupling in both groups, but invasively assessed PA pressure correlated with BNP levels only in ASD pts. Conclusions Patients with ASD as a model of chronic RV volume overload have not only preserved RV longitudinal function but also better global RV function, PAS and RV-PA coupling compared to pts with chronic RV pressure overload. The BNP levels are significantly more impaired in pts with chronic RV pressure overload.

Abstract In patients with pulmonary hypertension (PH) right ventricular (RV) function is a well-recognized determinant of clinical outcome, including cardiac death. Data regarding the prognostic value of comorbidities in patients with PH are scarce. Purpose. To identify predictors of cardiac death and all-cause mortality on mid-term follow-up in PH patients receiving specific vasodilator therapy. Methods. Sixty-eight patients (40 ± 16 years, 51 women) with PH (12 with idiopathic pulmonary arterial hypertension-PAH, 32 with congenital heart disease, 10 ith connective tissue disease, 7 with chronic thromboembolic PH and 7 with other forms of PAH) treated with pulmonary vasodilators were studied. Clinical parameters (NYHA class, 6-minutes walking distance, 6MWD), biological (hemoglobin, B-type natriuretic peptide-BNP), and echocardiographic parameters of RV function (TAPSE, tissue-Doppler imaging-derived free-RV wall S- and e’-wave, RV fractional area change (FAC), Tei index, I/H, RV isovolumic acceleration-IVA) and cardiac index were assessed. RV global longitudinal strain (RV-GLS) was measured from the apical 4-chamber view as the average of 3 (free-wall) and 6 (free-wall and interventricular septum) segments by 2D speckle-tracking echocardiography. Parameters of pulmonary artery stiffness (PAS) were assessed: pulsatility, capacitance, elastic modulus, dynamic compliance. Two endpoints were defined: cardiac death and all-cause mortality. Results. PH patients were followed-up for 35 months (4-81). Comorbidities were identified in 15 patients (non-cardiac surgery, anemia, chronic kidney disease, neoplasia, respiratory infections). During follow-up, 20 patients died, 14 deaths being cardiac deaths. At univariable analysis, the following parameters correlated with all-cause mortality: age (51 ± 14 in deceased patients vs 36 ± 15 years in surviving patients, p < 0.001), 6MWD (262 ± 171 vs 369 ± 155 m, p = 0.015), FAC (30 ± 9 vs 38 ± 8%, p < 0.001), 6 segments RV-GLS (-12.3 ± 5.1 vs -15.1 ± 4.5%, p = 0.025), S-RV (9.9 ± 2.2 vs 11.4 ±2.3 cm/s, p = 0.016), TAPSE (16.3 ± 3.3 vs 18.6 ± 3.5 mm, p = 0.014), right atrial (RA) area (30.6 ± 13.1 vs 21.2 ± 8.8 cm2, p = 0.001) hemoglobin (12.9 ± 2.6 vs 15.2 ± 3.0 g/dl, p = 0.004) and BNP levels (lnBNP, 5.8 ± 1.3 vs 4.1 ± 1.4, p < 0.001), presence of comorbidities (10 vs 5, p = 0.001). At multivariable analysis, only comorbidities (p = 0.05) and FAC (p = 0.07) seemed to independently predict all-cause mortality. Predictors of cardiac death at univariate analysis were age, RV function parameters, RA area, PA capacitance (1.02 ± 0.56 vs 1.48 ± 0.80 ml/mmHg, p = 0.05) and cardiac index (2.48 ± 0.64 vs 3.31 ± 1.34 l/min/m2, p = 0.03) but only age (p = 0.03) and cardiac index (0.06) emerged as independent predictors of cardiac death. Conclusions: In PH patients, RV function and comorbidities predict all-cause mortality, while age and cardiac index are independent determinants of cardiac death. Identifying and properly treating associated pathologies could impact survival in this setting.

Noncardiac complications significantly contribute to the morbidity and mortality of adults with congenital heart disease (CHD), mainly because life expectancy and quality of life for those born with CHD have greatly improved in the last decades. Double-outlet right ventricle (DORV) with a subaortic ventricular septal defect (VSD) associated with subpulmonary stenosis is a complex cyanotic congenital heart disease from the spectrum of tetralogy of Fallot. We present the case of a young male who was diagnosed at the age of 18 with DORV with subaortic VSD (Figure A), subpulmonary stenosis (Figure C) and mitral valve malformation (anterior leaflet cleft (Figure B) and both leaflets prolapse) with secondary moderate mitral regurgitation (MR), associated with major aortopulmonary collaterals, without pulmonary hypertension. There was no surgical correction at that moment. At the age of 26 he presented with recurrent hemoptysis and embolisation of bronchial arteries was performed. In the same year, a cerebral MRI showed signs of multiple infratentorial and supratentorial ischemic strokes. One year later, he was diagnosed with brain abscess and otomastoiditis that were surgically managed; multiple microorganisms were isolated from the two sources (M. morganii, K. pneumoniae, P . aeruginosa) and the patient received prolonged antibiotic therapy. Two months later he presented with clinical, biological and echocardiographic signs of infective endocarditis (IE) and blood cultures confirmed the diagnosis of Candida albicans associated mitral valve IE (Figure D, E, F, G). Antifungal therapy was administered for one month, with the complete disappearance of the vegetation, but worsening of the mitral regurgitation and moderate left ventricular systolic dysfunction. Thus, the patient had a clear indication for mitral valve replacement together with complete repair of the cardiac malformation that seemed still feasible as the pulmonary arteries were well developed, there was no pulmonary vascular disease and the systolic function of the RV was normal. Correction of CHD was performed in March 2019 consisting of repair of the DORV (Figure H), mitral valve replacement (33 mm bileaflet mechanical valve) (Figure I), tricuspid valve repair with RVOT remodelling. Postoperative, the patient clinical status improved significantly and transthoracic echocardiography revealed a mild residual subpulmonary stenosis (Figure J) and normally functioning mitral prosthesis. There are frequent, various complications in the natural history of congenital heart diseases, especially unrepaired. This case illustrates how vicious the circle of complications can get in a case of unrepaired cyanotic cardiac malformation and the difficulty of breaking this circle. The correction of CHD is the best prevention method and should be performed whenever it is feasible, even at an adult age. Abstract P719 Figure.

Patients with Eisenmenger’s syndrome (ES) have better survival than other patients with pulmonary arterial hypertension (PAH) probably due to the preservation of right ventricular (RV) function. As in PAH patients RV remodeling and function depend not only on pulmonary artery (PA) pressure but also on the intrinsic properties of PA wall, there is also a possible role of PA stiffness (PAS) as outcome predictor in this setting. Purpose. To study the prognostic role of PAS parameters assessed by 2D transthoracic echocardiography in patients with ES compared to other patients with pulmonary hypertension (PH) receiving specific vasodilator therapy. Methods. Sixty-eight PH patients were enrolled: 27 ES patients and 41 non-ES patients, including patients with other types of PAH (12 idiopathic PAH, 5 operated congenital heart disease, 10 connective tissue disease, 7 other forms of PAH) or chronic thromboembolic PH (7 patients) receiving oral vasodilator therapy. Clinical data, B-type natriuretic peptide (BNP), RV function and PAS parameters were assessed: pulmonary capacitance (PC), PC indexed to body surface area (PC/BSA), pulsatility, elastic modulus (EP), beta-index. PH patients were followed-up for 2.9 years (4 months-6.8 years). Results. Pulmonary vascular resistance (PVR) assessed by right heart catheterization was similar in both groups (11.9 ± 8.0 vs 11.0 ± 6.4 Wood units, p = 0.68). ES patients had lower BNP levels (lnBNP 3.63 ± 1.31 vs 5.31 ± 1.33, p < 0.001) and better RV function than non-ES patients: RV-free wall S wave, RV-S (12.2 ± 2.3 vs 10.2 ± 2.0 cm/s, p < 0.001), RV fractional area change, RV-FAC (40 ± 7 vs 32 ± 9%, p < 0.001), RV global longitudinal strain (RV-GLS) on 3 segments (-20.2 ± 4.4 vs -14.8 ± 6.0%, p = 0.001) or 6 segments (-16.2 ± 4.2 vs -13.1 ± 4.9%, p = 0.011). In ES patients PAS parameters were less impaired than in non-ES group (PC 1.68 ± 0.86 vs 1.18 ± 0.66 ml/mmHg, p = 0.014; PC/BSA 1.05 ± 0.53 vs 0.68 ± 0.37 ml/mmHg m2, p = 0.003; pulsatility 18.8 ± 8.4 vs 13.8 ± 6.4%, p = 0.007, EP 390.7 ± 198.6 vs 578.8 ± 341.6 mmHg, p = 0.007; beta index 6.09 ± 2.85 vs 10.77 ± 6.21, p < 0.001). During follow-up, 12 cardiac deaths occurred: 1 in ES group and 11 in non-ES group (p = 0.021). In non-ES group, predictors of cardiac death were parameters of RV function and PAS: BNP levels (lnBNP 6.20 ± 1.10 in deceased patients vs 4.97 ± 1.27 in survivors, p = 0.007), RV-S (9.1 ± 2.0 vs 10.6 ± 1.9 cm/s, p = 0.038), RV-FAC (25 ± 8 vs 35 ± 7%, p = 0.001), RV-GLS on 3 segments (-11.1 ± 4.4 vs -16.2 ± 6.0%, p = 0.015) or 6 segments (-9.0 ± 3.7 vs -14.6 ± 4.4%, p = 0.001), PC (0.86 ± 0.29 vs 1.32 ± 0.72 ml/mmHg, p = 0.01; PC/BSA (0.51 ± 0.17 vs 0.76 ± 0.41 ml/mmHg m,2 p = 0.013). Conclusion: Patients with ES have better RV function and less impaired PAS compared to patients with other types of PH and similar PVR. Moreover, besides RV function, PAS parameters emerged as predictors of cardiac death in non-ES patients that had worse prognosis than ES patients. The impact of these findings on clinical outcomes in ES patients remains to be further studied.

In patients (pts) with pulmonary hypertension (PH) different right ventricular (RV) function parameters predict outcome. Data regarding the prognostic value of averaged 6-segments RV longitudinal strain (RV-GLS) in PH pts are scarce.

Introduction.Double-chambered right ventricle (DCRV) is a very rare congenital defect causing right ventricular outflow tract (RVOT) obstruction where the right ventricle (RV) is divided by aberrant muscle bundles into a high-pressure and low-pressure chamber.Case report.A 44-year-old man was referred to our center for progressive exertional dyspnea.He was previously diagnosed with Eisenmenger syndrome secondary to an uncorrected ventricular septal defect (VSD).Clinical evaluation showed cyanosis, a harsh systolic murmur along the left sternal border and hepatomegaly.The electrocardiogram revealed atrial fibrillation, right bundle branch block and right ventricular hypertrophy.Transthoracic echocardiography (TTE) showed the presence of a discrete obstruction in the RVOT (Fig. A), adjacent to the pulmonary valve, caused by an anomalous muscle bundle, consistent with DCRV.Both the right atrium (RA) and the RV were severely dilated, with marked RV trabeculations and moderate to severe RV systolic dysfunction.Moderate to severe secondary tricuspid regurgitation was also identified.The RV-RA systolic gradient (108 mmHg) was used as a surrogate for the intraventricular gradient.The left ventricular ejection fraction was normal.The patient had a large circumferential pericardial effusion with no signs of cardiac tamponade.The patient underwent right heart catheterization that showed severely increased right ventricular systolic pressure (108 mmHg) but normal pressure and arterial resistance in the pulmonary artery.The right ventriculogram identified an obstruction between the RV and the pulmonary valve (Fig. B).There was no VSD.Since the patient was cyanotic, the presence of an intracardiac shunt was suspected.Transoesophageal echocardiography (TOE) confirmed the diagnosis of DCRV and identified a right-to-left atrial shunt at the level of a patent foramen ovale.For further anatomical assessment of the RV obstruction and RV systolic function, a cardiac magnetic resonance (CMR) study was performed.It showed subinfundibular muscular obstruction and severe RV systolic dysfunction (Fig. C).Surgery was indicated (with a high perioperative risk), and the patient underwent resection of the muscle bundle and tricuspid annuloplasty.Unfortunately, the postoperative evolution was unfavorable, with nonresponsive RV failure and death.Discussion.DCRV is frequently associated (80-90%) with VSD/pulmonary valve stenosis.Isolated DCRV is very uncommon, therefore ruling out associated lesions is paramount.This disease is usually diagnosed in childhood.Hence, there is little data available in adults.Moreover, the presentation is frequently nonspecific.Differential diagnosis must be made with infundibular/ pulmonary valve stenosis.TTE is diagnostic in a few patients (15%), while TOE is superior, defining the full morphological details, including associated defects.Since surgical correction before RV failure carries a small risk and is curative, misdiagnosis must be prevented by accurate imaging of the whole RV, RVOT and pulmonic valve.While TTE is the first-line imaging tool, use of additional imaging techniques: TOE, cardiac catheterization, ventriculography and CMR adds useful information in many cases.Conclusion.This is a very rare presentation of an isolated DCRV in an adult patient.It underlines the importance of using multiple and complementary imaging techniques to establish the correct diagnosis.

In patients with pulmonary hypertension (PH) right ventricular (RV) remodeling and function depend not only on pulmonary pressure but also on intrinsic properties of pulmonary artery wall. RV function is a well-recognized determinant of clinical outcome in PH patients. Data regarding the impact of pulmonary artery stiffness (PAS) assessed by echocardiography on PH patients’ outcome are scarce. Due to the com- mon embryological origin of the proximal segments of the great vessels, aortic elastic properties may also be impaired in this setting. Purpose. To assess pulmonary and aortic stiffness parameters in PH patients receiv-ing specific vasodilator therapy using 2D transthoracic echocardiography and the PAS impact on clinical outcome in these patients. Methods. Forty-seven patients (40 6 15 years, 35 women) with PH (13 with idiopathic pulmonary arterial hypertension (PAH), 17 with congenital heart disease, 6 with con-nective tissue disease, 6 with chronic thromboembolic PH and 5 with other forms of PAH) treated with either bosentan, sildenafil, or both and 33 healthy volunteers with similar age and gender (40 6 11 years, 21 women) were studied. Clinical parameters, B-type natriuretic peptide (BNP) and PAS parameters were assessed: pulsatility, capacitance, elastic modulus, compliance, dynamic compliance, beta-index, pulmo- nary strain. Moreover, following parameters of aortic stiffness (AS) were also assessed: strain, pulse pressure, elastic modulus and beta index. PH patients were followed-up 23 months (2-49). endpoint of cardiac death defined. Results. and end-diastolic PAS parameters compared to healthy volunteers All parameters of AS were also significantly impaired patients with PH compared to with p from 0,002 to 0,049. At the end-of-follow-up, there was a significant impairment in compliance mmHg-1, distensibility mmHg-1, 0.018), modulus 0.004), beta (9.96 6.04 15.46 14.34, p ¼ 0.011), strain 3.08 vs 5.31 6 3.20 %, p ¼ 0.043) and pulsatility (13.45 6 6.61 vs 11.01 6 6.81 %, p ¼ 0.044). During follow-up, 7 patients died. Amongst PAS parameters, patients who the endpoint had at baseline a PA capacitance (0.54 6 0.20 vs 0.86 6 0.51 ml/(mmHg*m2), p ¼ 0.011). baseline PA capacitance significantly correlated with end-of-follow-up 6 minutes walk distance (r ¼ 0.34, p ¼ 0.032) and BNP levels ¼ p ¼ 0,021). In clinical out- Background: Right atrial pressure (RAP) is an essential component in the hemody- namic assessment of patients and a requisite for the noninvasive estimation of the pulmonary artery pressures. Estimation of RAP using ultrasound measurement of the inferior vena cava (IVC) diameter along with its respiratory variation is commonly per-formed because it is simple and non-invasive technique, despite the paucity of data that evaluates this technique compared to simultaneous invasive methods. Purpose: The purpose of the present study was to establish cut-off values for esti- mating elevated RAP (RAP (cid:2) 10mmHg) from IVC parameters obtained concomitantly with invasive value. Methods: One-hundred and twenty patients (55 6 17 years, 60 male) with heart dis- eases who were scheduled for right-heart catheterization or central venous catheter insertion were prospectively enrolled. Patients on mechanical ventilation and with heart transplantation were excluded. IVC ultrasound parameters were obtained simultaneously with invasive measurements. The measurement of an IVC diameter were made at end-expiration and just proximal to the junction of the hepatic veins that lie approximately 0.5 to 3.0 cm proximal to the ostium of the right atrium recom- mended in current American Society of Echocardiography guidelines. To assess IVC collapse, the changes in diameter of the IVC with a brief sniff were also measured. Results: Mean RAP was 6 6 4 mmHg (range 1 to 22 mmHg). Indications for invasive measurements were pulmonary hypertension (48%), ischemic heart dis- ease?(19%), congenital heart disease (16%), cardiomyopathy 6 11.46 years) over 12 years (median 4.5 þ /- 0.27 years). Survival curves of Kaplan-Meier were constructed. Mortality in the first year was 9.2% and at 5 years 45.4% with a mean survival time of 5.9 years. Independent predictors of mortality were established using a logistic regression Cox. Clinically relevant variables were progressively chosen to build the best multivariate model using a stepwise procedure, identifying as prognostic variables the age (HR 1.95 CI 1.58 to 2.42), ischemic etiology (HR 1.61 CI 1.32 to 1.98), functional class NYHA II (HR 1.88 CI 1.15 to 3.04) and III-IV (HR 2,73 CI 1.68 to 4.43), the NT-pro-BNP (HR 1.85 CI 1.50 to 2.28), hospitalization prior to the inclusion (1,82 HR IC 1.28 to 2.58) and tricuspid gradient (HR 1.92 CI 1.32 to 2.18). Conclusions: Pulmonary hypertension measured by echocardiogram tricuspid gra- dient is an important independent predictor of mortality. Other echocardiographic variables do not add value to the multivariate model. Methods: 309 participants of the SKIPOGH study (Swiss Kidney Project On Genes in Hypertension), a multi-center population-based study investigating the genetic determinants of arterial hypertension underwent a complete TTE study including 3D- acquisitions of the atria. 3D datasets of the RA were acquired in multi-beat mode to obtain a minimal volume rate of 25/s and were analyzed with a commercial software. Participants with a dilated right ventricle (RV area > 25 cm2), a reduced RV function (FAC < 35%) or an increased pulmonary pressure (velocity of the tricuspid regurgita- tion jet > 2.8 m/s) or with an otherwise abnormal echocardiogram were excluded. No participant had more than mild tricuspid regurgitation. Image quality was graded for each 3D dataset and agreement between 3D and single-plane 2D RA volumes was assessed with Bland-Altman statistics and calculation of the coefficient of variation (CV). techniques, especially in RA that are not spherical in shape. Purpose: Right ventricular (RV) function assessment is a challenge by its complex morphology. Right ventricular free wall longitudinal strain (RVLS) might be more sen-sitive to detect RV dysfunction and proved to be a good prognosticator in heart failure. Organic mitral regurgitation (MR) is a frequent heart valve disease in western coun-tries which impairs cardiovascular prognosis when patients are referred for rescue surgery (with symptoms or LV dysfunction). Lately we demonstrated that RV EF alter-ation assessed by radionuclide angiography is frequent and impairs post-operative prognosis in organic MR. We sought to assess RV function and determinants of RV dysfunction in organic MR based on RVLS. Methods and Results: One hundred ninety-two patients (60 6 15 years, 125 males) with mild to severe chronic organic MR were prospectively enrolled and had a com- plete echocardiography including a RVLS assessment. In addition, RV ejection fraction (EF) was measured by cardiac magnetic resonance (CMR) imaging (n ¼ 128) or radionuclide angiography (n ¼ 6) in 134 patients (70%). Patients were stratified according to RVLS < -26% (normal RVLS function) or > -26% (impaired function). Right ventricular LS was impaired in 96 patients (50%). Patients with RV dysfunction exhibit a more severe condition with greater MR (SOR MR: 0.46 6 0.25 cm 2 versus 0.38 6 0.22 p ¼ 0.023), were more symptomatic (NYHA 2 6 0.9 versus 1.5 6 Background: right ventricular (RV) systolic dysfunction is an important prognostic factor in pulmonary hypertension (PH) patients. However, the progression of RV mechanical changes that leads to the RV pump dysfunction remains to be clarified. We developed a software package that applied to the three-dimensional echocardiographic (3DE) beutel of the RV allows to discriminate between the relative contribution of the longitudinal and radial displacement to the global RV pump function. Purpose: to investigate the mechanics of RV by 3DE in PH patients. Methods: 81 3DE of the RV were acquired in 59 consecutive PH patients (79% females; 59 6 14 years) and compared with 57 age- and gender-matched controls. Exclusion criteria was PH type 2. 3DE data sets of the RV have been analyzed by RV function 2.0 (TomTec Imaging Systems, Unterschleissheim, Germany) in order to obtain volumes and ejection fraction (RV-EF). Then PH have been divided according to RV-EF: preserved ( (cid:2) 45%) or impaired ( < 45%). Finally, the 3DE RV beutels were analyzed by our custom made software package obtaining the longitudinal EF (LongEF) and radial EF (RadEF) and their relative contribution to RV-EF by calculat- ing the ratios LongEF/RV-EF and RadEF/RV-EF. Results: PH patients showed larger 3DE RV end-diastolic and end-systolic volumes (106 6 39 ml vs 67 6 14 ml and 65 6 33 vs 28 6 7 ml, respectively; p < 0.0001) and lower 3D RV-EF (41 6 11% vs 58 6 4%; p < 0.0001) than controls. Similarly, both LongEF and RadEF were lower (18 6 7% vs 27 6 4% and 15 6 7% vs 27 6 5%, respectively; p < 0.0001) in PH patients. However, only the RadEF/RV-EF appeared impaired (36 6 11 vs 47 6 6, p < 0.0001), whereas LongEF/RV-EF (47 6 9 vs 47 6 6, p ¼ NS) was similar among groups. Looking at the subgroup of patients with reduced RV-EF, RV pump dysfunction was mainly driven by progressive reduction of the radial component (Table). Conclusions: the radial component of RV wall displacement is as important as the longitudinal one to determine global RV pump function. In patients with PH, the impairment of RV pump function seems to be mainly driven by the progressive reduc- tion of RV radial displacement. Background. Right atrial (RAP) and pulmonary artery pressures (PAP) are independ- ent predictors of outcome in pts with cardiovascular disease. In current practice, evaluation of RAP plays a key role in the hemodynamic assessment of patients and is a prerequisite for the noninvasive evaluation of PAP. Echocardiographic estimates of RAP and PAP have been validated against right heart catheterization (RHC) in gen- eral population,

Cardiovascular manifestations of tertiary syphilis infections are uncommon, but represent an important cause of mortality and morbidity. Syphilitic aortitis is characterized by aortic regurgitation, dilatation of ascending aorta and ostial coronary artery lesions. We report a case of 36 years old man admitted to our hospital for acute anterior ST segment elevation myocardial infarction complicated with cardiogenic shock (hypotension 75/50 mmHg). Transthoracic echocardiography revealed a dilated left ventricle with severe systolic dysfunction (ejection fraction = 25%), severe mitral regurgitation, moderate aortic regurgitation and mildly dilated ascending aorta. Coronary angiography showed a severe ostial lesion of left main coronary artery which was treated by urgent stent implantation and an intra-aortic contrapulsation balloon was implanted. Blood tests for syphilitic infection were positive. The patient was discharged with treatment including benzathine penicillin. In our case, we present an acute manifestation of a syphilitic ostial left main stenosis treated by primary percutaneous coronary intervention in acute myocardial infarction. Long term follow-up of the patient is crucial as a result of potential rapid in-stent restenosis caused by continuous infection of the ascending aorta. This case is particular because it shows that syphilitic aortitis can be diagnosed in acute settings, like ST segment elevation myocardial infarction.

Objective: Fibromuscular dysplasia (FMD) mostly affects young women and more specifically, renal arteries in the distal or segmental segments. Preeclampsia is a hypertensive disorder unique in pregnancy, consistently involving the kidney. The renin-angiotensin system is implicated in the pathogenesis of both preeclampsia and FMD. When these two entities meet, the outcome can be poor. Design and method: We report the case of a 27 years old female with a history of hypertension, recent pregnancy loss (27 weeks) due to preeclampsia and Hellp syndrome, admitted for uncontrolled hypertension (max. 240 mmHg). Results: Upon admission, high blood pressure (BP = 160/110mmHg), normal cardiovascular (including ECG), respiratory and central nervous system examinations were determined, as well as no detectable heart or vascular (including abdominal) bruits. The blood tests revealed elevation of plasmatic renine (286 pg/ml), negative urinary metanephrines, thyroid hormones, prolactine, testosterone, dehydroepiandrosterone, dehydroepiandrosterone-sulfate and 17-OH progesterone, as well as normal creatinine clearance. Ecocardiography showed moderate concentric hypertrophy and normal heart function. The probability of secundary hypertension was outlined. Renal ecography showed a difference between kidneys >1.5 cm (left kidney 12.6 cm > right kidney 9.75 cm). Renal arteriography revealed important stenosis (90%) in the distal segment of the right renal artery. Consequently, balloon angioplasty for the stenotic lesion was applied. Patient's evolution improved dramatically after the procedure (absent headaches and controlled BP). Two weeks after discharge, 24 hours’ Holter monitoring BP showed values below 140/90mmHg without any additional antihypertensive treatment other than beta-blockers and double platelet anti-aggregant. Conclusions: The case was characterized by renovascular hypertension due to fibromuscular dysplasia of renal artery diagnosed in a young female after 27-weeks’ pregnancy loss due to preeclampsia. The report underlines the difficulty of reaching a correct diagnosis and the importance of its’ timing.

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary arterial hypertension (PAH). Because of the similar clinical picture of dyspnea on exertion and signs of right heart failure, PVOD is difficult to distinguish from idiopathic PAH. However, the distinction is mandatory because PVOD has a worse prognosis and, more importantly, the administration of PAH specific therapy (vasodilators) can precipitate severe acute pulmonary oedema. We present a challenging case of PAH in a patient with systemic sclerosis in whom a marked decrease in functional capacity after the initiation of bosentan therapy led to the diagnosis of PVOD. Management of PVOD patients is challenging and referral for lung transplantation should be done at the moment of diagnosis.

Objective: Fibromuscular dysplasia (FMD) mostly affects young women and more specifically, renal arteries in the distal or segmental segments. Preeclampsia is a hypertensive disorder unique in pregnancy, consistently involving the kidney. The renin-angiotensin system is implicated in the pathogenesis of both preeclampsia and FMD. When these two entities meet, the outcome can be poor. Design and method: We report the case of a 27 years old female with a history of hypertension, recent pregnancy loss (27 weeks) due to preeclampsia and Hellp syndrome, admitted for uncontrolled hypertension (max. 240 mmHg). Results: Upon admission, high blood pressure (BP = 160/110mmHg), normal cardiovascular (including ECG), respiratory and central nervous system examinations were determined, as well as no detectable heart or vascular (including abdominal) bruits. The blood tests revealed elevation of plasmatic renine (286 pg/ml), negative urinary metanephrines, thyroid hormones, prolactine, testosterone, dehydroepiandrosterone, dehydroepiandrosterone-sulfate and 17-OH progesterone, as well as normal creatinine clearance. Ecocardiography showed moderate concentric hypertrophy and normal heart function. The probability of secundary hypertension was outlined. Renal ecography showed a difference between kidneys >1.5 cm (left kidney 12.6 cm > right kidney 9.75 cm). Renal arteriography revealed important stenosis (90%) in the distal segment of the right renal artery. Consequently, balloon angioplasty for the stenotic lesion was applied. Patient's evolution improved dramatically after the procedure (absent headaches and controlled BP). Two weeks after discharge, 24 hours’ Holter monitoring BP showed values below 140/90mmHg without any additional antihypertensive treatment other than beta-blockers and double platelet anti-aggregant. Conclusions: The case was characterized by renovascular hypertension due to fibromuscular dysplasia of renal artery diagnosed in a young female after 27-weeks’ pregnancy loss due to preeclampsia. The report underlines the difficulty of reaching a correct diagnosis and the importance of its’ timing.

Contact address: Cristina Bucşa, MD, Fundeni Clinical Institute, Bucharest. E-mail: cristinabucsa@yahoo.com INTRODUCTION Many studies show that the renal transplantation is now seen as the best treatment for patients with end-stage renal disease (ESRD) because the renal transplant recipients have a better quality of life and a longer survival compared to patients on dialysis1. Still, the cardiovas-