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Undifferentiated connective tissue disease at risk for systemic sclerosis (UCTD-risk-SSc), otherwise referred to as very early-early SSc, is a condition characterized by Raynaud's phenomenon with serum SSc marker autoantibodies and/or typical capillaroscopic findings and unsatisfying classification criteria for the disease. The aim of the present study was to assess the prevalence of right (RV) or left ventricular (LV) systolic and/or diastolic dysfunction by standard echocardiography and tissue Doppler imaging (TDI). Thirty patients with UCTD-risk-SSc (28 female, mean age 47 ± 13 years, range 21-70) and 30 age- and sex-matched controls underwent cardiac assessment by standard echocardiography and TDI. UCTD-risk-SSc patients and controls did not show any difference at standard echocardiography. Despite results falling within the respective normal ranges, TDI pointed out a mild impairment of LV and RV diastolic (E m 15 ± 4 vs. 19 ± 5, p = 0.0004; E/E m 6.1 ± 1.7 vs. 4.8 ± 1.2, p = 0.001; E t 14 ± 3 vs. 16 ± 2, p = 0.02; E t/A t 0.9 ± 0.4 vs. 1.3 ± 0.3, p = 0.002; E/E t 3.5 ± 1.2 vs. 4.2 ± 0.9, p = 0.02) and systolic function (S m 13 ± 3 vs. 15 ± 2 cm/s, p

Aim: A fluid challenge with rapid saline infusion during right heart catheterization has been shown to be useful for the differential diagnosis between pre- and post-capillary pulmonary hypertension. The aim of this study was to evaluate the prognostic relevance of fluid challenge-induced changes in pulmonary hemodynamics in patients with pulmonary arterial hypertension (PAH).Methods: Overall, 118 PAH patients (mean age 57 +/- 15 years, 80 female) underwent hemodynamic measurements before and after rapid saline infusion (7 mL/kg in 10 min) and were followed up for 19 +/- 4 months.Results: Thirty-two patients (27%) had a clinical worsening event defined as the occurrence of one of the following: death, lung transplantation, initiation of parenteral prostanoids, or worsening of PAH (defined as the presence of all of the three following components: a decrease in the 6-minute walk distance of at least 15% from baseline, worsening of PAH symptoms, and need for new PAH treatment). Cardiac index (CI), stroke volume and pulmonary artery compliance were lower whereas right atrial pressure (RAP), the ratio of RAP to pulmonary artery wedge pressure (PAWP) and pulmonary vascular resistance were higher in patients with a clinical worsening event versus patients without events, both at baseline and after fluid challenge (all p < 0.01). At multivariable Cox proportional hazards regression analysis, a post-fluid challenge CI

Several studies have described the adaptive remodeling of the heart during exercise. In some more practiced endurance athletes, there is a disproportionate load on the right ventricle (RV), at least during exercise, and this might be the basis for a chronic pro-arrhythmic RV remodeling. Especially, in these kinds of athletes the recovery after detraining might be incomplete, in particular for RV changes. The observation of acute myocardial injury based on transient elevation of biomarkers and chronic myocardial scar, not completely reversible changes of the RV and an increased prevalence of some arrhythmias support the existence of an "exercise-induced cardiomyopathy." The aim of this paper is to review current knowledge about changes in the right heart in highly trained athletes and how these change influence cardiac function.

Background: Initial combination treatment with ambrisentan and tadalafil (up-front therapy) offers clinical benefits in pulmonary arterial hypertension (PAH) and reduces the risk of clinical failure compared with monotherapy in naive patients. Aims: To assess the efficacy of 12 month up-front therapy with ambrisentan and tadalafil to improve hemodynamic changes in incident PAH patients. Methods: This is a multicentre retrospective analysis of real-world Italian clinical data in 56 patients with newly diagnosed PAH. Clinical evaluation, including demographics, medical history, World Health Organization (WHO) functional class, 6-minutes walk distance (6MWD), and right heart catheterization (RHC) were collected from medical records at baseline and 12 month of follow up. Results: WHO functional class at baseline was: II in 12 patients, III in 38 and IV in 6. Over a median follow-up period of 12 months, 53 (95%) patients are still alive, 6 (11%) of whom received parenteral prostanoids. Ambrisentan-tadalafil association was a associated with significant improvements in WHO-functional class (2.2±0.8 vs 2.8±0.6, p Conclusion: This study shows that initial combination with oral medications ambrisentan and tadalafil offers clinical benefits, and significant haemodynamic improvement in newly diagnosed PAH patients.

Aim: To determine whether right atrial (RA) function has prognostic value in patients with idiopathic pulmonary arterial hypertension (PAH).Methods and results: Overall, 104 patients (70 female, mean age 58 +/- 13 years) with idiopathic PAH underwent standard Doppler echocardiography and strain and strain rate (SR) analysis before right heart catheterization. At a mean follow-up of 22 +/- 7 months, 30 patients (29%) had clinical worsening. On Cox multivariable proportional-hazards regression analysis, RA reservoir function measured as peak longitudinal SR (hazard ratio [HR] 0.5; P < 0.0001), RA area (HR 1.2; P < 0.01), right ventricular (RV) SR (HR 0.6; P < 0.0001), cardiac index (HR 0.79; P < 0.01), and mixed venous oxygen saturation (HR 0.82; P < 0.01) were found to be independent correlates of cardiac events. A RA SR reservoir cut-off value of

AIMS A simple echocardiographic score was designed for diagnosing precapillary vs postcapillary pulmonary hypertension and for discriminating between isolated postcapillary pulmonary hypertension (Ipc-PH) and combined precapillary and postcapillary pulmonary hypertension (Cpc-PH). METHODS The score comprised 7 points (2 for E/e' ratio ≤10, 2 for a dilated non-collapsible inferior vena cava, 1 for a left ventricular eccentricity index ≥1.2, 1 for a right-to-left heart chamber dimension ratio >1 and 1 for the right ventricle forming the heart apex) and was applied to 230 consecutive patients referred for evaluation of pulmonary hypertension. RESULTS Precapillary pulmonary hypertension and postcapillary pulmonary hypertension were diagnosed in 160 and 70 patients, respectively. In the latter, Ipc-PH was found in 51 and Cpc-PH in 19. The echo score was higher in precapillary vs postcapillary pulmonary hypertension patients (4.2 ± 1.7 vs 1.6 ± 1.7, P

Background Fluid challenge may help in the differential diagnosis between pre- and postcapillary pulmonary hypertension (PH). However, the test is still in need of standardization and better defined clinical relevance. Methods Two hundred twelve patients referred for PH underwent a right-sided heart catheterization with measurements before and after rapid infusion of 7mL/kg of saline. PH was defined as mean pulmonary artery pressure≥ 25mmHg, and postcapillary PH was defined as pulmonary artery wedge pressure (PAWP) > 15mmHg. An increase in PAWP≥ 18mmHg was considered diagnostic for postcapillary PH. At baseline, 66 patients received a diagnosis of no PH; 22, of postcapillary PH; and 124, of precapillary PH (mostly pulmonary arterial hypertension). Results After fluid challenge, five of 66 patients with no PH (8%) and eight of 124 with precapillary PH (6%) had the diagnosis reclassified as postcapillary PH. Fluid challenge was associated with an increase in PAWP by 7 ± 2mmHg in postcapillary PH and 3 ± 1mmHg in both precapillary PH and no-PH groups. Between-group differences were significant, but there was overlap. There were no adverse events related to fluid challenge. Prediction bands calculated from quadratic fits of the PAWP responses in pooled control subjects with no PH and patients with precapillary PH helped confirm 18mmHg as the cutoff for diagnosing postcapillary PH. Conclusions Fluid challenge with 7mL/kg saline increases PAWP, more in postcapillary than in precapillary PH or in control subjects with no PH. A cutoff value of 18mmHg allows reclassification of 6%to 8%of patients with precapillary PH or normal hemodynamic characteristics at baseline.

Background: Echocardiography is a valid tool for predicting the presence of pre- or post-capillary pulmonary hypertension (PH). A diastolic pulmonary gradient (DPG) equal or higher than 7 mmHg is a marker of pulmonary vascular disease also in patients with post-capillary PH. Aim: To detect if an echocardiographic score is able to allow a differential diagnosis between isolated post-capillary PH (Ipc-PH) and combined post- and pre-capillary PH (Cpc-PH). Methods: An echo-score, including right-to-left-heart chamber dimension >1 (2 points), dilation and no collapsibility of inferior vena cava (2 points), left ventricular eccentricity index ≥1.2 (1 point), E/e9 ratio ≤10 (1 point), and the right ventricle forming the heart apex (1 point), was applied to 230 consecutive patients referred for evaluation of PH and quasi-simultaneous right heart catheterization RHC (within 1 hour). Results: One-hundred-sixty/230 (70%) patients had pre-capillary PH and 70/230 (30%) had post-capillary PH. Within the 70 patients with post-capillary PH, 51/70 had Ipc-PH and 19/70 had Cpc-PH. There was a moderate linear correlation between Echo score and DPG (R 2 = 0.34). Echo score was higher in pre- vs post-capillary PH patients (4.0±2.0 vs 1.6±1.8, p The sensitivity and the specificity of Echo score ≥3 for pre-capillary PH were respectively 74% and 73%. Considering the 70 patients with post-capillary PH, the sensitivity and the specificity of Echo score ≥3 for Cpc-PH were respectively 68% and 88%. Conclusions: Echocardiography allows a satisfactory differential diagnosis not only between pre- and post-capillary PH, but also between Ipc-PH and Cpc-PH.

Background: Right ventricular (RV) contractile reserve measured as exercise-induced increase in the pulmonary artery systolic pressure (PASP) has been shown to predict survival in severe pulmonary hypertension. However, RV contractile reserve can also be measured by changes in stroke volume (SV), tricuspid annular plane excursion (TAPSE) or tricuspid annulus systolic velocity (S). Aim: To explore the limits of normal and functional significance of these changes in healthy subjects, that is not exactly known so far. Methods and results: We measured PASP, TAPSE, SV, and S in 90 subjects aged 39 ± 13 yrs, 50% female, at rest and at maximum exercise, and estimated limits of normal as mean – 2SD to mean + 2SD. Normal values of exercise-induced changes (Δ) were 4 to 10 mm for TAPSE, 6 to 14 cm/s for S, 0 to 96 ml for SV, 12 to 57 mmHg for PASP and – 1.2 to 0 mm/mmHg for TAPSE/PASP. Limits of normal (shaded) and exercise-induced changes as a function of workload in TAPSE, S, SV, and PASP are shown in Figure 1. As compared to men (n=45), women had decreased ΔS, ΔTAPSE/PASP, ΔPASP and ΔSV, but increased TAPSE/PASP at peak exercise. Aging was associated with decreased ΔS, ΔTAPSE, ΔPASP, ΔSV and TAPSE/PASP. Changes in S, TAPSE/PASP, PASP and SV were directly correlated to workload. Conclusions: These results provide age- and sex-related limits of normal RV contractile reserve as assessed by stress echocardiographic PASP, TAPSE, S and TAPSE/PASP. Right ventricular contractile reserve, however measured, correlates with exercise capacity.

Percutaneous closure is widely recognized as the first therapeutic option in the majority of cases of secundum atrial septal defect (ASD) because of its high effectiveness and safety. Nonetheless, with the progressive increase of implanted devices and follow-up duration, several adverse events, some of them potentially life-threatening, have been reported. We report the case of an asymptomatic aortic erosion that occurred 13 years after the procedure. The main feature of our case is the very late occurrence of a life-threatening asymptomatic complication of ASD percutaneous closure, which should prompt lifelong surveillance in this population.