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Background: Pulmonary arterial hypertension (PAH) is a severe complication among adult patients with congenital heart disease (ACHD). This study presents real-world data on risk stratification, pharmacotherapy and survival rates in PAH-ACHD., Methods: Data from PAH-ACHD patients were analyzed using The Hellenic Pulmonary Hypertension Registry (HOPE), spanning eight specialized centers between 2015 and 2023. Patients were categorized into low, intermediate, and high-risk groups using the ESC/ERS three-strata model to assess 1-year mortality risk., Results: A total of 93 PAH-ACHD patients were included (median age 38.5 years, 60.2 % women). Most patients had an atrial septal defect (37.6 %) or a ventricular septal defect (35.5 %), with 11.8 % presenting with complex ACHD. Eisenmenger syndrome was present in 35.5 % of patients. The proportion of low-risk patients nearly doubled from first to last assessment (24.7 % vs. 40.9 %, p = 0.001). Initially, 52.7 % of patients were on PAH monotherapy, with a subsequent shift towards combination therapy (73.1 %) during follow-up. Over a median follow-up of 5.9 years, 29 patients (31.1 %) died, with 1- and 5-year survival rates of 95.5 % and 81.9 %, respectively. Compared to the high-risk group, low- and intermediate-risk patients exhibited a 70 % and 50 % lower hazard of death, respectively. The lowest survival rates were observed in patients with Eisenmenger physiology., Conclusion: Survival prospects were favorable for the non-high risk patients in this nationwide cohort of PAH-ACHD patients. The observed shift towards combination therapy use may have contributed to the improvement in 1-year ESC mortality risk, underscoring the importance of timely combination therapy with PAH drugs., Competing Interests: Declaration of competing interest Dr. A. Arvanitaki has received travel grants from MSD. Dr. Eftychia Demerouti received honorarium and consultation fees from Actelion Pharmaceuticals-Janssen Hellas, MSD Merck Hellas, Elpen and Galenica. Dr. A. Frogoudaki has received travel grants from Boehringer, ELPEN, Genesis Pharma and Pfizer. Dr. I. Tsangaris has received fees for lectures and/or consultations from Actelion, Bayer, ELPEN, GSK, Janssen, MSD, Pfizer and United Therapeutics. Dr. K. Naka reports lecture fees from Amgen, Astra, Bayer, BMS, Boehringer Ingelheim, Janssen, Merck, Novartis, Novo Nordisk, Pfizer, Sanofi, Servier. Athanasios Manginas reports consultation fees from Astra Zeneca, Bayer, Elpen, Janssen, MSD and Novartis. Dr. Giannakoulas has received fees for lectures and/or consultations from Actelion/Janssen, Bayer, Boehringer Ingelheim, ELPEN Pharmaceuticals, Ferrer/Galenica, GlaxoSmithKline, Gossamer-Bio, Merck Sharp and Dohme, Pfizer, Lilly, and United Therapeutics. The rest of the authors report no conflicts of interest., (Copyright © 2024. Published by Elsevier B.V.)

Background: Continuous ultrafiltration consists a decongestion method for patients with refractory decompensated heart failure with diuretic resistance as it enables the energetic withdrawal of isotonic fluid under controlled rate according to the patient's vital signs, offering decongestion without exceeding plasma refill rate., Case Summary: A 62-year-old male with history of Holt-Oram syndrome with Eisenmenger physiology presented with worsening dyspnoea. Patient initial clinical and laboratory examination, renal vein ultrasound, and echocardiogram were consistent with significant congestion. A combined strategy of intravenous furosemide with early initiation of continuous ultrafiltration at an adjustable rate for 4 days was finally selected. Patient remained haemodynamically stable during the total treatment time and exhibited significant clinical and laboratory improvement. Consecutive renal vein ultrasounds and echocardiograms demonstrated a continuous and steady recession of congestion. During the 4 days of ultrafiltration, total fluid loss was estimated at 42 L. Patient remained asymptomatic without signs of worsened congestion at 1, 3, and 5 months follow-up., Discussion: Our case depicts that continuous ultrafiltration without exceeding plasma refill rate allows an impaired right ventricle to maintain significant preload. This suggests that it might be considered for patients in whom a session of short classic ultrafiltration might have detrimental results regarding cardiac output., Competing Interests: Conflict of interest: None declared., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Whereas younger female patients were diagnosed with idiopathic pulmonary arterial hypertension (IPAH) in 1980s, it is now frequently encountered in elderly patients with cardiovascular comorbidities (CVCs) associated with increased risk for left heart disease. We present data until November 2019 regarding specific features and clinical outcomes of IPAH population from the Hellenic Pulmonary Hypertension Registry (HOPE). Patients were divided into two groups based on the presence of ≥ or <3 CVCs, arterial hypertension, diabetes mellitus, obesity, presence of coronary artery disease, or atrial fibrillation. Overall, 77 patients with IPAH (55.1 [interquartile range, IQR: 24.1] years, 62.8% women) have been recorded. Fifteen patients (19.2%) had ≥3 CVCs, while 25 (32%) were over 65 years old. Patients with ≥3 CVCs were older, presented an almost equal female to male ratio, walked less in 6-min walk test, and had lower mean arterial pulmonary pressure and pulmonary vascular resistance at baseline than patients with less CVCs. Fewer patients with ≥3 CVCs received PAH-specific treatment compared to patients with less comorbidities ( n  = 11 [73.3%] versus n  = 58 [95.5%], p  = 0.02). During a median follow-up period of 3.8 (IQR: 2.7) years, 18 patients died (all-cause mortality 24.3%). Male sex and older age were independent predictors of mortality and/or lung transplantation, while CVCs did not have a significant impact on clinical outcomes. In this nationwide, register-based study, the epidemiology of IPAH involves older patients with CVCs, who seem to have less hemodynamic compromise, but worse functional impairment and are treated less aggressively with PAH pharmacotherapy., Competing Interests: Alexandra Arvanitaki has been the recipient of the International Training and Research Fellowship EMAH Stiftung Karla Voellm, Krefeld, Germany. Anastasia Anthi reports receiving honoraria and consultancy fees from Actelion Pharmaceuticals Hellas, Bayer, ELPEN, GSK, MSD, Lilly, and United Therapeutics. Eftychia Demerouti has been an advisory board member for Actelion Pharmaceuticals Hellas, MSD Hellas, and GlaxoSmithKline and an honorarium speaker for Actelion Pharmaceutical Hellas and MSD Hellas. Ioanna Mitrouska reports receiving honoraria and consultancy fees from Actelion Pharmaceuticals Hellas, Bayer, ELPEN, MSD, and GSK. Katerina K. Naka reports lecture fees from Novartis, Medtronic, and Abbott, while she serves as an investigator in randomized clinical trials and/or registries sponsored by Novartis, Merck, Amgen, BMS, Boehringer Ingelheim, Pfizer, and Actelion Pharmaceutical Hellas, outside the submitted work. Stylianos E. Orfanos reports has been receiving research grants and/or honoraria and/or consultancy fees from Actelion Pharmaceutical Hellas, Bayer, ELPEN, Galenica‐Ferrer, GSK, MSD, Pharmaserve Lilly, PharmaSwiss, Pfizer, and United Therapeutics. Georgia Pitsiou reports receiving honoraria and consultancy fees from Actelion Pharmaceutical Hellas, Bayer, ELPEN, MSD, and GSK. Ioannis Stanopoulos reports receiving honoraria and consultancy fees from Actelion Pharmaceuticals Hellas. Iraklis Tsangaris reports has been receiving honoraria and consultancy fees from Actelion Pharmaceuticals Hellas, Bayer, ELPEN, GSK, Pfizer, Lilly, and United Therapeutics. Athanasios Manginas reports consultation fees from Astra Zeneca, Bayer, ELPEN, Actelion Pharmaceuticals Hellas, and MSD. George Giannakoulas reports has been receiving honoraria and consultancy fees from Actelion Pharmaceuticals Hellas, Bayer, ELPEN, Galenica‐Ferrer, GSK, Pfizer, Lilly, MSD, and United Therapeutics. The remaining authors declare no conflicts of interest. The remaining authors declare no conflicts of interest., (© 2022 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)

Background: Supraventricular and ventricular tachyarrhythmias represent a common complication in grown-up patients with congenital heart defects (CHD) associated with worsening of functional capacity and increased morbidity. The aim of this study was to present the experience of our department on catheter ablation (CA) addressing safety and efficacy concerns., Methods: Consecutive patients with CHD treated with CA between 2002 and 2021 in our hospital were included in this study. The established ablation techniques modified according to the patient's special anatomic characteristics were applied. NYHA functional status along with the underlying cardiac rhythm were recorded during the follow-up., Results: In total, 35 patients (44.9 ​± ​2.7 years, 22 males) underwent CA for the management of either supraventricular or ventricular (n ​= ​2) arrhythmia during the study period. The most common anatomical diagnoses were atrial septal defect (13), Tetralogy of Fallot (5) and transposition of great arteries (3). Atrial fibrillation or atrial flutter was the most frequent baseline diagnosis, observed in 15 (42.8%) patients, followed by intra-atrial reentrant tachycardia in 9 (25.7%) patients and atrioventricular nodal reentry tachycardia (AVNRT) in 5 (14.2%). No significant complication occurred during CA. Patients were followed for a median period of 9 years after the index procedure. At this time, arrhythmia recurrence was observed in 13 patients (37,1%) of whom 6 underwent a redo procedure. Furthermore, 4 patients underwent a permanent pacemaker implantation, while 6 were offered an ICD treatment., Conclusion: CA is a safe method offering significant benefits regarding the management of tachyarrhythmias and preserving functional capacity in adult patients with CHD., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2022 The Authors.)

Rituximab is a monoclonal antibody against the protein CD20. Various lymphomas as well as non-malignant immune disorders are treated with this antibody. Hypersensitivity reactions associated with the use of rituximab include urticaria, hypotension, chest tightness, vomiting, oxygen desaturation and bronchospasm. A very uncommon case of hypertensive crisis and pulmonary edema following rituximab-induced hypersensitivity reaction in an 80-year-old man receiving rituximab for non-Hodgkin lymphoma is reported. Anaphylaxis manifesting as coronary vasospasm following drug treatment, including rituximab, could be proved a serious condition in patients who need specific treatment. In these patients desensitization protocols seem to be mandatory.

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a rare disease with poor prognosis if left untreated, characterized by pulmonary vascular bed obstruction due to unresolving thromboembolic material. The Hellenic pulmonary hypertension registry (HOPE) was launched in Greece in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. In total, 98 patients with CTEPH were enrolled from January 2015 until November 2019. Of these patients, 55.1% represented incident population, 50% were classified in the World Health Organization functional class II and 49% had a history of acute pulmonary embolism. The median values of pulmonary vascular resistance (PVR) and cardiac index were 7.4 (4.8) WU and 2.4 (1.0) L/min/m 2 , respectively, the mean diffusing capacity for carbon monoxide was 74.8 ± 20.6%, the median 6-minute walk distance was 347 (220) meters and the median value of N Terminal-pro brain natriuretic peptide was 506.0 (1450.0) pg/mL. In total, 60.2% of the patients were under pulmonary arterial hypertension-targeted therapy at the time of enrolment; specifically, riociguat was received by 35.7% of the patients and combination therapy was the preferred strategy for 16% of the patients. In total, 74 patients were evaluated for pulmonary endarterectomy (PEA), 34 (45.9%) were assessed as operable but only 23 of those (31.1%) finally underwent PEA. The remaining 40 patients were ineligible for PEA according to the operability assessment and 13 (17.6%) of them underwent balloon pulmonary angioplasty. The age of the non-operable patients was significantly higher than the operable patients ( p < 0.001), while there was no significant difference with regard to the history of coagulopathies between the operable and non-operable patients ( p = 0.33).

The perception that women represent a low-risk population for cardiovascular (CV) disease (CVD) needs to be reconsidered. Starting from risk factors, women are more likely to be susceptible to unhealthy behaviors and risk factors that have different impact on CV morbidity and mortality as compared to men. Despite the large body of evidence as regards the effect of lifestyle factors on the CVD onset, the gender-specific effect of traditional and non-traditional risk factors on the prognosis of patients with already established CVD has not been well investigated and understood. Furthermore, CVD in women is often misdiagnosed, underestimated, and undertreated. Women also experience hormonal changes from adolescence till elder life that affect CV physiology. Unfortunately, in most of the clinical trials women are underrepresented, leading to the limited knowledge of CV and systemic impact effects of several treatment modalities on women's health. Thus, in this consensus, a group of female cardiologists from the Hellenic Society of Cardiology presents the special features of CVD in women: the different needs in primary and secondary prevention, as well as therapeutic strategies that may be implemented in daily clinical practice to eliminate underestimation and undertreatment of CVD in the female population., (Copyright © 2020 Hellenic Society of Cardiology. Published by Elsevier B.V. All rights reserved.)

Objective: Despite the progress in the management of patients with adult congenital heart disease (ACHD), a significant proportion of patients still develop pulmonary hypertension (PH). We aimed to highlight the rate of the complications in PH-ACHD and the predicting factors of cumulative mortality risk in this population., Methods: Data were obtained from the cohort of the national registry of ACHD in Greece from February 2012 until January 2018., Results: Overall, 65 patients receiving PH-specific therapy were included (mean age 46.1±14.4 years, 64.6% females). Heavily symptomatic (New York Heart Association (NYHA) class III/IV) were 53.8% of patients. The majority received monotherapy, while combination therapy was administered in 41.5% of patients. Cardiac arrhythmia was reported in 30.8%, endocarditis in 1.5%, stroke in 4.6%, pulmonary arterial thrombosis in 6.2%, haemoptysis in 3.1% and hospitalisation due to heart failure (HF) in 23.1%. Over a median follow-up of 3 years (range 1-6), 12 (18.5%) patients died. On univariate Cox regression analysis history of HF hospitalisation emerged as a strong predictor of mortality (HR 8.91, 95% CI 2.64 to 30.02, p<0.001), which remained significant after adjustment for age and for NYHA functional class., Conclusions: Long-term complications are common among patients with PH-ACHD. Hospitalisations for HF predict mortality and should be considered in the risk stratification of this population., Competing Interests: Competing interests: GG has acted as a consultant and/or received unrestricted educational or research grants from Actelion, Bayer, MSD, GlaxoSmithKline, Lilly, Pfizer and United Therapeutics. AF received travel grants from Actelion and GlaxoSmithKline., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Myocardial redox state is a critical determinant of atrial biology, regulating cardiomyocyte apoptosis, ion channel function, and cardiac hypertrophy/fibrosis and function. Nevertheless, it remains unclear whether the targeting of atrial redox state is a rational therapeutic strategy for atrial fibrillation prevention., Objective: To review the role of atrial redox state and anti-oxidant therapies in atrial fibrillation., Method: Published literature in Medline was searched for experimental and clinical evidence linking myocardial redox state with atrial fibrillation pathogenesis as well as studies looking into the role of redoxtargeting therapies in the prevention of atrial fibrillation., Results: Data from animal models have shown that altered myocardial nitroso-redox balance and NADPH oxidases activity are causally involved in the pathogenesis of atrial fibrillation. Similarly experimental animal data supports that increased reactive oxygen / nitrogen species formation in the atrial tissue is associated with altered electrophysiological properties of atrial myocytes and electrical remodeling, favoring atrial fibrillation development. In humans, randomized clinical studies using redox-related therapeutic approaches (e.g. statins or antioxidant agents) have not documented any benefits in the prevention of atrial fibrillation development (mainly post-operative atrial fibrillation risk)., Conclusion: Despite strong experimental and translational data supporting the role of atrial redox state in atrial fibrillation pathogenesis, such mechanistic evidence has not been translated to clinical benefits in atrial fibrillation risk in randomized clinical studies using redox-related therapies., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Background: The majority of patients with congenital heart disease (CHD), nowadays, survives into adulthood and is faced with long-term complications. We aimed to study the basic demographic and clinical characteristics of adult patients with congenital heart disease (ACHD) in Greece., Methods: A registry named CHALLENGE (Adult Congenital Heart Disease Registry. A registry from Hellenic Cardiology Society) was initiated in January 2012. Patients with structural CHD older than 16years old were enrolled by 16 specialized centers nationwide., Results: Out of a population of 2115 patients with ACHD, who have been registered, (mean age 38years (SD 16), 52% women), 47% were classified as suffering from mild, 37% from moderate and 15% from severe ACHD. Atrial septal defect (ASD) was the most prevalent diagnosis (33%). The vast majority of ACHD patients (92%) was asymptomatic or mildly symptomatic (NYHA class I/II). The most symptomatic patients were suffering from an ASD, most often the elderly or those under targeted therapy for pulmonary arterial hypertension. Elderly patients (>60years old) accounted for 12% of the ACHD population. Half of patients had undergone at least one open-heart surgery, while 39% were under cardiac medications (15% under antiarrhythmic drugs, 16% under anticoagulants, 16% under medications for heart failure and 4% under targeted therapy for pulmonary arterial hypertension)., Conclusions: ACHD patients are an emerging patient population and national prospective registries such as CHALLENGE are of unique importance in order to identify the ongoing needs of these patients and match them with the appropriate resource allocation., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Introduction: Regular physical activity has been associated with less severity of an acute coronary syndrome (ACS), lower in-hospital mortality rates, and an improved short term prognosis. This study evaluated the relationship between physical activity status and the development of left ventricular systolic dysfunction (LVSD) according to inflammation and sex in elderly patients who had had an ACS., Methods: We analyzed prospectively collected data from 355 male (age 74 ± 6 years) and 137 female (76 ± 6 years) patients who were hospitalized with an ACS. LVSD was evaluated by echocardiography on the 5th day of hospitalization and physical activity status was assessed by a self-reported questionnaire. Inflammatory response was evaluated by measuring C-reactive protein levels. Logistic regression models were applied to evaluate the effect of physical activity status on the development of LVSD and inflammatory response at entry., Results: Physical inactivity had a higher prevalence in women who developed LVSD than in the female patients with preserved systolic function (46% vs. 20%, p=0.02). There was a significant positive association between physical activity levels and ejection fraction in women (p=0.06), but not in men (p=0.30). Multiadjusted logistic regression showed that women who were physically active had 76% lower odds (95%CI: 1-94%) of developing LVSD compared to their sedentary counterparts. Furthermore, physical activity was inversely associated with C-reactive protein levels in both sexes (p=0.08)., Conclusions: Long-term involvement in a physically active lifestyle seems to confer further cardio-protection by reducing the inflammatory response and preserving left ventricular systolic function in elderly female, but not male patients with an ACS.

We assessed the effect of left atrial (LA) function index, LA ejection fraction, LA kinetic energy, and maximal LA volume on 6 months clinical outcome in patients with newly diagnosed systolic heart failure (HF). During a 36-month period, 179 consecutive patients (17% female, mean age 63 ± 14 years) were enrolled. During the follow-up, 46 patients had an event (32% event rate; 15 were fatal). Those with an adverse event were older, had lower creatinine clearance, advanced New York Heart Association stage, higher prevalence of ischemic HF, and lower values for right ventricle systolic wave in the tissue Doppler imaging evaluation, compared with those without an event. Multivariate analysis revealed that LA function and ischemic etiology of HF were the most significant prognostic indicators after index hospitalization. This study reveals the important role of LA function for the short-term prognosis of patients with newly diagnosed systolic HF in sinus rhythm., (© The Author(s) 2013.)

Background: Previous studies using speckle tracking-derived strain for quantification of right ventricular (RV) function in pulmonary hypertension (PHT) have focused on the magnitude of global and regional peak longitudinal systolic strains (PLSS) and systolic strain-related indices of dyssynchrony. The aim of our study was to investigate the pattern of RV contraction and relaxation with the use of the contour and timing of strain and velocity curves in PHT., Methods: The study population consisted of thirty-seven patients with PHT (45 ± 18 years, 16 women) and thirty-seven controls. A complete two-dimensional echo with speckle-tracking-derived longitudinal strain of the basal RV free wall and interventricular septum (IVS) was performed and the cycle length-corrected time to PLSS (SST) and time from PLSS to 50% of PLSS (systolic strain half time-SSHT) in both regions were calculated., Results: Patients with PHT had significantly reduced PLSS (-24.9 ± 2.0% vs -43.2 ± 3.0%, p<0.001) and increased SST (0.47 ± 0.02 vs 0.39 ± 0.02, p=0.043) and SSHT (0.22 ± 0.02 vs 0.16 ± 0.02, p=0.047) in the basal RV free wall compared to controls. Furthermore, peak systolic velocities were observed earlier in the cardiac cycle in both regions in patients with PHT compared to controls., Conclusions: Longitudinal strain curves in the RV free wall reach peak values later in the cardiac cycle and return slower towards the baseline in PHT. Furthermore, peak systolic velocities are observed earlier in the cardiac cycle in both the basal RV free wall and the basal IVS. The above observations effectively illustrate changes in patterns of RV contraction and relaxation caused by PHT., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

We present the case of a young female with a large sporadic left atrial myxoma. Interestingly, despite the tumor's large size, this patient had only mild exertional dyspnea without any embolic events or constitutional symptoms.

Objective: To investigate the effects of atorvastatin on endothelial function and low-grade systemic inflammation in subjects with successful surgery for aortic coarctation repair (SCR)., Design: Open-label study., Setting: Outpatients visiting the adult congenital heart disease department of our hospital., Patients: 34 young people with SCR., Interventions: Patients with SCR received atorvastatin 10 mg/day (n=17) or no treatment (n=17) for 4 weeks. At baseline and at 4 weeks, endothelial function was assessed by flow-mediated dilatation (FMD) of the right brachial artery, and blood samples were obtained. Serum levels of interleukin (IL) 1b, IL-6 and soluble vascular cell adhesion molecule-1 (sVCAM-1) were determined by ELISA., Main Outcome Measures: Effects of treatment on FMD and serum levels of IL-1b, IL-6 and sVCAM-1., Results: FMD in the atorvastatin group was significantly improved after 4 weeks (from 6.46±0.95% to 11.24±1.38%, p<0.01), while remaining unchanged in the control group (from 6.74±0.58% to 6.95±0.53%, p=NS). Even though atorvastatin had no effect on serum IL-6 levels (0.62 (0.37-0.88) pg/ml to 0.53 (0.28-0.73) pg/ml, p=NS), it significantly reduced circulating levels of IL-1b (from 1.17 (0.92-1.77) pg/ml to 1.02 (0.75-1.55) pg/ml, p<0.05) and sVCAM-1 (from 883.4 (660.3-1093.1) ng/ml to 801.4 (566.7-1030.2) ng/ml, p<0.05). No changes were seen in serum levels of IL-6, IL-1b and sVCAM-1 in the control group after 4 weeks compared with baseline (p=NS for all)., Conclusions: Atorvastatin treatment for 4 weeks in subjects with SCR significantly improved endothelial function and suppressed systemic inflammatory status by decreasing circulating levels of IL-1b and sVCAM-1.

Background: Right ventricular (RV) systolic function has been recognized as a prognostic factor in endstage heart failure (HF) patients and in the present study we evaluated the effect of this dysfunction on prognosis in patients with newly-diagnosed systolic HF., Methods and Results: We enrolled 180 consecutive patients with newly diagnosed systolic HF (ischemic or dilated cardiomyopathy). Echocardiographic evaluation was performed to assess biventricular function. Pulse-wave tissue Doppler imaging (TDI) readings were obtained from the lateral tricuspid annulus and the peak systolic annular velocity (Stv) was recorded. Patients were followed for a 2-year period and events (death or HF hospitalization) were recorded. During the follow-up, 79 patients (44%) had an adverse event. An inverse relationship was observed between the height of Stv and the probability of an event (odds ratio (OR) 0.716, 95% confidence interval (CI) 0.583-0.880, P=0.001), after controlling for potential confounders. Furthermore, creatinine clearance (CrCl) was inversely associated with the outcome: a 1-unit increase in CrCl was associated with a 0.98-times lower likelihood of having an event. When the analysis was stratified by CrCl < 60 ml/min or ≥ 60 ml/min, Stv predicted adverse events in both groups (CrCl < 60 ml/min: OR 0.62, 95%CI 0.39-0.98, P = 0.04; CrCl ≥ 60 ml/min: OR 0.78, 95%CI 0.61-1.01, P=0.06)., Conclusions: Pulse-wave TDI readings of peak systolic velocity at the lateral tricuspid annulus, reflecting RV systolic function, has prognostic significance in newly-diagnosed systolic HF patients.

Introduction: transcatheter aortic valve implantation (TAVI) is an emerging technique for the treatment of aortic stenosis. With the advent of percutaneous suture devices for the access point and prosthesis delivery systems of smaller diameter, TAVI has become a truly percutaneous procedure: percutaneous aortic valve replacement (PAVR). Thus, PAVR may be conducted without general anaesthesia (GA)., Methods: We report two centres' experience from PAVR without GA. CoreValve aortic bioprostheses were utilised. The patient selection process and PAVR procedure are described in detail., Results: a total of 30 patients (pts) were treated with PAVR. In 4 pts correction of the initial malposition of the prosthesis required a special technique (2 pts: "snare"; 2 pts: "removing and reinserting"). At 1-month follow up, haemodynamic and clinical improvements were observed: left ventricular ejection fraction increased from 50.8 ± 9.3% to 54.3 ± 8.3% (p=0.02); peak aortic valve gradient decreased from 90.3 ± 26.4 mmHg to 14.8 ± 9.7 mmHg, (p<0.001); NYHA functional class decreased from 3.53 ± 0.93 to 1.45 ± 0.94 (p<0.001). Overall 1-month mortality was 3.3% (1 patient died)., Conclusion: PAVR without general anaesthesia is a feasible technique, however the role of anaesthesiologists is still important.

Viral myocarditis may have various clinical presentations, sometimes mimicking acute myocardial infarction or ischaemia. We describe the case of a young man presenting with acute heart failure, who had electrocardiographic changes suggesting myocardial ischaemia and an episode of sustained ventricular tachycardia. The diagnosis of acute myocarditis was confirmed using cardiac magnetic resonance imaging.

We sought to evaluate the relationship between plasma cytokine levels (sCD14, tumor necrosis factor [TNF]-alpha, and interleukin [IL]-6) and tissue Doppler derived indices of left ventricular systolic and diastolic function in patients with newly diagnosed heart failure. We enrolled 101 consecutive patients (mean age 65+/-13 years) with newly diagnosed heart failure who were hospitalized in our institute. Echocardiographic assessment was performed in all patients during the third day of their initial hospitalization. The pulsed tissue Doppler imaging (TDI) of the systolic and diastolic function of mitral annulus was characterized by the systolic wave Smv, and the diastolic waves: Emv and Amv. Left atrial kinetic energy (LAKE), an index of left atrial function, was calculated using the equation 1/2 x LASV x 1.06 x Amv(2); where LASV is left atrial systolic volume. Furthermore the ratio E/Emv and the flow propagation velocity were also calculated; where E is the rapid mitral filling wave, detected by pulse Doppler. Soluble plasma levels of CD14, TNF-alpha, and IL-6 were measured in all patients during their third day of hospitalization. Linear regression analysis, after adjustment for sex, age, left ventricular ejection function, body mass index, arterial hypertension, smoking, physical activity, creatinine clearance, diabetes mellitus, and blood lipid levels, revealed that IL-6 levels were inversely associated with LAKE (b= - 5422.4+/-2031.5, P=0.03), Sm (b= -0.375+/-0.1, P=0.03), and flow propagation (b= -5.404+/-0.621, P=0.001). CD14 levels were inversely associated with flow propagation (b = -17.655+/-2.6, P=0.001), and positively associated with E/Emv ratio (b=2.58+/-3.6, P=0.002) and A/Amv ratio (b=0.629+/-0.6, P=0.04). TNF-alpha was inversely associated with Smv (b-1.189+/-0.3, P=0.005). This study reveals that increased plasma levels of CD14, IL-6 and TNF-alpha are associated with impaired left atrial function and more advanced left ventricular diastolic and systolic dysfunction, in patients with newly diagnosed heart failure.

A 52-year-old diabetic male was admitted due to 1-month history of fever, fatigue, and mild shortness of breath. Three months prior to admission, he had undergone aortic valve replacement, with a prosthetic one, because of streptococcus viridans endocarditis complicated by severe aortic regurgitation. Transesophageal echocardiogram revealed prosthetic valve endocarditis with dehiscence of the aortic valve and an abscess cavity extending from the aortic root into the ascending aorta. Blood cultures and serology were negative. Due to clinical deterioration, despite antibiotic therapy, the patient was reoperated on and the aortic valve and ascending aorta were replaced with a homograft. Valve culture grew Aspergillus flavus. This case is an example of a rare but of increasing frequency complication after cardiac surgery. Considering the high mortality from this complication, early recognition is of paramount importance.

Background: The role of atherosclerosis in thoracic aortic dissection has not been established yet. Transesophageal echocardiography (TEE) is an imaging modality widely used in the diagnostic evaluation of thoracic aortic dissection, and it can detect aortic atherosclerotic plaques and assess their size and specific characteristics., Methods and Results: One hundred consecutive patients with thoracic aortic dissection and adequate imaging of the thoracic aorta by TEE were studied. The type of dissection (proximal or distal) and the presence and the degree of aortic atherosclerosis were defined. Proximal aortic dissection (Stanford type A) was found in 64 patients. Patients with proximal dissection were younger than those with distal (type B; 58+/-13 vs 67+/-11 years, p<0.001). The prevalence of arterial hypertension was higher in patients with distal dissection compared with those with proximal. Aortic atherosclerosis was present in less patients with proximal than with distal dissection (67% vs 94%, p<0.002). Logistic regression analysis revealed that patients with severe atherosclerosis were 7.6-fold more probable to have type B than type A dissection (p<0.001)., Conclusion: Aortic atherosclerosis is more associated with distal than with proximal aortic dissection.

Background: The longstanding pulmonary regurgitation in patients with repaired tetralogy of Fallot (RTOF) results in right ventricular (RV) failure. The estimation of RV function and reserve in these patients is of great importance, especially for the determination of the proper timing of pulmonary valve replacement. Tissue Doppler imaging (TDI) of the tricuspid annulus has been proved a valuable tool in the evaluation of these patients. Dobutamine stress echocardiography (DSE) in low doses detects the contractility reserve of cardiac myocytes. The aim of our study was to estimate RV reserve in patients with RTOF with the use of DSE and TDI and to examine whether this is related to baseline TDI indices of the tricuspid annulus., Methods: We studied 21 patients with RTOF and 21 age- and gender-matched controls with TDI Doppler at the tricuspid annulus during DSE. TDI measurements were made at baseline and at infusion rates of 10 and 20 microg x kg x min., Results: Patients with RTOF had lower values of TDI indices at baseline and during dobutamine infusion and smaller dobutamine-induced increase of Sa (DeltaSa) (3.8 +/- 1.2 vs. 10.8 +/- 3.6 cm/sec, P < .001) and Aa (3.5 +/- 2.2 vs. 10.0 +/- 3.2 cm/sec, P < .001). A value of DeltaSa < or = 6 cm/sec clearly discriminated patients from controls and could be predicted by values of Sa < 11.5 cm/sec with sensitivity of 95% and specificity of 100%., Conclusions: In patients with RTOF, impaired RV contractile reserve can be documented with TDI of tricuspid annular motion during DSE and is predicted by TDI indices at rest. Its serial estimation may contribute to optimal timing of reoperation.

The authors sought to investigate whether serum uric acid levels are associated with systolic left and right ventricular function, as well as left atrial function in patients with newly diagnosed heart failure. The authors enrolled 106 consecutive patients (mean age 65+/-13 years). Echocardiographic and biochemical assessment was performed during the third day of hospitalization. Pulsed tissue Doppler imaging of the systolic function of mitral and tricuspid annulus was characterized by the systolic waves (Smv and Stv, respectively), expressed in cm/s, and the left atrial function by the Amv wave. Left atrial kinetics was calculated using an equation. Serum uric acid levels were inversely correlated with Stv (P=.005) and left atrial kinetics (P=.05), after controlling for potential confounders. Uric acid levels appear to be correlated with more impaired right ventricular systolic function and decreased left atrial work in patients with heart failure.

Objectives: The purpose of this study was to evaluate the effect of ramipril on endothelial function and inflammatory process in a group of normotensive subjects with successfully repaired coarctation of the aorta (SCR)., Background: Subjects with SCR experience higher long-term cardiovascular risk as a result of the relapse of arterial hypertension or owing to nonreversible structural changes in the pre-coarctation arterial tree. These subjects experience endothelial dysfunction in the right forearm and appear to have elevated levels of proatherogenic inflammatory markers, even in the absence of arterial hypertension., Methods: Twenty young individuals age 27.3 +/- 2.4 years old with SCR 13.9 +/- 2.2 years previously, received ramipril 5 mg/day for 4 weeks in a randomized, cross-over, controlled trial. Endothelial function was evaluated in the right forearm by gauge-strain plethysmography, and serum levels of interleukin (IL)-1b, IL-6, soluble CD40 ligand (sCD40L), and soluble vascular cell adhesion molecule (sVCAM)-1 were determined by enzyme-linked immunosorbent assay., Results: Ramipril improved endothelial function (p < 0.001) and decreased the expression of proinflammatory cytokine IL-6 (p < 0.05) and sCD40L (p < 0.01). Furthermore, ramipril decreased serum levels of sVCAM-1 (p < 0.01) but failed to affect serum levels of C-reactive protein. These effects were independent of blood pressure lowering., Conclusions: Ramipril reversed the impaired endothelial function and decreased the expression of proinflammatory cytokine IL-6, sCD40L, and adhesion molecules in normotensive subjects with SCR. These findings imply that ramipril treatment may have antiatherogenic effects in subjects with SCR, even in the absence of arterial hypertension.

Coronary arteriovenous fistulas are rare. The right coronary artery (RCA) seems to be the most common site of origin, while the right ventricle, right atrium and the main pulmonary artery are the most common draining chambers. We report on a patient with fistulas in both coronary arteries: one arising from the circumflex artery and draining into the coronary sinus (CS), and a fistulous connection between the proximal aorta and the CS, represented by an enlarged and cirsoid RCA. Despite the factthat the non-invasive diagnosis of coronary fistulas has traditionally been difficult, in this case echocardiography led to the suspicion of the anomaly.

Objective: To compare real-time three-dimensional echocardiography (RT3DE) with two-dimensional dobutamine stress echocardiography (2DE) for the detection of myocardial ischaemia, with angiographic validation of the results., Methods: 56 patients (mean (SD) age 64.5 (6.2) years, 38 males), referred for coronary angiography, were examined by 2DE and RT3DE during the same dobutamine stress protocol., Results: All 56 patients completed the stress protocol uneventfully. The mean (SD) acquisition time for the necessary views to evaluate all segments was 26.3 (2.5) s for RT3DE and 58.8 (3.7) s for 2DE (p<0.001). At peak stress, RT3DE had a higher wall-motion score index (1.25 (0.24) by 2DE, 1.30 (0.27) by RT3DE; p = 0.014). The regional wall-motion score for the four apical segments at peak stress was compared; it was 1.35 (0.55) by 2DE and 1.52 (0.69) by RT3DE (p = 0.003). The diagnostic parameters of 2DE versus RT3DE were: sensitivity 73% vs 78%, specificity 93% vs 89% and overall accuracy 86% vs 85%, respectively. In the left anterior descending artery territory, in particular, where RT3DE had higher regional wall-motion scores, it showed a tendency towards higher sensitivity (85% vs 78%), although this difference did not achieve statistical significance., Conclusion: RT3DE identifies wall-motion abnormalities more readily in the apical region than 2DE, which may explain the tendency towards higher sensitivity in the left anterior descending artery territory. RT3DE results were validated using angiography as reference and findings indicate diagnostic equivalence to 2DE, with the advantage of considerable shorter acquisition times.

Introduction: We tested the hypothesis that low dose dobutamine stress echocardiography (LDDSE) combined with tissue Doppler imaging (TDI) can be used for the quantitative assessment of the content of viable myocardium., Methods: Forty-one patients with coronary artery disease and left ventricular dysfunction (ejection fraction < or =40%), already scheduled for revascularisation, underwent echocardiographic assessment of viability at rest and during low-dose dobutamine infusion (2.5 microg/kg/min up to 10 micro/kg/min) at two time points, 2 days before and 3 months after revascularisation. Pulsed-wave TDI was performed at rest and during LDDSE; ejection (Ej), pre-ejection (pre-Ej) and diastolic velocities (Ea, Aa) were recorded at rest and at 10 microg/kg/min dobutamine infusion. Recovery of regional function was defined as improvement of one or more grades 3 months post-revascularisation., Results: A total of 112 vessels were revascularised. Out of 492 segments, 274 segments were characterised as viable and the remaining 218 as non-viable, according to postoperative functional myocardial recovery. Conventional qualitative LDDSE showed a sensitivity of 78% and specificity of 85% in predicting myocardial recovery. Ej, pre-Ej and Ea velocities increased significantly during LDDSE, while Aa velocity did not change significantly. Using ROC curves, the optimal cut-off value for viability assessment was an increase of 0.5 cm/s in Ej during LDDSE (80% sensitivity and 88% specificity, area under the curve 0.801), 0.6 cm/s in pre-Ej (91% sensitivity and 90% specificity, area under the curve 0.890), and 0.44 cm/s in Ea velocity (80% sensitivity and 81% specificity, area under the curve 0.780)., Conclusions: Despite its technical limitations, the measurement of ejection and pre-ejection velocities during dobutamine stimulation appears to be an effective way of predicting myocardial segmental recovery following reperfusion.

Background: Aortic elastic properties are compromised in various states that induce functional and histological changes in the aortic wall. Aortic stenosis is frequent and often requires replacement of the stenotic valve. The purpose of this study was to examine the effect of aortic valve replacement on the aortic root function., Methods: 31 patients, mean+/-SD age 67.2+/-9.1 years with severe aortic stenosis, who underwent aortic valve replacement with a bileaflet mechanical prosthesis, were studied. Aortic root function indices such as aortic cross-sectional compliance (CSC), aortic root distensibility (ARD), and aortic stiffness index (ASI) were calculated with the use of M-mode echocardiography in three sessions: one preoperatively (pre-op), one on day 7 postoperatively (early post-op), and one 6 months postoperatively (late post-op)., Results: Aortic root function deteriorated early post-op (p<0.001 for all) and returned towards pre-op levels late post-op (p=NS for all). CSC changed from 2.84+/-1.98 to 1.37+/-0.92, and 2.30+/-1.11 cm2/mmHg, ARD from 2.21+/-5.60 to 1.01+/-0.67, and 1.79+/-0.96 cm2/dyne, and ASI from 9.72+/-5.60 to 24.65+/-19.10, and 11.51+/-7.85, respectively. Correlations were found between early changes in some aortic root indices and the degree of aortic stenosis, denoting that aortic function deteriorated less in more severe cases of aortic stenosis. None of the late changes were related to aortic valve or left ventricular indices., Conclusions: Aortic valve replacement with a mechanical valve results in a significant but transient impairment of aortic distensibility.

We report a case of a young female patient with congenitally unguarded tricuspid orifice and underdeveloped pulmonary valve leaflets, who had developed severe right-sided congestive heart failure and cardiac cirrhosis early in life. She was admitted to our hospital with deterioration of dyspnea and abdominal tenderness. Hepatocellular carcinoma was diagnosed on the basis of computed tomography findings, high plasma alpha-fetoprotein levels and evidence of chronic hepatitis B viral infection. Transthoracic echocardiogram revealed a mass into the right atrium, considered to be an extension of the tumor.

Objective: We aimed to evaluate myocardial diastolic function in patients with beta-thalassemia major (beta-TM) using pulsed-tissue Doppler imaging (TDI) and flow propagation (VP), in relation to BNP levels., Methods: We enrolled 192 consecutive patients with beta-TM (88 men (25 +/- 6 years) and 104 women (26 +/- 6 years), with normal left ventricular (LV) systolic function. By TDI, diastolic myocardial velocities were sampled at the lateral section of the mitral annulus (Smv, Emv, Amv). From the apical four-chamber view diastolic transmitral flow velocities, including measures of E- and A-waves were performed. The propagation velocity of early flow into the LV cavity was measured by color M-mode Doppler. Plasma BNP levels were measured in all patients., Results: A nonlinear relationship was found between BNP levels and E/A ratio (beta coefficient for the second-order term = 1.4 +/- 0.4, P = 0.001). We also found a positive association of BNP levels with Amv (r = 0.28, P = 0.023), and a reverse with Smv (r =-0.59, P = 0.01) and Emv/Amv (r =-0.36, P = 0.019). Moreover, an inverse relationship was observed between BNP and Vp (r =-0.43, P = 0.012) levels. Finally, a strong positive linear relationship was found between E/Vp ratio and BNP levels (r = 0.76, P < 0.001)., Conclusion: A U-curved association of E/A ratio of transmitral Doppler velocity was revealed with BNP levels, while the tissue Doppler of the Emv/Amv of the mitral annulus movement showed a negative linear association with BNP levels.

Background: Accurate estimation of right ventricular (RV) function in patients with repaired tetralogy of Fallot (RTOF) is difficult, partly due to the presence of tricuspid regurgitation and pulmonary regurgitation and/or stenosis. The aim of the present study was to evaluate RV systolic and diastolic function of adult asymptomatic patients with RTOF by means of tissue Doppler imaging (TDI) and brain natriuretic peptide (BNP) values., Methods: 25 adult patients with RTOF and 25 healthy controls were studied. The following echocardiographic measurements were obtained: RV diameter/left ventricular (LV) diameter (RVD/LVD) and systolic (Sa) and diastolic (Ea, Aa) velocities at the RV free wall tricuspid annulus site. Serum BNP levels were measured as well., Results: Patients with RTOF demonstrated reduced TDI velocities: Sa, 8.16 +/- 1.15 versus 16.43 +/- 1.15 cm/sec (P < .001); Ea, 10.00 +/- 2.18 versus 18.99 +/- 1.00 cm/sec (P < .001); Aa, 5.64 +/- 1.77 vs. 13.69 +/- 0.86 cm/sec (P < .001). Patients with RTOF also had higher BNP levels than controls (85.0 +/- 87.0 vs 5.36 +/- 1.0 pg/mL; P < .001). The increased BNP levels in RTOF patients correlated with the RVD/LVD ratio (r = .521; P < .01)., Conclusions: Our results indicate that although our cohort of patients was asymptomatic, using TDI and BNP allowed us to easily discriminate them from the healthy controls. The ability of TDI to assess ventricular function even in the presence of valvular lesions, as in RTOF patients, makes it a valuable tool in the investigation and follow-up of these patients.