A 65 year-old woman with an echodense mitral annular mass.

Caseous calcification of the mitral annulus (CCMA) is a rare variant of mitral annular calcification which usually represents an incidental finding during cardiac imaging. Differential diagnosis from significant lesions such as myocardial abscesses or tumours may be problematic. Herein, we present the case of a 65 year-old woman with CCMA who was managed conservatively. Besides providing useful diagnostic clues, we briefly discuss management issues for this under-recognised clinical entity.

Left ventricular geometry and systolic function improvement after percutaneous closure of aortic prosthetic paravalvular leak.

Paravalvular leak (PVL) represents a potential complication of valve replacement surgery. The 'gold standard' treatment for patients with severe prosthetic dysfunction, hemolysis or symptoms, is surgery. Reoperative valve surgery for such patients is related to high morbidity and mortality rate. The percutaneous closure of PVL is feasible and safe, when performed at experienced interventional cardiologic centers. Herein, the case is reported of a patient who showed a significant improvement in left ventricular systolic function after transcatheter closure of an aortic prosthetic PVL with an Amplatzer duct occluder II (ADO II). A review of the current literature is also provided.

Updated knowledge and practical implementations of stress echocardiography in ischemic and non-ischemic cardiac diseases: An expert consensus of the Working Group of Echocardiography of the Hellenic Society of Cardiology.

Stress echocardiography (SE) is a well established and valid technique, widely used for the diagnostic evaluation of patients with ischemic and nonischemic cardiac diseases. This statement of the Echocardiography Working Group of the Hellenic Society of Cardiology summarizes the consensus of the writing group regarding the applications of SE, based on the expertise of their members and on a critical review of present medical literature. The main objectives of the consensus document include a comprehensive review of SE methodology and training-which focus on the preparation, the protocols used, the analysis of the SE images, and updated, evidence-based knowledge about SE applications on ischemic and nonischemic heart diseases, such as in cardiomyopathies, heart failure, and valvular heart disease.

Echocardiography in Pulmonary Arterial Hypertension: Is It Time to Reconsider Its Prognostic Utility?

Pulmonary arterial hypertension (PAH) is characterized by an insult in the pulmonary vasculature, with subsequent right ventricular (RV) adaptation to the increased afterload that ultimately leads to RV failure. The awareness of the importance of RV function in PAH has increased considerably because right heart failure is the predominant cause of death in PAH patients. Given its wide availability and reduced cost, echocardiography is of paramount importance in the evaluation of the right heart in PAH. Several echocardiographic parameters have been shown to have prognostic implications in PAH; however, the role of echocardiography in the risk assessment of the PAH patient is limited under the current guidelines. This review discusses the echocardiographic evaluation of the RV in PAH and during therapy, and its prognostic implications, as well as the potential significant role of repeated echocardiographic assessment in the follow-up of patients with PAH.

Reduced global longitudinal strain at rest and inadequate blood pressure response during exercise treadmill testing in male heterozygous familial hypercholesterolemia patients.

BACKGROUND: Heterozygous familial hypercholesterolemia (heFH) is a genetic disorder leading to premature coronary artery disease (CAD). We hypothesized that the subclinical pathophysiologic consequences of hypercholesterolemia may be detected before the occurrence of clinically overt CAD by stress testing and myocardial strain imaging. PATIENTS-METHODS: We evaluated the treadmill tests (ETTs) of 46 heFH men without known arterial hypertension/diabetes mellitus/vasculopathy like CAD and of 39 healthy men matched for age, baseline systolic/diastolic blood pressure (BP) and heart rate (HR), using Bruce protocol. Global longitudinal strain (GLS) of the left ventricle (LV) additionally to ejection fraction was obtained. RESULTS: heFH men reached a significantly higher peak systolic and diastolic BP compared to controls (p = 0.002 and p < 0.001, respectively). Mean rate pressure product was significantly higher in heFH patients (p = 0.038). Both duration of the ETT and workload in metabolic equivalents was lower in the heFH group (p < 0.001 and p < 0.001, respectively). Baseline to peak rise of systolic and diastolic BP in heFH men was higher (p = 0.008 and p < 0.001 for systolic and diastolic BP, respectively). Furthermore, heFH men had higher rise of HR from baseline to peak, compared to controls; (p = 0.047). GLS in heHF men was slightly decreased (p = 0.014), although the ejection fraction was similar in both groups. CONCLUSION: heFH men have a higher rise in systolic/diastolic BP during ETT, which may reflect early, preclinical hypertension. Furthermore, slight impairment of LV GLS is present, despite the absence of apparent myocardial dysfunction in conventional 2D echocardiography.

Epidemiology and Management of Chronic Thromboembolic Pulmonary Hypertension in Greece. Real-World Data from the Hellenic Pulmonary Hypertension Registry (HOPE).

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/m2, 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).

Intracoronary infusion of selected autologous bone marrow stem cells improves longitudinal myocardial strain and strain rate in patients with old anterior myocardial infarction without recent revascularization.

AIMS: We sought to evaluate the efficacy of intracoronary infusion of selected bone marrow stem cells (BMSCs) in patients with remote, anterior non-viable MI by the use of tissue Doppler imaging. METHODS AND RESULTS: We infused selected CD133+ and CD133-CD34+ BMSCs in 10 patients enrolled in the study. Peak systolic strain rate, maximum strain during the cardiac cycle (epsilon(max)), strain during ejection time (epsilon(et)), and post-systolic strain (epsilon(ps)) were measured. Peak systolic strain rate (-0.69 +/- 0.2 vs. -1.15 +/- 0.27, P = 0.001), epsilon(max) (-9.87 +/- 3.30 vs. -15.57 +/- 5, P = 0.006), and epsilon(et) (-7.45+/-2.86 vs. -10.92 +/- 4.45, P = 0.015) improved significantly during the rest study 6 months after cell infusion. Low-dose inotropic challenge also showed significant improvement of longitudinal deformation indices in the follow-up study. Global ejection fraction did not improve significantly after cell therapy. CONCLUSION: Intracoronary infusion of selected BMSCs in patients with remote, anterior, non-viable myocardial infarction is safe and leads to improvement of longitudinal deformation indices 6 months after the infusion.

Improving Survival in Patients with Pulmonary Arterial Hypertension: Focus on Intravenous Epoprostenol.

Pulmonary arterial hypertension represents a devastating disease, causing progressive increase of pulmonary vascular resistance leading to right ventricular dysfunction and death. Therapeutic management has rapidly advanced in recent years due to improved understanding of pathophysiology and new drugs have been developed; however, survival remains poor. Oral agents as phosphodiesterase type V inhibitors, the soluble guanylyl cyclase stimulator riociguat, the prostacyclin receptor agonist selexipag and the endothelin receptor antagonists have each achieved evidence-based validation and are recommended for pulmonary arterial hypertension. Initial oral monotherapy or combination therapy is recommended for patients with low or intermediate risk according to each patient's risk stratification. Intravenous epoprostenol is a synthetic prostacyclin and the first drug approved for the disease. Although it represents the only treatment shown to reduce mortality, it is underused. Survival rates for patients treated with oral combination drug therapies are lower than those for patients treated with initial combination therapies including intravenous epoprostenol. This raises the interesting question of whether intermediate risk pulmonary arterial hypertension patients should be routinely introduced to therapies including intravenous epoprostenol rather than combination oral therapies.

Pulmonary arterial hypertension associated with interferon-beta treatment for multiple sclerosis. Case report and literature review.

Drug-Induced Pulmonary Arterial Hypertension (PAH) represents a well-known entity, predominantly related to anorexigens. Interferon-ß (IFN) is considered to be a drug with a possible risk of inducing PAH. We report a patient with Multiple Sclerosis treated with IFN-ß who diagnosed with PAH and her course of disease under specific PAH drug therapy. A review of the literature in IFN-ß-induced PAH is provided.

Prognostic significance of renal function in patients undergoing dobutamine stress echocardiography.

BACKGROUND: Dobutamine stress echocardiography (DSE) is used for risk stratification of patients with suspected coronary artery disease (CAD). However, the prognostic value of DSE among the entire strata of renal function has yet to be determined. We assessed the prognostic value of renal function relative to DSE findings. METHODS: We studied 2292 patients, divided into 729 (32%) patients with normal renal function [creatinine clearance (CrCl) >90 ml/min] and 1563 (68%) with renal dysfunction, classified as mild (CrCl: 60-90 ml/min) in 933, moderate (CrCl: 30-60 ml/min) in 502 and severe (CrCl < 30 ml/min) in 128 patients. All patients underwent DSE for the evaluation of known or suspected CAD and were followed for a mean of 8 years. RESULTS: New wall motion abnormalities during DSE and mildly, moderately and severely abnormal CrCl were powerful independent predictors for all-cause mortality, cardiac death and hard cardiac events (cardiac death and non-fatal myocardial infarction). Kaplan-Meier curves demonstrated that patients with normal DSE and renal dysfunction have greater probability for cardiac death and hard cardiac events compared to those with normal renal function. The warranty of a normal DSE in the presence of moderate renal dysfunction was 15 and 36 months for 10 and 20% risk for cardiac death and hard cardiac events, respectively. CONCLUSIONS: The presence and severity of renal dysfunction has additional independent prognostic value over DSE findings. The low-risk warranty period after a normal DSE is determined by the severity of renal dysfunction.

Transthoracic Doppler echocardiography assessment of left anterior descending artery flow in patients with previous anterior myocardial infarction.

AIM: We tested the hypothesis that shortening of diastolic pressure half time (PHT) of left anterior descending (LAD) coronary flow in patients with old reperfused anterior myocardial infarction (MI) is related to the presence of permanent myocardial damage of the reperfused area. METHODS AND RESULTS: We studied 49 patients divided into: group A: 15 patients with previous anterior MI and evidence of myocardial scar; group B: 10 patients with previous anterior MI and no evidence of myocardial scar and group C: 24 patients without anterior MI. All patients underwent coronary angiography at least 6 months after an index event and any reperfusion procedure. Group A patients had lower PHT (199 +/- 62 ms) than group C (377 +/- 103 ms, p = 0.0001) and group B (316 +/- 154 ms, p = 0.029) patients. No other LAD flow velocity parameter differed among the 3 groups. A PHT value of 265 ms discriminated patients with scarred anterior wall with a sensitivity of 79% and a specificity of 94% (0.88, p < 0.001). CONCLUSION: Shortening of the LAD flow diastolic PHT in patients with remote, reperfused anterior MI reflects scarred myocardial tissue in the anteroapical wall while patients who maintain diastolic wall thickness after an acute coronary syndrome have PHT similar to patients without anterior MI.

Value of myocardial viability estimation using dobutamine stress echocardiography in assessing risk preoperatively before noncardiac vascular surgery in patients with left ventricular ejection fraction <35%.

Patients with heart failure (HF) scheduled for vascular surgery have an increased risk of adverse postoperative outcome, and stratification usually depends on dichotomous risk factors. A quantitative prognostic model for patients with HF was developed using wall motion patterns during dobutamine stress echocardiography (DSE). A total of 295 consecutive patients (mean age 67 +/- 12 years) with ejection fraction < or =35% were studied. During DSE, wall motion patterns of dysfunctional segments were scored as scar, ischemia, or sustained improvement. Cardiac death and myocardial infarction were noted perioperatively and during 5 years of follow-up. Of 4,572 dysfunctional segments; 1,783 (39%) had ischemia, 1,280 (28%) had sustained improvement, and 1,509 (33%) had scar. In 212 patients, > or =1 ischemic segment was present; 83 had only sustained improvement. Perioperative and late cardiac event rates were 20% and 30%, respectively. Using multivariate analysis, number of ischemic segments was associated with perioperative cardiac events (odds ratio per segment 1.6, 95% confidence interval 1.05 to 1.8), whereas number of segments with sustained improvement was associated with improved outcome (odds ratio per segment 0.2, 95% confidence interval 0.04 to 0.7). Multivariate independent predictors of late cardiac events were age and ischemia. Sustained improvement was associated with improved survival. In conclusion, DSE provides accurate risk stratification of patients with HF undergoing vascular surgery.

Outcomes after a left anterior descending artery endarterectomy in advanced coronary artery disease.

BACKGROUND: Coronary endarterectomy albeit infrequently utilized remains a pivotal treatment modality for advanced atherosclerotic heart disease. Benefits of coronary endarterectomy are explored in terms of better mid-term survival, freedom of major adverse cardiac and cerebrovascular events and improved left ventricular ejection fraction. METHODS: 50 patients with coronary artery disease including extensive diffuse LAD disease underwent a left anterior descending artery endarterectomy with coronary by-pass grafting and left internal mammary artery as conduit between 2006 and 2014. Prospective evaluation was performed on an outpatient basis with physical examination, echo recordings of ejection fraction and LAD flow reserve for 24 up to 60months. RESULTS: Study group was constituted by a male to female ratio 4:1 and mean age 62.4years old. Pre-operative characteristics included patients with age<60years old and gensini score>60 in 42.1% while patients with age>60years old had gensini score (21-60) in 63.4%. Furthermore, males were affected more severely by atherosclerosis than females. Postoperative anterior wall contractility of left ventricle was improved (56% pre-op vs. 66% post-op) and hypokinesis reduced (34% pre-op vs. 24% post-op). No deaths were recorded for a mean follow-up of 48months. Also, MACCE were recorded in 8% patients. Post-operative LAD flow reserve was normal in 66% and reduced in 33% of cases. Finally, gensini score preoperatively affects mid-term flow reserve postoperatively (p<0.05). CONCLUSION: Coronary endarterectomy presents a viable modality that preserves myocardial function and restores LAD flow in patients with diffuse atherosclerotic LAD. Also postoperative adverse effects were minimal while mid-term flow reserve was affected by preoperative factors.

Percutaneous transcatheter closure of aorto-right ventricular fistula using the Amplatzer duct occluder.

The occurrence of aorto-right ventricular fistula after an aortic valve replacement is rare. If it remains untreated, this condition can result in heart failure and could thus significantly compromise patient survival. Surgical closure is the treatment of choice; however, transcatheter closure has been attempted with relatively acceptable results. Here, we report on a patient who presented with heart failure with an aorto-right ventricular fistula that was present for nine years following aortic valve replacement. Successful transcatheter closure of the fistula with the use of the Amplatzer duct occluder was performed, suggesting that the percutaneous approach is an efficient technique for the treatment of such fistulae.

Cardiac Dual-source Computed Tomography for the Detection of Left Main Compression Syndrome in Patients with Pulmonary Hyper-tension.

INTRODUCTION: Left Main Compression Syndrome (LMCS) represents an entity described as the extrinsic compression of the left main coronary artery (LMCA) by a dilated pulmonary artery (PA) trunk. We examined the presence of LMCS in patients with pulmonary hypertension (PH) using dual-source computed tomography (DSCT), as a non-invasive diagnostic tool. METHODS: The following parameters were measured: PA trunk diameter (PAD), the distance between PAD and LMCA (LMPA) and the distance between PA and aorta (AoPA). These measurements were related with demographic, echocardiographic, hemodynamic and clinical parameters. Angiography was performed in two patients with LMCS suspected by cardiac computed tomographic angiography. Patients without PH but with angina were examined as controls, using DSCT cardiac angiography to assess the same measurements and to detect the prevalence of coronary artery disease. RESULTS: PA diameter value over 40.00 mm has been associated with PH and LMCS. Furthermore, LMCS did not occur at a distance smaller than 0.50 mm between the PA and the LMCA, and did not correlate with the distance between the PA and the aorta or with cardiac index and NT-proBNP. CONCLUSION: DSCT may represent the initial testing modality in PH patients with dilated PA trunk to exclude LMCS. A periodical rule-out of this rare entity, as assessed by DSCT, in patients with a severely dilated PA seems to be mandatory for PH patients contributing to survival improvement.

First application of coronary flow reserve measurement for the assessment of left main compression syndrome in pulmonary hypertension.

Left main compression syndrome (LMCS) refers to extrinsic compression of the left main coronary artery because of a dilated pulmonary artery trunk. The condition represents an unusual cause of angina, left ventricular dysfunction, and sudden cardiac death in patients with pulmonary hypertension. We present 2 patients with the syndrome who were followed with serial assessments of coronary flow reserve by transthoracic echocardiography to screen for LMCS-related ischemia.

Frequency of coronary artery ectasia in patients undergoing surgery for ascending aortic aneurysms.

We retrospectively studied the coronary arteriograms of 82 consecutive patients who underwent planned surgical repair of an ascending aorta aneurysm and an age-matched control group of 92 consecutive patients who underwent coronary angiography during the same time period. The present study examines the incidence of coronary artery ectasia in patients with aneurysms of the ascending aorta.

Outcome of percutaneous mitral balloon valvuloplasty: comparison of the inoue and retrograde non-transseptal techniques. A single-center experience.

INTRODUCTION: The transseptal Inoue (IN) and to a lesser extend retrograde non-transseptal (RNT) techniques are established procedures for percutaneous mitral balloon valvuloplasty (PBMV) in patients with mitral stenosis. However, a head to head comparison of these two techniques, especially from a single center, has not yet been reported. METHODS: Seventy-two consecutive patients (n = 35 IN and n = 37 RNT) underwent PMBV in our clinic from October 1993 to December 1999. All baseline and procedural characteristics were compared, as well as immediate and long-term outcomes (mean follow-up, 42 12 months) of the patients. RESULTS: Baseline characteristics were similar in the two groups. A successful immediate result was achieved in 91% of IN patients and 89% of RNT patients. After the PMBV, mitral valve area (MVA) increased from 1.04 0.16 cm2 to 1.6 0.3 cm2 and from 1.06 0.23 cm2 to 1.55 0.3 cm2 in the IN group and RNT group, respectively (p = NS). There was a higher percentage of mild mitral regurgitation (MR) after the RNT technique (p = 0.03). Mean fluoroscopy time was 31 16 minutes in the IN group and 39 11 minutes in the RNT group (p = 0.02). After discharge, major adverse cardiac events (MACE: mitral valve replacement, repeat PMBV) occurred in 3 patients (8%) patients in the IN group and 5 patients (13.5%) in the RNT group (p = NS). Follow-up echocardiographic evaluation revealed no significant changes regarding MVA in either group. CONCLUSIONS: The IN and RNT techniques are comparable regarding the achieved MVA, with slightly more frequent MR post-RNT PBMV. IN requires significantly less fluoroscopy time. MACE and event-free survival rates at follow-up were similar in the two groups.

Resolution of Thrombi in Left Atrial Appendage Aneurysm.

Atrial appendage aneurysm is a rare cardiac disease and may be complicated by embolic events and rhythm disturbances. We describe a case of a congenital left atrial aneurysm presenting with a cerebrovascular accident in a 57-year-old female. The diagnosis was made by transesophageal echocardiography, which revealed the presence of intraaneurysmal thrombi. The patient initially received anticoagulant therapy and the thrombi were resolved. Subsequently, she was successfully operated on and the aneurysm was removed. This is the first report of thrombi resolution in a left atrial appendage aneurysm.