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.

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).

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.

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.

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.

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.

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.

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.

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 pseudoaneurysm formation: Two cases and review of the literature.

Left ventricular wall rupture (LVWR) comprises a complication of acute myocardial infarction (AMI). Acute LVWR is a fatal condition, unless the formation of a pseudoaneurysm occurs. Several risk factors have been described, predisposing to LVWR. High index of suspicion and imaging techniques, namely echocardiography and computed tomography, are the cornerstones of timely diagnosis of the condition. As LVWR usually leads to death, emergency surgery is the treatment of choice, resulting in significant reduction in mortality and providing favorable short-term outcomes and adequate prognosis during late follow-up. Herein, we present two patients who were diagnosed with LVWR following AMI, and subsequent pseudoaneurysm formation. In parallel, we review the aforementioned condition.

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.

Successful epoprostenol withdrawal in pulmonary arterial hypertension: case report and literature review.

Pulmonary arterial hypertension is a rare and devastating disease characterized by vascular proliferation and remodeling. Epoprostenol, the drug counterpart of the eicosanoid prostacyclin, produced by the vascular endothelial cells, is the drug of choice for this disease. Its capacity to act rapidly and to significantly improve survival prospects in severe pulmonary hypertension patients has been supported by a wealth of evidence. Intravenous epoprostenol was believed to require therapy of indefinite duration. Since 2001, oral drugs have been approved for specific treatment. The availability of newer and less invasive drug therapies for pulmonary arterial hypertension led physicians to withdraw epoprostenol in carefully selected patients. We report a case of successful intravenous epoprostenol interruption in a patient with idiopathic disease. A literature review on epoprostenol withdrawal in pulmonary hypertension in adult patients is also provided.

Complications leading to sudden cardiac death in pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a disease of small pulmonary arteries, characterized by vascular proliferation and remodeling. Progressive increase in pulmonary vascular resistance ultimately leads to right ventricular heart failure and death. PAH-specific drug therapy has improved clinical outcomes and survival. While the survival is better, progression of pulmonary vasculopathy contributes to pulmonary artery dilatation. Left main compression syndrome, pulmonary artery dissection, pulmonary artery rupture, and severe hemoptysis are reported as complications leading to sudden cardiac death, an event encountered more often in PAH patients. The advent of PAH-targeted drug therapy has reduced referral for lung transplantation; however, severe complications require rapid diagnosis, decision making, and possible registration on a lung transplantation waiting list. PAH referral centers provide multidisciplinary emergency care and specific therapeutic management, contributing to improved quality of life and survival for PAH patients. We review the complications leading to sudden death in PAH.

Postpartum pulmonary arterial hypertension: two cases covering a wide spectrum of presentations.

We present two clinical cases of pulmonary arterial hypertension (PAH) diagnosed after pregnancy. Both patients experienced exertional breathlessness during the third month after their uncomplicated delivery, and idiopathic PAH was diagnosed after an integrated assessment. Our first patient, a 30-year-old woman, was vasoreactive, treated with optimally tolerated doses of calcium channel blockers with adequate response, and she has remained in a stable and satisfactory condition for the last seven years. The second patient, a 34-year-old woman, was non-vasoreactive and had an inadequate response to combination therapy, including prostanoids. Balloon atrial septostomy was performed as a bridge to lung transplantation, however she expired five months after her delivery.

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.

Transcatheter aortic valve implantation: first Greek experience.

INTRODUCTION: Percutaneous aortic valve replacement represents an alternative to conventional open-heart surgery for selected high-risk patients without the need for sternotomy, aortotomy, or cardiopulmonary bypass. We present the first Greek series of transcatheter prosthetic aortic valve implantation procedures, performed in our centre. METHODS: All 12 patients (age 81 +/- 5 years) had severe, symptomatic, calcific aortic stenosis and were judged not to have a reasonable surgical option by a medical team including experienced cardiac surgeons. The patients' mean logistic EuroSCORE was 34 +/- 15% (min 11%, max 61%). Eight (8) of them underwent transfemoral (SAPIEN, Edwards 23 mm valve in 7 and 26 mm in 1 patient) and 4 transapical (26 mm in 2 and 23 mm in 2 patients) prosthetic aortic valve implantation, all in the cardiac catheterisation laboratory under general anaesthesia. RESULTS: The procedural, in-hospital and 2-month (mean follow up 50 days, min 17, max 122 days) mortality was 0%. The length of hospital stay was 8 +/- 2 days (min 5, max 12 days). The aortic valve area increased from 0.64 +/- 0.14 cm2 to 1.83 +/- 0.14 cm2 and the mean pressure gradient decreased from 57 +/- 23 mmHg to 10 +/- 3 mmHg post-implantation (p<0.001 for both). The patients' mean NYHA functional status improved from 2.8 +/- 0.7 to 1.3 +/- 0.5 at follow-up (p<0.001). CONCLUSIONS: Our initial experience with transcatheter prosthetic aortic valve implantation demonstrates that it can be performed safely and with excellent short and mid-term clinical outcomes.

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.