Haemolytic Anaemia-Related Pulmonary Hypertension.

Haemolytic anaemia represents a risk factor for the development of pulmonary hypertension (PH), currently classified as World Health Organization group 5 PH, and data regarding appropriate therapeutic strategy are limited. A total of 28 patients, 85.7% with thalassaemia and 14.

Transoesophageal echocardiography beyond the echo-laboratory. An expert consensus paper of the working group of echocardiography of the hellenic society of cardiology.

Transoesophageal echocardiography (TOE) is a well-established and valid imaging modality, providing more accurate and of higher quality information than transthoracic echocardiography (TTE) for several specific diagnoses and recently a useful guide of an increasing number of catheter-based and surgical interventions. The present paper represents an effort by the Echocardiography Working Group (WG) of the Hellenic Society of Cardiology to state the essential steps of the TOE exam performed beyond the echo lab: a) in the operating rooms intraoperatively during either transcatheter interventions, or cardiothoracic surgery and b) in the intensive care unit for critically ill patients' monitoring. This paper includes information and tips and tricks about the pre-procedural evaluation, the procedural echocardiographic guidance, and post-procedural evaluation of the result and potential complications.

Practical guidance and clinical applications of transoesophageal echocardiography. A position paper of the working group of echocardiography of the Hellenic Society of Cardiology.

Transoesophageal echocardiography (TOE) is a well-established imaging modality, providing more accurate and of higher quality information than transthoracic echocardiography (TTE) for a wide spectrum cardiac and extra-cardiac diseases. The present paper represents an effort by the Echocardiography Working Group (WG) of the Hellenic Cardiology Society to state the essential steps of the typical TOE exam performed in echo lab. This is an educational text, describing the minimal requirements and the preparation of a meticulous TOE examination.

Caseous calcification of the mitral annulus presenting as recurrent strokes.

Unlabelled: Although caseous calcification of the mitral annulus is usually diagnosed incidentally, it can cause embolic complications. The current report describes the case of caseous calcification revealed by recurrent episodes of stroke in a 64-year-old female patient. After her last ischemic episode, cerebral magnetic resonance imaging confirmed the presence of a thrombus in the right middle cerebral artery.

The impact of cardiovascular comorbidities associated with risk for left heart disease on idiopathic pulmonary arterial hypertension: Data from the Hellenic Pulmonary Hypertension Registry (HOPE).

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.

Balloon Pulmonary Angioplasty in Patients with Chronic Thromboembolic Pulmonary Hypertension in Greece: Data from the Hellenic Pulmonary Hypertension Registry.

Balloon pulmonary angioplasty (BPA) is a novel and promising treatment option for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are ineligible for pulmonary endarterectomy (PEA) and for those with persistent or recurrent pulmonary hypertension after PEA. We present the results of BPA procedures in CTEPH patients included in the Greek Pulmonary Hypertension Registry, evaluating the real-life efficacy and safety. We analyzed data from 180 BPA procedures (2−17/patient, mean 8 ± 4/patient, 1248 dilated vessels, 0−18/session).

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.

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.

The wide spectrum of β-thalassaemia intermedia-induced pulmonary hypertension: two case reports on the possible role of specific pulmonary arterial hypertension therapy.

Pulmonary hypertension (PH) development remains a significant cardiovascular complication of haemoglobinopathies, severely affecting the morbidity and mortality of such patients. According to the 5th World Symposium on PH, PH related with chronic haemolytic anaemias is classified in group 5, mainly due to the multifactorial pathophysiology of PH in this patient population. There are no clear guidelines regarding the management of PH in patients with haemoglobinopathies; the use of specific pulmonary arterial hypertension (PAH) therapy in patients with β-thalassaemia and PH is based on data derived from other forms of PH, expert opinion and small series or case reports.

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.

Sickle cell disease related chronic thromboembolic pulmonary hypertension: challenging clinical scenario.

Sickle cell disease (SCD), a haemoglobinopathy characterized by chronic haemolysis with acute exacerbations and vascular occlusion episodes, may be complicated by pulmonary hypertension. The latter may be caused by chronic thromboembolic disease of pulmonary artery branches and its management is not well-defined. Herein, we present a case of SCD complicated by chronic thromboembolic pulmonary hypertension of subsegmental pulmonary artery branches successfully treated with endothelin receptor antagonists, orally administered prostacyclin analogs and balloon pulmonary angioplasty.

Epidemiology and initial management of pulmonary arterial hypertension: real-world data from the Hellenic pulmOnary hyPertension rEgistry (HOPE).

Pulmonary arterial hypertension (PAH) is a heterogenous clinical entity with poor prognosis, despite recent major pharmacological advances. To increase awareness about the pathophysiology, epidemiology, and management of the disease, large national registries are required. The Hellenic pulmOnary hyPertension rEgistry (HOPE) was launched in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece.

Phenotypic Heterogeneity within Members of a Family Carrying the Same RBM20 Mutation R634W.

Objective: We present the genotypic and phenotypic characterization of a family displaying dilated cardiomyopathy (DCM).

Methods: The proband and his relatives underwent full cardiological assessment. Genetic analysis of the proband was performed with the use of next-generation sequencing technology.

A new phenotype of severe dilated cardiomyopathy associated with a mutation in the LAMP2 gene previously known to cause hypertrophic cardiomyopathy in the context of Danon disease.

Danon disease is a rare X-linked cardiac and skeletal muscle disorder with multisystem clinical manifestations. Genetic defects at the lysosome-associated membrane 2 protein (LAMP2) are the cause of the disorder. Due to the rarity of the disease, there is limited progress in understanding the correlation between genotype and phenotype, and explaining the large variability of the clinical features of the disease.

Ischemic Stroke in a Patient With Quadricuspid Aortic Valve and Patent Foramen Ovale.

Quadricuspid aortic valve (QAV) is a rare congenital aortic valve abnormality. It is less common as compared to bicuspid or unicuspid aortic valve abnormality. QAV causes aortic regurgitation usually in the fifth to sixth decade of life.

Primary cardiac intimal sarcoma masquerading as mitral stenosis.

Intimal sarcomas are a very rare medical entity and usually arise from the pulmonary artery or the thoracic aorta. Sarcomas should be considered in the differential diagnosis in patients with suspected mitral valve disease. Echocardiography should be performed as early as possible to establish a prompt diagnosis and management.

Atrial Myxoma Mimicking Mitral Stenosis.

Myxoma is the most common primary cardiac tumor and is usually located in the left atrium. Clinical manifestation relies in large part on the size, location, and architecture of the tumor. There are many reports in the literature of cardiac myxomas causing syncope, embolism, even myocardial infarction.

Correction of human phospholamban R14del mutation associated with cardiomyopathy using targeted nucleases and combination therapy.

A number of genetic mutations is associated with cardiomyopathies. A mutation in the coding region of the phospholamban (PLN) gene (R14del) is identified in families with hereditary heart failure. Heterozygous patients exhibit left ventricular dilation and ventricular arrhythmias.

Growth rate of an apical left ventricular myxoma using serial two dimensional echocardiographic and computed tomography observations over twelve months: a case report.

Introduction: Myxomas are the most common benign tumors of the heart. They vary widely in size, and little is known about their growth rate. The present case report is, we believe the first in bibliography that provides images of an apical left ventricular myxoma from transthoracic echocardiography and computed tomography scans taken a year apart.

Acute and chronic effects of smoking on myocardial function in healthy heavy smokers: a study of Doppler flow, Doppler tissue velocity, and two-dimensional speckle tracking echocardiography.

Background: The purpose of the study was to evaluate the acute and chronic effect of smoking on left ventricular function in healthy heavy smokers by conventional Doppler flow, tissue Doppler, and two-dimensional speckle tracking echocardiography (2D-STE).

Methods: Echocardiograms were performed in 42 healthy heavy (>20 cigarettes/day) smokers (age 34 ± 5 years), before (group SM-1), 15 minutes (SM-2) and 30 minutes (SM-3) after starting smoking 2 cigarettes. Nonsmokers (n = 41, age 33 ± 4 years) served as controls.

Long term effects of cardiac resynchronization therapy in non-ambulatory NYHA IV heart failure patients.

Background: We aimed at evaluating the long-term effects of cardiac resynchronization therapy (CRT) in nonambulatory New York Heart Association (NYHA) IV heart failure patients (NAIVHFP).

Methods: Eighteen patients, 15 men and three women, eight with ischemic and 10 with nonischemic cardiomyopathy, who underwent biventricular pacemaker implantation while they were in nonambulatory NYHA IV class, were studied. Patients' age was 58 ± 9 years and left ventricular ejection fraction (LVEF) 18 ± 3%.