Multi-modality imaging of the systemic right ventricle in congenital heart disease.

A comprehensive and structured imaging approach in the evaluation of the systemic right ventricle (sRV) in patients with complete transposition of the great arteries (TGA) after atrial switch procedure and congenitally corrected transposition of the great arteries (ccTGA) is a key for their optimal lifelong surveillance. Despite the improvements in cardiovascular imaging of adults with congenital heart disease (ACHD), the imaging of sRV remains an ongoing challenge due to its complex morphology and the difficulty in applying the existing knowledge for the systemic left ventricle. While cardiac magnetic resonance (CMR) is considered the gold standard imaging method, echocardiographic evaluation is primarily preferred in everyday clinical setting.

Marfan Syndrome beyond Aortic Root-Phenotyping Using Cardiovascular Magnetic Resonance Imaging and Clinical Implications.

Marfan syndrome (MFS) is an inherited autosomal-dominant connective tissue disorder with multiorgan involvement including musculoskeletal, respiratory, cardiovascular, ocular, and skin manifestations. Life expectancy in patients with MFS is primarily determined by the degree of cardiovascular involvement. Aortic disease is the major cardiovascular manifestation of MFS.

Diagnostic and therapeutic dilemmas in a patient with myocarditis, Brugada syndrome and arrhythmic syncope.

We present a case of a previously healthy 23-year-old male who presented with chest pain, palpitations and spontaneous type 1 Brugada electrocardiographic (ECG) pattern. Positive family history for sudden cardiac death (SCD) was remarkable. Initially, clinical symptoms in combination with myocardial enzymes elevation, regional myocardial oedema with late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) and inflammatory lymphocytoid-cell infiltrates in the endomyocardial biopsy (EMB) suggested the diagnosis of a myocarditis-induced Brugada phenocopy (BrP).

Cardiomyopathies in children: An overview.

Paediatric cardiomyopathies form a heterogeneous group of disorders characterized by structural and electrical abnormalities of the heart muscle, commonly due to a gene variant of the myocardial cell structure. Mostly inherited as a dominant or occasionally recessive trait, they might be part of a syndromic disorder of underlying metabolic or neuromuscular defects or combine early developing extracardiac abnormalities (i.e.

Arrhythmia risk stratification late after Tetralogy of Fallot repair with pulmonary regurgitation.

Among the most feared sequelae of repaired Tetralogy of Fallot (TOF) are ventricular arrhythmias and sudden cardiac death (SCD) [1]. The presence of pulmonary regurgitation as a potential risk factor for arrhythmias has sparked interest in a more aggressive strategy for reoperation in TOF patients and chronic pulmonary regurgitation. Although this strategy was initially suggested to be protective against the development of ventricular arrhythmias, timely pulmonary valve replacement (PVR) alone, does not appear to abort the SCD risk as myocardial fibrosis, a clear arrhythmic substrate remains.

Complicated coarctation repair: The importance of three-dimensional cross-sectional imaging in late postoperative assessment.

Coarctation of the aorta (CoA) represents 5%-8% of congenital heart disease patients and is one of the most common causes of neonatal surgical intervention. These patients require close lifelong follow-up due to frequent long-term complications. Although transthoracic echocardiography is the first-line technique for its diagnosis and follow-up, cross-sectional imaging with cardiovascular magnetic resonance (CMR) gives excellent anatomical and functional information, especially in complex CoA.

Right Ventricular Dysfunction and the Effect of Defibrillator Implantation in Patients With Nonischemic Systolic Heart Failure.

Background Patients with nonischemic systolic heart failure are at an increased risk of sudden cardiac death, but more discriminating tools are needed to identify those patients likely to benefit from implantable cardioverter-defibrillator (ICD) implantation. Whether right ventricular (RV) ejection fraction (RVEF) can identify patients with nonischemic systolic heart failure more likely to benefit from ICD implantation is not yet known. Methods In this post hoc analysis of the DANISH trial (Danish Study to Assess the Efficacy of ICDs in Patients with Nonischemic Systolic Heart Failure on Mortality), patients with nonischemic systolic heart failure randomized to ICD or control underwent cardiovascular magnetic resonance.

Identification of myocardial diffuse fibrosis by 11 heartbeat MOLLI T mapping: averaging to improve precision and correlation with collagen volume fraction.

Objectives: Our objectives involved identifying whether repeated averaging in basal and mid left ventricular myocardial levels improves precision and correlation with collagen volume fraction for 11 heartbeat MOLLI T mapping versus assessment at a single ventricular level.

Materials And Methods: For assessment of T mapping precision, a cohort of 15 healthy volunteers underwent two CMR scans on separate days using an 11 heartbeat MOLLI with a 5(3)3 beat scheme to measure native T and a 4(1)3(1)2 beat post-contrast scheme to measure post-contrast T , allowing calculation of partition coefficient and ECV. To assess correlation of T mapping with collagen volume fraction, a separate cohort of ten aortic stenosis patients scheduled to undergo surgery underwent one CMR scan with this 11 heartbeat MOLLI scheme, followed by intraoperative tru-cut myocardial biopsy.

Myocardial edema in Takotsubo syndrome mimicking apical hypertrophic cardiomyopathy: An insight into diagnosis by cardiovascular magnetic resonance.

Myocardial edema is one of the characteristic features in the pathogenesis of Takotsubo syndrome. We report a middle aged man who presented with typical clinical and echocardiographic features of apical variant of Takotsubo syndrome. However, a cardiovascular magnetic resonance study performed 10 days after presentation did not show any apical 'ballooning' but revealed features of an apical hypertrophic cardiomyopathy on cine images.

Effect of personalized external aortic root support on aortic root motion and distension in Marfan syndrome patients.

Objective: Personalized external aortic root support (PEARS) is a novel surgical approach with the aim of stabilizing the aortic root size and decreasing risk of dissection in Marfan syndrome patients. A bespoke polymer mesh tailored to each patient's individual aorta shape is produced by modeling and then surgically implanted. The aim of this study is to assess the mechanical effects of PEARS on the aortic root systolic downward motion (an important determinant of aortic wall stress), aortic root distension and on the left ventricle (LV).

The value of E/Em ratio in the estimation of left ventricular filling pressures: impact of acute load reduction: a comparative simultaneous echocardiographic and catheterization study.

Background: The ratio of the early transmitral flow velocity to the early diastolic tissue velocity (E/Em) has been suggested as a reliable estimate of left ventricular diastolic pressures (LVDP). However, the evidence regarding the ability of E/Em to detect LVDP changes is relatively equivocal. Our aim was to evaluate the validity of the ratio following acute load reduction.

Diagnosing acute aortic syndromes: the role of specific biochemical markers.

Acute aortic syndromes (AAS) remain one of the most challenging medical emergencies. Making a prompt and accurate diagnosis is a race against time, where delay may be disastrous for the patient's life. Prompt and accurate diagnosis using imaging modalities has been available for many years, but the major concern is how the clinician's suspicion should be aroused concerning the possibility of an acute aortic syndrome, especially in cases of atypical clinical presentation and/or poor signs during clinical examination.

Asymptomatic papillary fibroelastoma of the aortic valve in a young woman - a case report.

Echocardiography represents an invaluable diagnostic tool for the detection of intracardiac masses while simultaneously provides information about their size, location, mobility and attachment site as well as the presence and extent of any consequent hemodynamic derangement. A 29-year-old asymptomatic young woman with incidental transthoracic echocardiographic (TTE) discovery of an aortic valve mass is presented. The 2-dimensional TTE showed a mobile, pedunculated mass, attached by a thin stalk to the aortic surface of the right coronary aortic cusp at the junction of its base with the anterior aortic wall.

Are measurements of systolic myocardial velocities and displacement with colour and spectral Tissue Doppler compatible?

Background: Tissue Doppler (TD) in pulsed mode (spectral TD) and colour TD are the two modalities today available in tissue velocity echocardiography (TVE). Previous studies have shown poor agreement between these two methods when measuring myocardial velocities and displacement. In this study, the concordance between the myocardial velocity and displacement measurements using colour TD and different spectral TD procedures was evaluated.

Association of impaired glucose tolerance with increased heart rate and subclinical inflammation.

Introduction: This study was designed to assess possible alterations in heart rate (HR), heart rate variability (HRV) and circulating serum levels of proinflammatory cytokines in patients with impaired glucose tolerance (IGT).

Methods: Forty-five patients, aged 34-68 years, with IGT were compared with 28 age-matched healthy controls. Using a 24-hour ambulatory electrocardiogram, we calculated mean HR during daytime (HR-D), night-time (HR-N) and the entire 24-hour period (HR-24h), as well as time domain HRV parameters.