Ana G. Almeida

Reduced myocardial 123-iodine metaiodobenzylguanidine uptake: a prognostic marker in familial amyloid polyneuropathy.

Background— Transthyretin familial amyloid polyneuropathy is a hereditary form of amyloidosis characterized by sensorimotor and autonomic neuropathy, cardiac conduction defects, and infiltrative cardiomyopathy. Previous studies have suggested that myocardial sympathetic denervation assessed by 123-iodine metaiodobenzylguanidine (MIBG) imaging occurs early in disease progression. However, its prognostic significance was never evaluated. We aimed to study the long-term prognostic value of myocardial sympathetic denervation detected by MIBG imaging in transthyretin familial amyloid polyneuropathy. Methods and Results— A total of 143 individuals with V30M transthyretin mutation underwent Holter, ambulatory blood pressure monitoring, echocardiography, and MIBG imaging. Time to all-cause death was compared with late heart-to-mediastinum MIBG uptake ratio (H/M; either in relation to the estimated lower limit of normal [1.60] or as a continuous variable) using Cox proportional hazards regression. Multivariable analyses were performed to test the prognostic accuracy of clinical, neurological, and cardiovascular parameters. During a median follow-up of 5.5 years, 32 (22%) patients died. Five-year mortality rate was 42% for late H/M <1.60 and 7% for late H/M ≥1.60 (hazard ratio, 7.19; P <0.001). Late H/M was identified as an independent prognostic predictor. Fifty-three patients were submitted to liver transplantation. In comparison with neurophysiological score–matched controls, transplanted patients had lower long-term mortality (hazard ratio, 0.32; P =0.012). Patients with late H/M<1.60 were at higher risk of unfavorable outcome but seemed to have benefited from liver transplantation. Conclusions— Cardiac sympathetic denervation as assessed by MIBG imaging is a useful prognostic marker in transthyretin familial amyloid polyneuropathy.Background— Transthyretin familial amyloid polyneuropathy is a hereditary form of amyloidosis characterized by sensorimotor and autonomic neuropathy, cardiac conduction defects, and infiltrative cardiomyopathy. Previous studies have suggested that myocardial sympathetic denervation assessed by 123-iodine metaiodobenzylguanidine (MIBG) imaging occurs early in disease progression. However, its prognostic significance was never evaluated. We aimed to study the long-term prognostic value of myocardial sympathetic denervation detected by MIBG imaging in transthyretin familial amyloid polyneuropathy. Methods and Results— A total of 143 individuals with V30M transthyretin mutation underwent Holter, ambulatory blood pressure monitoring, echocardiography, and MIBG imaging. Time to all-cause death was compared with late heart-to-mediastinum MIBG uptake ratio (H/M; either in relation to the estimated lower limit of normal [1.60] or as a continuous variable) using Cox proportional hazards regression. Multivariable analyses were performed to test the prognostic accuracy of clinical, neurological, and cardiovascular parameters. During a median follow-up of 5.5 years, 32 (22%) patients died. Five-year mortality rate was 42% for late H/M <1.60 and 7% for late H/M ≥1.60 (hazard ratio, 7.19; P<0.001). Late H/M was identified as an independent prognostic predictor. Fifty-three patients were submitted to liver transplantation. In comparison with neurophysiological score–matched controls, transplanted patients had lower long-term mortality (hazard ratio, 0.32; P=0.012). Patients with late H/M<1.60 were at higher risk of unfavorable outcome but seemed to have benefited from liver transplantation. Conclusions— Cardiac sympathetic denervation as assessed by MIBG imaging is a useful prognostic marker in transthyretin familial amyloid polyneuropathy.

Training and accreditation in cardiovascular magnetic resonance in Europe: a position statement of the working group on cardiovascular magnetic resonance of the European Society of Cardiology

Cardiovascular magnetic resonance (CMR) has become an established imaging modality which provides often unique information on a wide range of cardiovascular diseases. The European Society of Cardiology (ESC) training curriculum reflects the emerging role of CMR by recommending that all trainees obtain a minimum level of training in CMR and by defining criteria for subspecialty training in CMR.1 The wider use of CMR requires the definition of standards for data acquisition, reporting, and training in CMR across Europe. At the same time, training and accreditation in all cardiac imaging methods should be harmonized and integrated to promote the training of cardiac imaging specialists. The recommendations presented in this document are intended to inform the discussion about standards for accreditation and certification in CMR in Europe and the discussion on integrated imaging training. At present, the recommendations in this position statement are not to be interpreted as guidelines. Until such guidelines are available and nationally ratified, physicians will be able to train and practice CMR according to current national regulations. This document has been prepared by the working group (WG) on CMR of the ESC. It aims to make proposals for a European standard for competency and excellence in CMR acquisition and reporting. This is to be achieved by providing recommendations for training of individuals wishing to perform and report CMR studies as well as for the accreditation of centres performing CMR studies and wishing to offer CMR training. The document has been written in concordance with the ESC processes on accreditation and certification. However, it is emphasized that European certification of CMR competency or institutional accreditation in CMR as outlined in this document are not compulsory or regulatory certificates. An individuals right to report and sign clinical studies and an institutions right to provide a CMR service in individual …

Non-compaction cardiomyopathy

Non-compaction cardiomyopathy (NCM) is a myocardial disorder, which is thought to occur due to the failure of left ventricle (LV) compaction during embryogenesis, leading to distinct morphological characteristics in the ventricular chamber.1 It was first described about 80 years ago, in association with complex congenital heart diseases. More recently, Chin et al 2 reported the isolated form of non-compaction LV, and since then many other reports have been published. The involvement of the right ventricle in the non-compaction process has been increasingly identified and the condition is now included among the cardiomyopathies. The nomenclature of this entity has been variable, being known as ‘spongy myocardium’ or ‘persistent embryonic myocardium’, but more frequently known as ‘LV non-compaction’ or NCM. Therefore, the latter term will be the one used in this article. The characteristic features of NCM have been described as including a two-layered ventricular wall, comprising a thinner compact epicardial layer and an inner non-compacted layer, with prominent trabeculations associated with deep, intertrabecular recesses that communicate with the ventricular cavity but not with the coronary circulation.2 ,3 The in vivo diagnosis requires the detection of these typical characteristics using imaging techniques.2 ,3 However, there has been considerable controversy regarding the differentiation from normal LV trabeculation and the relationship with other cardiomyopathies, such as dilated and hypertrophic cardiomyopathies, which may share the same genetic basis and be associated with NCM. The prevalence of NCM varies considerably among different series and is still unknown. Several limitations for this assessment are the different diagnostic criteria, the heterogeneous populations, and the retrospective design of most studies. The reported prevalence of NCM in patients referred to echocardiography laboratories ranges between 0.014–1.26%,4 while in a population based retrospective study in children NCM accounted for 9.5% among cardiomyopathies,5 and in a large …

Non-compaction Cardiomyopathy: Prevalence, Prognosis, Pathoetiology, Genetics, and Risk of Cardioembolism

Left ventricular non-compaction (LVNC) is thought to arise from arrest of the normal process of trabecular remodeling or “compaction” that takes place during embryonic life and is characterized by the presence of a two-layered ventricular wall, with a compact epicardial layer and a non-compacted endocardial layer. It is an uncommon condition that can occur isolated or in association with other disorders, including congenital heart anomalies and mitochondrial or musculoskeletal disorders. Both familial and sporadic forms are recognized, and several responsible genes have been identified, although only a minority of patients can be successfully genotyped. The diagnosis is usually made by echocardiography, but cardiac magnetic resonance imaging has been used increasingly. Management is mainly empirical and directed at the major clinical manifestations: heart failure, arrhythmias, and systemic embolic events. This article will review the major features of LVNC and present new trends in the diagnosis and management of this intriguing condition.

An exploratory panel of biomarkers for risk prediction in pulmonary hypertension: emerging role of CT-proET-1.

BACKGROUND Pulmonary arterial hypertension (PAH) is a rare, deadly condition. Although risk stratification is extremely important for assessment of prognosis and to guide therapy, there is lack of evidence concerning the role of novel biomarkers. In a pivotal study, we sought to comparatively investigate the predictive power of several new biomarkers in PAH. METHODS Patients with prevalent PAH were enrolled in the study protocol, which included clinical, functional and echocardiographic assessment. Blood samples were collected at baseline for determination of NT-proBNP, CT-proET-1, MR-proANP, MR-proADM, copeptin and troponin I. Patients were clinically followed-up up to 12 months for first occurrence of hospital admission due to PAH-related clinical worsening, heart/lung transplantation or all-cause mortality. RESULTS Among the 28 included patients the pre-specified end-point occurred in 8 (29% event rate). There were higher baseline levels of CT-proET-1, copeptin, MR-proANP, NT-proBNP and troponin I in patients who reached the composite end-point. They also had larger right atria. In multivariate Cox regression, CT-proET-1 was the only biomarker associated with increased hazard of reaching the primary composite end-point (hazard ratio per tertile increase = 10.1; 95% CI 2.0 to 50.6). CONCLUSIONS CT-proET-1 provided prognostic information independent of other biomarkers. Importantly, we have provided the first evidence that CT-proET-1 may be superior to commonly used biomarkers.

Update of the European Association of Cardiovascular Imaging (EACVI) Core Syllabus for the European Cardiovascular Magnetic Resonance Certification Exam

An updated version of the European Association of Cardiovascular Imaging (EACVI) Core Syllabus for the European Cardiovascular Magnetic Resonance (CMR) Certification Exam is now available online. The syllabus lists key elements of knowledge in CMR. It represents a framework for the development of training curricula and provides expected knowledge-based learning outcomes to the CMR trainees, in particular those intending to demonstrate CMR knowledge in the European CMR exam, a core requirement in the CMR certification process.

Diastolic Function in Several Stages of Chronic Kidney Disease in Patients with Autosomal Dominant Polycystic Kidney Disease: A Tissue Doppler Imaging Study

Background: This study evaluates the prevalence of diastolic dysfunction (DD) in several stages of chronic kidney disease (CKD) in patients with autosomal dominant polycystic kidney disease (ADPKD). Methods: 107 ADPKD patients performed echocardiographic and Doppler studies and a tissue Doppler imaging (TDI) study. Patients were divided in three groups: group 1, 57 patients with CKD stage I, group 2, 37 patients in stages II and III, and group 3, 13 patients with CKD stages IV and V (not on dialysis). Results: In transmitral Doppler, 1 patient in group 1 compared to 5 in group 2, and 4 in group 3 exhibited DD (p < 0.005); moreover, E/A ratio decreases progressively from group 1 to 3 (p < 0.0001). In TDI, DD was observed in 8 patients in group 1, 17 in group 2, and 8 in group 3 had DD (p < 0.001). Em velocity, the best TDI parameter for DD, correlated with age, renal function and blood pressure. When adjusted for age, increased left ventricular mass index and decreased renal function were independent risk factors of DD. Conclusions: DD occurred progressively as renal function deteriorates in patients with ADPKD and this effect is independently related to age and blood pressure.

Predictors of Functional Capacity in Patients with Pulmonary Hypertension

Introduction: The 6-minute walk test (6 MWT) distance is frequently used in the prediction of pulmonary hypertension (PH) prognosis. However, potential surrogates of this measure have not been established. We aim to describe the clinical, echocardiographic, and laboratorial criteria determining the 6 MWT distance in patients with PH. Methods: In 22 consecutive PH patients, functional capacity was evaluated by the 6 MWT distance and compared with levels of neurohormonal activation biomarkers and echocardiographic parameters for right ventricular (RV) function. Results: A correlation between the 6 MWT distance and several clinical parameters was found: Borg’s pre-test classification (R=-0.46; p=0.038); WHO functional class (p=0.029). Patients with higher levels of aldosterone (R=-0.46; p=0.030), renin (R=-0.43; p=0.046) and mid-regional pro-adrenomedullin (MR-proADM, R=-0.53; p=0.009) had worse 6 MWT performance. The opposite was true for natremia levels (R=0.55; p=0.006). There was a positive correlation between the 6 MWT distance and RV global longitudinal peak systolic strain rate and early diastolic strain rate (R=0.49; p=0.038 and R=-0.55; p=0.018, respectively). On multivariate analysis, only natremia and early diastolic strain rate were predictors of the 6 MWT distance. Conclusion: The 6 MWT distance correlated with renin-angiotensin-aldosterone system activation and parameters for RV myocardial deformation. The new biomarker MR-proADM proved to be useful in the prediction of the functional capacity.

The role of cardiovascular magnetic resonance in takotsubo syndrome

Takotsubo syndrome (TS) is a transient form of left ventricular dysfunction associated with a distinctive contraction pattern in the absence of significant coronary artery disease triggered by stressful events. Several aspects of its clinical profile have been described but it still remains difficult to quickly establish the diagnosis at admission.Cardiovascular magnetic resonance (CMR) has achieved great improvements in the last years, which in turn has made this imaging technology more attractive in the diagnosis and evaluation of TS. With its superior tissue resolution and dynamic imaging capabilities, CMR is currently the most useful imaging technique in this setting.In this review, we propose to comprehensively define the role of CMR in the evaluation of patients with TS and to summarize a set of criteria suitable for diagnostic decision making in this clinical setting.

Simplifying cardiovascular magnetic resonance pulse sequence terminology.

We propose a set of simplified terms to describe applied Cardiovascular Magnetic Resonance (CMR) pulse sequence techniques in clinical reports, scientific articles and societal guidelines or recommendations. Rather than using various technical details in clinical reports, the description of the technical approach should be based on the purpose of the pulse sequence. In scientific papers or other technical work, this should be followed by a more detailed description of the pulse sequence and settings. The use of a unified set of widely understood terms would facilitate the communication between referring physicians and CMR readers by increasing the clarity of CMR reports and thus improve overall patient care. Applied in research articles, its use would facilitate non-expert readers’ understanding of the methodology used and its clinical meaning.