RESUMEN
Heart failure (HF) and chronic kidney disease (CKD) are two pathological conditions with a high prevalence in the general population. When they coexist in the same patient, a strict interplay between them is observed, such that patients affected require a clinical multidisciplinary and personalized management. The diagnosis of HF and CKD relies on signs and symptoms of the patient but several additional tools, such as blood-based biomarkers and imaging techniques, are needed to clarify and discriminate the main characteristics of these diseases. Improved survival due to new recommended drugs in HF has increasingly challenged physicians to manage patients with multiple diseases, especially in case of CKD. However, the safe administration of these drugs in patients with HF and CKD is often challenging. Knowing up to which values ââof creatinine or renal clearance each drug can be administered is fundamental. With this review we sought to give an insight on this sizable and complex topic, in order to get clearer ideas and a more precise reference about the diagnostic assessment and therapeutic management of HF and CKD.
Asunto(s)
Insuficiencia Cardíaca , Insuficiencia Renal Crónica , Humanos , Insuficiencia Cardíaca/terapia , Insuficiencia Cardíaca/tratamiento farmacológico , Insuficiencia Renal Crónica/complicaciones , Insuficiencia Renal Crónica/epidemiología , BiomarcadoresRESUMEN
INTRODUCTION: Micra AV Transcatheter Pacing System (TPS) represents an innovative second-generation leadless pacemaker which represents an effective alternative to conventional devices in selected cases. Intrinsic malfunctions of these devices are rare, requiring sometimes their retrieval. When performed in experienced centers, this procedure is safe. CASE PRESENTATION: We describe a case of sudden battery malfunction of a Micra AV TPS, which required the extraction and the placement of a new pacing system in the right ventricle. DISCUSSION: This case, which has never been reported, highlights the need to a careful fluoroscopic evaluation and the usefulness of remote monitoring.
Asunto(s)
Arritmias Cardíacas , Marcapaso Artificial , Humanos , Resultado del Tratamiento , Diseño de Equipo , Marcapaso Artificial/efectos adversos , Estimulación Cardíaca ArtificialRESUMEN
BACKGROUND AND AIM: Our aim was to evaluate the fluoroscopy time (FT), procedure time (PT) safety and efficacy when using intracardiac echocardiography (ICE) in comparison to transesophageal echocardiography (TEE) guidance for transcatheter closure of Ostium Secundum Atrial Septal Defect (OS-ASD). METHOD: Ninety patients (n = 90) diagnosed with OS-ASD underwent transcatheter closure between March 2006 and October 2021. Fifty-seven patients were treated under ICE guidance, while 33 patients were treated under TEE guidance. RESULTS: Mean age was 43 ± 15 years and 42 ± 10 years in the ICE and TEE groups, respectively. The majority of patients had a centrally placed defect. Median FT was 8.40 min versus 11.70 min (p < .001) in the ICE group compared to the TEE group, respectively. Median PT was 43 min versus 94 min (p < .001) in the ICE group compared to the TEE group, respectively. Both ICE and TEE provided high quality images. All interventions were completed successfully, except for one patient in the ICE group who experienced a device migration, the development of atrial tachycardia in one patient and atrial fibrillation in two patients in the ICE group which spontaneously cardioverted. There were no other complications. CONCLUSION: This study on a consistent cohort of patients with OS-ASD undergoing percutaneous closure suggests that use of ICE is safe and efficacious. Compared to TEE, ICE demonstrated significantly shorter FT and PT, decreasing the entire duration of the procedure and x-ray exposure. No relevant differences were observed in terms of success rate and complications.
Asunto(s)
Fibrilación Atrial , Defectos del Tabique Interatrial , Humanos , Adulto , Persona de Mediana Edad , Ecocardiografía Transesofágica/métodos , Cateterismo Cardíaco/métodos , Defectos del Tabique Interatrial/diagnóstico por imagen , Defectos del Tabique Interatrial/cirugía , Fluoroscopía , Resultado del TratamientoRESUMEN
Telemedicine and remote monitoring devices, including implantable loop recorders (ILR), are increasingly adopted in the cardiologic setting. These are valuable tools in the arrhythmic stratification of patients at risk of sudden cardiac death, providing a tailored therapeutic management to prevent lethal arrhythmias. We report a case of an asymptomatic 18-year-old boy with a family history of syncope and cardiac arrest, who had a diagnosis of Brugada syndrome with an inducible type 1 pattern and carrier of a missense mutation of the SCN5A gene. In light of the risk factors, although not recommended by current guidelines, we decided to proceed with the implantation of an ILR with remote monitoring service. A few months later, an episode of asymptomatic sustained polymorphic ventricular tachycardia was promptly observed by the remote monitoring, leading to a timely implantation of a subcutaneous cardiac implantable defibrillator.
Asunto(s)
Síndrome de Brugada , Desfibriladores Implantables , Telemedicina , Humanos , Masculino , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/genética , Síndrome de Brugada/terapia , Adolescente , Telemedicina/métodos , Medición de Riesgo/métodos , Canal de Sodio Activado por Voltaje NAV1.5/genética , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/terapia , Electrocardiografía , Electrocardiografía Ambulatoria/instrumentación , Electrocardiografía Ambulatoria/métodos , Mutación Missense , Muerte Súbita Cardíaca/prevención & control , Muerte Súbita Cardíaca/etiologíaRESUMEN
PURPOSE OF REVIEW: Heart failure (HF) and erectile dysfunction (ED) are two common conditions that affect millions of men worldwide and impair their quality of life. ED is a frequent complication of HF, as well as a possible predictor of cardiovascular events and mortality. ED deserves more attention from clinicians and researchers. RECENT FINDINGS: The pathophysiology of ED in HF involves multiple factors, such as endothelial dysfunction, reduced cardiac output, neurohormonal activation, autonomic imbalance, oxidative stress, inflammation, and drug side effects. The diagnosis of ED in HF patients should be based on validated questionnaires or objective tests, as part of the routine cardiovascular risk assessment. The therapeutic management of ED in HF patients should be individualized and multidisciplinary, considering the patient's preferences, expectations, comorbidities, and potential drug interactions. The first-line pharmacological treatment for ED in HF patients with mild to moderate symptoms (NYHA class I-II) is phosphodiesterase type 5 inhibitors (PDE5Is), which improve both sexual function and cardiopulmonary parameters. PDE5Is are contraindicated in patients who use nitrates or nitric oxide donors for angina relief, and these patients should be advised to avoid sexual activity or to use alternative treatments for ED. Non-pharmacological treatments for ED, such as psychotherapy or couples therapy, should also be considered if there are significant psychosocial factors affecting the patient's sexual function or relationship. This review aims to summarize the most recent evidence regarding the prevalence of ED, the pathophysiology of this condition with an exhaustive analysis of factors involved in ED development in HF patients, a thorough discussion on diagnosis and management of ED in HF patients, providing practical recommendations for clinicians.
Asunto(s)
Disfunción Eréctil , Insuficiencia Cardíaca , Masculino , Humanos , Disfunción Eréctil/epidemiología , Disfunción Eréctil/etiología , Disfunción Eréctil/terapia , Insuficiencia Cardíaca/terapia , Insuficiencia Cardíaca/tratamiento farmacológico , Calidad de Vida , Inhibidores de Fosfodiesterasa 5/uso terapéutico , Medición de RiesgoRESUMEN
The association between cardiomyopathies (CMPs) and psychiatric disorders is a complex and bidirectional phenomenon that involves multiple mechanisms and factors. CMPs may raise the risk of psychiatric disorders due to the psychological stress, physical limitations, social isolation, or poor prognosis associated with the underlying disease. Psychiatric disorders, on the other hand, can increase the possibility of developing or worsening CMPs due to the behavioral, neuroendocrine, inflammatory, or pharmacological effects of mental illness or its treatment. Moreover, some common genetic or environmental factors may have a relevant influence on both conditions. With this comprehensive review, we sought to provide an overview of the current evidence on the strict and intriguing interconnection between CMPs and psychiatric disorders, focusing on the epidemiology, pathophysiology, clinical implications, and management strategies.
RESUMEN
Historically, cardiovascular prevention has been predominantly focused on stress-induced ischemia, but recent trials have challenged this paradigm, highlighting the emerging role of vulnerable, non-flow-limiting coronary plaques, leading to a shift towards integrating plaque morphology with functional data into risk prediction models. Coronary computed tomography angiography (CCTA) represents a high-resolution, low-risk, and largely available non-invasive modality for the precise delineation of plaque composition, morphology, and inflammatory activity, further enhancing our ability to stratify high-risk plaque and predict adverse cardiovascular outcomes. Coronary artery calcium (CAC) scoring, derived from CCTA, has emerged as a promising tool for predicting future cardiovascular events in asymptomatic individuals, demonstrating incremental prognostic value beyond traditional cardiovascular risk factors in terms of myocardial infarction, stroke, and all-cause mortality. Additionally, CCTA-derived information on adverse plaque characteristics, geometric characteristics, and hemodynamic forces provides valuable insights into plaque vulnerability and seems promising in guiding revascularization strategies. Additionally, non-invasive assessments of epicardial and pericoronary adipose tissue (PCAT) further refine risk stratification, adding prognostic significance to coronary artery disease (CAD), correlating with plaque development, vulnerability, and rupture. Moreover, CT imaging not only aids in risk stratification but is now emerging as a screening tool able to monitor CAD progression and treatment efficacy over time. Thus, the integration of CAC scoring and PCAT evaluation into risk stratification algorithms, as well as the identification of high-risk plaque morphology and adverse geometric and hemodynamic characteristics, holds promising results for guiding personalized preventive interventions, helping physicians in identifying high-risk individuals earlier, tailoring lifestyle and pharmacological interventions, and improving clinical outcomes in their patients.
RESUMEN
Aim: Micra AV represents a leadless endocardial pacing system able to detect atrial contractions providing atrioventricular synchrony. A reduction of myocardial contractility may be detected in case of first-degree atrioventricular block (AVB). Materials & methods: In six patients with first-degree AVB (PQ interval ≥220 msec) was evaluated the left ventricle global longitudinal strain (LV GLS) by speckle tracking (ST) echocardiography during single-lead atrial sensing ventricular pacing (VDD) stimulation as compared with spontaneous rhythm (SR), 24-48 h after Micra AV implantation. Results: A statistically significant difference between the two modalities was observed (LV GLS during SR: -14.7% [interquartile range (IQR) 5.5], LV GLS during VDD pacing: -16.1% [IQR 5.2]; p value = 0.041). Conclusion: Our preliminary results suggest an improvement of myocardial contractility with VDD pacing as compared with SR.
What is this article about? The Micra AV is an electronic device placed in the heart chambers capable to supply the electrical activity of the heart. A reduction of cardiac contractility may be observed in patients with electrical disorders of the heart. What were the results? In six patients affected by electrical cardiac disorders, we observed an improvement of cardiac contractility using Micra AV as compared with the spontaneous electrical activity of the heart. What do the results of the study mean? The results of this study suggest that in patients carrying this electronic device should be preferred a specific modality of activation of the device as compared with the spontaneous electrical activity of the heart in order to improve the contractility of the cardiac walls.
Asunto(s)
Bloqueo Atrioventricular , Defectos de los Tabiques Cardíacos , Marcapaso Artificial , Humanos , Ventrículos Cardíacos/diagnóstico por imagen , Estimulación Cardíaca Artificial/métodos , Bloqueo Atrioventricular/terapia , Atrios Cardíacos/diagnóstico por imagenRESUMEN
Cardiac magnetic resonance (CMR) imaging has witnessed substantial progress with the advent of parametric mapping techniques, most notably T1 and T2 mapping. These advanced techniques provide valuable insights into a wide range of cardiac conditions, including ischemic heart disease, cardiomyopathies, inflammatory cardiomyopathies, heart valve disease, and athlete's heart. Mapping could be the first sign of myocardial injury and oftentimes precedes symptoms, changes in ejection fraction, and irreversible myocardial remodeling. The ability of parametric mapping to offer a quantitative assessment of myocardial tissue properties addresses the limitations of conventional CMR methods, which often rely on qualitative or semiquantitative data. However, challenges persist, especially in terms of standardization and reference value establishment, hindering the wider clinical adoption of parametric mapping. Future developments should prioritize the standardization of techniques to enhance their clinical applicability, ultimately optimizing patient care pathways and outcomes. In this review, we endeavor to provide insights into the potential contributions of CMR mapping techniques in enhancing the diagnostic processes across a range of cardiac conditions.
RESUMEN
The interventricular septum (IVS) is a core myocardial structure involved in biventricular coupling and performance. Physiologically, during systole, it moves symmetrically toward the center of the left ventricle (LV) and opposite during diastole. Several pathological conditions produce a reversal or paradoxical septal motion, such as after uncomplicated cardiac surgery (CS). The postoperative paradoxical septum (POPS) was observed in a high rate of cases, representing a unicum in the panorama of paradoxical septa as it does not induce significant ventricular morpho-functional alterations nor negative clinical impact. Although it was previously considered a postoperative event, evidence suggests that it might also appear during surgery and gradually resolve over time. The mechanism behind this phenomenon is still debated. In this article, we will provide a comprehensive review of the various theories generated over the past fifty years to explain its pathological basis. Finally, we will attempt to give a heuristic interpretation of the biventricular postoperative motion pattern based on the switch of the ventricular anchor points.
RESUMEN
One of the most common complications of tricuspid valve replacement is atrioventricular block (AVB), often requiring permanent pacing. The endocardial pacemaker lead, placed in the right ventricle, may sometimes interfere with the implanted prosthesis, causing its early dysfunction and the need for alternative sites of pacing. To the best of our knowledge, we present the first case of a successful combined percutaneous procedure consisting of the implantation of two leads in the coronary sinus for univentricular bifocal pacing and a transcatheter tricuspid valve-in-valve implantation in a young patient with severe dysfunction of the tricuspid bioprosthesis, requiring permanent pacing for a postsurgical complete atrioventricular block.
Tricuspid valve replacement with surgery can often lead to cardiac rhythm disorders requiring a permanent pacemaker. This device may occasionally damage the tricuspid prosthesis. We present the first case of a combined procedure of tricuspid valve replacement and device implantation distant from the prosthesis without the need for a surgical approach in a young patient with severe tricuspid prosthesis malfunctioning and permanent pacing.
Asunto(s)
Cateterismo Cardíaco , Estimulación Cardíaca Artificial , Seno Coronario , Implantación de Prótesis de Válvulas Cardíacas , Válvula Tricúspide , Humanos , Válvula Tricúspide/cirugía , Implantación de Prótesis de Válvulas Cardíacas/métodos , Seno Coronario/cirugía , Cateterismo Cardíaco/métodos , Estimulación Cardíaca Artificial/métodos , Bloqueo Atrioventricular/terapia , Bloqueo Atrioventricular/etiología , Bioprótesis , Prótesis Valvulares Cardíacas , Insuficiencia de la Válvula Tricúspide/cirugía , Insuficiencia de la Válvula Tricúspide/diagnóstico , Marcapaso Artificial , Femenino , Masculino , AdultoRESUMEN
Premature ventricular contractions (PVCs) are a common form of arrhythmic events, often representing an idiopathic and benign condition without further therapeutic interventions. However, in certain circumstances PVCs may represent the epiphenomenon of a concealed structural heart disease (SHD). Surface 12leads EKG and 24-h dynamic EKG are necessary to assess their main characteristics such as site of origin, frequency and complexity. Echocardiography represents the first-line imaging tool recommended to evaluate cardiac structures and function. Cardiac Magnetic Resonance (CMR) is recognized as a superior modality for detecting structural cardiac alterations, that might evade detection by conventional echocardiography. Moreover, in specific populations such as athletes, CMR may have a crucial role to exclude a concealed SHD and the risk of serious arrhythmic events during sport activity. Some clinical characteristics such as male sex, older age or family history of sudden cardiac death (SCD) or cardiomyopathy, and some electrocardiographic features of PVCs, in particular a right branch bundle block (RBBB) with superior/intermediate axis morphology, the reproducibility of VAs during exercise test (ET) or the evidence of complex ventricular arrhythmias, may warrant a CMR evaluation, due to the high probability of SHD. In this systematic review our objective was to provide an exhaustive overview on the role of CMR in detecting a concealed SHD in patients with high daily burden of PVCs and a normal echocardiographic evaluation, paving the way for a more extensive utilization of CMR in presence of certain high-risk clinical and/or EKG features identified during the diagnostic workup.
Asunto(s)
Ecocardiografía , Complejos Prematuros Ventriculares , Humanos , Cardiopatías/diagnóstico por imagen , Cardiopatías/fisiopatología , Imagen por Resonancia Cinemagnética/métodos , Complejos Prematuros Ventriculares/fisiopatología , Complejos Prematuros Ventriculares/diagnóstico por imagen , Complejos Prematuros Ventriculares/diagnósticoRESUMEN
Cardiomyopathies (CMPs) are a group of myocardial disorders that are characterized by structural and functional abnormalities of the heart muscle. These abnormalities occur in the absence of coronary artery disease (CAD), hypertension, valvular disease, and congenital heart disease. CMPs are an increasingly important topic in the field of cardiovascular diseases due to the complexity of their diagnosis and management. In 2023, the ESC guidelines on cardiomyopathies were first published, marking significant progress in the field. The growth of techniques such as cardiac magnetic resonance imaging (CMR) and genetics has been fueled by the development of multimodal imaging approaches. For the diagnosis of CMPs, a multimodal imaging approach, including CMR, is recommended. CMR has become the standard for non-invasive analysis of cardiac morphology and myocardial function. This document provides an overview of the role of CMR in CMPs, with a focus on tissue mapping. CMR enables the characterization of myocardial tissues and the assessment of cardiac functions. CMR sequences and techniques, such as late gadolinium enhancement (LGE) and parametric mapping, provide detailed information on tissue composition, fibrosis, edema, and myocardial perfusion. These techniques offer valuable insights for early diagnosis, prognostic evaluation, and therapeutic guidance of CMPs. The use of quantitative CMR markers enables personalized treatment plans, improving overall patient outcomes. This review aims to serve as a guide for the use of these new tools in clinical practice.
RESUMEN
Arrhythmic risk stratification in patients with Lamin A/C gene (LMNA)-related cardiomyopathy influences clinical decisions. An implantable cardioverter defibrillator (ICD) should be considered in patients with an estimated 5-year risk of malignant ventricular arrhythmia (MVA) of ≥10%. The risk prediction score for MVA includes non-missense LMNA mutations, despite their role as an established risk factor for sudden cardiac death (SCD) has been questioned in several studies. The purpose of this study is to investigate cardiac features and find gene-phenotype correlations that would contribute to the evidence on the prognostic implications of non-missense vs. missense mutations in a cohort of LMNA mutant patients. An observational, prospective study was conducted in which 54 patients positive for a Lamin A/C mutation were enrolled, and 20 probands (37%) were included. The median age at first clinical manifestation was 41 (IQR 19) years. The median follow-up was 8 years (IQR 8). The type of LMNA gene mutation was distributed as follows: missense in 26 patients (48%), non-frameshift insertions in 16 (30%), frameshift deletions in 5 (9%), and nonsense in 7 (13%). Among the missense mutation carriers, two (8%) died and four (15%) were admitted onto the heart transplant list or underwent transplantation, with a major adverse cardiovascular event (MACE) rate of 35%. No statistically significant differences in MACE prevalence were identified according to the missense and non-missense mutation groups (p value = 0.847). Our data shift the spotlight on this considerable topic and could suggest that some missense mutations may deserve attention regarding SCD risk stratification in real-world clinical settings.
RESUMEN
Isotretinoin or 13-cis-retinoic acid (RA) is one of the most effective and widely used drugs for the treatment of severe acne vulgaris. Despite being deemed safe, no definite consensus has been reached on the cardiovascular risk of RA derivatives. We report a case of heart failure due to dilated cardiomyopathy (DCM) and concomitant renal infarction occurring after 5 months of isotretinoin use in a previously healthy 18-year-old male. The patient, with a history of acne vulgaris, presented to our emergency department with left iliac fossa pain and effort dyspnea. A trans-thoracic echocardiogram showed DCM and severely reduced left ventricle ejection fraction (LVEF: 29%). During hospitalization, a total body computed tomography (CT) showed an ischemic lesion in the left kidney. Ischemic, autoimmune, infective, and heritable causes of DCM were ruled out. Cardiac magnetic resonance (CMR) evidenced LV circumferential mid-wall late gadolinium enhancement. Heart failure therapy was promptly started and up-titrated, but only poor LVEF improvement was detected overtime. Our case aims to raise awareness on rare life-threatening cardiovascular events possibly associated with isotretinoin use. To the best of our knowledge, this is the first described case of renal thromboembolism and severe DCM leading to implantable cardioverter-defibrillator (ICD) implantation occurring during isotretinoin treatment.
RESUMEN
Cangrelor, the first intravenous P2Y12 inhibitor (P2Y12-I), has been approved on the basis of three large RCTs from the CHAMPION program which nevertheless have been criticized for the low bleeding risk of the enrolled patients, the large quote of chronic coronary syndromes, and the use of Clopidogrel as control arm even in the setting of acute coronary syndromes (ACS). We sought to investigate, in the setting of ACS, the comparative performance of Cangrelor in terms of in-hospital ischemic and haemorrhagic outcomes compared with the current gold-standard of oral P2Y12-I. The study retrospectively enrolled 686 consecutive patients admitted to the Divisions of Cardiology of Policlinico of Bari and L. Bonomo Hospital of Andria for ACS and treated with percutaneous coronary intervention. The study population was divided according to the P2Y12-I treatment strategy in two groups: patients given an oral P2Y12-I and patients receiving Cangrelor in the cath lab followed by an oral P2Y12-I. Clinical endpoints included death, ischemic and bleeding events occurring during hospital stay. Cangrelor treated patients presented higher clinical risk profile at presentation and faced higher death rate. However, after PS matching, in-hospital mortality resulted comparable between the groups and Cangrelor use was associated with reduced in-hospital definite stent thrombosis (p = 0.03). Data from our real-world registry highlight that, in the setting of ACS, Cangrelor is prevalently used in patients with very challenging clinical presentations. The adjusted analysis provides for the first time promising data on stent thrombosis reduction associated with Cangrelor use.
Asunto(s)
Síndrome Coronario Agudo , Intervención Coronaria Percutánea , Humanos , Síndrome Coronario Agudo/cirugía , Estudios Retrospectivos , Sistema de RegistrosRESUMEN
(1) Background: Glucagone-Like Peptide-1 Receptor Agonists (GLP-1 RAs) (GLP-1 RAs) are incretine-based medications recommended in the treatment of type 2 Diabetes Mellitus (DM2) with atherosclerotic cardiovascular disease (ASCVD) or high or very high cardiovascular (CV) risk. However, knowledge of the direct mechanism of GLP-1 RAs on cardiac function is modest and not yet fully elucidated. Left ventricular (LV) Global Longitudinal Strain (GLS) with Speckle Tracking Echocardiography (STE) represents an innovative technique for the evaluation of myocardial contractility. (2) Methods: an observational, perspective, monocentric study was conducted in a cohort of 22 consecutive patients with DM2 and ASCVD or high/very high CV risk, enrolled between December 2019 and March 2020 and treated with GLP-1 RAs dulaglutide or semaglutide. The echocardiographic parameters of diastolic and systolic function were recorded at baseline and after six months of treatment. (3) Results: the mean age of the sample was 65 ± 10 years with a prevalence of the male sex (64%). A significant improvement in the LV GLS (mean difference: -1.4 ± 1.1%; p value < 0.001) was observed after six months of treatment with GLP-1 RAs dulaglutide or semaglutide. No relevant changes were seen in the other echocardiographic parameters. (4) Conclusions: six months of treatment with GLP-1 RAs dulaglutide or semaglutide leads to an improvement in the LV GLS in subjects with DM2 with and high/very high risk for ASCVD or with ASCVD. Further studies on larger populations and with a longer follow-up are warranted to confirm these preliminary results.
RESUMEN
Non-ischemic dilated cardiomyopathy (DCM) is a disease characterized by left ventricular dilation and systolic dysfunction. Patients with DCM are at higher risk for ventricular arrhythmias and sudden cardiac death (SCD). According to current international guidelines, left ventricular ejection fraction (LVEF) ≤ 35% represents the main indication for prophylactic implantable cardioverter defibrillator (ICD) implantation in patients with DCM. However, LVEF lacks sensitivity and specificity as a risk marker for SCD. It has been seen that the majority of patients with DCM do not actually benefit from the ICD implantation and, on the contrary, that many patients at risk of SCD are not identified as they have preserved or mildly depressed LVEF. Therefore, the use of LVEF as unique decision parameter does not maximize the benefit of ICD therapy. Multiple risk factors used in combination could likely predict SCD risk better than any single risk parameter. Several predictors have been proposed including genetic variants, electric indexes, and volumetric parameters of LV. Cardiac magnetic resonance (CMR) can improve risk stratification thanks to tissue characterization sequences such as LGE sequence, parametric mapping, and feature tracking. This review evaluates the role of CMR as a risk stratification tool in DCM patients referred for ICD.
RESUMEN
AIM: To investigate the prevalence of amyloid cardiomyopathy (AC) and the diagnostic accuracy of echocardiographic red flags of AC among consecutive adult patients undergoing transthoracic echocardiogram for reason other than AC in 13 Italian institutions. METHODS AND RESULTS: This is an Italian prospective multicentre study, involving a clinical and instrumental work-up to assess AC prevalence among patients ≥55 years old with an echocardiogram suggestive of AC (i.e. at least one echocardiographic red flag of AC in hypertrophic, non-dilated left ventricles with preserved ejection fraction). The study was registered at ClinicalTrials.gov (NCT04738266). Overall, 381 patients with an echocardiogram suggestive of AC were identified among a cohort of 5315 screened subjects, and 217 patients completed the investigations. A final diagnosis of AC was made in 62 patients with an estimated prevalence of 29% (95% confidence interval 23%-35%). Transthyretin-related AC (ATTR-AC) was diagnosed in 51 and light chain-related AC (AL-AC) in 11 patients. Either apical sparing or a combination of ≥2 other echocardiographic red flags, excluding interatrial septum thickness, provided a diagnostic accuracy >70%. CONCLUSION: In a cohort of consecutive adults with echocardiographic findings suggestive of AC and preserved left ventricular ejection fraction, the prevalence of AC (either ATTR or AL) was 29%. Easily available echocardiographic red flags, when combined together, demonstrated good diagnostic accuracy.
Asunto(s)
Amiloidosis , Cardiomiopatías , Insuficiencia Cardíaca , Amiloidosis/diagnóstico , Cardiomiopatías/diagnóstico por imagen , Cardiomiopatías/epidemiología , Humanos , Persona de Mediana Edad , Prevalencia , Estudios Prospectivos , Volumen Sistólico , Función Ventricular IzquierdaRESUMEN
BACKGROUND: Laminopathies are rare diseases, whose cardiac manifestations are heterogeneous and, especially in their initial stage, similar to those of more common conditions, such as ischemic heart disease. Early diagnosis is essential, as these conditions can first manifest themselves with sudden cardiac death. Electrical complications usually appear before structural complications; therefore, it is important to take into consideration these rare genetic disorders for the differential diagnosis of brady and tachyarrhythmias, even when left ventricle systolic function is still preserved. CASE SUMMARY: A 60-year-old man, without history of previous disorders, presented in September 2019 to the emergency department because of the onset of syncope associated with hypotension. The patient was diagnosed with a high-grade atrioventricular block. A dual chamber pacemaker was implanted, but after the onset of a sustained ventricular tachycardia during physical exertion, a drug eluting stent was implanted on an intermediate stenosis on the left anterior descending artery, which had previously been considered non-haemodynamically significant. During the follow-up, the treating cardiologist, suspicious of the overall clinical picture, recommended a genetic test for the diagnosis of cardiomyopathies, which tested positive for a pathogenetic mutation of the lamin A/C gene. While awaiting the result of the genetic test and, later, the pacemaker to be upgraded to a biventricular defibrillator, a remote monitoring device was given to the patient in order to minimize in-person clinical evaluations during the coronavirus disease 2019-related lockdown. CONCLUSION: This case aims to raise awareness of the cardiological manifestations of laminopathies, which can be dangerously misdiagnosed as other, more common conditions.