Sarcoidosis – ABC CARDIOL

ABCIC

Review Article

Cardiac Sarcoidosis: The Role of Multimodal Imaging

Lara Cristiane Terra Ferreira Carreira et al. Lívia Carreira Adriana Soares Xavier de Brito

ABSTRACT

Cardiac sarcoidosis (CS) is a potentially severe manifestation of systemic sarcoidosis, associated with advanced atrioventricular block, ventricular arrhythmias, heart failure, and sudden death. Diagnosis remains challenging due to phenotypic variability and the limitations of conventional diagnostic methods. Advances in imaging techniques, especially the combination of positron emission tomography/computed tomography (PET/CT) using ¹⁸F-FDG and cardiac magnetic resonance imaging (CMR), have revolutionized the diagnostic approach and therapeutic monitoring of CS. This article reviews current concepts of CS and its diagnosis, with a focus on the role of PET/CT, the importance of appropriate patient preparation, and integration with CMR.

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ABC

Case Report

Cardiac Sarcoidosis Reactivation in a Transplanted Heart: An Unusual Case of New Graft Dysfunction

Manuela Cristina Ribeiro Dias Barroso et al. Heleutério da Conceição Nicolau Madogolele Danielle Louvet Guazzelli Luis Fernando Bernal da Costa Seguro … Fabiana Goulart Marcondes-Braga Sandrigo Mangini Monica Samuel Ávila Gabriel Barros Aulicino Iascara Wozniak Campos Samuel Padovani Steffen Fabio Antonio Gaiotto Fernando Bacal

Introduction

Cardiac sarcoidosis (CS) is an uncommon yet potentially life-threatening manifestation of systemic sarcoidosis. Recurrence of CS following heart transplantation (HTx) is an exceedingly rare event and presents a significant diagnostic challenge.

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ABCHF

Case Report

Cardiac Sarcoidosis and Complex Arrhythmias

Anna Luiza Souza et al. Renata dos Santos Oliveira Ana Paula Lindoso Lima Barreiro Mauricio Lopes Prudente … Rodrigo Paashaus de Andrade Giulliano Gardenghi

Introduction

Sarcoidosis is a multisystem inflammatory granulomatous disease of unknown cause, related to genetic factors, with an incidence of 1 to 30 cases/100,000 people, and is more prevalent in women over 50 years of age. The formation of scar tissue characterizes it and can affect any organ, with a predilection for the lungs in approximately 90% of cases. Patients with cardiac involvement are mainly male and, in general, have a worse prognosis, with high mortality rates observed in this population (85% of deaths).^,

The scar substrate in the myocardium, which presents as multifocal and heterogeneous, is related to several cardiac manifestations. Among the most common, we can mention left ventricular dysfunctions, atrioventricular conduction abnormalities, and ventricular and supraventricular arrhythmias, such as ventricular tachycardia and atrial fibrillation, which can lead, in some cases, to sudden death. In view of this, the use of antiarrhythmic drugs is necessary in clinical practice in most patients, aiming at the control of arrhythmias, especially those of ventricular origin, and consequently improving the quality of life and reducing mortality. In this work, we report the therapeutic management of a case of cardiac sarcoidosis associated with prolonged QT interval that evolved with complex ventricular tachycardia (VT).

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ABCIC

Case Report

A Rare Case of Pulmonary Sarcoidosis With Extracranial Carotid System Involvement

Fanilda Souto Barros et al. Halsted Alarcão Gomes Pereira da Silva Daniela Souto Barros Simone Nascimento dos Santos

Introduction

Sarcoidosis is a disease of unknown etiology, whose pathogenesis appears to involve genetic, immunologic, infectious, and environmental factors. It was first described in 1889 by Ernest Besnier and is also called Besnier-Boeck-Schaumann disease.^,

In sarcoidosis, a noninfectious inflammatory process leads to the formation of granulomas, which predominantly affect the lungs and intrathoracic lymph nodes in approximately 90% to 95% of cases. Close to 30% of patients with sarcoidosis present with extrapulmonary involvement, which may be the primary manifestation of the disease or occur in isolation.^–

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ABCIC

Review Article

Cardiac Sarcoidosis: The Chameleon of Cardiology

Diego Moraes De Moura et al. Aluísio José De Oliveira Monteiro Neto Marcelo Dantas Tavares de Melo Fábio Fernandes

Abstract

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology, characterized by the formation of non-caseating granulomas in multiple organs. Cardiac involvement, an important cause of morbidity and mortality in these patients, has been generating interest in cardiology, because it is a cause of heart failure, atrioventricular blocks, and ventricular arrhythmias with unfavorable prognosis; however, there are specific treatments with the potential to change the natural history of this condition. The main challenge of cardiac sarcoidosis (CS) is diagnosis, given that the gold standard method of endomyocardial biopsy has limited sensitivity due to the focal nature of the pathology.

Accordingly, cardiovascular imaging methods play the role of guiding most diagnoses of CS. In this scenario, knowledge about these methods, their main findings, and their rational use are essential to the diagnosis of this disease with such diverse presentations.

Electrocardiogram and echocardiography are practical and widely available exams; however, they provide greater diagnostic capacity in patients with clinically manifest disease. On the other hand, to identify incipient forms, which are often silent, it is necessary to use advanced imaging methods, such as positron emission tomography with 18F-fluorodeoxyglucose and cardiac magnetic resonance, which primarily identify signs of active inflammatory activity and fibrosis, respectively. Despite the advances in these imaging methods, due to the lack of studies comparing them with the gold standard (endomyocardial biopsy), the diagnosis of CS currently remains a major challenge.

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ABCIC

Review Article

What Is Important in the Echocardiographic Evaluation of Patients With Cardiac Sarcoidosis?

Nathalia Conci Santorio et al. Pandreli Testa Santorio Fabio Fernandes Viviane Tiemi Hotta

Abstract

Sarcoidosis is a systemic condition, of unclear etiology, associated with the formation of non-necrotizing granulomas in several organs, and thoracic involvement in 90% of the cases. Cardiac impairment is detected in approximately 10% of the patients, reaching 25% in autopsy studies. It is in charge of about half the deaths in patients with sarcoidosis, and an important prognostic factor. Interventricular septum and the left ventricle free wall are the most affected regions, especially in the subepicardial portion. The development of changes in conduction (including atrioventricular block and ventricular arrhythmia) and heart failure are the most common manifestations. Diagnosis is challenging and often requires more advanced imaging examinations, such as positron emission tomography or late-enhancement cardiac magnetic resonance imaging. However, these examinations have high cost and are not so available. The conventional transthoracic echocardiography, on the other hand, is widely accessible, but presents later and little specific findings. The most important ones are the reduction of the left ventricle ejection fraction < 50% and the presence of abnormal tapering of the basal interventricular septum. Other segmental changes, especially when not correspondent to coronary territories, and aneurisms, are also relevant. Besides, there may be diastolic dysfunction, pericardial effusion and right ventricular dysfunction, both due to impairment primary or secondary to pulmonary hypertension. The most advanced ultrasound techniques, such as myocardial strain, myocardial work and elastography, are promising in the search of an earlier diagnosis at a lower cost.

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ABCIC

Case Report

8F-FDG PET/CT in Diagnosis, Therapeutic Evaluation and Definition of Management in Cardiac Sarcoidosis: Learn From This Case Report

Simone Cristina Soares Brandão et al. Matheus de Araújo Lucena Christiane Wiefels Pedro Rodrigues da Silva … Claudio Tinoco Mesquita Maria Fernanda Rezende Fabio Mastrocola

Introduction

Sarcoidosis is a non-caseating granulomatous inflammatory disease of unknown etiology. The presentation can range from an asymptomatic condition with nonspecific findings on chest radiography to organ failure. Any part of the body can be affected, but the most common sites are the mediastinal lymph nodes and the lungs, followed by the skin and ocular structures such as the uvea. Other systems, such as the nervous, endocrine, gastrointestinal, urinary, musculoskeletal, and cardiovascular systems, can also be affected.

Cardiac sarcoidosis is a disease that is still under-researched in cardiology. It is related to cardiomyopathy conditions that can be confused with hypertrophic, infiltrative, or even ischemic cardiomyopathy, and it courses with ventricular arrhythmias and conduction disorders with possible progression to heart failure and/or sudden death. It is worth emphasizing that cardiac sarcoidosis is responsible for up to 35% of cases of complete atrioventricular block (CAVB) in patients under 60 years of age. These patients benefit from the implantation of a pacemaker and implantable cardioverter defibrillator (ICD). Immunosuppressive therapy is indicated to control the inflammatory process.

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ABCHF

Review Article

Cardiac Sarcoidosis, the Great Chameleon of Myocardiopathies

Diogo Coutinho Suassuna et al. Felipe J. de Oliveira Fraga Carlos Eduardo L. Montenegro

Sarcoidosis is a chronic multisystemic disease of unknown etiology, histologically determined by the formation of non-caseating granulomas in various organs of the body, which can also affect heart tissues, resulting in cardiac sarcoidosis (CS).

This disease continues to be enigmatic in such a way that, since its first description (1929), and over the years, the exact mechanism of its pathogenesis has still not been clarified, with the possibility of the existence of an important component of genetic predisposition added to types of environmental exposure.

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ABCHF

Viewpoint

Restrictive Cardiomyopathies: What’s New on the War Front?

Luiz Claudio Danzmann et al. Liciani de Mello Feliciano Elisa Kalil Paula Loredo Siminovich Marianna de Moura Nora

A rare – or not so rare – condition?

Restrictive cardiomyopathies, caused by primary pathologies of the heart muscle, are considered rare diseases, corresponding to about 5% of all cardiomyopathies. The most frequent form of restrictive cardiomyopathy is cardiac amyloidosis (CA), which can be considered a benchmark since, with the availability of diagnostic technologies, its detection has been made much more frequent, associated with the fact that there are effective treatment possibilities.

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ABCIC

Case Report

Cardiac Sarcoidosis as a Cause of Total Atrioventricular Block: Importance of Differential Diagnosis

Jorge Elias Neto et al. Márcio Augusto Silva Ricardo Ryoshim Kuniyoshi Guilherme Futuro … Erick Sessa Merçon Petherson Susano Grativvol Fátima Cristina Pedrotti

Introduction

Sarcoidosis is a systemic granulomatous disorder of unknown cause that can affect virtually any organ. The extracardiac forms are usually benign and subjected to spontaneous remission. However, the prognosis may be unfavorable in case of cardiac involvement.

Symptomatic cardiac sarcoidosis (CS) is diagnosed in approximately 5% of patients with sarcoidosis. However, based on some autopsy series, the prevalence of subclinical CS can reach 25–30%.

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IJCS

Review Article

The Role of 18F-FDG PET/CT in Cardiac Sarcoidosis

Christiane Wiefels et al. Olabimpe Lamai Riina Kandolin David Birnie … Eugene Leung Claudio Tinoco Mesquita Rob Beanlands

Introduction

Systemic sarcoidosis (SS) was first described more than a century ago by the Norwegian dermatologist Caesar Boeck. He correlated skin nodules with epithelioid cells with large nuclei and giant cells as “multiple benign sarcoid of the skin”, for their resemblance with sarcoma. Today, the cause of the disease remains unknown, but evidence points toward immunological response to an unidentified antigenic trigger in individuals with genetic susceptibility. The estimation of the prevalence of SS in the population varies in the literature also depending on the type of study: epidemiologic, autopsy or imaging.

Some previous data showed prevalence as high as 100-330 cases per 100,000 inhabitants.^, Environmental factors have been attributed to those differences, including sex, age and ethnicity, showing a predisposition for environmental and genetic factors. The reported incidence is similar across sex in North American population but greater in females in Scandinavian and Japanese populations,^, but more likely to be chronic and fatal in black Americans. The disease usually develops before the age of 50 years, with a peak incidence at 20 to 39 years old,^, and is very uncommon under the age or 15 or older than 70. Symptomatic cardiac sarcoidosis (CS) has been reported in 2-5% of the patients with the systemic form.^– However, with the advance of new cardiac imaging techniques, cases of asymptomatic (clinically silent) cardiac involvement have been diagnosed. The disease has been reported in at least 20% of the autopsies and imaging report in the United States to as much as 50% in Japan.^,

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ABC

Artigo Original

Cardiac electrophysiological assessment in patients with sarcoidosis

Jefferson Curimbaba et al. Silvia Carla Souza Rodrigues José Marcos Moreira Lenine Ângelo Alves Silva … Carlos Alberto de Castro Pereira João Pimenta

BACKGROUND: Sarcoidosis is a multisystem granulomatous disease of unknown origin that can cause sudden death. OBJECTIVE: Electrophysiological evaluation of patients with suspected sarcoidosis with cardiac involvement. METHODS: We studied 22 patients with mean age of 55.32 ± 13.13 years, diagnosed with sarcoidosis and suspected cardiac involvement. These patients underwent clinical evaluation, laboratory tests, electrocardiogram, echocardiogram, 24-hour Holter, technetium or gallium scintigraphy and electrophysiological study. In selected cases, we performed positron emission tomography or magnetic resonance imaging. Patients were followed up in the outpatient care service with quarterly visits. RESULTS: Cardiac involvement was confirmed in four (18.2%) patients. Ventricular extrasystoles with density > 100/24h were documented in 24-Holter monitoring in 12 (54.5%) patients. Electrophysiological studies revealed an increased HV interval in seven patients (31.8%) and increased Wenckebach point in four (18.2%) patients. There was induction of atrial fibrillation in seven patients (31.8%) and sustained ventricular tachycardia in one patient (4.5%). Four patients with confirmed cardiac sarcoidosis had documented ventricular extrasystoles with density > 100/24h. Out of these, two had prolonged HV interval and atrial fibrillation was induced in two of them. Sustained ventricular tachycardia was not induced in any of these patients. After mean follow-up period of 20.9 ± 15.7 months, one patient with cardiac sarcoidosis had sudden death. CONCLUSION: Patients with sarcoidosis and suspected cardiac involvement have a high prevalence of ventricular extrasystoles (VEs) and conduction system disorders.

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