scholarly journals Atypical presentation of cardiac sarcoidosis: role of multimodality imaging and review of literature

2020 ◽  
Vol 13 (2) ◽  
pp. e232047
Author(s):  
Deepti Bhandare ◽  
Anupama Kottam

Cardiac sarcoidosis (CS) is challenging to determine, consequently is under-recognised in clinical practice. The accurate prevalence of CS is possibly underestimated due to unspecific symptoms, subclinical illness and the dearth of universally accepted diagnostic criteria. Totally, non-invasive diagnosis of CS was proposed in 2015 by the Japanese Ministry of Health and Welfare using positron emission tomography and cardiac MRI findings as major criteria and substituting histological verification. We present a case of a 60-year-old woman with pulmonary sarcoidosis presenting with progressively worsening palpitations and recurrent syncope. Her initial evaluation at another hospital facility revealed normal cardiac testing. A detailed evaluation with echocardiography and cardiac MRI helped us arrive at the diagnosis of CS, which resulted in appropriate treatment and resolution of symptoms. We discuss CS in general, the clinical disease, diagnostic algorithms, latest guidelines and management

Heart ◽  
2021 ◽  
pp. heartjnl-2020-318001
Author(s):  
Mahesh K Vidula ◽  
Paco E Bravo

Infiltrative cardiomyopathies result from the deposition or anomalous storage of specific substances in the heart, leading to impaired cardiac function and heart failure. In this review, we describe the utility of a variety of imaging modalities for the diagnosis of infiltrative cardiomyopathies and provide algorithms for clinicians to use to evaluate patients with these disorders. We have divided infiltrative cardiomyopathies into two different categories: (1) infiltrative cardiomyopathies characterised by increased wall thickness (eg, cardiac amyloidosis and Anderson-Fabry disease (AFD)) and (2) infiltrative cardiomyopathies that can mimic ischaemic or dilated cardiomyopathies (eg, cardiac sarcoidosis (CS) and iron overload cardiomyopathy). Echocardiography is the first modality of choice for the evaluation of cardiomyopathies in either category, and the differential can be narrowed using cardiac magnetic resonance (CMR) and nuclear imaging techniques. The diagnosis of cardiac amyloidosis is supported with key findings seen on echocardiography, CMR and nuclear imaging, whereas AFD can be suggested by unique features on CMR. CMR and nuclear imaging are also important modalities for the diagnosis of CS, while iron overload cardiomyopathy is mostly diagnosed using tissue characterisation on CMR. Overall, multimodality imaging is necessary for the accurate non-invasive diagnosis of infiltrative cardiomyopathies, which is important to ensure appropriate treatment and prognostication.


2019 ◽  
Vol 92 (1100) ◽  
pp. 20190247 ◽  
Author(s):  
Dario Genovesi ◽  
Matteo Bauckneht ◽  
Corinna Altini ◽  
Cristina Elena Popescu ◽  
Paola Ferro ◽  
...  

The myocardium and the cardiovascular system are often involved in patients with sarcoidosis. As therapy should be started as early as possible to avoid complications such as left ventricular dysfunction, a prompt and reliable diagnosis by means of non-invasive tests would be highly warranted. Among other techniques,18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) has emerged as a high sensitive tool to detect sites of inflammation before morphological changes are visible to conventional imaging techniques. We therefore aim at summarizing the most relevant findings in the literature on the use of18F-fluorodeoxyglucose PET in the diagnostic workup of cardiac sarcoidosis and to underline future perspectives.


2021 ◽  
pp. 201010582110061
Author(s):  
Raja Ezman Raja Shariff ◽  
Hafisyatul Aiza Zainal Abidin ◽  
Sazzli Kasim

Cardiac amyloidosis is a severely underdiagnosed cause of heart failure with preserved ejection fraction. We report a case of highly probable transthyretin (ATTR) cardiac amyloidosis (ATTR-CA) diagnosed through the assistance of non-invasive multimodality imaging. An 81-year-old man presented with worsening dyspnoea, reduced effort tolerance and limb swelling. Examination and bedside investigations demonstrated congestive cardiac failure. On arrival, N-terminal-pro B-type natriuretic peptide was 2400 ng/L, and high-sensitivity troponin T was 78 mmol/L. Echocardiography showed severe left and right ventricular hypertrophy, and a Doppler study revealed diastolic dysfunction. Cardiac magnetic resonance imaging revealed on non-conventional dark blood sequence an abnormal inversion time for nulling myocardium suggestive of infiltrative disease, including amyloidosis. The patient was referred for nuclear-based studies involving technetium-99m pyrophosphate which demonstrated changes highly diagnostic of ATTR-CA. Early diagnosis of ATTR-CA remains paramount due to the increasing availability of disease-modifying therapies. Current guidelines recognise the role of multimodality imaging in confidently recognising the disease without the need for histological evidence in the appropriate context, providing an alternative means of diagnosis.


2020 ◽  
Vol 7 (1) ◽  
pp. e000608
Author(s):  
Juan Carlos Quijano-Campos ◽  
Lynne Williams ◽  
Sharad Agarwal ◽  
Katharine Tweed ◽  
Robert Parker ◽  
...  

IntroductionSarcoidosis is a multisystem disease, predominantly affecting the lungs but can involve the heart, resulting in cardiac sarcoidosis (CS). Patients require MRI/Positron Emission Tomography (PET) scans for diagnosis. Echocardiography, ECG and Holter monitoring may be indicative but not diagnostic alone. Patients can present late with conduction defects, heart failure or sudden death. The CASPA (CArdiac Sarcoidosis in PApworth) study protocol aims to (1) use MRI to identify CS prevalence; (2) use speckle-tracking echocardiography, signal averaged ECG and Holter monitoring to look for diagnostic pathways; and (3) identify serum proteins which may be associated with CS.Methods and analysisParticipants with pulmonary sarcoidosis (and no known cardiac disease) from Royal Papworth Hospital will have the following: cardiac MRI with late gadolinium, two-dimensional transthoracic echocardiography with speckle tracking, signal averaged ECG and 24-hour Holter monitor. They will provide a serum sample for brain natriuretic peptide levels and proteomics by liquid chromatography coupled to high-resolution mass spectrometry. All data will be collected on OpenClinica platform and analysed approximately 6 months after final patient recruitment.Ethics and disseminationThe Camden & Kings Cross Research Ethics Committee approved the protocol (REC number: 17/LO/0667). Integrated Research Approval System (IRAS) 222 720. Dissemination of findings will be via conference presentations and submitted to peer-reviewed journals.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A Caforio ◽  
S Gianstefani ◽  
A Baritussio ◽  
R Marcolongo ◽  
M Seguso ◽  
...  

Abstract Background Sarcoidosis is an immune-mediated disease; cardiac involvement, a granulomatous form of myocarditis, is under-recognised and prognostically relevant, as it can present with significant morbidity and mortality. Anti-heart autoantibodies (AHA) and anti-intercalated disk autoantibodies (AIDA) are reliable autoimmune markers in non-sarcoidosis myocarditis forms. Purpose The aim of this study was to assess the potential role of serum AHA and AIDA in cardiac sarcoidosis. Methods This is a cross-sectional study on a series of 29 patients with biopsy proven extra-cardiac sarcoidosis and with biopsy-proven or clinically suspected cardiac involvement, who were tested for AHA and AIDA. Patients were recruited in two recruiting tertiary centres, in USA and Italy. AHA and AIDA were detected by indirect immunofluorescence on human myocardium and skeletal muscle. Controls included sera from patients with non-inflammatory cardiac disease (NICD) (n=160), with ischemic heart failure (IHF) (n=141) and normal blood donors (NBD) (n=270). Results The frequencies of AHA and of AIDA were higher in sarcoidosis (86%; 62%) than in NICD (8%; 4%), IHF (7%; 2%), NBD (9%; 0%) (p=0.0001; p=0.0001 respectively). Sensitivity and specificity were: 86% and 92% for positive AHA and 62% and 98% for positive AIDA, respectively (see figure). Figure 1 Conclusions The detection of serum AHA and AIDA in biopsy-proven or clinically suspected cardiac sarcoidosis supports the involvement of heart-specific autoimmunity in the majority of our cases and may provide a novel non invasive diagnostic marker.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
T M Matajira Chia ◽  
M Castineira Busto ◽  
M Cespon Fernandez ◽  
B Gimena Reyes ◽  
F Calvo Iglesias ◽  
...  

Abstract A 61-year-old man was admitted to the hospital with an atypical pain lumbar with oligoanuria. Other comorbidities were: arterial hypertension, diabetes mellitus and smoking .On examination the patient was comfortable at rest, with a heart rate of 89 b.p.m. and a blood pressure of 147/2 mmHg. Normal S1 and S2 heart sounds were present. There were no signs of heart failure present. Patients complained of pain in hypogastrium on palpation. Creatinine 2.33 mg / dL. PCR 72. The immunological studies were normal (including IgG and IgA serological levels, antinuclear antibodies, extractable nuclear antigens, anti-neutrophil cytoplasmic antibodies. An Body CT was performed, it shows mass that includes the ureters as well as the iliac arteries and parietal thickening in aorta wall. The positron emission tomography–computed tomography (PET CT) scans was performed that evidences pathological hypermetabolism that surrounds both primitive iliac arteries with maximum SUV 12 g / ml. Pathological hypermetabolism in ascending aorta until reaching arch with maximum SUV of 9.1 mg / ml compatible with periaortitis in the ascending aorta. A study was completed with retroperitoneal mass biopsy that showed areas of retroperitoneal fibrosis with predominantly lymphoplasmacytic areas. IgG4 / IgG> 40% , Obliterative involvement of small venules suggestive of IgG4 disease. A transthoracic echocardiogram was performed which showed normal biventricular function, absence of significant valvular disease and thickening of the aortic wall compatible with periaortitis. The patient started glucocorticoid therapy with favorable response. A PET CT control was performed that showed disappearing retroperitoneal masses around iliac vessels and disappearance of activity in lateral wall of aorta and decrease activity about ascendent aorta. DIAGNOSIS : IgG4 -related aortitis Abstract P273 Figure.


2014 ◽  
Vol 21 (3) ◽  
pp. 655-659
Author(s):  
Hanna N. Ahmed ◽  
Yehuda Edo Paz ◽  
Jason Stuck ◽  
Mathew Maurer ◽  
Sabahat Bokhari

Author(s):  
Bernard Cosyns ◽  
Bernard Paelinck

The ability of ultrasound to elucidate the functional and structural abnormalities of pericardial disease is powerful. Due to multimodality imaging possibilities and to its portability, echocardiography is the technique of choice for the diagnosis of pericardial disease. Although other non-invasive technologies have been developed to provide information about the pericardium, echocardiography remains the first and often only diagnostic method needed to make a definitive diagnosis and guide appropriate treatment in patients with pericardial effusion, cardiac tamponade, or constrictive pericarditis. It allows differential diagnosis with restrictive cardiomyopathy and can easily be performed for guiding pericardiocentesis.


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