Cardiomyopathies

2020 ◽  
pp. 120-244
Author(s):  
Perry Elliott ◽  
Pier D. Lambiase ◽  
Dhavendra Kumar
Keyword(s):  
Risk Stratification ◽  
Heart Muscle ◽  
Takotsubo Syndrome ◽  
Arrhythmogenic Cardiomyopathy ◽  
Ongoing Debate ◽  
Family Screening ◽  
Adults And Children ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

The chapter covers heart muscle diseases, starting from the classification and the importance of a cardiomyopathy-focused mindset for diagnostic work up. It goes on to examine genetics, diagnosis, common challenging differentials, and family screening in a number of specific cardiomyopathies in both adults and children. Management of specific cardiomyopathies is discussed in detail, including symptomatic and prognostic treatment with drugs, devices, and surgery, with focus on areas of ongoing debate, such as arrhythmic risk stratification and overlap between dilated and arrhythmogenic cardiomyopathy. In the final section it discusses the pathophysiology, diagnosis, and management of a distinct condition (and arguably not a cardiomyopathy), Takotsubo syndrome.

Typical and reverse Takotsubo syndromes as initial manifestations of consecutive Addisonian crises in a 38-year-old patient: the heart has its reasons!

2021 ◽  
Vol 14 (1) ◽  
pp. e238189
Author(s):  
Melissa Tso ◽  
Shravan Nosib
Keyword(s):  
Case Report ◽  
Interesting Case ◽  
Syndrome Type ◽  
Type Ii ◽  
Takotsubo Syndrome ◽  
Refractory Shock ◽  
First Case ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

We report an interesting case of a 38-year-old woman presenting with reverse Takotsubo syndrome (TTS) secondary to an Addisonian crisis, her second such episode. A few years prior, she had presented with typical TTS in the setting of Addisonian crisis; diagnostic work-up revealing Auto-Immune Polyglandular Syndrome Type II (APS II). We believe this to be the first case report of typical and variant phenotypes of TTS in a patient with APS II. The pathogenic link between these two conditions is explored. In patients presenting with Addisonian crises and refractory shock, the possibility of concurrent TTS should be considered. TTS muddies the diagnostic waters and poses therapeutic challenges as outlined.

P5460Prevalence of left atrial enlargement in patients undergoing an echocardiogram for cardiovascular risk stratification at an esh excellence centre in italy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M L Muiesan ◽  
M Salvetti ◽  
A Paini ◽  
C Aggiusti ◽  
F Bertacchini ◽  
...  
Keyword(s):  
Cardiovascular Risk ◽  
Risk Stratification ◽  
Arterial Hypertension ◽  
Left Atrial ◽  
Cardiovascular Risk Stratification ◽  
Large Sample ◽  
Hypertension Guidelines ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

Abstract Background Left atrial (LA) enlargement (LAE) is associated to an increased risk of cardiovascular complications, and in particular of atrial fibrillation. The 2018 ESH/ESC Hypertension guidelines suggested the use of LA volume instead of linear dimensions, and for the first time proposed the indexation to height2 (h2) The aim of our study was to assess the prevalence of left atrial dilatation in a large sample of patients undergoing an echocardiogram for cardiovascular risk stratification at an ESH excellence centre in Italy. Design and methods 3872 subjects undergoing a diagnostic work-up for arterial hypertension (known or suspect) were analysed. The mean age was 56±15 years, BMI 26±5 kg/m, 44% normal weight, 39% overweight, 17% obese, 53% males. Left atrial volume was measured by the area-length method using the apical 4-chamber and 2-chamber views. Results The prevalence of left ventricular hypertrophy (LVH) was 11% when indexing for BSA and 12% when indexing for height2.7. LAE was observed in 30% of subjects when indexing for h2 and in 9% when indexing for BSA. In obese or overweight subjects the prevalence of LAE was 38% of subjects when indexing for h2 and in 11% when indexing for BSA. The different prevalence of LAE was particularly evident in extremely obese patients. LAE was very common in patients with LVH: 62% and 26% when indexing for h2 and for BSA, respectively. Interestingly, it was frequent also in patients without LVH, in particular when the indexation for h2 was used (25% as compared to 7% when indexing for BSA). Conclusions In a large sample of subjects undergoing a diagnostic work-up for arterial hypertension LAE was frequently observed, particularly when the new indexation proposed by the 2018 ESH/ESC hypertension guidelines was used. Even in the absence of clear-cut LVH, LAE was observed in one quarter of subjects. The indexation to BSA leads to an under-recognition of LAE, in particular in patients with overweight and/or obesity

scholarly journals Diagnostic Work-Up and Risk Stratification in X-Linked Dilated Cardiomyopathies Caused by Dystrophin Defects

2011 ◽  
Vol 58 (9) ◽  
pp. 925-934 ◽  
Author(s):  
Marta Diegoli ◽  
Maurizia Grasso ◽  
Valentina Favalli ◽  
Alessandra Serio ◽  
Fabiana Isabella Gambarin ◽  
...  
Keyword(s):  
Risk Stratification ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

scholarly journals Stroke masquerading as cardiac arrest: the artery of Percheron

2021 ◽  
Vol 14 (1) ◽  
pp. e238681
Author(s):  
Megan Quetsch ◽  
Sureshkumar Nagiah ◽  
Stephen Hedger
Keyword(s):  
Cardiac Arrest ◽  
Clinical Assessment ◽  
Altered Consciousness ◽  
Cognitive Changes ◽  
Consciousness Level ◽  
Artery Of Percheron ◽  
Conscious Level ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

The artery of Percheron (AOP) is a rare arterial variant of the thalamic blood supply. Due to the densely packed collection of nuclei it supplies, an infarction of the AOP can be devastating. Here we highlight a patient who had an AOP stroke in the community, which was initially managed as cardiac arrest. AOP strokes most often present with vague symptoms such as reduced conscious level, cognitive changes and confusion without obvious focal neurology, and therefore are often missed at the initial clinical assessment. This case highlights the importance of recognising an AOP stroke as a cause of otherwise unexplained altered consciousness level and the use of MRI early in the diagnostic work-up.

scholarly journals Death of unknown cause? Post-mortem diagnosis of fulminant course of an EBV-associated secondary hemophagocytic lymphohistiocytosis

2021 ◽  
Author(s):  
Josia Fauser ◽  
Stefan Köck ◽  
Eberhard Gunsilius ◽  
Andreas Chott ◽  
Andreas Peer ◽  
...  
Keyword(s):  
Immune Response ◽  
Case Report ◽  
Macrophage Activation ◽  
Post Mortem ◽  
Life Threatening ◽  
Appropriate Therapy ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up ◽  
Secondary Hemophagocytic Lymphohistiocytosis

SummaryHLH is a life-threatening disease, which is characterized by a dysregulated immune response with uncontrolled T cell and macrophage activation. The often fulminant course of the disease needs a fast diagnostic work-up to initiate as soon as possible the appropriate therapy. We present herein the case of a 71-year-old patient with rapidly progressive hyperinflammatory syndrome, which post mortem resulted in the diagnosis of EBV-associated HLH. With this case report, we intend to highlight the relevance of the HScore in the diagnosis of HLH, to create a greater awareness for EBV as a trigger of HLH, and to demonstrate the importance of treating EBV-associated HLH as early as possible.

scholarly journals COVID-19 lung injury as a primer for immune checkpoint inhibitors (ICIs)-related pneumonia in a patient affected by squamous head and neck carcinoma treated with PD-L1 blockade: a case report

2021 ◽  
Vol 9 (2) ◽  
pp. e001870
Author(s):  
Angelo Dipasquale ◽  
Pasquale Persico ◽  
Elena Lorenzi ◽  
Daoud Rahal ◽  
Armando Santoro ◽  
...  
Keyword(s):  
Lung Injury ◽  
Head And Neck ◽  
Checkpoint Inhibitors ◽  
Experimental Therapy ◽  
Common Mechanism ◽  
Nasopharyngeal Swab ◽  
Immune Mediated ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

By the beginning of the global pandemic, SARS-CoV-2 infection has dramatically impacted on oncology daily practice. In the current oncological landscape, where immunotherapy has revolutionized the treatment of several malignancies, distinguishing between COVID-19 and immune-mediated pneumonitis can be hard because of shared clinical, radiological and pathological features. Indeed, their common mechanism of aberrant inflammation could lead to a mutual and amplifying interaction.We describe the case of a 65–year-old patient affected by metastatic squamous head and neck cancer and candidate to an experimental therapy including an anti-PD-L1 agent. COVID-19 ground-glass opacities under resolution were an incidental finding during screening procedures and worsened after starting immunotherapy. The diagnostic work-up was consistent with ICIs-related pneumonia and it is conceivable that lung injury by SARS-CoV-2 has acted as an inflammatory primer for the development of the immune-related adverse event.Patients recovered from COVID-19 starting ICIs could be at greater risk of recall immune-mediated pneumonitis. Nasopharyngeal swab and chest CT scan are recommended before starting immunotherapy. The awareness of the phenomenon could allow an easier interpretation of radiological changes under treatment and a faster diagnostic work-up to resume ICIs. In the presence of clinical benefit, for asymptomatic ICIs-related pneumonia a watchful-waiting approach and immunotherapy prosecution are suggested.

scholarly journals Long-Term Follow-Up of Thyroid Incidentalomas Visualized with 18F-Fluorodeoxyglucose Positron Emission Tomography—Impact of Thyroid Scintigraphy in the Diagnostic Work-Up

Diagnostics ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 557
Author(s):  
Kirsten Korsholm ◽  
Michala Reichkendler ◽  
Louise Alslev ◽  
Åse Krogh Rasmussen ◽  
Peter Oturai
Keyword(s):  
Emission Tomography ◽  
Thyroid Scintigraphy ◽  
Fdg Uptake ◽  
Positron Emission ◽  
Pet Ct ◽  
18F Fdg ◽  
Diagnostic Work ◽  
Fdg Pet Ct ◽  
Work Up ◽  
Diagnostic Work Up

Our objective was to evaluate the frequency of malignancy in incidental thyroidal uptake on 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) in a cohort of Danish patients, and furthermore to evaluate the impact of thyroid scinti-graphy in the diagnostic work-up. All whole-body PET/CT reports from 1 January 2010 to 31 December 2013 were retrospectively reviewed and further analyzed if visually increased thyroidal FDG uptake was reported. Patient electronic files were searched for further thyroid evaluation. Of 13,195 18F-FDG-PET/CT scans in 9114 patients, 312 PET/CT reports mentioned incidental thyroid FDG-uptake, and 279 patients were included in the study (3.1%). The thyroid was further investigated in 137 patients (49%), and 75 patients underwent thyroid scintigraphy. A total of 57 patients had a thyroid biopsy and 21 proceeded to surgery. Surgical specimens displayed malignancy in 10 cases, and one thyroid malignancy was found by autopsy. Hence, 11 patients were diagnosed with thyroid malignancies among 279 patients with incidental thyroid 18F-FDG uptake (3.9%). In 34 patients, a biopsy was avoided due to the results of the thyroid scintigraphy. We conclude that patients with thyroid incidentalomas can benefit from further diagnostic work-up including a thyroid scintigraphy.

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