restrictive cardiomyopathy
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2022 ◽  
Vol 13 ◽  
pp. 215013192110626
Author(s):  
Michel Juarez ◽  
Gaspar Del Rio-Pertuz ◽  
Kanak Parmar ◽  
Melanie C. Bois ◽  
Scott Shurmur ◽  
...  

Background: Transthyretin amyloid cardiomyopathy (ATTR-CM) is one of the most common types of cardiac amyloidosis. Amyloid cardiomyopathy more commonly affects men, elderly, and 3% to 4% of the African-American population. ATTR-CM suspicion and diagnosis is challenging; however, awareness of the disease is increasing, and best practices to identify it are being proposed. The approach to suspected cases of ATTR-CM relies on the presence of heart failure, red flag signs and symptoms, and age >65 or >70 for men and women respectively. Little is known about cases when it presents in early ages. Case: We report a 62-year-old African American male with past medical history of hyperlipidemia, prostate cancer, hypertension, bilateral carpal tunnel surgery that had debuted with a cardiac arrhythmia at age 55 and was diagnosed with heart failure several years later. Restrictive cardiomyopathy was suspected, and genetic screening was sent for ATTRm which confirmed a pathogenic trasnthyretin gene mutation. Endomyocardial biopsy was performed which confirmed cardiac amyloid deposition. Discussion: ATTR-CM is a rare disease with an increasing prevalence. Cases with out of proportion signs and symptoms of heart failure with preserved ejection fractions should raise the suspicion of ATTR-CM despite age.


2021 ◽  
Vol 27 (4) ◽  
pp. 7-15
Author(s):  
Monika Shumkova ◽  
Kiril Karamfiloff ◽  
Raya Ivanova ◽  
Kristina Stoyanova ◽  
Dobrin Vassilev

Cardiomyopathies are a heterogeneous group of diseases. The main pathogenetic mechanism is myocardial damage due to genetic mutations. Cardiomyopathies are one of the leading causes of heart failure, sudden cardiac death, and life-threatening arrhythmias. Certain factors associated with poor prognosis determined the prognosis in this group of patients. Survival in different types of cardiomyopathies depends on the time of diagnosis and initial treatment. The types of cardiomyopathies discussed in this review are hypertrophic cardiomyopathy, dilative cardiomyopathy, restrictive cardiomyopathy, left ventricle non-compaction, and arrhythmogenic right ventricular cardiomyopathy.


Heart ◽  
2021 ◽  
pp. heartjnl-2021-320181
Author(s):  
Jack RW Brownrigg ◽  
Vincenzo Leo ◽  
Joel Rose ◽  
Eric Low ◽  
Sarah Richards ◽  
...  

AimsThe population prevalence of cardiomyopathies and the natural history of symptomatic heart failure (HF) and arrhythmia across cardiomyopathy phenotypes is poorly understood. Study aims were to estimate the population-diagnosed prevalence of cardiomyopathies and describe the temporal relationship between a diagnosis of cardiomyopathy with HF and arrhythmia.MethodsPeople with cardiomyopathy (n=4116) were identified from linked electronic health records (~9 million individuals; 2000–2018) and categorised into hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), restrictive cardiomyopathy (RCM) and cardiac amyloidosis (CA). Cardiomyopathy point prevalence, rates of symptomatic HF and arrhythmia and timing relative to a diagnosis of cardiomyopathy were determined.ResultsIn 2018, DCM was the most common cardiomyopathy. DCM and HCM were twice as common among men, with the reverse trend for ARVC. Between 2010 and 2018, prevalence increased for ARVC by 180% and HCM by 9%. At diagnosis, more patients with CA (66%), DCM (56%) and RCM (62%) had pre-existing HF compared with ARVC (29%) and HCM (27%). Among those free of HF at diagnosis of cardiomyopathy, annualised HF incidence was greatest in CA and DCM. Diagnoses of all cardiomyopathies clustered around the time of HF onset.ConclusionsThe recorded prevalence of all cardiomyopathies increased over the past decade. Recognition of CA is generally preceded by HF, whereas individuals with ARVC or HCM more often developed HF after their cardiomyopathy diagnosis suggesting a more indolent course or better asymptomatic recognition. The clustering of HF and cardiomyopathy diagnoses suggests opportunities for presymptomatic or earlier diagnosis.


2021 ◽  
Author(s):  
Hiroki Mori ◽  
Shigetoyo Kogaki ◽  
Hidekazu Ishida ◽  
Tadahiro Yoshikawa ◽  
Takahiro Shindo ◽  
...  

2021 ◽  
pp. 60-72
Author(s):  
Irina V. Opalinskaya ◽  
Galina V. Danilova ◽  
Natalya Yu. Isaeva ◽  
Ekaterina S. Petrova ◽  
Elena I. Busalaeva ◽  
...  

Primary amyloidosis is a disease with a complex and not fully understood pathogenesis, which is characterized by a wide range of clinical manifestations. Light chain amyloidosis is the most common form of systemic amyloidosis. At this, the heart is the dominant target organ in systemic amyloidosis. Cardiac amyloidosis (amyloid cardiomyopathy) is most often manifested by diastolic heart failure resulting from restrictive cardiomyopathy. Therapy of amyloid cardiopathy includes optimal treatment of heart failure and chemotherapy. To reduce the symptoms of heart failure, diuretics are the main means, since other pathogenetic agents cannot be used due to hypotension and a possible decrease in cardiac output. With the introduction of new medicinal products into clinical practice, such as the proteosome inhibitor Bortezomib, the prognosis for patients has improved. However, amyloidosis remains a difficult disease to diagnose and treat.


Author(s):  
Archit Dahiya ◽  
Deepesh Agarwal ◽  
Naveen Gupta ◽  
Harsh Wardhan ◽  
Jai Chowdhary

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Beatrice Bacchi ◽  
Francesco Cappelli ◽  
Federico Perfetto ◽  
Marco Allinovi ◽  
Samantha Innocenti ◽  
...  

Abstract Aims Multiple myeloma (MM) is a malignant neoplasm with a frequent cardiac involvement characterized by restrictive cardiomyopathy. Kidney and thromboembolic complications are also described. Methods and results A 52-year-old woman with a history of kidney disease was admitted to nephrology department due to worsening of renal function and refractory hypertension. A bone marrow biopsy revealed the diagnosis of MM. A renal and fat pad biopsy with Congo red staining was performed but amyloid deposition was not observed. Increased cardiac enzymes suggested a cardiac involvement. Indeed, two intracardiac right-sided masses and massive pulmonary embolism were detected. Surgical cardiac intervention was promptly performed and a chemotherapy regimen was started. Meantime, a kidney biopsy revealed a light-chain deposition disease. Conclusions This case highlights that multiple organ involvement in patients with MM may result from a combination of paraprotein-dependent and -independent factors. Moreover, in patients with not acute massive pulmonary embolism and intracardiac right masses, surgical pulmonary embolectomy should be promptly performed to preserve RV function and to avoid prevent pulmonary hypertension development.


2021 ◽  
pp. 102639
Author(s):  
Kseniya Perepelina ◽  
Aleksandr Khudiakov ◽  
Nataliia Rodina ◽  
Aleksandr Boytsov ◽  
Tatiana Vavilova ◽  
...  

2021 ◽  
Vol 34 (4) ◽  
Author(s):  
Débora Pereira Galvêas Negr ◽  
◽  
Patrick Ventorim Costa ◽  
Mateus Oliveira Potratz ◽  
Carina Massariol Belinassi ◽  
...  

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