The outlook for transthyretin amyloidosis (ATTR) is changing with the availability of new and emerging treatments. ATTR now appears to be more common than previously thought and is no longer viewed as an obscure diagnosis with a grim prognosis. Now more than ever, there is growing emphasis on the need for early diagnosis because the treatments appear to be most effective if started in earlier stages of the disease. Diagnosing ATTR is a challenge as it may initially present with nonspecific symptoms and it is often thought of as a diagnosis of exclusion. Increased awareness is imperative as new treatments offer hope and have the potential to change the disease trajectory. ATTR commonly presents with neurological and cardiac features. Transthyretin (TTR) is a protein produced in the liver which misfolds either due to genetic mutations or due to aging and results in deposition of amyloid fibrils in organs and tissues. Apart from the traditional imaging modalities, newer techniques including echocardiographic strain imaging, magnetic resonance imaging (MRI), and nuclear scintigraphy, as well as the increased availability of genetic testing are aiding in making a timely diagnosis. In this review, we present the current understanding of the ATTR disease process, diagnostic and surveillance approaches, newer treatment modalities, and the future directions.
We describe a unique case of fulminant myocarditis in a patient with presumed SARS-CoV-2 reinfection. Patient had initial infection 4 months backand had COVID-19 antibody at the time of presentation. Endomyocardial biopsy showed lymphocytic myocarditis, that is usually seen in viral myocarditis. The molecular diagnostic testing of the endomyocardial biopsy for cardiotropic viruses was positive for Parvovirus and negative for SARS-CoV-2. Authors highly suspect co-infection of SARS-CoV-2 and Parvovirus, that possibly triggered the immune cascade resulting in fulminant myocarditis. Patient was hemodynamically unstable with ventricular tachycardia and was supported on VA ECMO and Impella CP. There was impressive recovery of left ventricular function within 48 hours, leading to decannulation of VA ECMO in 72 h. This unique case was written by the survivor herself.
Myocardial injury, diagnosed by troponin elevation, is common in COVID-19 patients, but cardiac involvement with clinical manifestations occurs less frequently. We analyzed the literature on COVID-19 (2020) and systematically reviewed the cases where individual patient data were presented. We searched PubMed and Google Scholar for "COVID," "COVID-19," and "coronavirus" in combination with "myocarditis," "heart failure," "takotsubo," "cardiomyopathy," and "cardiogenic shock." We identified 90 cases of COVID-19 with myocardial involvement, mean age 52.9 ± 18.3 years, 54.5% males. Of them, 55 survived (61.1%), 20 died (22.2%), and in 15 (16.7%) the outcome was unknown at the time of publication. Among patients with known outcome, mortality was 26%. The nadir LVEF was 31.7 ± 13.1% and recovered to 50.1 ± 16.0%. Pericardial effusion was a common finding, reported in 21 (23.3%) of patients, including moderate size effusion in 8.9% and large in 7.8%. The effusion caused tamponade in 11 (12.2%) of patients. Out of 83 patients who experienced a decrease in LVEF, 30 could be classified as takotsubo syndrome. The takotsubo patients were older than those with myocarditis, and with relatively high proportion of males. About one third of the cases was complicated by cardiogenic shock. Myocardial involvement in COVID-19 patients most often presents as a new, rapid decrease in LVEF, although normal LVEF or takotsubo-like wall motion pattern does not rule out myocarditis. Moderate and large pericardial effusion is common, and cardiac tamponade occurs in 12.2% of patients. Cardiogenic shock develops in one third of the patients. Mortality appears to be high at 26%.
Introduction: Takotsubo is often described as stress-induced cardiomyopathy and is a known cause of heart failure. Objective: Review the clinical course of a young coronavirus disease 2019 (COVID-19) patient who developed Takotsubo following cardiac tamponade. Case presentation: A 42-year-old woman presented to the emergency department with fever, altered mental status and hypoxia. She was ultimately found to be in cardiac tamponade and within 2 hours of a pericardiocentesis she developed Takotsubo and was in cardiogenic shock. Her family decided to place her on comfort measures and she died the same day. Discussion: This case illustrates the increasing number of cardiovascular complications being reported in COVID-19 and highlights the importance of clinicians to be aware of these challenges. Conclusion: Here, we report a distinct presentation of cardiogenic shock in a young COVID-19 patient. The rapid onset of her suspected Takotsubo and the severity of her disease were striking features in this case.
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