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Pubblicazioni recenti - cardiac amyloidosis
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[Suspected cardiac amyloidosis - diagnostic steps for evaluation].
Dtsch Med Wochenschr2020 Aug;145(16):1162-1168. doi: 10.1055/a-1019-2072.
Feldmann Korinna, Hamm Christian W, Aßmus Birgit,
Abstract
Cardiac amyloidosis is a rare cause of cardiomyopathy, however, it is part of an underdiagnosed underlying systemic disease. For transthyretin (ATTR) amyloidosis, which is caused by the deposition of incorrectly folded transthyretin, either as the wild-type (wtATTR) or mutated (mATTR) form, novel evidence-based treatment options were recently shown to reduce disease progression as well as hospitalisation rates for heart failure. Thus, it is important to establish early and reliable diagnosis of cardiac involvement with ATTR amyloidosis.Modern non-invasive imaging (cardio magnetic resonance (CMR) and scintigraphic methods) together with immune fixation with determination of free light chains allow fast and reliable clinical screening for cardiac amyloidosis, whereas endomyocardial biopsy and genetics are used for confirmation of the underlying diagnosis. Interdisciplinary teams including hemato-oncology, neurology, nephrology in addition to cardiology are essential to enable personalized targeted treatment strategies.
© Georg Thieme Verlag KG Stuttgart · New York.
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Spectrum of transthyretin gene mutations and clinical characteristics of Polish patients with cardiac transthyretin amyloidosis.
Cardiol J2020 Aug;():. doi: 10.5603/CJ.a2020.0104.
Gawor Monika, Holcman Katarzyna, Franaszczyk Maria, Lipowska Marta, Micha?ek Piotr, Teresi?ska Anna, Bili?ska Zofia T, Rubi? Pawe?, Kostkiewicz Magdalena, Szot Wojciech, Podolec Piotr, Grzybowski Jacek,
Abstract
BACKGROUND:
Transthyretin amyloidosis (ATTR) is a rare, life-threatening systemic disorder. We present first findings on the cardiac hereditary ATTR in Poland.
METHODS:
Sixty-eight consecutive patients with suspected or known cardiac amyloidosis were evaluated, including blood tests, standard 12-lead electrocardiography (ECG) and transthoracic echocardiography. ATTR was confirmed histologically or non-invasively using 99mTc-DPD scintigraphy. Transthyretin (TTR) gene sequencing was performed.
RESULTS:
In 2017-2019, 10 unrelated male patients were diagnosed with hereditary ATTR. All patients had very uncommon TTR gene mutations: 7 patients had p.Phe53Leu mutation, 2 patients had p.Glu109Lys mutation and 1 patient had p.Ala101Val mutation. The age of onset ranged from 49 to 67 years (mean [SD] age, 58.7 [6.4] years). On ECG, most patients (70%) had pseudoinfarct pattern and/or low QRS voltage. The maximal wall thickness (MWT) on echocardiography varied considerably among the patients from moderate (16 mm) to massively increased (30 mm). Most patients (90%) had decreased LV ejection fraction (mean [SD], 43 [11] %). On follow-up, we observed progressive heart failure in almost all cases. The first patient with p.Phe53Leu mutation died of heart failure, the second died suddenly, the third successfully underwent combined heart and liver transplant with 15 months survival from the surgery. The patient with p.Ala101Val mutation died of stroke.
CONCLUSIONS:
According to available research, this is the first time types of TTR mutations and clinical characteristics of Polish patients with cardiac hereditary ATTR have been reported. Previous literature data about Polish background in families with p.Phe53Leu mutation and the present results, suggest that this TTR mutation might be endemic in the Polish population.
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Hearts and bones: choose your tracers wisely.
Eur Heart J2020 Aug;():. doi: ehaa653.
Chandrashekar Pranav, Al-Rashdan Lana, Dale Zack, Masri Ahmad,
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Imaging Techniques As An Aid In The Early Detection Of Cardiac Amyloidosis.
Curr Pharm Des2020 Aug;():. doi: 10.2174/1381612826666200813133557.
Santarelli M F, Scipioni M, Genovesi D, Giorgetti A, Marzullo P, Landini L,
Abstract
The idea that performing a proper succession of imaging tests and techniques allows an accurate and early diagnosis of cardiac amyloidosis, avoiding the need to perform myocardial biopsy, is becoming increasingly popular. Furthermore, being imaging techniques non-invasive, it is possible to perform the follow-up of the pathology through repeated image acquisitions. In the present review, the various innovative imaging methodologies are presented, and it is discussed how they have been applied for early diagnosis of cardiac amyloidosis (CA), also to distinguish the two most frequent subtypes in CA: immunoglobulin light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR); this allows to perform the therapy in a targeted and rapid manner.
Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.
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A Review of Patisiran (ONPATTRO®) for the Treatment of Polyneuropathy in People with Hereditary Transthyretin Amyloidosis.
Neurol Ther2020 Aug;():. doi: 10.1007/s40120-020-00208-1.
Urits Ivan, Swanson Daniel, Swett Michael C, Patel Anjana, Berardino Kevin, Amgalan Ariunzaya, Berger Amnon A, Kassem Hisham, Kaye Alan, Viswanath Omar,
Abstract
Hereditary variant transthyretin amyloidosis (ATTRv) is a rare genetic defect that affects about 5000-10,000 people worldwide, causing amyloidosis secondary to misfolding of mutant transthyretin (TTR) protein fibrils. TTR mutations can cause protein deposits in many extracellular regions of organs, but those deposits in cardiac and axonal cells are the primary cause of this clinical syndrome. Treatment options are limited, but new drugs are being developed. Patisiran, a novel drug, is a liposomal siRNA against TTR that specifically targets this protein, reducing the accumulation of TTR in tissues, with subsequent improvement in both neuropathy and cardiac function. Patisiran is likely to serve as a prototype for the development of further intelligent drug solutions for use in targeted therapy. In this review we summarize the evidence currently available on the treatment of polyneuropathy in people with ATTRv with patisiran. We review the evidence on its efficacy, safety, and indications of use, citing novel and seminal papers on these subjects.
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Analysis of cardiopulmonary findings in COVID-19 fatalities: High incidence of pulmonary artery thrombi and acute suppurative bronchopneumonia.
Cardiovasc Pathol2020 Jul;49():107263. doi: S1054-8807(20)30067-3.
Grosse Claudia, Grosse Alexandra, Salzer Helmut J F, Dünser Martin W, Motz Reinhard, Langer Rupert,
Abstract
Since its recognition in December 2019, coronavirus disease 2019 (COVID-19) caused by SARS-CoV-2 has rapidly spread globally causing a pandemic that represents the greatest medical challenge in decades. The aim of the study was to evaluate the spectrum of cardiopulmonary pathology of COVID-19 based on (non-minimal invasive) autopsies performed on 14 COVID-19 decedents. Bilateral diffuse alveolar damage (DAD) was found in all patients. Superimposed acute bronchopneumonia was present in 11 of 14 (78.6%) patients and was considered the major cause of death in 2 patients. A key finding was the presence of thrombotic/thromboembolic vascular occlusions. We classified 5 types of pulmonary thrombi: 1. capillary microthrombi (11/14, 78.6%); 2. partially organized thrombi in mid-sized pulmonary arteries with complete vessel occlusion; 3. non-organized thrombi in mid-sized pulmonary arteries that did not completely fill out the vessel lumen and probably represented thromboemboli rather than thrombosis; 4. bone marrow emboli (1/14, 7.1%); and 5. septic pulmonary thromboemboli (1/14, 7.1%). Pulmonary thrombi in mid-sized arteries were noted in 5 of 14 (35.7%) patients, causing pulmonary infarction and/or pulmonary hemorrhage. All patients had evidence of chronic cardiac disease, including myocardial hypertrophy (13/14, 92.9%), mild to marked coronary artery atherosclerosis (14/14, 100%) and focal myocardial fibrosis (3/14, 21.4%). Acute myocardial infarction was found as concurrent cause of death in 3 (21.4%) patients, and significant cardiac hypertrophy (heart weight 750 g) was present in 1 (7.1%) patient with ATTR-positive cardiac amyloidosis. The autopsy findings confirm that COVID-19 is a systemic disease, with major involvement of the lungs, that increases the risk of cardiac and vascular complications including acute myocardial injury and thrombotic/thromboembolic events. Secondary acute bronchopneumonia is a common complication in patients with COVID-19 and may be the major cause of death.
Copyright © 2020. Published by Elsevier Inc.
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Next generation flow cytometry for MRD detection in patients with AL amyloidosis.
Amyloid2020 Aug;():1-5. doi: 10.1080/13506129.2020.1802713.
Kastritis Esftathios, Kostopoulos Ioannis V, Theodorakakou Foteini, Fotiou Despina, Gavriatopoulou Maria, Migkou Magdalini, Tselegkidi Maria Irini, Roussou Maria, Papathoma Alexandra, Eleutherakis-Papaioakovou Evangelos, Dialoupi Ioanna, Kanellias Nikolaos, Ntalianis Argyrios, Rousakis Pantelis, Trougakos Ioannis P, Tsitsilonis Ourania, Gakiopoulou Charikleia, Terpos Evangelos, Dimopoulos Meletios A,
Abstract
The treatment of AL amyloidosis aims to eradicate the plasma cell clone and eliminate toxic free light chain production. Only in a minority of patients the plasma cell clone is completely eradicated; residual light chain production may still exist while clonal relapse may occur. We used sensitive next-generation flow cytometry (NGF) to detect minimal residual disease (MRD) in AL amyloidosis patients at complete haematologic response. MRD evaluation was feasible in 51 of 52 (98%) tested patients and at a median sensitivity of 2.3?×?10 MRD was undetectable in 23 (45%). An organ response occurred in 86% of MRDneg vs 77% in MRDpos; renal response in 15/17(88%) of MRDneg vs in 14/16(87.5%) of MRDpos and cardiac response in 10/10(100%) of MRDneg vs 11/15(73%) of MRDpos patients. After a median follow-up of 24?months post MRD testing, no MRDneg patient had a haematologic relapse vs 6/28(21%) MRDpos (?=?.029). Pooling haematologic and organ progressions, 9 (32%) MRDpos patients had disease progression vs only 1 (4%) MRDneg patient (?=?.026). In conclusion, MRD detection using NGF has profound clinical implications, so that AL patients with undetectable MRD have a very high probability of organ response and a very low probability of haematologic relapse.
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Sudden death in lambda light chain AL cardiac amyloidosis: a review of literature and update for clinicians and pathologists.
Int J Clin Exp Pathol2020 ;13(7):1474-1482.
D'Errico Stefano, Mazzanti Andrea, Baldari Benedetta, Maiese Aniello, Frati Paola, Fineschi Vittorio,
Abstract
Light chain (AL) amyloidosis is the most common type of systemic amyloidosis, affecting around 10 people per million per year. In Europe, approximately 5000 new diagnosis per year are reported. Deposition of amyloid fibrils derived from antibody light chains are key pathogenic agents in AL amyloidosis. They can be deposited in multiple organs but cardiac involvement carries a major risk of mortality. The prognosis is poor in cases associated with multiple myeloma. The average survival is around 1 year. Up to half of all patients with cardiac amyloidosis die suddenly; 75% ofthose deaths are due to heart failure. Ventricular arrhythmia is also associated with cardiac amyloidosis and unexpected death. It is crucial to make a diagnosis and start treatment at an early stage. Recent data suggest that cardiac amyloidosis has become a treatable and curable condition with a combination of agents targeting multiple steps of the amyloid cascade. ICD implantation may not be as effective for the therapy of light chain (AL) cardiac amyloidosis as supposed earlier. In cases of unexpected and sudden death, autopsy may show unknown conditions and is valuable to assess existing risks for family members. Even after careful autopsy, a proportion of sudden deaths, ranging from 2 to 54%, remain unexplained and this broad range of values is likely due to the heterogeneity of autopsy protocols. Post mortem diagnosis of cardiac amyloidosis still represents a challenge for forensic pathologists. Detailed morphologic study of the heart and a complete histopathologic study are mandatory. Immunohistochemistry is essential for amyloid subclassification. A review of existing literature is performed by the authors and a methodological approach in post mortem diagnosis of light chain AL cardiac amyloidosis is proposed. Both macroscopic and microscopic findings are discussed.
IJCEP Copyright © 2020.
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Trends, burden, and impact of arrhythmia on cardiac amyloid patients: A 16-year nationwide study from 1999 to 2014.
J Arrhythm2020 Aug;36(4):727-734. doi: 10.1002/joa3.12376.
Isath Ameesh, Correa Ashish, Siroky Gregory P, Perimbeti Stuthi, Mohammed Selma, Chahal C Anwar A, Padmanabhan Deepak, Mehta Davendra,
Abstract
Background:
Patients with cardiac amyloidosis (CA) have increased mortality, which can be explained in part by an increased risk of arrhythmias. The burden of arrhythmias in CA, their predictors, and impact on in-hospital outcomes remains unclear. The role of implantable cardioverter-defibrillators (ICD) in this population is also uncertain.
Methods:
We queried the National Inpatient Sample (NIS) using ICD-9-CM codes 277.39 and 425.7 to identify CA. Twelve common arrhythmias were extracted using appropriate, validated ICD-9-CM codes. ICD implantation was identified using procedure ICD-9 codes 37.94 to 37.98, 00.51 and 00.54.
Results:
There were a total of 145,920 CA hospitalizations between 1999 and 2014 in the United States and 56,199 (38.5%) of them were associated with arrhythmias. The prevalence of arrhythmias remained relatively constant from 41.5% in 1999 to 40.2% in 2014. The most common arrhythmia was atrial fibrillation (25.4%). In-patient mortality was significantly higher in CA patients with arrhythmias (10.4% vs 6.5%, < .001). ICD implantation was performed in 1,381 (0.94%) patients with CA and analysis revealed an incremental trend in implantation over the study period (0.48% in 1999 to 0.65% in 2014). In-hospital mortality was significantly lower in patients who underwent ICD implantation (3.7% vs 8%; = .0078). CA patients with arrhythmias also had an increased cost of hospitalization and length of stay ($65,046 ± 1,079 vs $53,322 ± 687 and 8.3 ± 0.1 vs 7.4 ± 0.1 days, respectively; < .0001).
Conclusion:
Cardiac arrhythmias are common in patients with CA and are associated with worse in-hospital outcomes, increased length of stay, and cost of hospitalization.
© 2020 The Authors. Journal of Arrhythmia published by John Wiley & Sons Australia, Ltd on behalf of the Japanese Heart Rhythm Society.
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Significant role of magnetic resonance imaging for the diagnosis and evaluation of cardiac amyloidosis in primary light chain amyloidosis.
J Clin Exp Hematop2020 Aug;():. doi: 10.3960/jslrt.19040.
Sekiguchi Yasunobu, Wakabayashi Mutsumi, Iizuka Hiroko, Takizawa Haruko, Sugimoto Keiji, Sakajiri Sakura, Inano Tadaaki, Fukuda Yasutaka, Hamano Yasuharu, Tomita Shigeki, Izumi Hiroshi, Isogai Hiroyuki, Okubo Mitsuo, Nakamura Noriko, Sawada Tomohiro, Matsumoto Kimihiro, Noguchi Masaaki,
Abstract
A 47-year-old male with macroglossia presented with dyspnea on effort and chest pain at rest. Cardiac MRI revealed diffuse global subendocardial late gadolinium enhancement below the left ventricular endocardium and a dark blood pool of intracardiac contrast medium. Tongue biopsy revealed amyloid deposition, which was limited in the myocardium. He was diagnosed with primary light chain amyloidosis. His condition was stage I according to the Mayo Clinic staging system. He underwent autologous peripheral blood stem cell transplantation. On Day 10, he developed chest pain and died suddenly on Day 11. Postmortem examination revealed amyloid deposition throughout the heart.
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Amyloidosis Masquerading as Alcohol-Related Cirrhosis.
Cureus2020 Jul;12(7):e8976. doi: 10.7759/cureus.8976.
Nagra Navroop, Burman Blaire, Gault Christopher R, Dorer Russell, Siddique Asma,
Abstract
Amyloidosis can affect multiple organs, and involvement of the heart is the most common cause of death. Signs and symptoms vary depending upon the organ system affected by amyloid. Liver involvement is often seen, but symptoms are usually mild and nonspecific in isolated hepatic amyloidosis. None of the laboratory markers and imaging is characteristic of this condition; therefore, diagnosis is often delayed. Tissue biopsy is required for definitive diagnosis. Herein, we report a case where the patient's symptoms had been attributed to alcohol-related cirrhosis; however, further workup ultimately led to a diagnosis of systemic amyloidosis with multi-organ involvement.
Copyright © 2020, Nagra et al.
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Insight from an autopsy in a patient with rapidly worsening heart failure due to amyloid light-chain cardiac amyloidosis: A case report.
J Cardiol Cases2020 Aug;22(2):48-51. doi: 10.1016/j.jccase.2020.04.002.
Yokoyama Hiroaki, Shishido Koki, Ito Junko, Kamata Wataru, Katoh Nagaaki, Saito Shigeru,
Abstract
Amyloid light-chain (AL) amyloidosis is a systemic disease characterized by the deposition of misfolded immunoglobulin light chain, causing organ failure, and in particular cardiac involvement is a leading cause of morbidity and mortality. We report the case of a 47-year-old man without prior cardiovascular events who presented with shortness of breath. He was diagnosed with primary AL cardiac amyloidosis (CA) from the laboratory test, the endomyocardial biopsy, the bone marrow examination, and the cardiovascular imaging. Only a week after discharge of the first heart failure (HF) admission, he was readmitted for the exacerbation of HF. Finally, he died 2 weeks after the second admission, that is 3 months after the onset of HF. Autopsy, which was performed to investigate the causes of rapid worsening HF, implied the impairment of ventricular function and coronary microcirculation dysfunction. We could diagnose CA immediately by using diagnostic tools, however, we recognized that there was the fulminant type in CA, and considered the insight from autopsy. ? This case demonstrates a young patient with cardiac amyloidosis (CA) developed rapid worsening heart failure (RWHF), and then he died 1 month after diagnosis, that is 3 months after the onset of heart failure. This case deems to be a fulminant type in amyloid light-chain CA, and autopsy suggested the mechanisms of RWHF, which are the impairment of ventricular function and coronary microcirculation dysfunction.?.
© 2020 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
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F-Florbetaben and PET/CT Holds Promise for the Identification and Differentiation Among Cardiac Amyloidosis Entities.
JACC Cardiovasc Imaging2020 Aug;():. doi: S1936-878X(20)30519-2.
Schindler Thomas H, Sharma Vijay, Imperiale Alessio,
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[18F]-Florbetaben PET/CT for Differential Diagnosis Among Cardiac Immunoglobulin Light Chain, Transthyretin Amyloidosis, and Mimicking Conditions.
JACC Cardiovasc Imaging2020 Aug;():. doi: S1936-878X(20)30521-0.
Genovesi Dario, Vergaro Giuseppe, Giorgetti Assuero, Marzullo Paolo, Scipioni Michele, Santarelli Maria Filomena, Pucci Angela, Buda Gabriele, Volpi Elisabetta, Emdin Michele,
Abstract
OBJECTIVES:
This study aimed to test the diagnostic value of fludeoxyglucose F 18 ([18F])-florbetaben positron emission tomography (PET) in patients with suspicion of CA.
BACKGROUND:
Diagnosis of cardiac involvement in immunoglobulin light-chain-derived amyloidosis (AL) and transthyretin-related amyloidosis (ATTR), which holds major importance in risk stratification and decision making, is frequently delayed. Furthermore, although diphosphonate radiotracers allow a noninvasive diagnosis of ATTR, demonstration of cardiac amyloidosis (CA) in AL may require endomyocardial biopsy.
METHODS:
Forty patients with biopsy-proven diagnoses of CA (20 ALs, 20 ATTRs) and 20 patients referred with the initial clinical suspicion and later diagnosed with non-CA pathology underwent a cardiac PET/computed tomography scan with a 60-min dynamic [18F]-florbetaben PET acquisition, and 4 10-min static scans at 5, 30, 50, and 110 min after radiotracer injection.
RESULTS:
Visual qualitative assessment showed intense early cardiac uptake in all subsets. Patients with AL displayed a high, persistent cardiac uptake in all the static scans, whereas patients with ATTR and those with non-CA showed an uptake decrease soon after the early scan. Semiquantitative assessment demonstrated higher mean standardized uptake value (SUV) in patients with AL, sustained over the whole acquisition period (early SUV: 5.55; interquartile range [IQR]: 4.00 to 7.43; vs. delayed SUV: 3.50; IQR: 2.32 to 6.10; p = NS) compared with in patients with ATTR (early SUV: 2.55; IQR: 1.80 to 2.97; vs. delayed SUV: 1.25; IQR: 0.90 to 1.60; p < 0.001) and in patients with non-CA (early SUV: 3.50; IQR: 1.60 to 3.37; vs. delayed SUV: 1.40; IQR: 1.20 to 1.60; p < 0.001). Similar results were found comparing heart-to-background ratio and molecular volume.
CONCLUSIONS:
Delayed [18F]-florbetaben cardiac uptake may discriminate CA due to AL from either ATTR or other mimicking conditions. [18F]-florbetaben PET/computed tomography may represent a promising noninvasive tool for the diagnosis of AL amyloidosis, which is still often challenging and delayed. (A Prospective Triple-Arm, Monocentric, Phase-II Explorative Study on Evaluation of Diagnostic Efficacy of the PET Tracer [18F]-Florbetaben [Neuraceq] in Patients With Cardiac Amyloidosis [FLORAMICAR2]; EudraCT number: 2017-001660-38).
Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
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Identifying Cardiac Amyloid in Aortic Stenosis: ECV Quantification by CT in TAVR Patients.
JACC Cardiovasc Imaging2020 Aug;():. doi: S1936-878X(20)30509-X.
Scully Paul R, Patel Kush P, Saberwal Bunny, Klotz Ernst, Augusto João B, Thornton George D, Hughes Rebecca K, Manisty Charlotte, Lloyd Guy, Newton James D, Sabharwal Nikant, Kelion Andrew, Kennon Simon, Ozkor Muhiddin, Mullen Michael, Hartman Neil, Cavalcante João L, Menezes Leon J, Hawkins Philip N, Treibel Thomas A, Moon James C, Pugliese Francesca,
Abstract
OBJECTIVES:
To validate computed tomography measured ECV (ECV) as part of routine evaluation for the detection of cardiac amyloid in patients with aortic stenosis (AS)-amyloid.
BACKGROUND:
AS-amyloid affects 1 in 7 elderly patients referred for transcatheter aortic valve replacement (TAVR). Bone scintigraphy with exclusion of a plasma cell dyscrasia can diagnose transthyretin-related cardiac amyloid noninvasively, for which novel treatments are emerging. Amyloid interstitial expansion increases the myocardial extracellular volume (ECV).
METHODS:
Patients with severe AS underwent bone scintigraphy (Perugini grade 0, negative; Perugini grades 1 to 3, increasingly positive) and routine TAVR evaluation CT imaging with ECV using 3- and 5-min post-contrast acquisitions. Twenty non-AS control patients also had ECV performed using the 5-min post-contrast acquisition.
RESULTS:
A total of 109 patients (43% male; mean age 86 ± 5 years) with severe AS and 20 control subjects were recruited. Sixteen (15%) had AS-amyloid on bone scintigraphy (grade 1, n = 5; grade 2, n = 11). ECV was 32 ± 3%, 34 ± 4%, and 43 ± 6% in Perugini grades 0, 1, and 2, respectively (p < 0.001 for trend) with control subjects lower than lone AS (28 ± 2%; p < 0.001). ECV accuracy for AS-amyloid detection versus lone AS was 0.87 (0.95 for Tc-3,3-diphosphono-1,2-propanodicarboxylic acid Perugini grade 2 only), outperforming conventional electrocardiogram and echocardiography parameters. One composite parameter, the voltage/mass ratio, had utility (similar AUC of 0.87 for any cardiac amyloid detection), although in one-third of patients, this could not be calculated due to bundle branch block or ventricular paced rhythm.
CONCLUSIONS:
ECV during routine CT TAVR evaluation can reliably detect AS-amyloid, and the measured ECV tracks the degree of infiltration. Another measure of interstitial expansion, the voltage/mass ratio, also performed well.
Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.
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Teaching an Old Dog New Tricks: Using Cardiac CT for Comprehensive Imaging.
JACC Cardiovasc Imaging -
The Role of Echocardiography in the Cancer Patient.
Curr Cardiol Rep2020 Aug;22(10):103. doi: 10.1007/s11886-020-01373-3.
Palaskas Nicolas L, Lopez-Mattei Juan,
Abstract
PURPOSE OF REVIEW:
To review the uses of echocardiography in patients with cancer and how it has expanded beyond the typical monitoring of systolic function during potentially cardiotoxic cancer therapeutics.
RECENT FINDINGS:
In addition to myocardial strain imaging being a predictor of subsequent left ventricular dysfunction, it can be used for pattern recognition to help identify patients with cardiac amyloidosis or Takotsubo cardiomyopathy. Echocardiography is essential for diagnosis and planning of intervention for aortic stenosis in radiation-induced valvular disease, for which transcutaneous aortic valve replacement that gives many cancer patients that are not surgical candidates an option for treatment. The safety of transesophageal echocardiography has recently been demonstrated in patients with cancer with thrombocytopenia and depleted white blood cell counts who are at increased risk of endocarditis. Echocardiography is an essential tool for evaluating common conditions in cancer patients such as pericardial disease, radiation-induced heart disease, and intracardiac tumors-with specific uses of specialized echocardiography techniques such as deformation imaging, transesophageal echocardiography, and point-of-care ultrasound.
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Review of Transthyretin Silencers, Stabilizers, and Fibril Removal Agents in the Treatment of Transthyretin Cardiac Amyloid.
Curr Cardiol Rep2020 Aug;22(10):106. doi: 10.1007/s11886-020-01374-2.
Hough Augustus, Wearden Jessica, de Almeida Kristen, Kaiser Stephanie,
Abstract
PURPOSE OF REVIEW:
To provide a functional review for practicing clinicians on the current and emerging treatment considerations for transthyretin (TTR) cardiac amyloidosis (ATTR-CA).
RECENT FINDINGS:
Current treatment considerations are characterized as those silencing TTR translation, stabilizing TTR tetramers, and disrupting amyloid fibril deposition. Historically considered a rare disease state, ATTR-CA is increasingly recognized as an important mediator of heart failure morbidity and mortality. The emergence of widely available therapies for ATTR-CA has developed hope for patients where little was previously present. Thus, it is important that all cardiology clinicians have a functional understanding of the disease state and treatment options. This review will discuss agents within each of the above classes with expanded discussion on tafamidis given its favorable efficacy, safety, and availability. ATTR-CA diagnostic considerations are reviewed with regard to the identification of potential tafamidis candidates, and practical economic considerations are also reviewed.
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Protective Effects of PACAP in Peripheral Organs.
Front Endocrinol (Lausanne)2020 ;11():377. doi: 10.3389/fendo.2020.00377.
Toth Denes, Szabo Edina, Tamas Andrea, Juhasz Tamas, Horvath Gabriella, Fabian Eszter, Opper Balazs, Szabo Dora, Maugeri Grazia, D'Amico Agata G, D'Agata Velia, Vicena Viktoria, Reglodi Dora,
Abstract
Pituitary adenylate cyclase activating polypeptide (PACAP) is a neuropeptide widely distributed in the nervous system, where it exerts strong neuroprotective effects. PACAP is also expressed in peripheral organs but its peripheral protective effects have not been summarized so far. Therefore, the aim of the present paper is to review the existing literature regarding the cytoprotective effects of PACAP in non-neuronal cell types, peripheral tissues, and organs. Among others, PACAP has widespread expression in the digestive system, where it shows protective effects in various intestinal pathologies, such as duodenal ulcer, small bowel ischemia, and intestinal inflammation. PACAP is present in both the exocrine and endocrine pancreas as well as liver where it reduces inflammation and steatosis by interfering with hepatic pathology related to obesity. It is found in several exocrine glands and also in urinary organs, where, with its protective effects being mainly published regarding renal pathologies, PACAP is protective in numerous conditions. PACAP displays anti-inflammatory effects in upper and lower airways of the respiratory system. In the skin, it is involved in the development of inflammatory pathology such as psoriasis and also has anti-allergic effects in a model of contact dermatitis. In the non-neuronal part of the visual system, PACAP showed protective effects in pathological conditions of the cornea and retinal pigment epithelial cells. The positive role of PACAP has been demonstrated on the formation and healing processes of cartilage and bone where it also prevents osteoarthritis and rheumatoid arthritis development. The protective role of PACAP was also demonstrated in the cardiovascular system in different pathological processes including hyperglycaemia-induced endothelial dysfunction and age-related vascular changes. In the heart, PACAP protects against ischemia, oxidative stress, and cardiomyopathies. PACAP is also involved in the protection against the development of pre-senile systemic amyloidosis, which is presented in various peripheral organs in PACAP-deficient mice. The studies summarized here provide strong evidence for the cytoprotective effects of the peptide. The survival-promoting effects of PACAP depend on a number of factors which are also shortly discussed in the present review.
Copyright © 2020 Toth, Szabo, Tamas, Juhasz, Horvath, Fabian, Opper, Szabo, Maugeri, D'Amico, D'Agata, Vicena and Reglodi.
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Cardiac Imaging in the Post-ISCHEMIA Trial Era: A Multisociety Viewpoint.
JACC Cardiovasc Imaging2020 Aug;13(8):1815-1833. doi: S1936-878X(20)30343-0.
Shaw Leslee, Kwong Raymond Y, Nagel Eike, Salerno Michael, Jaffer Farouc, Blankstein Ron, Dilsizian Vasken, Flachskampf Frank, Grayburn Paul, Leipsic Jonathan, Marwick Tom, Nieman Koen, Raman Subha, Sengupta Partho, Zoghbi William, Pellikka Patricia A, Swaminathan Madhav, Dorbala Sharmila, Thompson Randall, Al-Mallah Mouaz, Calnon Dennis, Polk Donna, Soman Prem, Beanlands Rob, Garrett Kirk N, Henry Timothy D, Rao Sunil V, Duffy Peter L, Cox David, Grines Cindy, Mahmud Ehtisham, Bucciarelli-Ducci Chiara, Plein Sven, Greenwood John P, Berry Colin, Carr James, Arai Andrew E, Murthy Venkatesh L, Ruddy Terrence D, Chandrashekhar Y,
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