Pubblicazioni recenti - dilated cardiomyopathy
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Acute heart failure due to dilated cardiomyopathy exacerbated by systemic parechovirus-A1 infection in an infant.
Int J Infect Dis2021 Jan;():. doi: S1201-9712(21)00031-X.
Maki Shun, Aizawa Yuta, Ito Yuki, Suda Masashi, Saitoh Akihiko,
Abstract
Parechovirus-A1 (PeV-A1) often causes mild respiratory or gastrointestinal disease. Herein we report a case of acute heart failure due to dilated cardiomyopathy (DCM) exacerbated by acute PeV-A1 infection in a 10-month-old infant. He presented to our hospital with acute respiratory distress and compensated shock. Echocardiogram showed a dilated left ventricle and severe mitral regurgitation, consistent with DCM. PeV-A1 infection was confirmed by 1) positive PCR for PeV-A in multiple anatomical sites including blood, stool, and throat swab samples, 2) genetic sequence of viral protein, and 3) an increase in paired serum PeV-A1-specific neutralizing antibody titers. A few, scattered case reports in infants and young children also indicate the association between myocarditis and/or DCM and PeV-A1 infection. In conclusion, PeV-A1 infection could be associated with exacerbation of myocardial diseases in infants and young children; thus PeV-A1 needs to be evaluated as a viral cause of such condition.
Copyright © 2021. Published by Elsevier Ltd.
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-Associated Ventricular Arrhythmias in a Patient with Structurally Normal Heart.
Genes (Basel)2021 Jan;12(1):. doi: E94.
Vakhrushev Yuriy, Kozyreva Alexandra, Semenov Andrey, Sokolnikova Polina, Lubimtseva Tamara, Lebedev Dmitry, Smolina Natalia, Zhuk Sergey, Mitrofanova Lubov, Vasichkina Elena, Kostareva Anna,
Abstract
(RNA-binding motif protein 20) is a splicing factor targeting multiple cardiac genes, and its mutations cause cardiomyopathies. Originally, mutations were discovered to cause the development of dilated cardiomyopathy by erroneous splicing of the gene (titin). Titin is a giant protein found in a structure of the sarcomere that functions as a molecular spring and provides a passive stiffness to the cardiomyocyte. Later, mutations were also described in association with arrhythmogenic right ventricular cardiomyopathy and left ventricular noncompaction cardiomyopathy. Here, we present a clinical case of a rare arrhythmogenic phenotype and no structural cardiac abnormalities associated with a genetic variant of uncertain significance.
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Catecholaminergic polymorphic ventricular tachycardia complicated by dilated cardiomyopathy: a case report.
Eur Heart J Case Rep2020 Dec;4(6):1-6. doi: 10.1093/ehjcr/ytaa299.
Christina Granitz, Peter Jirak, Bernhard Strohmer, Gerhard Pölzl,
Abstract
Background :
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a severe genetic arrhythmogenic disorder characterized by adrenergically induced ventricular tachycardia manifesting as stress-induced syncope and sudden cardiac death. While CPVT is not associated with dilated cardiomyopathy (DCM) in most cases, the combination of both disease entities poses a major diagnostic and therapeutic challenge.
Case summary :
We present the case of a young woman with CPVT. The clinical course since childhood was characterized by repetitive episodes of exercise-induced ventricular arrhythmias and a brady-tachy syndrome due to rapid paroxysmal atrial fibrillation and sinus bradycardia. Medical treatment included propranolol and flecainide until echocardiography showed a dilated left ventricle with severely depressed ejection fraction when the patient was 32?years old. Cardiac magnetic resonance imaging revealed non-specific late gadolinium enhancement. Myocardial inflammation, however, was excluded by subsequent endomyocardial biopsy. Genetic analysis confirmed a mutation in the cardiac ryanodine receptor but no pathogenetic variant associated with DCM. Guideline-directed medical therapy for HFrEF was limited due to symptomatic hypotension. Over the next months, the patient developed progressive heart failure symptoms that were finally managed by heart transplantation.
Discussion :
Management in patients with CPVT and DCM is challenging, as Class I antiarrhythmic drugs are not recommended in structural heart disease and prophylactic internal cardioverter-defibrillator implantation without adjuvant antiarrhythmic therapy can be detrimental. Regular echocardiographic screening for DCM is recommendable in patients with CPVT. A multidisciplinary team of heart failure specialists, electrophysiologists, geneticists, and imaging specialists is needed to collaborate in the delivery of clinical care.
© The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology.
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A case report of advanced heart failure refractory to pharmacological therapy who was successfully recovered by combinatory usage of cardiac resynchronizing therapy, Impella and MitraClip.
Eur Heart J Case Rep2020 Dec;4(6):1-5. doi: 10.1093/ehjcr/ytaa418.
Sobajima Mitsuo, Fukuda Nobuyuki, Ueno Hiroshi, Kinugawa Koichiro,
Abstract
Background :
The safety and efficacy of MitraClip for advanced heart failure (HF) patients who are inotrope-dependent or mechanically supported are unknown.
Case summary :
The patient was a 71-year-old man diagnosed as dilated cardiomyopathy in 2003. He was admitted due to worsening HF in January 2019 and became dependent upon intravenous infusion of inotropes. During the 8-month hospitalization, his haemodynamics were relatively static with bed rest and continuous inotropes, but he was definitely dependent on them. Our multidisciplinary team decided to perform both cardiac resynchronization therapy (CRT) and MitraClip under Impella support. First, Impella was inserted from left subclavian artery. After a week, CRT was implanted from right subclavian vein, and the QRS duration of electrocardiogram became remarkably narrow. MitraClip was performed 2 weeks after Impella, and functional mitral regurgitation improved from severe to mild, and Impella was removed on the same day. Inotropes could be ceased, and he was discharged 2 months after MitraClip.
Discussion :
During inotrope-dependent status, there was a risk that HF would worsen with haemodynamic collapse when performing CRT implantation, and we firstly supported his haemodynamics by Impella. Cardiac resynchronization therapy implantation before MitraClip seemed to be crucial. In fact, the mitral valve morphology before Impella insertion had very poor coaptation of the anterior and posterior leaflets that was not optimal for MitraClip procedure. But the Impella support and correction of dyssynchrony by CRT markedly improved the coaptation of those leaflets. The combination therapy of CRT and MitraClip unloading with Impella maybe a new therapeutic option for advanced HF.
© The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology.
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Clinical Considerations for a Family with Dilated Cardiomyopathy, Sudden Cardiac Death, and a Novel Frameshift Mutation.
Int J Mol Sci2021 Jan;22(2):. doi: E670.
Micaglio Emanuele, Monasky Michelle M, Bernardini Andrea, Mecarocci Valerio, Borrelli Valeria, Ciconte Giuseppe, Locati Emanuela T, Piccoli Marco, Ghiroldi Andrea, Anastasia Luigi, Pappone Carlo,
Abstract
Dilated cardiomyopathy (DCM) is the leading indication for heart transplantation. gene truncating mutations account for about 25% of familial DCM cases and for 18% of sporadic DCM cases. The clinical relevance of specific variants in has been difficult to determine because of the sheer size of the protein for which encodes, as well as existing extensive genetic variation. Clinicians should communicate novel clinically-relevant variants and genotype-phenotype associations, so that animal studies evaluating the molecular mechanisms are always conducted with a focus on clinical significance. In the present study, we report for the first time the novel truncating heterozygous variant NM_001256850.1:c.72777_72783del (p.Phe24259Leufs*51) in the gene and its association with DCM in a family with sudden death. This variant occurs in the A-band region of the sarcomere, in a known mutational hotspot of the gene. Truncating titin variants that occur in this region are the most common cause of DCM and have been rarely reported in asymptomatic individuals, differently from other pathogenic gene variants. Further studies are warranted to better understand this particular clinically-relevant variant.
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Storm and STEMI: a case report of unexpected cardiac complications of thyrotoxicosis.
Eur Heart J Case Rep2020 Dec;4(6):1-5. doi: 10.1093/ehjcr/ytaa414.
Brown Josiah, Cham Matthew D, Huang Gary S,
Abstract
Background :
Thyroid storm is a rare condition with well-known cardiovascular manifestations including tachycardia, atrial fibrillation, heart failure, and myocardial infarction (MI). Several uncommon conditions that can mimic MI are associated with thyrotoxicosis and discussed in this case.
Case summary :
A 23-year-old previously healthy male presented after the onset of generalized weakness and inability to rise from bed in the setting of 35?kg of unintentional weight loss, and was found to have profound hypokalaemia, elevated thyroid hormone, and suppressed thyroid-stimulating hormone consistent with thyrotoxicosis secondary to Grave's disease. Following hospital admission, he developed worsening tachycardia with dynamic anteroseptal ST-segment elevations and elevated cardiac biomarkers concerning for MI. He was treated with aspirin, ticagrelor, and a heparin infusion, but was unable to tolerate beta-blockade acutely due to hypotension. Echocardiography demonstrated a severely dilated left ventricle (left ventricular end-diastolic volume index 114?mL/m) and severely reduced systolic function (ejection fraction 23%) with global hypokinesis. Following initiation of propylthiouracil, iodine solution, and stress-dosed steroids his tachycardia and ST-elevations resolved. Computed tomography (CT) coronary angiography demonstrated no evidence of coronary stenosis. He was discharged on methimazole, metoprolol, and lisinopril and found to have recovered left ventricular systolic function at 2-month follow-up.
Discussion :
Thyrotoxicosis can rarely cause coronary vasospasm, stress cardiomyopathy, and autoimmune myocarditis. These conditions should be suspected in hyperthyroid patients with features of MI and normal coronary arteries. Workup should include laboratory evaluation, electrocardiography (ECG), echocardiography, and non-invasive or invasive ischaemic evaluation.
© The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology.
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A case report of acute myopericarditis associated with Graves' thyrotoxicosis.
Eur Heart J Case Rep2020 Dec;4(6):1-5. doi: 10.1093/ehjcr/ytaa465.
Anderton Thomas S, Saunderson Christopher E D, Jain Manali, Sengupta Anshuman,
Abstract
Background :
The effects of hyperthyroidism on the heart are well documented, primarily consisting of supraventricular tachycardias, congestive heart failure, and dilated cardiomyopathy. Acute myopericarditis resulting from a hyperthyroid state is an uncommon but recognized association.
Case summary :
A 29-year-old man with a history of Graves' disease presented with chest pain and electrocardiogram changes suggestive of an infero-lateral ST-elevation myocardial infarction. However, emergent coronary angiography and bedside echocardiography were normal. Troponin-I was found to be >25 000?ng/L (normal value <57). Thyroid function tests showed a significantly raised free T4 and undetectable thyroid-stimulating hormone. Cardiovascular magnetic resonance (CMR) showed extensive myocardial oedema and late gadolinium enhancement (LGE) in keeping with acute myopericarditis, alongside an enlarged thyroid gland consistent with goitre. Propylthiouracil in combination with an angiotensin-converting enzyme inhibitor and beta-blocker were commenced and eventually definitive treatment with thyroidectomy was performed. Follow-up CMR at 6?months showed complete resolution of the prior noted oedema and a reduction in the location and extent of LGE with significant residual fibrosis.
Discussion :
Acute myopericarditis is a common diagnosis in young patients presenting with symptoms of chest pain with elevated troponin and is frequently related to a viral illness. Hyperthyroid states are also associated with acute myopericarditis and should be particularly considered in patients with a pre-existing thyroid condition or in those with symptoms suggestive of hyperthyroidism. Given the specific treatments required in a case of myopericarditis associated with hyperthyroidism, it is important to be aware of this association and consider screening where appropriate.
© The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology.
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Adriamycin inhibits glycolysis through downregulation of key enzymes in .
3 Biotech2021 Jan;11(1):15. doi: 10.1007/s13205-020-02530-9.
Mohan Uma Priya, Kunjiappan Selvaraj, Tirupathi Pichiah P B, Arunachalam Sankarganesh,
Abstract
Adriamycin is a widely used drug for the treatment of various types of cancers, but its clinical application is limited because of irreversible dilated cardiomyopathy. The incidence of cardiomyopathy is a consequence of disrupted energy production, which could be related to the defects in glycogen, lipid and mucopolysaccharide metabolism. We explored the effect of Adriamycin on enzymes involved in glycolysis and apoptotic genes through molecular docking. We used as model organism and studied the effect of Adriamycin on selected enzymes involved in glycolysis. The docking studies revealed that Adriamycin interacts with phosphofructokinase and enolase in an efficient manner. In phosphofructokinase, Adriamycin binds at the active site and with enolase the drug interacts at the cofactor-binding site (Mg) which might impair the activity of the enzyme. Gene expression studies revealed that Adriamycin causes the dysregulation of glycolysis through dysregulation of hexokinase, phosphoglycerate mutase, enolase and downregulation of pyruvate kinase. The drug shows a biphasic effect on the expression of genes enolase and pyruvate kinase. The impairment in glycolysis might reduce the ATP synthesis, and the cells might be deprived of energy. The condition is further worsened by elevated ROS levels triggering the cell to undergo apoptosis evidenced by downregulation of SOD and upregulation of BAX and caspase. In conclusion, our study reveals that Adriamycin impairs glycolysis and cause cell to undergo apoptosis due to oxidative stress in yeast cells.
© King Abdulaziz City for Science and Technology 2021.
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Simultaneous infection of abandoned leads and newly implanted leadless cardiac pacemaker: Why did this occur?
J Cardiol Cases2021 Jan;23(1):35-37. doi: 10.1016/j.jccase.2020.09.005.
Okada Ayako, Shoda Morio, Tabata Hiroaki, Kobayashi Hideki, Shoin Wataru, Okano Takahiro, Yoshie Koji, Kato Ken, Motoki Hirohiko, Kuwahara Koichiro,
Abstract
An 80-year-old man with a history of dilated hypertrophic cardiomyopathy received a dual-chamber pacemaker for sick sinus syndrome and atrioventricular block in February 2010. On May 30, 2019, he developed pocket erosion, with streaks of pus exuding from the pocket. The pacemaker generator was removed, although both capping leads were left buried under the skin, and a leadless pacemaker was implanted into the right ventricular (RV) apex the next day. Blood and pus cultures on July 15, 2019 indicated methicillin-resistant (MRSA). The patient was transferred to our hospital for simultaneous removal of both devices in August 2019. The RV lead and right atrial lead were extracted using a laser sheath and a mechanical sheath. A 23 Fr MICRA® sheath was inserted from the right femoral vein to accommodate an 8.5 Fr Agillis sheath. An Osypka LASSO snare catheter was advanced through the sheath to catch the distal aspect of the MICRA® body. Finally, the MICRA® device was completely removed through the sheath. Culture results for the lead tip and MICRA® were both MRSA positive. This is the first report of late-phase simultaneous infection of abandoned leads and implanted leadless cardiac pacemaker extraction. < Leadless pacemakers are becoming increasingly popular in high-risk patients due to no lead-associated complications. As a result of the incomplete removal, the remaining leads caused a drug-refractory blood stream infection, which secondarily infected the MICRA® device. Thus, an insufficiently treated pocket infection resulted in persistent methicillin-resistant bacteremia in this case.>.
© 2020 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
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Bocavirus Infection in a Young Pregnant Woman: A Case Report and Literature Review.
Am J Case Rep2021 Jan;22():e928099. doi: 10.12659/AJCR.928099.
Al Bishawi Ahmad, Ben Abid Fatma, Ibrahim Wanis,
Abstract
BACKGROUND Human bocavirus (HBoV) is a parvovirus found primarily in children and was first identified in 2005. It usually causes mild upper- and lower-respiratory tract infections. HBoV infection seems to be rare during adulthood, probably due to high antibody titers resulting from childhood infection and seroconversion. The clinical significance, possible complications, and consequences of an adulthood infection are still unclear. Furthermore, the consequences of HBoV infection during pregnancy are seldom reported in the literature. CASE REPORT We report the case of a 22-year-old pregnant woman in her third trimester who presented with a 1-week history of fever and cough followed by progressive shortness of breath. She was treated initially as a case of severe pneumonia; however, her condition deteriorated rapidly, resulting in hypoxic respiratory failure that required intensive care support. The patient was found to have dilated cardiomyopathy on echocardiography, and her fetal ultrasound showed no fetal heart activity; subsequently, labor induction for stillbirth was performed. An extensive workup for an underlying cause was unrevealing apart from positive respiratory viral PCR assay for human bocavirus, performed twice. A provisional diagnosis of HBoV pneumonia complicated by dilated cardiomyopathy, stillbirth, and multiorgan failure was made. Fortunately, the patient had a good recovery and was discharged home in good clinical condition. CONCLUSIONS In addition to severe pneumonia, HBoV infection may result in other life-threatening complications. Although the infection is rare during adulthood, infection in a pregnant woman should be taken seriously and close monitoring of such patients is advised.
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Thyrotoxic dilated cardiomyopathy: personal experience and case collection from the literature.
Endocrinol Diabetes Metab Case Rep2020 Dec;2020():. doi: 10.1530/EDM-20-0068.
Molinaro Giuseppina, De Vecchis Renato, Badolati Elio, Giannattasio Raffaele,
Abstract
Summary:
The authors examine several reports of the literature concerning thyrotoxic dilated cardiomyopathy. In particular, it is pointed out that this clinical manifestation of hyperthyroidism is rare in readily diagnosed and properly treated hyperthyroidism. Case reports are analyzed comparatively. A case deriving from the direct experience of the authors is also presented.
Learning points:
Dilated cardiomyopathy has been reported as the initial presentation of hyperthyroidism in only 6% of patients although <1% developed severe LV dysfunction. Clinical picture of thyrotoxic dilated cardiomyopathy can degenerate into an overt cardiogenic shock sometimes requiring the use of devices for mechanical assistance to the circulation, or extracorporeal membrane oxygenation. For thyrotoxic dilated cardiomyopathy, evidence-based pharmacologic measures valid for heart failure should always be supplemented by the administration of specific thyroid therapies such as thionamides (methimazole, carbimazole or propylthiouracil), whose relatively long latency of action should be supported by the i.v. administration of small doses of beta-blocker. In cases of cardiogenic shock, the administration of beta-blocker should be carried out only after the restoration of satisfactory blood pressure levels- with the prudent use of synthetic catecholamines, if necessary.
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Malaria and encephalopathy in a heart transplant recipient: a case report in the context of multiorgan donation.
Transpl Infect Dis2021 Jan;():e13565. doi: 10.1111/tid.13565.
Vernaza Armando, Pinilla-Monsalve Gabriel, Cañas Felipe, Carrillo Diana, López Juan David, Flórez Noel, Gómez Juan Esteban,
Abstract
INTRODUCTION:
Malaria is an endemic infection in tropical circles. It can be transmitted from mosquitoes bite, but exceptional cases have been attributed to multiorgan transplantation.
CASE REPORT:
This is a 34 years-old woman who received a heart transplant for final stage dilated cardiomyopathy. Over the hospitalization, she developed fever, cephalalgia, and tonic-clonic seizures with MRI findings compatible with posterior reversible encephalopathy. A thick blood smear revealed hemoparasitic forms of Plasmodium vivax. Afterward, malaria was also diagnosed in recipients of one kidney and liver of the same organ-donor. First-line treatment with artesunate was prescribed for three days and chloroquine with primaquine thereafter for fourteen days. The patient was discharged and returned to the emergency department five days later, complaining of gastrointestinal symptoms and develop multiorgan failure that led to death.
CONCLUSION:
We report a case of malaria transmission through heart transplantation. Despite adequate and supervised treatment, it can be related to a fatal outcome. Malaria screening in organ donors should be considered in regions where endemicity can lead to rare cases of transmission by transplantation.
This article is protected by copyright. All rights reserved.
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Plasminogen activator inhibitor-1 reduces cardiac fibrosis and promotes M2 macrophage polarization in inflammatory cardiomyopathy.
Basic Res Cardiol2021 Jan;116(1):1. doi: 10.1007/s00395-020-00840-w.
Baumeier Christian, Escher Felicitas, Aleshcheva Ganna, Pietsch Heiko, Schultheiss Heinz-Peter,
Abstract
Plasminogen activator inhibitor-1 (PAI-1) has a cardioprotective function in mice by repressing cardiac fibrosis through TGF-? and plasminogen-mediated pathways. In addition it is known to be involved in the recruitment and polarization of monocytes/macrophages towards a M2 phenotype in cancer. Here, we investigated the expression of PAI-1 in human dilated cardiomyopathy (DCM) and inflammatory dilated cardiomyopathy (DCMi) and its effect on cardiac fibrosis and macrophage polarization. We retrospectively analyzed endomyocardial biopsies (EMBs) of patients with DCM or DCMi for PAI-1 expression by immunohistochemistry. Furthermore, EMBs were evaluated for the content of fibrotic tissue, number of activated myofibroblasts, TGF-? expression, as well as for M1 and M2 macrophages. Patients with high-grade DCMi (DCMi-high, CD3 lymphocytes?>?30 cells/mm) had significantly increased PAI-1 levels compared to DCM and low-grade DCMi patients (DCMi-low, CD3 lymphocytes?=?14-30 cells/mm) (15.5?±?0.4% vs. 1.0?±?0.1% and 4.0?±?0.1%, p???0.001). Elevated PAI-1 expression in DCMi-high subjects was associated with a diminished degree of cardiac fibrosis, decreased levels of TGF-? and reduced number of myofibroblasts. In addition, DCMi-high patients revealed an increased proportion of non-classical M2 macrophages towards classical M1 macrophages, indicating M2 macrophage-favoring properties of PAI-1 in inflammatory cardiomyopathies. Our findings give evidence that elevated expression of cardiac PAI-1 in subjects with high-grade DCMi suppresses fibrosis by inhibiting TGF-? and myofibroblast activation. Moreover, our data indicate that PAI-1 is involved in the polarization of M2 macrophages in the heart. Thus, PAI-1 could serve as a potential prognostic biomarker and as a possible therapeutic target in inflammatory cardiomyopathies.
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Eosinophilic heart disease: diagnostic and prognostic assessment by cardiac magnetic resonance.
Eur Heart J Cardiovasc Imaging2021 Jan;():. doi: jeaa346.
Antonopoulos Alexios S, Azzu Alessia, Androulakis Emmanuel, Tanking Chonthicha, Papagkikas Panagiotis, Mohiaddin Raad H,
Abstract
AIMS :
Eosinophilic heart disease (EHD) is a rare cardiac condition with a wide spectrum of phenotypes. The diagnostic and prognostic value of cardiac magnetic resonance (CMR) in EHD remains unknown.
METHODS AND RESULTS :
This was a retrospective analysis of 250 patients with eosinophilia referred for a CMR scan (period 2000-2020). CMR data sets and clinical/laboratory data were collected. Patients were followed up for a mean of 24?months (range 1-224) for the composite endpoint of death, acute coronary syndrome, hospitalization for acute heart failure, malignant ventricular arrhythmias, or the need for implantable cardiac defibrillator/pacemaker. The main objectives were to explore the diagnostic value of CMR in EHD; relationships between cardiac function, late gadolinium enhancement (LGE), and EHD phenotypes; and the prognostic value of fibrosis and oedema by CMR. The prevalence of findings compatible with EHD was 39% (patients with cardiac symptoms: 57% vs. screening: 20%, P?0.001). EHD phenotypes included subendocardial LGE (n?=?58), mid-wall/subepicardial LGE (n?=?26), pericarditis (n?=?5) or dilated cardiomyopathy (n?=?8). Myocardial oedema was present in 10% of patients. Intracardiac thrombi (7%) were associated with EHD phenotype (?2=47.3, P?=?1.3×10-8). LGE extent correlated with LVEDVi (rho?=?0.268, P?=?5.3×10-5) and LVEF (rho=-0.415, P?=?8.6×10-11). A CMR scan positive for EHD [hazard ratio (HR)?=?5.61, 95% confidence interval (CI): 1.82-17.89, P?=?0.0026] or a subendocardial LGE pattern (HR?=?5.13, 95% CI: 1.29-20.38, P?=?0.020) were independently associated with the composite clinical endpoint.
CONCLUSION :
The diagnostic yield of CMR screening in patients with persistent eosinophilia, even if asymptomatic, is high. The extent of subendocardial fibrosis correlates with LV remodelling and independently predicts clinical outcomes in patients with eosinophilia.
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2021. For permissions, please email: journals.permissions@oup.com.
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Remote monitoring in heart failure: current and emerging technologies in the context of the pandemic.
Heart2021 Jan;():. doi: heartjnl-2020-318062.
Mohebali Donya, Kittleson Michelle M,
Abstract
The incidence of heart failure (HF) remains high and patients with HF are at risk for frequent hospitalisations. Remote monitoring technologies may provide early indications of HF decompensation and potentially allow for optimisation of therapy to prevent HF hospitalisations. The need for reliable remote monitoring technology has never been greater as the COVID-19 pandemic has led to the rapid expansion of a new mode of healthcare delivery: the virtual visit. With the convergence of remote monitoring technologies and reliable method of remote healthcare delivery, an understanding of the role of both in the management of patients with HF is critical. In this review, we outline the evidence on current remote monitoring technologies in patients with HF and highlight how these advances may benefit patients in the context of the current pandemic.
© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.
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Mechanisms, features, and significance of diastolic mitral regurgitation: a case series.
Eur Heart J Case Rep2020 Oct;4(5):1-8. doi: 10.1093/ehjcr/ytaa203.
Li Quan, Liu Yu, Zuo Wuxu, Chen Haiyan, Zhao Weipeng, Dong Lili, Pan Cuizhen, Shu Xianhong,
Abstract
Background:
Diastolic mitral regurgitation (DMR) is a type of functional mitral regurgitation. Its occurrence in the diastolic phase of cardiac cycle renders DMR an easily ignored entity. Confusing it with systolic mitral regurgitation occasionally happens. The reversal of left atrioventricular pressure gradient during diastole and the incomplete closure of mitral valve are the essential conditions for DMR. Diastolic mitral regurgitation develops under various situations, where the mechanisms of diastolic reversal of left atrioventricular pressure gradient differ.
Case summary:
Patient 1 was a 50-year-old man diagnosed with 2:1?second-degree atrioventricular block (AVB). Patient 2 was a 70-year-old man diagnosed with first-degree AVB. Patient 3 was a 66-year-old man diagnosed with atrial fibrillation with long intermission and occasional atrial flutter with unequal conduction. Patient 4 was a 54-year-old woman diagnosed with dilated cardiomyopathy with complete left bundle branch block. Patient 5 was a 36-year-old man diagnosed with severe acute aortic regurgitation secondary to subacute bacterial endocarditis.
Discussion:
Although the degree of DMR is relatively mild, its appearance generally prompts further clinical considerations. The appreciation of DMR has an incremental value for diagnosing and evaluating the underlying cardiovascular disease.
© The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology.
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Peripartum cardiomyopathy and HELLP syndrome in a previously healthy multiparous woman: A case report.
SAGE Open Med Case Rep2020 ;8():2050313X20979288. doi: 10.1177/2050313X20979288.
Quevedo Shany, Bekele Caroline, Thompson Patrice D, Philkhana Megan, Virani Sana, Consuegra Andrea, Douglass Paul, Gertz Alida M,
Abstract
Peripartum cardiomyopathy is a type of dilated cardiomyopathy in which the exact etiology is uncertain. HELLP syndrome is characterized by a constellation of different clinical and laboratory findings, including hemolysis, elevated liver enzymes, and low platelets. Few case reports exist detailing successful diagnosis and management of postpartum HELLP syndrome, peripartum cardiomyopathy, and multisystem organ failure in a previously healthy woman. We herein report the case of a 39-year-old multiparous female with mild gestational hypertension, who presented in the third trimester with vaginal bleeding and was subsequently suspected to have intrapartum placental abruption leading to immediate Cesarean section, complicated by massive postpartum hemorrhage, necessitating care in the intensive care unit. HELLP syndrome, disseminated intravascular coagulation, and acute kidney injury requiring hemodialysis subsequently developed along with respiratory failure and peripartum cardiomyopathy. After diagnosis and proper management, the patient made a full recovery. Peripartum cardiomyopathy should remain on the differential for women with heart failure symptoms.
© The Author(s) 2020.
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The results of a single-center experience with HeartMate 3 in a biventricular configuration.
J Heart Lung Transplant2020 Dec;():. doi: S1053-2498(20)31881-7.
McGiffin David, Kure Christina, McLean Janelle, Marasco Silvana, Bergin Peter, Hare James L, Leet Angeline, Patel Hitesh, Zimmet Adam, Rix Julia, Taylor Andrew, Kaye David,
Abstract
BACKGROUND:
Right ventricular (RV) failure after left ventricular assist device (VAD) implantation is a difficult problem. One solution is the implantation of continuous-flow VADs in a biventricular configuration. Disappointing survival and a concerning incidence of right-sided pump thrombosis have been previously reported.
METHODS:
From May 2017 to April 2020, a total of 12 patients underwent implantation of HeartMate 3 (HM3) biventricular VADs (BiVADs) as a bridge to cardiac transplantation. The right-sided pump was implanted in the right atrium in all cases. Adverse events and patient outcomes were determined.
RESULTS:
Patients were male, and the mean age was 44 years. The etiology was dilated cardiomyopathy (6 patients), sarcoid heart disease (2 patients), ischemic cardiomyopathy (1 patient), anthracycline cardiomyopathy (1 patient), non-compaction cardiomyopathy (1 patient), and arrhythmogenic RV cardiomyopathy with biventricular involvement (1 patient). There was 1 death from multisystem failure. There were 3 episodes of right VAD thrombus (thrombosis or clot ingestion); 1 managed medically, 1 recognized intraoperatively treated with clot retrieval, and 1 requiring pump exchange. There were 3 driveline infections. At 18 months after the procedure, 5 patients (41.7%) had undergone cardiac transplantation, 5 patients (41.7%) were alive and on biventricular support, 1 patient had died (8.3%), and 1 patient had VAD explantation for myocardial recovery (8.3%). Actuarial survival at 18 months was 91.7%.
CONCLUSIONS:
In this small study, HM3 BiVAD in these critically ill patients was used with low mortality. This suggests that the timely deployment of biventricular support with HM3 can be associated with favorable outcomes.
Copyright © 2020 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.
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Mandibuloacral Dysplasia Type A in Five Tunisian Patients.
Eur J Med Genet2021 Jan;():104138. doi: S1769-7212(21)00004-5.
R Sakka, H Marmouch, M Trabelsi, A Achour, M Golli, I Hannachi, E Kerkeni, K Monastiri, F Maazoul, R M'rad,
Abstract
Mandibuloacral dysplasia with type A lipodystrophy is a rare autosomal recessive disorder characterized by craniofacial dysmorphism, type A lipodystrophy, clavicular dysplasia, and acroostelolysis. It is caused by homozygous or compound heterozygous missense mutations in LMNA gene. We report five Tunisian patients harboring the same homozygous c.1580G > A; p. (Arg527His) mutation in LMNA gene. The patients presented with typical features of mandibuloacral dysplasia including, prominent eyes, thin or beaked nose, dental overcrowding, mandibular hypoplasia, short and broad finger's distal phalanges with round tips and lipodystrophy type A. Newly recognized signs are growth hormone deficiency and dilated cardiomyopathy. Genotype-phenotype correlation found that at least one of the disease's LMNA mutant alleles involve one of the highly conserved aminoacids, residing in a key site domain for protein function within the C-terminal globular domain of A-type lamins. Also, the severity of the disease depends on the position in the protein's domain and on the type of substitution of the concerned aminoacid.
Copyright © 2021 Elsevier Masson SAS. All rights reserved.
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Current use of cardiac magnetic resonance in tertiary referral centres for the diagnosis of cardiomyopathy: the ESC EORP Cardiomyopathy/Myocarditis Registry.
Eur Heart J Cardiovasc Imaging2021 Jan;():. doi: jeaa329.
Mizia-Stec Katarzyna, Charron Philippe, Gimeno Blanes Juan Ramon, Elliott Perry, Kaski Juan Pablo, Maggioni Aldo P, Tavazzi Luigi, Tendera Micha?, Felix Stephan B, Dominguez Fernando, Ojrzynska Natalia, Losi Maria-Angela, Limongelli Giuseppe, Barriales-Villa Roberto, Seferovic Petar M, Biagini Elena, Wybraniec Maciej, Laroche Cecile, Caforio Alida L P, ,
Abstract
AIMS :
Cardiac magnetic resonance (CMR) is recommended in the diagnosis of cardiomyopathies, but it is time-consuming, expensive, and limited in availability in some European regions. The aim of this study was to determine the use of CMR in cardiomyopathy patients enrolled into the European Society of Cardiology (ESC) cardiomyopathy registry [part of the EURObservational Research Programme (EORP)].
METHODS AND RESULTS :
Three thousand, two hundred, and eight consecutive adult patients (34.6% female; median age: 53.0?±?15?years) with cardiomyopathy were studied: 1260 with dilated (DCM), 1739 with hypertrophic (HCM), 66 with restrictive (RCM), and 143 with arrhythmogenic right ventricular cardiomyopathy (ARVC). CMR scans were performed at baseline in only 29.4% of patients. CMR utilization was variable according to cardiomyopathy subtypes: from 51.1% in ARVC to 36.4% in RCM, 33.8% in HCM, and 20.6% in DCM (P?0.001). CMR use in tertiary referral centres located in different European countries varied from 1% to 63.2%. Patients undergoing CMR were younger, less symptomatic, less frequently had implantable cardioverter-defibrillator (ICD)/pacemaker implanted, had fewer cardiovascular risk factors and comorbidities (P?0.001). In 28.6% of patients, CMR was used along with transthoracic echocardiography (TTE); 67.6% patients underwent TTE alone, and 0.9% only CMR.
CONCLUSION :
Less than one-third of patients enrolled in the registry underwent CMR and the use varied greatly between cardiomyopathy subtypes, clinical profiles of patients, and European tertiary referral centres. This gap with current guidelines needs to be considered carefully by scientific societies to promote wider availability and use of CMR in patients with cardiomyopathies.
© The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology.
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