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Pubblicazioni recenti - cardiac sarcoidosis
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Unusual case of cerebral embolism after initiation of selexipag for sarcoidosis-related pulmonary hypertension: a case report.
Radiol Case Rep2022 Sep;17(9):3009-3014. doi: 10.1016/j.radcr.2022.05.064.
Inoue Naoya, Morikawa Shuji,
Abstract
: Pulmonary hypertension is a rare complication of sarcoidosis. The pathogenesis of sarcoidosis-related pulmonary hypertension is multifactorial, and patients with sarcoidosis-related pulmonary hypertension can have variable treatment responses and prognoses. While selexipag (Nippon Shinyaku / Kyoto / Japan) was recently approved in Japan for the treatment of pulmonary hypertension, the risk of cerebral infarction has not been clearly reported. : A 63-year-old Asian female with a diagnosis of ocular and cutaneous sarcoidosis developed shortness of breath and was referred to our department to rule out cardiac sarcoidosis. Swan-Ganz catheterization was performed, and she was diagnosed with pulmonary arterial hypertension and started on selexipag. A few days after starting treatment, she presented with hemiplegia and was diagnosed with cardiogenic cerebral embolism by using magnetic resonance imaging. As there was no evidence of pre-existing intracardiac thrombosis, we suspected unusual cerebral embolism. Echocardiography revealed a deep venous thrombus and a bubble study revealed a right-left shunt through a patent foramen ovale. : The initiation of selexipag improved pulmonary blood flow and caused cerebral embolism, which was an unusual and unexpected event. This report highlights the importance of confirming the presence of patent foramen ovale and a deep venous thrombus before starting treatment for pulmonary hypertension.
© 2022 The Authors. Published by Elsevier Inc. on behalf of University of Washington.
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Characterization of the PF-ILD phenotype in patients with advanced pulmonary sarcoidosis.
Respir Res2022 Jun;23(1):169. doi: 10.1186/s12931-022-02094-7.
Schimmelpennink M C, Meek D B, Vorselaars A D M, Langezaal L C M, van Moorsel C H M, van der Vis J J, Veltkamp M, Grutters J C,
Abstract
BACKGROUND:
Advanced pulmonary sarcoidosis causes significant morbidity and can lead to death. Large trials demonstrated efficacy of antifibrotics in patients with progressive fibrosing interstitial lung diseases (PF-ILD), including a few with sarcoidosis. To date, little is known about this progressive fibrosing phenotype in sarcoidosis. Diffusion capacity of carbon monoxide (DLCO) may be a useful functional marker to screen for advanced pulmonary sarcoidosis. In this study, we describe a cohort with advanced pulmonary sarcoidosis and we gain insights in the progressive fibrosing phenotype in sarcoidosis.
METHODS:
Patients with sarcoidosis and a DLCO?50% predicted were included in this retrospective cohort study. First measurement of DLCO?50% predicted was the baseline. Lung function data, HRCT, pulmonary hypertension (PH) and mortality were collected. Patients with?>?10% fibrosis on HRCT meeting the criteria for ILD-progression within 24 months were labelled as PF-ILD. With Cox-regression analysis predictors of mortality were established.
RESULTS:
106 patients with a DLCO?50% predicted were included. Evolution of forced vital capacity (FVC) varied widely between patients from - 34% to?+?45% after 2 years follow-up, whereas change in DLCO varied between - 11% and?+?26%. Fourteen patients (15%) met the PF-ILD criteria, of whom 6 (43%) died within 10 years versus 10 (13%) in the non PF-ILD group (p?=?0.006). PH was present 12 (11%), 56 (53%) demonstrated?>?10% fibrosis on HRCT. Independent predictors of mortality and lung transplantation in the whole cohort are PH, PF-ILD and UIP-like pattern.
CONCLUSION:
In conclusion, within this group with advanced pulmonary sarcoidosis disease course varied widely from great functional improvement to death. PF-ILD patients had higher mortality rate than the mortality in the overall pulmonary sarcoidosis group. Future research should focus on the addition of antifibrotics in these patients. Trial registration retrospectively registered.
© 2022. The Author(s).
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Integration and application of clinical practice guidelines for the diagnosis of idiopathic pulmonary fibrosis and fibrotic hypersensitivity pneumonitis.
Chest2022 Jun;():. doi: S0012-3692(22)01102-3.
Marinescu Daniel-Costin, Raghu Ganesh, Remy-Jardin Martine, Travis William D, Adegunsoye Ayodeji, Beasley Mary Beth, Chung Jonathan H, Churg Andrew, Cottin Vincent, Egashira Ryoko, Fernández Pérez Evans R, Inoue Yoshikazu, Johannson Kerri A, Kazerooni Ella A, Khor Yet H, Lynch David A, Müller Nestor L, Myers Jeffrey L, Nicholson Andrew G, Rajan Sujeet, Saito-Koyama Ryoko, Troy Lauren, Walsh Simon L F, Wells Athol U, Wijsenbeek Marlies S, Wright Joanne L, Ryerson Christopher J,
Abstract
Recent clinical practice guidelines have addressed the diagnosis of idiopathic pulmonary fibrosis (IPF) and fibrotic hypersensitivity pneumonitis (fHP). These disease-specific guidelines were developed independently, without clear direction on how to concurrently apply their respective recommendations within a single patient, where discrimination between these two fibrotic interstitial lung diseases represents a frequent diagnostic challenge. The objective of this document, created by an international group of experts, was to suggest a pragmatic approach on how to apply existing guidelines to distinguish IPF and fHP. Key clinical, radiological, and pathological features described in previous guidelines are integrated in a set of diagnostic algorithms, which are then placed in the broader context of multidisciplinary discussion to guide the generation of a consensus diagnosis. While these algorithms necessarily reflect some uncertainty wherever strong evidence is lacking, they provide insight into the current approach favored by experts in the field based on currently available knowledge. The authors further identify priorities for future research to clarify ongoing uncertainties in the diagnosis of fibrotic interstitial lung diseases.
Copyright © 2022. Published by Elsevier Inc.
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What cardiologists should know about cardiac sarcoidosis in 2022?
Curr Opin Cardiol2022 Jul;37(4):380-387. doi: 10.1097/HCO.0000000000000970.
Lemay Sylvain, Marchand Laurie, Sénéchal Mario,
Abstract
PURPOSE OF REVIEW:
Cardiac sarcoidosis (CS) is a potentially fatal condition when unrecognized or not treated adequately. The purpose of this review is to provide new strategies to increase clinical recognition of CS and to present an updated overview of the immunosuppressive treatments using most recent data published in the last 18?months.
RECENT FINDINGS:
CS is an increasingly recognized pathology, and its diagnostic is made 20 times more often in the last two decades. Recent studies have shown that imaging alone usually lacks specificity to distinguish CS from other inflammatory cardiomyopathies. However, imaging can be used to increase significantly diagnostic yield of extracardiac and cardiac biopsy. Recent reviews have also demonstrated that nearly 25% of patients will be refractory to standard treatment with prednisone and that combined treatment with a corticosteroid-sparing agent is often necessary for a period that remains undetermined.
SUMMARY:
CS is a complex pathology that should always require a biopsy attempt to have a histological proven diagnosis before starting immunosuppressive therapy consisting of corticosteroids with or without a corticosteroid-sparing agent.
Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.
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Arrhythmia Monitoring and Outcomes in Patients With Cardiac Sarcoidosis: Insights From the Cardiac Sarcoidosis Consortium.
J Am Heart Assoc2022 Jun;():e024924. doi: 10.1161/JAHA.121.024924.
Bressi Edoardo, Crawford Thomas C, Bogun Frank M, Gu Xiaokui, Ellenbogen Kenneth A, Chicos Alexandra B, Roukoz Henri, Zimetbaum Peter J, Kalbfleisch Steven J, Murgatroyd Francis D, Steckman David A, Rosenfeld Lynda E, Garlitski Ann C, Soejima Kyoko, Bhan Adarsh K, Vedantham Vasanth, Dickfeld Timm M, De Lurgio David B, Platonov Pyotr G, Zipse Matthew M, Nishiuchi Suguru, Ortman Matthew L, Narasimhan Calambur, Patton Kris K, Rosenthal David G, Mukerji Siddharth S, Hoogendoorn Jarieke C, Zeppenfeld Katja, Sauer William H, Kron Jordana, ,
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Effectiveness of methotrexate as a second-line treatment for cardiac sarcoidosis assessed via F-FDG PET: a case report.
Eur Heart J Case Rep2022 Jun;6(6):ytac226. doi: 10.1093/ehjcr/ytac226.
Dotare Taishi, Maeda Daichi, Matsue Yuya, Minamino Tohru,
Abstract
Background:
Although methotrexate has been widely used as a second-line therapy for cardiac sarcoidosis (CS), it is not clear if it has a direct anti-inflammatory effect.
Case summary:
A 65-year-old man presented to our hospital with symptomatic ventricular tachycardia (VT). After cardioversion, electrocardiography showed a first-degree atrioventricular block with a right bundle branch block, and transthoracic echocardiography revealed left ventricular dilatation. After extensive investigations, including fluorine-18 fluorodeoxyglucose positron emission tomography (F-FDG PET), the patient was diagnosed with CS according to the Japanese Circulation Society guidelines. After the implantation of a transvenous implantable cardioverter defibrillator, corticosteroid therapy was introduced at a starting dose of 30?mg/day. After corticosteroid therapy was tapered to a maintenance dose of 10?mg/day, he had an uneventful clinical course without symptoms for the 1st year after hospital discharge. However, symptomatic VT recurred and F-FDG PET showed abnormal patterns of cardiac FDG uptake. Although he was treated with corticosteroid therapy once more, which was gradually up-titrated to a dose of 20?mg/day over a 1-month period, myocardial uptake of F-FDG PET remained unchanged. As the patient was considered steroid refractory, second-line treatment with 6?mg/week of methotrexate was introduced, whereas maintaining the dose of corticosteroid therapy at 20?mg/day. After 1 month, F-FDG PET showed remarkable reduction in FDG uptake, and the patient had a good clinical course without further episodes of arrhythmia or other symptoms during an 8-month follow-up.
Discussion:
Methotrexate may have a direct anti-inflammatory effect in patients with CS refractory to regular corticosteroid therapy.
© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology.
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Accelerated cardiac T1 mapping with recurrent networks and cyclic, model-based loss.
Med Phys2022 Jun;():. doi: 10.1002/mp.15801.
Le Johnathan V, Mendes Jason K, McKibben Nicholas, Wilson Brent D, Ibrahim Mark, DiBella Edward V R, Adluru Ganesh,
Abstract
BACKGROUND:
Using the spin-lattice relaxation time (T1) as a biomarker, the myocardium can be quantitatively characterized using cardiac T1 mapping. The modified Look-Locker inversion (MOLLI) recovery sequences have become the standard clinical method for cardiac T1 mapping. However, the MOLLI sequences require an 11-heartbeat breath-hold that can be difficult for subjects, particularly during exercise or pharmacologically induced stress. Although shorter cardiac T1 mapping sequences have been proposed, these methods suffer from reduced precision. As such, there is an unmet need for accelerated cardiac T1 mapping.
PURPOSE:
To accelerate cardiac T1 mapping MOLLI sequences by using neural networks to estimate T1 maps using a reduced number of T1-weighted images and their corresponding inversion times.
MATERIALS AND METHODS:
In this retrospective study, 911 pre-contrast T1 mapping datasets from 202 subjects (128 males, 56 ± 15 years; 74 females, 54 ± 17 years) and 574 T1 mapping post-contrast datasets from 193 subjects (122 males, 57 ± 15 years; 71 females, 54 ± 17 years) were acquired using the MOLLI-5(3)3 sequence and the MOLLI-4(1)3(1)2 sequence, respectively. All acquisition protocols used similar scan parameters: , , and , gadoteridol (ProHance, Bracco Diagnostics) dose . A bidirectional multilayered long short-term memory (LSTM) network with fully connected output and cyclic model-based loss was used to estimate T1 maps from the first three T1-weighted images and their corresponding inversion times for pre- and post-contrast T1 mapping. The performance of the proposed architecture was compared to the three-parameter T1 recovery model using the same reduction of the number of T1-weighted images and inversion times. Reference T1 maps were generated from the scanner using the full MOLLI sequences and the three-parameter T1 recovery model. Correlation and Bland-Altman plots were used to evaluate network performance in which each point represents averaged regions of interest in the myocardium corresponding to the standard American Heart Association 16-segment model. The precision of the network was examined using consecutively repeated scans. Stress and rest pre-contrast MOLLI studies as well as various disease test cases, including amyloidosis, hypertrophic cardiomyopathy, and sarcoidosis were also examined. Paired t-tests were used to determine statistical significance with .
RESULTS:
Our proposed network demonstrated similar T1 estimations to the standard MOLLI sequences (pre-contrast: vs. with ; post-contrast: vs. with ). The precision of standard MOLLI sequences was well preserved with the proposed network architecture ( vs. ). Network-generated T1 reactivities are similar to stress and rest pre-contrast MOLLI studies ( vs. with ). Amyloidosis T1 maps generated using the proposed network are also similar to the reference T1 maps (pre-contrast: vs. with ; post-contrast: vs. with ).
CONCLUSIONS:
A bidirectional multilayered LSTM network with fully connected output and cyclic model-based loss was used to generate high-quality pre- and post-contrast T1 maps using the first three T1-weighted images and their corresponding inversion times. This work demonstrates that combining deep learning with cardiac T1 mapping can potentially accelerate standard MOLLI sequences from 11 to 3 heartbeats.
© 2022 American Association of Physicists in Medicine.
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Cardiac Sarcoidosis Diagnostic Challenges and Management: A Case Report and Literature Review.
Cureus2022 May;14(5):e24850. doi: 10.7759/cureus.24850.
Jaiswal Richa, Vaisyambath Laseena, Khayyat Azadeh, Unachukwu Nkechinyere, Nasyrlaeva Bibimariyam, Asad Muhammad, Fabara Stephanie P, Balan Irina, Kolla Sree, Rabbani Rizwan,
Abstract
Sarcoidosis can be presented as cardiac sarcoidosis (CS), which is challenging to diagnose due to its clinical silence. Ventricular arrhythmias and atrioventricular blocks can be fatal and cause sudden death in patients with cardiac sarcoidosis. Five percent of sarcoidosis patients have clinically evident cardiac sarcoidosis. However, autopsy reports and imaging studies have shown a higher prevalence of cardiac involvement. Early recognition is important to prevent such detrimental consequences. Cardiac sarcoidosis is increasingly being diagnosed owing to increased awareness among physicians and new diagnostic tools like MRI and positron emission tomography (PET) scan replacing traditional endomyocardial biopsy. A definitive diagnosis of CS remains challenging due to the non-specific clinical findings that can present similar symptoms of common cardiac disease; therefore, the imaging and biopsies are substantial for diagnosis confirmation. Pharmacological and Implantable devices are two main therapeutic approaches in cardiac sarcoidosis, in which steroids and pacemaker therapy have shown better outcomes. This review summarizes the available data related to the prevalence, prognosis, diagnosis, and management of cardiac sarcoidosis.
Copyright © 2022, Jaiswal et al.
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Effect of Corticosteroids on Left Ventricular Function in Patients With Cardiac Sarcoidosis.
Am J Cardiol2022 Jun;():. doi: S0002-9149(22)00530-6.
Wand Alison L, Pavlovic Noelle, Duvall Chloe, Rosen Natalie S, Chasler Jessica, Griffin Jan M, Okada David R, Jefferson Artrish, Chrispin Jonathan, Tandri Harikrishna, Mathai Stephen C, Sharp Michelle, Chen Edward S, Kasper Edward K, Hays Allison G, Gilotra Nisha A,
Abstract
Cardiac sarcoidosis (CS) is an important cause of cardiomyopathy. The trajectory of left ventricular ejection fraction (LVEF) in patients with CS undergoing treatment remains unclear. Patients with CS who were treated with corticosteroids and who underwent transthoracic echocardiography were studied. Baseline characteristics, treatment, echocardiographic data (including baseline to follow-up change in LVEF), and outcomes were retrospectively evaluated. Among 100 patients, 55 had baseline reduced LVEF (
Copyright © 2022 Elsevier Inc. All rights reserved.
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Catastrophic Events of Cardiac Sarcoidosis: A Case Report.
Cureus2022 May;14(5):e24902. doi: 10.7759/cureus.24902.
Riasat Maria, Khan Arshan, Ehtesham Moiz, Meghrajani Vineet, Hafez Anthony,
Abstract
Cardiac sarcoidosis (CS) can be silent in most patients with extrapulmonary sarcoidosis. Atrioventricular (AV) block is the most common clinical presentation, but it can also present as fatal ventricular arrhythmias and sudden cardiac death. Endomyocardial biopsy is the gold standard; however, it is not sensitive since CS can involve the myocardium in a patchy distribution. Our case depicts a female who presented with syncope; however, her hospital course was complicated by multiple cardiac arrests. Her initial laboratory tests, including an autoimmune workup, were unremarkable. Cardiac magnetic resonance and fluorodeoxyglucose (FDG) positron emission tomography (PET) imaging revealed intramyocardial delayed enhancement of the basal anteroseptal (non-ischemic distribution) and patchy foci of increased uptake in the anteroseptal and inferior myocardial region, respectively. The patient was started on intravenous methylprednisolone, and her condition slowly improved. Post-discharge, the patient followed in the outpatient clinic with a repeat FDG-PET scan revealing resolution of myocardial FDG uptake. She also underwent bronchoscopy with lymph node biopsy showing granulomas and endobronchial biopsy confirming pulmonary sarcoidosis.
Copyright © 2022, Riasat et al.
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Bidirectional Ventricular Tachycardia: Challenges and Solutions.
Vasc Health Risk Manag2022 ;18():397-406. doi: 10.2147/VHRM.S274857.
Almarzuqi Ahmed, Kimber Shane, Quadros Kenneth, Senaratne Janek,
Abstract
Bidirectional ventricular tachycardia (BiVT) is a rare form of ventricular tachycardia that manifests on surface electrocardiogram by dual QRS morphologies alternating on a beat-to-beat basis. It was first reported in the 1920s as a complication of digoxin, and since then, it has been reported in other conditions including fulminant myocarditis, sarcoidosis, catecholaminergic polymorphic ventricular tachycardia, and Andersen-Tawil syndrome. The mechanism for BiVT is not as well known as other forms of ventricular tachycardia but appears to include typical mechanisms including triggered activity from afterdepolarizations, abnormal automaticity, or reentry. This review will go beyond the definition, surface electrocardiogram, mechanisms, causes, and treatment of BiVT as per our current understanding.
© 2022 Almarzuqi et al.
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Role of serum soluble interleukin 2-receptor in the evaluation of myocardial inflammation in patients with cardiac sarcoidosis and ventricular arrhythmias.
J Interv Card Electrophysiol2022 Jun;():. doi: 10.1007/s10840-022-01271-4.
Reithmann Christopher, Min Suhui, Kling Theresia, Herkommer Bernhard, Ulbrich Michael,
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Isolated cardiac sarcoidosis associated with coronary vasomotion abnormalities: a case report.
Eur Heart J Case Rep2022 Feb;6(2):ytac083. doi: 10.1093/ehjcr/ytac083.
Godo Shigeo, Hasebe Yuhi, Takahashi Jun, Shimokawa Hiroaki, Yasuda Satoshi,
Abstract
Background :
Cardiac sarcoidosis is a chronic, inflammatory disease that can affect the heart and often results in heart failure and lethal arrhythmias. A multimodality imaging approach without endomyocardial biopsy allows for the diagnosis of isolated cardiac sarcoidosis. Coronary vasomotion abnormalities are highly prevalent in various cardiovascular and inflammatory diseases. It remains unknown whether active myocardial inflammation due to cardiac sarcoidosis is associated with coronary vasomotion abnormalities.
Case summary :
A 68-year-old man without a past medical history experienced an out-of-hospital cardiac arrest due to ventricular fibrillation and was successfully resuscitated without neurologic sequelae. Coronary angiography showed normal coronary arteries; however, intracoronary acetylcholine provocation testing demonstrated both epicardial coronary and coronary microvascular spasm. He was diagnosed with isolated cardiac sarcoidosis by fulfilling the diagnostic criteria proposed by the Japanese Circulation Society 2016 diagnostic guidelines, including fatal ventricular arrhythmia, focal left ventricular wall asynergy, increased myocardial fluorodeoxyglucose uptake by positron emission tomography, and late gadolinium enhancement by cardiac magnetic resonance in the heart. He was treated with calcium-channel blocker for coronary artery spasm and prednisolone for cardiac sarcoidosis and underwent implantation of an implantable cardioverter-defibrillator for secondary prevention. Following the treatment, the severity of coronary artery spasm was reduced along with regression of the myocardial inflammation.
Discussion :
Epicardial coronary artery and coronary microvascular spasm can be accompanied by active myocardial inflammation of isolated cardiac sarcoidosis, and the treatment with corticosteroid and calcium-channel blocker may be effective for relieving the severity of coronary artery spasm in association with regression of myocardial inflammation of the disease.
© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology.
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Cardiac Magnetic Resonance Imaging-Based Screening for Cardiac Sarcoidosis in Patients With Atrioventricular Block Requiring Temporary Pacing.
J Am Heart Assoc2022 Jun;11(11):e024257. doi: 10.1161/JAHA.121.024257.
Vuorinen Aino-Maija, Lehtonen Jukka, Pakarinen Sami, Holmström Miia, Kivistö Sari, Kaasalainen Touko,
Abstract
Background Some myocardial diseases, such as cardiac sarcoidosis, predispose to complete atrioventricular block. The European Society of Cardiology Guidelines on cardiac pacing in 2021 recommend myocardial disease screening in patients with conduction disorder requiring pacemaker with multimodality imaging, including cardiac magnetic resonance (CMR) imaging. The ability of CMR imaging to detect myocardial disease in patients with a temporary pacing wire is not well documented. Methods and Results Our myocardial disease screening protocol is based on using an active fixation pacing lead connected to a reusable extracorporeal pacing generator (temporary permanent pacemaker) as a bridge to a permanent pacemaker. From 2011 to 2019, we identified 17 patients from our CMR database who underwent CMR imaging with a temporary permanent pacemaker for atrioventricular block. We analyzed their clinical presentations, CMR data, and pacemaker therapy. All CMRs were performed without adverse events. Pacing leads induced minor artifacts to the septal myocardial segments. The extent of late gadolinium enhancement in CMR imaging was used to screen patients for the presence of myocardial disease. Patients with evidence of late gadolinium enhancement underwent endomyocardial biopsy. If considered clinically indicated, also 18-F-fluorodeoxyglucose positron emission tomography and extracardiac tissue biopsy were performed if sarcoidosis was suspected. Eventually, 8 of 17 patients (47.1%) were diagnosed with histologically confirmed granulomatous inflammatory cardiac disease. Importantly, only 1 had a previously diagnosed extracardiac sarcoidosis at the time of presentation with high-degree atrioventricular block. Conclusions CMR imaging with temporary permanent pacemaker protocol is an effective and safe early screening tool for myocardial disease in patients presenting with atrioventricular block requiring immediate, continuous pacing for bradycardia.
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Not All That Glitters Is Sarcoidosis: Septal Perforator Myocardial Infarction Mimicking Isolated Cardiac Sarcoidosis.
Circ Cardiovasc Imaging2022 Jun;15(6):e014050. doi: 10.1161/CIRCIMAGING.122.014050.
Vidula Mahesh K, O'Quinn Rupal P, Sanghavi Monika, Litt Harold, Han Yuchi, Bravo Paco E,
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The discrepancies between clinical and histopathological diagnoses of cardiomyopathies in patients with stage D heart failure undergoing heart transplantation.
PLoS One2022 ;17(6):e0269019. doi: 10.1371/journal.pone.0269019.
Lertsuttimetta Thana, Tumkosit Monravee, Kaveevorayan Peerapat, Chantranuwatana Poonchavist, Theerasuwipakorn Nonthikorn, Chattranukulchai Pairoj, Puwanant Sarinya,
Abstract
BACKGROUND:
This study aimed to determine the etiology of stage-D heart failure (HF) and the prevalence and prognosis of misdiagnosed cardiomyopathy in patients undergoing heart transplantation.
METHODS AND RESULTS:
We retrospectively reviewed 127 consecutive patients (mean age, 42 years; 90 [71%], male) from February 1994 to September 2021 admitted for heart transplant in our tertiary center. Pre-transplant clinical diagnosis was compared with post-transplant pathological diagnosis. The most common misdiagnosed cardiomyopathy was nonischemic cardiomyopathy accounting for 6% (n = 8) of all patients. Histopathological examination of explanted hearts in misdiagnosed patients revealed 2 arrhythmogenic cardiomyopathy, 2 sarcoidosis, 1 hypertrophic cardiomyopathy, 1 hypersensitivity myocarditis, 1 noncompacted cardiomyopathy, and 1 ischemic cardiomyopathy. Pre-transplant cardiac MRI and endomyocardial biopsy (EMB) were performed in 33 (26%) and 6 (5%) patients, respectively, with both performed in 3 (3% of patients). None of the patients undergoing both cardiac tests were misdiagnosed. During the 5-years follow-up period, 2 (25%) and 44 (37%) patients with and without pretransplant misdiagnosed cardiomyopathy died. There was no difference in survival rate between the groups (hazard ratio: 0.52; 95% CI:0.11-2.93; P = 0.314).
CONCLUSIONS:
The prevalence of misdiagnosed cardiomyopathy was 6% of patients with stage-D HF undergoing heart transplantation, the misdiagnosis mostly occurred in nonischemic/dilated cardiomyopathy. An accurate diagnosis of newly detected cardiomyopathy gives an opportunity for potentially reversing cardiomyopathy, including sarcoidosis or myocarditis. This strategy may minimize the need for advanced HF therapy or heart transplantation. With advances in cardiac imaging, improvements in diagnostic accuracy of the etiology of HF can improve targeting of treatment.
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Safety and tolerability of pirfenidone in asbestosis: a prospective multicenter study.
Respir Res2022 May;23(1):139. doi: 10.1186/s12931-022-02061-2.
Miedema Jelle R, Moor Catharina C, Veltkamp Marcel, Baart Sara, Lie Natascha S L, Grutters Jan C, Wijsenbeek Marlies S, Mostard Rémy L M,
Abstract
BACKGROUND:
Pirfenidone slows down disease progression in idiopathic pulmonary fibrosis (IPF). Recent studies suggest a treatment effect in progressive pulmonary fibrosis other than IPF. However, the safety and effectiveness of pirfenidone in asbestosis patients remain unclear. In this study, we aimed to investigate the safety, tolerability and efficacy of pirfenidone in asbestosis patients with a progressive phenotype.
METHODS:
This was a multicenter prospective study in asbestosis patients with progressive lung function decline. After a 12-week observational period, patients were treated with pirfenidone 801 mg three times a day. Symptoms and adverse events were evaluated weekly and patients completed online patient-reported outcomes measures. At baseline, start of therapy, 12 and 24 weeks, in hospital measurement of lung function and a 6 min walking test were performed. Additionally, patients performed daily home spirometry measurements.
RESULTS:
In total, 10 patients were included of whom 6 patients (66.7%) experienced any adverse events during the study period. Most frequently reported adverse events were fatigue, rash, anorexia and cough, which mostly occurred intermittently and were reported as not very bothersome. No significant changes in hospital pulmonary function (forced vital capacity (FVC), diffusion capacity of the lung for carbon monoxide (DLCO), 6 min walking test or patient-reported outcomes measures before and after start of pirfenidone were found. Home spirometry demonstrated a FVC decline in 12 weeks before start of pirfenidone, while FVC did not decline during the 24 week treatment phase, but this difference was not statistically significant.
CONCLUSIONS:
Treatment with pirfenidone in asbestosis has an acceptable safety and tolerability profile and home spirometry data suggest this antifibrotic treatment might attenuate FVC decline in progressive asbestosis. Trial registration MEC-2018-1392; EudraCT number: 2018-001781-41.
© 2022. The Author(s).
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Impact of lung function and baseline clinical characteristics on patient-reported outcome measures in systemic sclerosis-associated interstitial lung disease.
Rheumatology (Oxford)2022 May;():. doi: keac325.
Kreuter Michael, Hoffmann-Vold Anna-Maria, Matucci-Cerinic Marco, Saketkoo Lesley Ann, Highland Kristin B, Wilson Hilary, Alves Margarida, Erhardt Elvira, Schoof Nils, Maher Toby M,
Abstract
OBJECTIVE:
The SENSCIS® trial demonstrated a significant reduction of lung function decline in patients with systemic sclerosis (SSc)-associated interstitial lung disease (SSc-ILD) treated with nintedanib, but no significant effect on health-related quality of life (HRQoL). To assess whether SSc/SSc-ILD severity and large changes in lung function correlate with HRQoL, a post-hoc analysis of SENSCIS®, aggregating treatment arms, was undertaken.
METHODS:
Patient-reported outcome (PRO) measures (St. George's Respiratory Questionnaire [SGRQ], Functional Assessment of Chronic Illness Therapy [FACIT]-Dyspnoea, and Health Assessment Questionnaire-Disability Index [HAQ-DI], incorporating the Scleroderma Health Assessment Questionnaire visual analogue scale [SHAQ VAS]) at baseline and week 52 were assessed for associations to SSc-ILD severity.
RESULTS:
At baseline and at week 52, forced vital capacity (FVC) 30% fibrosis on high-resolution computed tomography at baseline demonstrated worse PRO measure scores at week 52. After 1?year, patients with a major (>10%) improvement/worsening in FVC demonstrated corresponding improvement/worsening in SGRQ and other PRO measures, significant for the SGRQ symptom domain (p
CONCLUSION:
Severe SSc-ILD and major deteriorations in lung function have important impacts on HRQoL. Treatments that slow lung function decline and prevent severe SSc-ILD are important to preserve HRQoL.
TRIAL REGISTRATION:
clinicaltrials.gov, www.clinicaltrials.gov, NCT02597933.
© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology.
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ERS International Congress 2021: highlights from the Interstitial Lung Diseases Assembly.
ERJ Open Res2022 Apr;8(2):. doi: 00640-2021.
Guler Sabina A, Cuevas-Ocaña Sara, Nasser Mouhamad, Wuyts Wim A, Wijsenbeek Marlies S, Froidure Antoine, Bargagli Elena, Renzoni Elisabetta A, Veltkamp Marcel, Spagnolo Paolo, Nunes Hilario, McCarthy Cormac, Molina-Molina Maria, Bonella Francesco, Poletti Venerino, Kreuter Michael, Antoniou Katerina M, Moor Catharina C,
Abstract
This article provides an overview of scientific highlights in the field of interstitial lung disease (ILD), presented at the virtual European Respiratory Society Congress 2021. A broad range of topics was discussed this year, ranging from translational and genetic aspects to novel innovations with the potential to improve the patient pathway. Early Career Members summarise a selection of interesting findings from different congress sessions, together with the leadership of Assembly 12 - Interstitial Lung Disease.
Copyright ©The authors 2022.
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Systemic sarcoidosis with subcutaneous, muscular, cardiac, and lymph node involvement.
Clin Rheumatol2022 May;():. doi: 10.1007/s10067-022-06222-y.
Zoshima Takeshi, Okada Hirofumi, Kawano Mitsuhiro,
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