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Pubblicazioni recenti - dilated cardiomyopathy
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FUNDC1 protects against doxorubicin-induced cardiomyocyte PANoptosis through stabilizing mtDNA via interaction with TUFM.
Cell Death Dis2022 Dec;13(12):1020. doi: 10.1038/s41419-022-05460-x.
Bi Yaguang, Xu Haixia, Wang Xiang, Zhu Hong, Ge Junbo, Ren Jun, Zhang Yingmei,
Abstract
Doxorubicin (DOX) is an effective anthracycline chemotherapeutic anticancer drug with its life-threatening cardiotoxicity severely limiting its clinical application. Mitochondrial damage-induced cardiomyocyte death is considered an essential cue for DOX cardiotoxicity. FUN14 domain containing 1 (FUNDC1) is a mitochondrial membrane protein participating in the regulation of mitochondrial integrity in multiple diseases although its role in DOX cardiomyopathy remains elusive. Here, we examined whether PANoptosis, a novel type of programmed cell death closely associated with mitochondrial damage, was involved in DOX-induced heart injury, and FUNDC1-mediated regulation of cardiomyocyte PANoptosis, if any. FUNDC1 was downregulated in heart tissues in patients with dilated cardiomyopathy (DCM) and DOX-challenged mice. FUNDC1 deficiency aggravated DOX-induced cardiac dysfunction, mitochondrial injury, and cardiomyocyte PANoptosis. Further examination revealed that FUNDC1 countered cytoplasmic release of mitochondrial DNA (mtDNA) and activation of PANoptosome through interaction with mitochondrial Tu translation elongation factor (TUFM), a key factor in the translational expression and repair of mitochondrial DNA, via its 96-133 amino acid domain. TUFM intervention reversed FUNDC1-elicited protection against DOX-induced mtDNA cytosolic release and cardiomyocyte PANoptosis. Our findings shed light toward a beneficial role of FUNDC1 in DOX cardiotoxicity and cardiomyocyte PANoptosis, thus offering therapeutic promises in DOX-induced cardiotoxicity.
© 2022. The Author(s).
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Peripartum Cardiomyopathy-Induced Cardiogenic Shock Causing Hypoxic-Ischemic Encephalopathy in a COVID-19 Patient.
Case Rep Crit Care2022 ;2022():7114732. doi: 7114732.
Le Minh Khoi, Nguyen Thanh Hieu,
Abstract
BACKGROUND:
Cardiogenic shock complicating peripartum cardiomyopathy (PPCM) is a rare but lethal syndrome. The etiology of PPCM is not fully elucidated and is probably multifactorial, and viral infection might play some role. It has been documented that the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) directly invades the cardiomyocytes and most commonly damages this vital organ via complex systemic devastating mechanisms. . A 28-year-old pregnant female was admitted to a COVID-19 field hospital due to a SARS-CoV-2 infection. She gave birth by spontaneous vaginal delivery at 34 gestational weeks. Six hours after the delivery, she presented signs of hemodynamic collapse and became comatosed, requiring a transfer to the COVID-19 intensive care center. The brain magnetic resonance imaging excluded thromboembolism, intracerebral hemorrhage, and central nervous system infection and revealed a hypoxic-ischemic encephalopathy. Bedside echocardiography documented a dilated left ventricle and severely reduced left ventricular systolic function with an ejection fraction of 24%. The management was aimed at a cardiogenic shock secondary to peripartum cardiomyopathy. The clinical course was favorable: the hemodynamics stabilized, the cognitive function fully recovered, and the patient was extubated on the second day of admission to the intensive care unit. The patient was discharged from the hospital ten days after admission. Neurological and cardiovascular checkups six months after discharge showed full recovery.
CONCLUSION:
Peripartum cardiomyopathy-induced cardiogenic shock with severe neurological consequences in COVID-19 patients was rare but did exist. A systemic approach and vigorous efforts to pinpoint the accurate diagnosis played important roles in the prompt and appropriate management.
Copyright © 2022 Minh Khoi Le and Thanh Hieu Nguyen.
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A Case of Neonatal Lupus Presenting with Myocardial Dysfunction in the Absence of Congenital Heart Block (CHB): Clinical Management and Brief Literature Review of Neonatal Cardiac Lupus.
Pediatr Cardiol2022 Dec;():. doi: 10.1007/s00246-022-03056-y.
Jain Samhita, Spadafora Ruggero, Maxwell Sarah, Botas Carlos, Nawaytou Hythem, von Scheven Emily, Crouch Elizabeth E,
Abstract
Neonatal lupus (NLE) is a rare acquired autoimmune disorder caused by transplacental passage of maternal autoantibodies to Sjogren's Syndrome A or B (SSA-SSB) autoantigens (Vanoni et al. in Clin Rev Allerg Immunol 53:469-476, 2017) which target fetal and neonatal tissues for immune destruction. The cardiac trademark of NLE is autoimmune heart block, which accounts for more than 80% of cases of complete atrioventricular heart block (AVB) in newborns with a structurally normal heart (Martin in Cardiol Young 24: 41-46, 2014). NLE presenting with cardiac alterations not involving rhythm disturbances are described in the literature, but they are rare. Here, we report a case of a neonate with high anti-SSA antibodies who developed severe ventricular dysfunction in the absence of rhythm abnormalities, endocardial fibroelastosis, and dilated cardiomyopathy (Trucco et al. in J Am Coll Cardiol 57:715-723, https://doi.org/10.1016/j.jacc.2010.09.044 , 2011), the most common cardiac presentations of NLE. The patient developed severe multiorgan dysfunction syndrome that required prolonged critical care support but fully recovered and was discharged home. We highlight the unusual clinical features of this NLE case and the importance of timely treatment of NLE allowing complete recovery of a critically ill neonate.
© 2022. The Author(s).
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Normal and excessive muscle sympathetic nerve activity in heart failure: implications for future trials of therapeutic autonomic modulation.
Eur J Heart Fail2022 Dec;():. doi: 10.1002/ejhf.2749.
Badrov Mark B, Keir Daniel A, Tomlinson George, Notarius Catherine F, Millar Philip J, Kimmerly Derek S, Shoemaker J Kevin, Keys Evan, Floras John S,
Abstract
AIMS:
Patients with sympathetic excess are those most likely to benefit from novel interventions targeting the autonomic nervous system. To inform such personalized therapy, we identified determinants of augmented muscle sympathetic nerve activity (MSNA) in heart failure, versus healthy controls.
METHODS AND RESULTS:
We compared data acquired in 177 conventionally-treated, stable non-diabetic patients in sinus rhythm, aged 18-79?years (149 males; 28 females; LVEF: 25±11% [mean±SD]; range 5-60%), and, concurrently, under similar conditions, in 658 healthy, normotensive volunteers (398 males; 260 females; aged 18-81?years). In heart failure, MSNA ranged between 7 and 90 bursts·min , proportionate to heart rate (P
CONCLUSION:
Burst frequency and incidence exceeded normative values in only ~53% and ~33% of patients. Such diversity encourages selective deployment of sympatho-modulatory therapies. Clinical characteristics can highlight individuals who may benefit from future personalized interventions targeting pathological sympathetic activation.
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Too much heterogeneity: envisioning a new approach to methamphetamine associated heart failure.
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Methamphetamine-associated heart failure: a systematic review of observational studies.
Heart2022 Dec;():. doi: heartjnl-2022-321610.
Manja Veena, Nrusimha Ananya, Gao Ya, Sheikh Aleesha, McGovern Mark, Heidenreich Paul A, Sandhu Alex Tarlochan Singh, Asch Steven,
Abstract
OBJECTIVE:
To conduct a systematic review of observational studies on methamphetamine-associated heart failure (MethHF) .
METHODS:
Six databases were searched for original publications on the topic. Title/abstract and included full-text publications were reviewed in duplicate. Data extraction and critical appraisal for risk of bias were performed in duplicate.
RESULTS:
Twenty-one studies are included in the final analysis. Results could not be combined because of heterogeneity in study design, population, comparator, and outcome assessment. Overall risk of bias is moderate due to the presence of confounders, selection bias and poor matching; overall certainty in the evidence is very low. MethHF is increasing in prevalence, affects diverse racial/ethnic/sociodemographic groups with a male predominance; up to 44% have preserved left-ventricular ejection fraction. MethHF is associated with significant morbidity including worse heart failure symptoms compared with non-methamphetamine related heart failure. Female sex, methamphetamine abstinence and guideline-directed heart failure therapy are associated with improved outcomes. Chamber dimensions on echocardiography and fibrosis on biopsy predict the extent of recovery after abstinence.
CONCLUSIONS:
The increasing prevalence of MethHF with associated morbidity underscores the urgent need for well designed prospective studies of people who use methamphetamine to accurately assess the epidemiology, clinical features, disease trajectory and outcomes of MethHF. Methamphetamine abstinence is an integral part of MethHF treatment; increased availability of effective non-pharmacological interventions for treatment of methamphetamine addiction is an essential first step. Availability of effective pharmacological treatment for methamphetamine addiction will further support MethHF treatment. Using harm reduction principles in an integrated addiction/HF treatment programme will bolster efforts to stem the increasing tide of MethHF.
© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.
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Cardiovascular magnetic resonance imaging characteristics in patients with methamphetamine-associated cardiomyopathy.
J Cardiovasc Magn Reson2022 Dec;24(1):67. doi: 67.
Stokes Michael B, Thoi Fiona, Scherer Daniel J, Win Kyi T H, Kaye David M, Teo Karen S, Sanders Prashanthan,
Abstract
BACKGROUND:
Methamphetamine-associated cardiomyopathy (MA-CMP) is an increasingly recognised aetiology of cardiomyopathy. Cardiovascular magnetic resonance (CMR) is a specialised cardiac imaging modality commonly used in assessment of cardiomyopathy. We aimed to identify specific CMR features associated with MA-CMP.
METHODS:
A retrospective cohort study of CMR scans was performed in a single centre between January 2015 and December 2020. Thirty patients with MA-CMP who had undergone CMR were identified. MA-CMP was defined as those with a history of significant methamphetamine use hospitalised with acute decompensated heart failure (other causes of cardiomyopathy excluded). A retrospective analysis of index admission CMRs was performed. All studies were performed on a 1.5 T CMR scanner.
RESULTS:
The mean age of MA-CMP patients was 43.7?±?7.5 years, and 86.7% were male. The mean left ventricular (LV) volume obtained in this cohort was consistent with severe LV dilatation (LV end-diastolic volume (334?±?99 ml); LV end-systolic volume: 269?±?98 ml), whilst the right ventricular (RV) volume indicated moderate-to-severe dilatation (RV end-diastolic volume: 272?±?91 ml; RV end-systolic volume: 173?±?82 ml). Mean LV ejection fraction (20.9?±?9.2%) indicated severe LV dysfunction, with moderate-to-severe RV dysfunction also detected (RV ejection fraction: 29.4?±?13.4%). 22 patients (73.3%) had myocardial late gadolinium enhancement (LGE), of which 59.1% were located in the mid-wall, with all of these involving the interventricular septum. 22.7% displayed localised regions of sub-endocardial LGE in a variety of locations, and 18.2% had transmural regions of LGE that were located in the inferior and inferolateral segments. 6 patients (20%) had intracardiac thrombus (4 LV, 2 both LV and RV).
CONCLUSION:
MA-CMP was associated with severe biventricular dilatation and dysfunction, with a high prevalence of intraventricular thrombus. This cohort study highlights that MA-CMP patients have a high prevalence of CMR findings.
© 2022. The Author(s).
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UEDA Heart Awards for 2022.
Int Heart J2022 ;63(6):1217. doi: 10.1536/ihj.63-6_UEDA.
Abstract
We are pleased to announce that the following 3 articles have been selected for the UEDA Heart Awards for the Year 2022.FIRST PLACEChronic HDAC6 Activation Induces Atrial Fibrillation Through Atrial Electrical and Structural Remodeling in Transgenic MiceYohei Sawa, Naoko Matsushita, Sachiko Sato, Nanae Ishida, Maki Saito, Atsushi Sanbe, Yoshihiro Morino, Eiichi Taira, Mami Obara, Masamichi HiroseInt Heart J 2021; 62 (3): 616-626.SECOND PLACEA Novel Titin Truncation Variant Linked to Familial Dilated Cardiomyopathy Found in a Japanese Family and Its Functional Analysis in Genome-Edited Model CellsKayoko Hirayama-Yamada, Natsuko Inagaki, Takeharu Hayashi, Akinori KimuraInt Heart J 2021; 62 (2): 359-366.THIRD PLACECytokine Signaling and Matrix Remodeling Pathways Associated with Cardiac Sarcoidosis Disease Activity Defined Using FDG PET ImagingBryan D. Young, Hannah Moreland, Kelsie E. Oatmen, Lisa A. Freeburg, Zartashia Shahab, Erica Herzog, Edward J. Miller, Francis G. SpinaleInt Heart J 2021; 62 (5): 1096-1105.
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Drug-refractory Heart Failure in Female Carrier of Duchenne Muscular Dystrophy: A Case of X-linked Dilated Cardiomyopathy.
Intern Med2022 Nov;():. doi: 10.2169/internalmedicine.0745-22.
Ohtani Hayato, Saotome Masao, Sakamoto Atsushi, Suwa Kenichiro, Maekawa Yuichiro,
Abstract
A 56-year-old woman was referred to our hospital for the further evaluation of drug-refractory heart failure with a reduced ejection fraction. A family history interview revealed that men in her family had died of Duchenne muscular dystrophy (DMD), whereas she had no skeletal muscle disorder. Myocardial histopathology revealed a reduced dystrophin expression in the cardiomyocyte membrane, and a dystrophin (DMD) gene analysis identified a duplication in exon 8-9 on Xp21, suggesting that she had a cardiac-specific phenotype of dystrophinopathy, i.e. X-linked dilated cardiomyopathy (XLDCM). In conclusion, careful family history interviews and an investigation of dystrophinopathy are required to detect XLDCM in women.
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Dilated Cardiomyopathy With Multiple Left Ventricular Thrombi and Embolic Stroke After Mild COVID-19.
Tex Heart Inst J2022 Nov;49(6):. doi: e207488.
Yalamanchi Radhapriya, Murugan Madhan Kumar, Chandrasekharan Krishnaswamy, Showkathali Refai,
Abstract
COVID-19 is a novel disease with multisystem involvement, but most patients have pulmonary and cardiovascular involvement in the acute stages. The cardiovascular impact of acute COVID-19 is well recognized and ranges from myocarditis, arrhythmias, and thrombotic occlusion of coronary arteries to spontaneous coronary artery dissection and microthrombi in small coronary vessels on autopsy. We report a case of a 37-year-old man who recovered from mild COVID-19 only to present a few weeks later with devastating cardiovascular involvement that included severe left ventricular impairment resulting from nonischemic cardiomyopathy, multiple left ventricular thrombi, and embolic stroke.
© 2022 by the Texas Heart® Institute, Houston.
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What acute cardiac care physicians need to know from the latest 2022 ESC Guidelines for Ventricular Tachycardia and Sudden Cardiac Death.
Eur Heart J Acute Cardiovasc Care2022 Dec;():. doi: zuac149.
Goette Andreas, Lip Gregory Y H, Gorenek Bulent,
Abstract
The present paper summarizes and comments on the latest 2022 ESC guidelines on ventricular tachycardia and sudden cardiac death. Most relevant recommendations for acute cardiovascular care physicians are addressed, particularly, in the fields of coronary artery disease, dilated cardiomyopathy and inflammatory diseases. New recommendations encompass the implantation of a defibrillator (ICD) in the setting of an acute myocarditis. Furthermore, the pathophysiology of electrical storm including involved molecular pathways as well as the angry Purkinje fiber syndrome are presented and discussed.
© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.
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Dilated Cardiomyopathy and Systolic Heart Failure in a Female Patient With Danon Disease.
Cureus2022 Oct;14(10):e30803. doi: e30803.
Stack-Pyle Taylor J, Shah Joel, Stack Lindsey, Arcara Victoria,
Abstract
Danon disease commonly manifests as isolated hypertrophic cardiomyopathy in female patients. The diagnosis is easily missed as it is rare and its pathophysiology is poorly understood. Without early diagnosis and treatment with heart transplantation, cardiomyopathy may progress to heart failure. We present the case of an adolescent female with Danon disease who presented with heart failure with reduced ejection fraction secondary to dilated cardiomyopathy. Her original presentation included a six-month history of shortness of breath, vomiting, and abdominal pain. Further workup revealed pelvic ascites and dilated cardiomyopathy with signs of heart failure. Genetic testing confirmed the diagnosis, revealing a previously undocumented amino acid substitution in the lysosomal-associated membrane protein-2 (LAMP2) gene. The patient received a heart transplant which led to an improvement in her symptoms. The patient's unique symptomatology illustrates the importance of early cardiac monitoring and transplantation if Danon disease is suspected. This uncommon presentation of dilated cardiomyopathy followed by psychiatric impairment, developmental disability, and unique LAMP2 genetic substitution provides a rare phenotype of Danon disease.
Copyright © 2022, Stack-Pyle et al.
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Automated Measurement of Native T1 and Extracellular Volume Fraction in Cardiac Magnetic Resonance Imaging Using a Commercially Available Deep Learning Algorithm.
Korean J Radiol2022 Dec;23(12):1251-1259. doi: 10.3348/kjr.2022.0496.
Chang Suyon, Han Kyunghwa, Lee Suji, Yang Young Joong, Kim Pan Ki, Choi Byoung Wook, Suh Young Joo,
Abstract
OBJECTIVE:
T1 mapping provides valuable information regarding cardiomyopathies. Manual drawing is time consuming and prone to subjective errors. Therefore, this study aimed to test a DL algorithm for the automated measurement of native T1 and extracellular volume (ECV) fractions in cardiac magnetic resonance (CMR) imaging with a temporally separated dataset.
MATERIALS AND METHODS:
CMR images obtained for 95 participants (mean age ± standard deviation, 54.5 ± 15.2 years), including 36 left ventricular hypertrophy (12 hypertrophic cardiomyopathy, 12 Fabry disease, and 12 amyloidosis), 32 dilated cardiomyopathy, and 27 healthy volunteers, were included. A commercial deep learning (DL) algorithm based on 2D U-net (Myomics-T1 software, version 1.0.0) was used for the automated analysis of T1 maps. Four radiologists, as study readers, performed manual analysis. The reference standard was the consensus result of the manual analysis by two additional expert readers. The segmentation performance of the DL algorithm and the correlation and agreement between the automated measurement and the reference standard were assessed. Interobserver agreement among the four radiologists was analyzed.
RESULTS:
DL successfully segmented the myocardium in 99.3% of slices in the native T1 map and 89.8% of slices in the post-T1 map with Dice similarity coefficients of 0.86 ± 0.05 and 0.74 ± 0.17, respectively. Native T1 and ECV showed strong correlation and agreement between DL and the reference: for T1, = 0.967 (95% confidence interval [CI], 0.951-0.978) and bias of 9.5 msec (95% limits of agreement [LOA], -23.6-42.6 msec); for ECV, = 0.987 (95% CI, 0.980-0.991) and bias of 0.7% (95% LOA, -2.8%-4.2%) on per-subject basis. Agreements between DL and each of the four radiologists were excellent (intraclass correlation coefficient [ICC] of 0.98-0.99 for both native T1 and ECV), comparable to the pairwise agreement between the radiologists (ICC of 0.97-1.00 and 0.99-1.00 for native T1 and ECV, respectively).
CONCLUSION:
The DL algorithm allowed automated T1 and ECV measurements comparable to those of radiologists.
Copyright © 2022 The Korean Society of Radiology.
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Prognostic value of cardiac magnetic resonance derived global longitudinal strain analysis in patients with ischaemic and non-ischaemic dilated cardiomyopathy: a systematic review and meta-analysis.
Int J Cardiovasc Imaging2022 Dec;38(12):2707-2721. doi: 10.1007/s10554-022-02679-9.
Fong Lucas Chun Wah, Lee Nicholas Ho Cheung, Poon Jessica Wai Ling, Chin Calvin W L, He Baroc, Luo Lin, Chen Catherine, Wan Eric Yuk Fai, Pennell Dudley J, Mohiaddin Raad, Ng Ming-Yen,
Abstract
Cardiac magnetic resonance (CMR) derived left ventricular global longitudinal strain (LV-GLS) for evaluating dilated cardiomyopathy patients has been addressed in studies with contradictory results. We therefore performed the first systematic review evaluating evidence on the prognostic value of CMR derived LV-GLS for ischaemic (IDCM) and non-ischaemic dilated cardiomyopathy (NDCM) patients. Systematic review (PROSPERO CRD42020171582) identified studies up to January 2021 that measured LV-GLS for predicting major adverse cardiac events among dilated cardiomyopathy patients. Studies were identified from MEDLINE, Embase and PubMed by two independent reviewers. 2099 studies were screened. Three prospective and three retrospective observational studies comprising of 1758 patients (29% IDCM patients; 71% NDCM patients) with a weighted mean follow up of 3 years (SD?=?1 year) were identified. All six studies included mortality in the primary composite outcome. LV-GLS was associated with increase primary composite outcome among mild to moderately impaired left ventricular ejection fraction (LVEF) IDCM and NDCM patients (>?30%) in univariable and multivariable analysis. Association was lost among severely impaired LVEF patients (30%). From sensitivity analysis, LV-GLS showed significant association with death among NDCM patients (HR 1.27; 95% CI 1.10-1.46; p?=?0.001; I?=?59%) but insignificant for heart transplant outcome (HR 1.23; 95% CI 0.46-3.33; p?=?0.68, I?=?44%). LV-GLS threshold for effectively stratifying patients is - 12.5% to - 13.5%. LVEF in IDCM and NDCM became an insignificant prognostic marker in multivariable analysis. CMR LV-GLS shows promise as an independent predictor of mortality in IDCM and NDCM patients. However, in patients with LVEF?30% LV-GLS may have less prognostic value.Prospero Registration: CRD42020171582.
© 2022. The Author(s), under exclusive licence to Springer Nature B.V.
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Association Between Right Ventricular Dysfunction and Adverse Outcomes in Peripartum Cardiomyopathy: Insights From the BRO-HF Quebec Cohort Study.
CJC Open2022 Nov;4(11):913-920. doi: 10.1016/j.cjco.2022.05.004.
Pacheco Christine, Tremblay-Gravel Maxime, Marquis-Gravel Guillaume, Couture Etienne, Avram Robert, Desplantie Olivier, Bibas Lior, Simard François, Malhamé Isabelle, Poulin Anthony, Tran Dan, Senechal Mario, Afilalo Jonathan, Farand Paul, Bérubé Lyne, Jolicoeur E Marc, Ducharme Anique, Tournoux François,
Abstract
BACKGROUND:
Peripartum cardiomyopathy (PPCM) is associated with severe morbidity and mortality, and the significance of right ventricular (RV) involvement is unclear. We sought to determine whether RV systolic dysfunction or dilatation is associated with adverse clinical outcomes in women with PPCM.
METHODS:
We conducted a multicentre retrospective cohort study examining the association between echocardiographic RV systolic dysfunction or dilatation at the time of PPCM diagnosis and clinical outcomes. Clinical endpoints of interest were the need for mechanical support, recovery of left ventricular ejection fraction at follow-up, and a combined endpoint of hospitalization for heart failure, cardiac transplant, or death.
RESULTS:
A total of 67 women, median age 30 years (interquartile range: 7), were diagnosed with PPCM between 1994 and 2015 in 17 participating centres. Twin pregnancies occurred in 11%; 62% of women were multiparous; and 24% had preeclampsia. RV systolic function was impaired in 18 (27%) and dilated in 8 (12%). Seven women required ventricular assistance, and 8 experienced the composite outcome during follow-up (25 [interquartile range 61] months). RV dysfunction was associated with the need for mechanical support (odds ratio 10.10 (95% confidence interval: 1.86-54.81), = 0.007), but neither RV dysfunction nor dilatation was associated with left ventricular ejection fraction recovery, the need for cardiac transplant, heart failure hospitalization, or death.
CONCLUSIONS:
RV dysfunction is associated with the need for mechanical support in women with PPCM. These findings may improve risk stratification of complications and clinical management.
© 2022 The Authors.
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[The new ESC guidelines on the management of ventricular tachyarrhythmias : Implications for daily practice].
Herz2022 Nov;():. doi: 10.1007/s00059-022-05148-0.
Könemann Hilke, Frommeyer Gerrit, Zeppenfeld Katja, Eckardt Lars,
Abstract
The recently published guidelines of the European Society of Cardiology (ESC) on the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death is an update of the 2015 guideline. For the first time a new section is dedicated to public basic life support. In the acute treatment of ventricular arrhythmias electrical cardioversion is upgraded, and there is a new focus on the management of electrical storm. Recommendations for the comprehensive diagnostic evaluation of patients with first manifestations of ventricular arrhythmias structured according to common clinical scenarios are also new. Both genetic testing and cardiac magnetic resonance imaging are upgraded, not only for diagnostic evaluation but also for risk stratification. In the long-term management, recommendations for pharmacotherapy are aligned with current heart failure guidelines. Catheter ablation has gained relevance not only for recurrent ventricular tachycardia under amiodarone treatment and as an alternative to implantable cardioverter defibrillation (ICD) implantation in selected patients with coronary artery disease but also particularly in the treatment of idiopathic ventricular extrasystoles and tachycardia. The ICD treatment remains an essential component of primary and secondary prevention of sudden cardiac death. Of note, the recommendation on primary preventive ICD treatment for patients with dilated cardiomyopathy and left ventricular ejection fraction (LVEF)???35% has been downgraded. In addition to LVEF a combination of risk factors and risk calculators is included in the recommendations on primary prophylactic ICD implantation. Overall, due to numerous tables and practical algorithms, the guidelines have become a user-oriented reference book.
© 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.
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Prenatal case report of Barth syndrome caused by novel mutation: Clinical characteristics of fetal dilated cardiomyopathy with ascites.
Front Pediatr2022 ;10():1004485. doi: 1004485.
Zhao Xuliang, Li Xu, Sun Weiwei, Jia Jian-An, Yu Min, Tian Ruixia,
Abstract
Barth syndrome (BTHS) is a rare X-linked recessive genetic disease, which appears in infancy with myocardial and skeletal muscle diseases, neutropenia, growth retardation, and other clinical features. is the pathogenic gene of BTHS, which encodes the tafazzin protein of the inner membrane of the mitochondria, a phosphatidyltransferase involved in cardiolipin remodeling and functional maturation. At present, BTHS has been widely reported, but prenatal cases are rare. We report a 24-week fetus with clinical manifestations including left ventricular insufficiency and ascites. After induced labor, whole exome sequencing detection of fetal skin tissue showed that had the mutation c.311A?>?C/.His104Pro and that his mother was the carrier. This His104Pro mutation has hitherto not been reported, and it is rated as likely to be pathogenic according to the American College of Medical Genetics and Genetics guidelines. Molecular dynamics and protein expression experiments on the His104Pro mutation showed that the stability of the local protein structure and protein expression were reduced. In conclusion, the case presented in this study enriches our knowledge of the mutation spectrum and suggests that His104Pro may lead to cardiac structural abnormalities in the early embryo. The possibility of BTHS should be considered when an abnormal cardiac structure or ascites appear in a prenatal ultrasound.
© 2022 Zhao, Li, Sun, Jia, Yu and Tian.
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Risk factors and a Bayesian network model to predict ischemic stroke in patients with dilated cardiomyopathy.
Front Neurosci2022 ;16():1043922. doi: 1043922.
Fan Ze-Xin, Wang Chao-Bin, Fang Li-Bo, Ma Lin, Niu Tian-Tong, Wang Ze-Yi, Lu Jian-Feng, Yuan Bo-Yi, Liu Guang-Zhi,
Abstract
OBJECTIVE:
This study aimed to identify risk factors and create a predictive model for ischemic stroke (IS) in patients with dilated cardiomyopathy (DCM) using the Bayesian network (BN) approach.
MATERIALS AND METHODS:
We collected clinical data of 634 patients with DCM treated at three referral management centers in Beijing between 2016 and 2021, including 127 with and 507 without IS. The patients were randomly divided into training (441 cases) and test (193 cases) sets at a ratio of 7:3. A BN model was established using the Tabu search algorithm with the training set data and verified with the test set data. The BN and logistic regression models were compared using the area under the receiver operating characteristic curve (AUC).
RESULTS:
Multivariate logistic regression analysis showed that hypertension, hyperlipidemia, atrial fibrillation/flutter, estimated glomerular filtration rate (eGFR), and intracardiac thrombosis were associated with IS. The BN model found that hyperlipidemia, atrial fibrillation (AF) or atrial flutter, eGFR, and intracardiac thrombosis were closely associated with IS. Compared to the logistic regression model, the BN model for IS performed better or equally well in the training and test sets, with respective accuracies of 83.7 and 85.5%, AUC of 0.763 [95% confidence interval (CI), 0.708-0.818] and 0.822 (95% CI, 0.748-0.896), sensitivities of 20.2 and 44.2%, and specificities of 98.3 and 97.3%.
CONCLUSION:
Hypertension, hyperlipidemia, AF or atrial flutter, low eGFR, and intracardiac thrombosis were good predictors of IS in patients with DCM. The BN model was superior to the traditional logistic regression model in predicting IS in patients with DCM and is, therefore, more suitable for early IS detection and diagnosis, and could help prevent the occurrence and recurrence of IS in this patient cohort.
Copyright © 2022 Fan, Wang, Fang, Ma, Niu, Wang, Lu, Yuan and Liu.
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Outcomes of catheter ablation of ventricular tachycardia in non-ischemic idiopathic dilated cardiomyopathy: A systematic review and meta-analysis.
Front Cardiovasc Med2022 ;9():1007392. doi: 1007392.
Ammar Ahmed, Sharief Mohamed, Abouelmagd Khaled, Riad Omar, Ibrahim Mokhtar,
Abstract
OBJECTIVE:
To perform a systematic review and meta-analysis of available trials regarding the outcomes of ventricular tachycardia (VT) ablation in patients with non-ischemic dilated cardiomyopathy (NIDCM).
METHODS:
A comprehensive database search of large four electronic databases, including PubMed, Cochrane, Scopus, and Institute for Scientific Information network meta-analysis, identified five studies enrolling 666 patients for patients with idiopathic dilated cardiomyopathy (IDCM) underwent catheter ablation (CA) for VT. The short-term outcomes assessed included procedural success, VT non-inducibility and procedural complications, whereas the long-term outcomes assessed included VT recurrence, heart transplantation, antiarrhythmic drugs (AAD) use after ablation and death.
RESULTS:
A total of 5 observational studies reported outcomes in 666 patients with NIDCM undergoing VT CA. The complete procedural success was moderately high; 65.5% of the patients (95% CI 0.402- 0.857,
CONCLUSION:
Ventricular tachycardia CA is effective and safe approach for management of patients with NIDCM with the epicardial approach to be considered as initial strategy especially in presence of ECG and CMR findings suggestive of epicardial substrate. A multicenter randomized trial is crucial to look at the short- and long-term outcomes of VT ablation in NIDCM especially with the advances in mapping and ablation techniques and predictors of success.
Copyright © 2022 Ammar, Sharief, Abouelmagd, Riad and Ibrahim.
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ECG-based techniques to enhance clinical practice in cardiac genetic disease management.
J Electrocardiol2022 Nov;76():55-60. doi: 10.1016/j.jelectrocard.2022.10.013.
Boonstra Machteld, Kloosterman Manon, van der Schaaf Iris, Roudijk Rob, van Dam Peter, Loh Peter,
Abstract
INTRODUCTION:
Inherited cardiomyopathies are associated with a broad spectrum of potentially lethal phenotypes characterized by structural and electrical myocardial remodeling. Increased awareness and genetic cascade screening lead to more genotype-positive, yet phenotype-negative individuals to be evaluated and followed up. The predictive value of genetic testing is hampered by incomplete penetrance and high variability in disease onset, progression and severity.
CLINICAL CHALLENGES:
Dilated cardiomyopathy usually manifests with symptoms of heart failure and ventricular arrhythmias (VA) develop in advanced disease. In arrhythmogenic cardiomyopathy (ACM), electrical remodeling can precede structural and functional changes and life-threatening VA can be the first disease manifestation. Early signs and symptoms may be subtle and go unnoticed. Physicians are in great need of appropriate screening and risk-stratification strategies. Task Force Criteria (TFC) were established to standardize the clinical diagnosis of ACM but risk-stratification remains challenging. Accurate prediction of disease progression in variation carriers is currently beyond the capabilities of diagnostic tests.
PROPOSED DIAGNOSTIC TECHNIQUES:
We propose three ECG-based techniques; isopotential mapping, inverse ECG and CineECG, to enhance risk-stratification in ACM. With the use of isopotential mapping abnormal spatio-temporal activation and repolarization may be identified. Furthermore, by combining subject specific ?12?lead ECG data with cardiothoracic imaging using inverse ECG techniques, the direct link between ECG and cardiac anatomy can be obtained.
CONCLUSION:
New ECG techniques may prove more sensitive to detect early de- and repolarization abnormalities in yet asymptomatic variation carriers. Early electrical signs of disease progression may be identified prior to symptoms. Furthermore, individualized risk-stratification may be enhanced.
Copyright © 2022. Published by Elsevier Inc.
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