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Li M, Liu W, Mi S, Wang M, Wang Y, Guo Q, Lian Z, Ge J. Clinical and Echocardiographic Risk Factors of Adverse Outcomes in Young Patients With Dilated Cardiomyopathy. Cardiovasc Ther 2025; 2025:2122089. [PMID: 40225593 PMCID: PMC11986925 DOI: 10.1155/cdr/2122089] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/30/2024] [Revised: 01/31/2025] [Accepted: 02/20/2025] [Indexed: 04/15/2025] Open
Abstract
Purpose: This study is aimed at identifying clinical and echocardiographic factors associated with all-cause mortality or heart transplantation (HTx) in young patients with dilated cardiomyopathy (DCM). Methods: We conducted a retrospective analysis of hospitalized patients (aged 18-45 years) diagnosed with DCM between January 2012 and December 2022. All patients underwent a 2-year medical therapy for heart failure, followed by at least 1 year of follow-up. Clinical and echocardiographic data were collected at baseline and after the 2-year treatment period. Multivariate Cox proportional hazards regression with a backward stepwise method was used to identify risk factors for all-cause mortality or HTx. Results: The study cohort comprised 67 patients. Over a median follow-up of 38 months (range 18-50), 15 patients died and 24 underwent HTx. Significant risk factors for all-cause mortality/HTx included smoking, digoxin use, elevated N-terminal pro-brain natriuretic peptide (NT-proBNP, ≥ 5678 pg/mL), higher C-reactive protein (CRP, ≥ 3.0 mg/L), higher uric acid (UA, ≥ 570 μmol/L), lower left ventricular ejection fraction (LVEF, ≤25%), and enlarged end-diastolic left ventricular diameter (LVD, ≥ 65 mm). Among these, elevated CRP (hazard ratio, HR = 6.727, p < 0.001) and enlarged LVD (HR = 3.038, p = 0.007) were the strongest independent risk factors, irrespective of other risk factors. Moreover, each 5 mm annual increase in end-systolic left atrial diameter (LAD, HR = 3.641, p < 0.001) and each unit annual increase in Ln(NT-proBNP) (HR = 4.069, p < 0.001) were the strongest predictors of all-cause mortality/HTx, even after accounting for the effects of body mass index, duration of treatment, and baseline CRP level. Conclusions: Intensive monitoring and medical care may be beneficial for young adult DCM patients with defined risk factors such as smoking, elevated NT-proBNP and CRP, lower LVEF, and enlarged LV diameter. Our findings suggest that personalized intensive monitoring and medical care based on identified risk factors may improve outcomes in young adult DCM patients.
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Affiliation(s)
- Mengwan Li
- Department of Cardiology, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, China
- Qingdao Municipal Key Laboratory of Hypertension (Key Laboratory of Cardiovascular Medicine), Qingdao, Shandong Province, China
| | - Wenheng Liu
- Department of Cardiology, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, China
- Qingdao Municipal Key Laboratory of Hypertension (Key Laboratory of Cardiovascular Medicine), Qingdao, Shandong Province, China
| | - Shouling Mi
- Department of Cardiology, Zhongshan Hospital, Fudan University, Shanghai, China
- Shanghai Institute of Cardiovascular Diseases, Shanghai, China
- State Key Laboratory of Cardiovascular Diseases, Shanghai, China
- NHC Key Laboratory of Ischemic Heart Diseases, Shanghai, China
- Key Laboratory of Viral Heart Diseases, Chinese Academy of Medical Sciences, Shanghai, China
- National Clinical Research Center for Interventional Medicine, Shanghai, China
| | - Meng Wang
- Department of Cardiology, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, China
- Qingdao Municipal Key Laboratory of Hypertension (Key Laboratory of Cardiovascular Medicine), Qingdao, Shandong Province, China
| | - Yanli Wang
- Department of Cardiology, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, China
- Qingdao Municipal Key Laboratory of Hypertension (Key Laboratory of Cardiovascular Medicine), Qingdao, Shandong Province, China
| | - Qilong Guo
- Department of Cardiology, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, China
- Qingdao Municipal Key Laboratory of Hypertension (Key Laboratory of Cardiovascular Medicine), Qingdao, Shandong Province, China
| | - Zhexun Lian
- Department of Cardiology, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, China
- Qingdao Municipal Key Laboratory of Hypertension (Key Laboratory of Cardiovascular Medicine), Qingdao, Shandong Province, China
| | - Junhua Ge
- Department of Cardiology, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, China
- Qingdao Municipal Key Laboratory of Hypertension (Key Laboratory of Cardiovascular Medicine), Qingdao, Shandong Province, China
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Amemiya K, Matsuyama TA, Ishibashi-Ueda H, Morita Y, Matsumoto M, Ohta-Ogo K, Ikeda Y, Tsukamoto Y, Fukushima N, Fukushima S, Fujita T, Hatakeyama K. Can right ventricular endomyocardial biopsy predict left ventricular fibrosis beforehand in dilated cardiomyopathy? ESC Heart Fail 2024; 11:1001-1008. [PMID: 38234242 DOI: 10.1002/ehf2.14642] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/05/2023] [Revised: 10/18/2023] [Accepted: 12/05/2023] [Indexed: 01/19/2024] Open
Abstract
AIMS Myocardial fibrosis of the left ventricle (LV) is a prognostic factor in dilated cardiomyopathy (DCM). This study aims to evaluate whether fibrosis of right ventricular (RV) endomyocardial biopsy (EMB) can predict the degree of LV fibrosis beforehand in DCM. METHODS AND RESULTS Fibrosis extent in 70 RV-EMB specimens of DCM diagnosis was compared with that in the whole cross-sectional LV of excised hearts in the same patients (52 explanted hearts for transplant and 18 autopsied hearts). The median interval between biopsy and excision was 4.1 (0.13-19.3) years. The fibrosis area ratio of the EMBs and excised hearts were evaluated via image analysis. The distribution of cardiovascular magnetic resonance-late gadolinium enhancement (LGE) in the intraventricular septum was classified into four quartile categories. The fibrosis area ratio in RV-EMB correlated significantly with that in the short-axis cut of the LV of excised hearts (r = 0.82, P < 0.0001) and with a diffuse pattern of LGE (r = 0.71, P = 0.003). In a multivariate model, after adjusting for the interval between biopsy performance and heart excision, the fibrosis area ratio in RV-EMB was associated with that in LV-excised heart (regression coefficient, 0.82; 95% confidence interval, 0.68-0.95; P < 0.0001). CONCLUSIONS The fibrosis observed in RV-EMB positively correlated with the extent of fibrosis in the LV of excised hearts in patients with DCM. The study findings may help predict LV fibrosis, considered a prognostic factor of DCM through relatively accessible biopsy techniques.
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Affiliation(s)
- Kisaki Amemiya
- Department of Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan
- Department of Legal Medicine, Showa University School of Medicine, Tokyo, Japan
| | - Taka-Aki Matsuyama
- Department of Legal Medicine, Showa University School of Medicine, Tokyo, Japan
| | - Hatsue Ishibashi-Ueda
- Department of Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan
- Division of Pathology, Hokusetsu General Hospital, Osaka, Japan
| | - Yoshiaki Morita
- Department of Radiology, National Cerebral and Cardiovascular Center, Osaka, Japan
| | - Manabu Matsumoto
- Department of Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan
| | - Keiko Ohta-Ogo
- Department of Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan
| | - Yoshihiko Ikeda
- Department of Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan
| | - Yasumasa Tsukamoto
- Department of Transplant Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan
| | - Norihide Fukushima
- Department of Transplant Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan
- Senri Kinran University, Osaka, Japan
| | - Satsuki Fukushima
- Department of Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan
| | - Tomoyuki Fujita
- Department of Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan
- Department of Cardiovascular Surgery, Tokyo Medical and Dental University, Tokyo, Japan
| | - Kinta Hatakeyama
- Department of Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan
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Xin XX, Se YY. Caution in the use of sedation and endomyocardial biopsy for the management of pediatric acute heart failure caused by endocardial fibroelastosis. World J Clin Cases 2023; 11:5412-5415. [PMID: 37621580 PMCID: PMC10445076 DOI: 10.12998/wjcc.v11.i22.5412] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/30/2023] [Revised: 07/04/2023] [Accepted: 07/17/2023] [Indexed: 08/04/2023] Open
Abstract
Endocardial fibroelastosis (EFE) is commonly considered to be an inflammatory reactive lesion of hyperplasia and deposition of tissue fibers and collagen in the endocardium and/or subendocardium, which is strongly associated with endocardial sclerosis, ventricular remodeling and acute and chronic heart failure, and is one of the important causes for pediatric heart transplantation. Early diagnosis and treatment are the key factors in determining the prognosis of the children. In this paper, we would like to highlight the potential unintended consequences of the use of sedation and biopsy for pediatric acute heart failure caused by EFE and the comprehensive considerations prior to clinical diagnosis.
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Affiliation(s)
- Xiao-Xuan Xin
- School of Hulunbuir Clinical Medicine, Inner Mongolia Minzu University, Hulunbuir 021000, Inner Mongolia Autonomous Region, China
| | - Yo-Yeng Se
- Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong 999077, China
- Jockey Club College of Veterinary Medicine and Life Sciences, City University of Hong Kong, Hong Kong 999077, China
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Xin XX, Se YY. Caution in the use of sedation and endomyocardial biopsy for the management of pediatric acute heart failure caused by endocardial fibroelastosis. World J Clin Cases 2023; 11:5406-5409. [DOI: 10.12998/wjcc.v11.i22.5406] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/30/2023] [Revised: 07/04/2023] [Accepted: 07/17/2023] [Indexed: 08/03/2023] Open
Abstract
Endocardial fibroelastosis (EFE) is commonly considered to be an inflammatory reactive lesion of hyperplasia and deposition of tissue fibers and collagen in the endocardium and/or subendocardium, which is strongly associated with endocardial sclerosis, ventricular remodeling and acute and chronic heart failure, and is one of the important causes for pediatric heart transplantation. Early diagnosis and treatment are the key factors in determining the prognosis of the children. In this paper, we would like to highlight the potential unintended consequences of the use of sedation and biopsy for pediatric acute heart failure caused by EFE and the comprehensive considerations prior to clinical diagnosis.
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Affiliation(s)
- Xiao-Xuan Xin
- School of Hulunbuir Clinical Medicine, Inner Mongolia Minzu University, Hulunbuir 021000, Inner Mongolia Autonomous Region, China
| | - Yo-Yeng Se
- Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong 999077, China
- Jockey Club College of Veterinary Medicine and Life Sciences, City University of Hong Kong, Hong Kong 999077, China
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Sarohi V, Srivastava S, Basak T. A Comprehensive Outlook on Dilated Cardiomyopathy (DCM): State-Of-The-Art Developments with Special Emphasis on OMICS-Based Approaches. J Cardiovasc Dev Dis 2022; 9:jcdd9060174. [PMID: 35735803 PMCID: PMC9225617 DOI: 10.3390/jcdd9060174] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/08/2021] [Revised: 05/12/2022] [Accepted: 05/19/2022] [Indexed: 02/04/2023] Open
Abstract
Dilated cardiomyopathy (DCM) remains an enigmatic cardiovascular disease (CVD) condition characterized by contractile dysfunction of the myocardium due to dilation of the ventricles. DCM is one of the major forms of CVD contributing to heart failure. Dilation of the left or both ventricles with systolic dysfunction, not explained by known causes, is a hallmark of DCM. Progression of DCM leads to heart failure. Genetic and various other factors greatly contribute to the development of DCM, but the etiology has still remained elusive in a large number of cases. A significant number of studies have been carried out to identify the genetic causes of DCM. These candidate-gene studies revealed that mutations in the genes of the fibrous, cytoskeletal, and sarcomeric proteins of cardiomyocytes result in the development of DCM. However, a significant proportion of DCM patients are idiopathic in nature. In this review, we holistically described the symptoms, causes (in adults and newborns), genetic basis, and mechanistic progression of DCM. Further, we also summarized the state-of-the-art diagnosis, available biomarkers, treatments, and ongoing clinical trials of potential drug regimens. DCM-mediated heart failure is on the rise worldwide including in India. The discovery of biomarkers with a better prognostic value is the need of the hour for better management of DCM-mediated heart failure patients. With the advent of next-generation omics-based technologies, it is now possible to probe systems-level alterations in DCM patients pertaining to the identification of novel proteomic and lipidomic biomarkers. Here, we also highlight the onset of a systems-level study in Indian DCM patients by applying state-of-the-art mass-spectrometry-based “clinical proteomics” and “clinical lipidomics”.
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Affiliation(s)
- Vivek Sarohi
- Indian Institute of Technology (IIT)-Mandi, School of Basic Sciences (SBS), Mandi 175075, HP, India; (V.S.); (S.S.)
- BioX Centre, Indian Institute of Technology (IIT)-Mandi, Mandi 175075, HP, India
| | - Shriya Srivastava
- Indian Institute of Technology (IIT)-Mandi, School of Basic Sciences (SBS), Mandi 175075, HP, India; (V.S.); (S.S.)
| | - Trayambak Basak
- Indian Institute of Technology (IIT)-Mandi, School of Basic Sciences (SBS), Mandi 175075, HP, India; (V.S.); (S.S.)
- BioX Centre, Indian Institute of Technology (IIT)-Mandi, Mandi 175075, HP, India
- Correspondence: ; Tel.: +91-1905-267826
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6
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Amara RS, Dickfeld T. Acute Myocarditis. JACC: CARDIOVASCULAR IMAGING 2022; 15:1781-1783. [DOI: 10.1016/j.jcmg.2022.04.004] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 03/31/2022] [Accepted: 04/07/2022] [Indexed: 11/25/2022]
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Abou Hassan OK, Sheng CC, Wang TKM, Cremer PC. SARS-CoV-2 Myocarditis: Insights Into Incidence, Prognosis, and Therapeutic Implications. Curr Cardiol Rep 2021; 23:129. [PMID: 34342728 PMCID: PMC8330199 DOI: 10.1007/s11886-021-01551-x] [Citation(s) in RCA: 17] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 04/11/2021] [Indexed: 12/16/2022]
Abstract
PURPOSE OF REVIEW In coronavirus disease 2019 (COVID-19), myocardial injury occurs frequently in severe or critically ill hospitalized patients, yet myocarditis is much less common. In this context, revisiting the definition of myocarditis is appropriate with a specific focus on diagnostic and management considerations in patients infected with SARS-CoV-2. RECENT FINDINGS Pathologic cardiac specimens from patients with COVID-19 suggest a mixed inflammatory response involving lymphocytes and macrophages, and importantly, cellular injury occurs predominantly at the level of pericytes and endothelial cells, less often involving direct myocyte necrosis. In COVID-19, the diagnosis of myocarditis has understandably been based predominantly on clinical criteria, and the number of patients with clinically suspected myocarditis who would meet diagnostic histological criteria is unclear. Echocardiography and cardiac magnetic resonance are important diagnostic tools, although the prognostic implications of abnormalities are still being defined. Importantly, SARS-CoV2 myocarditis should be diagnosed within an appropriate clinical context and should not be based on isolated imaging findings. Therapies in COVID-19 have focused on the major clinical manifestation of pneumonia, but the promotion of viral clearance early in the disease could prevent the development of myocarditis, and further study of immunosuppressive therapies once myocarditis has developed are indicated. A strict and uniform approach is needed to diagnose myocarditis due to SARS-CoV-2 to better understand the natural history of this disease and to facilitate evaluation of potential therapeutic interventions. A methodological approach will also better inform the incidence of COVID-19 associated myocarditis and potential long-term health effects.
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Affiliation(s)
- Ossama K. Abou Hassan
- Section of Cardiovascular Imaging, Robert and Suzanne Tomsich Department of Cardiovascular Medicine, Sydell and Arnold Miller Family Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH 44195 USA
| | - Calvin C. Sheng
- Section of Cardiovascular Imaging, Robert and Suzanne Tomsich Department of Cardiovascular Medicine, Sydell and Arnold Miller Family Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH 44195 USA
| | - Tom Kai Ming Wang
- Section of Cardiovascular Imaging, Robert and Suzanne Tomsich Department of Cardiovascular Medicine, Sydell and Arnold Miller Family Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH 44195 USA
| | - Paul C. Cremer
- Section of Cardiovascular Imaging, Robert and Suzanne Tomsich Department of Cardiovascular Medicine, Sydell and Arnold Miller Family Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH 44195 USA
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8
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Zarifa A, Kim JW, Lopez-Mattei J, Palaskas N, Iliescu C, Kim PY. Cardiac Toxicities Associated with Immune Checkpoints Inhibitors: Mechanisms, Manifestations and Management. Korean Circ J 2021; 51:579-597. [PMID: 34227272 PMCID: PMC8263294 DOI: 10.4070/kcj.2021.0089] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/14/2021] [Revised: 05/02/2021] [Accepted: 05/07/2021] [Indexed: 12/26/2022] Open
Abstract
Immune checkpoint inhibitor (ICI) associated cardiovascular adverse events (CVAE) have become more frequent with the growing use of cancer immunotherapy. CVAEs include a wide spectrum of diseases such as myocarditis, pericarditis, heart failure, arrhythmias, coronary artery disease, and hypertension. The induction of cardiovascular side effects by ICI use is hypothesized to occur due to inflammation and immune dysregulation of normal tissue in response to immunotherapy. Management of ICI-associated CVAEs mitigates an overactive immune response by utilizing steroids, immunomodulatory drugs and hemodynamic stabilization. However, few controlled studies on the cardiovascular safety of ICIs exist and treatment of their side effects are mostly from limited case series. Our review seeks to provide the most recent understanding of ICI-associated CVAEs and their management.
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Affiliation(s)
- Abdulrazzak Zarifa
- Department of Medicine, Brookdale Hospital University and Medical Center, Brooklyn, NY, USA
| | - Jin Wan Kim
- Department of Medicine, University of Texas Health Science Center at Houston, Houston, TX, USA
| | - Juan Lopez-Mattei
- Department of Cardiology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
| | - Nicolas Palaskas
- Department of Cardiology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
| | - Cezar Iliescu
- Department of Cardiology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
| | - Peter Y Kim
- Department of Cardiology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
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Shah Z, Mohammed M, Vuddanda V, Ansari MW, Masoomi R, Gupta K. National Trends, Gender, Management, and Outcomes of Patients Hospitalized for Myocarditis. Am J Cardiol 2019; 124:131-136. [PMID: 31060730 DOI: 10.1016/j.amjcard.2019.03.036] [Citation(s) in RCA: 35] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/18/2019] [Revised: 03/14/2019] [Accepted: 03/19/2019] [Indexed: 12/31/2022]
Abstract
Myocarditis is a major cause of acute and chronic cardiomyopathy. Data on patient characteristics utilization of healthcare, and outcomes of myocarditis-related hospitalizations are limited. We sought to analyze the outcomes of patients hospitalized with myocarditis from a large diverse, multicentric, nationwide cohort using Nationwide Inpatient Sample database. A total of 27,129 hospitalizations involving adult patients (age ≥ 18 years) with the primary discharge diagnosis of myocarditis from years 2007 through 2014 were included and patients who had diagnosis of myocardial infarction or coronary syndromes (including unstable angina) during the same hospitalization were excluded. More men were hospitalized compared with women (66% vs 34%, p <0.05). Patients hospitalized were young with a mean age of 37.3 ± 18.8 years with women being older compared with men (45.2 ± 20.9 vs 33.2 ± 16.2, p <0.001). In-hospital complications of cardiogenic shock and ventricular fibrillation/cardiac arrest occurred in 6.5% and 2.5% of hospitalizations, respectively, with females being affected significantly more than males (10.2% vs 4.6%; 3.6% vs 2%, respectively, p <0.001 for both comparisons). A total of 640 (2.4%) patients died during index hospitalization. Mortality was significantly higher in females compared with males (3.5% vs 1.8%; p <0.001). Multiple logistic regression analysis demonstrated female gender as an independent predictor of in-hospital mortality (odds ratio: 1.69, 95% confidence interval: 1.1 to 2.6; p = 0.007). In conclusion, myocarditis-related hospitalizations have increased during the study years and mostly affect young population with no significant co-morbidities. Female gender remains at high risk for myocarditis-related complications and in-hospital mortality.
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10
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Histopathological and Morphometric Study of Fibrosis and Nuclear Pleomorphism in Dilated Cardiomyopathy. CURRENT HEALTH SCIENCES JOURNAL 2019; 45:73-78. [PMID: 31297266 PMCID: PMC6592661 DOI: 10.12865/chsj.45.01.10] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 02/03/2018] [Accepted: 01/21/2019] [Indexed: 11/23/2022]
Abstract
Histopathological changes associated with dilated cardiomyopathy (CMD) are frequently nonspecific and often only present in the terminal stage of the disease. The study followed the histopathological and morphometric quantification of fibrosis and nuclear pleomorphism in CMD. We analyzed left ventricle myocardial fragments harvested during autopsy, from 35 cases with clinical diagnosis of CMD and 5 cases of normal myocardium. Fibrosis was present in all CMD cases, with higher values compared with control cases. Nuclear pleomorphism was identified in 18 cases (45%), two of the analyzed parameters, respectively the ratio of nuclear diameters and roundness of nucleus, revealing significant differences in CMD compared to the control cases. Myocardial fibrosis present in all cases of CMD represents a major feature of the disease. The nuclear pleomorphism due to the nuclei change in diameters and size was more pronounced in the vicinity of fibrosis areas, possibly related to this alteration.
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11
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Toce MS, Farias M, Powell AJ, Daly KP, Vargas SO, Burns MM. Myocardial Infarct After Marijuana Inhalation in a 16-year-old Adolescent Boy. Pediatr Dev Pathol 2019; 22:80-86. [PMID: 29958511 DOI: 10.1177/1093526618785552] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Many reports of marijuana-associated myocardial infarct (MI) are limited by incomplete evaluation of the toxicologic exposure, a lack of definitive anatomic findings, and the potential for comorbid coronary atherosclerosis inherent in an adult population. We report a 16-year-old adolescent boy who presented with chest pain after smoking marijuana and was found to have acute MI. Electrocardiogram showed diffuse ST-segment elevations. Exhaustive toxicologic testing confirmed the presence of Δ-9-tetrahydrocannabinol metabolite and ruled out other drugs of abuse. Echocardiography demonstrated moderate global left ventricular systolic dysfunction. Coronary angiography demonstrated no focal coronary lesions or obstruction. Right ventricular septal endomyocardial samples biopsied 36 hours after the onset of pain showed a subendocardial acute MI with a sparse neutrophilic infiltrate. One month after the event, magnetic resonance imaging showed a severely dilated left ventricle and moderately to severely depressed global systolic function. Late gadolinium enhancement consistent with myocardial fibrosis was seen in nearly all myocardial segments. Our unusually well-documented findings strengthen the potential association between marijuana and MI. Furthermore, we demonstrate a disease distribution supporting a process that affects the coronary circulation globally, likely at the distal, small-vessel level.
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Affiliation(s)
- Michael S Toce
- 1 Harvard Medical Toxicology Program, Boston Children's Hospital, Boston, Massachusetts.,2 Division of Emergency Medicine, Department of Medicine, Boston Children's Hospital, Boston, Massachusetts
| | - Michael Farias
- 3 Department of Cardiology, The Heart Center, Arnold Palmer Hospital for Children, Orlando, Florida
| | - Andrew J Powell
- 4 Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts
| | - Kevin P Daly
- 4 Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts
| | - Sara O Vargas
- 5 Department of Pathology, Boston Children's Hospital, Boston, Massachusetts
| | - Michele M Burns
- 1 Harvard Medical Toxicology Program, Boston Children's Hospital, Boston, Massachusetts.,2 Division of Emergency Medicine, Department of Medicine, Boston Children's Hospital, Boston, Massachusetts
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12
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Sudden death following endomyocardial biopsy of cardiac metastasis from malignant melanoma. Forensic Sci Med Pathol 2016; 12:217-9. [DOI: 10.1007/s12024-016-9755-3] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/09/2016] [Indexed: 02/06/2023]
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Pollack A, Kontorovich AR, Fuster V, Dec GW. Viral myocarditis--diagnosis, treatment options, and current controversies. Nat Rev Cardiol 2015; 12:670-80. [PMID: 26194549 DOI: 10.1038/nrcardio.2015.108] [Citation(s) in RCA: 402] [Impact Index Per Article: 40.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/08/2023]
Abstract
Myocarditis--a frequent cause of dilated cardiomyopathy and sudden cardiac death--typically results from cardiotropic viral infection followed by active inflammatory destruction of the myocardium. Characterization of this disease has been hampered by its heterogeneous clinical presentations and diverse aetiologies. Advances in cardiac MRI and molecular detection of viruses by endomyocardial biopsy have improved our ability to diagnose and understand the pathophysiological mechanisms of this elusive disease. However, therapeutic options are currently limited for both the acute and chronic phases of myocarditis. Several randomized, controlled trials have demonstrated potential benefit with immunosuppressive and immunomodulatory therapies, but further investigations are warranted. In this Review, we explore the pathophysiology, natural history, and modes of diagnosis of myocarditis, as well as evidence-based treatment strategies. As novel imaging techniques and human in vitro models of the disease emerge, the landscape of therapies for myocarditis is poised to improve.
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Affiliation(s)
- Ari Pollack
- Zena and Michael A. Wiener Cardiovascular Institute, Icahn School of Medicine at Mount Sinai, 1 Gustave L. Levy Place, New York, NY 10029, USA
| | - Amy R Kontorovich
- Zena and Michael A. Wiener Cardiovascular Institute, Icahn School of Medicine at Mount Sinai, 1 Gustave L. Levy Place, New York, NY 10029, USA
| | - Valentin Fuster
- Zena and Michael A. Wiener Cardiovascular Institute, Icahn School of Medicine at Mount Sinai, 1 Gustave L. Levy Place, New York, NY 10029, USA
| | - G William Dec
- Cardiology Division, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA
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Kupsky DF, Tweet MS, Anavekar NS. 30-year-old man with chest pain and nausea. Mayo Clin Proc 2014; 89:1579-83. [PMID: 25444489 DOI: 10.1016/j.mayocp.2014.03.020] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/30/2014] [Revised: 03/13/2014] [Accepted: 03/17/2014] [Indexed: 11/19/2022]
Affiliation(s)
- Daniel F Kupsky
- Resident in Internal Medicine, Mayo School of Graduate Medical Education, Mayo Clinic, Rochester, MN
| | - Marysia S Tweet
- Fellow in Cardiovascular Diseases, Mayo School of Graduate Medical Education, Mayo Clinic, Rochester, MN
| | - Nandan S Anavekar
- Adviser to resident and Consultant in Cardiovascular Diseases, Mayo Clinic, Rochester, MN.
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Abstract
Myocarditis is most often caused by a viral infection. Less common causes include other infectious agents and autoimmune diseases. Fulminant myocarditis is an unusual complication with a rapidly progressive course resulting in severe heart failure and cardiogenic shock. Fulminant myocarditis should be treated with full supportive care, using aggressive pharmacologic therapy and mechanical circulatory support, because significant improvement in left ventricular function will often occur. Cardiac transplantation is required in a small minority of patients. Cardiac magnetic resonance imaging is becoming a frequently used modality to aid in the diagnosis of myocarditis.
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Affiliation(s)
- Fredric Ginsberg
- Division of Cardiovascular Medicine, Cooper University Hospital, One Cooper Plaza, Camden, NJ 08103, USA.
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Yoshida A, Ishibashi-Ueda H, Yamada N, Kanzaki H, Hasegawa T, Takahama H, Amaki M, Asakura M, Kitakaze M. Direct comparison of the diagnostic capability of cardiac magnetic resonance and endomyocardial biopsy in patients with heart failure. Eur J Heart Fail 2013; 15:166-75. [PMID: 23329703 DOI: 10.1093/eurjhf/hfs206] [Citation(s) in RCA: 61] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
AIMS The diagnostic performance of cardiac magnetic resonance (CMR) has not been compared with that of other imaging modalities. Therefore, this study investigated the diagnostic capabilities of CMR and endomyocardial biopsy (EMB) in patients with heart failure (HF). METHODS AND RESULTS We studied 136 patients with cardiomyopathy who underwent both CMR and EMB. Independent diagnoses were made according to the results of (i) CMR alone; (ii) EMB alone; (iii) clinical data plus echocardiogram; (iv) clinical data, echocardiogram, plus CMR; and (v) clinical data, echocardiogram, plus EMB. These diagnoses were then compared with the final diagnosis (gold standard) that was made using the complete clinical data, including EMB and CMR. The sensitivities of the diagnosis strategies of (i-v) relative to the final diagnosis were 67, 79, 86, 97, and 100%, respectively. CMR alone demonstrated better sensitivity for cardiac sarcoidosis and greater specificity for dilated cardiomyopathy than EMB alone. CMR also tended to show better sensitivity for hypertensive heart disease. There was no difference between the diagnostic capability of CMR and EMB for hypertrophic cardiomyopathy (HCM). However, CMR showed excellent sensitivity (100%) for apical and obstructive HCM, whereas EMB displayed better sensitivity for dilated HCM. Moreover, combined diagnosis with clinical data, echocardiogram, plus CMR achieved superior agreement with the final diagnosis in comparison with EMB alone. CONCLUSION Non-invasive CMR demonstrated excellent diagnostic capability for patients with HF and was as effective as or superior to EMB. In particular, the use of CMR in combination with clinical data unrelated to EMB may provide excellent diagnostic accuracy for HF.
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Affiliation(s)
- Akemi Yoshida
- Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan
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Saji T, Matsuura H, Hasegawa K, Nishikawa T, Yamamoto E, Ohki H, Yasukochi S, Arakaki Y, Joo K, Nakazawa M. Comparison of the clinical presentation, treatment, and outcome of fulminant and acute myocarditis in children. Circ J 2012; 76:1222-8. [PMID: 22307381 DOI: 10.1253/circj.cj-11-1032] [Citation(s) in RCA: 70] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Abstract
BACKGROUND Myocarditis (MC) is an important cause of cardiac dysfunction in children. Fulminant MC is sometimes fatal, and sequelae may develop during follow-up. We conducted a nationwide survey to determine the clinico-epidemiological features of MC in Japanese children and adolescents. METHODS AND RESULTS Survey questionnaires were mailed to 627 hospitals, which were asked if they had treated MC patients aged between 1 month and 17 years during the period from January 1997 through December 2002. Responses were collected until December 2005, and data were collected and analyzed until January 2008. A total of 169 patients were reported: 64 fulminant cases, 89 acute cases, and 8 chronic cases. Incidence was 43.5 cases/year and 0.26 cases/100,000. Pathogens were identified in 37 patients; coxsackie virus accounted for 60%. Major cardiovascular manifestations at onset were congestive heart failure, refractory arrhythmia, and syncope in 70, 37, and 17 patients, respectively. Intravenous immunoglobulin was administered to 73 patients. Mechanical support seemed to be effective and life-saving. Among the 169 patients, 123 survived. Cardiovascular sequelae were reported in 49 patients. CONCLUSIONS The survival rate for children with fulminant MC was disappointing. Overall, two-thirds of survivors had no sequelae. Mechanical support may reduce the mortality and the risk of clinical worsening.
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Affiliation(s)
- Tsutomu Saji
- Department of Pediatrics, Toho University Medical Center, Omori Hospital, 6-11-1 Omori-Nishi, Ota-ku, Tokyo 143-8541, Japan.
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18
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Endomyocardial biopsy in recent dilated cardiomyopathy - evaluation of baseline characteristics of the first 50 patients. COR ET VASA 2011. [DOI: 10.33678/cor.2011.157] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022]
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Kumar K, Guirgis M, Zieroth S, Lo E, Menkis AH, Arora RC, Freed DH. Influenza myocarditis and myositis: case presentation and review of the literature. Can J Cardiol 2011; 27:514-22. [PMID: 21652168 DOI: 10.1016/j.cjca.2011.03.005] [Citation(s) in RCA: 55] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/06/2010] [Accepted: 02/18/2011] [Indexed: 11/26/2022] Open
Abstract
Myocarditis, a general inflammatory condition of the heart muscle, can result from a variety of etiologies, the most common being viral. Despite common pathogens, concomitant myocarditis and myositis remains a rare event. Although a common cause of respiratory illness, extrapulmonary infections with influenza are infrequent. We describe the case of a patient who presented to our centre with concomitant "seasonal" H1N1 influenza A myocarditis further complicated by pan-myositis. The patient's condition rapidly declined, eventually requiring biventricular mechanical support, in addition to multilimb fasciotomies. The cardiac support required was progressive, from a percutaneous left ventricular assist device, to extracorporeal membrane oxygenation, to eventual biventricular assist device support for bridge-to-transplantation. This case motivated a detailed review of the literature (a total of 29 cases were identified), in which we found that patients with influenza myocarditis/myositis were predominantly female (63%) and young (mean age 33.2 years) and continue to have a high incidence of morbidity and mortality (27%). As a result of its atypical pattern, the 2009 H1N1 pandemic strain has gained attention. From our review, we found 7 patients with of 2009 H1N1 pandemic influenza myocarditis. Serial serum cytokine analysis did not demonstrate a "cytokine storm," which has been associated with other virulent influenza strains. The PB1-F2 marker in particular has been associated with a vigorous cytokine response. The 2009 H1N1 and "seasonal" influenza strains lack this marker. In those patients with community-acquired influenza, interleukin-6 has been shown to correlate with symptoms. For patients with myocarditis resulting in shock, mechanical circulatory support has gained acceptance as a means to recovery or transplantation.
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Affiliation(s)
- Kanwal Kumar
- Institute of Cardiovascular Sciences, St. Boniface Research Centre, Winnipeg, Manitoba, Canada
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20
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Ultrasound-guided serial transabdominal cardiac biopsies in cats. Vet J 2011; 191:341-6. [PMID: 21498093 DOI: 10.1016/j.tvjl.2011.02.022] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2010] [Revised: 01/18/2011] [Accepted: 02/24/2011] [Indexed: 11/24/2022]
Abstract
A percutaneous/transdiaphragmatic core needle biopsy technique was developed in cats to obtain serial biopsies from different locations of the left ventricle, through which morphological and molecular changes within the same individual can be studied to unravel the mechanisms of feline cardiomyopathies. Transmural left ventricular myocardial samples were obtained from 29 anesthetized, healthy, adult cats with ultrasound guidance. An 18G automatic biopsy needle was inserted between the last left rib and the sternum through the diaphragm into the thorax. Biopsies were obtained from the left ventricular wall. In five cats, three single biopsies were taken with 4-week intervals. Autopsy was performed on six cats, of which three cats had serial biopsies. In total, 87 biopsies were obtained without long-term effects on cardiac function or structure. The biopsies caused transient single ventricular premature complexes and mild pericardial effusion without tamponade. Necropsy revealed a minimal amount of fibrous connective tissue in the diaphragm and the heart without any significant microscopic lesions in the adjacent muscle tissue. The high quality biopsy material was suitable for morphological and molecular studies. This minimally invasive, ultrasound-guided cardiac biopsy technique thus allows for the safe collection of serial biopsies to study feline cardiomyopathies in an experimental setting.
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Kobayashi Y, Giles JT, Hirano M, Yokoe I, Nakajima Y, Bathon JM, Lima JAC, Kobayashi H. Assessment of myocardial abnormalities in rheumatoid arthritis using a comprehensive cardiac magnetic resonance approach: a pilot study. Arthritis Res Ther 2010; 12:R171. [PMID: 20836862 PMCID: PMC2990998 DOI: 10.1186/ar3131] [Citation(s) in RCA: 79] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/24/2009] [Revised: 12/27/2009] [Accepted: 09/13/2010] [Indexed: 01/18/2023] Open
Abstract
Introduction Rheumatoid arthritis (RA) is a multi-organ inflammatory disorder associated with high cardiovascular morbidity and mortality. We sought to assess cardiac involvement using a comprehensive cardiac magnetic resonance imaging (cMRI) approach and to determine its association with disease characteristics in RA patients without symptomatic cardiac disease. Methods RA patients with no history and/or clinical findings of systemic or pulmonary hypertension, coronary artery disease, severe valvular heart disease, atrial fibrillation, diabetes mellitus, or echocardiographic abnormalities underwent contrast-enhanced cMRI on a 1.5T scanner. Adenosine triphosphate was used to assess perfusion defects due to microvascular impairment or ischemia, and delayed enhanced imaging was obtained for the assessment of myocardial inflammation/fibrosis. We explored the associations of cMRI abnormalities with RA disease activity and severity measures. Results Eighteen patients (78% female) with a mean age of 57 ± 10 years were studied. Eight patients (45%) demonstrated a myocardial abnormality. Perfusion defects under pharmacologic stress were seen in two patients (11%), one of whom had a circumferential subendocardial perfusion defect and one had a non-segmental subendocardial perfusion defect. Seven patients (39%) were found to have delayed enhancement, only one of whom also demonstrated a perfusion defect. Mean disease activity score (DAS)28 was significantly higher in the group with delayed enhancement compared to the group without by an average of 1.32 DAS28 units (4.77 vs. 3.44 units, respectively; P = 0.011). Corresponding trends to statistical significance were noted in systemic inflammatory markers, with both C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) quantitatively higher in the group with delayed enhancement. Other RA characteristics, such as disease duration, autoantibody status, and current treatments were not significantly associated with cardiac involvement. Conclusions Myocardial abnormalities, as detected by cMRI, were frequent in RA patients without known cardiac disease. Abnormal cMRI findings were associated with higher RA disease activity, suggesting a role for inflammation in the pathogenesis of myocardial involvement in RA.
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Affiliation(s)
- Yasuyuki Kobayashi
- Department of Radiology, St Marianna University School of Medicine, 2-16-1 Sugao, Miyamae-ku, Kawasaki, Kanagawa 216-8511, Japan.
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Luk A, Metawee M, Ahn E, Gustafsson F, Ross H, Butany J. Do clinical diagnoses correlate with pathological diagnoses in cardiac transplant patients? The importance of endomyocardial biopsy. Can J Cardiol 2009; 25:e48-54. [PMID: 19214301 DOI: 10.1016/s0828-282x(09)70484-2] [Citation(s) in RCA: 30] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022] Open
Abstract
BACKGROUND Heart transplantation remains the last treatment option for patients with end-stage cardiac disease. Such diseases include ischemic cardiomyopathy, nonischemic cardiomyopathy and other conditions such as arrhythmogenic right ventricular dysplasia, cardiac sarcoidosis and cardiac amyloidosis. OBJECTIVE To review the changes that have occurred over time in the etiology of heart disease in patients requiring heart transplantation, and to compare the clinical and histological diagnoses of explanted hearts from patients with progressive cardiac disease. METHODS The pathological findings of 296 surgically excised hearts over a 20-year period (January 1987 to July 2006) at one institution were examined. Patients were separated into groups based on year of heart transplantation. The tissue was examined to determine the underlying cardiac pathology leading to congestive heart failure. Patient records were reviewed for preoperative clinical diagnoses and other relevant data, including pretransplant endomyocardial biopsy (EMB) results, information regarding left ventricular assist devices and, finally, evidence of disease recurrence in the grafted heart. RESULTS A shift in the underlying etiology was found in patients who underwent heart transplantation from 1992 to 1996, and 1997 to 2001. Between 1987 and 1997, the majority of transplant cases consisted of ischemic cardiomyopathies. From 1997 to 2001, the majority of patients had nonischemic cardiomyopathies, and this trend continued to 2006. A majority of patients with ischemic and hypertrophic cardiomyopathy were diagnosed correctly (96.5% and 82%, respectively) before transplantation. Most patients diagnosed post-transplant with lymphocytic (viral, 15%), hypersensitive/ eosinophilic (25%) and giant cell (100%) myocarditis, arrhythmogenic right ventricle dysplasia (100%), cardiac sarcoidosis (83%) and iron overload toxicity- associated cardiomyopathy (100%) had been misdiagnosed in pretransplantation investigations. Investigations before transplantation did not include an EMB. Of all 296 patients, 51 patients (17%) were misdiagnosed. Excluding the patients with ischemic cardiomyopathy, 46 of 152 patients (30%) were misdiagnosed before transplantation. CONCLUSIONS Although cardiac transplantation is a viable treatment option for patients with a variety of cardiac diseases, accurate diagnosis of patients before transplantation remains a priority. Accurate diagnosis of particular diseases (sarcoidosis, myocarditis, iron toxicity-associated cardiomyopathy and others) allows for proper treatment before transplantation, which may slow down disease progression and improve patient outcomes. Furthermore, it is important to accurately diagnose patients with diseases such as sarcoidosis, amyloidosis and particular types of myocarditis because these can readily recur in the grafted heart. The risk for recurrence must be known to practitioners and, most importantly, to the patient. We strongly recommend the use of EMB if a nonischemic cardiomyopathy is suspected, because the results may alter the diagnosis and modify the treatment strategy.
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Affiliation(s)
- Adriana Luk
- Department of Pathology, Toronto General Hospital/University Health Network, Toronto, Ontario
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25
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Kochar M, López-Candales A, Ramani G, Rajagopalan N, Edelman K. Unusual echocardiographic features seen in a case of giant cell myocarditis. Can J Cardiol 2008; 24:855-6. [PMID: 18987760 DOI: 10.1016/s0828-282x(08)70195-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
Abstract
The case of an 18-year-old college football player with a recent history of streptococcal pharyngitis who was experiencing progressive disabling dyspnea on exertion with easy fatigability and lack of stamina, and was taken to the hospital after a syncopal episode is described. The patient was initially diagnosed with heart failure and treated accordingly. However, because of a fulminant clinical deterioration, an endomyocardial biopsy was recommended, which showed focal giant cell transformation consistent with giant cell myocarditis. Treatment with methylprednisolone and cyclosporine was promptly initiated. Several apical clots were noted during treatment, but the patient attained full recovery with treatment.
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Affiliation(s)
- Minisha Kochar
- Department of Medicine, University of Pittsburgh, Pennsylvania, USA
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27
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Abstract
Cardiovascular manifestations of Lyme disease were first reported nearly 30 years ago. This article describes Lyme carditis, its epidemiology, pathophysiology, methods of diagnosis, and treatment options.
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Affiliation(s)
- Airley E Fish
- Division of Cardiology, Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USA
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28
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Imazio M, Trinchero R. Myopericarditis: Etiology, management, and prognosis. Int J Cardiol 2008; 127:17-26. [PMID: 18221804 DOI: 10.1016/j.ijcard.2007.10.053] [Citation(s) in RCA: 145] [Impact Index Per Article: 8.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/30/2007] [Revised: 09/19/2007] [Accepted: 10/27/2007] [Indexed: 12/16/2022]
Abstract
Acute pericarditis is often accompanied by some degree of myocarditis. In clinical practice both pericarditis and myocarditis coexist because they share common etiologic agents, mainly cardiotropic viruses. The term "myopericarditis" indicates a primarily "pericarditic syndrome" and it is responsible for the majority of cases. The clinical presentation is varied, reflecting the variability of myocardial involvement, that may be focal or diffuse, affecting any or all cardiac chambers. Probably many cases may be subclinical and subtle cardiac symptoms and signs may be overshadowed by the systemic manifestations of the viral infection. Echocardiography is essential for the diagnosis of left ventricular dysfunction in even subclinical cases and for follow-up of patients with apparently normal left ventricular function. Magnetic resonance imaging holds promise for an effective non-invasive diagnostic tool. Either for acute pericarditis or myopericarditis there is a lack for adequate controlled clinical trials. In myopericarditis the use of NSAID should be cautious, because in animal models of myocarditis, NSAID are not effective and may actually enhance the myocarditic process and increase mortality. In clinical practice lower anti-inflammatory doses are mainly considered to control symptoms. The natural history of myopericarditis in large populations is not known with accuracy. On follow-up, the majority of these cases had objective normalization of echocardiography, electrocardiography, laboratory testing, and functional status, although up to 14% may report atypical, non-limiting chest discomfort. Unfortunately, few data have been published on myopericarditis, the paper reviews current available evidence on the presentation, management, and prognosis of myopericarditis.
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Affiliation(s)
- Massimo Imazio
- Cardiology Department, Maria Vittoria Hospital, Via Cibrario 72, 10141 Torino, Italy.
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29
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Oh SS, Youn HJ, Park JH, Lee DH, Choi YS, Park CS, Oh YS, Chung WS, Hong SJ. Endomyocardial Biopsy: One Center's Report About Its Role. Korean Circ J 2008. [DOI: 10.4070/kcj.2008.38.7.374] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022] Open
Affiliation(s)
- Su-Sung Oh
- Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Ho-Joong Youn
- Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Jeong-Hwan Park
- Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Dong-Hyun Lee
- Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Yun-Seok Choi
- Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Chul-Soo Park
- Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Yong-Seog Oh
- Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Wook-Sung Chung
- Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Soon-Jo Hong
- Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
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Abstract
Recently, there has been an increased recognition of the association of clozapine with myocarditis and myocardiopathy. Commonly used diagnostic tests have very limited sensitivity in diagnosing this potentially life-threatening complication. We present 3 case reports of clozapine-induced myocarditis/cardiomyopathy that illustrate the development of a combined approach involving a clinical questionnaire and diagnostic testing at our hospital. This combination approach helped in the early recognition and successful treatment of clozapine myocarditis in one of our patients. Given the increasing recognition of this adverse reaction, we felt it timely to report our experience in diagnosing and treating this clinical syndrome.
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Abstract
Myocarditis represents a heterogeneous final common pathway for myocardial inflammation of diverse etiologies and accounts for up to one-third of cases of dilated cardiomyopathy. The pathophysiology of viral myocarditis can be disaggregated into the effects of direct viral mediated injury, triggered acute and chronic autoimmune responses, and subsequent adverse remodeling. Recent research highlights the pathogenic role of persistent viral genome expression, Fas-ligand, tumor necrosis factor-alpha receptor 1, and antimyosin autoantibodies in the evolution of chronic systolic and diastolic heart failure. Recent refinements in endomyocardial biopsy evaluation, cardiac magnetic resonance imaging, and cytokine assays augment existing diagnostic modalities. Novel specific immunosuppressive targets aimed at interrupting myocyte injury and apoptosis, including interferon-beta seem promising to date in small clinical studies performed on selected patients.
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Affiliation(s)
- Christopher R Ellis
- From the Vanderbilt Heart and Vascular Institute, Vanderbilt University Medical Center, Nashville, Tennessee 37232-8802, USA
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32
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Lewis GD, Holmes CB, Holmvang G, Butterton JR. Case records of the Massachusetts General Hospital. Case 8-2007. A 48-year-old man with chest pain followed by cardiac arrest. N Engl J Med 2007; 356:1153-62. [PMID: 17360994 DOI: 10.1056/nejmcpc079002] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/19/2022]
Affiliation(s)
- Gregory D Lewis
- Cardiology Division, Department of Medicine, Massachusetts General Hospital, USA
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Han J, Park Y, Lee H, Kang H, Kim H, Yang DH, Park HS, Cho Y, Chae SC, Jun JE, Park WH. Complications of 2-D echocardiography guided transfemoral right ventricular endomyocardial biopsy. J Korean Med Sci 2006; 21:989-94. [PMID: 17179674 PMCID: PMC2721952 DOI: 10.3346/jkms.2006.21.6.989] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/05/2022] Open
Abstract
Endomyocardial biopsy (EMBx) is a useful tool for diagnosing various cardiac pathologies. However, the routine use of EMBx has not gained widespread acceptance due to the possible complications related to the EMBx. Thus, not much information is available on the complications related to the EMBx. We prospectively evaluated 90 consecutive patients who underwent 2-D echocardiography guided transfemoral right ventricular EMBx at Kyungpook National University Hospital between March 2002 and November 2005 to determine the incidence, nature and subsequent management of complications related to EMBx. The clinical diagnoses before the EMBx were arrhythmogenic right ventricular dysplasia in 54, dilated cardiomyopathy in 19, Brugada syndrome in 9, myocarditis in 6 and miscellaneous in 2 patients. The overall major complication rate was 5.6% and no procedure-related mortality occurred. Myocardial perforation (n=3), which was the most frequent complication, did not progress to cardiac tamponade requiring pericardiocentesis in any patient. Hemodynamically unstable ventricular tachycardia occurred in 1 patient. New and persistent right bundle branch block occurred in another. Our findings suggest that 2-D echocardiography guided transfemoral right ventricular EMBx is a relatively safe procedure.
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Affiliation(s)
- Juyup Han
- Department of Internal Medicine, Kyungpook National University Hospital, Daegu, Korea
| | - Yongwhi Park
- Department of Internal Medicine, Kyungpook National University Hospital, Daegu, Korea
| | - Hyunsang Lee
- Department of Internal Medicine, Kyungpook National University Hospital, Daegu, Korea
| | - Hyunjae Kang
- Department of Internal Medicine, Kyungpook National University Hospital, Daegu, Korea
| | - Hyungseop Kim
- Department of Internal Medicine, Kyungpook National University Hospital, Daegu, Korea
| | - Dong Heon Yang
- Department of Internal Medicine, Kyungpook National University Hospital, Daegu, Korea
| | - Hun Sik Park
- Department of Internal Medicine, Kyungpook National University Hospital, Daegu, Korea
| | - Yongkeun Cho
- Department of Internal Medicine, Kyungpook National University Hospital, Daegu, Korea
| | - Shung-Chull Chae
- Department of Internal Medicine, Kyungpook National University Hospital, Daegu, Korea
| | - Jae-Eun Jun
- Department of Internal Medicine, Kyungpook National University Hospital, Daegu, Korea
| | - Wee-Hyun Park
- Department of Internal Medicine, Kyungpook National University Hospital, Daegu, Korea
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Tepe SM, Glockner JF. A Case of Acute Myocarditis with Evaluation of Magnetic Resonance Imaging. Int J Cardiovasc Imaging 2006; 23:233-5. [PMID: 16847738 DOI: 10.1007/s10554-006-9118-3] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/16/2005] [Accepted: 06/05/2006] [Indexed: 01/01/2023]
Abstract
A 42-year-old male with acute chest pain referred for acute myocarditis versus myocardial infarction (MI) was examined with magnetic resonance imaging (MRI). Clinical presentation and MRI findings are reviewed.
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Affiliation(s)
- Savas M Tepe
- Department of Radiology, Mayo Clinic, 200 First Street Southwest, Rochester, MN 55905, USA.
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35
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Affiliation(s)
- Jared W Magnani
- Cardiology Division, Massachusetts General Hospital, Boston, Massachusetts, USA
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36
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Isbell DC, Kramer CM. The Evolving Role of Cardiovascular Magnetic Resonance Imaging in Nonischemic Cardiomyopathy. Semin Ultrasound CT MR 2006; 27:20-31. [PMID: 16562569 DOI: 10.1053/j.sult.2005.11.002] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
Over the last several years, the role of cardiac magnetic resonance imaging in the diagnosis and management of heart failure has been rapidly expanding. The techniques unrivaled flexibility, accuracy in defining ventricular structure and function, and capacity to characterize tissue makes it particularly well suited for the study of the nonischemic cardiomyopathies. In this article, we provide an overview of the existing literature highlighting the diagnostic utility and prognostic power of cardiac magnetic resonance imaging in the nonischemic cardiomyopathies.
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Affiliation(s)
- David C Isbell
- Department of Medicine, University of Virginia Health System, Charlottesville, VA 22908, USA.
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Cooper LT, Virmani R, Chapman NM, Frustaci A, Rodeheffer RJ, Cunningham MW, McNamara DM. National Institutes of Health-sponsored workshop on inflammation and immunity in dilated cardiomyopathy. Mayo Clin Proc 2006; 81:199-204. [PMID: 16471075 DOI: 10.4065/81.2.199] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
Abstract
Nonischemic dilated cardiomyopathy (DCM) is an uncommon cause of heart failure but has widespread importance because it is the cause of 45% of heart transplantations. Multiple experimental and clinical lines of evidence have implicated altered immunity in the pathogenesis of DCM. However, advances in the understanding of the mechanisms of altered immunity have not affected the diagnosis or treatment of DCM. In recognition of this problem, the National Institutes of Health sponsored an expert workshop with 2 aims: to review the current understanding of inflammation and immunity as they relate to DCM and to identify the most promising areas for future clinical research efforts in the field. This report summarizes the scientific opportunities, perceived needs and barriers, and workshop recommendations on research directions in DCM. The major recommendations from the members of the workshop are organized according to the following themes: cardiotropic viruses, innate and acquired immune responses, environmental factors, novel diagnostics, and novel therapeutics.
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Affiliation(s)
- Leslie T Cooper
- Division of Cardiovascular Diseases, Mayo Clinic College of Medicine, 200 First St SW, Rochester, MN 55905, USA.
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Magnani JW, Danik HJS, Dec GW, DiSalvo TG. Survival in biopsy-proven myocarditis: a long-term retrospective analysis of the histopathologic, clinical, and hemodynamic predictors. Am Heart J 2006; 151:463-70. [PMID: 16442915 DOI: 10.1016/j.ahj.2005.03.037] [Citation(s) in RCA: 119] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/14/2004] [Accepted: 03/28/2005] [Indexed: 01/25/2023]
Abstract
OBJECTIVE We hypothesized that histopathology predicts survival without cardiac transplantation in patients with biopsy-proven myocarditis. BACKGROUND The role of endomyocardial biopsy in diagnosing myocarditis remains controversial. Histopathology has been integrated with clinical and hemodynamic features to predict prognosis. However, the influence of histopathology on survival > 5 years has not been explored. METHODS We retrospectively identified 112 consecutive patients with histopathologic confirmation of myocarditis. We examined these patients' clinical presentation, hemodynamic assessment, hospital course, and treatment. We selected 14 variables that might influence survival without cardiac transplantation. RESULTS A total of 62 (55%) of 112 patients had lymphocytic myocarditis; 88 (79%) and 63 (56%) were alive without cardiac transplantation at 1 and 5 years, respectively. Median follow-up was a mean 95.5 months and median 74.5 months. Among the 55 with complete data of the 14 candidate predictor variables, age, sex, and clinical presentation with congestive heart failure and ventricular (ventricular tachycardia or fibrillation) or atrial arrhythmias (atrial fibrillation or flutter) did not predict the study end point of death or need for transplantation. In univariate analysis, pulmonary capillary wedge pressure > or = 15 mm Hg significantly predicted the study end point. In multivariate analysis, pulmonary capillary wedge pressure > or = 15 mm Hg and histopathology of lymphocytic, granulomatous, or giant cell myocarditis each significantly predicted mortality or transplant (P = .047, P = .013, and P = .054, respectively) on cumulative survival without cardiac transplantation. CONCLUSIONS Histopathology predicts long-term survival in patients with myocarditis. Clinical presentation, including presentation with congestive heart failure, ventricular tachycardia/ventricular fibrillation, or atrial fibrillation/atrial flutter, does not predict survival without transplantation. Endomyocardial biopsy can play a role in predicting transplant-free survival in patients with myocarditis.
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Affiliation(s)
- Jared W Magnani
- Department of Internal Medicine, Massachusetts General Hospital, Boston, MA, USA.
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Lemaitre F, Close L, Plein D, Silance PG, Vandenbossche JL. [Acute myocarditis: from chest pain to cardiogenic shock]. Ann Cardiol Angeiol (Paris) 2005; 54:97-102. [PMID: 15828465 DOI: 10.1016/j.ancard.2004.09.012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
Abstract
We report the observations of three patients with myocarditis. The first one with chest pain, the second one with a pseudo-infarct presentation and the third one with a cardiogenic shock. We discuss the different anatomo-clinical presentations of myocarditis, the diagnosis, the indications of endomyocardial biopsies and the prognosis of this pathology.
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Affiliation(s)
- F Lemaitre
- Clinique de cardiologie, département de médecine interne, CHU Saint-Pierre, 322, rue Haute, 1000 Bruxelles, Belgique.
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Cassimatis DC, Atwood JE, Engler RM, Linz PE, Eckart RE, Vernalis MN, Grabenstein JD. Reply. J Am Coll Cardiol 2005. [DOI: 10.1016/j.jacc.2004.10.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
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Gami AS, Murphy J, Cooper LT. Cautious interpretation of data regarding myopericarditis associated with smallpox vaccination. J Am Coll Cardiol 2005; 45:160; author reply 160-1. [PMID: 15629393 DOI: 10.1016/j.jacc.2004.10.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
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Law KK, Binder J, Cooper LT, Specks U, Tazelaar HD, Seward JB. Echocardiographic features of atrial myocarditis with giant cells: A case report. J Am Soc Echocardiogr 2004; 17:1073-6. [PMID: 15452474 DOI: 10.1016/j.echo.2004.04.029] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
Abstract
We report a case of restrictive cardiomyopathy in which a distinct endothelial thickening of the atrial wall and pulmonary vein orifices was noted on transesophageal echocardiography. Echocardiographically guided endomyocardial biopsy of the thickening revealed an inflammatory infiltrate that was rich in giant cells and provided important clues about an underlying immune mechanism for the pathogenesis. Positive results from the antineutrophil cytoplasmic autoantibody assay supported the diagnosis of Wegener's granulomatosis. After immunosuppressive therapy, the endomyocardial thickening completely resolved, but the restrictive cardiomyopathy did not reverse.
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Affiliation(s)
- Kwan-K Law
- Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA
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Abstract
The diagnosis of peripartum cardiomyopathy should be considered whenever women present with heart failure during the peripartum period. This cardiomyopathy is distinguished by rapid onset, occurrence in the peripartum period, and significant improvement in up to 50% of affected women. The cause and pathogenesis of this dilated cardiomyopathy remain unknown. Treatment is similar to medical therapy for other forms of dilated cardiomyopathy. Worsening of heart failure may require management in the ICU with support by vasodilators, inotropes, and ventricular assist devices. Patients with severe ventricular dysfunction are less likely to survive and recover normal cardiac function. Subsequent pregnancies may provoke a recurrence, even in patients who apparently recover.
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Affiliation(s)
- Mark Tidswell
- Division of Adult Critical Care, Department of Medicine, Baystate Medical Center, Porter Building, Room 2983, 759 Chestnut Street, Springfield, MA 01199, USA.
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Ardehali H, Qasim A, Cappola T, Howard D, Hruban R, Hare JM, Baughman KL, Kasper EK. Endomyocardial biopsy plays a role in diagnosing patients with unexplained cardiomyopathy. Am Heart J 2004; 147:919-23. [PMID: 15131552 DOI: 10.1016/j.ahj.2003.09.020] [Citation(s) in RCA: 89] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
BACKGROUND The etiology of cardiomyopathy is usually inferred from clinical information and preliminary laboratory studies. Patients with unexplained cardiomyopathy may be referred for endomyocardial biopsy (EMBx). It is unknown whether pathological information obtained from EMBx is beneficial or alters the diagnosis established clinically. This study was undertaken to evaluate the utility of EMBx in confirming or excluding a clinically suspected diagnosis. METHODS We evaluated 845 patients with initially unexplained cardiomyopathy who underwent EMBx between 1982 and 1997 at The Johns Hopkins Hospital. For each patient, an initial clinical diagnosis, an EMBx diagnosis, and a final diagnosis prior to discharge based on all available data were established. RESULTS The final diagnosis differed from the initial clinical diagnosis in 264 (31%) of these patients; EMBx made the diagnosis in 196 (75%) of these cases. Initial diagnoses most frequently altered were myocarditis (34%) and idiopathic cardiomyopathy (25%). Initial diagnoses least likely to be altered were those in which biopsy was used to confirm or grade a previously documented illness, such as hemochromatosis (11%), amyloidosis (18%), or cardiomyopathy secondary to doxorubicin toxicity (0%). EMBx was more sensitive than clinical diagnosis in detecting myocarditis and amyloidosis, and proved to be very specific in detecting ischemic cardiomyopathy, myocarditis, amyloidosis, and hemochromatosis. CONCLUSIONS In patients with unexplained cardiomyopathy after a standard evaluation, the clinical assessment of the etiology is inaccurate in 31% of patients. EMBx establishes the final diagnosis in 75% of these patients with a high degree of specificity.
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Affiliation(s)
- Hossein Ardehali
- Division of Cardiology, Department of Medicine, The Johns Hopkins Hospital, Baltimore, Md, USA.
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Cassimatis DC, Atwood JE, Engler RM, Linz PE, Grabenstein JD, Vernalis MN. Smallpox vaccination and myopericarditis: a clinical review. J Am Coll Cardiol 2004; 43:1503-10. [PMID: 15120802 DOI: 10.1016/j.jacc.2003.11.053] [Citation(s) in RCA: 126] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/11/2003] [Revised: 11/05/2003] [Accepted: 11/13/2003] [Indexed: 11/19/2022]
Abstract
Smallpox is a devastating viral illness that was eradicated after an aggressive, widespread vaccination campaign. Routine U.S. childhood vaccinations ended in 1972, and routine military vaccinations ended in 1990. Recently, the threat of bioterrorist use of smallpox has revived the need for vaccination. Over 450,000 U.S. military personnel received the vaccination between December 2002 and June 2003, with rates of non-cardiac complications at or below historical levels. The rate of cardiac complications, however, has been higher than expected, with two confirmed cases and over 50 probable cases of myopericarditis after vaccination reported to the Department of Defense Smallpox Vaccination Program. The practicing physician should use the history and physical, electrocardiogram, and cardiac biomarkers in the initial evaluation of a post-vaccination patient with chest pain. Echocardiogram, cardiac catheterization, magnetic resonance imaging, nuclear imaging, and cardiac biopsy may be of use in further workup. Treatment is with non-steroidal anti-inflammatory agents, four to six weeks of limited exertion, and conventional heart failure treatment as necessary. Immune suppressant therapy with steroids may be uniquely beneficial in myopericarditis related to smallpox vaccination, compared with other types of myopericarditis. If a widespread vaccination program is undertaken in the future, many more cases of post-vaccinial myopericarditis could be seen. Practicing physicians should be aware that smallpox vaccine-associated myopericarditis is a real entity, and symptoms after vaccination should be appropriately evaluated, treated if necessary, and reported to the Vaccine Adverse Events Reporting System.
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Cooper LT, Gersh BJ. Viral infection, inflammation, and the risk of idiopathic dilated cardiomyopathy: can the fire be extinguished? Am J Cardiol 2002; 90:751-4. [PMID: 12356390 DOI: 10.1016/s0002-9149(02)02603-6] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
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Shields RC, Tazelaar HD, Berry GJ, Cooper LT. The role of right ventricular endomyocardial biopsy for idiopathic giant cell myocarditis. J Card Fail 2002; 8:74-8. [PMID: 12016630 DOI: 10.1054/jcaf.2002.32196] [Citation(s) in RCA: 63] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
Abstract
BACKGROUND Idiopathic giant cell myocarditis (GCM) is an uncommon cause of cardiac failure distinguished clinically from lymphocytic myocarditis by rapidly progressive heart failure, arrhythmias, and heart block. Unlike fulminant lymphocytic myocarditis, patients with fulminant cardiac failure caused by GCM may respond to certain immunosuppressive agents; however, right ventricular endomyocardial biopsy (EMB) is infrequently used to establish the diagnosis partly because the sensitivity of EMB for GCM is unknown. The purpose of this study was to estimate the sensitivity of right ventricular EMB for GCM in a referral population. METHODS AND RESULTS Twenty subjects (of 63 total) in the Multicenter Giant Cell Myocarditis Registry underwent both right ventricular EMB and heart pathology (HRTP) evaluation from apical wedge, explantation, or autopsy. The false-negative rate of right ventricular EMB was defined as the ratio of negative EMB to positive HRTP results. Ten of the 20 subjects were women. The mean age was 38 years (range, 16-53 years). Twelve (60%) subjects had a positive EMB and positive HRTP confirming GCM. Three (15%) had a negative EMB and positive HRTP for GCM. Five had a positive EMB and negative HRTP evaluation for GCM. The resulting sensitivity of EMB for GCM was 80% (12/15) with a positive predictive value of 71%. Assuming the 5 subjects with a positive EMB and negative HRTP are true positives, the sensitivity improves to 85% (17/20). Predictors of negative HRTP after positive EMB were time from symptom onset to HRTP (P.006) and time from EMB to HRTP (P.03). CONCLUSIONS The sensitivity of right ventricular EMB is high in patients with GCM who have early disease presentation and a fulminant clinical course. Although these results may not apply to individuals with less aggressive disease, EMB may be used selectively to distinguish fulminant heart failure caused by GCM from other causes in which the prognosis may differ.
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