Left Ventricular Septal Aneurysm in Cardiac Sarcoidosis

2010 ◽  
pp. 216-217
Author(s):  
Xing Sheng Yang ◽  
Jing Ping Sun ◽  
John D. Merlino
Keyword(s):  
Cardiac Sarcoidosis ◽  
Left Ventricular

scholarly journals Cardiac sarcoidosis: systematic review of literature on corticosteroid and immunosuppressive therapies

2021 ◽  
pp. 2100449
Author(s):  
Julien Stievenart ◽  
Guillaume Le Guenno ◽  
Marc Ruivard ◽  
Virginie Rieu ◽  
Marc André ◽  
...  
Keyword(s):  
Adverse Events ◽  
Cardiac Sarcoidosis ◽  
Positive Impact ◽  
Immunosuppressive Agents ◽  
Left Ventricular ◽  
Randomised Control Trial ◽  
Cochrane Library ◽  
Prisma Statement ◽  
Threatening Condition ◽  
Life Threatening

BackgroundCardiac sarcoidosis (CS) is a life-threatening condition in which clear recommendations are lacking. We aimed to review systematically the literature on cardiac sarcoidosis treated by corticosteroids and/or immunosuppressive agents in order to update the management of CS.MethodsUsing Pubmed, Embase and Cochrane Library databases, we found original articles on corticosteroid and/or standard immunosuppressive therapies for CS which provided at least fair SIGN overall assessment of quality and analyse the relapse rate, major cardiac adverse events (MACEs) and adverse events. We base our methods on Prisma statement and checklist.ResultsWe retrieved 21 studies. Mean quality provided by SIGN assessment was 6.8/14 (range 5–9). Corticosteroids appeared to have a positive impact on left ventricular function, atrioventricular block, and ventricular arrhythmias. For corticosteroids alone, nine (45%) studies (n=351) provided data on relapses, representing an incidence of 34% (n=119). Three studies (14%, n=73) provided data on MACEs (n=33), representing 45% of MACEs in patients treated by corticosteroid alone. Nine studies provided data on adjunctive immunosuppressive therapy in which four studies (n=78) provided data on CS relapse, representing an incidence of 33% (n=26). Limitations consisted in no randomised control trial retrieved and unclear data on MACEs in patients treated by combined immunosuppressive agents and corticosteroids.ConclusionsCorticosteroids should be started early after diagnosis but the exact scheme is still unclear. Studies concerning adjunctive conventional immunosuppressive therapies are lacking and benefits of adjunctive immunosuppressive therapies are unclear. Homogenous data on CS long-term outcomes under corticosteroids, immunosuppressive therapies and other adjunctive therapies are lacking.

Abstract 5935: Influence of Recent Advances in the Management of Heart Failure and Ventricular Arrhythmias on Survival in Patients with Cardiac Sarcoidosis

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Yoshikazu Yazaki ◽  
Mitsuaki Horigome ◽  
Kazunori Aizawa ◽  
Takeshi Tomita ◽  
Hiroki Kasai ◽  
...  
Keyword(s):  
Heart Failure ◽  
Ventricular Tachycardia ◽  
Cardiac Sarcoidosis ◽  
Severe Heart Failure ◽  
Left Ventricular ◽  
Cardiac Resynchronization ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Predictors Of Mortality ◽  
Recent Advances

Background : We previously described severity of heart failure and ventricular tachycardia (VT) as independent predictors of mortality in patients with cardiac sarcoidosis (CS). Medical treatment for chronic heart failure has been established over the last few decades. Prophylactic use of implantable cardioverter defibrillator (ICD) and cardiac resynchronization therapy (CRT or CRT-D) have been introduced in patients with severe heart failure. We therefore hypothesized that the prognosis of CS improves due to such advances in the management of heart failure and VT. Methods : To confirm our hypothesis, we analyzed 43 CS patients diagnosed between 1988 and 2006 and treated with corticosteroids. We classified two sequential referral patients diagnosed between 1988 and 1997 (n=19) and between 1998 and 2006 (n=24), and compared treatment and prognosis between the two cohorts. Results : Left ventricular ejection fraction (LVEF) and dimensions were similar between the two cohorts. Although age in the 1988–1997 referral cohort was significantly younger than that in the 1998–2006 referral cohort (54±14years versus 62±10years, p<0.05), survival in the earlier cohort was significantly worse (log-rank=4.41, p<0.05). The 1- and 5-year mortality rates were 88% and 71% in the 1988–1997 referral cohort, and 96% and 92% in the 1998–2006 referral cohort, respectively. The 1998–2006 referral cohort showed significantly higher incidence of ICD or CRT-D implantation (29% versus 6%, p<0.05), β-blocker use (46% versus 6%, p<0.01) and addition of methotrexate (21% versus 0%, p<0.05), and increased maintenance dose (7.0±1.9mg/day versus 5.0±0.9mg/day, p<0.01) compared to the 1988–1997 referral cohort. Multivariate analysis including age, LVEF, and sustained ventricular tachycardia (sVT) identified diagnosis between 1988 and 1997 (hazard ratio [HR]: 19.8, p<0.01) and LVEF (HR: 0.83/1% increase, p<0.01) as independent predictors of mortality. Conclusions : Survival in the recent CS patients is significantly better than previously described. Recent advances in the device therapies and medical treatments including modified immunosuppression alter the clinical outcome in patients with CS.

Cardiac sarcoidosis mimicking left ventricular noncompaction: An approach to acquired apical hypertrabeculation

2019 ◽  
Vol 36 (4) ◽  
pp. 791-793
Author(s):  
Tomoo Nagai ◽  
Hitomi Horinouchi ◽  
Azusa Kitayama ◽  
Yuji Ikari
Keyword(s):  
Cardiac Sarcoidosis ◽  
Left Ventricular ◽  
Left Ventricular Noncompaction ◽  
Ventricular Noncompaction

Insight form cardiovascular magnetic resonance on cardiac sarcoidosis: a single centre experience

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
C Alderighi ◽  
A Baritussio ◽  
O Ozden Tok ◽  
M Perazzolo Marra ◽  
S Iliceto ◽  
...  
Keyword(s):  
Cardiovascular Magnetic Resonance ◽  
Magnetic Resonance ◽  
T1 Mapping ◽  
Cardiac Involvement ◽  
Cardiac Sarcoidosis ◽  
T2 Mapping ◽  
Left Ventricular ◽  
Lv Function ◽  
Post Contrast ◽  
Lv Volumes

Abstract Background Clinically manifest cardiac sarcoidosis (CS) has a prevalence of 5%, but is more frequent in autoptic series (25%). Diagnosis is multiparametric and relies on clinical criteria and imaging findings, although a certain diagnosis, especially in the case of isolated CS (ICS), can only be based on endomyocardial biopsy. Cardiovascular magnetic resonance (CMR) has a comprehensive role in the assessment of CS: left ventricular (LV) dysfunction and extent of late gadolinium enhancement (LGE)are important predictors of prognosis, T2 mapping provides information on disease activity and global longitudinal strain (GLS) analysis can uncover subclinical LV impairment. Purpose To assess the prevalence of CS by CMR in patients with biopsy-proven extracardiacsarcoidosis (ECS); to describe the imaging characteristics of patients with ECS and those with high clinical suspicionof ICS; to investigate the contribution of more recent techniques to the diagnosis of CS alongside traditional LGE assessment. Methods We retrospectively enrolled 84 patients (66% males, mean age 59±13 years) referred to our centreforsuspected CS (biopsy-proven ECS, n=61; clinical presentation suggestive of CS,, n=23). CMR was performed on a 1.5T scanner, with a protocol comprehensive of biventricular functional assessment and post-contrast images; T2-STIR images (n=30), native myocardial T1 mapping (n=24) and T2 mapping (n=19) were also performed in selected patients. Tissue tracking analysis was perfomed in all patients using a dedicated software. Results Based on CMR findings, 35 patients (42%) with ECS did not show cardiac involvement (SS), 26 (31%) showed both cardiac and systemic involvement (CS-SS) and 23 (27%) had evidence of ICS (ICS). 43% of patients had history of arrhythmias, but life-threatening tachyarrhythmiaswere more frequent in patients with CS (p=0.02).Patients with CS had significantly lower LVEF (p&lt;0,01), larger LV volumes (p&lt;0,01) and greater LV mass (p&lt;0,01). GLS values were impaired in all the groups but significantly more in patients with CS (p&lt;0,01). With regards to LGE distribution, ICS patients showed a higher number of segments involved (p=0,011) as compared to CS patients. T2-STIRimages were positive in 3 out of 30 patients; T2 mapping detected myocardial oedema in 1 patient with negative T2- STIR and was positive in 7 who did not undergo traditional oedema evaluation. T1 mapping mainly confirmed the results provided by LGE, but was altered in 1 patient who could not receive gadolinium. Conclusions CMR findings consistent with CS were found in 49 patients referred for suspected CS. Patients with cardiac involvement, particularly if isolated, had significantly lower LVEF, greater LV volumes and more impaired GLS. Patients with SS, despite a normal LV function, showed mildly impaired GLS, subtending subclinical cardiac involvement. Funding Acknowledgement Type of funding source: None

Cardiac Sarcoidosis

2020 ◽  
Vol 41 (05) ◽  
pp. 626-640 ◽  
Author(s):  
David H. Birnie
Keyword(s):  
Heart Failure ◽  
Cardiac Involvement ◽  
Cardiac Sarcoidosis ◽  
Positron Emission Tomography Imaging ◽  
Left Ventricular ◽  
Implantable Cardioverter Defibrillators ◽  
Heart Failure Treatment ◽  
Positron Emission ◽  
Minimal Data ◽  
Cardioverter Defibrillators

AbstractApproximately 5% of patients with sarcoidosis will have clinically manifest cardiac involvement presenting with one or more of ventricular arrhythmias, conduction abnormalities, and heart failure. It is estimated that another 20 to 25% of pulmonary/systemic sarcoidosis patients have asymptomatic cardiac involvement (clinically silent disease). Cardiac presentations can be the first (and/or an unrecognized) manifestation of sarcoidosis in a variety of circumstances. Immunosuppression therapy (usually with corticosteroids) has been suggested for the treatment of clinically manifest cardiac sarcoidosis (CS) despite minimal data supporting it. Positron emission tomography imaging is often used to detect active disease and guide immunosuppression. Patients with clinically manifest disease often need device therapy, typically with implantable cardioverter defibrillators (ICDs). The extent of left ventricular dysfunction seems to be the most important predictor of prognosis among patients with clinically manifest CS. In the current era of earlier diagnosis, modern heart failure treatment, and use of ICD therapy, the prognosis from CS is much improved. In a recent Finnish nationwide study, 10-year cardiac survival was 92.5% in 102 patients.

P1807Manifestations and outcome of cardiac sarcoidosis - does gender matter?

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
P Simonen ◽  
J Lehtonen ◽  
M Kupari
Keyword(s):  
Cardiac Sarcoidosis ◽  
Hot Spot ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Diagnostic Process ◽  
Term Outcome ◽  
Ventricular Ejection Fraction ◽  
Long Term Outcome

Abstract Background Sarcoidosis is characterized by the formation of inflammatory epithelioid-cell granulomas in various organs with cardiac involvement as its most ominous manifestation. A female preponderance in the prevalence of cardiac sarcoidosis (CS) is well known but other possible gender differences remain poorly studied. Purpose We set out to evaluate gender-related differences in the manifestations and long-term outcome of CS. Methods We reviewed the history, diagnostic procedures, details of treatment and outcome of 158 consecutive patients with histologically confirmed CS diagnosis between 1988 and 2017 at our hospital. Follow-up data were collected up to the end of 2018. Results The study population consisted of 51 men and 107 women (68%). At presentation, men were younger than women (mean age 47 years vs 51 years, p=0.045) and had more often a history of pre-existing extracardiac sarcoidosis (25% vs 10%, p=0.013). Isolated CS remained less common in men even after the complete diagnostic process (50% vs 75%, p=0.001). The main presenting CS manifestations were atrioventricular block, ventricular tachyarrhythmias and heart failure in 39%, 30% and 18% of men vs in 54%, 23% and 17% of women, respectively (p=0.183). Left ventricular ejection fraction at presentation averaged 49±11% in men and 49±13% in women (p=0.845). Troponin T was elevated more often in men at the presentation (46% vs 26%, p=0.024). At magnetic resonance imaging, pathological myocardial late gadolinium enhancement was observed in 87% of men and 84% of women (p=0.615). Myocardial “hot spot” at 18-F fluorodeoxyglucose positron emission tomography was also equally common (87% in men, 92% in women, p=0.468). An intracardiac cardioverter-defibrillator was implanted in 78% of men and 75% of women (p=0.693) and nearly all patients (99%, no gender difference) received immunosuppressive therapy. During the mean follow-up of 64 months, 10 of 51 men versus 30 of 107 women either died of a cardiac cause, suffered an aborted sudden cardiac death or underwent transplantation. The composite event-free survival did not differ between genders (Figure 1. Log-rank p=0.852). Conclusions Two thirds of CS patients are women. At disease presentation, women are older than men and their sarcoidosis is more often isolated to the heart but the clinical manifestations, diagnostic findings and long-term outcome are comparable in the two genders.

The Many Faces of Cardiac Sarcoidosis

2019 ◽  
Vol 153 (3) ◽  
pp. 294-302 ◽  
Author(s):  
Virian D Serei ◽  
Billie Fyfe
Keyword(s):  
Literature Review ◽  
Cardiac Sarcoidosis ◽  
Interventricular Septum ◽  
Left Ventricular ◽  
Review Literature ◽  
Sampling Errors ◽  
End Stage Heart Failure ◽  
The Many ◽  
End Stage ◽  
Current Guidelines

Abstract Objectives The objective of this study was to review and illustrate the sometimes diagnostically challenging features of cardiac sarcoidosis. We emphasize variable phenotypes presented at explant and biopsy evaluation and review literature regarding ancillary clinical and pathologic studies to enhance diagnostic accuracy. Methods A literature review was performed and two cardiac sarcoidosis cases were illustrated. Results Our cases and literature review demonstrate the pathologic spectrum of cardiac sarcoidosis. Irregular left ventricular free wall involvement is most common, followed by the interventricular septum and right ventricle. Although granulomas are often composed of tight epithelioid macrophage aggregates, early granulomas comprise loosely associated macrophages with lymphocyte predominance. Chronic disease leads to fibrosis and end-stage heart failure. Sampling errors and variable histology cause low endomyocardial biopsy sensitivity. Conclusions Current guidelines use clinical, radiologic, and immunohistologic criteria for diagnosing cardiac sarcoidosis. Knowledge of these guidelines will assist pathologists in making accurate diagnosis of this disease.

scholarly journals A case series on inflammatory cardiomyopathy and suspected cardiac sarcoidosis: role of cardiac PET in management

2020 ◽  
Vol 4 (4) ◽  
pp. 1-9
Author(s):  
Peter J Kennel ◽  
Farhan Raza ◽  
Jiwon Kim ◽  
Parmanand Singh ◽  
Alain Borczuk ◽  
...  
Keyword(s):  
Cardiac Sarcoidosis ◽  
Significant Coronary Artery Disease ◽  
Left Ventricular ◽  
Pet Scan ◽  
Left Ventricular Ejection ◽  
Cardiomyocyte Hypertrophy ◽  
Inflammatory Cardiomyopathy ◽  
Ventricular Ejection Fraction ◽  
New Onset

Abstract Background Presentation of life-threatening arrhythmias concomitantly with a new-onset non-ischaemic cardiomyopathy raises concern for an inflammatory cardiomyopathy such as cardiac sarcoidosis or cardiac manifestations of connective tissue disease. Comprehensive workup for specific aetiologies may be unrevealing except for signs of myocardial inflammation identified on cardiac positron emission tomography (PET). Here, we present five cases of such subjects and their clinical course. Case summary We collected clinical, imaging, pathological, and follow-up data of five subjects presenting with arrhythmias and unexplained new-onset cardiomyopathy. Mean age was 56.2 ± 5.8 years. Three subjects presented with ventricular tachycardia and two with atrial arrhythmias. Echocardiography showed a mean left ventricular ejection fraction of 37 ± 9%. Significant coronary artery disease was ruled out in all cases as the cause of the cardiomyopathy. All patients underwent cardiac magnetic resonance imaging (MRI) and PET scan at presentation and follow-up. In all patients, cardiac MRI revealed hyperenhancement in epicardial and mid-myocardial pattern in a non-coronary distribution, while PET scan revealed fluorodeoxyglucose (FDG) mismatch defects in multiple foci in a non-coronary distribution. Right ventricular biopsy was obtained in all patients and revealed interstitial fibrosis and cardiomyocyte hypertrophy. On median follow-up of 210 days, all subjects had improvement in both heart failure symptoms and arrhythmias and repeat PET in four out of five patients showed decreased inflammation. Discussion A high level of suspicion for inflammatory cardiomyopathy is needed in patients presenting with new unexplained cardiomyopathy and arrhythmias. A cardiac FDG-PET should be considered for diagnosis if cardiac inflammation is in the differential. This can inform further decisions regarding targeted immunomodulation therapy that may be helpful in this cohort.

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