Prognosis Of Cardiac Sarcoidosis Research Articles

Association Between Right Bundle Branch Block and Ventricular Arrhythmia in Patients With Cardiac Sarcoidosis

BackgroundVentricular arrhythmia (VA) is a life-threatening condition associated with cardiac sarcoidosis (CS). Right bundle branch block (RBBB) is a common conduction disorder in CS; however, its association with VA remains unknown. ObjectivesThis study aimed to investigate the relationship between RBBB and VA in patients with CS. MethodsThis was a post hoc analysis of ILLUMINATE-CS (Illustration of the Management and Prognosis of Japanese Patients with Cardiac Sarcoidosis), a multicenter, retrospective, and observational study that evaluated the clinical characteristics and prognosis of CS. Eligible patients were divided into two groups based on the presence or absence of RBBB at the time of diagnosis. The primary outcome was serious ventricular arrhythmia events (SVAEs), defined as a combination of sudden cardiac death and documented ventricular fibrillation, sustained ventricular tachycardia, or appropriate implantable cardioverter-defibrillator therapy. ResultsOverall, 312 patients were studied, with 155 (49.7%) patients presenting with RBBB (RBBB group). Patients in the RBBB group had a higher prevalence of basal interventricular septum (IVS) thinning and prominent late gadolinium enhancement in the basal IVS on cardiac magnetic resonance imaging than those in the non-RBBB group. During a median follow-up of 3.0 years (IQR: 1.6-6.0 years), 66 patients experienced SVAE. In multivariable Cox regression analysis, the RBBB group was independently associated with a higher incidence of SVAEs (HR: 1.93 [95% CI: 1.14-3.28]; P = 0.015). ConclusionsIn patients with CS, RBBB was an independent predictor of SVAEs, which might reflect the specific scar distribution that is predominant in the IVS.

The prognostic value of atrial fibrillation in patients with cardiac sarcoidosis

Abstract Background/Introduction The prognostic value of the presence of atrial fibrillation (AF) at the time of diagnosis in patients with cardiac sarcoidosis (CS) is unknown. Purpose The aim of this study is to investigate the association between the presence of AF at the time of diagnosis of CS and prognosis. Methods This study is a post-hoc analysis of ILLUMINATE-CS (Illustration of the Management and Prognosis of Japanese Patients With Cardiac Sarcoidosis), a multicenter, retrospective, and observational study that evaluated the clinical characteristics and prognosis of cardiac sarcoidosis. The primary endpoint was the combined endpoint of all-cause death and heart failure hospitalization. Results After excluding 32 patients with missing data about the presence of AF, 480 patients with CS (61.6±11.4 years, 36% men) diagnosed according to current guidelines were analyzed, and 86 primary endpoints were observed during a median follow-up period of 3.3 (interquartile range, 1.8-6.0) years. Patients with AF were associated with older age, higher levels of brain natriuretic peptide (BNP), and the prevalence of heart failure admission compared to those without AF. Kaplan-Meier curve analysis indicated that AF was significantly associated with a high incidence of primary endpoints (log-rank P=0.002), and this association was retained even after adjusting for known risk factors including log-transformed BNP and left ventiricular ejection fraction (LVEF) (hazard ratio, 2.10 [95% CI, 1.16-3.82]; P=0.015). Conclusion The presence of atrial fibrillation at the time of diagnosis in patients with cardiac sarcoidosis is independently associated with adverse events.

The Role of Echocardiography in the Contemporary Diagnosis and Prognosis of Cardiac Sarcoidosis: A Comprehensive Review.

Cardiac sarcoidosis (CS) is a rare inflammatory disorder characterised by the presence of non-caseating granulomas within the myocardium. Contemporary studies have revealed that 25-30% of patients with systemic sarcoidosis have cardiac involvement, with detection rates increasing in the era of advanced cardiac imaging. The use of late gadolinium enhancement cardiac magnetic resonance and 18fluorodeoxy glucose positron emission tomography (FDG-PET) imaging has superseded endomyocardial biopsy for the diagnosis of CS. Echocardiography has historically been used as a screening tool with abnormalities triggering the need for advanced imaging, and as a tool to assess cardiac function. Regional wall thinning or aneurysm formation in a noncoronary distribution may indicate granuloma infiltration. Thinning of the basal septum in the setting of extracardiac sarcoidosis carries a high specificity for cardiac involvement. Abnormal myocardial echotexture and eccentric hypertrophy may be suggestive of active myocardial inflammation. The presence of right-ventricular involvement as indicated by free-wall aneurysms can mimic arrhythmogenic right-ventricular cardiomyopathy. More recently, the use of myocardial strain has increased the sensitivity of echocardiography in diagnosing cardiac involvement. Echocardiography is limited in prognostication, with impaired left-ventricular (LV) ejection fraction and LV dilatation being the only established independent predictors of mortality. More research is required to explore how advanced echocardiographic technologies can increase both the diagnostic sensitivity and prognostic ability of this modality in CS.

The Prognostic Value of B-Type Natriuretic Peptide in Patients With Cardiac Sarcoidosis Without Heart Failure: Insights From ILLUMINATE-CS.

Background The prognostic role of BNP (B-type natriuretic peptide) in patients with cardiac sarcoidosis without evident heart failure is unknown. Methods and Results This is a post hoc analysis of ILLUMINATE-CS (Illustration of the Management and Prognosis of Japanese Patients With Cardiac Sarcoidosis), a multicenter, retrospective, and observational study that evaluated the clinical characteristics and prognosis of cardiac sarcoidosis. We analyzed patients with cardiac sarcoidosis without evident heart failure at the time of diagnosis. The association between baseline BNP levels and prognosis was investigated. The primary end point was the combined end point of all-cause death, heart failure hospitalization, and fatal ventricular arrhythmia. In total, 238 patients (61.0±11.1 years, 37% men) were analyzed, and 61 primary end points were observed during a median follow-up period of 3.0 (interquartile range, 1.7-5.8) years. Patients with high BNP (BNP above the median value of BNP) were older and had a lower renal function and left ventricular ejection fraction than those with low BNP values. Kaplan-Meier curve analysis indicated that high BNP levels were significantly associated with a high incidence of primary end points (log-rank P=0.004), and this association was retained even in multivariable Cox regression (hazard ratio, 2.06 [95% CI, 1.19-3.55]; P=0.010). Log-transformed BNP as a continuous variable was associated with the primary end point (hazard ratio, 2.12 [95% CI, 1.31-3.43]; P=0.002). Conclusions High baseline BNP level was an independent predictor of future adverse events in patients with cardiac sarcoidosis without heart failure at the time of diagnosis. Registration URL: https://www.umin.ac.jp/english/; Unique Identifier: UMIN-CTR: UMIN000034974.

Cardiac involvement in sarcoidosis: Evolving concepts in diagnosis and treatment

Clinically evident cardiac involvement has been noted in at least 2 to 7% of patients with sarcoidosis, but occult involvement is much higher (> 20%). Cardiac Sarcoidosis (CS) is often not recognized as an antemortem, as sudden death may be the presenting feature. Cardiac involvement may occur at any point during the course of sarcoidosis and may occur in the absence of pulmonary or systemic involvement. Sarcoidosis can involve any part of the heart. The prognosis of CS is related to the extent and site(s) of involvement. Most deaths due to CS are due to arrhythmias or conduction defects, but granulomatous infiltration of the myocardium may cause progressive and ultimately lethal cardiomyopathy. The definitive diagnosis of isolated CS is difficult and the yield of Endomyocardial Biopsies (EMB) is low. Treatment of CS is often warranted even in the absence of histologic proof. Radionuclide scans are integral to the diagnosis. Gadolinium-enhanced cardiac magnetic imaging scans and 18Fluorodeoxyglucose (18FDG)-Positron Emission Tomography (PET) are the key imaging modalities to diagnose CS. The prognosis of CS is variable, but mortality rates of untreated CS are high. Randomized therapeutic trials have not been done, but corticosteroids (alone or combined with additional immunosuppressive agents) are the mainstay of therapy. Additionally, anti-arrhythmic agents and therapy for heart failure are often required. Because of the potential for sudden cardiac death, an Implantable Cardioverter-Defibrillator (ICD) should be placed in any patient with CS and serious ventricular arrhythmias or heart block and should be considered for cardiomyopathy. Cardiac transplantation is a viable option for patients with end-stage CS refractory to medical therapy.

Management of ventricular tachycardia in patients with cardiac sarcoidosis.

Sarcoidosis is a multisystem granulomatous disease with 2 different phases (inflammation and scar). In the current era of targeted use of implantable cardioverter-defibrillators and modern heart failure therapy, recent data indicate the prognosis of cardiac sarcoidosis (CS) is much improved, and hence more patients are presenting with recurrent ventricular tachycardia (VT). This review highlights our current understanding of the pathophysiology and management of ventricular arrhythmias in CS with the major focus on indications, techniques, and outcomes of ablation.It is likely macroreentry phenomena around areas of fibrosis is the most frequent mechanism of ventricular arrhythmia in CS. It is also possible that inflammation may play a role in initiating reentry with ventricular ectopy in CS patients, or by slowing conduction in diseased tissue. The best available data would suggest annual rates of VT of perhaps 1%–2% and 10%–15% in patients with initially clinically silent and clinically manifest disease, respectively. Current guidelines recommend a stepwise approach to VT management. The first suggested step is treatment with immunosuppression if there is evidence of active inflammation. Antiarrhythmic medications are often started at the same time, with catheter ablation considered if VT cannot be controlled. Activation and entrainment mapping and ablation are favored in the setting of hemodynamically tolerated VT. Substrate ablation targets areas of abnormal electrogram and favorable pace mapping using linear and/or cluster lesion sets with the goal of abolishing critical isthmuses and/or blocking VT exit sites. Epicardial mapping ablation is required in 20%–35% of cases. In general, more morphologies of VT are induced (often 3–4) and subsequent outcomes (recurrence rates 40%–50%) are less favorable than in other forms of nonischemic cardiomyopathy.The prognosis of CS is much improved and, as a result, more patients are developing VT during follow-up. Likely principally related to the complex disease substrate, VT ablation is technically challenging, with moderate outcomes, and much remains to be learned.

Management and outcomes of cardiac sarcoidosis: a 20-year experience in two tertiary care centres.

Cardiac sarcoidosis (CS) often presents with ventricular arrhythmias, heart block, and cardiomyopathy. The prognosis of CS with contemporary management is uncertain. We estimated the impact of baseline and treatment variables on left ventricular ejection fraction (LVEF), ventricular assist device placement, heart transplant, and death. We identified patients with CS seen from 1994-2014 at two large academic medical centres. All met the 2014 Heart Rhythm Society expert consensus criteria for diagnosis. From the 574 patients identified, 91 met inclusion criteria. Twenty-two (24.2%) were diagnosed by endomyocardial biopsy. Cardiomyopathy was the primary presentation in 47 patients (51.6%). Within 90 days of diagnosis, 41 patients (45.0%) received prednisone alone, 29 (31.9%) received alternative immunosuppression with or without prednisone, and 21 (23.1%) received no immunosuppression. During follow-up, 31 of 47 cardiomyopathy patients experienced improvement in LVEF, while 23 experienced decline in LVEF or clinical exacerbation, and 15 of 22 patients presenting with ventricular arrhythmia had recurrence. These results did not differ by treatment group. During a median follow-up of 44 months for our cohort, 14 patients reached the composite endpoint of ventricular assist device placement, heart transplant, or death. Survival without the composite outcome did not differ by treatment group, but was worse among patients presenting with cardiomyopathy (log-rank = 0.005). In a large series of CS subjects, rates of ventricular arrhythmia and heart failure events remain high with no treatment regimen clearly associated with better outcome. Patients with cardiomyopathy at diagnosis were more likely to reach the composite endpoint.

Cardiac Involvement in Sarcoidosis: Evolving Concepts in Diagnosis and Treatment.

AbstractClinically evident sarcoidosis involving the heart has been noted in at least 2 to 7% of patients with sarcoidosis, but occult involvement is much higher (> 20%). Cardiac sarcoidosis is often not recognized antemortem, as sudden death may be the presenting feature. Cardiac involvement may occur at any point during the course of sarcoidosis and may occur in the absence of pulmonary or systemic involvement. Sarcoidosis can involve any part of the heart, with protean manifestations. Prognosis of cardiac sarcoidosis is related to the extent and site(s) of involvement. Most deaths due to cardiac sarcoidosis are due to arrhythmias or conduction defects, but granulomatous infiltration of the myocardium may be lethal. The definitive diagnosis of isolated cardiac sarcoidosis is difficult. The yield of endomyocardial biopsies is low; treatment of cardiac sarcoidosis is often warranted even in the absence of histologic proof. Radionuclide scans are integral to the diagnosis. Currently, 18F-fluorodeoxyglucose–positron emission tomography/computed tomography and gadolinium-enhanced magnetic resonance imaging scans are the key imaging modalities to diagnose cardiac sarcoidosis. The prognosis of cardiac sarcoidosis is variable, but mortality rates of untreated cardiac sarcoidosis are high. Although randomized therapeutic trials have not been done, corticosteroids (alone or combined with additional immunosuppressive medications) remain the mainstay of treatment. Because of the potential for sudden cardiac death, implantable cardioverter defibrillators should be placed in any patient with cardiac sarcoidosis and serious ventricular arrhythmias or heart block, and should be considered for cardiomyopathy. Cardiac transplantation is a viable option for patients with end-stage cardiac sarcoidosis refractory to medical therapy.

Complementary Role of CMR to Conventional Screening in the Diagnosis and Prognosis of Cardiac Sarcoidosis

ObjectivesThe goal of this study was to assess the independent and collective diagnostic value of various modalities in cardiac sarcoidosis, delineate the role of cardiac magnetic resonance (CMR), and identify patients at risk. BackgroundCardiac sarcoidosis is associated with increased morbidity and mortality. CMR is a key modality in the evaluation of patients with cardiac symptoms, but the complementary role of CMR to conventional tests for the diagnosis of cardiac sarcoidosis is not fully defined. MethodsPatients (N = 321) with biopsy-proven sarcoidosis underwent conventional cardiac testing and CMR with late gadolinium enhancement (LGE) and were followed up for primary (composite of all-cause mortality, sustained ventricular tachycardia [VT] episodes, or hospitalization for heart failure) and secondary (nonsustained VT episodes) endpoints. ResultsCardiac sarcoidosis was diagnosed in 29.9% of patients according to the Heart Rhythm Society consensus criteria. CMR was the most sensitive and specific test (area under the curve: 0.984); it detected 44 patients with cardiac symptoms and/or electrocardiogram (ECG) abnormalities but normal echocardiogram, as well as 15 asymptomatic patients with normal baseline testing. Echocardiography added to cardiac history and ECG did not change sensitivity of the initial screening strategy (68.8% vs. 72.9%). Despite a high positive predictive value (83.9%), echocardiography had a low sensitivity (27.1%). During follow-up, 7.2% of patients reached the primary endpoint and another 3.4% reached the secondary endpoint. LGE was and independent predictor of primary endpoints (hazard ratio: 5.68; 95% CI: 1.74 to 18.49; p = 0.004). LGE, age, and baseline nonsustained VT were independent predictors of all events. In patients with cardiac symptoms and/or an abnormal ECG, CMR increased diagnostic accuracy and independently predicted primary endpoints (hazard ratio: 12.71; 95% confidence interval: 1.48 to 109.35; p = 0.021). ConclusionsOf all cardiac tests, CMR was the most valuable in the diagnosis and prognosis of cardiac sarcoidosis in a general sarcoidosis population. Echocardiography had an overall limited diagnostic value as a screening test, and an abnormal study, despite a high positive predictive value, may still need confirmation with CMR.

Caractéristiques cliniques, cardiologiques et pronostiques des patients atteints de sarcoïdose cardiaque. Étude de cas-témoins

IntroductionCardiac involvement is the second cause of mortality associated with sarcoidosis in France. The objective of this study was to evaluate the clinical signs, the main cardiological investigations and the prognosis of cardiac sarcoidosis (CS) in a retrospective case-control study. Methods278 suspected CS patients underwent a cardiac MRI (cmR) between January 2007 and December 2013. The diagnosis was made in 114 cases before the combination of consistent clinical and paraclinical signs and the presence of delayed enhancement on the cmRI. The cases were compared to 118 patients in whom the diagnosis of CS was not confirmed. ResultsTwo factors were associated with the CS: female sex (54% vs 34%; p= 0.0019) and a young age at diagnosis (45 years vs 49 years; p= 0.013). Patients with CS were not different from controls in terms of cardiac symptoms, extrathoracic involvement or radiological stage. Conduction abnormalities on ECG and ventricular arrhythmias on Holter-ECG were more frequent in cases of CS (34% vs 20.3%; p= 0.020 and 50.6 % vs. 6.4 %; p <0.001). Focal or focal on diffuse uptake on FDG-PET was associated with CS (95 % vs 19 %; p < 0.001). Cardiac complications were identified in 23 of the patients. No deaths or serious ventricular rhythm disorders were observed during the 4.9 years of average follow-up. The cmR and FDG-PET are useful to confirm the diagnosis of CS but this cannot be achieved consistently. The lack of a simple algorithm to identify patients at risk for CS makes it difficult to benefit from these studies.

Cardiac sarcoidosis: epidemiology, characteristics, and outcome over 25 years in a nationwide study.

This study was designed to assess the epidemiology, characteristics, and outcome of cardiac sarcoidosis (CS) in Finland. We identified in retrospect all adult (>18 years of age) patients diagnosed with histologically confirmed CS in Finland between 1988 and 2012. A total of 110 patients (71 women) 51±9 years of age (mean±SD) were found and followed up for outcome events to the end of 2013. The annual detection rate of CS increased >20-fold during the 25-year period, reaching 0.31 in 1×10(5) adults between 2008 and 2012. The 2012 prevalence of CS was 2.2 in 1×10(5). Nearly two thirds of patients had clinically isolated CS. Altogether, 102 of the 110 patients received immunosuppressive therapy, and 56 received an intracardiac defibrillator. Left ventricular function was impaired (ejection fraction <50%) in 65 patients (59%) at diagnosis and showed no overall change over 12 months of steroid therapy. During follow-up (median, 6.6 years), 10 patients died of a cardiac cause, 11 patients underwent transplantation, and another 11 patients suffered an aborted sudden cardiac death. The Kaplan-Meier estimates for 1-, 5-, and 10-year transplantation-free cardiac survival were 97%, 90%, and 83%, respectively. Heart failure at presentation predicted poor outcome (log-rank P=0.0001) with a 10-year transplantation-free cardiac survival of only 53%. The detection rate of CS has increased markedly in Finland over the last 25 years. With current therapy, the prognosis of CS appears better than generally considered, but patients presenting with heart failure still have poor long-term outcome.

Cardiac involvement in sarcoidosis: evolving concepts in diagnosis and treatment.

Clinically evident sarcoidosis involving the heart has been noted in at least 2 to 7% of patients with sarcoidosis, but occult involvement is much higher (> 20%). Cardiac sarcoidosis is often not recognized antemortem, as sudden death may be the presenting feature. Cardiac involvement may occur at any point during the course of sarcoidosis and may occur in the absence of pulmonary or systemic involvement. Sarcoidosis can involve any part of the heart, with protean manifestations. Prognosis of cardiac sarcoidosis is related to extent and site(s) of involvement. Most deaths due to cardiac sarcoidosis are due to arrhythmias or conduction defects, but granulomatous infiltration of the myocardium may be lethal. The definitive diagnosis of isolated cardiac sarcoidosis is difficult. The yield of endomyocardial biopsies is low; treatment of cardiac sarcoidosis is often warranted even in the absence of histologic proof. Radionuclide scans are integral to the diagnosis. Currently, 18F-fluorodeoxyglucose positron emission tomography/computed tomography and gadolinium-enhanced magnetic resonance imaging scans are the key imaging modalities to diagnose cardiac sarcoidosis. The prognosis of cardiac sarcoidosis is variable, but mortality rates of untreated cardiac sarcoidosis are high. Although randomized therapeutic trials have not been done, corticosteroids (alone or combined with additional immunosuppressive medications) remain the mainstay of treatment. Because of the potential for sudden cardiac death, implantable cardioverter-defibrillators should be placed in any patient with cardiac sarcoidosis and serious ventricular arrhythmias or heart block, and should be considered for cardiomyopathy. Cardiac transplantation is a viable option for patients with end-stage cardiac sarcoidosis refractory to medical therapy.

Cardiac sarcoidosis is a slowly progressive cardiomyopathy

Purpose: To evaluate the course and prognosis of cardiac sarcoidosis (CS). Methods: The cases were identified from the hospital discharge and pathology registries from university and central hospitals in Finland between 1988-2011. Their diagnoses, including details of the diagnostic procedures conducted, were reviewed, and the diagnoses were retrospectively evaluated. Results: Of the 104 patients, 54/104 (52%) were diagnosed based on endomyocardial biopsy. 50 (48%) had histological verification of sarcoidosis from noncardiac tissue. They had symptoms and imaging findings consistent with CS in ECHO, cMRI, or 18FDG PET. The mean age (± SD) was 50.1±8.6 years (range, 33-73). 66 (63%) were female 38 (37%) were male. The main presenting clinical manifestations were atrioventricular block in 50 patients of the 104 (49%), sustained monomorphic VT in 23 cases (22%), VF in 5 (5%), congestive heart failure in 19 cases (18%), and frequent ventricular extrasystole in 6/104 (6%). Of the 104 cases of CS, 64 (62%) had a normal LV function (EF > 50%) at the time of first manifestation (CHB, VT, or VPCs) and 32/64 (50%) developed LV dysfunction by the end of the follow-up. The mean and median time to LV dysfunction (EF < 40%) for patients with initially normal LV function after the initial presentation were 77.6 months and 71.0 months, respectively. 18 patients (17%) either died (n=6) or had cardiac transplant (n=12). Figure shows Kaplan-Maier curves for survival free of death or transplantation. General prognostic signs of heart failure, low LV function, high pro-BNP and LBBB, were associated with adverse outcome in CS. ![Figure][1] Conclusions: CS is progressive cardiomyopathy that leads to LV dysfunction despite normal LV function at the time of initial presentation. The rate of death or cardiac transplant is 17%. [1]: pending:yes

心臓サルコイドーシスにおける診断，治療，予後に関する臨床的検討

我々は多数例の心臓サルコイドーシスを長期経過観察する中で，診断，治療，予後などの包括的研究を20 年以上継続し，国内外の雑誌や学会にその結果を報告してきた．新旧診断の手引きの作成にかかわり，心臓サルコイドーシスの臨床的特徴を，病理組織所見や各種画像診断を分析することにより，拡張型心筋症や肥大型心筋症などと対比しながら明らかにした．心臓サルコイドーシスに対する治療は，１）ステロイドを中心とした免疫抑制療法，２）心不全治療，３）不整脈治療に要約される．ステロイド治療が行われた心臓サルコイドーシスの予後を検討し，予後規定因子を明らかにした．また，最近の心不全治療や不整脈治療の進歩，免疫抑制薬の工夫などによりその予後が改善してきたことを報告した．

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

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&lt;0.05), survival in the earlier cohort was significantly worse (log-rank=4.41, p&lt;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&lt;0.05), β-blocker use (46% versus 6%, p&lt;0.01) and addition of methotrexate (21% versus 0%, p&lt;0.05), and increased maintenance dose (7.0±1.9mg/day versus 5.0±0.9mg/day, p&lt;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&lt;0.01) and LVEF (HR: 0.83/1% increase, p&lt;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 Involvement in Sarcoidosis

Cardiac sarcoidosis is a challenging diagnostic entity. Manifestations range from an incidentally discovered, benign condition to fatal cardiomyopathy and arrhythmias. In this review, we discuss the salient clinical features, diagnostic evaluation, treatment, and prognosis of cardiac sarcoidosis. A definitive histologic diagnosis of myocardial sarcoidosis is difficult, and treatment may be necessary in some patients with suspected cardiac involvement even in the absence of histologic confirmation. Optimal strategies to diagnose cardiac involvement have not been clarified. Thallium (201) radionuclide scintigraphy has been most extensively studied; 2-D echocardiography is less sensitive, but may have a complementary role. Additional radionuclide techniques (e.g., gallium (67) or technetium (99) ) may have a role in complex cases. Given the life-threatening nature of cardiac involvement, prompt and aggressive treatment with corticosteroids or immunosuppressive agents, or both, is warranted. Long-term, possibly lifelong, therapy may be required. Serious, recurrent tachyarrhythmias may require implantation of an automatic cardioverter-defibrillator. Recognizing cardiac sarcoidosis and selecting a sensible management strategy remain the major obstacles facing clinicians.

Comparison of clinical features and prognosis of cardiac sarcoidosis and idiopathic dilated cardiomyopathy

In the present study, clinical findings of 15 patients with cardiac sarcoidosis presenting as dilated cardiomyopathy were compared with those of 30 consecutive patients with idiopathic dilated cardiomyopathy. The sarcoidosis patients had different clinical features, including female predominance, a high incidence of grave conduction disturbance and abnormal wall thickness, uneven wall motion abnormalities, and perfusion defects preferentially affecting the anteroseptal and apical regions, and poor prognosis compared with those with idiopathic dilated cardiomyopathy.