Cardiac Sarcoidosis

Zhou, Ying; Lower, Elyse E.; Li, Huiping; Costea, Alexandru; Attari, Mehran; Baughman, Robert P.

doi:10.1016/j.chest.2016.08.1457

Cited by 85 publications

(34 citation statements)

References 28 publications

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“…Demographic factors such as older age,29 male sex,33 and black race9 were associated with in‐hospital mortality. Our findings are in agreement with an informative study that examined sarcoidosis mortality from 1988 to 2007 at a population level using data from the National Center for Health Statistics 34.…”

Section: Discussionmentioning

confidence: 98%

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Hospitalization Rates, Prevalence of Cardiovascular Manifestations, and Outcomes Associated With Sarcoidosis in the United States

Patel

Kalra

Doshi

et al. 2018

JAHA

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BackgroundRecent trends of hospitalizations and in‐hospital mortality are not well defined in sarcoidosis. We examined aforementioned trends and prevalence of cardiovascular manifestations and explored rates of implantable cardioverter‐defibrillator implantation in hospitalizations with sarcoidosis.Methods and ResultsUsing data from the National Inpatient Sample, a retrospective population cohort from 2005 to 2014 was studied. To identify sarcoidosis, an International Classification of Diseases, Ninth Revision, Clinical Modification (ICD‐9‐CM) diagnosis code was used. We excluded hospitalizations with myocardial infarction, coronary artery disease, and ischemic cardiomyopathy. Cardiovascular manifestations were defined by the presence of diagnosis codes for conduction disorders, arrhythmias, heart failure, nonischemic cardiomyopathy, and pulmonary hypertension. A total of 609 051 sarcoidosis hospitalizations were identified, with an age of 55±14 years, 67% women, and 50% black. The number of sarcoidosis hospitalizations increased from 2005 through 2014 (138 versus 175 per 100 000, P trend<0.001). We observed declining trends of unadjusted in‐hospital mortality (6.5 to 4.9 per 100 sarcoidosis hospitalizations, P trend<0.001). Overall ≈31% (n=188 438) of sarcoidosis hospitalizations had coexistent cardiovascular manifestations of one or more type. Heart failure (≈16%) and arrhythmias (≈15%) were the most prevalent cardiovascular manifestations. Rates of implantable cardioverter‐defibrillator placement were ≈7.5 per 1000 sarcoidosis hospitalizations (P trend=0.95) during the study period. Black race was associated with 21% increased risk of in‐hospital mortality (odds ratio, 1.21; 95% confidence interval, 1.16–1.27 [P<0.001]).ConclusionsSarcoidosis hospitalizations have increased over the past decade with a myriad of coexistent cardiovascular manifestations. Black race is a significant predictor of in‐hospital mortality, which is declining. Further efforts are needed to improve care in view of low implantable cardioverter‐defibrillator rates in sarcoidosis.

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Section: Discussionmentioning

confidence: 98%

“…It is well recognized that age is perhaps the most significant role player in the development of clinical cardiovascular disease 28, 29. Thus, it follows with sarcoidosis.…”

Section: Discussionmentioning

confidence: 99%

Hospitalization Rates, Prevalence of Cardiovascular Manifestations, and Outcomes Associated With Sarcoidosis in the United States

Patel

Kalra

Doshi

et al. 2018

JAHA

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“…Our study is the first to include data on T2‐weighted oedema imaging. Active myocardial inflammation may increase arrhythmogenicity, but conflicting data exist concerning the efficacy of current immune‐suppressive and anti‐arrhythmic treatment in actually improving long‐term outcomes in patients with active disease 11, 12, 18, 22, 24, 25, 26. The presence of increased T2 signal in our cohort, managed with corticosteroids and methotrexate, did not correlate with adverse events during follow‐up.…”

Section: Discussionmentioning

confidence: 60%

“…Patel's cohort mainly consisted of African American women with RV LGE in 67%, a potentially high‐risk scenario. Long‐term outcomes in patients with CS have markedly improved because of modern heart failure management, including device therapy and arrhythmia ablation in selected patients 10, 11, 18, 25, 26. Current annual mortality rates range from 0–4.2/100 patients compared with 7.5–12/100 patients as previously reported by Yazaki (2001) and Fleming (1987) 22.…”

Section: Discussionmentioning

confidence: 82%

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Right ventricular involvement and the extent of left ventricular enhancement with magnetic resonance predict adverse outcome in pulmonary sarcoidosis

et al. 2017

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AimsCardiac involvement is the main determinant of poor outcomes in sarcoidosis. Right ventricular (RV) dysfunction and left ventricular (LV) late gadolinium enhancement (LGE) have been reported to be predictive of adverse outcome in non‐ischaemic cardiomyopathies. The aim of our study was to determine whether delayed RV LGE with cardiovascular magnetic resonance would be predictive of adverse events in addition to LV LGE during the long‐term follow‐up of pulmonary sarcoidosis patients.Methods and resultsEighty‐four consecutive biopsy‐proven pulmonary sarcoidosis patients were followed for a median of 56 months [38–74] after baseline delayed contrast‐enhanced cardiac magnetic resonance. The composite primary endpoint consisted of admission for congestive heart failure, sustained ventricular tachycardia, appropriate implantable cardioverter defibrillator therapy, pacemaker implantation for high degree atrio‐ventricular block, or cardiac death. The composite secondary endpoint included all‐cause mortality in addition to the primary endpoint. RV and LV LGE were demonstrated in respectively 12 and 27 patients. Five of 10 events included in the primary endpoint occurred in the group with RV LGE. RV LGE, LV, or biventricular LGE yielded Cox hazard ratios of 8.71 [95% confidence interval (CI) 1.90–23.81], 9.22 (95% CI 1.96–43.45), and 12.09 (95% CI 3.43–42.68) for the composite primary endpoint. In a multivariate model, the predictive value of biventricular LGE for the composite primary and secondary endpoints was strongest. Kaplan–Meier event‐free survival curves were most significant for RV LGE and biventricular LGE (log rank with P < 0.001).ConclusionsBiventricular LGE at presentation is the strongest, independent predictor of adverse outcome during long‐term follow‐up. Asymptomatic myocardial scar <8% of LV mass carried a favourable long‐term outcome.

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

Fussner

Karlstedt

Hodge

et al. 2018

European J of Heart Fail

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AimsCardiac 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.Methods and resultsWe 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).ConclusionIn 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.

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Cardiac Sarcoidosis

Cited by 85 publications

References 28 publications

Hospitalization Rates, Prevalence of Cardiovascular Manifestations, and Outcomes Associated With Sarcoidosis in the United States

Hospitalization Rates, Prevalence of Cardiovascular Manifestations, and Outcomes Associated With Sarcoidosis in the United States

Right ventricular involvement and the extent of left ventricular enhancement with magnetic resonance predict adverse outcome in pulmonary sarcoidosis

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

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