Sudden death in cardiac sarcoidosis: an analysis of nationwide clinical and cause-of-death registries

Abstract

Aims The present study was done to assess the role of sudden cardiac death (SCD) among the presenting manifestations of and fatalities from cardiac sarcoidosis (CS). Methods and results We analysed altogether 351 cases of CS presenting from year 1998 through 2015 in Finland. There were 262 patients with a clinical diagnosis and treatment of CS, 27 patients with an initial lifetime diagnosis of giant cell myocarditis that was later converted to CS, and 62 cases detected at autopsy and identified by screening >820 000 death certificates from the national cause-of-death registry. The total case series comprised 253 females and 98 males aged on average 52 years at presentation. High-grade atrioventricular block was the most common first sign of CS (n = 147, 42%) followed by heart failure (n = 58, 17%), unexpected fatal (n = 38) or aborted (n = 12) SCD (14%), and sustained ventricular tachycardia (n = 48, 14%). Severe coronary artery disease was found at autopsy concomitant with CS in four of the 38 cases presenting with fatal SCD. Of all deaths recorded till the end of 2015, 64% (n = 54/84) were unexpected SCDs from CS that had either been silent during life or defied all attempts at diagnosis. The Kaplan–Meier estimate (95% CI) of survival from symptom onset was 85% (80–90%) at 5 years and 76% (68–84%) at 10 years. Conclusion Together fatal and aborted SCD constitute 14% of the presenting manifestations of CS. Nearly two-thirds of all fatalities from CS are caused by undiagnosed granulomas in the heart.

Folate Receptor β–Targeted PET Imaging of Macrophages in Autoimmune Myocarditis

“…Myocardial samples from five patients who died of cardiac sarcoidosis and one heart explanted due to sarcoidosis described earlier (19) were cut into serial 4 μm paraffin sections. Sections were immunohistochemically stained with an anti-CD68 antibody to detect macrophages and with an anti-FR-β antibody to study localization of FR-β.…”

“…Double immunofluorescence staining was performed with an anti-CD68 antibody and with an anti-FR-β antibody (20). The use of human tissues was done in accordance with Finnish law, the principles of Declaration of Helsinki, and was approved by The National Authority for Medicolegal Affairs and the National Institute for Health and Welfare (19).…”

Role of CMR Mapping Techniques in Cardiac Hypertrophic Phenotype

“…Noncaseating granulomas are the histopathological hallmark, typically affecting the lungs, as up to 90% of patients could present pulmonary involvement [ 74 ]; however, being multisystemic, also other organs as cardiac muscle, liver, spleen, eyes, parotid gland, and skin can be involved. Cardiac sarcoidosis (CS) represents one of the most relevant drivers of prognosis, considering the high rate of atrioventricular blocks, heart failure development and sudden cardiac death if heart is affected, as demonstrated by Ekstrӧm et al in a large registry of 351 subjects with definite diagnosis of CS [ 75 ]. In light of such potential negative prognosis, timely diagnosis of myocardial involvement is of paramount clinical relevance, and thus an updated Expert Consensus Document was released in 2014 suggesting diagnostic algorithms in several scenarios in which a cardiac localization could be present [ 76 ]: according to clinical likelihood of CS (clinical history, EKG findings), CMR is suggested as first or second line test.…”

Cardiac magnetic resonance imaging to predict arrhythmic events in cardiac sarcoidosis: Unmasking the great pretender?

“…Cardiac sarcoidosis can produce both abnormalities of cardiac conduction/atrioventricular (AV) block and ventricular arrhythmias, both of which may be life threatening . Treatment of resulting tachyarrhythmias and brady‐arrhythmias may require permanent pacing and/or the use of implantable cardiac defibrillators .…”

“…both of which may be life threatening. 1 Treatment of resulting tachyarrhythmias and brady-arrhythmias may require permanent pacing and/or the use of implantable cardiac defibrillators. 2 In this regard, better risk stratification to identify patients prone to such arrhythmic events is therefore of great clinical importance to guide appropriate device therapy.…”