Evaluation and Management of Heart Rhythm Disturbances Due to Cardiac Sarcoidosis

Bridging the Knowledge Gaps in Arrhythmogenic Cardiovascular Conditions: The Critical Role of Registries

“…CS affects a small minority of patients with pulmonary or systemic sarcoidosis, but when present is often associated with a spectrum of clinically significant conduction abnormalities and arrhythmias. [57][58][59][60][61][62][63][64][65][66][67][68] Unlike other inherited arrhythmogenic cardiovascular conditions reviewed, sarcoidosis is not clearly identified as an inherited disorder. However, epidemiologic data suggest some genetic predisposition.…”

“…However, epidemiologic data suggest some genetic predisposition. 57,60 It has become evident through registry data that the cardinal manifestations of CS are conduction disturbances and arrhythmias. [57][58][59][60][61][62][63][64][65][66][67][68] Although sarcoidosis is typically a multisystem granulomatous disease with cardiac involvement in <10 % of patients with multisystem disease, isolated CS is a definite clinical entity.…”

“…57,60 It has become evident through registry data that the cardinal manifestations of CS are conduction disturbances and arrhythmias. [57][58][59][60][61][62][63][64][65][66][67][68] Although sarcoidosis is typically a multisystem granulomatous disease with cardiac involvement in <10 % of patients with multisystem disease, isolated CS is a definite clinical entity. [57][58][59][60][61][62][63][64][65][66][67][68][69] As CS commonly presents with life-threatening heart block, malignant arrhythmias, and congestive heart failure, detection and appropriate treatment are essential to ensure the best possible patient outcomes.…”

“…Registries have played an essential role in filling the knowledge gaps in many domains related to CS. [57][58][59][60][61][62][63][64][65][66][67][68][69] A recent consensus statement provides guidance for clinicians on the diagnosis and management of arrhythmias associated with CS including indications for ICDs. 57 The current diagnostic criteria, natural history, and risk stratification process for CS are all based on data from multiple registries.…”

“…57 In the therapeutic domain, pacemakers, ICD implantation, and early implementation of corticosteroid therapy have led to an improvement in the overall prognosis and clinical outcomes of CS based on observations from registries. [57][58][59][60][61][62][63][64][65][66][67][68][69] Contemporary registries contributed to the recent establishment of CS diagnostic criteria in 2014. 57,58 The 2014 criteria place greater emphasis on patients with proven systemic sarcoidosis, which is supported by the findings of Schuller et al 58 Blankstein et al showed a correlation between 18F-fluorodeoxyglucose (18F-FDG) uptake on positron emission tomography (PET) scans and poor outcomes, thus allowing patients to now be officially diagnosed via PET scan evidence.…”