Myocardial Involvement in Patients With Histologically Diagnosed Cardiac Sarcoidosis: A Systematic Review and Meta‐Analysis of Gross Pathological Images From Autopsy or Cardiac Transplantation Cases

Abstract

Background In patients with suspected cardiac sarcoidosis, late gadolinium enhancement on cardiovascular magnetic resonance imaging and/or 18 F‐fluorodeoxyglucose uptake on positron emission tomography are often used to reach a clinical diagnosis of cardiac sarcoidosis. On the basis of data from the imaging literature of clinical cardiac sarcoidosis, no specific features of myocardial involvement are regarded as pathognomonic for cardiac sarcoidosis. Thus, a diagnosis of cardiac sarcoidosis is challenging to make. There has been no systematic analysis of histologically diagnosed cardiac sarcoidosis for patterns of myocardial involvement. We hypothesized that certain patterns of myocardial involvement are more frequent in histologically diagnosed cardiac sarcoidosis. Methods and Results We performed a systematic review and meta‐analysis of gross pathological images from the published literature of patients with histologically diagnosed cardiac sarcoidosis who underwent autopsy or cardiac transplantation. Thirty‐three eligible articles provided images of 49 unique hearts. Analysis of these hearts revealed certain features of myocardial involvement in >90% of cases: left ventricular (LV) subepicardial, LV multifocal, septal, and right ventricular free wall involvement. In contrast, other patterns were seen in 0% to 6% of cases: absence of gross LV myocardial involvement, isolated LV midmyocardial involvement, isolated LV subendocardial involvement, isolated LV transmural involvement, absence of septal involvement, or isolated involvement of only one LV level. Conclusions In this systematic review and meta‐analysis of histologically diagnosed cardiac sarcoidosis, we identified certain features of myocardial involvement that occurred frequently and others that occurred rarely or never. These patterns could aid the interpretation of cardiovascular magnetic resonance imaging and positron emission tomography imaging and improve the diagnosis and the prognostication of patients with suspected cardiac sarcoidosis.

Challenges in diagnosing cardiac sarcoidosis: should we increase our index of suspicion?

“…A meta-analysis of histologically diagnosed CS concluded that there were certain patterns of myocardial involvement in >90% of cases. These findings can improve the diagnosis and prognostication of patients with suspected CS, using CMR and FDG-PET [1].…”

“…Sarcoidosis is characterized by a non-caseating granuloma, which is formed via an inflammatory reaction resulting in an accumulation of epithelial cells, monocytes, lymphocytes, macrophages and fibroblasts [1]. There are various histological subtypes [1].…”

“…Sarcoidosis is characterized by a non-caseating granuloma, which is formed via an inflammatory reaction resulting in an accumulation of epithelial cells, monocytes, lymphocytes, macrophages and fibroblasts [1]. There are various histological subtypes [1]. Pulmonary sarcoidosis (PS) affects the upper lobes of the lung and is associated with hilar and mediastinal adenopathy [2].…”

Clinical characteristics and organ system involvement in sarcoidosis: comparison of the University of Minnesota Cohort with other cohorts

“…Similarly, patchy uptake on a dedicated cardiac PET scan has also been incorporated into the HRS and the WASOG criteria. A crucial element in establishing a diagnosis of cardiac sarcoidosis, even with histology proven extra-cardiac disease, is excluding other causes of LGE, such as coronary artery disease or other non-ischemic cardiomyopathies such as genetic arrhythmogenic cardiomyopathies which could also demonstrate increased myocardial uptake on FDG-PET imaging [42]. Challenges also remain regarding the ideal dietary preparation to suppress normal glucose uptake by the myocardium and avoid false positive FDG-PET.…”

Cardiac sarcoidosis: modern diagnostics and therapy

“…При этом излюбленной локализацией является стенка левого желудочка (ЛЖ) (преимущественно ее средняя часть и субэпикардиальная область) и межжелудочковая перегородка (МЖП), особенно базальные сегменты [2]. Описаны редкие случаи вовлечения перикарда (с возможным плевральным выпотом) и эндокарда (в т. ч. и клапанного), но обычно при распространении из толщи миокарда [10].…”