Clinical Features and Diagnosis of Cardiac Sarcoidosis

Abstract

Cardiac sarcoidosis (CS) is an unusual, but potentially harmful, manifestation of systemic sarcoidosis (SA), a chronic disease characterized by organ involvement from noncaseating and nonnecrotizing granulomas. Lungs and intrathoracic lymph nodes are usually the sites that are most frequently affected, but no organ is spared and CS can affect a variable portion of SA patients, up to 25% from post-mortem studies. The cardiovascular involvement is usually associated with a bad prognosis and is responsible for the major cause of death and complications, particularly in African American patients. Furthermore, the diagnosis is often complicated by the occurrence of non-specific clinical manifestations, which can mimic the effect of more common heart disorders, and imaging and biopsies are the most valid approach to avoid misdiagnosis. This narrative review summarizes the main clinical features of CS and imaging findings, particularly of CMR and 18-Fluorodeoxyglucose Positron Emission Tomography (18F-FDG PET) that can give the best cost/benefit ratio in terms of the diagnostic approach. Imaging can be very useful in replacing the endomyocardial biopsy in selected cases, to avoid unnecessary, and potentially dangerous, invasive maneuvers.

Comorbidities of sarcoidosis

“…FDG-PET scan is useful to detect the presence of active myocardial inflammation, and is usually performed if CMR shows evidence of cardiac involvement or there is a high degree of clinical suspicion of CS [ 86 ]. Despite this, the recommendation for serial PET scans to monitor cardiac sarcoidosis is controversial.…”

Sarcoidosis and COVID-19: At the Cross-Road between Immunopathology and Clinical Manifestation

“…Both pathologies can cause cardiac involvement. While cardiac sarcoidosis is characterized by the presence of myocyte necrosis (acute phase) and interstitial fibrosis (chronic phase) with both non-ischemic (intramyocardial or subepicardial) and ischemic (subendocardial or transmural) patterns in more than one coronary territory, peri-myocarditis following Coronavirus Disease 2019 (COVID-19) revealed a typical mid-subepicardial non-ischemic pattern mainly at the left ventricle lateral wall [ 87 , 88 , 89 , 90 , 91 ].…”

Clinical Features, Histopathology and Differential Diagnosis of Sarcoidosis

“…Echocardiographic abnormalities can include some nonspecific findings such as wall thickening, segmental abnormalities or ventricular aneurysms. Cardiac magnetic resonance imaging (cMRI) can instead reveal with higher accuracy the presence of edema as gadolinium uptake at T2-weighted images, and findings of chronic disease such as thinning of basal septum wall, ventricular dilatation and systolic dysfunction [ 3 ].…”

“…Recent data about epidemiology show that Sweden is the country at the highest incidence and prevalence (respectively, 11 and 160 per 100,000 inhabitants), while it is rarely found in countries such as Japan and South Korea (1–5 per 100,000) [ 2 ] The worst prognosis is found in African Americans, since they can have an early cardiac involvement with high risk of complications, hospitalization and mortality [ 3 ]. Furthermore, black people can more frequently experience a multiorgan involvement and an aggressive form of pulmonary disease [ 4 ].…”