Isolated cardiac sarcoidosis: A focused review of an under-recognized entity.
There is accumulating evidence for the existence of a phenotype of isolated cardiac sarcoidosis (ICS), or sarcoidosis that only involves the heart. In the absence of biopsy-confirmed cardiac sarcoidosis (CS), existing diagnostic criteria require the presence of extra-cardiac sarcoidosis as an inclusion criterion for the diagnosis of CS. Consequently, in the absence of a positive endomyocardial biopsy, ICS is not diagnosable by current guidelines. Therefore, there is uncertainty regarding the epidemiology, pathobiology, clinical characteristics, prognosis, and optimal treatment of ICS. This review will summarize the available data related to the prevalence and prognosis of ICS and will discuss challenges surrounding the diagnosis and management of this under-recognized entity.
Division of Cardiology, Department of Medicine, Johns Hopkins Hospital, Baltimore, MD, USA.
David R
DR
Okada
Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital, 75 Francis St., Boston, MA, 02115, USA.
Paco E
PE
Bravo
Department of Radiology, Brigham and Women's Hospital, Boston, MA, USA.
Tomas
T
Vita
Department of Radiology, Brigham and Women's Hospital, Boston, MA, USA.
Vikram
V
Agarwal
Cardiology Division, Department of Medicine, Massachusetts General Hospital, Boston, MA, USA.
Michael T
MT
Osborne
Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital, 75 Francis St., Boston, MA, 02115, USA.
Viviany R
VR
Taqueti
Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital, 75 Francis St., Boston, MA, 02115, USA.
Hicham
H
Skali
Division of Cardiovascular Diseases, Department of Medicine, Mayo Clinic, Rochester, MN, USA.
Panithaya
P
Chareonthaitawee
Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital, 75 Francis St., Boston, MA, 02115, USA.|Department of Radiology, Brigham and Women's Hospital, Boston, MA, USA.
Sharmila
S
Dorbala
Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital, 75 Francis St., Boston, MA, 02115, USA.
Garrick
G
Stewart
Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital, 75 Francis St., Boston, MA, 02115, USA.|Department of Radiology, Brigham and Women's Hospital, Boston, MA, USA.
Marcelo
M
Di Carli
Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital, 75 Francis St., Boston, MA, 02115, USA. rblankstein@partners.org.|Department of Radiology, Brigham and Women's Hospital, Boston, MA, USA. rblankstein@partners.org.
Ron
R
Blankstein
D001706:Biopsy; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D049268:Positron-Emission Tomography; D011379:Prognosis; D012507:Sarcoidosis / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy
Cardiac sarcoidosis; cardiac MRI; cardiac PET