Complementary Role of CMR to Conventional Screening in the Diagnosis and Prognosis of Cardiac Sarcoidosis.
The goal of this study was to assess the independent and collective diagnostic value of various modalities in cardiac sarcoidosis, delineate the role of cardiac magnetic resonance (CMR), and identify patients at risk.



Cardiac sarcoidosis is associated with increased morbidity and mortality. CMR is a key modality in the evaluation of patients with cardiac symptoms, but the complementary role of CMR to conventional tests for the diagnosis of cardiac sarcoidosis is not fully defined.



Patients (N = 321) with biopsy-proven sarcoidosis underwent conventional cardiac testing and CMR with late gadolinium enhancement (LGE) and were followed up for primary (composite of all-cause mortality, sustained ventricular tachycardia [VT] episodes, or hospitalization for heart failure) and secondary (nonsustained VT episodes) endpoints.



Cardiac sarcoidosis was diagnosed in 29.9% of patients according to the Heart Rhythm Society consensus criteria. CMR was the most sensitive and specific test (area under the curve: 0.984); it detected 44 patients with cardiac symptoms and/or electrocardiogram (ECG) abnormalities but normal echocardiogram, as well as 15 asymptomatic patients with normal baseline testing. Echocardiography added to cardiac history and ECG did not change sensitivity of the initial screening strategy (68.8% vs. 72.9%). Despite a high positive predictive value (83.9%), echocardiography had a low sensitivity (27.1%). During follow-up, 7.2% of patients reached the primary endpoint and another 3.4% reached the secondary endpoint. LGE was and independent predictor of primary endpoints (hazard ratio: 5.68; 95% CI: 1.74 to 18.49; p = 0.004). LGE, age, and baseline nonsustained VT were independent predictors of all events. In patients with cardiac symptoms and/or an abnormal ECG, CMR increased diagnostic accuracy and independently predicted primary endpoints (hazard ratio: 12.71; 95% confidence interval: 1.48 to 109.35; p = 0.021).



Of all cardiac tests, CMR was the most valuable in the diagnosis and prognosis of cardiac sarcoidosis in a general sarcoidosis population. Echocardiography had an overall limited diagnostic value as a screening test, and an abnormal study, despite a high positive predictive value, may still need confirmation with CMR.
Outpatient Sarcoidosis Clinic, General Hospital of Chest Diseases "Sotiria," Athens, Greece; Interstitial Lung Disease Unit, Royal Brompton Hospital, London, United Kingdom. Electronic address: billkouranos@gmail.com.
Vasileios
V
Kouranos
Department of Pathophysiology, Laiko Hospital, University of Athens Medical School, Athens, Greece.
George E
GE
Tzelepis
Outpatient Sarcoidosis Clinic, General Hospital of Chest Diseases "Sotiria," Athens, Greece.
Aggeliki
A
Rapti
Onassis Cardiac Surgery Center, Athens, Greece.
Sofia
S
Mavrogeni
1(st) Cardiology Department, University of Athens Medical School, Athens, Greece.
Konstantina
K
Aggeli
Bioiatriki Magnetic Resonance Unit, Athens, Greece.
Marousa
M
Douskou
Cardiac MRI Department, Royal Brompton Hospital, London, United Kingdom.
Sanjay
S
Prasad
First Department of Respiratory Medicine, University of Athens, Medical School, General Hospital of Chest Diseases "Sotiria," Athens, Greece.
Nikolaos
N
Koulouris
First Department of Propaedeutic and Internal Medicine, Laiko Hospital, University of Athens Medical School, Athens, Greece.
Petros
P
Sfikakis
Interstitial Lung Disease Unit, Royal Brompton Hospital, London, United Kingdom.
Athol
A
Wells
Outpatient Sarcoidosis Clinic, General Hospital of Chest Diseases "Sotiria," Athens, Greece.
Elias
E
Gialafos
D000328:Adult; D019540:Area Under Curve; D001706:Biopsy; D009202:Cardiomyopathies / Q000150:complications / Q000000981:diagnostic imaging* / Q000401:mortality / Q000473:pathology; D002423:Cause of Death; D003287:Contrast Media / Q000008:administration & dosage; D004452:Echocardiography; D015716:Electrocardiography, Ambulatory; D005260:Female; D006333:Heart Failure / Q000209:etiology; D006760:Hospitalization; D006801:Humans; D053208:Kaplan-Meier Estimate; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology*; D011237:Predictive Value of Tests; D011379:Prognosis; D012372:ROC Curve; D015203:Reproducibility of Results; D012307:Risk Factors; D012507:Sarcoidosis / Q000150:complications / Q000000981:diagnostic imaging* / Q000401:mortality / Q000473:pathology; D017180:Tachycardia, Ventricular / Q000209:etiology; D013997:Time Factors
D003287:Contrast Media
cardiac MRI; cardiac sarcoidosis; diagnosis; echocardiography; screening