Diagnostic utility of fusion 18F-fluorodeoxyglucose positron emission tomography/cardiac magnetic resonance imaging in cardiac sarcoidosis.

BACKGROUND: Although each 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) and cardiac magnetic resonance (CMR) imaging with late gadolinium enhancement (LGE) has been used to diagnose cardiac sarcoidosis (CS), active CS is still misdiagnosed. METHODS: Active CS, diagnosed by PET alone, was defined as focal or focal on diffuse FDG uptake pattern. In fusion PET/CMR imaging, using a regional analysis with AHA 17-segment model, the patients were categorized into four groups: (1) PET-/LGE-, (2) PET+/LGE-, (3) PET+/LGE+, and (4) PET-/LGE+. PET+/LGE+ was defined as active CS. RESULTS: 74 Patients with suspected CS were enrolled. Between PET alone and fusion PET/CMR imaging, 20 cases had mismatch evaluations of active CS, and most had diffuse or focal on diffuse FDG uptake pattern on PET alone imaging. 40 Patients fulfilled the 2016 the Japanese Circulation Society diagnostic criteria for CS. The interobserver diagnostic agreement was excellent (κ statistics 0.89) and the overall accuracy for diagnosing CS was 87.8% in fusion PET/CMR imaging, which were superior to those in PET alone imaging (0.57 and 82.4%, respectively). In a sub-analysis of diffuse and focal on diffuse patterns, the agreement (κ statistics 0.86) and overall accuracy (81.8%) in fusion PET/CMR imaging were still better. CONCLUSIONS: Fusion PET/CMR imaging with regional analysis offered reliable and accurate diagnosis of CS, covering low diagnostic area by FDG-PET alone.

Increased Left Ventricular Trabeculation Is Associated With Increased B-Type Natriuretic Peptide Levels and Impaired Outcomes in Nonischemic Cardiomyopathy.

BACKGROUND: The clinical significance of left ventricular (LV) trabeculation remains unknown in cardiomyopathies. B-Type natriuretic peptide (BNP) strongly reflects LV end-diastolic wall stress and is a useful prognostic marker of cardiovascular diseases. The enhanced identification of LV trabeculae (T) with the use of cardiac magnetic resonance and the evaluation of its relationship with BNP may elucidate the biologic significance and clinical impact of trabeculation in patients with nonischemic cardiomyopathy (NICM). METHODS: The LV volume and mass of 515 patients with NICM and 36 control subjects were analyzed with the use of a steady-state free precession sequence, and individual T mass was planimetred. Major adverse cardiac events (MACE) were assessed. RESULTS: T mass index correlated with LV end-diastolic volume index (EDVI), LV mass index, and papillary muscle mass index (all P < 0.001). Also, T mass index was positively correlated with BNP level (R = 0.381; P < 0.001) and was an independent determinant of BNP after adjusting for age, sex, body mass index (BMI), etiology, LV ejection fraction, and LV EDVI (P < 0.001). Kaplan-Meier analysis during a median follow-up of 17.3 months showed that higher T mass index and increased BNP level correlated with MACE. On multivariate analysis, T mass index (P = 0.031) and BNP (P < 0.001) remained associated with poor outcomes when combined with age, sex, BMI, and etiology. CONCLUSIONS: Increased LV trabeculation was associated with LV dysfunction/remodelling and impaired outcomes in NICM of various etiologies. This may support the biologic significance of LV trabeculation and could be attributed to its association with BNP through LV wall stress.

Right ventricular involvement is an important prognostic factor and risk stratification tool in suspected cardiac sarcoidosis: analysis by cardiac magnetic resonance imaging.

BACKGROUND: Late gadolinium enhancement imaging (LGE) of the left ventricle (LV) by cardiac magnetic resonance (CMR) has prognostic value for patients with cardiac sarcoidosis (CS). Right ventricle (RV) dysfunction is also associated with adverse outcomes in patients with heart failure. Therefore, we sought to determine if RV LGE and dysfunction predicted adverse events in patients with suspected CS. METHODS: In 103 consecutive patients with suspected CS who underwent CMR, functional and remodeling indexes of both the LV and RV were measured and the extent and localization of LGE were also analyzed. Major adverse cardiac events (MACE) were defined as cardiovascular mortality, severe ventricular tachyarrhythmia, hospitalization with heart failure, and advanced atrioventricular block. RESULTS: During a median follow-up of 20.6 months, Kaplan-Meier analysis showed that decreased RV ejection fraction (EF) was associated with MACE (P < 0.001) and receiver operating characteristics curve (ROC) analysis indicated good predictive performance of RV EF for MACE (area under the ROC = 0.834). RV EF operated independently of LV EF or LGE extent for predicting MACE. In addition, the presence of LGE in RV was independently associated with MACE (P = 0.011), and a combined analysis of RV EF and RV LGE showed better risk stratification for MACE (P < 0.001). CONCLUSIONS: Both RV EF and LGE were independently associated with MACE and enhanced risk stratification in patients with suspected CS. CMR may be a useful tool for detecting myocardial function and fibrosis in both the LV and RV.

Clinical and histological features of different types of Budd-Chiari syndrome: a comparison of 4 cases.

Budd-Chiari syndrome (BCS) is a rare condition characterized by hepatic venous outflow obstruction. In this report, we present 4 cases of BCS with complete and incomplete obstruction of the inferior vena cava (IVC) and hepatic vein (HV). Each case showed different and unique features of liver damage, which were attributed to the site and degree of obstruction. Interestingly, improved liver functions such as increased serum albumin levels, decreased hyaluronic acid levels and a normal indocyanine green clearance test were evident within 1 month of the balloon angioplasty. Pericellular fibrosis and hypervascular regenerative nodules were also reversible after obstruction removal. Therefore, it is very important to manage this rare disease before it progresses to liver cirrhosis.