Impact of late gadolinium enhancement extent, location, and pattern on ventricular tachycardia and major adverse cardiac events in patients with ischemic vs. non-ischemic cardiomyopathy.

Background: Left ventricular late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) has been associated with increased risk for life-threatening ventricular tachyarrhythmias. The differences in association between LGE characteristics and prognosis in patients with ischemic (ICM) vs. non-ischemic (NICM) cardiomyopathy is incompletely understood. Methods: A total of 168 consecutive patients who underwent CMR imaging with either ICM or NICM were included in our study. LGE extent, location and pattern were examined for association to the primary endpoint of ventricular tachycardia (VT) and secondary endpoint of major adverse cardiac events (MACE). Results: Of 68 (41%) patients with ICM and 97 (59%) patients with NICM, median LGE mass was 15% (IQR 9-28) for the ICM group and 10% (IQR 6-15) for the NICM group. On multivariate analysis for both groups, LGE characteristics were prognostic while LVEF was not. In patients with ICM, septal and apical segment LGE, and involvement of multiple walls predicted both endpoints on multivariate analysis. LGE extent (≥median) and inferior wall LGE independently predicted the primary endpoint. In patients with NICM, anterior, inferior and apical segment LGE, and involvement of multiple walls predicted both endpoints on multivariate analysis. LGE extent (≥median, number of LGE segments, LGE stratified per 5% increase) and midwall LGE were independent predictors of the primary endpoint. Conclusions: Although LGE was an independent predictor of prognosis in both groups, LGE extent, location, and pattern characteristics were more powerful correlates to worse outcomes in patients with NICM than ICM.

Rare primary pulmonary angiosarcoma with aberrant neuroendocrine differentiation.

Angiosarcomas are aggressive, malignant endothelial cell tumors derived from the vascular or lymphatic epithelium. Angiosarcomas makes up less than 1% of all soft tissue sarcomas. Primary pulmonary angiosarcoma is a rare tumor that most commonly presents as metastatic disease and carries a grim prognosis. The disease rarity and low index of suspicion make the diagnosis problematic. In this report, we present a case of primary pulmonary angiosarcoma with aberrant neuroendocrine differentiation. The diagnostic images, pathology and management strategy are discussed in this case with overall longer survival. The unique neuroendocrine pathologic subtype may contribute to the overall longer survival, however, there are only a few limited cases published. Further recognition and investigation of primary pulmonary angiosarcoma with aberrant neuroendocrine differentiation is warranted to further characterize diagnostic markers, determine more effective treatment strategies, and establish a possible link to improved survival.