Vascular Permeability Assay in Human Coronary and Mouse Brachiocephalic Arteries.

Coronary artery disease remains an important cause of morbidity and mortality. Previous work, including ours, has focused on the role of intraplaque hemorrhage, particularly from immature microvessel angiogenesis, as an important contributor to plaque progression via increases in vascular permeability leading to further intraplaque hemorrhage, which increases red cell membrane-derived free cholesterol in plaque content and inflammatory cell recruitment. Evans Blue Dye (EBD) assay is widely used as a standard assay for vasculature permeability. However, the method has not been established in fresh human coronary artery autopsy samples to evaluate intraplaque microvessel permeability and angiogenesis. In this protocol, we describe a method to evaluate human coronary samples for microvascular permeability, including procedures to perfuse coronary arteries, collection of artery samples for histological analysis and immunostaining as well as the use of appropriate methodology to analyze the images. An optional procedure is also provided for the use of FITC-dextran in mouse model to evaluate vascular permeability. These Evans Blue Dye procedures may be useful in providing functional measure of the endothelium integrity and permeability in both human samples and animal models in various pathological conditions.

Cardiovascular pathologies in mucopolysaccharidosis type VII (Sly Syndrome).

We present the cardiac findings from the autopsy of a 28-year-old male with mucopolysaccharidosis VII (MPS VII), also known as Sly Syndrome, whose diagnosis was confirmed by biochemical testing. The patient died a sudden cardiac death. Autopsy showed thickened and stenotic aortic valve leaflets as well as marked concentric intimal thickening of the aorta and muscular arteries. There was left ventricular hypertrophy as well as mild papillary muscle thickening and fusion. Increased colloid iron staining was seen in the small- and medium-sized arteries of the heart and at the intercalated discs. We discuss the patient's premortem echocardiographic and electrocardiographic studies. In addition, we discuss the pathogenesis of MPS VII and review previous literature on its anatomic and pathologic features.

Fetal death resulting from an isolated congenital partial pericardial defect.

BACKGROUND: Congenital pericardial defects occur from a defect in the formation of the pleuropericardial membrane during embryonic development. This defect may be asymptomatic but can be fatal if complicated by herniation of any portion of the heart. CASES: We report two cases in which herniation of a portion of the heart occurred through a partial left pericardial defect and resulted in fetal death. In case one, there were no fetal symptoms, and in case two, an irregular heartbeat was detected prompting a fetal echocardiogram that was negative for heart abnormalities. CONCLUSION: Although isolated congenital pericardial defects are rare, they can result in fetal death. Awareness may help to refine ultrasonography or other diagnostic modalities to evaluate possible congenital pericardial defects in utero.