PubMed · 6553939
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C V Ram. 1983. Hypertensive crises.. https://pubmed.ncbi.nlm.nih.gov/6553939/
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Dissecting aortic aneurysm is the hallmark of Marfan syndrome (MFS) and the result of mutations in fibrillin-1, the major constituent of elastin-associated extracellular microfibrils. It is yet to be established whether dysfunction of fibrillin-1 perturbs the ability of the elastic vessel wall to sustain hemodynamic stress by disrupting microfibrillar assembly, by impairing the homeostasis of established elastic fibers, or by a combination of both mechanisms. The pathogenic sequence responsible for the mechanical collapse of the elastic lamellae in the aortic wall is also unknown. Targeted mutation of the mouse fibrillin-1 gene has recently suggested that deficiency of fibrillin-1 reduces tissue homeostasis rather than elastic fiber formation. Here we describe another gene-targeting mutation, mgR, which shows that underexpression of fibrillin-1 similarly leads to MFS-like manifestations. Histopathological analysis of mgR/mgR specimens implicates medial calcification, the inflammatory-fibroproliferative response, and inflammation-mediated elastolysis in the natural history of dissecting aneurysm. More generally, the phenotypic severity associated with various combinations of normal and mutant fibrillin-1 alleles suggests a threshold phenomenon for the functional collapse of the vessel wall that is based on the level and the integrity of microfibrils.
Penetrating aortic ulcers and intramural hematomas cannot always be differentiated by transesophageal echocardiography. We suggest that color and pulsed-wave Doppler flow can be used for identifying penetrating aortic ulcers.
We report a case of acute aortic dissection that occurred in the late course of surgically untreated atrial septal defect. A 60-year-old man with acute aortic dissection and atrial septal defect was operated on successfully, and we discuss the causal relationship between these two unusual conditions.