PMC8652058_01
A pre-operative electrocardiogram showed sinus rhythm with a heart rate of 75 beats per minute and p mitrale (noted by the negative q wave deflection of more than 1 mm in the lead V1, suggestive of left atrial enlargement).
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F1
The electrocardiogram showed sinus rhythm with a rate of 75 per minute and p mitrale (Figure 1).
Diagnosis of two fusiform aortic aneurysms. (A) Computerized tomography (CT) aortogram section (axial view) below the level of the aortic arch showed marked dilatation of the ascending (A1) and the descending (A2) aorta (scale 1:5). (B) CT aortogram shows the sagittal view of the dilated aneurysms as described in (A). (C) Reconstructed maximum intensity projection images of a contrast-enhanced CT showed 2 fusiform aneurysms. The larger aneurysm (A1) ranges for a length of about 12 cm, involving the ascending aorta and extending to proximal arch along with root involvement. The smaller (A2) aneurysm was noted extending from the distal arch to the proximal descending aorta (scale 1:5). The prominent "sausage link appearance" (*), can be appreciated between the 2 aneurysms. The rest of the descending aorta showed a tortuous course, atypical for the age of the patient. (D) CT images (3D volume rendered) show the aneurysms as described in (B).
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A computerized tomography (CT) aortogram revealed two fusiform aortic aneurysms involving the ascending aorta and arch, and another involving the distal arch and the proximal descending thoracic aorta with cardiomegaly (Figure 2A). A "sausage link" appearance of the aneurysmal aorta together with the observed arterial tortuosity raised a strong suspicion for hereditary aortopathy (Figures 2B-D).
Images after the valve-sparing operation along with an aortic arch replacement. (A) Contrast-enhanced CT image (volume rendered) showed significant reduction in the caliber of the aorta (AR:aortic root, AA:Ascending Aorta, DA:Descending Aorta, LCA:Left common Carotid Artery, LSA:Left Subclavian Artery, IA:Innominate Artery). (B) Post valve-sparing surgery along with the aortic arch replacement, a CT aortogram section (axial view) at the level and just below the main pulmonary artery (MPA) showed significant reduction in the caliber of ascending aorta (A1) and descending aorta (A2). Minimally enhancing soft tissue thickening was noted surrounding the great vessels, suggestive of postoperative changes (scale 1:5).
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She underwent a valve-sparing (David V procedure) operation along with an aortic arch replacement (Figure 3).
Histopathological assessment of the resected aorta. (A) The wall of the aorta stained with hematoxylin and eosin stain showed thickened tunica media with myxoid change (100x). (B) Masson's trichome staining showed disorganization and fragmentation of collagen (40x) with, (C) an increase in fibroblasts in the tunica media (400x). (D) Verhoeff-van Gieson stain showed deficient and fragmented elastic fibers (red arrows, 40x), with (E) increased fibroblasts (400x).
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Histopathological assessments of the resected tissue samples demonstrated disorganization and fragmentation of elastin and collagen fibers (Figure 4).