Autopsy in the era of advanced cardiovascular imaging

Abstract

Historically, autopsy contributed to our current knowledge of cardiovascular anatomy, physiology, and pathology. Major advances in the understanding of cardiovascular diseases, including atherosclerosis and coronary artery disease, congenital heart diseases, and cardiomyopathies, were possible through autopsy investigations and clinicopathological correlations. In this review, the importance of performing clinical autopsies in people dying from cardiovascular disease, even in the era of advanced cardiovascular imaging is addressed. Autopsies are most helpful in the setting of sudden unexpected deaths, particularly when advanced cardiovascular imaging has not been performed. In this setting, the autopsy is often the only chance to make the correct diagnosis. In previously symptomatic patients who had undergone advanced cardiovascular imaging, autopsies still play many roles. Post-mortem examinations are important for furthering the understanding of key issues related to the underlying diseases. Autopsy can help to increase the knowledge of the sensitivity and specificity of advanced cardiovascular imaging modalities. Autopsies are particularly important to gain insights into both the natural history of cardiovascular diseases as well as less common presentations and therapeutic complications. Finally, autopsies are a key tool to quickly understand the cardiac pathology of new disorders, as emphasized during the recent coronavirus disease 2019 pandemic.

Keywords: Autopsy; Cardiovascular pathology; Cause of death; Devices; Imaging; Therapy.

Graphical abstract

Clinical role of autopsy in patients who die unexpectedly or of known cardiac disease: identification of cardiac disease; confirmation of cardiac disease; and assessment of treatment and disease progression. Top: myocardial disease. Stage 1: Asymptomatic, subclinical (not detectable by current imaging, but detectable at autopsy). Stage 2: Asymptomatic, mildly dilated (detectable by imaging and at autopsy). Stage 3: Symptomatic, dilated (detectable by imaging, confirmed at autopsy). Stage 4: Symptomatic, dilated, post-treatment (assessment of therapy, verification of diagnosis). Bottom: coronary artery disease. Stage 1: Asymptomatic, mild atherosclerosis, complicated by acute thrombosis. Stage 2: Asymptomatic, moderate atherosclerosis, complicated by thrombosis. Stage 3: Symptomatic, severe atherosclerosis. Stage 4: Symptomatic, atherosclerosis post treatment (stent), complicated by thrombosis.

Figure 1

Validation of cardiac imaging findings in dilated cardiomyopathy with pulmonary hypertension. (A) Presence of late gadolinium enhancement in the right ventricular junctional insertion point of the interventricular septum (arrowhead). (B) At autopsy no scar is visible but a marked expansion of the interstitial space due to oedema in the context of disorderly arranged myocytes (modified from De Lazzari et al.).

Figure 2

Distinct patterns of amyloid deposition in the heart. (A) Congo red stain showing amyloid in a nodular/interstitial pattern (arrows). When extensive, this pattern of amyloidosis is likely identifiable by imaging. (B) Congo red stain showing amyloid in a microvascular specific pattern (arrows). It is unclear if imaging studies can identify this pattern of amyloidosis.

Figure 3

Unexpected histological findings in dilated cardiomyopathy with extensive cardiac imaging investigation during life. (A) Post-contrast cardiac magnetic resonance, two-chamber view: severe left ventricular dilatation and focal late gadolinium enhancement compatible with fibrosis (not coronary-related ischaemic pattern; arrowhead; courtesy of Professor Perazzolo Marra). (B) Histology of the myocardium shows patchy replacement-type fibrosis (arrows) and thickened wall of the small intra-myocardial vessels with lumen subocclusion. (C) Amyloid deposits in the vascular wall are confirmed by Congo red stain by fluorescent microscopy (apple-green birefringence).

Figure 4

Immune checkpoint inhibitor-associated myocarditis treated with solumedrol and abatacept. Histology of the myocardium at autopsy confirms persistent high-grade myocarditis after treatment. (A) Haematoxylin and eosin-stained section showing infiltration of the myocardium by lymphocytes (arrows) and associated myocyte injury (arrowheads). (B) Immunohistochemical stain for the cytoxic T-cell marker CD8 shows numerous CD8⁺ lymphocytes.

Figure 5

Peri-procedural complications in patients undergoing transcatheter aortic valve implantation. (A) Intra-operative aortic annulus rupture soon after the transcatheter aortic valve implantation procedure with cardiac tamponade (boxed area, external rupture in the pericardial cavity, arrow): in the left ventricular outflow tract region, note the topographic relationship between the bulky calcifications at the level of right and left fibrous trigone (arrowheads) and the rupture at the nadir of the left-coronary cusp (where the probe is passing through; modified from van Tarantini et al.). (B) Deep implantation of the prosthesis in the left ventricular outflow tract causes compression of subaortic septum, where the specialized conduction system (left bundle branch, white dotted lines) runs superficially with increased incidence of iatrogenic atrioventricular block (arrow indicates atrioventricular node, modified from Fraccaro et al.). LCC, left-coronary cusp; IVS, interventricular septum; AMV, anterior leaflet of the mitral valve.