Advertisement

International Journal of Legal Medicine

, Volume 131, Issue 6, pp 1565–1572 | Cite as

Sudden aortic death—proposal for a comprehensive diagnostic approach in forensic and in clinical pathology practice

  • Hans H. de Boer
  • Fabrice Dedouit
  • Nina Chappex
  • Allard C. van der Wal
  • Katarzyna MichaudEmail author
Original Article

Abstract

Backgrounds

Aortic rupture or dissection as immediate cause of sudden death is encountered in forensic and clinical autopsy practice. Despite a common denominator of ‘sudden aortic death’ (SAD), we expect that in both settings the diagnostic workup, being either primarily legal or primarily disease related, differs substantially, which may affect the eventual diagnoses.

Methods

We retrospectively reviewed case records of deceased persons who fitted a diagnosis of SAD in the continuous autopsy cohorts in a forensic (Suisse) and a clinical setting (The Netherlands). Clinical characteristics, data from post-mortem imaging, tissue blocks for histological analysis and results of ancillary studies were reviewed for its presence and outcome.

Results

SAD was found in 7.7% in the forensic versus 2.2% in the clinical autopsies. In the forensic setting, autopsy was always combined with post-mortem imaging, showing variable outcome on detection of aortic disruption and/or pericardial bleeding. Histology of aorta was performed in 12/35 cases, mostly in the natural deaths. In the clinical setting, histology of the aorta was available in all cases, but post-mortem imaging in none. In both settings, underlying aortic disease was mostly cystic medial degeneration, atherosclerosis or a combination of both, with occasional rare unexpected diagnosis. Also in both, a genetic cause of aortic dissection was revealed in a minority (three cases).

Conclusion

Sudden aortic death (SAD) is more commonly encountered in a forensic than in a clinical setting. Major differences in the approach of SAD between these settings coincide with similarities in causes of death and underlying diseases. To ensure a correct diagnosis, we recommend that the investigation of SAD includes a study of the medical history, a full autopsy with histology of major organs including aorta, and storage of material for toxicological and genetic testing. Post-mortem radiological examination, useful for documentation and screening purposes, is feasible as non-invasive alternative when autopsy is not possible, but cannot substitute a full autopsy.

Keywords

Aorta Dissection Rupture Sudden death Forensic autopsy 

References

  1. 1.
    Halushka MK, Angelini A, Bartoloni G et al (2016) Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association For European Cardiovascular Pathology: II. Noninflammatory degenerative diseases—nomenclature and diagnostic criteria. Cardiovasc Pathol 25:247–257. doi: 10.1016/j.carpath.2016.03.002 CrossRefPubMedGoogle Scholar
  2. 2.
    Stone JR, Bruneval P, Angelini A et al (2015) Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases. Cardiovasc Pathol 24:267–278. doi: 10.1016/j.carpath.2015.05.001 CrossRefPubMedGoogle Scholar
  3. 3.
    Virmani R, Burke AP, Farb A (2001) Sudden cardiac death. Cardiovasc Pathol 10:275–282. doi: 10.1016/s1054-8807(01)00108-9 CrossRefPubMedGoogle Scholar
  4. 4.
    Ripperger T, Troger HD, Schmidtke J (2009) The genetic message of a sudden, unexpected death due to thoracic aortic dissection. Forensic Sci Int 187:1–5. doi: 10.1016/j.forsciint.2009.01.020 CrossRefPubMedGoogle Scholar
  5. 5.
    Klintschar M, Bilkenroth U, Arslan-Kirchner M, Schmidtke J, Stiller D (2009) Marfan syndrome: clinical consequences resulting from a medicolegal autopsy of a case of sudden death due to aortic rupture. Int J Legal Med 123:55–58. doi: 10.1007/s00414-008-0288-5 CrossRefPubMedGoogle Scholar
  6. 6.
    Hugar BS, Praveen S, Kainoor SK, Shetty ARS (2014) Sudden death in Marfan syndrome. J Forensic Sci 1126–8Google Scholar
  7. 7.
    Lindsay ME, Dietz HC (2011) Lessons on the pathogenesis of aneurysm from heritable conditions. Nature 473:308–316CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Dean JH, Woznicki EM, O'Gara P et al (2014) Cocaine-related aortic dissection: lessons from the International Registry of Acute Aortic Dissection. Am J Med 127:878–885. doi: 10.1016/j.amjmed.2014.05.005 CrossRefPubMedGoogle Scholar
  9. 9.
    Grabherr S, Doenz F, Steger B et al (2010) Multi-phase post-mortem CT angiography: development of a standardized protocol. Int J Leg Med: 1–12. doi: 10.1007/s00414-010-0526-5
  10. 10.
    Brinkmann B (1999) Harmonisation of medico-legal autopsy rules. Int J Legal Med 113 (1):1–14. doi: 10.1007/s004140050271
  11. 11.
    Basso C, Burke M, Fornes P et al (2008) Guidelines for autopsy investigation of sudden cardiac death. Virchows Arch 452:11–18. doi: 10.1007/s00428-007-0505-5 CrossRefPubMedGoogle Scholar
  12. 12.
    Zerlauth JB, Doenz F, Dominguez A et al (2013) Surgical interventions with fatal outcome: utility of multi-phase postmortem CT angiography. Forensic Sci Int 225:32–41. doi: 10.1016/j.forsciint.2012.05.013 CrossRefPubMedGoogle Scholar
  13. 13.
    Sieswerda-Hoogendoorn T, van Rijn RR (2010) Current techniques in postmortem imaging with specific attention to paediatric applications. Pediatr Radiol 40:141–152. quiz 259. doi: 10.1007/s00247-009-1486-0 CrossRefPubMedGoogle Scholar
  14. 14.
    Krentz BV, Alamo L, Grimm J et al (2016) Performance of post-mortem CT compared to autopsy in children. Int J Legal Med 130:1089–1099. doi: 10.1007/s00414-016-1370-z CrossRefPubMedGoogle Scholar
  15. 15.
    Blokker BM, Wagensveld IM, Weustink AC, Oosterhuis JW, Hunink MGM (2016) Non-invasive or minimally invasive autopsy compared to conventional autopsy of suspected natural deaths in adults: a systematic review. Eur Radiol 26:1159–1179. doi: 10.1007/s00330-015-3908-8 CrossRefPubMedGoogle Scholar
  16. 16.
    Kurra V, Lieber ML, Sola S et al (2010) Extent of thoracic aortic atheroma burden and long-term mortality after cardiothoracic surgery: a computed tomography study. JACC Cardiovascular Imaging 3:1020–1029. doi: 10.1016/j.jcmg.2010.08.006 CrossRefPubMedGoogle Scholar
  17. 17.
    Xu L, Burke A (2013) Acute medial dissection of the ascending aorta: evolution of reactive histologic changes. Am J Surg Pathol 37:1275–1282. doi: 10.1097/PAS.0b013e318294adc3 CrossRefPubMedGoogle Scholar
  18. 18.
    Lucena J, Blanco M, Jurado C et al (2010) Cocaine-related sudden death: a prospective investigation in south-west Spain. Eur Heart J 31:318–329. doi: 10.1093/eurheartj/ehp557 CrossRefPubMedGoogle Scholar
  19. 19.
    Elefteriades JA, Pomianowski P (2013) Practical genetics of thoracic aortic aneurysm. Prog Cardiovasc Dis 56:57–67. doi: 10.1016/j.pcad.2013.06.002 CrossRefPubMedGoogle Scholar
  20. 20.
    Albornoz G, Coady MA, Roberts M et al (2006) Familial thoracic aortic aneurysms and dissections—incidence, modes of inheritance, and phenotypic patterns. Ann Thorac Surg 82:1400–1405. doi: 10.1016/j.athoracsur.2006.04.098 CrossRefPubMedGoogle Scholar
  21. 21.
    Lindsay ME, Dietz HC (2014) The genetic basis of aortic aneurysm. Cold Spring Harb Perspect Med. 4(9):a015909. doi: 10.1101/cshperspect.a015909
  22. 22.
    Maleszewski JJ, Tazelaar HD, Horcher HM et al (2015) IgG4-related disease of the aortic valve: a report of two cases and review of the literature. Cardiovasc Pathol 24(1):56-59. doi: 10.1016/j.carpath.2014.08.001
  23. 23.
    Hirani R, Koszyca B, Byard RW (2008) Marfan syndrome and sudden death within a family—aetiologic, molecular and diagnostic issues at autopsy. J Forensic Legal Med 15:205–209. doi: 10.1016/j.jflm.2007.07.010 CrossRefGoogle Scholar
  24. 24.
    Savall F, Dedouit F, Piercecchi-Marti M-D, Leonetti G, Rougé D, Telmon N (2014) Acute aortic dissection diagnosed after embalming: macroscopic and microscopic findings. J Forensic Sci 59:1423–1426. doi: 10.1111/1556-4029.12485 CrossRefPubMedGoogle Scholar
  25. 25.
    Halushka MK (2012) Single gene disorders of the aortic wall. Cardiovasc Pathol 21:240–244. doi: 10.1016/j.carpath.2011.09.004 CrossRefPubMedGoogle Scholar
  26. 26.
    Bjorck M, Wanhainen A (2013) Pathophysiology of AAA: heredity vs environment. Prog Cardiovasc Dis 56:2–6. doi: 10.1016/j.pcad.2013.05.003 CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  1. 1.Department of PathologyAcademic Medical Center, University of AmsterdamAmsterdamThe Netherlands
  2. 2.University Hospital of LausanneUniversity Center of Legal Medicine, Lausanne-GenevaLausanne 25Switzerland

Personalised recommendations