Korean J Leg Med.  2019 May;43(2):81-85. 10.7580/kjlm.2019.43.2.81.

Sudden Death due to Rupture of Pulmonary Trunk Aneurysm in a Patient with Eisenmenger Syndrome

• Kim SZ
• Lee S

• KMID: 2448943
• DOI: http://doi.org/10.7580/kjlm.2019.43.2.81

Abstract

Proximal pulmonary artery aneurysms and dissection are rare and life-threatening conditions, which are usually detected only during autopsy examination in cases of sudden death. These pathological entities often occur as complications of chronic pulmonary hypertension and most commonly result from pulmonary arterial hypertension associated with various congenital cardiac lesions involving left-to-right shunting. This study describes an autopsy case of a 38-year-old man who was diagnosed with Eisenmenger syndrome 5 years prior to sudden death secondary to cardiac tamponade following a ruptured pulmonary trunk aneurysm.

Keyword

Eisenmenger syndrome; Sudden death; Pulmonary hypertension; Autopsy

MeSH Terms

Adult
Aneurysm*
Autopsy
Cardiac Tamponade
Death, Sudden*
Eisenmenger Complex*
Humans
Hypertension
Hypertension, Pulmonary
Pulmonary Artery
Rupture*

Figure

• 

  Fig. 1 The pulmonary trunk shows aneurysmal dilatation (arrowheads) with thrombus and is dissected circumferentially (arrows).

• 

  Fig. 2 The right pulmonary artery shows fatty streaks and atheromas on the intimal surface along its branches.

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  Fig. 3 The lung parenchyma shows two organizing thrombi (arrows) in the branches of the pulmonary artery.

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  Fig. 4 A large perimembranous ventricular septal defect (arrows) measuring 25 mm at its maximal diameter is seen below the pulmonary valves in the right ventricle.

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  Fig. 5 The microscopic examination of small pulmonary artery shows concentric medial hypertrophy (H&E stain, ×400).

• 

  Fig. 6 The microscopic examination of small pulmonary artery shows a typical plexiform lesion in the pulmonary hypertension (H&E stain, ×400).

Reference

1. Khattar RS, Fox DJ, Alty JE, et al. Pulmonary artery dissection: an emerging cardiovascular complication in surviving patients with chronic pulmonary hypertension. Heart. 2005; 91:142–145.

2. Graham JK, Shehata B. Sudden death due to dissecting pulmonary artery aneurysm: a case report and review of the literature. Am J Forensic Med Pathol. 2007; 28:342–344.

3. Senbaklavaci O, Kaneko Y, Bartunek A, et al. Rupture and dissection in pulmonary artery aneurysms: incidence, cause, and treatment: review and case report. J Thorac Cardiovasc Surg. 2001; 121:1006–1008.

4. Butto F, Lucas RV Jr, Edwards JE. Pulmonary arterial aneurysm: a pathologic study of five cases. Chest. 1987; 91:237–241.

5. Jones SL. Dissecting hematomas of the pulmonary artery: rare and fatal catastrophies. Am J Forensic Med Pathol. 1997; 18:349–353.

6. Walley VM, Virmani R, Silver MD. Pulmonary arterial dissections and ruptures: to be considered in patients with pulmonary arterial hypertension presenting with cardiogenic shock or sudden death. Pathology. 1990; 22:1–4.

7. Inayama Y, Nakatani Y, Kitamura H. Pulmonary artery dissection in patients without underlying pulmonary hypertension. Histopathology. 2001; 38:435–442.

8. Lopez-Candales A, Kleiger RE, Aleman-Gomez J, et al. Pulmonary artery aneurysm: review and case report. Clin Cardiol. 1995; 18:738–740.

9. Arena V, De Giorgio F, Abbate A, et al. Fatal pulmonary arterial dissection and sudden death as initial manifestation of primary pulmonary hypertension: a case report. Cardiovasc Pathol. 2004; 13:230–232.

10. Sakuma M, Demachi J, Suzuki J, et al. Proximal pulmonary artery aneurysms in patients with pulmonary artery hypertension: complicated cases. Intern Med. 2007; 46:1789–1793.

11. Katzenstein AL. Katzenstein and Askin's surgical pathology of non-neoplastic lung disease. Volume 13 in the major problems in pathology series. 4th ed. Philadelphia: Elsevier Saunders;2006. p. 351–384.

12. Vongpatanasin W, Brickner ME, Hillis LD, et al. The Eisenmenger syndrome in adults. Ann Intern Med. 1998; 128:745–755.

13. Sreeram N. Eisenmenger syndrome: towards identifying the risk factors for death. Eur Heart J. 2006; 27:1644–1645.

14. Broberg CS, Ujita M, Prasad S, et al. Pulmonary arterial thrombosis in eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity. J Am Coll Cardiol. 2007; 50:634–642.

15. Kumar V, Abbas AK, Fausto N. Robbins and Cotran pathologic basis of disease. 7th ed. Philadelphia: Elsevier Saunders;2004. p. 119–144.