International Journal of Cardiology 190 (2015) 18–19

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Letter to the Editor

Ascending aortic aneurysm and acute type A aortic dissection in a patient with congenital severe coarctation of the aorta Yaowen Jiang a,1, Yunfei Ling b,1, Dan Zhu a, Yu Cao a,⁎ a b

Emergency Department, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, People's Republic of China Department of Cardiovascular Surgery, West China Hospital, Sichuan University, No. 37 GuoXue Xiang, Chengdu, Sichuan 610041, People's Republic of China

a r t i c l e

i n f o

Article history: Received 12 April 2015 Accepted 14 April 2015 Available online 15 April 2015 Keywords: Ascending aortic aneurysm Aortic dissection Coarctation of the aorta

Coarctation of the aorta is a congenital narrowing of the upper descending thoracic aorta adjacent to the site of attachment of the ductus arteriosus. The aortic lumen may be atretic in the most severe form of this defect, but aortic walls above and below the atresia are in continuity. It is frequently associated with diffuse hypoplasia of the aortic arch and isthmus [1]. The incidence of coarctation of the aorta is 4 in 10,000 live births, accounting for 5–8% of the children with congenital heart defects [2,3]. The mean age of death for persons with this condition is 34 years if untreated and is usually due to heart failure, aortic dissection or rupture, endocarditis, endarteritis, cerebral haemorrhage or concomitant biscuspid aortic valve disease in uncomplicated case. Symptoms may not be present in adults [4]. We report the case of ascending aortic aneurysm and acute type A aortic dissection in association with congenital severe coarctation of the aorta in an adult. A 41-year-old man presented to the emergency department with a history of severe acute central chest pain for 4 h. A physical examination revealed the following: heart rate, 100/min, blood pressure, 175/90 and 180/95 mm Hg in the right and left upper extremities, respectively, and 126/71 and 109/70 mm Hg in the right and left lower extremities, respectively, and saturation, 97% on air.

⁎ Corresponding author. 1 These authors contributed equally to this work and should be considered co-first authors.

http://dx.doi.org/10.1016/j.ijcard.2015.04.118 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.

Systolic murmur was heard in the aortic area. Analgesia and sedation were obtained by morphine injection. Sodium nitroprusside and β-blocking agents were used for blood pressure control. Computerised tomography demonstrated a 7.34 × 6.16 cm ascending aorta and acute type A aortic dissection originating 2 cm distal from the aortic annulus and ending at the upper aortic arch with an intimal flap (Fig. 1A and B) that was associated with aneurysmal dilatation of the aortic arch. Three-dimensional computed tomography showed that the aortic lumen was atretic and aortic walls above and below the atresia are in continuity and numeral collateral vessels (Fig. 1C and D). The patients died of rupture of the aortic dissection before operation. Aortic coarctation is a relatively frequent congenital cardiovascular disease, defined as a narrowing or constriction or atresia of the lumen of the aorta. Hypertensive vascular complications, cerebrovascular emergencies, aortic valve destruction, aortic aneurysms and dissection of aortic aneurysm are usually seen in patients with unrepaired aortic coarctation [5,6]. The primary cause of death in unrepaired cases is aortic rupture and the location of the rupture is usually the ascending aorta [7]. Ascending aortic aneurysm and acute type A aortic dissection secondary to narrowing or constriction of the lumen of the aorta are much reported in the literature, however atresia of the lumen of the aorta, a severe kind of the aortic coarctation was not reported to our knowledge. The diagnosis and treatment of aortic coarctation are usually made in the early decades of life and complications are rarely seen. The dissection of an ascending aortic aneurysm is a rare but life-threatening event and has high mortality and unrepaired type I aortic dissection is associated with a nearly 88% mortality rate. The pre-hospital mortality rate was 22–33% and a 30-day mortality rate was approximately 36% in hospitalized patients. This mortality rate may be higher in cases with aortic dissections secondary to aortic coarctation than simple aortic dissections [6, 8] and most of them died of rupture of the aortic dissection before operation.

Conflict of interest The authors report no relationships that could be construed as a conflict of interest.

Y. Jiang et al. / International Journal of Cardiology 190 (2015) 18–19

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Fig. 1. A and B: ascending aortic aneurysm and acute type A aortic dissection. C and D: aortic lumen was atretic and aortic walls above and below the atresia are in continuity and numeral collateral vessels.

References [1] J.W.J. Vriend, B.J.M. Mulder, Late complications in patients after repair of aortic coarctation: implications for management [J], Int. J. Cardiol. 101 (3) (2005) 399–406. [2] J.I.E. Hoffman, S. Kaplan, The incidence of congenital heart disease, J. Am. Coll. Cardiol. 39 (2002) 1890–1900. [3] C. Zivelonghi, M. Pighi, S. Perandini, et al., Asymptomatic severe aortic coarctation at old age [J], Int. J. Cardiol. 173 (3) (2014) e56–e57. [4] S. Saunders, D. Harmse, M. Sheppard, Undiagnosed coarctation of the aorta as a cause of aortic dissection in the young [J], BMJ Case Rep. (2009)http://dx.doi.org/10.1136/ bcr.04.2009.1740.

[5] B.P. Frischhertz, P. Shamszad, C. Pedroza, et al., Thoracic aortic dissection and rupture in conotruncal cardiac defects: a population-based study [J], Int. J. Cardiol. 184 (2015) 521–527. [6] G. Heper, Y. Yorukoglu, M.E. Korkmaz, Clinical and hemodynamic follow-up of a patient after operation for dissection of an ascending aortic aneurysm secondary to coarctation of the aorta [J], Int. Heart J. 46 (6) (2005) 1123–1131. [7] G.H. Reifenstein, S.A. Levine, R.E. Gross, Coarctation of the aorta: a review of 104 autopsied cases of the ‘adult type’ 2 years of age or older, Am. Heart J. 33 (1947) 146–168. [8] M.E. Abbott, Coarctation of the aorta of the adult type, Am. Heart J. 3 (1928) 574–618.

Ascending aortic aneurysm and acute type A aortic dissection in a patient with congenital severe coarctation of the aorta.

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