Sudden death by Stanford type-A aortic dissection in a 10-year-old child
SOLANGEL GUTIERREZ EGUES, OMAIDA J. LÓPEZ BERNA,
LEIDELÉN ESQUIVEL SOSA
Hospital Pediátrico José Luis Miranda.
Villa Clara, Cuba E-mail
Recibido 19-NOV-2018 – ACEPTADO después de revisión el -21-DICIEMBRE-2018.
There are no conflicts of interest to disclose.
Ten-year-old male patient with history of apparent health that started presenting centro-thoracic, oppressive, piercing pain. He was taken to a health care center in the area where he lived, where he died suddenly. It was decided to move him to the Pediatric Hospital to determine the cause of death by necropsy.
The macroscopic examination showed pericardial sac increased in size, with hemorrhagic content of 400 cc. The heart was slightly increased in size. At its opening in the aortic emergence, 2 cm from the valve, an oblique tear with a length of 4 cm was observed, separating the intima from the middle layer with a large bruise dissecting the proximal portion of the ascending aorta until the emergence of the brachiocephalic trunk and flooding the pericardial sac (Stanford type-A aortic dissection  – DeBakey type II).
Currently, some authors prefer the Stanford classification  to the DeBakey classification, as they consider it more practical.
The microscopic examination displayed cystic degeneration of the media layer.
In the aortic dissection, a false lumen stood out in the media layer of the aortic wall, exceptional in children with no connective tissue disease .
Transesophageal echo is considered the initial test because of its availability, harmlessness and cost. It allows locating intimal tears among other complications. Multislice CAT presents a high sensitivity and specificity due to the reconstructions it enables. Magnetic resonance imaging defines ventricular volumes, functional valve alterations and dynamic behavior of the aortic wall .
The anatomopathological examination showed degeneration of smooth muscles of the media layer in type-B aortic dissection; more evident in hypertensive individuals and with an advanced age. Type-A aortic dissection has been associated to congenital anomalies of the conjunctive tissue, at the level of elastic tissue of the aortic media layer, frequently in young individuals, and very frequent in Marfan syndrome. Ehler-Danlos, Loeys-Dietz  syndromes and Turner syndrome are predisposing factors .
The risk of sudden death or aortic dissection is low in patients with Marfan syndrome and aortic diameter between 45 and 49 mm. The aortic diameter of 50 mm seems to be a reasonable threshold for prophylactic surgery .
Aortic dissection is considered a surgical emergency and it is properly documented that if not treated, the mortality rate is extremely high, close to 1% per hour during the first 48 hours and more than 80% during the first month .
Patel Himanshu J, Deeb MG. Ascending and arch aorta: Pathology, natural history, and treatment. Circulation 2008; 118: 188-95.
Mimoun L, Detaint D, Hamroun D, et al. Dissection in Marfan syndrome: the importance of the descending aorta. Eur Heart J 2011; 32 (4): 443-49.
Aalberts JJ, van den Berg MP, Bergman JE. The many faces of aggressive aortic pathology: Loeys-Dietz syndrome. Neth Heart J 2008; 16: 299-304.
Fikar CR, Koch S. Etiologic factors of acute aortic dissection in children and young adults. Clin Pediatr (Phila) 2000; 39: 71-80.
Jondeau G, Detaint D, Tubach F, et al. Aortic event rate in the Marfan population: a cohort study. Circulation 2012; 125 (2): 226-32.
Dinis Da Gama A, Marcelino JL, Damião A, et al. Surgical management of a dissecting aneurysm of the thoracic aorta in a patient with Marfan's syndrome - case report. Rev Port Cir Cardiotorac Vasc 2011; 18 (2): 115-21.