Inédita correção cirúrgica de dissecção aórtica em menina de 15 anos com Hipertensão Arterial Cronica

1. Letter to the Editor
Ascending aorta dissecting aneurysm in a teenager
with isolated systemic hypertension
Paulo Henrique Manso a,⁎, Fernando Tadeu Vasconcelos Amaral a
,
Walter Villela de Andrade Vicente b
, João Antônio Granzotti a
a
Division of Pediatric Cardiology, University Hospital, Ribeirão Preto Medical School, Ribeirão Preto, SP, Brazil
b
Division of Cardiac Surgery, University Hospital, Ribeirão Preto Medical School, Ribeirão Preto, SP, Brazil
Received 30 November 2006; accepted 1 January 2007
Available online 28 March 2007
Abstract
A teenager with longstanding arterial hypertension was admitted for acute pneumonia treatment. New onset atypical chest pain for the last
months and aortic valve regurgitation were also present. A dissecting aneurysm of the ascending aorta with moderate aortic valve
regurgitation was evidenced by laboratorial diagnostic. Aneurismectomy with aortic valve preservation and coronary artery reimplantation
was carried out.
© 2007 Elsevier Ireland Ltd. All rights reserved.
Keywords: Aortic aneurysm; Chronic hypertension; Aortic dissection; Children
1. Introduction
The high mortality rate and morbidity of ascending aortic
dissection makes it a dreadful entity. Although in elderly
patients chronic hypertension and progressive arteriosclerosis
are the main pathogenetic mechanisms, connective tissue
disorders e.g. Marfan and Ehlers–Danlos syndromes [4],
congenital heart disease [6,7], severe trauma [3] and weightlift-
ing are well known risk factors in children and young adults.
Isolate chronic arterial hypertension as the sole risk factor for
aortic dissection in young patients is exceedingly rare [8].
2. Case report
A 15-year-old girl had an episode of acute renal failure
due to hemolytic uremic syndrome when she was three and
has since been followed in the outpatient clinic with resilient
chronic hypertension. She was admitted due to acute
International Journal of Cardiology 118 (2007) e72–e74
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Fig. 1. Thorax X-ray showing mediastinal widening.
⁎ Corresponding author. Hospital das Clínicas, Faculdade de Medicina de
Ribeirão Preto-USP, Av. Bandeirantes 3900, Ribeirão Preto — SP, Brazil.
Tel.: +55 16 3602 2573; fax: +55 16 3602 2700.
E-mail address: phmanso@uol.com.br (P.H. Manso).
0167-5273/$ - see front matter © 2007 Elsevier Ireland Ltd. All rights reserved.
doi:10.1016/j.ijcard.2007.01.020

2. pneumonia and, during this period, she complained of a light
anterior chest pain present for the last 3 months. At physical
examination, a 39 kg white girl was in no distress. The blood
pressure was 140/60 mm Hg, and the heart rate was 82 bpm,
the pulses were normal and a ++/6+ diastolic murmur was
heard in the aortic area. The EKG showed left ventricular
hypertrophy with strain. Moderate left ventricular dilatation
and marked widening of the superior mediastinum were
noticed in the chest X-ray (Fig. 1). A transthoracic
echocardiogram displayed a 69 mm wide dissecting
aneurysm of the ascending aorta and moderate aortic valve
regurgitation (Fig. 2A and B). After the aneurysm was
screened by computerized tomography (CT) (Fig. 2C) and
magnetic resonance image (MRI), the patient was referred to
surgery. Under heart–lung bypass and systemic hypothermia,
the large ascending aorta dissecting aneurysm was exposed.
The partially thrombosed false lumen extended into the
dilated aortic root while the aortic annulus remained within
the normal diameter range. The valve leaflets gross anatomy
was preserved, but the severe dilatation of the sinotubular
junction prevented cusp coaptation. The entire ascending
aorta was replaced with a composite Dacron tube graft that
was proximally sutured to the aortic annulus. The aortic valve
was preserved and resuspended into the tube graft proximal
end. The coronary ostia were reimplanted and the patient was
uneventfully weaned from bypass. The chest pain disap-
peared and the post-operative echocardiogram showed mild
left ventricular dilation with normal systolic function and
mild to moderate aortic valve regurgitation. Histologic
analysis of the ascending aorta revealed colagen degeneration
and fibroblast proliferation compatible with aortic wall
degeneration secondary to chronic hypertension. Seventeen
days after surgery the patient was sent home on furosemide
and atenolol. After a two year follow up, she remains on
antihypertensive therapy. A recent echocardiogram revealed
left ventricular hypertrophy with mild dilatation and moderate
Fig. 2. Paraesternal transversal (A) and short axis view (B) of the dissecting aneurism in ascending aorta (arrows) in transthoracic echo. C) CT scan showing the
thrombosed false lumen (arrow) of the dissecting aneurism in ascending aorta. AAo: Ascending aorta; LA: left atrium; LV: left ventricle.
e73P.H. Manso et al. / International Journal of Cardiology 118 (2007) e72–e74

3. aortic valve regurgitation with normal left ventricular function.
A chest tomography and a magnetic resonance image of the
aorta showed a normal looking ascending aorta graft.
3. Discussion
Aortic dissection rarely affects children and adolescents.
This fact was highlighted by Zalstein et al. [9] who described
only 13 young individuals, diagnosed between 1970 and
2000, in four very active tertiary centers. The great majority
of these cases had degenerative or congenital cardiovascular
anomalies with a dilated and histologically abnormal
ascending aorta, predisposed to rupture [4,7]. Despite
systemic arterial hypertension being a common finding in
children with chronic renal disease, the occurrence of aortic
dissection among these patients is unusual. In fact, Vogt et al.
[8] recently described four such cases who presented with
acute aortic dissection in the emergency room. As well
known, it should be remembered that the typical case of
acute aortic dissection presents with intense chest pain,
associated with neurological manifestations and physical
signs mainly related to the cardiovascular system [1].
The case herein presented is interesting for the child had
no other risk factor for aortic dissection but the chronic
arterial hypertension. The acute pneumonia, on its turn, may
have blurred the relevance of the light, atypical, chest pain
that was present for the previous 3 months. An ascending
aorta aneurysm was only suspected when the new onset
diastolic aortic murmur and the mediastinal enlargement
were considered altogether. This findings make us think that
perhaps aortic dissection in patients with chronic hyperten-
sion may be undiagnosed. We thus consider that the
association of new onset chest pain and aortic valve
regurgitation should deserve cautious investigation in teen-
agers with longstanding arterial hypertension. On the other
hand, although chest pain is a common complaint in children
[5], it is usually not related to heart disease. However, in the
setting of chronic arterial hypertension, the association of
aortic valve insufficiency and chest pain should lead to
carefully cardiovascular screening.
The diagnosis of ascending aortic dissection and/or dis-
secting aortic aneurysm, as in this case, can be readily
accomplished by echocardiography, particularly by the
transesophageal approach. A chest CT scan or an MRI study
may also confirm the diagnosis and have the advantage of
being able to evaluate the entire thoracic aorta.
Prompt surgical treatment must be applied, whenever
feasible, and increases the chances of survival. Adjunct tech-
niques of aortic valve preservation, like the one here em-
ployed, may have a favorable impact on long term prognosis
[2].
In summary, aortic dissection is a rare finding in the
young. Patients with chronic arterial hypertension as the only
risk factor for this entity are even rarer and may progress
undiagnosed. On the basis of the present case, complaints of
chronic chest pain in patients with long term arterial
hypertension should be investigated in order to rule out
aortic dissection. On the other hand, a newly found aortic
valve regurgitation should be taken as a marker for important
proximal aorta dilatation in these patients. Finally, chroni-
cally hypertensive teenagers should have routine, serial
echocardiograms with a special focus onto the ascending
aorta. A prospective follow up study using modern imaging
techniques in young patients with chronic hypertension
seems to be warranted.
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