Ascending aortic aneurysm, descending aortic pseudoaneurysm
By David C. Isbell, MD; Klaus D. Hagspiel, MD; Christopher M. Kramer, MD
Three-dimensional surface-rendered reconstruction of the MR
angiogram revealed the interposition graft with a moderate degree
of stenosis at both the proximal and distal anastomoses as well as
a pseudoaneurysm (at the distal suture line) that measured 27 mm in
length and 7 mm at the neck (Figure 1). Velocity-encoded
phase-contrast MR imaging measured the gradient across the graft at
15 mm Hg (not shown), which was confirmed at cardiac
catheterization. Prominent intercostal vasculature was noted; also
noted were the pseudoaneurysm and an ascending aortic aneurysm that
measured 5.8 cm (Figure 2).
An aortic coarctation is a common cardiovascular lesion that
accounts for 5% to 7% of all congenital heart disease.1
Coarctation is more common in males than females and is known to
occur in conjunction with a variety of conditions, including
Turner's syndrome, Shone complex, ventricular septal defect,
bicuspid aortic valve, and aneurysms of the circle of
If left untreated, complications are common and
can include aortic dissection, infective endocarditis, severe
aortic insufficiency, hypertension, coronary artery disease, and
In patients with uncorrected
coarctation, as many as 90% die by the age of 60 years.4
Following surgical repair of aortic coarctation, close follow-up
of patients is recommended, as surgery is, in many ways, not
"curative." Late complications, as a consequence of the surgery
itself or the systemic arteriopathy, are not uncommon. Irrespective
of the success of the repair, hypertension frequently develops and
is a major contributor to long-term cardiovascular morbidity,
although early surgical intervention may reduce the risk of
developing late hypertension and other cardiovascular
sequelae.5,6 In a large surgical series, the most common
causes of death in patients with successful coarctation repair were
coronary artery disease (37%), congestive heart failure (9%), and
complications of reoperation (7%).2 The most frequent
indications for reoperation include recurrent coarctation,
ascending aortic aneurysm, valvular heart disease, and
Aortic imaging techniques are critical in ruling out pathology
in patients with a history of coarctation repair. MR imaging has
evolved into the modality of choice for screening these patients,
as it provides safe, high-quality images of the aorta.7
In the case reported here, several abnormalities associated with
this disease process have been identified, including an ascending
aortic aneurysm, narrowing of the graft at both the proximal and
distal anastomosis, and the unusual-appearing pseudoaneurysm at the
distal suture site. Although not identified here, true aneurysm
formation at the site of repair is not uncommon in patients who
have undergone prior synthetic patch aortoplasty, while it is
infrequent in those repaired with end-to-end anastamosis or
interposition grafting.8 Some have advocated routine
aortic screening with MR imaging every 12 to 24 months after
coarctation repair, particularly for those with prior patch
Following balloon angioplasty of native coarctation, the
incidence of aneurysm formation and recurrent coarctation is higher
than with surgical repair.9,10 Long-term experience and
follow-up of patients with aortic stent implantation in native
coarctation is limited,11,12 but short-term outcomes
have been encouraging.
Aortic coarctation is a potentially life-threatening congenital
lesion that often requires surgical or percutaneous intervention.
However, repair is frequently not "curative" in the traditional
sense, as long-term complications from hypertension, aneurysm
formation, associated valvular heart disease, and recoarctation are
common. Close follow-up by a cardiovascular specialist is warranted
in all cases.
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- Jenkins NP, Ward C. Coarctation of the aorta: Natural history
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- Attenhofer Jost CH, Schaff HV, Connolly HM, et al. Spectrum of
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implantation for aortic coarctation and recoarctation. Heart.
Ascending aortic aneurysm, descending aortic pseudoaneurysm.
December 16, 2006