After the advent of antibiotic treatment, tertiary syphilis is rarely observed over
the last several decades, and its cardiovascular manifestations are particularly rare.
Syphilitic aortitis typically involves the tubular portion of the ascending aorta,
aortic arch and descending thoracic aorta, sparing the sinuses of Valsalva.[1] Although
exceptional cases of treatment of syphilitic thoracic aortic aneurysm have been reported,[2–4]
the optimal technique for treatment is not established. Total arch replacement combined
with stented elephant trunk technique was proven to be feasible in patients with type
A aortic dissection. Herein, we described our experience of total arch replacement
with stented elephant trunk technique in three patients presenting with syphilitic
thoracic aortic aneurysm involving the aortic arch and proximal descending aorta.
Three male patients (aged 53, 64, and 67 years, respectively) who initially presented
with hoarseness or progressive dyspnea, were referred to our hospital. Their medical
history was significant for hypertension. Chest computed tomography (CT) scans showed
thoracic aortic aneurysm. Thereafter, computed tomographic angiography (CTA) demonstrated
a large saccular thoracic aortic aneurysm involving the aortic arch and proximal descending
aorta, as well as ascending aortic ulcer [Figure 1A and 1B]. The large aneurysm led
to compression of the adjacent organs, such as recurrent laryngeal nerve leading to
hoarseness, trachea, and left lung leading to progressive dyspnea. The cardiac examinations
were unremarkable with no aortic root aneurysm and aortic valve insufficiency. CTA
was used to exclude coronary artery disease. All data were collected from the database
of the Division of Cardiothoracic Surgery with the permission of the Institutional
Review Board of Changhai Hospital. This clinical series study was conducted after
receiving patients’ consent.
Figure 1
(A) Pre-operative computed tomographic angiography (CTA) demonstrated a large saccular
thoracic aortic aneurysm involving the aortic arch and proximal descending aorta.
(B) CTA showed the specific size of saccular thoracic aortic aneurysm. (C) Intra-operative
exploration showed no aortic root aneurysm and aortic valve insufficiency. (D) Intra-operative
exploration showed that the wall of ascending aorta and aortic arch aneurysm was moderately
thickened with moderate inflammation presumably due to syphilitic aortitis. (E) Post-operative
CTA showed successful repair with full exclusion of the aneurysm without endoleak.
At admission, the patients had no previous history or symptoms of syphilis. However,
the routine pre-operative laboratory test for syphilis was highly positive, and the
rapid plasma reagin (RPR) test was 1:64. Confirmatory fluorescent treponemal antibody
was positive. Hence, a presumptive diagnosis of tertiary syphilis was made. Antibiotic
therapy using penicillin was first administered, at least for two weeks before the
operation. Furthermore, prednisone was orally administered to alleviate the Jarisch-Herxheimer
reaction.
Ascending aorta and aortic arch replacement using a tetrafurcate graft, combined with
stented elephant trunk implantation, was performed in all patients via a median sternotomy
under cardiopulmonary bypass (CPB) with selective cerebral perfusion (SCP). Cannulation
of the right axillary artery was routinely conducted for CPB and SCP. CPB and cooling
were started after cannulation of the right axillary artery and the right atrium.
Circulatory arrest was established when the nasopharyngeal temperature reached 27°C.
The pathological ascending aorta was resected [Figure 1C and 1D]. The ascending aorta
and aortic arch were replaced with a tetrafurcate graft. The distal arch was circumferentially
transected proximal to the aortic aneurysm. If the aortic aneurysm involved the left
subclavian artery and/or the left common carotid artery, they were reconstructed with
a graft branch. Then, a 10 cm long stented graft (MicroPort Medical Co Ltd, Shanghai,
China) in a bound was inserted into the aneurysm, and the terminal end of the stented
graft was located at normal zone of descending aorta. Then the anastomosis between
the tetrafurcate graft and the distal arch was conducted by the open aortic technique.
The pathology examination of the aneurysmal wall revealed diffuse infiltration of
lymphocytes and plasma cells, which is characteristic of syphilitic aortitis.
There was no hospital death, and the patients recovered uneventfully. Stroke, paraparesis,
visceral malperfusion and lower extremity malfunction were not observed. Post-operatively,
a follow-up CTA demonstrated successful repair with full exclusion of the aneurysm
without endoleak [Figure 1E]. A serological test for syphilis was conducted, with
additional administration of antibiotic therapy if necessary.
Syphilitic aortic aneurysm is a relatively rare type of cardiovascular syphilis, which
is a tertiary manifestation, generally occurring 10 to 30 years after the original
untreated infection. Vascular involvement in late syphilis is due to an obliterative
small vessel endarteritis, usually of the vasa vasorum of the thoracic aorta, fostering
loss of structural integrity of the tunica media, and subsequent fibrosis and calcification.
Aortitis typically involves the tubular portion of the ascending aorta, aortic arch
and descending thoracic aorta, sparing the sinuses of Valsalva.[1] Definitive diagnosis
of syphilitic aortitis can be challenging owing to prolonged latency from primary
infection. Clinical diagnosis is most often made based upon serological confirmation
of syphilis and a characteristic pattern of vascular involvement.
Patients with syphilitic aortic aneurysm can be asymptomatic for many years without
significant clinical manifestations, but thoracic aortic aneurysm has high annual
mortality rates. Once the aortic aneurysm is diagnosed, surgical treatment is necessary.
The surgical procedure depends on the site involved in cardiovascular syphilis. Ascending
aorta replacement is the most commonly performed surgical procedure for the treatment
of syphilitic aortic aneurysm, because 50% of syphilitic aortic aneurysms involve
the ascending aorta. For the syphilitic aortic aneurysm located at distal aortic arch,
Yasuda et al
[2] adopted endovascular repair using a hand-made fenestrated stent graft. In order
to secure an adequate landing zone, the left subclavian artery had to be occluded,
which had the potential risk of ischemia of the brain and spinal cord. Additionally,
the intima of proximal aortic arch and ascending aorta in patients with cardiovascular
syphilis could be pathological, so serious complications may be observed after thoracic
endovascular aortic repair, such as retrograde type A dissection, if the proximal
landing zone of the stent is located in the proximal arch or the ascending aorta.
One method to avoid these limitations is the hybrid (or staged) procedure.[3] Although
placing the conventional elephant trunk into the descending aneurysm is not very difficult,
deaths caused by rupture of the remaining aortic aneurysm in the interval between
the two procedures have been reported.[5]
Given the above-mentioned problems, we adopted the total arch replacement with stented
elephant trunk technique for three patients presenting with syphilitic thoracic aortic
aneurysm involving the aortic arch and proximal descending aorta, which achieved satisfactory
outcomes. This procedure combined the advantages of open surgical treatment and interventional
methods while simultaneously avoiding the shortcomings of these approaches. First,
the pathological aortic segment (from the proximal aortic lesion to the distal arch),
which is susceptible to further dilation/rupture, was resected. Second, the procedure
was simplified, because the aneurysm does not need to be incised, which is an effective
way to prevent bleeding due to tissue weakness; the aortic arch was circumferentially
transected proximal to the aortic aneurysm, and then the stented graft was inserted
into the descending aorta during intra-operative view of selective antegrade brain
perfusion. Furthermore, the aneurysm was completely eliminated, because the proximal
end of the stent graft was firmly fixed to the distal end of the prosthetic graft
using suture line and the distal aorta was expanded by the rigidly spread surgical
stent. The proximal end of the stented graft had 1 cm of extravascular graft, facilitating
proximal anastomosis to the prosthetic graft or lengthening proximal graft using another
same-size graft in order to place the distal end of the stented graft at further landing
zone. If the descending aorta is diffusely expanded, a late-stage procedure for the
distal end is necessary.
Conflicts of interest
None.