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Giant Ascending Aortic Aneurysm Presented
with Acute Onset Superior Vena Cava Syndrome
Jorge Alcocer*, Nikita Mehta, María Ascaso, Eduard Quintana and Manuel Castellá
Department of Cardiovascular Surgery, ICCV, Hospital Clínic, Barcelona, Spain
Submission: July 30, 2019; Published: August 13, 2019
*Corresponding author: Jorge Alcocer, Hospital Clinic, University of Barcelona, Calle Villarroel 170, ZC 08036, Barcelona, Spain
How to cite this article:Jorge Alcocer, Nikita Mehta, María Ascaso, Eduard Quintana, Manuel Castellá. Giant Ascending Aortic Aneurysm Presented with Acute
Onset Superior Vena Cava Syndrome. J Cardiol & Cardiovasc Ther. 2019; 14(4): 555891. DOI: 10.19080/JOCCT.2019.14.555891
We present a case of a 50 year-old-male patient with past history of tissue aortic valve replacement presented at the emergency department with chest pain and acute onset superior vena cava syndrome. After physical examination and CT aortic angiogram a giant ascending aortic aneurysm complicated with aorta to superior vena cava fistula was identified. The patient underwent emergent surgery consisting of aortic root replacement with mechanical valve- conduit and total arch replacement under hypothermic cardiopulmonary bypass. The patient had an uneventful postoperative recovery and was discharged at postoperative day 8. Postoperative CT aortic angiogram showed an excellent surgical result.
The ascending aorta is the second most common localization of degenerative aortic aneurysms after infrarenal abdominal aorta. They represent a life-threatening condition since they can complicate with acute type A aortic dissection or rupture. According to clinical guidelines they must be surgically repaired when asymptomatic with diameter greater than 55mm or when symptomatic regardless their diameter. Aneurysms associated with elastopathies should be repaired with lower diameter (45-55mm) . Computed Tomography (CT) aortic angiography, magnetic resonance imaging and echocardiography represent the preferred diagnostic methods as they allow to detect aortic complications and to plan the best surgical approach.
Here, we report a patient with previous aortic valve replacement surgery and a giant ascending aortic aneurysm complicated with aorta to superior vena cava fistula. This is a very rare complication that has been previously described in association with syphilitic aortitis [2,3], acute aortic dissection  and aortic pseudoaneurysms  but infrequently associated to degenerative ascending aortic aneurysms.
A 50 year-old-male was admitted in the emergency department due to acute onset chest pain and hypotension.
Clinical examination was remarkable for upper body cyanosis, severe jugular vein distension and signs of low cardiac output. The patient was HIV seropositive due to past intravenous drug abuse and he had previous history of tissue aortic valve replacement for native bicuspid aortic valve endocarditis in 2011. Previous surgery was performed in an outside hospital and no follow-up data were available at the time of admission.
Chest-X-ray showed a huge mediastinal widening and transthoracic echocardiogram revealed a massively dilated aortic root and ascending aorta with normal functioning aortic bioprosthesis as well as good systolic function of both ventricles. A severe compression of the right atrium by the aortic aneurysm was also identified.
A CT aortic angiogram revealed a 14,7 x 13cm giant ascending aortic aneurysm (Figure 1, panel A) with a type A aortic dissection compressing the superior vena cava and right atrium. The CT also showed a fistula between the aneurysmal sac and superior vena cava (Figure 1, panel B, red and black arrows, respectively).
The patient underwent emergent surgery. Cardiopulmonary Bypass (CPB) was established after right axillary artery and right femoral vein cannulation. The re-sternotomy was performed under hypothermic CPB. Redo extensive aortic reconstruction was performed under deep hypothermic circulatory arrest with
antegrade selective cerebral perfusion. Myocardial protection
and cardiac standstill were obtained with selective antegrade
intermittent cold blood cardioplegia. Once the aneurismal sac was
incised, a communication between it and the superior vena cava
was confirmed (Figure 2, panel A, black arrow) and was repaired
with interrupted pledgeted 4/0 polypropilen sutures. After closing
the fistula, an open distal anastomosis with a 30mm branched
dacron graft was performed in arch zone 2 (just at the level of
the origin of left common carotid artery). Both brachiocephalic
trunk and left common carotid artery were independently
reimplanted (Figure 2, panel B). Since the anastomotic site was
slightly dilated, an elephant trunk technique was performed.
After completion of arch replacement and supraaortic vessels
reconstruction, systemic perfusion and rewarming were initiated.
Proximally, the previous aortic bioprosthesis was resected and
aortic root replacement was performed with 25mm mechanical
valved conduit (Bentall procedure). The great dilatation and
severe displacement of the aortic sinuses made coronary ostia
mobilization impossible for independent reimplantation in the
aortic root graft. Reimplantation of both ones with a separate
6mm dacron graft anastomosed laterally to the aortic graft was
needed, according to Cabrol´s modification of Bentall procedure
(Figure 2 & 3, black asterisk). The patient had an uneventful
postoperative course and was discharged in postoperative day
8. Postoperative CT aortic angiogram confirmed the excellent
surgical result (Figure 3, panel A & B). The patient is doing well
and remains asymptomatic 6 month after surgery.
Giant ascending aortic aneurysm, defined as an aneurysm
with more than 10cm diameter, is a rare condition. There is a
rapid increase in the risk of dissection or rupture when the aortic
diameter is > 6cm for the ascending aorta and > 7cm for the
descending aorta [1,6]. Most of the ascending aortic aneurysms are
asymptomatic at the time of diagnosis. However, giant aneurysms
are more frequently revealed by clinical signs of compression or
erosion of adjacent structures such as superior vena cava as in this
case, pulmonary artery, chest wall, oesophagus and trachea [7,8].
Superior Vena Cava (SVC) syndrome is the clinical presentation
derived from stenosis or obstruction of superior vena cava.
Head, face and upper body swelling is the most common sign,
but clinical presentation can be variable depending on the
velocity of instauration. In this case, the acute onset of chest
pain, hypotension and upper body cyanosis suggest aneurysmal
expansion with rupture to superior vena cava and aortocaval
fistula formation. Sudden increase in central venous pressure and
acute left to right shunt explain the rapid clinical presentation and
the poor hemodynamic tolerance.
The most frequent cause of SVC syndrome is a thoracic
malignancy (pulmonary and lymphoid neoplasms) while,
nowadays, cardiovascular disease is very unusual . It´s
been previously published its association with thoracic aortic
pathologies such as aortic dissection, aortic pseudoaneurysm,
giant ascending aneurysm and previous cardiac surgery .
Ascending aortic aneurysms in the setting of syphilitic
aortitis are inflammatory and often very large, therefore clinical
presentation related to compression and erosion of adjacent
structures are not uncommon in this clinical scenario. There are
previous reports of syphilitic aortic aneurysms presenting with
compression of surrounding structures as pulmonary artery, right
atrium, superior vena cava and trachea [7,8]. Sekine et al. 
reported SVC syndrome with aorta to superior vena cava fistula in
a patient with syphilitic aortitis .
In this case, the giant ascending aneurysm is probably
associated with bicuspid aortic valve aortopathy as this patient
had previous history of aortic valve replacement for native
bicuspid valve endocarditis. Moreover, pathologic analysis
revealed a medial degeneration with loss of smooth muscle
cells instead of endarteritis obliterans of adventitia, suggesting a
connective tissue disorder more than syphilitic histology changes
. Serologic testing performed after surgery was negative
in our patient. Although ascending aorta to superior vena cava
fistula has been previously published in association with syphilitic
aneurysm, our case is the first reported in a patient with bicuspid
aortic valve related aneurysm.
The large size of the aneurysm, which was very close to
the sternum, as well as the previous cardiac surgery made the
surgical approach very challenging. Peripheral cannulation,
establishment of cardiopulmonary bypass prior to chest opening
and hypothermia play an important role in surgical approach of
giant aortic aneurysms, especially in redo cases.
According to previously published similar cases, we decided
to initiate cardiopulmonary bypass prior to sternotomy in order
to decompress the aneurysm avoiding aortic injury during chest
This case demonstrates the potential of giant ascending
aortic aneurysms to cause superior vena cava syndrome and
right heart failure in the acute setting. Although improvements
in cardiovascular imaging and early detection of thoracic aortic pathologies have decrease the incidence of giant thoracic
aneurysms, we have to consider aortic diseases as possible cause
when a patient, especially with previous cardiac surgery, presents
with this clinical conundrum.