Case
report
Acute Stanford type A aortic dissection associated with aortic coarctation repaired by Tirone
E. David & debranching techniques
with combined axillar and femoral perfusion: a case
report
Disección aórtica
aguda Stanford A asociada a coartación de aorta reparada por técnica de Tirone E. David y debranchingcon perfusión
combinada axilar y femoral: reporte de un caso
Francisco
Guerrero1
https://orcid.org/0000-0002-7515-5993
Karla
Bautista1
https://orcid.org/0000-0003-2293-3218
Giuseppe
Salas1
https://orcid.org/0000-0002-2719-6483
Edmy Callalli1,
Josías
C. Ríos 2
https://orcid.org/0000-0002-4396-7954
DOI:
https://doi.org/10.47487/apcyccv.v2i3.154
Abstract
We present the case of a 38-year-old male with a diagnosis of Stanford A aortic dissection and associated coarctation of the thoracic
aorta. Acute dissection associated with coarctation of the aorta
is a rare problem and difficult to manage surgically.
Establishing a cardiopulmonary
bypass (CPB) with adequate flows is the
main objective of the procedure;
optimal cannulation ensures the protection of cerebral and
visceral organs. We successfully performed aortic valve re-implantation
surgery (T. David Surgery),
replacement of the ascending aorta and aortic arch, as well as debranching of the supra-aortic
trunks. The cannulation technique was axillary and femoral to guarantee flows
through the coarctation area.
Keywords: Aneurysm, dissecting; Aortic coarctation; Aortic valve insufficiency; Aortic valve prolapse
(source: MeSH NLM)
Resumen
Presentamos el caso de un varón de 38
años con diagnóstico de disección de aorta Stanford A y coartación de aorta
torácica asociada. La disección aguda asociada a la coartación de la aorta es
un problema raro y difícil de manejar quirúrgicamente. Establecer un bypass
cardiopulmonar con flujos adecuados es el principal objetivo del procedimiento;
una canulación óptima asegura la protección
de órganos cerebrales y viscerales. Realizamos con éxito una cirugía de
reimplante de válvula aórtica (cirugía de T. David), reemplazo de la aorta
ascendente y del arco aórtico, además de debranching
de los troncos supraaórticos. La técnica de
canulación fue axilar y femoral para garantizar flujos a través de la zona de coartación.
Palabras
clave:
Aneurisma Disecante; Coartación Aórtica; Insuficiencia de la Válvula Aórtica;
Prolapso de la Válvula Aórtica (fuente: DeCS BIREME).
Introduction
Acute aortic dissection is a rare and fatal disorder, due to
the separation of the layers
of the aortic
wall. A tear in the intimal layer
produces a retrograde or proximal blood
flow between intima and media,
which causes the formation of a false lumen within the middle
tunic. Statistics show that 60% of patients
die within 30 days if they do not
undergo surgery (1). The Stanford A dissection causing a rupture on the aortic
root, ascending aorta, aortic arch, supra- aortic trunks, and descending aorta is associated with very high mortality.
When aortic coarctation is also found, maintaining
good cerebral and systemic perfusion during surgery is a great
challenge (2). This type of dissection
should be repaired immediately with lifesaving emergency surgery. Therefore, an adequate cannulation
and temperature control strategy
must be planned to guarantee adequate
cerebral perfusion during the time of circulatory
arrest and also guarantee a good systemic perfusion during surgery (1,3).
Our surgical team used
both femoral and axillary cannulation strategies as a solution to perfusion problems due to coarctation of the aorta. We
performed replacement of the aortic
root, aortic arch and supra-aortic trunks with valve
preservation and coronary reimplantation.
Case report
A 38-year-old man,
without cardiovascular risk
factors, and no history of Marfan syndrome, was admitted to
the emergency unit after an episode
of chest and lumbar pain. On physical
examination, we found blood pressure
150/66 mmHg (right arm), 144/53 (left arm), and 125/45 in lower limbs; a heart rate of 72 beats
per minute, and a grade III systo-diastolic murmur in the right
parasternal focus. Transthoracic echocardiography revealed an aneurism
of the ascending
aorta and arch (diameter of 8 cm), with aortic root and arch dissection (Stanford A / De Bakey II). The flap prolapsed into the left
ventricle in the cardiac diastole. Severe aortic regurgitation
(type IA) and bicuspid aortic valve were
observed. The left ventricular ejection fraction (LVEF) was 55%, the right ventricle
systolic function was preserved, and no motility disorders were found. Computed
tomography angiogram showed a Stanford Type A aortic dissection (with supra-aortic trunks dissection extended to descending aorta) and coarctation of the thoracic aorta (Figure 1).
The cardiac team decided
emergency surgical treatment. A median sternotomy was performed, cannulation strategies for cardiopulmonary bypass included arterial cannulation
both in the the right axillary
(8 mm prosthesis as an interposed graft) and in the right femoral arteries, venous cannulation was performed in the right atrium directly
(Figure 2). Surgical findings
showed a severely dilated ascending aorta (approximately 80 mm) with dissection signs. After aortic clamping, we used HTK Custodiol®
for cardiac protection directly in both coronary arteries.
The sectioned and excised aorta showed a dissection flap extending to the
three supra-aortic trunks and into the coronary sinuses
near the border of both
coronary arteries. Bicuspid aortic valve (three sinuses,
right-left fusion, symmetric type) was observed.
We decided to repair
the root using the re-implantation
technique (David´s original
procedure) and replace the aortic arch
and the ascending aorta with a Dacron prosthesis.We used a 28 mm prosthesis for the aortic root replacement. After re-implantation of the aortic valve,
we repaired both aortic leaflets
(repair of the prolapse and suture of the raphe
of the fused
leaflet), and then coronary arteries were re-implanted. With the patient
in deep hypothermia (24 °C)
prepared for circulatory arrest, we clamped the brachiocephalic trunk, so that total anterograde cerebral perfusion was obtained with
bilateral carotid artery perfusion assured. We used a 28 mm prosthesis for ascending aorta
and arch replacement.
The anastomosis between the thoracic aorta and the graft was performed
under circulatory arrest. The next
step was the anastomosis of supra-aortic vessels (brachiocephalic and left carotid arteries),
performed by debranching technique with an adapted bifurcated
prosthesis (20 x 10 mm). The
proximal prothesis-to-prothesis anastomosis was then performed.
After systemic
rewarming, the aorta clamp was removed, and the myocardium was re-perfused again. Cardiopulmonary bypass was easily turned
off with low doses of inotropic support.
Total cardiopulmonary bypass
(CPB) time was 3 hours 39
minutes, aorta cross-clamp 3 hours
6 minutes, and cardiac arrest
26 minutes. In the critical
care unit, the patient´s evolution
was satisfactory with no neurological disorders. After a six-month follow-up, the patient was in functional class I, with normal left ventricular function and a mild aortic valve insufficiency
with adequate gradients (medium: 8 mmHg).
Discussion
Acute Stanford A aortic dissection is
a complex life-threatening disease, associated with high morbidity and mortality and is even more challenging when is combined
with coarctation of the aorta (3). The characteristics of the blood
flow in the ascending aorta make this region the
most frequently affected (4,5). Stanford A dissection
is always a surgical challenge due to the
involvement of the aortic root
(in some cases the coronary arteries), the ascending aorta, and the supra-aortic trunks, which puts
cerebral circulation at risk. If we
consider the existence of coarctation
of the thoracic
aorta, the difficulty in maintaining adequate cerebral and
systemic perfusion during surgery increases. In fact, the clue and the
key to good
results start with a good perfusion
strategy, to preserve cerebral
and systemic blood flow during surgery
and thus avoid cerebral,
renal and splanchnic ischemia
(6).
There are many reports about
cannulation techniques in these patients, we decided to
solve the problem by placing
a “Y” connection in the
arterial line and performed double
peripheral cannulation. We used axillary
cannulation to reduce complications in the nervous central system performing anterograde perfusion including the pre aortic coarctation zone (7). There are no doubts about the advantage
of this type
of cannulation, but to protect
the perfusion of the organs
beyond coarctation, we performed femoral cannulation (the site of choice
for many years in some centers), this is a safe
but non- physiological perfusion, and it is not recommended
only when the dissection extends to the
iliac or femoral arteries and when the thoracoabdominal aorta has severe atherosclerosis (1-3,8).
Lin-Chen et al., in a two-year prospective study, showed better
results (fewer neurological symptoms, renal and hepatic insufficiency, limb ischemia, and paraplegia) using axillary and femoral cannulation,
their complication rates did not
exceed 2.36% (7).
The American Association of Thoracic Surgery Consensus Guidelines for Bicuspid Aortic
Valve-Related Aortopathy states that patients
with a bicuspid aortic valve with
symmetric leaflets are candidates for valve-sparing surgery. In the case of valve
leaflet prolapse, this can be corrected with the plication
of the free edge. Long-term survival and reoperation-free survival results are greater than 80% at 10 years in large series (9-11).
The key
point of this case report is the incidental finding of coarctation
of the aorta in acute type A aortic
dissection, which in addition to being
a rare combination, increased the difficulty
in achieving adequate perfusion during surgery.