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Current Treatment Options in Cardiovascular Medicine
DOI 10.1007/s11936-012-0226-1
Vascular Disease (H Gornik and E Kim, Section Editors)
Update in the Management
of Aortic Dissection
Jip L. Tolenaar, MD1
Guido H. W. van Bogerijen, MD1
Kim A. Eagle, MD, FACC2
Santi Trimarchi, MD, PhD1,*
Address
*,1
Thoracic Aortic Research Center, IRCCS Policlinico San Donato, Cardiovascu-
lar Center E. Malan, University of Milan, Via Morandi, 30, 20097, San Donato
Milanese, MI, Italy
Email: Santi.trimarchi@unimi.it
2
University of Michigan Health System, Ann Arbor, MI, USA
* Springer Science+Business Media New York 2013
Keywords Thoracic/aortic I Thoracic aortic dissection I TEVAR (thoracic endovascular repair) I
Cardiopulmonary bypass I Open surgical repair I Medical treatment
Opinion statement
Recent improvements in diagnosis, peri-operative management, surgical techniques
and postoperative care have resulted in decreased mortality and morbidity in acute
aortic dissections (AAD). The classic treatment algorithm indicates that type A
patients require direct surgical intervention and type B patients should be treated
medically, in absence of complications. Initial medical treatment is adopted in all
AAD patients, as it reduces propagation of the dissection and aortic rupture. In
type A aortic dissection (TAAD) several techniques have contributed to major
changes in the surgical approach, such as cerebral protection using moderate circu-
latory arrest, selective cerebral perfusion, and aortic valve sparing with root re-
placement. In TAAD with involvement of the descending aorta, thoracic
endovascular aortic repair (TEVAR) can be performed as a part of a complex hybrid
procedure, in which surgical ascending/arch repair is combined with the placement
of a stent graft in the descending aorta. Future developments in stent graft tech-
nologies might broaden the usefulness of TEVAR for the total endovascular repair of
TAAD. In complicated type B aortic dissection (TBAD), the use of TEVAR has become
the therapy of first choice. By covering the proximal entry tear, the stent graft
reduces the pressurization of the false lumen, treating malperfusion and inducing
favorable aortic remodeling. In uncomplicated TBAD, TEVAR has been used to pre-
vent long term complications, such as aortic aneurysm, but this concept is not yet
routinely recommended. Regardless of their initial treatment, all AAD patients
should be administered with strict antihypertensive management combined with im-
aging surveillance and careful periodic clinical follow-up.
Introduction
Acute aortic dissection is a life-threatening disease
with an increasing incidence up to 14 per 100.000
people/year, due to aging population and im-
proved imaging modalities [1]. Prognosis of aortic
dissection in untreated patients is poor, with a
mortality of 20–30 % before hospital admission
and 50 % will expire within the first 48 hours
[1, 2]. The Stanford classification is most com-
monly used and classifies dissections into TAAD
involving the ascending aorta and TBAD, without
ascending involvement. Several conditions are as-
sociated with AAD, related to the increase of shear
stress on the aortic wall or the decrease of vascu-
lar wall strength. Systemic hypertension, increas-
ing age, and atherosclerosis are the most
important risk factors, while other risk factors like
congenital bicuspid or unicommissural aortic
valves, cocaine abuse and pregnancy are less com-
mon [3–5]. Connective tissue disorders like Mar-
fan, Ehlers-Danlos syndrome, and Loeys-Dietz
syndrome, result in structural weakness of the
aortic wall and are strongly associated with aortic
dissections [3, 4, 6, 7]. The diagnosis of aortic
dissection can be challenging because of the di-
verse clinical presentation, which can mimic more
common disorders such as myocardial infarction.
The majority of patients present with an abrupt
onset of pain, tearing or ripping in nature and lo-
cated in the chest and/or back [4, 6]. Obstruction
of the aortic branches can lead to malperfusion
and end-organ ischemia, which are the most
feared complications. Clinical presentation can in-
clude shock, cardiac tamponade, cardiac failure,
myocardial ischemia/infarction, spinal cord ische-
mia, cerebrovascular accidents, mesenteric malper-
fusion, renal failure, pulse deficits and limb
ischemia [4, 8].
In TAAD patients, transesophageal echocardio-
gram (TEE) is the preferred imaging modality as
it offers the advantage of immediate diagnosis.
The role of TEE can be expanded during the oper-
ation to assess cardiac and valve function. Com-
puted tomographic imaging (CT) is the most
widely adopted imaging modality to detect AAD,
as it provides a more detailed visualization of
the aorta and should be used at low threshold in
patients suspected of AAD. Despite current devel-
opments, the classic treatment algorithm, where
TAAD and complicated TBAD require surgical in-
tervention and uncomplicated TBAD can be treated
medically, remains intact. Improvements in the pe-
ri-operative management, surgical techniques and
postoperative care have resulted in a significant de-
crease in AAD mortality over the last decades. The
introduction of TEVAR has further improved the
outcome. In this review we report the up-to-date
knowledge regarding the current management
strategies of AAD.
Treatment
& In AAD patients the goal of the initial medical management is to
prevent progression of the dissection and aortic rupture. Blood
pressure should be regulated between 100 and 120 mmHg systolic
and ≤ 60–70 mmHg diastolic, and the heart rate G 60 beats/minute
[7].
& The pharmacologic management consists of intravenous β-blockers
or combined α and β-blockers, utilizing primarily short-acting agents
as they can be easier titrated to optimal levels [7].
& In patients who are unresponsive, have untoward side effects or
contra-indications to β-blockers, calcium-channel blockers and
angiotensin converting enzyme (ACE)-inhibitors are an alterna-
tive therapy. Most typically this would include a non-dihydro-
Vascular Disease (H Gornik and E Kim, Section Editors)
pyridine calcium-channel blocker (i.e., diltiazem or verapamil) to
maintain a low heart rate.
& Surgical and endovascular interventions are utilized to prevent car-
diac and aortic complications.
& Surgical repair is indicated in all TAAD, while TEVAR is reserved for
complicated TBAD. For uncomplicated TBAD, medical treatment is
currently the initial strategy of choice.
Diet and lifestyle
& Diet and lifestyle restrictions should be focused on avoiding situa-
tions in which the mean arterial pressure will increase.
& To effectively control blood pressure and cholesterol, low fat and low
salt diets are suggested.
& Cocaine or other stimulating drugs such as methamphetamine are
associated with aortic rupture and should be avoided.
& Heavy weight lifting, competitive sports, or a job involving isometric
exercise may induce hypertension and provoke aortic dissection or
aortic rupture and should be avoided.
& The importance of adherence to medical treatment must be em-
phasized, especially regarding the lifelong treatment with antihy-
pertensive agents.
Pharmacologic treatment
& The initial management of AAD is directed to clinically stabilize
the patient and prevent propagation of the dissection and aortic
rupture. Immediate adequate invasive haemodynamic monitor-
ing is advised, through an intra-arterial catheter and urinary
catheter [2, 4, 9].
& In patients presenting with refractory hypotension, rapid volume
expansion should administered in combination with vasopressors
such as nor-epinephrine or phenylepinephrine to maintain organ
perfusion, before the patient receives definitive surgical repair [6].
& In patients with hypertension at presentation, medical therapy is
administered to decrease aortic wall stress. This can be accom-
plished by decreasing the force of the left ventricular contraction
and lowering systolic blood pressure and heart rate. Blood pres-
sure should be regulated between 100 and 120 mmHg systolic
and ≤ 60–70 mmHg diastolic, and the heart rate G 60 beats/
minute [6].
& Pain should be treated promptly with intravenous opiates. Persisting
pain may indicate progression of the dissection or an impending
rupture. In TBAD patients, this condition should be considered as a
complication requiring intervention.
& During hospital stay the intravenous antihypertensive regimen is
converted into oral medication [10].
Update in the Management of Aortic Dissection Tolenaar et al.
& All AAD patients require life-long follow up for blood pressure reg-
ulation, as well imaging surveillance to detect aneurismal aortic en-
largement.
Specific drugs
Beta adrenoreceptor antagonists (β-blockers)
Intravenous β-blockers are the treatment of choice for patients suspected
for AAD. The β-blockade has a negative inotropic and chronotropic ef-
fect, which prolongs the diastolic filling time and results consequently in
an increased left ventricle end-diastolic volume, thereby lowering blood
pressure and heart rate.
Standard dosage Titrate dosage for symptom control, establish a target heart rate of 50–60
beats/minute and systolic blood pressure down to 100–120 mm Hg.
Contraindications Sick sinus syndrome, 2nd
and 3rd
degree atrioventricular block (AV block),
prolonged first degree AV block without pacemaker in place (PR interval 9
0.24 sec), cardiogenic shock, sinus bradycardia, hypotension (systolic blood
pressure of less than 100 mm Hg), severe bronchospastic disease, congestive
heart failure.
Main drug interactions Combined use of a β-blocker with verapamil or diltiazem and specific an-
tiarrhythmic medications (e.g., amiodarone) increases the risk of bradycardia
and AV block.
Main side effects Bradycardia, possible wheezing in susceptible patients. Rarely hyperkalemia
in elderly patients with diabetes or in combination with an angiotensin-
converting enzyme inhibitor (ACE-inhibitor).
Special points -
Cost effectiveness Adequate β-blockade can usually be achieved imntavenous with metoprolol
or propanolol which are relatively inexpensive medicine, while esmolol is
expensive and reserved for the very initial treatment.
Sodium nitroprusside
Nitroprusside is administered in the early setting of AAD patients with
refractory hypertension after initiation of β -blocker therapy. It induces
venous and arterial vasodilatation, thereby increasing the cardiac output.
Vasodilator therapy may be associated with reflex tachycardia that
increases the aortic wall stress, which potentially can cause expansion
and propagation of thoracic aortic dissection. For this reason, vasodilator
therapy should only be administered after adequate heart rate control.
Standard dosage Titrate dosage for symptom control, establish a target systolic blood pressure
down to 100–120 mm Hg.
Contraindications Significant hypotension, inadequate cerebral circulation, optic atrophy, de-
creased systemic vascular resistance (SVR), concomitant unstable angina or
recent myocardial infarction, renal or hepatic failure.
Main drug interactions Generally, nitroprusside will synergistically decrease the blood pressure if
used with other vasodilators, especially with PDE5-1 (e.g., sildenafil).
Main side effects Hypotension, metabolic acidosis, brady- and tachyarrhythmia’s, cyanide
toxicity, myocardial ischemia. With impaired renal function and infusion of
Vascular Disease (H Gornik and E Kim, Section Editors)
the agent for more than 48 hours, the risk of thiocyanate toxicity is increased.
Reduction of arterial oxygen saturation in some patients with hypoxic pul-
monary vasoconstriction.
Special points Do not use as a sole agent in patients with aortic dissection because the
vasodilating effect can cause reflex tachycardia and an increased force of left
ventricular contraction. Avoid the use in patients with renal failure. Further,
infusion periods should be as short as possible because of the possible
thiocyanate and cyanide toxicity. Nitroprusside is light sensitive, for this
reason infusion bags should be protected from light.
Cost effectiveness Sodium nitroprusside in combination with an intravenous β-blocker con-
trols the blood pressure effectively in the majority of patients with AAD.
Sodium nitroprusside is inexpensive in comparison to other intravenous
agents used for rapid control of hypertension.
Non-dihydropyridine calcium channel blockers (Ca-blockers)
In patients not responding, or poorly tolerating β-blockers, Ca-blockers
can be used as an alternative. In addition, usage of Ca-blockers in TBAD
might reduce aortic expansion over time [10, 11].
Standard dosage Titrate dosage for symptom control, establish a target heart rate of 50–60
beats/minute and systolic blood pressure down to 100–120 mm Hg.
Contraindications Acute myocardial infarction, 2nd
or 3rd
degree AV block, sino-atrial node
dysfunction, hypotension, sick sinus syndrome, Wolff-Parkinson-White
syndrome, congestive heart failure, wide QRS tachycardia of uncertain origin,
concomitant intravenous use of a β-blocker.
Main drug interactions β-blockers, digoxin and cyclosporine.
Main side effects Myocardial infarction, dyspnea, ileus, flushing.
Special points -
Cost effectiveness Inexpensive and effective.
Angiotensine-converting enzyme inhibitors (ACE- inhibitors)
In absence of adequate blood pressure control, ACE-inhibitors should be
administered to reduce the blood pressure and to maintain adequate
end-organ perfusion. Intravenous administration of an ACE-inhibitor is
rarely needed for the treatment of AAD.
Standard dosage Titrate dosage to establish a target systolic blood pressure down to 100–
120 mm Hg.
Contraindications Angioedema on previous therapy, pregnancy. Caution in patients with renal
insufficiency, bilateral renal artery stenosis or elevated potassium levels, as
these agents increase the risk of renal failure.
Main drug interactions Severe hyperkalemia can occur, if the agent is used in combination with
potassium-sparing diuretics or potassium supplements. First-dose hypoten-
sion can occur, if the agent is used in combination with α-blocking agents,
such as doxazosin, prazosin or terazosin. The antihypertensive effects of an
ACE-inhibitor are augmented by other antihypertensive agents.
Main side effects Impairment of renal function, hyperkalemia, potential for hypotension,
angioedema, dry cough.
Special points ACE-inhibitors can be useful in case of unirenal malperfusion with con-
comitant increasing renin levels.
Update in the Management of Aortic Dissection Tolenaar et al.
Cost effectiveness Generic agents are very inexpensive.
Angiotensin receptor blockers (ARBs)
ARBs can be used as an alternative for ACE-inhibitors, when patients
have persistent cough after administration of ACE-inhibitors. In ad-
dition, there is increasing evidence that patients with Marfan syn-
drome may benefit from ARBs instead of ACE-inhibitors, as losartan
looks to be associated with a reduced ascending aortic growth rate
[12].
Standard dosage Titrate dosage to establish a target systolic blood pressure down to 100–
120 mm Hg.
Contraindications Caution in patients with renal insufficiency, bilateral renal artery stenosis or
elevated potassium levels, as these agents increase the risk of renal failure.
Main drug interactions Severe hyperkalemia can occur, if the agent is used in combination with
potassium-sparing diuretics or potassium supplements. The antihypertensive
effects of ARBs are augmented by other antihypertensive agents.
Main side effects Potential for hypotension, impairment of renal function, hyperkalemia.
The incidence of cough is lower with ARBs (1 %) compared with ACE-
inhibitors (10 %). The incidence of angioedema with ARBs (very rare)
is lower in comparison with ACE-inhibitors. ARBs can be used as an
alternative in patients who suffered from angioedema due to ACE-
inhibitors.
Special points The administration of ARBs should be initiated during hospitalization.
Cost effectiveness Generic agents are inexpensive.
Alpha adrenergic blocking agents (α-blockers)
If the blood pressure is still uncontrolled despite using a combination of
the earlier mentioned antihypertensive agents, addition of α-blockers can
be a valuable option. It lowers the peripheral vascular resistance by di-
lation of the arterioles and venules.
Standard dosage Titrate upward to achieve a systolic blood pressure down to 100–120 mm
Hg.
Contraindications (Orthostatic) hypotension, heart failure, myocardial infarction.
Main drug interactions Simultaneous use with other antihypertensive agents can have a synergistic
effect. Combination of use with PDE-5 inhibitors, such as sildenafil, can
cause hypotension.
Main side effects (Orthostatic) hypotension, respiratory- or urinary tract infection, dizziness,
palpitations, tachycardia, dyspnea.
Cost effectiveness It is a valuable therapeutic adjunct and inexpensive.
Hydralazine
Hydralazine lowers blood pressure by exerting a peripheral vasodi-
lating effect through a direct relaxation of vascular smooth muscle.
However, it is associated with reflex tachycardia and increase in the
left ventricular contractility, plasma renin levels and the release of
norepinephrine. For these reasons, this arteriolar dilator is considered
to be relatively contraindicated for patients with suspected acute
Vascular Disease (H Gornik and E Kim, Section Editors)
AAD, except when the heart rate is well controlled and hypertension
persists.
Interventional procedures
Transcatheter fenestration
Standard procedure Through the groin, a guidewire is utilized to puncture the septum flap which
is then dilated with a balloon or catheter, resulting in the restoration of the
equilibrium of pressures between the false and true lumen. Complementary
aortic branch stenting can be used to treat malperfusion [13, 14].
Contraindications Malperfusion due to a static obstruction by thrombus in the false lumen
Complications The transcatheter fenestration may fail, not resolving malperfusion and the
pressurization of the false lumen, which also may lead to an increased risk of
aortic expansion and rupture [13, 14].
Special points Requires great expertise and strict blood tests and imaging follow-up in the
acute setting. This procedure can be used not only in TBAD patients, but also
in TAAD to relieve malperfusion before undergoing definitive surgical
management.
Cost/cost-effectiveness Because the procedure is entirely endovascular and usually does not require
stent grafts, it is relatively inexpensive compared to TEVAR or open surgery.
TEVAR (thoracic endovascular aortic repair)
Standard procedure In patients with complicated TBAD, TEVAR has become the preferred ther-
apeutic option [15, 16]. Femoral artery cut down or a percutaneous approach
is used to introduce the guidewires and catheters. After aortography and with
TEE control, a covered stent graft is deployed under fluoroscopic guidance,
covering the proximal entry tear. In this fashion blood flow is diverted into
the true lumen, depressurizing the false lumen and promoting false lumen
thrombosis and aortic remodeling. Post deployment angiography is
obtained to evaluate the correct position of the graft and resolution of the
aortic complications. In case of poor results, like persistence of malperfusion,
another covered stent graft can be deployed up to the level of the celiac
trunk. If necessary, an uncovered aortic stent might be extended more distally
to the abdominal aorta, at the level of the visceral and renal arteries.
Contraindications Unfavorable anatomy prohibiting stent graft delivery. Such as extreme tor-
tuosity, severe atherosclerotic disease or the necessity of coverage a major
branch vessel. Retrograde extension of the dissection into the aortic arch
should be considered a contra-indication for standard TEVAR.
Complications TEVAR is associated with an in-hospital mortality between 2.6-9.8 % and
neurologic complications between 0.6–3.1 % [17, 18]. Stroke, paraplegia
and paraparesis, incidental occlusion of the left subclavian artery and inad-
equate placement of the graft are all significant complications of TEVAR [19].
The risk of paraplegia is related to the extension of the aorta that is covered,
previous aortic surgery and hypotension. Stroke is often due the presence of
severe arch atherosclerosis and mural thrombosis, which can serve as source
of embolus, especially due to the manipulation of catheters in the arch and
ascending aorta [19]. Coverage of the left subclavian artery (LSA) may be
associated with a higher incidence of stroke, paraplegia and paraparesis.
Revascularization of the LSA, typically with left subclavian to common ca-
Update in the Management of Aortic Dissection Tolenaar et al.
rotid artery transposition or bypass grafting, should be performed in all non-
emergent cases [16, 19, 20] . Additionally, the false lumen may remain
patent, which seems to be associated with chronic aortic dilatation. Other
complications are related to the device or procedure, including endoleaks,
migration or collapse of the stent graft and development of a false aneurysm
[17, 21].
Special points Patients affected by TBAD with refractory or recurrent pain or refractory
hypertension are at increased risk of relevant in-hospital mortality and may
benefit from early TEVAR [22]. Serial CT and MRI studies are required due to
the increased risk of stent graft or aortic related complications.
Cost/cost-effectiveness TEVAR has shown to be cost-effective compared to open surgery for com-
plicated TBAD in the short term [23]. Multiple prospective studies are cur-
rently running to determine the long term durability and cost-effectiveness of
TEVAR.
Surgery
Type a dissection
Ascending aorta replacement
Standard procedure All patients are treated under general anesthesia through a medium ster-
notomy, using extracorpeal circulation with moderate or deep hypothermic
circulatory arrest. The pericardium is excised, cardiopulmonary bypass is
established and cardiac arrest is instituted. During patient cooling, the aorta
may be cross-clamped in order to inspect the aortic root and valve. If the
aortic vale and root are free of concomitant disease, the proximal aortic graft
anastomosis is performed at supracommisural level with possible aortic re-
inforcement using Teflon strips [24]. In TAAD patients, aortic regurgitation is
present in up to 44 %, frequently resulting from commissural dehiscence,
annular dilatation, sclerotic, stenosed or destructed aortic valves [4]. These
patients may require a Bentall procedure with composite aortic valve-graft
replacement. However, in some patients the function of the aortic valve is
preserved and they may be treated with valve-sparing procedures such as the
David or Yacoub technique [25, 26]. Such operations should be primarily
reserved for patients affected by connective tissue disorders, young patients,
and those with ectatic aortic root. Nevertheless, valve-sparing operations are
technically demanding and are indicated only in high-volume centers with
expertise. Aortic arch management requires moderate/deep hypothermic
circulatory arrest. For better cerebral protection, antegrade cerebral perfusion
maybe established through catheters that are selectively advanced into the
innominate artery and left common carotid artery. The inspection of the arch
is conducted to identify additional entry-tears. In the presence of an entry
tear in the inner curvature or absence of additional arch complications, a
hemi-arch replacement is performed, with the distal anastomosis sutured in
a transverse manner in order to resect the inner curvature of the arch and
sparing the supra-aortic branches. In case of extensive tears in the aortic arch,
which is present in 15–20 % of all TAAD patients, as well in presence of arch
aneurysm, complex arch dissection, connective tissue disease or atheroma-
teous disease, total arch replacement should be considered. Supra-aortic
vessels may be reimplanted separately or with the use of the island technique
[27, 28]. Total arch repair is also advocated in TAAD patients to reduce long
term aneurysmatic complications of the descending aorta, being associated
Vascular Disease (H Gornik and E Kim, Section Editors)
with increased false lumen thrombosis and aortic remodeling [29, 30]. Re-
duced patency of the false lumen can be achieved with the use of extensive
aortic procedures, such as the elephant trunk technique [31], which require a
staged operation for treating thoracic and thoraco-abdominal aortic seg-
ments. Recently TEVAR has been introduced as a minimally invasive option
to treat the descending aorta during the staged operation or with simulta-
neous descending stent-grafting as a hybrid procedure.
Contraindications Dense stroke and coma are considered contra-indications, although recent
studies report a relatively good outcome [32]. Patients presenting with severe
malperfusion have a relative contra-indication [33]. Surgery is also not in-
dicated in patients with end stage malignant disease or other major comor-
bid illness associated with decreased life expectancy.
Complications Although technical advances have helped to improve surgical outcomes,
mortality rates still range between 10–25 % [9, 34]. Coagulopathic hemor-
rhage remains a significant cause of mortality and morbidity and reexplo-
ration may be required. Extensive blood transfusion can be also associated
with post-operative pulmonary failure. Stroke still remains an important
complication of this surgery, ranging around 4–8 % [9, 34]. Other postop-
erative neuropsychiatric complications like delirium or changes in memory
and cognition may be present in one-third of the patients. The incidence of
these complications seems to be related to brain ischemia time. Antegrade
brain perfusion has been reported to be beneficial to prevent these compli-
cations [35]. Extracorporeal circulation, renal failure, end-organ ischemia
and hypothermia can result in significant electrolyte and/or acid–base dis-
turbance and may necessitate volume reduction with conventional diuretic
therapy or extracorporeal dialysis.
Special points Currently, cannulation of the axillary artery or right subclavian artery has
shown to be superior compared to femoral artery cannulation, as it is seldom
involved in the dissection or in extensive atherosclerotic lesions [36]. A sig-
nificant advantage of this approach is that it allows both antegrade brain
perfusion during circulatory arrest, which reduces cerebral ischemic events, as
well as antegrade distal perfusion. The use of a conduit in case of small,
deeply located, or damaged axillary arteries has been advocated. The optimal
temperature during hypothermic circulatory arrest remains debated, as the
advantages of deep hypothermic technique (e.g., brain protection) may be
outweighed by the complications (prolonged extracorporeal circulation
time, coagulation disorders and increased systemic inflammatory response
syndrome). A recent study showed no influence on outcome of cerebral
perfusion temperatures, ranging between 15–30 °C [9].
Cost/cost-effectiveness Surgical procedures for TAAD must be instituted as soon as possible as most
patients will expire after admission.
Type B dissection
Standard procedure Due to advancement in endovascular techniques, the treatment paradigm for
TBAD patients has shifted, with open surgery reserved for patients in whom
endovascular techniques are not feasible or have failed. During surgery, ex-
posure of the descending aorta is obtained through a left posterolateral
thoracotomy. Cardiopulmonary bypass can be established in a partial
manner through the left atrium and femoral artery. In patients with retro-
grade extension with partial arch involvement, full cardiopulmonary bypass,
using the femoral artery and vein, may be instituted. In the latter setting,
hypothermic circulatory arrest may be performed, with or without selective
Update in the Management of Aortic Dissection Tolenaar et al.
antegrade perfusion, for treating left hemi-arch using an open proximal
aortic anastomosis. After resection of the proximal entry tear, distal aortic
graft anastomosis is performed at the level of the diaphragm, with rein-
forcement of the aortic wall with Teflon-strips. At this level the aortic lamella
can be fixed to the dissected aortic wall or resected in order to create a single
aortic lumen which will balance the blood pressure between the true and
false lumen. After this, the aortic graft is sutured distally. In case of visceral/
renal malperfusion, a surgical fenestration is needed in order to restore blood
flow to the compromised branches. Extensive thoraco-abdominal aortic re-
placement is associated with higher risk of paraplegia, therefore, in patients
undergoing this surgery reimplantation of the intercostal arteries between T8
and L1is recommended. For those patients who present with lower extremity
ischemia, surgical management may include infrarenal aortic fenestration
through a median laparatomy. Older patients affected by limb ischemia may
benefit from a femoral-femoral arterial bypass graft.
Contraindications Patients presenting with severe visceral malperfusion and spinal cord ische-
mia may not have an indication for surgery given a low likelihood of clinical
recovery and grim overall prognosis. End stage malignant disease and severe
COPD are considered relative contra-indications for surgical aortic repair.
Complications In patients with TBAD, overall surgical mortality is around 30 % [34]. The
outcome of surgical repair may depend on the preoperative condition of the
patient, as risk factors like older age and hypotension/shock are strongly
associated with a poor outcome. A more beneficial outcome may be expected
in patients with radiating pain, normotension at surgery, and reduced hy-
pothermic circulatory arrest time. Neurologic complications, such as spinal
cord ischemia, paraparesis and stroke, are associated with the extent and
duration of the operation [16, 34, 37]. Other notable complications are
cardiac, respiratory and renal failure, visceral ischemia or peritonitis, sepsis,
and limb ischemia [16, 34, 37].
Special points For inpatients at higher risk for spinal cord ischemia, cerebrospinal fluid
drainage and perfusion pressure optimization are recommended [6]. Al-
though not routinely used, neurophysiologic monitoring of the spinal cord
can detect spinal cord ischemia, guiding hemodynamic optimization and the
potential reimplanation of intercostals arteries [38].
Cost/cost-effectiveness Surgery is only indicated in those TBAD patients that cannot be treated with
an endovascular approach.
Emerging therapies
TEVAR in uncomplicated TBAD
Standard procedure Among uncomplicated TBAD, 90 % survive hospital admission after
adequate antihypertensive therapy [5]. Conservative treatment in com-
bination with close surveillance is justified in order to detect aneurys-
matic dilatation or progression of the dissection, which is the most
feared complication in the long term, involving about 30–40 % of
patients [2, 39]. Based on these findings, it has been proposed that
uncomplicated TBAD might be treated with stent graft placement. Al-
though the INSTEAD (Investigation of STEnt Grafts in Patients with Type
B Aortic Dissection) trial did not show beneficial results for uncompli-
cated TBAD treated with TEVAR versus those managed medically in
terms of mortality [39] the one-year results of the ADSORB (A European
Vascular Disease (H Gornik and E Kim, Section Editors)
Study on Medical Management Versus TAG Device + Medical Manage-
ment for Acute Uncomplicated Type B Dissection) trial showed more
frequent false lumen thrombosis and aortic remodeling in those patients
with TBAD treated with TEVAR compared to those managed medically
[40].
Contraindications Comparable with TEVAR.
Complications Comparable with TEVAR.
Special points In order to stratify this cohort of patients, clinical, genetic and radiologic
predictors could identify those patients with TBAD that might benefit from
early intervention.
Cost/cost-effectiveness Currently TEVAR in uncomplicated TBAD has failed to show superior out-
come and is much more expensive than medical treatment alone. In the
absence of long-term outcome data, as well as the potential complications
related to TEVAR, medical treatment is preferred.
Hybrid procedures
Standard procedure As previously reported, TEVAR can be used in association with open
ascending/arch repair for treating extensive TAAD patients, simulta-
neously or in a staged procedure. In chronic arch and descending post-
dissecting aortic aneurysms, different hybrid approaches can be adopted,
consisting of aortic arch debranching, stented elephant trunk and frozen
elephant trunk [41, 42]. During debranching procedures, the supra-aortic
vessels are bypassed using surgical grafts from the ascending aorta and
the operation is finalized by placement of a stent graft into the arch, in
the same or later stage. TEVAR has been also adopted to complete the
second stage of the elephant trunk technique for treating descending
aortic diseases. Recently, hybrid “frozen elephant trunk” prostheses,
which are represented by a surgical graft with an incorporated stent graft,
have been introduced to manage extensive aortic diseases simultaneously
[31].
Contraindications In patients with previous coronary artery bypass grafting, where surgical
grafts for the aortic debranching needed to be sutured in the ascending aorta,
as well in those with unfavorable anatomy prohibiting safe stent graft delivery.
Complications Hybrid procedures for aortic arch dissections carry a considerable risk with a
pooled mortality of 9.8 %, perioperative stroke of 4.3 % and spinal cord
ischemia of 5.8 % [41].
Special points These procedures are not routinely performed, but are used for patients with
multiple comorbidities, considered unsuitable for open repair in experi-
enced/high volume centers.
Cost/cost-effectiveness Since initial results are comparable with open surgical repair among high risk
patients with multiple comorbidities, this approach seems justified although
long-term follow-up data should be awaited.
Total endovascular treatment of TAAD
Total endovascular treatment of TAAD is currently in an experimental
phase and only few case-reports have been reported [43, 44]. Stent
graft placement in the TAAD might be feasible in selected cases, as
the landing zone needs to be evaluated very accurately, being in the
vicinity of the supra-aortic branches and the coronary arteries.
Update in the Management of Aortic Dissection Tolenaar et al.
Developments of modular branched or fenestrated stent grafts, pos-
sibly with incorporated composite aortic valves, potentially harbor
the future in the treatment of TAAD patients.
Disclosure
Dr. Eagle is the Principal Investigator of IRAD which is supported by grants from the following organizations:
Gore, Mardigian Foundation, Hewlett Foundation, and Thomas Varbedian Fund for Aortic Research. He is also
a consultant to the National Institutes of Health Heart, Lund and Blood Institute where he is Study Chair of its
GenTAC (Genetically Triggered Aortic Conditions) Registry.
Dr. Trimarchi has been a speaker for Gore and Medtronic.
Dr. Jip L. Tolenaar and Dr. Guido H.W. van Bogerijen reported no conflicts of interest relevant to this article.
References and Recommended Reading
Papers of particular interest, published recently, have been
highlighted as:
• Of importance
•• Of major importance
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Current treatment options for aortic dissection

  • 1. Current Treatment Options in Cardiovascular Medicine DOI 10.1007/s11936-012-0226-1 Vascular Disease (H Gornik and E Kim, Section Editors) Update in the Management of Aortic Dissection Jip L. Tolenaar, MD1 Guido H. W. van Bogerijen, MD1 Kim A. Eagle, MD, FACC2 Santi Trimarchi, MD, PhD1,* Address *,1 Thoracic Aortic Research Center, IRCCS Policlinico San Donato, Cardiovascu- lar Center E. Malan, University of Milan, Via Morandi, 30, 20097, San Donato Milanese, MI, Italy Email: Santi.trimarchi@unimi.it 2 University of Michigan Health System, Ann Arbor, MI, USA * Springer Science+Business Media New York 2013 Keywords Thoracic/aortic I Thoracic aortic dissection I TEVAR (thoracic endovascular repair) I Cardiopulmonary bypass I Open surgical repair I Medical treatment Opinion statement Recent improvements in diagnosis, peri-operative management, surgical techniques and postoperative care have resulted in decreased mortality and morbidity in acute aortic dissections (AAD). The classic treatment algorithm indicates that type A patients require direct surgical intervention and type B patients should be treated medically, in absence of complications. Initial medical treatment is adopted in all AAD patients, as it reduces propagation of the dissection and aortic rupture. In type A aortic dissection (TAAD) several techniques have contributed to major changes in the surgical approach, such as cerebral protection using moderate circu- latory arrest, selective cerebral perfusion, and aortic valve sparing with root re- placement. In TAAD with involvement of the descending aorta, thoracic endovascular aortic repair (TEVAR) can be performed as a part of a complex hybrid procedure, in which surgical ascending/arch repair is combined with the placement of a stent graft in the descending aorta. Future developments in stent graft tech- nologies might broaden the usefulness of TEVAR for the total endovascular repair of TAAD. In complicated type B aortic dissection (TBAD), the use of TEVAR has become the therapy of first choice. By covering the proximal entry tear, the stent graft reduces the pressurization of the false lumen, treating malperfusion and inducing favorable aortic remodeling. In uncomplicated TBAD, TEVAR has been used to pre- vent long term complications, such as aortic aneurysm, but this concept is not yet routinely recommended. Regardless of their initial treatment, all AAD patients should be administered with strict antihypertensive management combined with im- aging surveillance and careful periodic clinical follow-up.
  • 2. Introduction Acute aortic dissection is a life-threatening disease with an increasing incidence up to 14 per 100.000 people/year, due to aging population and im- proved imaging modalities [1]. Prognosis of aortic dissection in untreated patients is poor, with a mortality of 20–30 % before hospital admission and 50 % will expire within the first 48 hours [1, 2]. The Stanford classification is most com- monly used and classifies dissections into TAAD involving the ascending aorta and TBAD, without ascending involvement. Several conditions are as- sociated with AAD, related to the increase of shear stress on the aortic wall or the decrease of vascu- lar wall strength. Systemic hypertension, increas- ing age, and atherosclerosis are the most important risk factors, while other risk factors like congenital bicuspid or unicommissural aortic valves, cocaine abuse and pregnancy are less com- mon [3–5]. Connective tissue disorders like Mar- fan, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome, result in structural weakness of the aortic wall and are strongly associated with aortic dissections [3, 4, 6, 7]. The diagnosis of aortic dissection can be challenging because of the di- verse clinical presentation, which can mimic more common disorders such as myocardial infarction. The majority of patients present with an abrupt onset of pain, tearing or ripping in nature and lo- cated in the chest and/or back [4, 6]. Obstruction of the aortic branches can lead to malperfusion and end-organ ischemia, which are the most feared complications. Clinical presentation can in- clude shock, cardiac tamponade, cardiac failure, myocardial ischemia/infarction, spinal cord ische- mia, cerebrovascular accidents, mesenteric malper- fusion, renal failure, pulse deficits and limb ischemia [4, 8]. In TAAD patients, transesophageal echocardio- gram (TEE) is the preferred imaging modality as it offers the advantage of immediate diagnosis. The role of TEE can be expanded during the oper- ation to assess cardiac and valve function. Com- puted tomographic imaging (CT) is the most widely adopted imaging modality to detect AAD, as it provides a more detailed visualization of the aorta and should be used at low threshold in patients suspected of AAD. Despite current devel- opments, the classic treatment algorithm, where TAAD and complicated TBAD require surgical in- tervention and uncomplicated TBAD can be treated medically, remains intact. Improvements in the pe- ri-operative management, surgical techniques and postoperative care have resulted in a significant de- crease in AAD mortality over the last decades. The introduction of TEVAR has further improved the outcome. In this review we report the up-to-date knowledge regarding the current management strategies of AAD. Treatment & In AAD patients the goal of the initial medical management is to prevent progression of the dissection and aortic rupture. Blood pressure should be regulated between 100 and 120 mmHg systolic and ≤ 60–70 mmHg diastolic, and the heart rate G 60 beats/minute [7]. & The pharmacologic management consists of intravenous β-blockers or combined α and β-blockers, utilizing primarily short-acting agents as they can be easier titrated to optimal levels [7]. & In patients who are unresponsive, have untoward side effects or contra-indications to β-blockers, calcium-channel blockers and angiotensin converting enzyme (ACE)-inhibitors are an alterna- tive therapy. Most typically this would include a non-dihydro- Vascular Disease (H Gornik and E Kim, Section Editors)
  • 3. pyridine calcium-channel blocker (i.e., diltiazem or verapamil) to maintain a low heart rate. & Surgical and endovascular interventions are utilized to prevent car- diac and aortic complications. & Surgical repair is indicated in all TAAD, while TEVAR is reserved for complicated TBAD. For uncomplicated TBAD, medical treatment is currently the initial strategy of choice. Diet and lifestyle & Diet and lifestyle restrictions should be focused on avoiding situa- tions in which the mean arterial pressure will increase. & To effectively control blood pressure and cholesterol, low fat and low salt diets are suggested. & Cocaine or other stimulating drugs such as methamphetamine are associated with aortic rupture and should be avoided. & Heavy weight lifting, competitive sports, or a job involving isometric exercise may induce hypertension and provoke aortic dissection or aortic rupture and should be avoided. & The importance of adherence to medical treatment must be em- phasized, especially regarding the lifelong treatment with antihy- pertensive agents. Pharmacologic treatment & The initial management of AAD is directed to clinically stabilize the patient and prevent propagation of the dissection and aortic rupture. Immediate adequate invasive haemodynamic monitor- ing is advised, through an intra-arterial catheter and urinary catheter [2, 4, 9]. & In patients presenting with refractory hypotension, rapid volume expansion should administered in combination with vasopressors such as nor-epinephrine or phenylepinephrine to maintain organ perfusion, before the patient receives definitive surgical repair [6]. & In patients with hypertension at presentation, medical therapy is administered to decrease aortic wall stress. This can be accom- plished by decreasing the force of the left ventricular contraction and lowering systolic blood pressure and heart rate. Blood pres- sure should be regulated between 100 and 120 mmHg systolic and ≤ 60–70 mmHg diastolic, and the heart rate G 60 beats/ minute [6]. & Pain should be treated promptly with intravenous opiates. Persisting pain may indicate progression of the dissection or an impending rupture. In TBAD patients, this condition should be considered as a complication requiring intervention. & During hospital stay the intravenous antihypertensive regimen is converted into oral medication [10]. Update in the Management of Aortic Dissection Tolenaar et al.
  • 4. & All AAD patients require life-long follow up for blood pressure reg- ulation, as well imaging surveillance to detect aneurismal aortic en- largement. Specific drugs Beta adrenoreceptor antagonists (β-blockers) Intravenous β-blockers are the treatment of choice for patients suspected for AAD. The β-blockade has a negative inotropic and chronotropic ef- fect, which prolongs the diastolic filling time and results consequently in an increased left ventricle end-diastolic volume, thereby lowering blood pressure and heart rate. Standard dosage Titrate dosage for symptom control, establish a target heart rate of 50–60 beats/minute and systolic blood pressure down to 100–120 mm Hg. Contraindications Sick sinus syndrome, 2nd and 3rd degree atrioventricular block (AV block), prolonged first degree AV block without pacemaker in place (PR interval 9 0.24 sec), cardiogenic shock, sinus bradycardia, hypotension (systolic blood pressure of less than 100 mm Hg), severe bronchospastic disease, congestive heart failure. Main drug interactions Combined use of a β-blocker with verapamil or diltiazem and specific an- tiarrhythmic medications (e.g., amiodarone) increases the risk of bradycardia and AV block. Main side effects Bradycardia, possible wheezing in susceptible patients. Rarely hyperkalemia in elderly patients with diabetes or in combination with an angiotensin- converting enzyme inhibitor (ACE-inhibitor). Special points - Cost effectiveness Adequate β-blockade can usually be achieved imntavenous with metoprolol or propanolol which are relatively inexpensive medicine, while esmolol is expensive and reserved for the very initial treatment. Sodium nitroprusside Nitroprusside is administered in the early setting of AAD patients with refractory hypertension after initiation of β -blocker therapy. It induces venous and arterial vasodilatation, thereby increasing the cardiac output. Vasodilator therapy may be associated with reflex tachycardia that increases the aortic wall stress, which potentially can cause expansion and propagation of thoracic aortic dissection. For this reason, vasodilator therapy should only be administered after adequate heart rate control. Standard dosage Titrate dosage for symptom control, establish a target systolic blood pressure down to 100–120 mm Hg. Contraindications Significant hypotension, inadequate cerebral circulation, optic atrophy, de- creased systemic vascular resistance (SVR), concomitant unstable angina or recent myocardial infarction, renal or hepatic failure. Main drug interactions Generally, nitroprusside will synergistically decrease the blood pressure if used with other vasodilators, especially with PDE5-1 (e.g., sildenafil). Main side effects Hypotension, metabolic acidosis, brady- and tachyarrhythmia’s, cyanide toxicity, myocardial ischemia. With impaired renal function and infusion of Vascular Disease (H Gornik and E Kim, Section Editors)
  • 5. the agent for more than 48 hours, the risk of thiocyanate toxicity is increased. Reduction of arterial oxygen saturation in some patients with hypoxic pul- monary vasoconstriction. Special points Do not use as a sole agent in patients with aortic dissection because the vasodilating effect can cause reflex tachycardia and an increased force of left ventricular contraction. Avoid the use in patients with renal failure. Further, infusion periods should be as short as possible because of the possible thiocyanate and cyanide toxicity. Nitroprusside is light sensitive, for this reason infusion bags should be protected from light. Cost effectiveness Sodium nitroprusside in combination with an intravenous β-blocker con- trols the blood pressure effectively in the majority of patients with AAD. Sodium nitroprusside is inexpensive in comparison to other intravenous agents used for rapid control of hypertension. Non-dihydropyridine calcium channel blockers (Ca-blockers) In patients not responding, or poorly tolerating β-blockers, Ca-blockers can be used as an alternative. In addition, usage of Ca-blockers in TBAD might reduce aortic expansion over time [10, 11]. Standard dosage Titrate dosage for symptom control, establish a target heart rate of 50–60 beats/minute and systolic blood pressure down to 100–120 mm Hg. Contraindications Acute myocardial infarction, 2nd or 3rd degree AV block, sino-atrial node dysfunction, hypotension, sick sinus syndrome, Wolff-Parkinson-White syndrome, congestive heart failure, wide QRS tachycardia of uncertain origin, concomitant intravenous use of a β-blocker. Main drug interactions β-blockers, digoxin and cyclosporine. Main side effects Myocardial infarction, dyspnea, ileus, flushing. Special points - Cost effectiveness Inexpensive and effective. Angiotensine-converting enzyme inhibitors (ACE- inhibitors) In absence of adequate blood pressure control, ACE-inhibitors should be administered to reduce the blood pressure and to maintain adequate end-organ perfusion. Intravenous administration of an ACE-inhibitor is rarely needed for the treatment of AAD. Standard dosage Titrate dosage to establish a target systolic blood pressure down to 100– 120 mm Hg. Contraindications Angioedema on previous therapy, pregnancy. Caution in patients with renal insufficiency, bilateral renal artery stenosis or elevated potassium levels, as these agents increase the risk of renal failure. Main drug interactions Severe hyperkalemia can occur, if the agent is used in combination with potassium-sparing diuretics or potassium supplements. First-dose hypoten- sion can occur, if the agent is used in combination with α-blocking agents, such as doxazosin, prazosin or terazosin. The antihypertensive effects of an ACE-inhibitor are augmented by other antihypertensive agents. Main side effects Impairment of renal function, hyperkalemia, potential for hypotension, angioedema, dry cough. Special points ACE-inhibitors can be useful in case of unirenal malperfusion with con- comitant increasing renin levels. Update in the Management of Aortic Dissection Tolenaar et al.
  • 6. Cost effectiveness Generic agents are very inexpensive. Angiotensin receptor blockers (ARBs) ARBs can be used as an alternative for ACE-inhibitors, when patients have persistent cough after administration of ACE-inhibitors. In ad- dition, there is increasing evidence that patients with Marfan syn- drome may benefit from ARBs instead of ACE-inhibitors, as losartan looks to be associated with a reduced ascending aortic growth rate [12]. Standard dosage Titrate dosage to establish a target systolic blood pressure down to 100– 120 mm Hg. Contraindications Caution in patients with renal insufficiency, bilateral renal artery stenosis or elevated potassium levels, as these agents increase the risk of renal failure. Main drug interactions Severe hyperkalemia can occur, if the agent is used in combination with potassium-sparing diuretics or potassium supplements. The antihypertensive effects of ARBs are augmented by other antihypertensive agents. Main side effects Potential for hypotension, impairment of renal function, hyperkalemia. The incidence of cough is lower with ARBs (1 %) compared with ACE- inhibitors (10 %). The incidence of angioedema with ARBs (very rare) is lower in comparison with ACE-inhibitors. ARBs can be used as an alternative in patients who suffered from angioedema due to ACE- inhibitors. Special points The administration of ARBs should be initiated during hospitalization. Cost effectiveness Generic agents are inexpensive. Alpha adrenergic blocking agents (α-blockers) If the blood pressure is still uncontrolled despite using a combination of the earlier mentioned antihypertensive agents, addition of α-blockers can be a valuable option. It lowers the peripheral vascular resistance by di- lation of the arterioles and venules. Standard dosage Titrate upward to achieve a systolic blood pressure down to 100–120 mm Hg. Contraindications (Orthostatic) hypotension, heart failure, myocardial infarction. Main drug interactions Simultaneous use with other antihypertensive agents can have a synergistic effect. Combination of use with PDE-5 inhibitors, such as sildenafil, can cause hypotension. Main side effects (Orthostatic) hypotension, respiratory- or urinary tract infection, dizziness, palpitations, tachycardia, dyspnea. Cost effectiveness It is a valuable therapeutic adjunct and inexpensive. Hydralazine Hydralazine lowers blood pressure by exerting a peripheral vasodi- lating effect through a direct relaxation of vascular smooth muscle. However, it is associated with reflex tachycardia and increase in the left ventricular contractility, plasma renin levels and the release of norepinephrine. For these reasons, this arteriolar dilator is considered to be relatively contraindicated for patients with suspected acute Vascular Disease (H Gornik and E Kim, Section Editors)
  • 7. AAD, except when the heart rate is well controlled and hypertension persists. Interventional procedures Transcatheter fenestration Standard procedure Through the groin, a guidewire is utilized to puncture the septum flap which is then dilated with a balloon or catheter, resulting in the restoration of the equilibrium of pressures between the false and true lumen. Complementary aortic branch stenting can be used to treat malperfusion [13, 14]. Contraindications Malperfusion due to a static obstruction by thrombus in the false lumen Complications The transcatheter fenestration may fail, not resolving malperfusion and the pressurization of the false lumen, which also may lead to an increased risk of aortic expansion and rupture [13, 14]. Special points Requires great expertise and strict blood tests and imaging follow-up in the acute setting. This procedure can be used not only in TBAD patients, but also in TAAD to relieve malperfusion before undergoing definitive surgical management. Cost/cost-effectiveness Because the procedure is entirely endovascular and usually does not require stent grafts, it is relatively inexpensive compared to TEVAR or open surgery. TEVAR (thoracic endovascular aortic repair) Standard procedure In patients with complicated TBAD, TEVAR has become the preferred ther- apeutic option [15, 16]. Femoral artery cut down or a percutaneous approach is used to introduce the guidewires and catheters. After aortography and with TEE control, a covered stent graft is deployed under fluoroscopic guidance, covering the proximal entry tear. In this fashion blood flow is diverted into the true lumen, depressurizing the false lumen and promoting false lumen thrombosis and aortic remodeling. Post deployment angiography is obtained to evaluate the correct position of the graft and resolution of the aortic complications. In case of poor results, like persistence of malperfusion, another covered stent graft can be deployed up to the level of the celiac trunk. If necessary, an uncovered aortic stent might be extended more distally to the abdominal aorta, at the level of the visceral and renal arteries. Contraindications Unfavorable anatomy prohibiting stent graft delivery. Such as extreme tor- tuosity, severe atherosclerotic disease or the necessity of coverage a major branch vessel. Retrograde extension of the dissection into the aortic arch should be considered a contra-indication for standard TEVAR. Complications TEVAR is associated with an in-hospital mortality between 2.6-9.8 % and neurologic complications between 0.6–3.1 % [17, 18]. Stroke, paraplegia and paraparesis, incidental occlusion of the left subclavian artery and inad- equate placement of the graft are all significant complications of TEVAR [19]. The risk of paraplegia is related to the extension of the aorta that is covered, previous aortic surgery and hypotension. Stroke is often due the presence of severe arch atherosclerosis and mural thrombosis, which can serve as source of embolus, especially due to the manipulation of catheters in the arch and ascending aorta [19]. Coverage of the left subclavian artery (LSA) may be associated with a higher incidence of stroke, paraplegia and paraparesis. Revascularization of the LSA, typically with left subclavian to common ca- Update in the Management of Aortic Dissection Tolenaar et al.
  • 8. rotid artery transposition or bypass grafting, should be performed in all non- emergent cases [16, 19, 20] . Additionally, the false lumen may remain patent, which seems to be associated with chronic aortic dilatation. Other complications are related to the device or procedure, including endoleaks, migration or collapse of the stent graft and development of a false aneurysm [17, 21]. Special points Patients affected by TBAD with refractory or recurrent pain or refractory hypertension are at increased risk of relevant in-hospital mortality and may benefit from early TEVAR [22]. Serial CT and MRI studies are required due to the increased risk of stent graft or aortic related complications. Cost/cost-effectiveness TEVAR has shown to be cost-effective compared to open surgery for com- plicated TBAD in the short term [23]. Multiple prospective studies are cur- rently running to determine the long term durability and cost-effectiveness of TEVAR. Surgery Type a dissection Ascending aorta replacement Standard procedure All patients are treated under general anesthesia through a medium ster- notomy, using extracorpeal circulation with moderate or deep hypothermic circulatory arrest. The pericardium is excised, cardiopulmonary bypass is established and cardiac arrest is instituted. During patient cooling, the aorta may be cross-clamped in order to inspect the aortic root and valve. If the aortic vale and root are free of concomitant disease, the proximal aortic graft anastomosis is performed at supracommisural level with possible aortic re- inforcement using Teflon strips [24]. In TAAD patients, aortic regurgitation is present in up to 44 %, frequently resulting from commissural dehiscence, annular dilatation, sclerotic, stenosed or destructed aortic valves [4]. These patients may require a Bentall procedure with composite aortic valve-graft replacement. However, in some patients the function of the aortic valve is preserved and they may be treated with valve-sparing procedures such as the David or Yacoub technique [25, 26]. Such operations should be primarily reserved for patients affected by connective tissue disorders, young patients, and those with ectatic aortic root. Nevertheless, valve-sparing operations are technically demanding and are indicated only in high-volume centers with expertise. Aortic arch management requires moderate/deep hypothermic circulatory arrest. For better cerebral protection, antegrade cerebral perfusion maybe established through catheters that are selectively advanced into the innominate artery and left common carotid artery. The inspection of the arch is conducted to identify additional entry-tears. In the presence of an entry tear in the inner curvature or absence of additional arch complications, a hemi-arch replacement is performed, with the distal anastomosis sutured in a transverse manner in order to resect the inner curvature of the arch and sparing the supra-aortic branches. In case of extensive tears in the aortic arch, which is present in 15–20 % of all TAAD patients, as well in presence of arch aneurysm, complex arch dissection, connective tissue disease or atheroma- teous disease, total arch replacement should be considered. Supra-aortic vessels may be reimplanted separately or with the use of the island technique [27, 28]. Total arch repair is also advocated in TAAD patients to reduce long term aneurysmatic complications of the descending aorta, being associated Vascular Disease (H Gornik and E Kim, Section Editors)
  • 9. with increased false lumen thrombosis and aortic remodeling [29, 30]. Re- duced patency of the false lumen can be achieved with the use of extensive aortic procedures, such as the elephant trunk technique [31], which require a staged operation for treating thoracic and thoraco-abdominal aortic seg- ments. Recently TEVAR has been introduced as a minimally invasive option to treat the descending aorta during the staged operation or with simulta- neous descending stent-grafting as a hybrid procedure. Contraindications Dense stroke and coma are considered contra-indications, although recent studies report a relatively good outcome [32]. Patients presenting with severe malperfusion have a relative contra-indication [33]. Surgery is also not in- dicated in patients with end stage malignant disease or other major comor- bid illness associated with decreased life expectancy. Complications Although technical advances have helped to improve surgical outcomes, mortality rates still range between 10–25 % [9, 34]. Coagulopathic hemor- rhage remains a significant cause of mortality and morbidity and reexplo- ration may be required. Extensive blood transfusion can be also associated with post-operative pulmonary failure. Stroke still remains an important complication of this surgery, ranging around 4–8 % [9, 34]. Other postop- erative neuropsychiatric complications like delirium or changes in memory and cognition may be present in one-third of the patients. The incidence of these complications seems to be related to brain ischemia time. Antegrade brain perfusion has been reported to be beneficial to prevent these compli- cations [35]. Extracorporeal circulation, renal failure, end-organ ischemia and hypothermia can result in significant electrolyte and/or acid–base dis- turbance and may necessitate volume reduction with conventional diuretic therapy or extracorporeal dialysis. Special points Currently, cannulation of the axillary artery or right subclavian artery has shown to be superior compared to femoral artery cannulation, as it is seldom involved in the dissection or in extensive atherosclerotic lesions [36]. A sig- nificant advantage of this approach is that it allows both antegrade brain perfusion during circulatory arrest, which reduces cerebral ischemic events, as well as antegrade distal perfusion. The use of a conduit in case of small, deeply located, or damaged axillary arteries has been advocated. The optimal temperature during hypothermic circulatory arrest remains debated, as the advantages of deep hypothermic technique (e.g., brain protection) may be outweighed by the complications (prolonged extracorporeal circulation time, coagulation disorders and increased systemic inflammatory response syndrome). A recent study showed no influence on outcome of cerebral perfusion temperatures, ranging between 15–30 °C [9]. Cost/cost-effectiveness Surgical procedures for TAAD must be instituted as soon as possible as most patients will expire after admission. Type B dissection Standard procedure Due to advancement in endovascular techniques, the treatment paradigm for TBAD patients has shifted, with open surgery reserved for patients in whom endovascular techniques are not feasible or have failed. During surgery, ex- posure of the descending aorta is obtained through a left posterolateral thoracotomy. Cardiopulmonary bypass can be established in a partial manner through the left atrium and femoral artery. In patients with retro- grade extension with partial arch involvement, full cardiopulmonary bypass, using the femoral artery and vein, may be instituted. In the latter setting, hypothermic circulatory arrest may be performed, with or without selective Update in the Management of Aortic Dissection Tolenaar et al.
  • 10. antegrade perfusion, for treating left hemi-arch using an open proximal aortic anastomosis. After resection of the proximal entry tear, distal aortic graft anastomosis is performed at the level of the diaphragm, with rein- forcement of the aortic wall with Teflon-strips. At this level the aortic lamella can be fixed to the dissected aortic wall or resected in order to create a single aortic lumen which will balance the blood pressure between the true and false lumen. After this, the aortic graft is sutured distally. In case of visceral/ renal malperfusion, a surgical fenestration is needed in order to restore blood flow to the compromised branches. Extensive thoraco-abdominal aortic re- placement is associated with higher risk of paraplegia, therefore, in patients undergoing this surgery reimplantation of the intercostal arteries between T8 and L1is recommended. For those patients who present with lower extremity ischemia, surgical management may include infrarenal aortic fenestration through a median laparatomy. Older patients affected by limb ischemia may benefit from a femoral-femoral arterial bypass graft. Contraindications Patients presenting with severe visceral malperfusion and spinal cord ische- mia may not have an indication for surgery given a low likelihood of clinical recovery and grim overall prognosis. End stage malignant disease and severe COPD are considered relative contra-indications for surgical aortic repair. Complications In patients with TBAD, overall surgical mortality is around 30 % [34]. The outcome of surgical repair may depend on the preoperative condition of the patient, as risk factors like older age and hypotension/shock are strongly associated with a poor outcome. A more beneficial outcome may be expected in patients with radiating pain, normotension at surgery, and reduced hy- pothermic circulatory arrest time. Neurologic complications, such as spinal cord ischemia, paraparesis and stroke, are associated with the extent and duration of the operation [16, 34, 37]. Other notable complications are cardiac, respiratory and renal failure, visceral ischemia or peritonitis, sepsis, and limb ischemia [16, 34, 37]. Special points For inpatients at higher risk for spinal cord ischemia, cerebrospinal fluid drainage and perfusion pressure optimization are recommended [6]. Al- though not routinely used, neurophysiologic monitoring of the spinal cord can detect spinal cord ischemia, guiding hemodynamic optimization and the potential reimplanation of intercostals arteries [38]. Cost/cost-effectiveness Surgery is only indicated in those TBAD patients that cannot be treated with an endovascular approach. Emerging therapies TEVAR in uncomplicated TBAD Standard procedure Among uncomplicated TBAD, 90 % survive hospital admission after adequate antihypertensive therapy [5]. Conservative treatment in com- bination with close surveillance is justified in order to detect aneurys- matic dilatation or progression of the dissection, which is the most feared complication in the long term, involving about 30–40 % of patients [2, 39]. Based on these findings, it has been proposed that uncomplicated TBAD might be treated with stent graft placement. Al- though the INSTEAD (Investigation of STEnt Grafts in Patients with Type B Aortic Dissection) trial did not show beneficial results for uncompli- cated TBAD treated with TEVAR versus those managed medically in terms of mortality [39] the one-year results of the ADSORB (A European Vascular Disease (H Gornik and E Kim, Section Editors)
  • 11. Study on Medical Management Versus TAG Device + Medical Manage- ment for Acute Uncomplicated Type B Dissection) trial showed more frequent false lumen thrombosis and aortic remodeling in those patients with TBAD treated with TEVAR compared to those managed medically [40]. Contraindications Comparable with TEVAR. Complications Comparable with TEVAR. Special points In order to stratify this cohort of patients, clinical, genetic and radiologic predictors could identify those patients with TBAD that might benefit from early intervention. Cost/cost-effectiveness Currently TEVAR in uncomplicated TBAD has failed to show superior out- come and is much more expensive than medical treatment alone. In the absence of long-term outcome data, as well as the potential complications related to TEVAR, medical treatment is preferred. Hybrid procedures Standard procedure As previously reported, TEVAR can be used in association with open ascending/arch repair for treating extensive TAAD patients, simulta- neously or in a staged procedure. In chronic arch and descending post- dissecting aortic aneurysms, different hybrid approaches can be adopted, consisting of aortic arch debranching, stented elephant trunk and frozen elephant trunk [41, 42]. During debranching procedures, the supra-aortic vessels are bypassed using surgical grafts from the ascending aorta and the operation is finalized by placement of a stent graft into the arch, in the same or later stage. TEVAR has been also adopted to complete the second stage of the elephant trunk technique for treating descending aortic diseases. Recently, hybrid “frozen elephant trunk” prostheses, which are represented by a surgical graft with an incorporated stent graft, have been introduced to manage extensive aortic diseases simultaneously [31]. Contraindications In patients with previous coronary artery bypass grafting, where surgical grafts for the aortic debranching needed to be sutured in the ascending aorta, as well in those with unfavorable anatomy prohibiting safe stent graft delivery. Complications Hybrid procedures for aortic arch dissections carry a considerable risk with a pooled mortality of 9.8 %, perioperative stroke of 4.3 % and spinal cord ischemia of 5.8 % [41]. Special points These procedures are not routinely performed, but are used for patients with multiple comorbidities, considered unsuitable for open repair in experi- enced/high volume centers. Cost/cost-effectiveness Since initial results are comparable with open surgical repair among high risk patients with multiple comorbidities, this approach seems justified although long-term follow-up data should be awaited. Total endovascular treatment of TAAD Total endovascular treatment of TAAD is currently in an experimental phase and only few case-reports have been reported [43, 44]. Stent graft placement in the TAAD might be feasible in selected cases, as the landing zone needs to be evaluated very accurately, being in the vicinity of the supra-aortic branches and the coronary arteries. Update in the Management of Aortic Dissection Tolenaar et al.
  • 12. Developments of modular branched or fenestrated stent grafts, pos- sibly with incorporated composite aortic valves, potentially harbor the future in the treatment of TAAD patients. Disclosure Dr. Eagle is the Principal Investigator of IRAD which is supported by grants from the following organizations: Gore, Mardigian Foundation, Hewlett Foundation, and Thomas Varbedian Fund for Aortic Research. He is also a consultant to the National Institutes of Health Heart, Lund and Blood Institute where he is Study Chair of its GenTAC (Genetically Triggered Aortic Conditions) Registry. Dr. Trimarchi has been a speaker for Gore and Medtronic. Dr. Jip L. Tolenaar and Dr. Guido H.W. van Bogerijen reported no conflicts of interest relevant to this article. References and Recommended Reading Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance 1.• Olsson C, Thelin S, Stahle E, Ekbom A, Granath F. Thoracic aortic aneurysm and dissection: in- creasing prevalence and improved outcomes reported in a nationwide population-based study of more than 14,000 cases from 1987 to 2002. Circulation. 2006;114(24):2611–8. This paper reports an increased incidence of AAD compared to previous reports, from 2–5 to 14 per 100.000 people/year. 2. Tsai TT, Trimarchi S, Nienaber CA. Acute aortic dis- section: perspectives from the International Registry of Acute Aortic Dissection (IRAD). Eur J Vasc Endo- vasc Surg. 2009;37(2):149–59. 3. Coady MA, Davies RR, Roberts M, et al. Familial patterns of thoracic aortic aneurysms. Arch Surg. 1999;134(4):361–7. 4. Hagan PG, Nienaber CA, Isselbacher EM, et al. The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. JAMA. 2000;283(7):897–903. 5. Suzuki T, Mehta RH, Ince H, et al. Clinical profiles and outcomes of acute type B aortic dissection in the current era: lessons from the International Registry of Aortic Dissection (IRAD). Circulation. 2003;108 Suppl 1:II312–7. 6.•• Hiratzka LF, Bakris GL, Beckman JA, et al. ACCF/ AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/ American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular An- giography and Interventions, Society of Interven- tional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation. 2010;121(13):e266–369. For AAD patients, these recent guidelines for thoracic aortic disease report risk factors, presentation, diagnosis, indication to treatment and preferred management. 7. von Kodolitsch Y, Schwartz AG, Nienaber CA. Clini- cal prediction of acute aortic dissection. Arch Intern Med. 2000;160(19):2977–82. 8. Okita Y, Takamoto S, Ando M, Morota T, Kawashima Y. Surgical strategies in managing organ malperfu- sion as a complication of aortic dissection. Eur J Cardiothorac Surg. 1995;9(5):242–6. 9. Rylski B, Suedkamp M, Beyersdorf F, et al. Outcome after surgery for acute aortic dissection type A in patients over 70 years: data analysis from the Ger- man Registry for Acute Aortic Dissection Type A (GERAADA). Eur J Cardiothorac Surg. 2011;40 (2):435–40. 10. Suzuki T, Isselbacher EM, Nienaber CA, et al. Type- selective benefits of medications in treatment of acute aortic dissection (from the International Reg- istry of Acute Aortic Dissection [IRAD]). Am J Car- diol. 2012;109(1):122–7. 11. Jonker FH, Trimarchi S, Rampoldi V, et al. Aortic expansion after acute type B aortic dissection. Ann Thorac Surg. 2012;94(4):1223–9. Vascular Disease (H Gornik and E Kim, Section Editors)
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