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E. Iannacone et al.
occurs from the dissecting ap or false lumen obstructing the ostia of the great vessels (Fig.4) [28].
Some consider cerebral malperfusion itself to be a contraindication to immediate
repair. The prominent concerns with immediate central repair, most often done with
concurrent hypothermic circulatory arrest and with full anticoagulation, include the
risk of hemorrhagic conversion and reperfusion injury worsening neurologic outcome. Patients with ATAAD and cerebral malperfusion are also more likely to present with other characteristics predictive of poor outcomes, including hypotension,
shock, tamponade, renal failure, myocardial ischemia, and limb ischemia. The risk
of delaying surgery for stabilization of the neurologic condition, however, includes
rupture and death. According to recent IRAD data, surgery is signicantly less
likely to be performed in patients with coma (66.7%) or stroke (75.9%) than those
without a brain injury (88.9%). However, patients with ATAAD and brain injury
perform miserably when managed medically, with a 100% mortality if presenting
with coma. Only 12.8% of those with CVA managed medically survive to discharge.
Although CVA and coma predict at least a two- or threefold higher mortality,
patients who receive surgical treatment have a 75% survival to discharge [29].
There are some reports of novel techniques for early cerebral reperfusion including direct carotid perfusion, endovascular stenting, or direct surgical fenestration,
followed by central repair [30–32]. Our standard approach is immediate central
repair of the dissection. Central cannulation of the true lumen is our preferred arterial cannulation strategy. We utilize a Seldinger technique and the guidance of both
epiaortic ultrasound and transesophageal echocardiography to ensure true lumen
perfusion [33]. Near-infrared spectroscopy (NIRS) conrms symmetric great vessel
ow, thus eliminating the need to manipulate the great vessels. In the small number
of patients in which we are unable to access the true lumen centrally, we utilize the
femoral artery for arterial inow and only use axillary artery cannulation as a last
resort. In our experience, the depth of the axillary artery, fragility of the vessel, and
the need to place a perfusion graft onto the artery a majority of the time leads to
additional cerebral ischemic time that can be avoided.
Fig. 4 Acute type A aortic
dissection with innominate
artery dissection and
occlusion of right carotid
artery (arrow)

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Intraoperative cerebral monitoring can include any combination of electroencephalogram, NIRS, and transcranial Doppler ultrasound. We routinely use NIRS
(Somanetics INVOS Cerebral/Somatic Oximeter, Covidien, IL, USA) to evaluate
cerebral perfusion, particularly during the initiation of cardiopulmonary bypass or
with aortic cross clamping. A sharp decrease in the cerebral oxygenation suggests
the need for alternative arterial cannulation or removal of the cross clamp for the
remainder of cooling. Although the optimal cerebral protection strategy during arch
surgery is often debated, a recent network meta-analysis of 26,968 patients, in
which dissections were included, compared deep hypothermic circulatory arrest
with antegrade (ACP) and retrograde cerebral perfusion (RCP). The authors found
no difference between ACP and RCP for stroke or operative mortality [34]. We
prefer RCP and deep hypothermic circulatory arrest (DHCA) with a systemic temperature of 20°C or less. In the setting of cerebral perfusion we prefer a conservative strategy of hemiarch reconstruction to limit cerebral ischemic time and quickly
reestablish antegrade great vessel ow.
Patient selection has been crucial to improving surgical outcomes over the last
two decades. Although quality of life data is not readily available for patients surviving immediate surgical management of ATAAD with cerebral malperfusion, several studies have reported favorable outcomes [28, 29, 32, 35]. Complete resolution
of neurologic decits have been reported in up to 84% of patients presenting with
focal decits. Patients with more devastating neurologic injury are signicantly less
likely to achieve neurologic improvement. Neither cerebral protection method nor
extent of aortic arch repair appear to be predictive of neurologic improvement. Early
intervention, however, particularly within 10h of presentation of stroke, is integral
to achieving neurologic recovery [28, 35]. This data should encourage surgeons to
offer emergent surgery to selected patients, particularly those with focal decits,
despite their higher risk prole.
357
Spinal Malperfusion
Spinal malperfusion complicating ATAAD is rare, occurring in less than 5% of
patients [4, 18]. It manifests as paraparesis or paraplegia, may present unilaterally,
and may be accompanied by urinary or bowel incontinence. Immediate central aortic repair is the mainstay of treatment, with complete resolution of spinal cord injury
occurring in 61% [18]. The presence of preoperative spinal malperfusion is associated with increased risk of postoperative complications, and signicantly increased
risk of mortality [4]. Resolution of spinal ischemia, however, is protective against
the increased risk of early mortality seen by those who do not experience neurologic
recovery [18]. The presence of preoperative spinal malperfusion should not deter
surgeons from offering a potentially life-saving procedure. Although nearly 40% of
patients do not experience complete resolution of their symptoms, there may be an
opportunity for those with partial recovery to experience additional return of lower
extremity function with extensive rehabilitation.

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E. Iannacone et al.
Mesenteric Malperfusion
Mesenteric malperfusion is fortunately a rare complication of ATAAD, occurring in
4–6% of patients [4, 36]. It can be insidious in presentation and frequently presents
with malperfusion of other vascular territories. Patients with mesenteric MPS may
present with abdominal pain, melena, metabolic acidosis, or elevated liver enzymes.
The etiology may be dynamic obstruction, occlusive, or thromboembolic. The presence of mesenteric MPS is highly lethal, with nearly two-thirds of patients dying
during hospitalization, a threefold increase over those without the complication
[4, 36].
Management strategies for mesenteric malperfusion are perhaps the most
strongly debated. The traditional approach of immediate aortic repair remains the
most commonly utilized. Despite this, nearly one third of patients diagnosed with
mesenteric ischemia are treated “medically” according to recent IRAD data. This
likely reects surgeons’ acknowledgement that, even with repair, mesenteric malperfusion is one of the most threatening dissection-related complications. Without
intervention, however, less than 5% survive [36].
Over recent decades, some groups have dedicated their efforts to a peripheral
revascularization rst strategy in order to resolve the MPS before moving on to
primary aortic repair [37–41]. This approach relies heavily on early identication of
patients who are at great risk of death from end-organ failure, and the availability of
proceduralists and facilities skilled at performing complex interventional procedures. The theoretical benet to this approach is that resolving the MPS will reduce
systemic inammation and metabolic derangements that otherwise would increase
the risk of central repair [16]. It may also prevent a futile attempt at open aortic
repair for the already unsalvageable patient who succumbs to organ failure despite
reperfusion of the affected vascular bed. Avoiding preventable aortic rupture while
awaiting resolution of MPS is the biggest challenge of the staged approach. Yang
and colleagues were able to eliminate fatal aortic rupture with their modied algorithm (Fig.5), noting that they enforced strict hemodynamic management during
the endovascular phase and waited only for downtrending rather than normalization
of ischemic markers before central repair. Despite this, total mortality for patients
with mesenteric MPS remained high, 33.3–40.3% [38]. Those presenting with
stroke (odds ratio [OR] 23), lactate >6mmol/L (OR 13.5), or with bowel necrosis at
laparotomy (OR 7) are the most difcult to salvage [37].
In many practices, including ours, the most expeditious means to restore endorgan function is rapid transfer to the operating room. We most often establish antegrade ow into the true lumen early by central cannulation, followed by rapid
conservative aortic repair. The risk of rupture or fatal tamponade complicating
delayed central repair is eliminated, and metabolic derangements can be corrected
while on bypass. Persistently elevated lactates in the operating room after central
aortic repair or high risk preoperative prole may warrant immediate laparotomy
after central repair. Ongoing postoperative clinical or biochemical evidence of

No
Management of Complicated Type A Aortic Dissection: The Cornell-New York…
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Acute type A aortic dissection
359
Hemodynamic instability
(aortic rupture, tamponade)?
No
Visceral or extremity
malperfusion syndrome (MPS)?
Ye s
Arterial obstruction?
Ye s
Endovascular treament
(fenestration/stenting)
Optimal medical support in ICU
Hemodynamic instability
(aortic rupture, tamponade)?
No
Resolution of organ failure?
Ye s
No
No
Open aortic repair
Ye s
Ye s
Fig. 5 Michigan algorithm for acute type A aortic dissection and mesenteric or extremity malperfusion syndrome (MPS). ICU indicates intensive care unit [38]
persistent bowel ischemia after central repair should prompt mesenteric angiography with interventional fenestration, angioplasty, or stenting, accompanied by
abdominal exploration and resection of any ischemic bowel. Patients presenting
with the particularly moribund risk factors of concomitant stroke or severely elevated lactate may be considered for a staged approach.
Mortality rates for patients with ATAAD complicated by mesenteric MPS are
dismal with medical and endovascular therapies alone [36]. Disappointingly, however, when comparing the endovascular rst to central repair rst approaches, the
overall mortality for patients with mesenteric MPS complicating ATAAD is still
alarmingly high [42]. Ongoing efforts supporting more prompt detection and restoration of mesenteric blood ow is paramount to improving outcomes for this
extremely high-risk cohort.

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E. Iannacone et al.
Peripheral Malperfusion
Peripheral malperfusion complicates ATTAD in 10–13% of patients in larger registries, and often accompanies malperfusion of other vascular beds [1, 3, 4, 17, 43].
Limb ischemia manifests early as a cool, pulseless extremity with mottled skin, later
as sensory and motor decits and, in its most advanced stage, as profound paralysis
of the limb [44]. Signicant preoperative elevations in creatine kinase may signal
potentially irretrievable tissue damage [45]. Sequelae of limb reperfusion are not
benign, and include shock, acidosis, rhabdomyolysis, and renal failure. For those
with advanced limb ischemia, the need to amputate may remain despite reperfusion.
Aggressive pursuit of fasciotomies after reperfusion are prudent to relieve or avoid
development of compartment syndrome and to assess the viability of the muscle.
Hemodynamically stable ATAAD with isolated and advanced limb malperfusion
as the presenting feature may benet from prioritizing limb reperfusion with a brief
period of recovery before central aortic repair [38]. For ATAAD patients with multiple vascular beds affected by malperfusion, and for those with isolated early
peripheral MPS, our preference is for immediate central aortic repair. Several studies report favorable results with immediate proximal aortic repair alone relieving
lower limb ischemia in 60–100% of patients [5, 46–48]. After central repair, intraoperative recognition of ongoing limb ischemia and expeditious revascularization
produces excellent outcomes comparable to those of ATAAD patients without malperfusion syndromes [43].
Conclusions
The ideal approach to the patient with ATAAD and malperfusion includes rapid
diagnosis and reperfusion of the ischemic vascular beds while minimizing the risk
of aortic rupture. In cases where there is radiographic and clinical evidence of multiorgan malperfusion, or with ongoing hemodynamic instability, an aortic repair rst
strategy optimizes the timing of true lumen reperfusion throughout the aorta and
eliminates the risk of rupture and inuence of pericardial tamponade. However,
when advanced single organ malperfusion syndromes are present (excluding coronary malperfusion), there may be opportunities to avoid the additional metabolic
and inammatory insult of open surgery by utilizing a percutaneous revascularization rst approach. The operative mortality with all approaches remains disappointingly high but a gratifying rate of salvage can be anticipated when patients are
triaged quickly to centers and surgeons with extensive experience caring for a wide
variety of aortic pathology.
Conicts No conicts of interest to report.
Funding No outside funding received.

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363

Management ofComplicated Acute
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Type A Aortic Dissection: The Stanford
Approach
AlbertJ.Pedroza andMichaelP.Fischbein
Introduction
Acute aortic dissection is a rare, life-threatening condition with an incidence ranging from 5 to 10/100,000 person-years [1]. Cardiac surgeons have long recognized
that this disease process is clinically challenging with a high mortality rate. Among
the simplest and earliest classication systems, the Stanford paradigm proposed in
1970 by Dailey etal. established surgical repair as the standard of care for the ‘Type
A’ variant involving the ascending aorta (Fig.1) [2]. While the wealth of experience
treating this entity over the subsequent ve decades has reafrmed the need for
prompt surgical intervention, one central theme remains certain: not all aortic dissections are created equal. Within the cohort of patients referred for prompt surgical
repair of acute type A aortic dissection (ATAAD), many potential complicating factors contribute to operative candidacy, optimal interventional strategy and morbidity/mortality risk. In particular, the presence of neurologic injury, mesenteric
malperfusion, limb ischemia or shock mandate rapid decisive action. When present,
these factors comprise a heterogeneous “complicated ATAAD” variant with heightened technical challenges and surgical risk. Whereas the debate around neurologic
status reects a question of ‘if’ an operation should be attempted, the presence of
malperfusion or limb ischemia raises important considerations of ‘how’ it should be
performed. Various institutional paradigms have been built around theories on optimal management, reecting the lack of clear consensus across the specialty about
how to optimally manage these difcult problems. The growing body of literature
surrounding complicated aortic dissection management underscores the need for
centralized cardiac surgery referral centers capable of interdisciplinary aortic interventions and the rapidly evolving practice of the modern aortic surgeon. This chapter presents pertinent lessons learned from institutional experience and multi-center
A. J. Pedroza · M. P. Fischbein (*)
Cardiothoracic Surgery, Stanford University, Stanford, CA, USA
e-mail: alpedroz@stanford.edu; mschbe@stanford.edu
J. S. Coselli et al. (eds.), Aortic Dissection and Acute Aortic Syndromes,
https://doi.org/10.1007/978-3-030-66668-2_26
365© Springer Nature Switzerland AG 2021

366
Stanford Classification
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Fig. 1 Stanford
classication for acute
aortic dissection. In the
“Type A” variant, the
primary intimal tear occurs
in the ascending aortic
segment, while in “Type
B”, the tear occurs distal to
the aortic arch, affecting
the descending thoracic
aorta
Type A Type B
A. J. Pedroza and M. P. Fischbein
databases to highlight branch points in the treatment algorithm for complicated ATAAD.
Pre-Operative Evaluation
Operative Candidacy
Decisions on operative candidacy for ATAAD in general are made difcult by the
very poor outcomes of medical management alone. While modern mortality estimates for medical management alone are implicitly limited by selection and reporting biases, data from the International Registry of Acute Aortic Dissection (IRAD)
database showed 57% mortality for patients treated medically. Surgical outcomes
have steadily improved since the inception of the IRAD database with reported
multi-center surgical mortality rates falling from 25% to 18% between 1995 and
2013 [3]. Findings from the IRAD database also highlight age-dependent increases
in mortality risk regardless of treatment modality but consistent superiority of surgical treatment up to 80years of age [4]. The paucity of data for patients over 80
within this cohort precludes robust determination of optimal management for octogenarians. Given these dichotomous outcomes, every ATAAD patient should be
considered for operative repair. With few exceptions, our default pathway is immediate transfer directly to a hybrid operating room and preparation for central aortic
repair. The presence of distal malperfusion, which may affect one or multiple organ
beds, represents a central branch point in treatment algorithm for patients presenting with ATAAD.
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