Distal Aortic Dissection Type Stanford B

and Reinhart T. Grundmann2



(1)
Department of Vascular Medicine, University Heart and Vascular Center at University Clinics Hamburg–Eppendorf, Hamburg, Germany

(2)
Former Medical Director, Community Hospital Altoetting-Burghausen, Burghausen, Germany

 



The Stanford classification system divides dissections into two categories. Type A dissections involve the ascending aorta regardless of the site of origin (surgery usually recommended). Type B dissections do not involve the ascending aorta (nonsurgical treatment usually recommended). Involvement of the aortic arch without involvement of the ascending aorta in the Stanford classification is labeled as Type B (Hiratzka et al. 2010). Note: in the following only dissections originating in the descending aorta distal to the left subclavian artery (distal aortic dissections Stanford type B), respectively type III dissections in the DeBakey classification system are discussed.


2.1 Guidelines



2.1.1 American Heart Association (AHA)


The guidelines of the American Heart Association (AHA) give the Class-I-recommendations (Hiratzka et al. 2010):



  • Acute thoracic aortic dissection involving the descending aorta should be managed medically unless life-threatening complications develop (e.g., malperfusion syndrome, progression of dissection, enlarging aneurysm, inability to control blood pressure or symptoms). (Level of Evidence: B)


  • For patients with chronic dissection, particularly if associated with a connective tissue disorder, but without significant comorbid disease, and a descending thoracic aortic diameter exceeding 5.5 cm, open repair is recommended. (Level of Evidence: B)


2.1.2 European Society of Cardiology (ESC)


The 2014 ESC guidelines on the diagnosis and treatment of aortic diseases recommend (Erbel et al. 2014):



  • In all patients with aortic dissection medical therapy including pain relief and blood pressure control is recommended. (Class I/Level of Evidence: B)


  • In uncomplicated Type B aortic dissection, medical therapy should always be recommended. (Class I/Level of Evidence: C)


  • In uncomplicated Type B aortic dissection, TEVAR (thoracic endovascular aortic repair) should be considered. (Class IIa/Level of Evidence: B)


  • In complicated Type B aortic dissection, TEVAR is recommended. (Class I/Level of Evidence: C). [The term ‘complicated’ means persistent or recurrent pain, uncontrolled hypertension despite full medication, early aortic expansion, malperfusion, and signs of rupture (haemothorax, increasing periaortic and mediastinal haematoma)].


  • In complicated Type B aortic dissection, surgery may be considered. (Class IIb/Level of Evidence: B)

According to this guideline, surgery is rare in cases of complicated Type B aortic dissection. Open repair has been replaced largely by endovascular therapy.


2.1.3 Society of Thoracic Surgeons Expert Consensus Document


The Society of Thoracic Surgeons Expert Consensus Document on the treatment of descending thoracic aortic disease using endovascular stent-grafts (Svensson et al. 2008) notes:



  • Acute descending (type B) aortic dissection is not as life-threatening as acute type A aortic dissection. Early survival is satisfactory using medical management alone, unless distal ischemic complications (“malperfusion”) or aortic rupture occurs. In patients with uncomplicated acute type B aortic dissection, this constitutes a benchmark that will be difficult to surpass, or even to match, by endovascular stent-graft treatment.


  • Patients with life-threatening complications of acute type B aortic dissection are at very high risk and require emergency treatment using thoracic aortic stent-grafting, open surgical aortic graft replacement, interventional or surgical flap fenestration, or catheter reperfusion or extra-anatomic surgical bypass, or both.


  • Once a patient survives 14 days after initial onset of an acute aortic dissection, it is defined as chronic. This definition is based on autopsy studies demonstrating that 74% of patients who die from dissections die within the first 2 weeks. The group of chronic dissection patients comprises those surviving surgery for acute indications and those initially treated with medical therapy alone.


  • Although primary medical therapy for uncomplicated type B dissection may improve hospital survival, it has not changed long-term survival. Most deaths are related to comorbid conditions, but late complications from distal aortic dissection are estimated to occur in 20–50% of patients. These sequelae include new dissection, with associated new complications, rupture of a weak false channel, and, most commonly, saccular or fusiform aneurysmal degeneration of the thinned walls of the false channel, which can lead to rupture and exsanguination.


  • Regardless of the approach used, as long as patients have residual dissected aorta, they remain at risk for late aneurysmal degeneration and rupture of the false lumen and require indefinite serial imaging surveillance, close blood pressure monitoring, and negative inotropic medical therapy.


2.1.4 Interdisciplinary Expert Consensus Document


An interdisciplinary expert consensus document on management of type B aortic dissections has been developed by Fattori et al. (2013b). The consensus describes algorithms for treatment of type B dissections.


Acute aortic dissection type B (first 2 weeks after onset of symptoms)





  • Patients with uncomplicated acute type B aortic dissection should be treated with medical therapy. At present, there is no evidence of advantage with TEVAR or open surgery.


  • TEVAR, when feasible, should be considered the first-line treatment in complicated acute type B dissection. A survival benefit is achieved by TEVAR in comparison with open surgery.


  • Aneurysmal evolution and eventual rupture may occur even in the absence of warning symptoms, and imaging follow-up must be performed at regular intervals. MDCT or MRI scan should be used to monitor uncomplicated dissections and should be performed at admission, 7 days, discharge, and 6 weeks, because the risk of instability is higher in the early phase.


  • Despite reasonably low early operative morbidity and mortality, there is the likelihood of aortic adverse events after TEVAR, and all patients need to be followed with imaging after treatment.


Panelists’ suggestions for definition of complicated type B acute aortic dissection





  • Malperfusion is indicative of impending organ failure and must be recognized early. Diagnosis of static or dynamic organ malperfusion is corroborated by laboratory markers (bilirubin, amylases, enzymes, creatinine) and imaging data.


  • Hypertension is indicative of complications in acute type B aortic dissection only when associated with malperfusion or persisting with uncontrolled high values despite full medical therapy.


  • Increases in perioaortic hematoma and hemorrhagic pleural effusion in two subsequent CT examinations during medical expectant management of acute type B aortic dissection are findings suggestive of impending rupture


Subacute aortic dissection type B





  • The subacute phase in aortic dissection (>2 to 6 weeks from onset) may sometimes reveal signs of instability, such as changes in aortic morphology (expanding diameter >4 mm, new onset of periaortic hematoma, and/or pleural hemorrhagic effusion), refractory hypertension, recurrent thoracic pain, and recurrent malperfusion. In these cases, TEVAR may be considered. However, data to support prognosis and complication rates in subacute type B aortic dissection are very limited.


Chronic aortic dissection type B





  • Most chronic type B aortic dissections are managed medically until complications develop. A tight control of systemic pressure with best medical treatment is of utmost importance to limit false lumen aneurysmal dilation over time.


  • Recurrence of symptoms, aneurysmal dilation (total aortic diameter >55 mm), or a yearly increase (> 4 mm) of aortic diameter should be considered signs of instability in the chronic phase and indication for TEVAR, or in unsuitable anatomy, indication for open surgery. Early mortality in complicated chronic type B aortic dissection is lower for TEVAR compared with open surgery. In uncomplicated chronic type B aortic dissection, yearly clinical and imaging follow-up is recommended, irrespective of diameter and treatment applied (TEVAR/medical/open surgery).


Panelists’ suggestions for definition of complications in chronic type B aortic dissection





  • In patients under medical management after the acute phase, recurrence of symptoms, aneurysmal dilation (>55 mm), or an aortic yearly increase of > 4 mm are indicative of higher worse prognosis without additional treatment (chronic complicated type B aortic dissections).


2.2 Results



2.2.1 Acute Uncomplicated Type B Aortic Dissection



2.2.1.1 Best Medical Treatment


In a systematic review and meta-analysis, outcome data of best medical therapy (BMT) were available for 2347 patients from 15 studies who underwent conservative medical management for acute type B aortic dissection (Moulakakis et al. 2014). The pooled 30-day/in-hospital mortality rate was 2.4%. The pooled rate for cerebrovascular events was 1%, for spinal cord ischemia 0.8% and for overall neurologic complications 2%. Survival rates ranged from 86.2% to 100% at 1-year and from 59.0% to 97.2% at 5-years, whereas freedom from aortic events ranged from 34% to 83.9%.

Although medical therapy is recommended for uncomplicated acute type B aortic dissection, the risk of aneurysm development with subsequent rupture in the long-term run should not to be neglected. Van Bogerijen et al. (2014) systematically reviewed and summarized the current available literature on prognostic variables related to aortic enlargement during follow-up in uncomplicated type B aortic dissection. A total of 18 full-text articles were found. The following predictors of aortic growth in these patients were identified: age <60 years, white race, Marfan syndrome, high fibrinogen-fibrin degradation product level (≥20 μg/mL) at admission, aortic diameter ≥40 mm on initial imaging, proximal descending thoracic aorta false lumen (FL) diameter ≥22 mm, elliptic formation of the true lumen, patent FL, partially thrombosed FL, saccular formation of the FL, presence of one entry tear, large entry tear (≥10 mm) located in the proximal part of the dissection, FL located at the inner aortic curvature, fusiform dilated proximal descending aorta, and areas with ulcer-like projection. In conclusion, a significant group of patients develops aneurysmal degeneration along the dissected segments during follow-up and might benefit from closer follow-up or early endovascular intervention.

Risk factors for failure of conservative treatment in acute type B aortic dissection (TBAD) were identified by Grommes et al. (2014) in a retrospective analysis of 104 patients. During the follow-up period, the initial medical treatment was converted to surgical treatment in 21 patients (20.2%) after a median of 333 days. In 5 patients (4.8%), endovascular surgery was performed during the acute dissection phase (within 14 days) because of acute complications, despite best medical treatment. In 16 patients (15.4%), surgery was performed after a median of 189 days. Surgical treatment was indicated because of aortic enlargement (n = 14), rupture (n = 1), or lower-limb ischemia (n = 1). In total, 16 patients (15.4%) died after a median of 774 days. Two patients died of aortic rupture during the acute phase of dissection, and further 14 patients died during the chronic phase of dissection, 6 of them due to dissection-related causes. Patients aged more than 66 years with a maximum aortic diameter greater than 40 mm at admission had a 6.87-fold higher mortality risk than younger patients and patients with smaller aortic diameters. Whether particularly older patients and those with early aortic dilatation benefit from prophylactic TEVAR, has to be questioned.

Durham et al. (2015b) identified a total of 298 patients with initially medical managed acute type B aortic dissections. Failure of medical therapy was defined as any death or aorta-related intervention. Early failure occurred within 15 days of presentation. There were 37 (12.4%) early failures, of which were 15 deaths and 25 were operative interventions. Thus, early mortality was 5%. The indication for early operation in a majority of patients was either renal ischemia (36.0%) or mesenteric ischemia (28.0%). Early aneurysmal degeneration was the indication for intervention in 24.0% of early operations. During a mean follow-up of 4.3 ± 3.5 years, failure of medical therapy occurred in 174 patients (58.4%). There were 87 (29.2%) aorta-related interventions and 119 (38.3%) deaths. An open operative approach was taken in 63 cases (72.4%). In 57 patients (65.5%), the indication for operation was aneurysmal degeneration. The actuarial freedom from intervention was 77.3% ± 2.4% after 3 years and 74.2% ± 2.5% after 6 years. Moreover, the intervention-free survival of the entire cohort was only 41% at 6 years, with end-stage renal disease being the only predictor of medical failure. These authors in addition presented 200 patients (61% men) with medically managed acute type B dissections receiving multiple imaging studies (Durham et al. 2015a). Mean follow-up was 5.3 years. At 5 years, only 51% of patients were free from aortic growth. Fifty-six patients (28%) required operative intervention (50 open, 6 endovascular repair) for aneurysmal degeneration, and the actuarial 5-year freedom from intervention was 76%. After excluding five patients (2.5%) with early rapid degeneration requiring intervention within the first 2 weeks, the mean rate of aortic growth was 12.3 mm/y for the total aortic diameter, 3.8 mm/y for the true lumen diameter, and 8.6 mm/y for the false lumen diameter. Complete thrombosis of the false lumen was protective against growth (odds ratio, 0.19). In conclusion, further study is needed to determine which patients presenting with acute type B dissection will benefit from early intervention (e. g., thoracic endovascular aortic repair) to prevent late aneurysm formation.

In contrast, Charilaou et al. (2016) reported nearly normal 6-year survival in patients with uncomplicated acute TBAD who underwent BMT. In this study, all 65 uncomplicated patients (100%) were treated medically and survived the initial hospitalization. Long-term survival in uncomplicated patients was 91%, 87%, 78%, and 55% at 1, 3, 5, and 8 years. That is, medically treated patients with uncomplicated acute TBAD achieved similar long-term survival as apparently healthy, age-matched and gender-matched controls. These good long-term results are contrary to the perspective of routine thoracic endovascular aortic repair for all TBAD patients.


2.2.1.2 Best Medical Treatment and Endovascular Aortic Repair


Luebke and Brunkwall (2014b) reviewed comparative studies of patients treated either with TEVAR or best medical treatment for uncomplicated type B aortic dissection (TBAD). Although the selected studies were not homogenous (with the risk of selection bias), the review strongly suggested that TEVAR may be beneficial compared to BMT in the treatment of uncomplicated Stanford Type B dissection, which is in agreement with the findings of the INSTEAD-XL trial. However, the early TEVAR-related deaths and complications, as well as trends toward higher paraplegia and stroke rates, raise concerns that moderate the better survival with TEVAR at 5 years.

The ADSORB (“acute dissection stent grafting or best medical treatment”) – study (Brunkwall et al. 2012) is the first prospective randomized multi-center-trial on acute dissections (symptom onset to diagnosis ≤14 days) comparing BMT with BMT and stent grafting of the proximal tear in patients having an uncomplicated acute dissection of the descending aorta. The objective of the study was to assess whether stent grafting will produce thrombosis and remodelling of the false lumen with a reduction in aneurysm formation and re-intervention. The primary endpoint of the study was a combination of incomplete/no false lumen thrombosis, aortic dilatation, or aortic rupture at 1 year. Thirty-one patients were randomised to the BMT group and 30 to the BMT + stent graft group (Brunkwall et al. 2014). During the first 30 days, no deaths occurred in either group, but there were three crossovers from the BMT to the BMT + stent graft group, all due to progression of disease within 1 week. At the 1-year follow up there had been another two failures in the BMT group: one malperfusion and one aneurysm formation. One death occurred in the BMT+ stent graft group. Incomplete false lumen thrombosis, was found in 13 (43%) of the BMT + stent graft group and 30 (97%) of the BMT group. The false lumen reduced in size in the BMT + stent graft group, whereas in the BMT group it increased. The true lumen increased in the BMT + stent graft group whereas in the BMT group it remained unchanged. The overall transverse diameter was the same at the beginning and after 1 year in the BMT group (42.1 mm), but in the BMT+ stent graft group it decreased (38.8 mm). In this trial remodelling with thrombosis of the false lumen and reduction of its diameter was induced by the stent graft. However, the question remains as to whether endovascular treatment with a stent graft in the acute phase of type B aortic dissection is associated with improved survival compared with medical treatment alone.

Shah et al. (2014) identified 4706 patients with uncomplicated TBAD from the National Inpatient Sample (NIS) for the years 2009 and 2010. Five-hundred and four patients were treated with TEVAR, 4202 were treated by medical management. The overall adjusted in-hospital mortality was similar for both groups (8.5% for TEVAR vs 10.3% for medical management). The TEVAR carried higher risk of stroke (odds ratio = 1.61). The TEVAR was associated with prolonged LOS (12 vs 5.6 days) and patients were less likely to be discharged home (odds ratio 0.73). Whether these findings support the more widespread use of TEVAR to treat patients with uncomplicated TBAD cannot be decided.


2.2.2 Acute Complicated Aortic Dissections Type B



2.2.2.1 Endovascular and Open Repair


A meta-analysis of the literature identified 2531 patients with acute complicated type B dissection that were treated with TEVAR (Moulakakis et al. 2014). The pooled rate for 30-day/in-hospital mortality was 7.3%. The pooled estimates for cerebrovascular events, spinal cord ischemia (SCI) and total neurologic events were 3.9%, 3.1% and 7.3%, respectively. Survival rates ranged from 62% to 100% at 1-year and from 61% to 87% at 5-years, whereas freedom from aortic events ranged from 45% to 77%. Comparative data for open treatment of acute complicated type B dissection in this meta-analysis were rather unfavorable. A total of 1276 patients from nine studies who underwent open surgical repair for acute complicated type B aortic dissection were analyzed. The pooled rate for 30-day/in-hospital mortality was 19.0%. The pooled rate for cerebrovascular events was 6.8%, for SCI 3.3% and for total neurologic complications 9.8%. Survival rates ranged from 74.1% to 86.0% at 1-year and from 44.0% to 82.6% at 5-years, whereas freedom from aortic events could not be estimated as there were no available data. The findings from Moulakakis et al. (2014) regarding in-hospital mortality with TEVAR were confirmed by a further systematic review where overall mortality and morbidity rates for TEVAR were 8.07% and 30.8%, respectively, in 1574 patients with symptomatic Stanford-B-dissection (Ramdass 2015).

Hogendoorn et al. (2014) assessed the comparative effectiveness of TEVAR vs. open surgical repair (OR) of complicated acute type B aortic dissections (cTBAD) using decision analysis. Main outcomes were quality-adjusted life years (QALYs). In the reference case, a cohort of 55-year-old men, TEVAR was preferred over OR: 7.07 QALYs vs. 6.34 QALYs for OR. The difference of 0.73 QALYs is equal to 8.5 months in perfect health. TEVAR was more effective in all analyzed cases and age groups. Perioperative mortality was the most important variable affecting the difference between OR and TEVAR, followed by the relative risk and percentage of aortic-related complications. Total expected reinterventions were 0.43/patient (TEVAR) and 0.35/patient (OR). The results of this decision model for the treatment of complicated acute TBAD suggest that TEVAR is preferred over OR. Although a higher number of reinterventions is expected, the total effectiveness of TEVAR is higher for all age groups. OR should be reserved for patients whose aortic anatomy is unsuitable for endovascular repair. Luebke and Brunkwall (2014a) also weighed the cost and benefit of TEVAR vs open repair (OR) in the treatment of acute complicated TBAD. In this cost-utility analysis OR appeared in terms of QALYs to be more expensive (incremental cost of €17,252.60) and less effective (−0.19 QALYs) compared with TEVAR. TEVAR yielded more QALYs and was associated with lower 1-year costs compared with OR in patients with an acute complicated TBAD and is therefore the dominant therapy over OR for this disease.


2.2.2.2 Clinical Studies and Case Series


Results of a prospective, nonrandomized, multicenter clinical trial for TEVAR of acute, complicated type B aortic dissection were reported by Bavaria et al. (2015). The trial enrolled 50 patients who were a mean age of 57.2 ± 12.9 years. All 50 patients had dissection-related symptoms at enrollment, including malperfusion (80%), rupture (14%), and malperfusion and rupture (6%). Thirty-day mortality was 8%. All 4 early deaths were considered dissection related. The Kaplan-Meier estimate of freedom from all-cause mortality was 14.6% at 12 months. Three patients (6%) required stent graft-related secondary procedures up to 30 days after implant, and an additional 3 patients (6%) underwent four late secondary procedures between 30 days and 12 months.

Excellent long-term results with TEVAR in 50 patients with acute complicated type B dissection, and in another 10 patients with acute complications, including rupture, end-organ ischaemia and acute dilatation during the primary hospitalisation, but >14 days after onset of symptoms were reported by Steuer et al. (2011). Within 30 days, two (3%) deaths, one (2%) paraplegia and three (5%) strokes were observed. Five-year survival was 87% and freedom from re-intervention at 5 years was 65%. Long-term results were also presented by Hanna et al. (2014). Fifty consecutive patients underwent TEVAR for management of acute complicated TBAD. Indications for intervention included rupture in 10 (20%), malperfusion in 24 (48%), and/or refractory pain/impending rupture in 17 (34%) patients. One patient (2%) had both rupture and malperfusion indications. In-hospital and 30-day rates of death were both 0%; 30-day/in-hospital rates of stroke, permanent paraplegia/paraparesis, and new-onset dialysis were 2%, 2%, and 4% respectively. Overall survival at 5 and 7 years was 84%, with no deaths attributable to aortic pathology. Thirteen (26%) patients required a total of 17 reinterventions over the study period. These data support the use of TEVAR for acute complicated type B aortic dissection but also highlight the importance of life-long aortic surveillance.

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Oct 14, 2017 | Posted by in CARDIOLOGY | Comments Off on Distal Aortic Dissection Type Stanford B

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