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Renal Malperfusion: Spontaneous Renal Dissection and with Aortic Dissection Amit Jain MBBS MS, Margaret C. Tracci MD JD, Dawn Coleman MD, Kenneth J. Cherry MD, Gilbert R. Upchurch Jr MD
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S0895-7967(14)00022-2 http://dx.doi.org/10.1053/j.semvascsurg.2014.06.004 YSVAS50429
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Cite this article as: Amit Jain MBBS MS, Margaret C. Tracci MD JD, Dawn Coleman MD, Kenneth J. Cherry MD, Gilbert R. Upchurch Jr MD, Renal Malperfusion: Spontaneous Renal Dissection and with Aortic Dissection, ĆSemin Vasc Surg , http://dx.doi.org/10.1053/j.semvascsurg.2014.06.004 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
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Renal malperfusion: Spontaneous renal dissection and with aortic dissection. Amit Jain MBBS MS, , Margaret C. Tracci MD JD, Dawn Coleman MD Kenneth J. Cherry MD, Gilbert R Upchurch Jr MD Division of Vascular and Endovascular Surgery University of Virginia Health System Charlottesville – VA Correspondence Address: Gilbert R Upchurch Jr MD PO Box 800679, Charlottesville, VA, 22908 Phone: 434.243.6333 Fax: 434.243.9941 Email: [email protected]
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Renal malperfusion: Spontaneous renal dissection and with aortic dissection. Amit Jain MBBS MS , Margaret C. Tracci MD JD, Dawn Coleman MD ,Kenneth J. Cherry MD, Gilbert R Upchurch Jr MD
Abstract Renal malperfusion associated with renal artery dissection may present as either an isolated disease process or in the setting of branch vessel stenosis complicating aortic dissection. Isolated renal artery dissection is a rare disorder whose clinical presentation often presents both a diagnostic and therapeutic challenge. The true incidence and natural history of this phenomenon also remain unclear. Multiple approaches to management have been described. Medical therapy typically consists of anticoagulation and blood pressure management and is reserved for cases with well controlled symptoms and blood pressure and preserved, stable renal function. Historically, surgical reconstruction with in‐situ or more complex ex‐vivo reconstruction has been described for the treatment of uncontrolled hypertension with preservation of renal perfusion. Nephrectomy, either partial or total, for control of hypertension, is reserved for cases where parenchymal injury necessitates this radical intervention. Recently, endovascular stenting of the renal artery has shown excellent and durable results and is now considered to be the first line intervention for renal artery dissection. Renal malperfusion associated with complicated aortic dissection is a different entity and one, which is consistently an independent predictor of poor prognosis. The pathogenesis of malperfusion can be dynamic, static or a combination. In addition, renal hypoperfusion may occur with or without extension of the intimal flap into the renal artery itself. Traditional open surgical interventions to treat aortic dissection with malperfusion have a very high perioperative mortality rate. Endovascular
Page 3 of 30 fenestration and stenting of both thoracic aortic and branch vessels have significantly improved clinical outcomes in complicated aortic dissections relative to open surgical fenestration. Although a significant body of long term data has yet to be accumulated, endovascular stent grafting has the added advantage over fenestration in that it may affect aortic remodeling, thus preventing the very morbid complication of aneurysmal degeneration.
Introduction and Epidemiology
Renal artery dissection (RAD) is an uncommon diagnosis. It was first reported in 1944 by
Bumpus et al.1 In most cases, RAD is clinically silent and hence its exact incidence is unknown. A small number of cases reported likely represent a more common, but likely underdiagnosed entity. With the increasing use of noninvasive imaging modalities and advanced endovascular interventions more renal artery dissections are being diagnosed. Isolated and spontaneous renal artery dissection was reported early in a number of small case series with a reported incidence of about 0.036 to 0.049% of all arterial dissections.2‐4 RAD can cause varying degree of renal parenchymal loss and hypertension. RAD, when symptomatic, typically presents as acute abdominal or flank pain. It may also present with hematuria mimicking renal colic secondary to renal stone, thus creating a diagnostic dilemma. Chronic RAD, on the other hand, is often diagnosed on the workup of abrupt onset of severe hypertension in a young adult that often requires multiple drug treatment. Aortic dissection can also cause renal artery malperfusion. About 25‐30% of the individuals with aortic dissections have some form of vascular insufficiency involving the visceral, renal, spinal, cerebral or limb.5‐7 Renal ischemia has been reported in approximately 8% of these cases.6,8,9 Renal malperfusion is an independent predictor of in‐hospital mortality in patients with aortic dissections.8, 10‐13 Hence, the identification and treatment of renal malperfusion with or without renal artery dissection is of paramount significance in patients with complicated aortic dissections.
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Etiology, Pathogenesis and Classification The etiology of isolated RAD is not clearly understood. Arterial dissection is caused by either bleeding in between the layers of the arterial wall via a primary intimal tear or hemorrhage from the vasa vasorum. Though the entity of RAD is relatively rare, renal arteries are one of the more common sites of primary dissection involving peripheral vessels and may be associated with atherosclerosis, fibro‐muscular dysplasia (FMD), Ehlers‐danlos syndrome, Marfan’ syndrome, malignant hypertension, blunt trauma and severe strenuous physical activities. 14‐20 Based on their presentation, RADs can be broadly categorized into two clinical categories, acute or chronic. RADs may also be classified based on their etiology (Table 1). Isolated RADs are localized to renal arteries. Primary or spontaneous RADs are usually associated with renal artery atherosclerosis or FMD.16 Sometimes, no underlying pathology can be identified and therefore classified as idiopathic (Figure 1). Spontaneous RADs in an otherwise healthy individuals have been reported with excessive physical exertion possibly playing a role in its causation.16‐19 Secondary RADs can be defined as those due to either blunt abdominal trauma or as a consequence of catheter and wire manipulation during an endovascular intervention. While renal artery thrombosis is the most frequent renal vascular pathology in blunt trauma, renal artery intimal disruption has also been reported.21‐24 Renal artery dissections are not uncommon after endovascular renal arterial interventions and may occur in 5‐24% individuals. 2,25, 26 Many of RAD associated with endovascular renal interventions may not be reported as they are treated with stent placement during the course of the procedure. Aortic dissection can cause renal malperfusion with or without associated renal artery dissection. The aortic flap may cause either a static or dynamic obstruction of renal artery blood flow depending on the configuration of the flap and its movement during the cardiac cycle.27 Static renal malperfusion is present throughout the cycle and is typically caused by the aortic dissection involving
Page 5 of 30 the renal artery ostium. The aortic wall hematoma may propagate into the renal artery, compromising the vessel lumen. Dynamic renal malperfusion varies with the pulsatile flow of blood in the aorta and is generally the result of cyclic prolapse of the dissection flap into the renal artery origin. Occasionally the obstruction may represent combination of varying degrees of both static and dynamic components.27,28 (Figure 2) Table 1: Classification of renal artery dissection.
Classification of renal artery dissection 1. Isolated RAD a. Primary – Spontaneous RAD i. Atherosclerosis ii. Fibromuscular Dysplasia (FMD) iii. Other connective tissue disorders (CTDs) iv. Idiopathic b. Secondary – i. Trauma ii. Iatrogenic Interventional procedures 2. Combined RAD with AD a. Static obstruction b. Dynamic obstruction c. Combined
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Presentation 1. Renal malperfusion with isolated renal artery dissection: Renal artery dissections are rare and often clinically silent. They usually affect young individuals in their third, fourth or fifth decade of life. Men are affected more than women (2.5:1).3,16,29 When clinically overt, they present acutely with various combinations of abdominal or flank pain, nausea, vomiting, headache, dysuria or hematuria (Table 2).2‐4,15,16 Spontaneous RAD can present with the sudden onset of malignant hypertension which may even be complicated by hypertensive encephalopathy.30,31 Abrupt onset of severe hypertension requiring three or four antihypertensive drugs was seen in majority of the patients requiring operative reconstruction of RADs.32,33 Hypertension may also be accompanied by varying degrees of renal dysfunction, up to and including frank renal failure.20,32‐ 35
Bilateral renal artery involvement is present in 12‐25% cases and is strongly associated with FMD as
the underlying pathology.16,32,33 The primary dissection event may be silent or associated with vague abdominal pain that goes undiagnosed until it presents in a delayed fashion as uncontrolled hypertension. Although RAD is typically isolated, it must be recognized that abdominal pain may also represent a rare simultaneous visceral arterial dissection.33 In a recent review of the clinical characteristics of the patients with SRADs, Afshinnia et al studied 17 patients who presented over a 12 years period.36 Of these, 11 (65%) were male and 14 (82%) white. Patients with SRAD were more likely to have higher mean serum creatinine, systolic and diastolic blood pressure and a lower body mass index at presentation. FMD was present in 4 patients, Ehlers‐ Danlos in 4 and polyarteritis nodosa was seen in 1 patient. The most common presenting symptom was pain (flank pain in 10 and abdominal pain in 2 patients).
Page 7 of 30 2. Renal malperfusion with aortic dissection with or without RAD:
Clinical suspicion for renal artery malperfusion in individuals with aortic dissection is based on
worsening hypertension and / or evolving renal insufficiency manifested by oliguria, anuria or a rise in serum BUN and creatinine. The renal circulation (8%) is the second most commonly malperfused arterial bed in aortic dissection, following the iliofemoral (11%) system.6,37
Investigation and Diagnosis 1. Renal malperfusion with isolated renal artery dissection: Symptoms of isolated spontaneous RADs often mimic other, more common etiologies of acute abdominal or flank pain and are often misdiagnosed. Reported misdiagnoses have included renal colic and even appendicitis, resulting in emergency appendectomy.33 A high index of suspicion in patients with abdominal or flank pain along with rising serum creatinine and uncontrolled hypertension can guide appropriate investigations. Measurement of serum LDH in the acute phase in addition to serum BUN and creatinine may indicate renal parenchymal loss.32 Duplex scanning to measure main renal artery flow velocity and renal intraparenchymal resistance indices provides additional data regarding renal perfusion and may even permit visualization of dissection. The kidney size can be evaluated by B mode ultrasound imaging. If renal function is not severely deranged, cross sectional imaging with CTA or MRA provides excellent resolution diagnostic imaging. Although CTA and MRA offer high sensitivity and specificity in detecting renal artery stenosis, digital subtraction angiography has been used widely and some authors continue to prefer this modality to evaluate extent of dissection and intimal flap morphology and to plan treatment strategy. 30,32,33,38 Muller et al32 have also suggested use of isotope renography to evaluate bilateral renal function and renal vein renin measurement to be helpful in selected cases. 2. Renal malperfusion with aortic dissection with or without RAD:
Page 8 of 30 CTA is the most widely used diagnostic modality for aortic dissection and is able to demonstrate renal hypoperfusion, where present, in most cases of AD with or without RAD. Evidence of renal malperfusion may include renal parenchymal infarction, differential contrast density nephrograms and visualization of an occlusive intimal flap. Barnes et al28 used contrast angiography to study 165 patients with clinically suspected malperfusion and confirmed true renal malperfusion by establishing the presence of a systolic gradient between aortic root and renal hilum of >10 mm Hg, failure of the renal artery to fill during contrast injection in the true and false lumen of the aorta, or intravascular ultrasound evidence of a “curtain‐like” occlusion of the renal ostium or the true lumen above its origin. Using these criteria, they diagnosed true renal artery malperfusion in 67% (n=59/88) of the patients clinically suspected to have renal artery malperfusion. Interestingly, they also diagnosed renal artery malperfusion in 39% (n=31/79) of those patients with suspected malperfusion of non‐renal tissues but without an initial clinical suspicion of renal hypoperfusion.
Treatment Options and Outcomes 1. Renal malperfusion with isolated renal artery dissection: The treatment options described for isolated spontaneous renal artery dissections (ISRAD) include medical management, open surgical reconstruction or endovascular intervention. Optimal management depends on the severity of the patient’s clinical condition, degree of renal infarction and extent of the renal artery dissection. In general, treatment seeks to preserve renal function and treat renovascular hypertension. Medical therapy. Ramamoorthy et al described successful non‐operative management of four patients with ISRAD.29 All of them were managed expectantly with anticoagulation. Blood pressure was easily controlled and renal function was stable in all 4 patients over a mean follow up of 14 month. However, one of the patients with FMD did develop a contralateral renal artery dissection. Edwards et
Page 9 of 30 al34 compared 13 patients treated medically with 11 patients who underwent surgery for ISRAD. They concluded that medical treatment with antihypertensive medications was equally effective. Afshinnia et al36 reported treating 8 patients out of 17 in their series of ISRAD, with medical management which consisted of pain control, control of hypertension and systemic anticoagulation. Others have also documented successful non‐operative management of ISRAD with or without anticoagulation.3,16,17,19 Medical management seems appropriate when renal function is stable and patient symptoms and hypertension can be controlled with medication. Open surgical therapy. Surgical treatment for the SRAD have been reported in several retrospective case series and, again, is aimed at preserving renal function and treating renovascular hypertension.16,20 32‐35 Smith et al16 described 10 renal artery bypass procedures in 9 patients with 100% immediate patency and maintenance or improvement in renal function. Five of these were ex‐vivo complex reconstructions due to renal branch vessel involvement. None needed nephrectomy. Lacombe33 treated 22 patients with 17 arterial bypasses and 8 nephrectomies (6 total, and 2 partial). Three patients had bilateral lesions. There were no post‐operative deaths. Arterial hypertension was cured in 9 patients (41%), improved in 11 (50), and unchanged in 2 (9%). On long term follow up (mean 10.1 years), one late thrombosis of a repaired polar artery and one spontaneous dissection of contralateral renal artery was seen. Muller et al32 described surgical treatment of RAD in a series of 25 patients with 22 SRADs and 3 with trauma with all but one reconstruction in situ. Hypertension resolved or improved in 86% of patients without preoperative renal damage and in only 38% of those with preoperative damaged kidneys. Renal function was preserved in 23 of 28 revascularized kidneys (82%) at a mean follow up of 55.3 months. The efficacy of extracorporeal reconstruction with autotransplantation of 19 kidneys was attempted in 15 patients by van Rooden et al.39 With a technical success rate of 94%, a favorable outcome was demonstrated in 79% of patients for blood pressure control and all the patients had normal renal functions over a 1 to 8 year follow up. There were no operative deaths and one primary nephrectomy was performed. In all of the above mentioned case series, surgical treatment was indicated for uncontrolled hypertension, persistent symptoms and / or
Page 10 of 30 deteriorating renal function. Both in‐situ and ex‐vivo repair are feasible and safe. Besides the preference of the surgeon, ex‐vivo repairs are used for complex reconstructions of RAD involving the branch vessels. Nephrectomy, either partial or total should be considered for severely damaged kidneys to prevent renovascular hypertension. Endovascular Therapy. Endovascular interventions have been also described for the treatment of both primary, spontaneous RADs, as well as secondary RADs either due to blunt trauma or iatrogenic endovascular interventions.21,30,36,38, 40‐43 Bilge et al43 and Lee et al40 each reported the earliest cases of SRAD treated by transcatheter intervention with adjunct stent placement. Pellerin et al30 subsequently described 16 patients with 17 SRAD treated with endovascular stent placement. In this later series all patients had uncontrolled hypertension and 10 patients had progressive renal insufficiency. With a 100% technical success, they reported clinical success in treating all the patients’ elevated blood pressure. More than 40% of them were cured and remained normotensive without taking any antihypertensive medications during a follow up of more than 8 years. All the patients had normal renal function and imaging of the renal arteries showed no sign of restenosis or occlusion in all patients. Three other recent case reports describe successful placement of renal artery stent for SRAD.38, 41,42 Afshinnia et al36 reported 17 cases of SRADs treated with supportive medical care, endovascular treatment and surgery for 8, 5 and 4 cases, respectively. However, of the 5 endovascular cases, thrombolysis and subsequent anticoagulation was required in one patient for acute thrombotic occlusion of the renal artery. The remaining 4 patients had Ehlers‐Danlos syndrome with leaking, dissecting renal artery aneurysms and were treated with endovascular coil embolization with immediate stabilization. Lee et al21 report a case of successful endovascular stent placement for a renal artery dissection secondary to blunt abdominal trauma. Bush et al25 treated 85 renal artery stenoses with 88 Palmaz stents. Although most stents were implanted for suboptimal balloon dilatation (52%), 24% were placed for secondary dissections caused by the balloon angioplasty. Thus, endovascular interventions for isolated RAD are now well established and safe in the setting of both primary and secondary etiologies.
Page 11 of 30 2. Renal malperfusion with aortic dissection with or without RAD: Aortic dissections complicated by renal artery malperfusion with or without RAD are associated with significantly increased mortality.8, 10‐13 In complicated aortic dissections, both open and endovascular approaches aim to restore flow to all the malperfused vascular beds, including the renal arteries. Open surgical therapy. Open procedures may involve aortic graft interposition, excision of the intimal flap and creation of an open fenestration, or performing aorta to branch vessel bypass to restore the blood flow to the malperfused organs. In multiple large series, open surgical treatment for complicated aortic dissection with visceral malperfusion have extremely high mortality rates (6‐ 69%).6,8,9,12,13 Lauterbach et al6 reported 187 patients with aortic dissection (101 proximal and 86 distal) with 53 patients (28%) having clinical evidence of organ or limb malperfusion. Of these, 12 patients had renal malperfusion. One third (17 patients) of those with malperfusion required specific intervention to treat the malperfusion. Twelve open procedures and 5 endovascular procedures (including 3 aortic fenestration and 2 renal stents) were performed. The in‐hospital mortality for the entire group was 18%. Panneton et al44 described open aortic fenestration for 14 patients with complicated aortic dissection (3 type A, 11 type B). Emergent fenestration was performed in 7 patients, 5 of them had renal malperfusion, 3 bowel and 4 lower extremity ischemia. Operative mortality was 43% (3/7) in the emergent group and there were no postoperative deaths in the elective group. The IRAD investigators reported open surgical repairs of complicated type B aortic dissection between 2006 and 2008 with an in‐hospital mortality of 29% and 34% for 82 and 59 open surgical interventions, respectively.45,46 These mortality rates were significantly higher compared to patients treated with endovascular therapy in the same database (11% in 2006 and 11% in 2008), as well as who were managed medically (10% in 2006 and 8.7% in 2008) during the same study period. The in‐hospital complication rate was 40% in those undergoing open surgical treatment compared to 21% in the endovascular group.46
Page 12 of 30 Endovascular aortic fenestration and stent therapy. Advances in endovascular techniques have enhanced the safety and efficacy of minimally invasive percutaneous endovascular interventions in the treatment of ischemic complications of aortic dissection. Endovascular aortic fenestration with or without branch vessel stenting was proposed and widely studied in late 1990s.47 Endovascular fenestration and stenting are technically challenging, requiring an advanced level of operator endovascular skill, a well‐equipped intervention suite, and experienced staff. The procedure involves extensive angiographic and IVUS evaluation of the anatomy of the dissected aorta, the intimal flap, and branch vessels and as well as the physiology of the dissection, including measurement of intravascular pressure gradients between the true and false lumens and the aortic branches. True malperfusion is confirmed by a systolic gradient between the aorta and the superior mesenteric artery or renal hilum of >15 mm Hg, failure of a branch artery to fill during injection of contrast in the true and false lumen of the aorta, evidence of a “curtain‐like” occlusion of the vessel origin or of the true lumen above the origin by IVUS, thrombosis, or evidence of embolization.47,48 The aortic intimal flap, once identified as causing the either a static or dynamic obstruction, is punctured with a trocar needle and the tear in the flap is dilated with a balloon. A persistent true lumen collapse or an unresolved pressure gradient can be further treated by placing a large diameter self‐expanding stent to open the aortic true lumen. Care should be taken to avoid covering the origins of renal and superior mesenteric arteries and or across fenestrations essential to branch vessel perfusion.48 Further branch vessel static obstructions should be treated by stent placement origin of these vessels. Williams et al47 treated 24 patients with aortic dissection complicated by ischemia of liver or bowel (n=15), kidney (n=18) or lower extremity (n=13). Treatment consisted of vascular stents alone (n=4), or balloon fenestration (n=20) without (n=8) or with (n=12) vascular stents. With a 30 day mortality of 25% (6 patients), there was a 92% success in restoring flow in the obstructed vessels (71 of 77 obstructed arteries). Slonim et al49 reported 40 patients with aortic dissection (10 type A and 30 type B) with peripheral ischemic complications. Thirty patients had renal, 22 had limb, 18 had mesenteric and 1 had upper arm ischemia. Fourteen patients were treated with combined stenting of true or false
Page 13 of 30 lumen with balloon fenestration of the flap, 24 with stenting alone and 2 with fenestration alone. Successful revascularization was achieved in 37 of 40 patients (93%) with a 25% (10 out of 40 patients) 30‐day mortality. The mortality was often related to ischemia at presentation. In one of the largest series on renal malperfusion Barnes et al28 reported a single center experience in treating renal malperfusion after aortic dissection with aortic fenestration and renal artery stenting. They included all patients with aortic dissection from 1996 to 2004 in whom there was sufficient clinical suspicion for peripheral malperfusion to require arteriography. The group comprised 165 patients (115 acute and 50 chronic), of which 75 had type A and 90 had type B dissection. Renal malperfusion was suspected in 88 patients secondary to worsening hypertension (n=34), renal insufficiency (n=37), CT evidence of renal ischemia (n=13) or a combination of above factors (n=4). Renal malperfusion, confirmed with a systolic gradient between the aortic root and renal hilum, was present in 59 of the suspected 88 patients (67%). However, renal malperfusion was also detected in 31 of 79 patients (39%) with suspected malperfusion of non‐renal tissue. Renal arteries were perfused by true lumen (70% right, 42% left), false lumen (7% right, 20% left), or both true and false lumen (23% right, 38% left). Of the 90 patients with confirmed renal malperfusion, 71 had endovascular treatment, including isolated renal artery stenting (n=31), proximal fenestration with or without stenting (n=24), or both renal and aortic intervention (n=16). Post‐intervention the pressure gradient across the aortic root and renal hilum decreased from an average of 44 mm Hg to 8.1 mm Hg. The peri‐procedure post‐ intervention mortality was 21% (n=15). In a separate report on 69 patients with complicated type B aortic dissection, Patel et al50 identified 185 malperfused vascular beds in 70% of patients, including spinal cord (5), mesenteric (40), renal (51), and lower extremity (47). Using aortic fenestration and true lumen or branch vessel stents, flow was restored in 96% of these vascular beds. Stroke and paraplegia rates were 4.3% and 2.9% and early mortality rate was 17%. Chavan et al51 described their experience of treating 45 patients with endovascular fenestration and stenting for complicated aortic dissection (13 type A and 32 type B). Out of total 88 vascular beds, 25 involved renal arteries, 22 mesenteric and 33 lower extremity vessels. Seven of 25 patients with renal ischemia required dialysis, but all of the patients
Page 14 of 30 were dialysis free 3 months following the intervention. The 30‐day mortality was 6.7%. Shiiya et al52 have reported on organ malperfusion in 38 of 135 patients with aortic dissection (31 type A). They emphasize a mechanism‐specific approach to treatment, noting the inability of a central aortic operation alone to adequately address renal branch malperfusion (15% success with 2 of 13 patients). However, a percutaneous endovascular approach was 100% successful in all vessels with branch type malperfusion. Endovascular stent graft therapy. Besides aortic fenestration and branch vessel stenting, endovascular graft placement to cover the site of aortic intimal tear has been shown to redirect flow into the true lumen and, in many cases, reverse organ malperfusion.53,54 During the last decade, the use of endovascular stent graft to treat complicated aortic dissection has been widely accepted with favorable results (Figure 3). Dake et al53 described 19 patients treated with stent grafts for aortic dissection (4 type A and 15 type B). Of these, 37% patients had symptomatic malperfusion. They reported a 76% improvement in malperfusion with a 16% 30‐day mortality rate. Thoracic stent graft placement resolved the obstruction in all of the 22 vessels with dynamic obstruction, but only 6 of the 15 vessels with combined static and dynamic obstruction. The benefit of aortic stent grafting in dynamic obstruction is clear, however there may be limitations in its ability to address branch vessel dissection or static obstruction of other etiologies.28 Since this report, several authors have published their experience of treating aortic dissection with visceral malperfusion syndrome with additional use of bare metal stents at the origin of visceral, renal and iliac vessels as necessary. Conrad et al55 reported treating 33 patients with thoracic endograft for complicated acute type B aortic dissection. Seventeen of these patients (53%) had malperfusion syndrome. All were repaired successfully with a 12% 30‐day mortality rate. Szeto et al56 examined treatment of acute complicated type B dissection in 35 patients, 17 of which had malperfusion. Technical success was achieved in 97% (34) of patients with 34% (12) of patients requiring additional procedures including bare metal stents in the infrarenal aorta, renal artery, celiac artery or iliac artery. One patient had permanent paraplegia (2.8%), one had stroke (2.8%) and 30‐ day mortality was 2.8%. White et al57 reported results of thoracic endovascular aneurysm repair (TEVAR)
Page 15 of 30 for complicated type B aortic dissection at 30 days and 1 year. They had 99 patients, 85 acute (symptom onset ≤ 14 days), 11 subacute (15‐30 days) and 3 chronic (31‐90 days). Of the acute 32 % had rupture and 72% had malperfusion, including 56% lower extremity, 36% renal, 19.7% visceral, 8.2% other and 3% spinal cord. Additional adjunctive procedures were necessary in 4.7% of the patients, including a carotid artery covered stent in one patient and bare metal stent in the true lumen of the right renal, left renal and left iliac arteries in three patients. Major adverse events at 30‐days were 10.6% death, 9.4% stroke, 9.4% renal failure and 9.4% paralysis / paraparesis. Actuarial mortality estimate at 1 year was 29.4. Lombardi et al58 described 40 patients with complicated type B aortic dissection treated with a composite endograft in the STABLE trial. Branch vessel malperfusion was present in 67.5% (27/40) patients, including mesenteric (5 of 27), renal (17 of 27), spinal cord (1 of 27) and limbs (15 of 27). All 40 patients received one or more endovascular stent grafts and 39 patients received at least one bare metal aortic stent. During the initial procedure, adjunctive stent placement was performed in 9 patients for 13 branch vessels (6 iliac arteries, 6 renal arteries, and one superior mesenteric artery). The 30‐day mortality rate was 5% (2 of 40), stroke (7.5%), transient ischemic attack (2.5%), paraplegia (2.5%), retrograde progression of dissection (5%), and renal failure (12.5%). None of the patients with renal failure became dialysis‐dependent. Many more authors have reported successful treatment of complicated aortic dissections with visceral malperfusion, including renal vessels, with endovascular stent graft with or without adjunctive stent placement in the branch vessels.59,60 A recently published report by Wilkinson et al61 analyzed 73 patients with type B dissection who underwent either open repair (n=24) or TEVAR(n=49) for malperfusion (n = 8), rupture (n = 22), or factors portending rupture, including rapid expansion (n = 26), uncontrolled pain (n = 18), aortic size greater than 5.0 cm (n = 26), or refractory hypertension (n = 2). The early and late mortality were similar between groups (16.7% for open and 10.2% for TEVAR, p=0.46). Ten‐year Kaplan‐Meier survival was 57.5% and similar between groups (p = 0.74). Comparing their results with other contemporary series they concluded that an early mortality benefit with TEVAR for complicated type B dissection (not seen in their study) was more when the intervention was performed for malperfusion than for rupture. They
Page 16 of 30 had a small number of patients with malperfusion in their study. They also concluded that TEVAR provided similar midterm treatment efficacy and late survival compared to open surgical repair of descending thoracic aorta.
Summary The management of renal malperfusion associated with both isolated RAD and aortic dissection has significantly evolved over time. Although successful medical management of RAD has been described in multiple case reports and small case series, it should be considered only in patients with stable renal function and well controlled hypertension. Aggressive attempts at revascularization of the renal arteries should be made whenever there is evidence of deteriorating renal function and/or in the presence of uncontrolled hypertension. Open surgical reconstruction of the renal arteries, both in‐situ and ex‐vivo has been performed with excellent results at experienced centers. However, endovascular therapy has proven effective, with limited morbidity, and is preferred modality of treatment where anatomically suitable. In the treatment of renal malperfusion with aortic dissection, an endovascular approach should be the first line of therapy as well, except in the unstable patient with a type A dissection who needs emergent open cardiac surgery. Both endovascular fenestration with stenting (“fen‐stent”) and endovascular stent grafting have been shown to be successful. DiMusto et al48 analyzed in detail the benefits and limitations of fen‐sten versus aortic endografts. In summary, fen‐sten is appropriate for treating malperfusion only, and is technically very demanding and time consuming. Endografting on the other hand, has broader indications in aortic dissections, including impending rupture, unstable or leaking false lumen, as well as malperfusion. However, the efficacy of endografts in treating distal branch type malperfusion may be limited. Fenestration has narrow clinical indications, but broad anatomical applicability, whereas thoracic stent graft has broad clinical indications, but perhaps
Page 17 of 30 narrower anatomical applicability.48 However, this limitation of endovascular stent grafts used alone can be overcome by adjunctive stenting of malperfused vascular beds, as necessary, at the time of stent graft deployment. The long term data on the durability of the stent graft is still evolving, but appears promising.
References: 1. HC Bumpus Jr. A case of renal hypertension. J Urol, 1944; 52: 295–299. 2. Gewertz BL, Stanley JC, Fry WJ. Renal Artery Dissections. Arch Surg. 1977; 112(4): 409‐414. 3. Beroniade, V., Roy, P., Froment, D., & Pison, C. Primary renal artery dissection. American journal of nephrology, 1987; 7(5): 382‐389. 4. Banitt PF, Clark CW. Isolated dissecting aneurysm of the renal artery. J Urol, 1986; 135: 998–999. 5. DeBakey, M.E., McCollum C.H., Crawfor E.S. et al. Dissection and dissecting aneurysms of the aorta: twenty‐year follow‐up of five hundred twenty‐seven patients treated surgically. Surgery, 1982; 92: 1118–1134. 6. Lauterbach, S. R., Cambria, R. P., Brewster, D. C., et al. Contemporary management of aortic branch compromise resulting from acute aortic dissection. Journal of vascular surgery, 2001; 33(6): 1185‐1192. 7. Girardi, L. N., Krieger, K. H., Lee, L. Y., et al Management strategies for type A dissection complicated by peripheral vascular malperfusion. The Annals of thoracic surgery, 2004; 77(4): 1309‐ 1314.
8. Fann, J. I., Sarris, G. E., Mitchell, R. S., et al. Treatment of patients with aortic dissection presenting with peripheral vascular complications. Annals of surgery, 1990; 212(6): 705. 9. Cambria, R. P., Brewster, D. C., Gertler, J., et al. Vascular complications associated with spontaneous aortic dissection. Journal of vascular surgery, 1988; 7(2): 199‐209. 10. Suzuki, T., Mehta, R. H., 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(10 suppl 1): II‐312. 11. Nienaber, C. A., & Eagle, K. A. Aortic Dissection: New Frontiers in Diagnosis and Management Part II: Therapeutic Management and Follow‐Up. Circulation, 2003; 108(6): 772‐778. 12. Yagdi T., Atay Y., Engin C., et al. Impact of organ malperfusion on mortality and morbidity in acute A aortic dissections. J Card Surg, 2006; 21: 363–369. 13. Fann, J. I., Smith, J. A., Miller, D. C., et al. Surgical management of aortic dissection during a 30‐year period. Circulation, 1995; 92(9): 113‐121.
Page 18 of 30 14. Foord, A. G., & Lewis, R. D. Primary dissecting aneurysms of peripheral and pulmonary arteries: dissecting hemorrhage of media. Archives of pathology, 1959; 68: 553. 15. Englund, G. W. Primary dissecting aneurysm of the renal artery. Report of a case and review of the literature. American journal of clinical pathology, 1966; 45(4): 472. 16. Smith, B. M., & Holcomb, G. W. Renal artery dissection. Annals of surgery, 1984; 200(2): 134. 17. Alamir, A., Middendorf, D. F., Baker, P., et al. Renal artery dissection causing renal infarction in otherwise healthy men. American journal of kidney diseases, 1997; 30(6): 851‐855. 18. Thomas MC, Walker RJ, Packer. Running repairs: renal artery dissection following extreme exertion. Nephrol Dial Transplant. 1999; 14(5): 1258‐9. 19. Iqbal FM, Goparaju M, Yemme S, et al. Review Renal artery dissection following marathon running. Angiology. 2009; 60(1): 122‐6. 20. Slavis, S. A., Hodge, E. E., Novick, A. C., et al. Surgical treatment for isolated dissection of the renal artery. The Journal of urology, 1990; 144(2 Pt 1): 233‐237. 21. Lee, J. T., & White, R. A. Endovascular management of blunt traumatic renal artery dissection. Journal of Endovascular Therapy, 2002; 9(3): 354‐358. 22. Clark, D. E., Georgitis, J. W., & Ray, F. S. Renal arterial injuries caused by blunt trauma. Surgery, 1981; 90(1): 87. 23. Kaufman, J. L., Dinerstein, C. R., Shah, D. M., et al. Renal artery intimal flaps after blunt trauma: indications for nonoperative therapy. Journal of vascular surgery, 1988; 8(1): 33‐37. 24. Sharples EJ, Sobeh M, Matson M, et al. Renal artery dissection after blunt abdominal trauma: a rare cause of acute cortical necrosis. Am J Kidney Dis, 2002; 40: E11. 25. Bush, R. L., Najibi, S., MacDonald, M. J., et al. Endovascular revascularization of renal artery stenosis: technical and clinical results. Journal of vascular surgery, 2001; 33(5): 1041‐1049. 26. Martin, L. G., Casarella, W. J., Alspaugh, J. P., et al. Renal artery angioplasty: increased technical success and decreased complications in the second 100 patients. Radiology, 1986; 159(3): 631‐634. 27. Williams, D. M., Lee, D. Y., Hamilton, B. H., et al. The dissected aorta: part III. Anatomy and radiologic diagnosis of branch‐vessel compromise. Radiology, 1997; 203(1): 37‐44. 28. Barnes, D. M., Williams, D. M., Dasika, N. L., et al. A single‐center experience treating renal malperfusion after aortic dissection with central aortic fenestration and renal artery stenting. Journal of Vascular Surgery, 2008; 47(5): 903‐910. 29. Ramamoorthy, S. L., Vasquez, J. C., Taft, P. M., et al. Nonoperative management of acute spontaneous renal artery dissection. Annals of vascular surgery, 2002; 16(2): 157‐162. 30. Pellerin O., Garçon P., Beyssen B., et al. Spontaneous renal artery dissection: long‐term outcomes after endovascular stent placement. J Vasc Interv Radiol, 2009; 20: 1024‐1030.
Page 19 of 30 31. Esayag‐Tendler, B., Yamase, H., Ramsby, G., et al. Accelerated hypertension with encephalopathy due to an isolated dissection of a renal artery branch vessel. American journal of kidney diseases, 1994; 23(6): 869‐873. 32. Müller, B. T., Reiher, L., Pfeiffer, T., et al. Surgical treatment of renal artery dissection in 25 patients: indications and results. Journal of vascular surgery, 2003; 37(4): 761‐768. 33. Lacombe M. Isolated spontaneous dissection of the renal artery. J Vasc Surg, 2001; 33: 385–391. 34. Edwards, B. S., Stanson A. W., Holley K. E., et al. "Isolated renal artery dissection, presentation, evaluation, management, and pathology." In Mayo Clinic proceedings. Mayo Clinic, 1982; 57(9): 564. 35. Reilly, L. M., Cunningham, C. G., Maggisano, R., et al. The role of arterial reconstruction in spontaneous renal artery dissection. Journal of vascular surgery, 1991; 14(4): 468‐479. 36. Afshinnia, F., Sundaram, B., Rao, P., et al. Evaluation of characteristics, associations and clinical course of isolated spontaneous renal artery dissection. Nephrology Dialysis Transplantation, 2013; 37. Oderich, G. S., & Panneton, J. M. Acute aortic dissection with side branch vessel occlusion: open surgical options. Seminars in vascular surgery, 2002; 15(2): 89‐96. 38. Kanofsky JA, Lepor H. Spontaneous renal artery dissection. Rev Urol. 2007; 9(3): 156‐60. 39. van Rooden, C. J., van Baalen, J. M., & van Bockel, J. H. Spontaneous dissection of renal artery: long‐ term results of extracorporeal reconstruction and autotransplantation. Journal of vascular surgery, 2003; 38(1): 116‐122. 40. Lee, S. H., Lee, H. C., Oh, S. J., et al. Percutaneous intervention of spontaneous renal artery dissection complicated with renal infarction: a case report and literature review. Catheterization and cardiovascular interventions, 2003; 60(3): 335‐338. 41. García‐Familiar, A., Ortiz‐Gutiérrez, F., De Blas‐Bravo, M., et al. Isolated Spontaneous Renal Artery Dissection: Endovascular Management. Annals of vascular surgery. 2013; 42. Tandon, G., & Sukhija, R. Isolated Spontaneous Renal Artery Dissection: A Case Report and Review. The International journal of angiology: official publication of the International College of Angiology, Inc, 2012; 21(2): 99. 43. Bilge, A. K., Nisanci, Y., Yilmaz, E., et al. Renovascular hypertension secondary to spontaneous renal artery dissection and treatment with stenting. International journal of clinical practice, 2003; 57(5): 435. 44. Panneton, J. M., Teh, S. H., Cherry Jr, K. J., et al. Aortic fenestration for acute or chronic aortic dissection: an uncommon but effective procedure. Journal of vascular surgery, 2000; 32(4): 711‐721. 45. Fattori, R., Tsai, T. T., Myrmel, T., et al. Complicated acute type B dissection: is surgery still the best option? A report from the International Registry of Acute Aortic Dissection. JACC: Cardiovascular Interventions, 2008; 1(4): 395‐402.
Page 20 of 30 46. Trimarchi, S., Nienaber, C. A., Rampoldi, V., et al. Role and results of surgery in acute type B aortic dissection insights from the International Registry of Acute Aortic Dissection (IRAD). Circulation, 2006; 114(1 suppl): I‐357. 47. Williams, D. M., Lee, D. Y., Hamilton, B. H., et al. The dissected aorta: percutaneous treatment of ischemic complications—principles and results. Journal of vascular and interventional radiology, 1997; 8(4): 605‐625. 48. DiMusto, P. D., Williams, D. M., Patel, H. J., et al. Endovascular management of type B aortic dissections. Journal of vascular surgery, 2010; 52(4): 26S‐36S. 49. Slonim, S. M., Miller, D. C., Mitchell, R. S., et al.. Percutaneous balloon fenestration and stenting for life‐threatening ischemic complications in patients with acute aortic dissection. The Journal of Thoracic and Cardiovascular Surgery, 1999; 117(6): 1118‐1127. 50. Patel, H. J., Williams, D. M., Meekov, M., et al. Long‐term results of percutaneous management of malperfusion in acute type B aortic dissection: implications for thoracic aortic endovascular repair. The Journal of Thoracic and Cardiovascular Surgery, 2009; 138(2): 300‐308. 51. Chavan, A., Rosenthal, H., Luthe, L., et al. Percutaneous interventions for treating ischemic complications of aortic dissection. European radiology, 2009; 19(2): 488‐494. 52. Shiiya, N., Matsuzaki, K. Management of vital organ malperfusion in acute aortic dissection: proposal of a mechanism‐specific approach. General thoracic and cardiovascular surgery, 2007;55(3): 85‐90. 53. Dake, M. D., Kato, N., Mitchell, R. S., et al. Endovascular stent–graft placement for the treatment of acute aortic dissection. New England Journal of Medicine, 1999; 340(20): 1546‐1552. 54. Dake, M. D., & Wang, D. S. Will stent‐graft repair emerge as treatment of choice for acute type B dissection?.Seminars in vascular surgery, 2006; 19(1): 40‐47. 55. Conrad, M. F., Crawford, R. S., Kwolek, C. J., et al. Aortic remodeling after endovascular repair of acute complicated type B aortic dissection. Journal of Vascular Surgery, 2009; 50(3): 510‐517. 56. Szeto, W. Y., McGarvey, M., Pochettino, A., et al. Results of a new surgical paradigm: endovascular repair for acute complicated type B aortic dissection. The Annals of thoracic surgery, 2008; 86(1): 87‐94. 57. White, R. A., Miller, D. C., Criado, F. J., et al. Report on the results of thoracic endovascular aortic repair for acute, complicated, type B aortic dissection at 30 days and 1 year from a multidisciplinary subcommittee of the Society for Vascular Surgery Outcomes Committee. Journal of Vascular Surgery, 2011; 53(4): 1082‐1090. 58. Lombardi, J. V., Cambria, R. P., Nienaber, C. A., et al. Prospective multicenter clinical trial (STABLE) on the endovascular treatment of complicated type B aortic dissection using a composite device design. Journal of Vascular Surgery, 2012; 55(3): 629‐640. 59. Sobocinski, J., Dias, N. V., Berger, L., et al. Endograft Repair of Complicated Acute Type B Aortic Dissections. European Journal of Vascular and Endovascular Surgery, 2013.
Page 21 of 30 60. Ehrlich, M. P., Rousseau, H., Heijmen, R., et al. Midterm results after endovascular treatment of acute, complicated type B aortic dissection: The Talent Thoracic Registry. The Journal of Thoracic and Cardiovascular Surgery, 2013; 145(1): 159‐165. 61. Wilkinson, D. A., Patel, H. J., Williams, D. M., et al. Early Open and Endovascular Thoracic Aortic Repair for Complicated Type B Aortic Dissection. The Annals of thoracic surgery, 2013. 62. Stawicki, S. P., Rosenfeld, J. C., Weger, N., et al. Spontaneous renal artery dissection: three cases and clinical algorithms. Journal of human hypertension, 2006; 20(9): 710‐718.
Legends Figure 1 A,B and C :Spontaneous renal artery dissection in a 39 yo otherwise healthy male. A and B– CT angiogram with poorly perfused left kidney (single arrow) and isolated left renal artery dissection (double arrow); C – Angiogram with evidence of dissection with no renal perfusion (single arrow). Figure 2 A and B: Renal artery malperfusion with aortic dissection. (A) Right renal artery malperfusion with static obstruction (arrow); and (B) Left renal artery malperfusion with both static and dynamic obstruction (arrows). Figure 3 A and B: Complicated type B aortic dissection with compressed true lumen (arrows)on pre op CTA (A) and expanded true lumen (arrows) on a post endograft CTA (B).
Page 22 of 30 Table 2 – Summary of a selected series and reviews of isolated renal artery dissections. Age Sex Bilater Etiology No. of al RAD patients range (No of RADs) 15(16) 3‐75 M=8; 1 Atherosclerosis yrs F=7; =5; FMD =3; Blunt trauma =7; Catheter induced =4; Atherosclerosis 35(41) 22‐58 M=3 6 =1; yrs 2; FMD =22; F=3; Idiopathic =12;
Author
Yea r
Gewertz et al2
197 7
Edwards et al34
198 2
Smith et al16*
198 3
9 (14)
39‐63 yrs
M=7; 5 F=2;
Smith et al16**
198 3
81 (91)
32‐65 yrs
M=5 10 1; F=30;
Beronaide 198 et al3 7
2(4)
37‐40 yrs
M=2; 2 F=0;
Idiopathic =2;
Alamir et al17
199 7
3(3)
36‐51 yrs
M=3; 0 F=0;
Physical exertion =2; FMD =1;
Lacombe3 3
200 1
22 (25)
20‐56 yrs
M=1 7; F=5;
Ramamoo rthy et al29
200 2
4 (5)
44‐58 yrs
M=3; 1 F=1;
3
Catheter induced=2; FMD=1; Spontaneous (idiopathic)=4; Agonal =2; Atherosclerosis =33; FMD =41; Catheter induced =22; Idiopathic =13;
Atherosclerosis =1; FMD =10; Cystic medial necrosis =1; Idiopathic =10; Atherosclerosis =1; FMD =1; Idiopathic =2;
Presentation
Treatment
Medical = 6; Open Surgery (RABP)=3; Nephrectomy (total or partial) =6; Medical = 13; HTN = 27; Open Surgery Abdominal or flank pain= 10; Hematuria (RABP)=3; Nephrectomy = 5; (total or partial) Headache=6 =9; Post mortem = 11; HTN=4; Abdominal or Medical = 2; Open surgical flank pain=3; (RABP)= 10; nausea=1; vomiting =2; hematuria=1; HTN = 12; Abdominal or flank pain=7; Hematuria=7; Nausea=1;
HTN = 81; Abdominal or flank pain= 21; Hematuria = 9; Nausea=8; Vomiting = 5; Asymptomatic =48; Post mortem = 19; Abdominal or flank pain=2; Nausea =2; Vomiting =2; Abdominal or Flank pain=3;
HTN =22; Abdominal or flank pain =17; Hematuria =4;
HTN =4; Abdominal or flank pain =4; Nausea =1; Hematuria =4;
Medical =12; Open surgery (RABP)=21; Nephrectomy (total or partial) =30;
Medical =2;
Medical =1; Open Surgery (nephrectomy) =1; Endovascular = 1; Open Surgery (RABP)=17; Nephrectomy (total or partial) =8; Medical = 4;
Page 23 of 30 200 15(18) van Rooden et 3 al39
3
35‐58 yrs
M=1 0; F=5;
M=1 3 8; F=7; M=3; 1 F=0;
Muller et al32
200 3
25 (30)
20‐56 yrs
Stawicki et al62
200 6
3(4)
37‐61 yrs
Pellerin et 200 al30 9
16 (20)
30‐70 yrs
M=1 3; F=3;
4
Afshinnia et al36
17(19)
26‐51 yrs
M=1 1; F=6;
2
238 (276)
‐
M=1 71; F = 67; M:F = 2.5:1
36 (15%)
201 3
Summary ‐ (%)
Atherosclerosis = HTN = 14; Abdominal or flank 1; pain= 8; FMD =14; Hematuria=5; Nausea=3; Vomiting=2; Headache=6; Blurred or loss of vision=4; Hypertensive encephalopathy=1; Blunt trauma =3; HTN =21; Idiopathic =22; Abdominal or Flank pain =11; FMD =1; HTN = 2; Idiopathic=2; Abdominal or flank pain=3; Hematuria=2; Nausea=1; Vomiting=1; Idiopathic =16; HTN=16; Abdominal or flank pain=9; Headache=4; Acute pulmonary edema=4; Hypertensive encephalopathy=4; FMD = 4; Abdominal or flank Ehlers‐Danlos =4; pain= 14; Hematuria = 2; PAN =1; Headache =1; Idiopathic =8; Asymptomatic =2;
Open Surgery (RABP)=30; Medical = 2; Endovascular (thrombolysis) =1;
Endovascular stent = 17; (3 arteries with
Renal Malperfusion: Spontaneous Renal Dissection and with Aortic Dissection Amit Jain MBBS MS, Margaret C. Tracci MD JD, Dawn Coleman MD, Kenneth J. Cherry MD, Gilbert R. Upchurch Jr MD
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S0895-7967(14)00022-2 http://dx.doi.org/10.1053/j.semvascsurg.2014.06.004 YSVAS50429
To appear in: ĆSemin Vasc Surg
Cite this article as: Amit Jain MBBS MS, Margaret C. Tracci MD JD, Dawn Coleman MD, Kenneth J. Cherry MD, Gilbert R. Upchurch Jr MD, Renal Malperfusion: Spontaneous Renal Dissection and with Aortic Dissection, ĆSemin Vasc Surg , http://dx.doi.org/10.1053/j.semvascsurg.2014.06.004 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
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Renal malperfusion: Spontaneous renal dissection and with aortic dissection. Amit Jain MBBS MS, , Margaret C. Tracci MD JD, Dawn Coleman MD Kenneth J. Cherry MD, Gilbert R Upchurch Jr MD Division of Vascular and Endovascular Surgery University of Virginia Health System Charlottesville – VA Correspondence Address: Gilbert R Upchurch Jr MD PO Box 800679, Charlottesville, VA, 22908 Phone: 434.243.6333 Fax: 434.243.9941 Email: [email protected]
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Renal malperfusion: Spontaneous renal dissection and with aortic dissection. Amit Jain MBBS MS , Margaret C. Tracci MD JD, Dawn Coleman MD ,Kenneth J. Cherry MD, Gilbert R Upchurch Jr MD
Abstract Renal malperfusion associated with renal artery dissection may present as either an isolated disease process or in the setting of branch vessel stenosis complicating aortic dissection. Isolated renal artery dissection is a rare disorder whose clinical presentation often presents both a diagnostic and therapeutic challenge. The true incidence and natural history of this phenomenon also remain unclear. Multiple approaches to management have been described. Medical therapy typically consists of anticoagulation and blood pressure management and is reserved for cases with well controlled symptoms and blood pressure and preserved, stable renal function. Historically, surgical reconstruction with in‐situ or more complex ex‐vivo reconstruction has been described for the treatment of uncontrolled hypertension with preservation of renal perfusion. Nephrectomy, either partial or total, for control of hypertension, is reserved for cases where parenchymal injury necessitates this radical intervention. Recently, endovascular stenting of the renal artery has shown excellent and durable results and is now considered to be the first line intervention for renal artery dissection. Renal malperfusion associated with complicated aortic dissection is a different entity and one, which is consistently an independent predictor of poor prognosis. The pathogenesis of malperfusion can be dynamic, static or a combination. In addition, renal hypoperfusion may occur with or without extension of the intimal flap into the renal artery itself. Traditional open surgical interventions to treat aortic dissection with malperfusion have a very high perioperative mortality rate. Endovascular
Page 3 of 30 fenestration and stenting of both thoracic aortic and branch vessels have significantly improved clinical outcomes in complicated aortic dissections relative to open surgical fenestration. Although a significant body of long term data has yet to be accumulated, endovascular stent grafting has the added advantage over fenestration in that it may affect aortic remodeling, thus preventing the very morbid complication of aneurysmal degeneration.
Introduction and Epidemiology
Renal artery dissection (RAD) is an uncommon diagnosis. It was first reported in 1944 by
Bumpus et al.1 In most cases, RAD is clinically silent and hence its exact incidence is unknown. A small number of cases reported likely represent a more common, but likely underdiagnosed entity. With the increasing use of noninvasive imaging modalities and advanced endovascular interventions more renal artery dissections are being diagnosed. Isolated and spontaneous renal artery dissection was reported early in a number of small case series with a reported incidence of about 0.036 to 0.049% of all arterial dissections.2‐4 RAD can cause varying degree of renal parenchymal loss and hypertension. RAD, when symptomatic, typically presents as acute abdominal or flank pain. It may also present with hematuria mimicking renal colic secondary to renal stone, thus creating a diagnostic dilemma. Chronic RAD, on the other hand, is often diagnosed on the workup of abrupt onset of severe hypertension in a young adult that often requires multiple drug treatment. Aortic dissection can also cause renal artery malperfusion. About 25‐30% of the individuals with aortic dissections have some form of vascular insufficiency involving the visceral, renal, spinal, cerebral or limb.5‐7 Renal ischemia has been reported in approximately 8% of these cases.6,8,9 Renal malperfusion is an independent predictor of in‐hospital mortality in patients with aortic dissections.8, 10‐13 Hence, the identification and treatment of renal malperfusion with or without renal artery dissection is of paramount significance in patients with complicated aortic dissections.
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Etiology, Pathogenesis and Classification The etiology of isolated RAD is not clearly understood. Arterial dissection is caused by either bleeding in between the layers of the arterial wall via a primary intimal tear or hemorrhage from the vasa vasorum. Though the entity of RAD is relatively rare, renal arteries are one of the more common sites of primary dissection involving peripheral vessels and may be associated with atherosclerosis, fibro‐muscular dysplasia (FMD), Ehlers‐danlos syndrome, Marfan’ syndrome, malignant hypertension, blunt trauma and severe strenuous physical activities. 14‐20 Based on their presentation, RADs can be broadly categorized into two clinical categories, acute or chronic. RADs may also be classified based on their etiology (Table 1). Isolated RADs are localized to renal arteries. Primary or spontaneous RADs are usually associated with renal artery atherosclerosis or FMD.16 Sometimes, no underlying pathology can be identified and therefore classified as idiopathic (Figure 1). Spontaneous RADs in an otherwise healthy individuals have been reported with excessive physical exertion possibly playing a role in its causation.16‐19 Secondary RADs can be defined as those due to either blunt abdominal trauma or as a consequence of catheter and wire manipulation during an endovascular intervention. While renal artery thrombosis is the most frequent renal vascular pathology in blunt trauma, renal artery intimal disruption has also been reported.21‐24 Renal artery dissections are not uncommon after endovascular renal arterial interventions and may occur in 5‐24% individuals. 2,25, 26 Many of RAD associated with endovascular renal interventions may not be reported as they are treated with stent placement during the course of the procedure. Aortic dissection can cause renal malperfusion with or without associated renal artery dissection. The aortic flap may cause either a static or dynamic obstruction of renal artery blood flow depending on the configuration of the flap and its movement during the cardiac cycle.27 Static renal malperfusion is present throughout the cycle and is typically caused by the aortic dissection involving
Page 5 of 30 the renal artery ostium. The aortic wall hematoma may propagate into the renal artery, compromising the vessel lumen. Dynamic renal malperfusion varies with the pulsatile flow of blood in the aorta and is generally the result of cyclic prolapse of the dissection flap into the renal artery origin. Occasionally the obstruction may represent combination of varying degrees of both static and dynamic components.27,28 (Figure 2) Table 1: Classification of renal artery dissection.
Classification of renal artery dissection 1. Isolated RAD a. Primary – Spontaneous RAD i. Atherosclerosis ii. Fibromuscular Dysplasia (FMD) iii. Other connective tissue disorders (CTDs) iv. Idiopathic b. Secondary – i. Trauma ii. Iatrogenic Interventional procedures 2. Combined RAD with AD a. Static obstruction b. Dynamic obstruction c. Combined
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Presentation 1. Renal malperfusion with isolated renal artery dissection: Renal artery dissections are rare and often clinically silent. They usually affect young individuals in their third, fourth or fifth decade of life. Men are affected more than women (2.5:1).3,16,29 When clinically overt, they present acutely with various combinations of abdominal or flank pain, nausea, vomiting, headache, dysuria or hematuria (Table 2).2‐4,15,16 Spontaneous RAD can present with the sudden onset of malignant hypertension which may even be complicated by hypertensive encephalopathy.30,31 Abrupt onset of severe hypertension requiring three or four antihypertensive drugs was seen in majority of the patients requiring operative reconstruction of RADs.32,33 Hypertension may also be accompanied by varying degrees of renal dysfunction, up to and including frank renal failure.20,32‐ 35
Bilateral renal artery involvement is present in 12‐25% cases and is strongly associated with FMD as
the underlying pathology.16,32,33 The primary dissection event may be silent or associated with vague abdominal pain that goes undiagnosed until it presents in a delayed fashion as uncontrolled hypertension. Although RAD is typically isolated, it must be recognized that abdominal pain may also represent a rare simultaneous visceral arterial dissection.33 In a recent review of the clinical characteristics of the patients with SRADs, Afshinnia et al studied 17 patients who presented over a 12 years period.36 Of these, 11 (65%) were male and 14 (82%) white. Patients with SRAD were more likely to have higher mean serum creatinine, systolic and diastolic blood pressure and a lower body mass index at presentation. FMD was present in 4 patients, Ehlers‐ Danlos in 4 and polyarteritis nodosa was seen in 1 patient. The most common presenting symptom was pain (flank pain in 10 and abdominal pain in 2 patients).
Page 7 of 30 2. Renal malperfusion with aortic dissection with or without RAD:
Clinical suspicion for renal artery malperfusion in individuals with aortic dissection is based on
worsening hypertension and / or evolving renal insufficiency manifested by oliguria, anuria or a rise in serum BUN and creatinine. The renal circulation (8%) is the second most commonly malperfused arterial bed in aortic dissection, following the iliofemoral (11%) system.6,37
Investigation and Diagnosis 1. Renal malperfusion with isolated renal artery dissection: Symptoms of isolated spontaneous RADs often mimic other, more common etiologies of acute abdominal or flank pain and are often misdiagnosed. Reported misdiagnoses have included renal colic and even appendicitis, resulting in emergency appendectomy.33 A high index of suspicion in patients with abdominal or flank pain along with rising serum creatinine and uncontrolled hypertension can guide appropriate investigations. Measurement of serum LDH in the acute phase in addition to serum BUN and creatinine may indicate renal parenchymal loss.32 Duplex scanning to measure main renal artery flow velocity and renal intraparenchymal resistance indices provides additional data regarding renal perfusion and may even permit visualization of dissection. The kidney size can be evaluated by B mode ultrasound imaging. If renal function is not severely deranged, cross sectional imaging with CTA or MRA provides excellent resolution diagnostic imaging. Although CTA and MRA offer high sensitivity and specificity in detecting renal artery stenosis, digital subtraction angiography has been used widely and some authors continue to prefer this modality to evaluate extent of dissection and intimal flap morphology and to plan treatment strategy. 30,32,33,38 Muller et al32 have also suggested use of isotope renography to evaluate bilateral renal function and renal vein renin measurement to be helpful in selected cases. 2. Renal malperfusion with aortic dissection with or without RAD:
Page 8 of 30 CTA is the most widely used diagnostic modality for aortic dissection and is able to demonstrate renal hypoperfusion, where present, in most cases of AD with or without RAD. Evidence of renal malperfusion may include renal parenchymal infarction, differential contrast density nephrograms and visualization of an occlusive intimal flap. Barnes et al28 used contrast angiography to study 165 patients with clinically suspected malperfusion and confirmed true renal malperfusion by establishing the presence of a systolic gradient between aortic root and renal hilum of >10 mm Hg, failure of the renal artery to fill during contrast injection in the true and false lumen of the aorta, or intravascular ultrasound evidence of a “curtain‐like” occlusion of the renal ostium or the true lumen above its origin. Using these criteria, they diagnosed true renal artery malperfusion in 67% (n=59/88) of the patients clinically suspected to have renal artery malperfusion. Interestingly, they also diagnosed renal artery malperfusion in 39% (n=31/79) of those patients with suspected malperfusion of non‐renal tissues but without an initial clinical suspicion of renal hypoperfusion.
Treatment Options and Outcomes 1. Renal malperfusion with isolated renal artery dissection: The treatment options described for isolated spontaneous renal artery dissections (ISRAD) include medical management, open surgical reconstruction or endovascular intervention. Optimal management depends on the severity of the patient’s clinical condition, degree of renal infarction and extent of the renal artery dissection. In general, treatment seeks to preserve renal function and treat renovascular hypertension. Medical therapy. Ramamoorthy et al described successful non‐operative management of four patients with ISRAD.29 All of them were managed expectantly with anticoagulation. Blood pressure was easily controlled and renal function was stable in all 4 patients over a mean follow up of 14 month. However, one of the patients with FMD did develop a contralateral renal artery dissection. Edwards et
Page 9 of 30 al34 compared 13 patients treated medically with 11 patients who underwent surgery for ISRAD. They concluded that medical treatment with antihypertensive medications was equally effective. Afshinnia et al36 reported treating 8 patients out of 17 in their series of ISRAD, with medical management which consisted of pain control, control of hypertension and systemic anticoagulation. Others have also documented successful non‐operative management of ISRAD with or without anticoagulation.3,16,17,19 Medical management seems appropriate when renal function is stable and patient symptoms and hypertension can be controlled with medication. Open surgical therapy. Surgical treatment for the SRAD have been reported in several retrospective case series and, again, is aimed at preserving renal function and treating renovascular hypertension.16,20 32‐35 Smith et al16 described 10 renal artery bypass procedures in 9 patients with 100% immediate patency and maintenance or improvement in renal function. Five of these were ex‐vivo complex reconstructions due to renal branch vessel involvement. None needed nephrectomy. Lacombe33 treated 22 patients with 17 arterial bypasses and 8 nephrectomies (6 total, and 2 partial). Three patients had bilateral lesions. There were no post‐operative deaths. Arterial hypertension was cured in 9 patients (41%), improved in 11 (50), and unchanged in 2 (9%). On long term follow up (mean 10.1 years), one late thrombosis of a repaired polar artery and one spontaneous dissection of contralateral renal artery was seen. Muller et al32 described surgical treatment of RAD in a series of 25 patients with 22 SRADs and 3 with trauma with all but one reconstruction in situ. Hypertension resolved or improved in 86% of patients without preoperative renal damage and in only 38% of those with preoperative damaged kidneys. Renal function was preserved in 23 of 28 revascularized kidneys (82%) at a mean follow up of 55.3 months. The efficacy of extracorporeal reconstruction with autotransplantation of 19 kidneys was attempted in 15 patients by van Rooden et al.39 With a technical success rate of 94%, a favorable outcome was demonstrated in 79% of patients for blood pressure control and all the patients had normal renal functions over a 1 to 8 year follow up. There were no operative deaths and one primary nephrectomy was performed. In all of the above mentioned case series, surgical treatment was indicated for uncontrolled hypertension, persistent symptoms and / or
Page 10 of 30 deteriorating renal function. Both in‐situ and ex‐vivo repair are feasible and safe. Besides the preference of the surgeon, ex‐vivo repairs are used for complex reconstructions of RAD involving the branch vessels. Nephrectomy, either partial or total should be considered for severely damaged kidneys to prevent renovascular hypertension. Endovascular Therapy. Endovascular interventions have been also described for the treatment of both primary, spontaneous RADs, as well as secondary RADs either due to blunt trauma or iatrogenic endovascular interventions.21,30,36,38, 40‐43 Bilge et al43 and Lee et al40 each reported the earliest cases of SRAD treated by transcatheter intervention with adjunct stent placement. Pellerin et al30 subsequently described 16 patients with 17 SRAD treated with endovascular stent placement. In this later series all patients had uncontrolled hypertension and 10 patients had progressive renal insufficiency. With a 100% technical success, they reported clinical success in treating all the patients’ elevated blood pressure. More than 40% of them were cured and remained normotensive without taking any antihypertensive medications during a follow up of more than 8 years. All the patients had normal renal function and imaging of the renal arteries showed no sign of restenosis or occlusion in all patients. Three other recent case reports describe successful placement of renal artery stent for SRAD.38, 41,42 Afshinnia et al36 reported 17 cases of SRADs treated with supportive medical care, endovascular treatment and surgery for 8, 5 and 4 cases, respectively. However, of the 5 endovascular cases, thrombolysis and subsequent anticoagulation was required in one patient for acute thrombotic occlusion of the renal artery. The remaining 4 patients had Ehlers‐Danlos syndrome with leaking, dissecting renal artery aneurysms and were treated with endovascular coil embolization with immediate stabilization. Lee et al21 report a case of successful endovascular stent placement for a renal artery dissection secondary to blunt abdominal trauma. Bush et al25 treated 85 renal artery stenoses with 88 Palmaz stents. Although most stents were implanted for suboptimal balloon dilatation (52%), 24% were placed for secondary dissections caused by the balloon angioplasty. Thus, endovascular interventions for isolated RAD are now well established and safe in the setting of both primary and secondary etiologies.
Page 11 of 30 2. Renal malperfusion with aortic dissection with or without RAD: Aortic dissections complicated by renal artery malperfusion with or without RAD are associated with significantly increased mortality.8, 10‐13 In complicated aortic dissections, both open and endovascular approaches aim to restore flow to all the malperfused vascular beds, including the renal arteries. Open surgical therapy. Open procedures may involve aortic graft interposition, excision of the intimal flap and creation of an open fenestration, or performing aorta to branch vessel bypass to restore the blood flow to the malperfused organs. In multiple large series, open surgical treatment for complicated aortic dissection with visceral malperfusion have extremely high mortality rates (6‐ 69%).6,8,9,12,13 Lauterbach et al6 reported 187 patients with aortic dissection (101 proximal and 86 distal) with 53 patients (28%) having clinical evidence of organ or limb malperfusion. Of these, 12 patients had renal malperfusion. One third (17 patients) of those with malperfusion required specific intervention to treat the malperfusion. Twelve open procedures and 5 endovascular procedures (including 3 aortic fenestration and 2 renal stents) were performed. The in‐hospital mortality for the entire group was 18%. Panneton et al44 described open aortic fenestration for 14 patients with complicated aortic dissection (3 type A, 11 type B). Emergent fenestration was performed in 7 patients, 5 of them had renal malperfusion, 3 bowel and 4 lower extremity ischemia. Operative mortality was 43% (3/7) in the emergent group and there were no postoperative deaths in the elective group. The IRAD investigators reported open surgical repairs of complicated type B aortic dissection between 2006 and 2008 with an in‐hospital mortality of 29% and 34% for 82 and 59 open surgical interventions, respectively.45,46 These mortality rates were significantly higher compared to patients treated with endovascular therapy in the same database (11% in 2006 and 11% in 2008), as well as who were managed medically (10% in 2006 and 8.7% in 2008) during the same study period. The in‐hospital complication rate was 40% in those undergoing open surgical treatment compared to 21% in the endovascular group.46
Page 12 of 30 Endovascular aortic fenestration and stent therapy. Advances in endovascular techniques have enhanced the safety and efficacy of minimally invasive percutaneous endovascular interventions in the treatment of ischemic complications of aortic dissection. Endovascular aortic fenestration with or without branch vessel stenting was proposed and widely studied in late 1990s.47 Endovascular fenestration and stenting are technically challenging, requiring an advanced level of operator endovascular skill, a well‐equipped intervention suite, and experienced staff. The procedure involves extensive angiographic and IVUS evaluation of the anatomy of the dissected aorta, the intimal flap, and branch vessels and as well as the physiology of the dissection, including measurement of intravascular pressure gradients between the true and false lumens and the aortic branches. True malperfusion is confirmed by a systolic gradient between the aorta and the superior mesenteric artery or renal hilum of >15 mm Hg, failure of a branch artery to fill during injection of contrast in the true and false lumen of the aorta, evidence of a “curtain‐like” occlusion of the vessel origin or of the true lumen above the origin by IVUS, thrombosis, or evidence of embolization.47,48 The aortic intimal flap, once identified as causing the either a static or dynamic obstruction, is punctured with a trocar needle and the tear in the flap is dilated with a balloon. A persistent true lumen collapse or an unresolved pressure gradient can be further treated by placing a large diameter self‐expanding stent to open the aortic true lumen. Care should be taken to avoid covering the origins of renal and superior mesenteric arteries and or across fenestrations essential to branch vessel perfusion.48 Further branch vessel static obstructions should be treated by stent placement origin of these vessels. Williams et al47 treated 24 patients with aortic dissection complicated by ischemia of liver or bowel (n=15), kidney (n=18) or lower extremity (n=13). Treatment consisted of vascular stents alone (n=4), or balloon fenestration (n=20) without (n=8) or with (n=12) vascular stents. With a 30 day mortality of 25% (6 patients), there was a 92% success in restoring flow in the obstructed vessels (71 of 77 obstructed arteries). Slonim et al49 reported 40 patients with aortic dissection (10 type A and 30 type B) with peripheral ischemic complications. Thirty patients had renal, 22 had limb, 18 had mesenteric and 1 had upper arm ischemia. Fourteen patients were treated with combined stenting of true or false
Page 13 of 30 lumen with balloon fenestration of the flap, 24 with stenting alone and 2 with fenestration alone. Successful revascularization was achieved in 37 of 40 patients (93%) with a 25% (10 out of 40 patients) 30‐day mortality. The mortality was often related to ischemia at presentation. In one of the largest series on renal malperfusion Barnes et al28 reported a single center experience in treating renal malperfusion after aortic dissection with aortic fenestration and renal artery stenting. They included all patients with aortic dissection from 1996 to 2004 in whom there was sufficient clinical suspicion for peripheral malperfusion to require arteriography. The group comprised 165 patients (115 acute and 50 chronic), of which 75 had type A and 90 had type B dissection. Renal malperfusion was suspected in 88 patients secondary to worsening hypertension (n=34), renal insufficiency (n=37), CT evidence of renal ischemia (n=13) or a combination of above factors (n=4). Renal malperfusion, confirmed with a systolic gradient between the aortic root and renal hilum, was present in 59 of the suspected 88 patients (67%). However, renal malperfusion was also detected in 31 of 79 patients (39%) with suspected malperfusion of non‐renal tissue. Renal arteries were perfused by true lumen (70% right, 42% left), false lumen (7% right, 20% left), or both true and false lumen (23% right, 38% left). Of the 90 patients with confirmed renal malperfusion, 71 had endovascular treatment, including isolated renal artery stenting (n=31), proximal fenestration with or without stenting (n=24), or both renal and aortic intervention (n=16). Post‐intervention the pressure gradient across the aortic root and renal hilum decreased from an average of 44 mm Hg to 8.1 mm Hg. The peri‐procedure post‐ intervention mortality was 21% (n=15). In a separate report on 69 patients with complicated type B aortic dissection, Patel et al50 identified 185 malperfused vascular beds in 70% of patients, including spinal cord (5), mesenteric (40), renal (51), and lower extremity (47). Using aortic fenestration and true lumen or branch vessel stents, flow was restored in 96% of these vascular beds. Stroke and paraplegia rates were 4.3% and 2.9% and early mortality rate was 17%. Chavan et al51 described their experience of treating 45 patients with endovascular fenestration and stenting for complicated aortic dissection (13 type A and 32 type B). Out of total 88 vascular beds, 25 involved renal arteries, 22 mesenteric and 33 lower extremity vessels. Seven of 25 patients with renal ischemia required dialysis, but all of the patients
Page 14 of 30 were dialysis free 3 months following the intervention. The 30‐day mortality was 6.7%. Shiiya et al52 have reported on organ malperfusion in 38 of 135 patients with aortic dissection (31 type A). They emphasize a mechanism‐specific approach to treatment, noting the inability of a central aortic operation alone to adequately address renal branch malperfusion (15% success with 2 of 13 patients). However, a percutaneous endovascular approach was 100% successful in all vessels with branch type malperfusion. Endovascular stent graft therapy. Besides aortic fenestration and branch vessel stenting, endovascular graft placement to cover the site of aortic intimal tear has been shown to redirect flow into the true lumen and, in many cases, reverse organ malperfusion.53,54 During the last decade, the use of endovascular stent graft to treat complicated aortic dissection has been widely accepted with favorable results (Figure 3). Dake et al53 described 19 patients treated with stent grafts for aortic dissection (4 type A and 15 type B). Of these, 37% patients had symptomatic malperfusion. They reported a 76% improvement in malperfusion with a 16% 30‐day mortality rate. Thoracic stent graft placement resolved the obstruction in all of the 22 vessels with dynamic obstruction, but only 6 of the 15 vessels with combined static and dynamic obstruction. The benefit of aortic stent grafting in dynamic obstruction is clear, however there may be limitations in its ability to address branch vessel dissection or static obstruction of other etiologies.28 Since this report, several authors have published their experience of treating aortic dissection with visceral malperfusion syndrome with additional use of bare metal stents at the origin of visceral, renal and iliac vessels as necessary. Conrad et al55 reported treating 33 patients with thoracic endograft for complicated acute type B aortic dissection. Seventeen of these patients (53%) had malperfusion syndrome. All were repaired successfully with a 12% 30‐day mortality rate. Szeto et al56 examined treatment of acute complicated type B dissection in 35 patients, 17 of which had malperfusion. Technical success was achieved in 97% (34) of patients with 34% (12) of patients requiring additional procedures including bare metal stents in the infrarenal aorta, renal artery, celiac artery or iliac artery. One patient had permanent paraplegia (2.8%), one had stroke (2.8%) and 30‐ day mortality was 2.8%. White et al57 reported results of thoracic endovascular aneurysm repair (TEVAR)
Page 15 of 30 for complicated type B aortic dissection at 30 days and 1 year. They had 99 patients, 85 acute (symptom onset ≤ 14 days), 11 subacute (15‐30 days) and 3 chronic (31‐90 days). Of the acute 32 % had rupture and 72% had malperfusion, including 56% lower extremity, 36% renal, 19.7% visceral, 8.2% other and 3% spinal cord. Additional adjunctive procedures were necessary in 4.7% of the patients, including a carotid artery covered stent in one patient and bare metal stent in the true lumen of the right renal, left renal and left iliac arteries in three patients. Major adverse events at 30‐days were 10.6% death, 9.4% stroke, 9.4% renal failure and 9.4% paralysis / paraparesis. Actuarial mortality estimate at 1 year was 29.4. Lombardi et al58 described 40 patients with complicated type B aortic dissection treated with a composite endograft in the STABLE trial. Branch vessel malperfusion was present in 67.5% (27/40) patients, including mesenteric (5 of 27), renal (17 of 27), spinal cord (1 of 27) and limbs (15 of 27). All 40 patients received one or more endovascular stent grafts and 39 patients received at least one bare metal aortic stent. During the initial procedure, adjunctive stent placement was performed in 9 patients for 13 branch vessels (6 iliac arteries, 6 renal arteries, and one superior mesenteric artery). The 30‐day mortality rate was 5% (2 of 40), stroke (7.5%), transient ischemic attack (2.5%), paraplegia (2.5%), retrograde progression of dissection (5%), and renal failure (12.5%). None of the patients with renal failure became dialysis‐dependent. Many more authors have reported successful treatment of complicated aortic dissections with visceral malperfusion, including renal vessels, with endovascular stent graft with or without adjunctive stent placement in the branch vessels.59,60 A recently published report by Wilkinson et al61 analyzed 73 patients with type B dissection who underwent either open repair (n=24) or TEVAR(n=49) for malperfusion (n = 8), rupture (n = 22), or factors portending rupture, including rapid expansion (n = 26), uncontrolled pain (n = 18), aortic size greater than 5.0 cm (n = 26), or refractory hypertension (n = 2). The early and late mortality were similar between groups (16.7% for open and 10.2% for TEVAR, p=0.46). Ten‐year Kaplan‐Meier survival was 57.5% and similar between groups (p = 0.74). Comparing their results with other contemporary series they concluded that an early mortality benefit with TEVAR for complicated type B dissection (not seen in their study) was more when the intervention was performed for malperfusion than for rupture. They
Page 16 of 30 had a small number of patients with malperfusion in their study. They also concluded that TEVAR provided similar midterm treatment efficacy and late survival compared to open surgical repair of descending thoracic aorta.
Summary The management of renal malperfusion associated with both isolated RAD and aortic dissection has significantly evolved over time. Although successful medical management of RAD has been described in multiple case reports and small case series, it should be considered only in patients with stable renal function and well controlled hypertension. Aggressive attempts at revascularization of the renal arteries should be made whenever there is evidence of deteriorating renal function and/or in the presence of uncontrolled hypertension. Open surgical reconstruction of the renal arteries, both in‐situ and ex‐vivo has been performed with excellent results at experienced centers. However, endovascular therapy has proven effective, with limited morbidity, and is preferred modality of treatment where anatomically suitable. In the treatment of renal malperfusion with aortic dissection, an endovascular approach should be the first line of therapy as well, except in the unstable patient with a type A dissection who needs emergent open cardiac surgery. Both endovascular fenestration with stenting (“fen‐stent”) and endovascular stent grafting have been shown to be successful. DiMusto et al48 analyzed in detail the benefits and limitations of fen‐sten versus aortic endografts. In summary, fen‐sten is appropriate for treating malperfusion only, and is technically very demanding and time consuming. Endografting on the other hand, has broader indications in aortic dissections, including impending rupture, unstable or leaking false lumen, as well as malperfusion. However, the efficacy of endografts in treating distal branch type malperfusion may be limited. Fenestration has narrow clinical indications, but broad anatomical applicability, whereas thoracic stent graft has broad clinical indications, but perhaps
Page 17 of 30 narrower anatomical applicability.48 However, this limitation of endovascular stent grafts used alone can be overcome by adjunctive stenting of malperfused vascular beds, as necessary, at the time of stent graft deployment. The long term data on the durability of the stent graft is still evolving, but appears promising.
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Page 21 of 30 60. Ehrlich, M. P., Rousseau, H., Heijmen, R., et al. Midterm results after endovascular treatment of acute, complicated type B aortic dissection: The Talent Thoracic Registry. The Journal of Thoracic and Cardiovascular Surgery, 2013; 145(1): 159‐165. 61. Wilkinson, D. A., Patel, H. J., Williams, D. M., et al. Early Open and Endovascular Thoracic Aortic Repair for Complicated Type B Aortic Dissection. The Annals of thoracic surgery, 2013. 62. Stawicki, S. P., Rosenfeld, J. C., Weger, N., et al. Spontaneous renal artery dissection: three cases and clinical algorithms. Journal of human hypertension, 2006; 20(9): 710‐718.
Legends Figure 1 A,B and C :Spontaneous renal artery dissection in a 39 yo otherwise healthy male. A and B– CT angiogram with poorly perfused left kidney (single arrow) and isolated left renal artery dissection (double arrow); C – Angiogram with evidence of dissection with no renal perfusion (single arrow). Figure 2 A and B: Renal artery malperfusion with aortic dissection. (A) Right renal artery malperfusion with static obstruction (arrow); and (B) Left renal artery malperfusion with both static and dynamic obstruction (arrows). Figure 3 A and B: Complicated type B aortic dissection with compressed true lumen (arrows)on pre op CTA (A) and expanded true lumen (arrows) on a post endograft CTA (B).
Page 22 of 30 Table 2 – Summary of a selected series and reviews of isolated renal artery dissections. Age Sex Bilater Etiology No. of al RAD patients range (No of RADs) 15(16) 3‐75 M=8; 1 Atherosclerosis yrs F=7; =5; FMD =3; Blunt trauma =7; Catheter induced =4; Atherosclerosis 35(41) 22‐58 M=3 6 =1; yrs 2; FMD =22; F=3; Idiopathic =12;
Author
Yea r
Gewertz et al2
197 7
Edwards et al34
198 2
Smith et al16*
198 3
9 (14)
39‐63 yrs
M=7; 5 F=2;
Smith et al16**
198 3
81 (91)
32‐65 yrs
M=5 10 1; F=30;
Beronaide 198 et al3 7
2(4)
37‐40 yrs
M=2; 2 F=0;
Idiopathic =2;
Alamir et al17
199 7
3(3)
36‐51 yrs
M=3; 0 F=0;
Physical exertion =2; FMD =1;
Lacombe3 3
200 1
22 (25)
20‐56 yrs
M=1 7; F=5;
Ramamoo rthy et al29
200 2
4 (5)
44‐58 yrs
M=3; 1 F=1;
3
Catheter induced=2; FMD=1; Spontaneous (idiopathic)=4; Agonal =2; Atherosclerosis =33; FMD =41; Catheter induced =22; Idiopathic =13;
Atherosclerosis =1; FMD =10; Cystic medial necrosis =1; Idiopathic =10; Atherosclerosis =1; FMD =1; Idiopathic =2;
Presentation
Treatment
Medical = 6; Open Surgery (RABP)=3; Nephrectomy (total or partial) =6; Medical = 13; HTN = 27; Open Surgery Abdominal or flank pain= 10; Hematuria (RABP)=3; Nephrectomy = 5; (total or partial) Headache=6 =9; Post mortem = 11; HTN=4; Abdominal or Medical = 2; Open surgical flank pain=3; (RABP)= 10; nausea=1; vomiting =2; hematuria=1; HTN = 12; Abdominal or flank pain=7; Hematuria=7; Nausea=1;
HTN = 81; Abdominal or flank pain= 21; Hematuria = 9; Nausea=8; Vomiting = 5; Asymptomatic =48; Post mortem = 19; Abdominal or flank pain=2; Nausea =2; Vomiting =2; Abdominal or Flank pain=3;
HTN =22; Abdominal or flank pain =17; Hematuria =4;
HTN =4; Abdominal or flank pain =4; Nausea =1; Hematuria =4;
Medical =12; Open surgery (RABP)=21; Nephrectomy (total or partial) =30;
Medical =2;
Medical =1; Open Surgery (nephrectomy) =1; Endovascular = 1; Open Surgery (RABP)=17; Nephrectomy (total or partial) =8; Medical = 4;
Page 23 of 30 200 15(18) van Rooden et 3 al39
3
35‐58 yrs
M=1 0; F=5;
M=1 3 8; F=7; M=3; 1 F=0;
Muller et al32
200 3
25 (30)
20‐56 yrs
Stawicki et al62
200 6
3(4)
37‐61 yrs
Pellerin et 200 al30 9
16 (20)
30‐70 yrs
M=1 3; F=3;
4
Afshinnia et al36
17(19)
26‐51 yrs
M=1 1; F=6;
2
238 (276)
‐
M=1 71; F = 67; M:F = 2.5:1
36 (15%)
201 3
Summary ‐ (%)
Atherosclerosis = HTN = 14; Abdominal or flank 1; pain= 8; FMD =14; Hematuria=5; Nausea=3; Vomiting=2; Headache=6; Blurred or loss of vision=4; Hypertensive encephalopathy=1; Blunt trauma =3; HTN =21; Idiopathic =22; Abdominal or Flank pain =11; FMD =1; HTN = 2; Idiopathic=2; Abdominal or flank pain=3; Hematuria=2; Nausea=1; Vomiting=1; Idiopathic =16; HTN=16; Abdominal or flank pain=9; Headache=4; Acute pulmonary edema=4; Hypertensive encephalopathy=4; FMD = 4; Abdominal or flank Ehlers‐Danlos =4; pain= 14; Hematuria = 2; PAN =1; Headache =1; Idiopathic =8; Asymptomatic =2;
Open Surgery (RABP)=30; Medical = 2; Endovascular (thrombolysis) =1;
Endovascular stent = 17; (3 arteries with
