|Year : 2015 | Volume
| Issue : 2 | Page : 60-65
Aortic Surgery for Patients with Connective Tissue Disorders
Michol A Cooper, James H Black
Associate Professor of Surgery, Chief of Vascular Surgery and Endovascular Therapy, Johns Hopkins Hospital and Johns Hopkins Medical Institutions, Halsted 668, 600 North Wolfe Street, Baltimore, Maryland 21287, USA
|Date of Web Publication||31-Jul-2015|
James H Black
Associate Professor of Surgery, Chief of Vascular Surgery and Endovascular Therapy, Johns Hopkins Hospital and Johns Hopkins Medical Institutions, Halsted 668, 600 North Wolfe Street, Baltimore, Maryland 21287
Source of Support: None, Conflict of Interest: None
Patients with connective tissue disorders have benefitted from refinements in surgical technique and progress in molecular biology research. As many patients with connective tissue disorders now enjoy a longer life expectancy, non aortic root manifestations of their conditions are becoming more commonplace and vascular surgeons are tooled to address them. In this review, we will elucidate the triage and diagnosis of patients with connective tissue disorders and advance practical treatment strategies for these challenging vascular surgery patients.
Keywords: Aortic surgery, connective tissue disorder, open aortic surgery
|How to cite this article:|
Cooper MA, Black JH. Aortic Surgery for Patients with Connective Tissue Disorders. Indian J Vasc Endovasc Surg 2015;2:60-5
| Introduction|| |
The management of patients with connective tissue disorder encompasses medical and surgical therapies. As a true example of "bench to bedside" translation, advances in research have allowed genetics experts, surgeons, and interventionalists to employ a variety of therapeutic solutions. In the era of genetic medicine, it is likely we will gain a better understanding of how individual genotypes confer the vascular phenotype and condition that the surgeon faces. In this article, we will review the current assessment and therapies for patients with vascular manifestations of an underlying connective tissue disorder - those which afflict the collagen and elastin building blocks of the human body.
| Aortic Homeostasis|| |
The cause of aortic aneurysm formation and aortic dissection is medial degeneration with disruption and loss of elastic fibers and deposition of proteoglycans.  Loss of elastic fibers is at least partially mediated by increased matrix metalloproteinases (MMPs), particularly MMP-2 and MMP-9, which have been found in the media of thoracic aortic aneurysms in patients with and without connective tissue disease (CTD). ,, Research into CTD has provided new insights into aneurysm formation that may have relevance to nonsyndromic patients. FBN1 mutations in Marfan syndrome have been shown to affect the aorta beyond the aortic root. Behavior of collagen alpha-1 (III) (COL3A1) mutations in vascular Ehlers-Danlos syndrome (EDS) is strongly influenced by genotype and more than 700 COL3A1 mutations have been identified.  Newly discovered genetic mutation subtypes in transforming growth factor (TGF) in Loeys-Dietz syndrome (LDS) may significantly affect the natural history of the disease.  Furthermore, we appreciate significant genetic heterogeneity in familial thoracic aortic aneurysm and dissection syndrome (FTAADS) leading to variable expression and decreased penetrance in women.  Aortic involvement in CTD may vary significantly based on both the specific genetic mutation and its clinical manifestations [Figure 1], which is why an individualized approach to patients with CTD is very important. 
|Figure 1: Arterial involvement in vascular Ehlers-Danlos syndrome patients by collagen alpha-1 (III) mutations. Collagen alpha-1 (III) mutations lead to a frameshift due to a glycine substitution. This destroys the three-dimensional architecture of the collagen III trimer leading to a total collagen III mass of <10% of normal. In haploinsufficient mutations, there is an early stop in transcription leading to improved collagen III production and an overall collagen III mass of 50% of normal. Although vascular Ehlers-Danlos syndrome involves medium-sized arteries more commonly, there is an increased prevalence of aortic involvement in the haploinsufficient cohort compared with the MIN cohort (P = 0.025) (reproduced with permission) |
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| Patient Selection|| |
An echocardiogram should be performed to determine the aortic root and ascending aortic diameters at the time of diagnosis of Marfan syndrome and at 6 months to determine the rate of enlargement. If the aortic diameter remains stable, annual imaging is recommended.  Surgical repair of the aortic root is recommended at 5 cm or with growth of >0.5 cm/year.  Repair can also be considered if the maximal cross-sectional area in square centimeters of the ascending aorta or root divided by the patient's height in meters exceeds 10 because shorter patients tend to dissect at smaller sizes and 15% of Marfan patients have dissections at <5 cm. , In addition, for women with Marfan syndrome contemplating pregnancy, prophylactic replacement of the aortic root and ascending aorta should be considered if the diameter exceeds 4 cm. 
All patients with LDS should have yearly echocardiography, strict blood pressure control, and exercise restrictions including avoidance of contact sports and exercising to the point of exhaustion. For blood pressure control, beta-blockade has been the traditional standard of care in aneurysmal disease.  However, in LDS patients, angiotensin-converting enzyme inhibitors may also be particularly useful due to their effects on the TGF-signaling cascade and should also be utilized. Congenital heart disease including bicuspid aortic valve, atrial septal defect, and patent ductus arteriosus, as well as mitral valve prolapse and insufficiency are more common in this patient population than the general population and should be managed per typical protocols. 
The decision to pursue aortic surgery is multi-factorial and based on genotype, absolute dimension of the aorta, rate of progression, valve function, severity of noncardiac features, and family history. , For adults, surgical repair is recommended if the aortic root reaches 4 cm or is rapidly expanding (>0.5 cm over 1-year), if the descending thoracic aorta reaches 4.5 cm or is rapidly expanding (>1 cm over 1-year) or if the abdominal aorta reaches 4 cm or is rapidly expanding (>1 cm over 1-year). For children with LDS, especially those with severe craniofacial features, surgical repair should be considered once the aorta exceeds the 99 th percentile for age and body surface area and the aortic annulus reaches 1.8 cm.  However, with aggressive antihypertensive regimens, the natural history of the disease may change allowing delay of surgical repair until the aortic annulus grows to 2-2.2 cm to allow placement of an adult-sized graft. 
In EDS patients, an echocardiogram and computed tomography angiography of the chest and abdomen should be performed at the initial evaluation for assessment of the aorta.  Traditionally, the recommendation when dealing with vascular complications of EDS has been conservative management with operative intervention only for acute, life-threatening situations. However, a study by Pepin et al. of 220 patients found that 92% of late deaths were from vascular complications and vascular complications shorten survival in EDS patients.  Our group has recommended elective interventions with rapid growth or aortic dissection and elective repairs when family history is notable for early rupture. 
Familial thoracic aortic aneurysm and dissection syndrome
For first-degree relatives of patients with thoracic aortic aneurysm, aortic imaging and genetic testing for TGFBR1, TGFBR2, COL3A1, ACTA2, MYH11, and FBN1 should be performed to identify family members with the asymptomatic disease. ,, If one or more first-degree relatives with known thoracic aortic aneurysm or dissection is also found to have a thoracic aortic aneurysm or dissection, then screening of second-degree relatives should also be considered. Due to the variable age of onset of the disease, imaging of family members at risk of the disease every 2 years should be performed. 
For patients with confirmed FTAADS and a TGFBR1 or TGFBR2 gene mutation, aortic replacement should be considered with an aortic root of 4.5 cm or rapid growth (>0.5 cm in 1-year).  In other FTAADS patients, aortic root dilatation of 5.5 cm, symptomatic disease, or growth of >0.5 cm/year is an indication for aortic root replacement. 
| Surgical Technique|| |
The debate of open versus endovascular
Open tube graft surgical repair for aortic aneurysm and dissection in patients with CTD is a well-established technique with low morbidity and mortality. In the largest studies evaluating open repair in CTD patients, perioperative mortality ranges from 0% to 11.5%, with an overall survival of 53-96%. Perioperative morbidity ranges from 0% to 6.5% for paraplegia, 0-13% for permanent renal failure, and 0-11% for re-exploration for bleeding. ,,,,, Endovascular surgical repair for aortic aneurysm and dissection in patients with CTD as a primary mode for aortic repair in CTD patients remains unproven. The fragility of the aortic wall can lead to stent graft induced trauma, retrograde dissection, and failure to control the aorta proximal and distal to the stent. , Unlike open surgical reconstructions with a universal 100% technical success rate, studies have shown that the technical success rate of endovascular treatment ranges from 38% to 100%. Perioperative mortality ranges from 0% to 14%, with overall survival of 71% to 100%. Perioperative morbidity ranges from 0% to 3.3% for paraplegia, 0-6.7% for permanent renal failure, and 0-14% for re-exploration for bleeding. However, the primary treatment failure rate is high at 44%. Type I endoleaks occur in 8-30% of patients, type II endoleaks occur in 17-30%, and new dissections occur in up to 17% of patients. ,,, In addition, a retrospective review of 650 patients undergoing thoracic endovascular aortic repair (TEVAR) for type B dissection found the incidence of stent graft induced entry tears to be 33% among patients with Marfan syndrome, in contrast to 3% in those without the disease with a mortality of 26%.  Overall, this evidence suggests TEVAR may be safe in short-term, but device issues are central in local aortic complications. In our practice, endovascular techniques are preferred when surgical graft anatomy allows proximal fixation inside a previously placed "open" graft.
Anesthesia and adjunct procedures
The approach to repair of aortic aneurysms relies on a multidisciplinary team of surgeons, anesthesiologists, nurses, surgical technicians, and perfusionists. Prior to starting the procedure, a spinal drain is placed by the anesthesia team to decrease the risk of spinal cord ischemia.  Large-bore venous access is placed under ultrasound guidance to reduce inadvertent arterial punctures.  In addition, prior to the operation, electrodes are placed for motor evoked potential (MEP) monitoring to reduce the risk of paraplegia. These are evaluated through the case to determine if re-implantation of intercostal vessels is necessary.  It is also important throughout the case to maintain stringent blood pressure control. 
During the operation, distal aortic perfusion removes the pressure of a proximal cross clamp. It can be performed either with direct visceral perfusion cannulas, femoral perfusion, or both. For patients with connective tissue disorders, branched grafts are used for direct bypasses to visceral artery aneurysm to avoid recurrent aneurysms seen with patch grafts.
Open graft replacement
The patient is placed in the right lateral decubitus position. For exposure of a type I thoracoabdominal aortic aneurysm (TAAA), a single skin incision is made to allow two thoracotomies in the 4 th and 7 th intercostal spaces. For a type II TAAA, the incision is made in the 6 th intercostal space with a single thoracotomy, and for type III TAAA in the 7 th intercostal space with a single thoracotomy [Figure 2]a. Once the chest is entered, the retroperitoneum is exposed, the costal margin is transected, and the diaphragm is divided in a circumferential fashion with a 2-3 cm rim left along the chest wall [Figure 2]b. The left renal artery is then identified for orientation and the lumbar vein that is usually present in this area is divided. This allows one to roll forward the left kidney and sharply divide the retroperitoneal tissue over the aorta with attention to not injure the vagus and recurrent laryngeal nerves as they course around the distal aortic arch. The inferior pulmonary ligament is divided, and the inferior pulmonary vein is isolated for use in partial left heart bypass. For the cannulation into the pulmonary vein, a purse string suture is placed at the cannulation site to accommodate a 22 or 24 French venous cannula.
|Figure 2: (a) Incision landmarks for type I, II, III, and IV thoracoabdominal aortic aneurysm repair. (b) Division of the diaphragm in a circumferential fashion with a 2-3 cm rim left along the chest wall|
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While the proximal exposure is being obtained, the left common femoral artery is simultaneously exposed via an oblique groin incision for use in partial left heart bypass. For the arterial cannulation, an 8 mm Dacron chimney graft is anastomosed end-to-side to the left common femoral artery for inflow. Partial left heart bypass is instituted with moderate hypothermia at 32°C, and distal pressure is maintained at 60-70 mmHg.
The aorta is clamped sequentially to isolate the proximal descending aorta for the proximal anastomosis. In patients with CTD, the same vessel position should never be clamped twice and Rommels should never be used. The abdominal aorta is opened longitudinally and posterior to the left renal artery with the distal clamp placed across the infrarenal aorta. At this point, the bypass circuit flows are decreased to avoid proximal hypotension due to excessive atrial drainage . The renal arteries are then intubated with a 12-14 French venous cannula and each flushed with 250 ml of cold renal perfusion solution (500 mg of solumedrol in 1 L of normal saline). The renal arteries are perfused with this solution via a slow drip until reconstruction. This method of cold renal perfusion during bypass has been shown in a randomized trial by Kφksoy et al. to lead to better outcomes than perfusion of normothermic blood in patients undergoing TAAA repair.  Back bleeding intercostals are oversewn except for those at the T8-T11 levels, which are controlled with Pruitt occlusion balloons for potential future re-implantation. MEP are monitored and any deterioration in MEP and any drop <75% of baseline is considered significant. If they do not improve with an increase in perfusion pressure, intercostal reconstruction is performed. If the MEPs remain unchanged, any patent intercostal vessels are oversewn. 
The proximal anastomosis is reinforced with felt pledgets circumferentially as the aorta is particularly thin in patients with CTD. Once this anastomosis is finished, the proximal aortic clamp is removed and placed on the graft. The renal and visceral anastomoses are then performed to the separate graft branches. Several branched grafts are available commercially, including the Vascutek ® Gelweave™ Plexus Graft made for the aortic arch, but adaptable for use in other types of aortic reconstruction [Figure 3]a and b and the Vascutek ® Gelweave™ Coselli Thoracoabdominal Graft made specifically for the thoracoabdominal aorta [Figure 3]c. The sequence of anastomoses is at the surgeon's discretion and can be adjusted to the progress of the operation. Renal arteries are usually reimplanted first to decrease the duration of renal ischemia and postoperative acute tubular necrosis. Intercostals are reimplanted if MEPs are lost during the conduct of the operation as above. Once all anastomoses are complete and distal perfusion is adequate, the patient is rewarmed, bypass is terminated, and hemostasis is ensured with the administration of additional products. The operation is then concluded with standard chest and abdominal closures.
|Figure 3: (a) Vascutek® Gelweave™ Plexus Graft made for the aortic arch illustrated in the configuration used for repair of the thoracoabdominal aortic aneurysm. (b) Final configuration of Vascutek® Gelweave™ Plexus Graft with wrapping of graft branches around the main tube graft to achieve optimal graft configuration in relation to visceral arteries once the retroperitoneum is allowed to fall back in place. (c) Illustration of Vascutek® Gelweave™ Coselli Thoracoabdominal Graft use in aortic reconstruction. Note the adjunctive use of felt for reinforcement of the anastomoses|
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Revision aortic surgery in patients with connective tissue disorders
In a series of 229 patients who underwent TAAA repair between 1992 and 2000 at Johns Hopkins Hospital, 107 underwent visceral patches instead of branched graft placement, including 17 patients with CTD. The celiac, superior mesenteric and right renal arteries were typically included in the patch as well as the left renal artery if anatomically feasible. Patch aneurysmal expansion was detected in eight patients (7.5%), of which 3 had CTD [Figure 4]. CTD was a predisposing factor for patch aneurysm formation (patch aneurysm formation 35% CTD patients vs. 5.6% atherosclerotic disease patients, P < 0.05).  Repair of the visceral patch in CTD patients is performed when the aneurysm is >5-6 cm. The rate of major morbidity of the repair is 20-30%, which is significantly higher than the index procedure. Repair options include open repair with refashioning of smaller inclusion patches, hybrid debranching procedures with stent graft into proximal Dacron graft and endovascular stent graft repair [Figure 5].
|Figure 4: Visceral patch aneurysm in a 60-year-old Marfan syndrome patient that is 7 years postthoracoabdominal aortic aneurysm repair|
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|Figure 5: (a) Three-dimensional computed tomography angiography of a 5.2 cm T9-T11 intercostal patch aneurysm in a 32-year-old man with Marfan syndrome 14 years after a thoracoabdominal aortic aneurysm repair. (b) Aortogram of the patient in A prior to repair. (c) Endovascular stent graft repair of the intercostal patch|
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Distal aortic failure after thoracic endovascular aortic repair
In patients with CTD who undergo TEVAR, the aorta distal to the stent graft continues to fail and patients develop recurrent aneurysms as well as acute type B dissections. In a study by Geisbüsch et al., 8 Marfan syndrome patients who underwent TEVAR had a 38% re-intervention rate and 50% developed de novo aneurysms.  In another study by Nordon et al., 7 Marfan syndrome patients who underwent TEVAR had a 33% re-intervention rate and all distal thoracic aneurysms continued to dilate at an average rate of 7 mm/year.  In the third study by Waterman et al., of 16 Marfan syndrome patients who underwent TEVAR, there was a 12% perioperative mortality and 44% of patients ultimately required conversion to an open repair due to retrograde aortic dissection, type I endoleak, aortic rupture, and type B aortic dissection.  When open conversion is required after stent graft placement, the preexisting endograft can be used as a platform for the proximal anastomosis of the surgical interposition graft [Figure 6].
|Figure 6: Open repair of distal type B dissection after thoracic endovascular aortic repair. (a) Clamping technique with a proximal clamp on descending thoracic aorta across the thoracic endovascular aortic repair and distal clamp supraceliac. (b) Opening of the aorta with identification and cannulation of intercostal arteries and of the distal extent of the previously placed graft. (c) Placement of the tube graft with reinforcement of the proximal anastomosis circumferentially with felt pledgets|
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| Conclusions|| |
Patients with connective tissue disorders are living longer and require durable aortic reconstruction. To achieve optimal outcomes in aortic surgery for patients with CTD, they should be evaluated using a multidisciplinary approach with a geneticist, an anesthesiologist, and a surgeon. Aortic repair using branched grafts leads to low morbidity and mortality as well as excellent branch patency and removes the risk of inclusion patch aneurysm. We use several adjunctive measures to decrease the risks of spinal and renovisceral ischemia including distal aortic perfusion via extracorporeal partial left heart bypass, cold renal perfusion, cerebrospinal fluid drainage, sequential aortic clamping, and MEP monitoring to determine the need for intercostal re-implantation. Endovascular approaches should be used with caution and in specific anatomic circumstances because of frequent endograft-related complications.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
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