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291.e4 PART IV Noncoronary Diseases: Diagnosis and Management
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97. Glicklich D, Lamba R, Pawar R. Hypertension in the kidney transplant recipient: overview of pathogenesis, clinical assessment and treatment. Cardiol Rev. 2016;Aug 19, [Epub ahead of print].
98. Boddi M. Renal ultrasound (and doppler sonography) in Hypertension: an update. Adv Exp Med Biol. 2016;Dec 14, [Epub ahead of print].
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100. Tumlin JA, Dunbar LM, Oparil S. Fenoldopam, a dopamine agonist, for hypertensive emergency: a multicenter randomized trial. Fenoldopam Study Group. Acad Emerg Med. 2000;7(6):653–662.
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102. Shusterman NH, Elliott WJ, White WB. Fenoldopam, but not nitroprusside, improves renal function in severely hypertensive patients with impaired renal function. Am J Med. 1993;95(2):161–168.
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104. Hijazi R, Chandar J, Nwobi O, et al. Renal manifestations in toddlers with Takayasu’s arteritis and malignant hypertension. Pediatr Nephrol. 2009;24(6):1227–1230.
105. Hutcheon JA, Lisonkova S, Joseph KS. Epidemiology of pre-eclampsia and the other hypertensive disorders of pregnancy. Best Pract Res Clin Obstet Gynaecol. 2011;25(4):391–403.
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107. Hypertension, Pregnancy-Induced—Practice Guideline. ACOG Task Force on Hypertension in Pregnancy. 2013; 1-100. Available at: http://www.acog.org/Resources-And-Publications/
Task-Force-and-Work-Group-Reports/Hypertension-in­Pregnancy. Accessed January 31, 2017.
108. Reither EN, Hauser RM, Yang Y. Do birth cohorts matter? Age-period-cohort analyses of the obesity epidemic in the United States. Soc Sci Med. 2009;69(10):1439–1448.
109. Ananth CV, Keyes KM, Wapner RJ. Pre-eclampsia rates in the United States, 1980-2010: age-period-cohort analysis. BMJ. 2013;347:f6564.
110. Bilano VL, Ota E, Ganchimeg T, et al. Risk Factors of Pre­Eclampsia/Eclampsia and Its Adverse Outcomes in Low- and Middle-Income Countries: A WHO Secondary Analysis. Young RC, ed. PLoS ONE. 2014;9(3):e91198.
111. Steegers EA, von Dadelszen P, Duvekot JJ, et al. Pre-eclampsia. Lancet. 2010;376(9741):631–644.
112. Minnerup J, Kleffner I, Wersching H. Late Onset Postpartum Eclampsia: it is really never too late-a case of eclampsia 8 weeks after delivery. Stroke Res Treat. 2010;2010:Available at: https://
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113. Meekins JW, Pijnenborg R, Hanssens M, et al. A study of placental bed spiral arteries and trophoblast invasion in normal and severe pre-eclamptic pregnancies. Br J Obstet Gynaecol. 1994;101(8):669–674.
114. Baumwell S, Karumanchi SA. Pre-eclampsia: clinical manifestations and molecular mechanisms. Nephron Clin Pract. 2007;106(2):c72–c81.
115. Vigil-De Gracia P, Tejada OR, et al. Expectant management of severe preeclampsia remote from term: the MEXPRE Latin Study, a randomized, multicenter clinical trial. Am J Obstet Gynecol. 2013;209(5):425.e1–425.e8.
116. Broekhuijsen K, van Baaren GJ, van Pampus MG. HYPITAT-II study group. Immediate delivery versus expectant monitoring for hypertensive disorders of pregnancy between 34 and 37 weeks of gestation (HYPITAT-II): an open-label, randomised controlled trial. Lancet. 2015;385(9986):2492–2501.
117. van Baaren GJ, Broekhuijsen K, van Pampus MG. HYPITAT-II Study Group. An economic analysis of immediate delivery and expectant monitoring in women with hypertensive disorders of pregnancy, between 34 and 37 weeks of gestation (HYPITAT-II). BJOG. 2017;24:453–461.
118. Magee LA, Cham C, Waterman EJ, Ohlsson A, von Dadelszen P. Hydralazine for treatment of severe hypertension in pregnancy: meta-analysis. BMJ. 2003;327(7421):955–960.
119. Aronson S. Perioperative hypertensive emergencies. Curr Hypertens Rep. 2014;16(7):448.
120. Buhari FS, Selvaraj V. Randomized controlled study comparing the hemodynamic response to laryngoscopy and endotracheal intubation with McCoy, Macintosh, and C-MAC laryngoscopes in adult patients. J Anaesthesiol Clin Pharmacol. 2016;32(4):505–509.
121. Momota Y, Kaneda K, Arishiro K, et al. Changes in blood pressure during induction of anesthesia and oral and maxillofacial surgery by type and timing of discontinuation of antihypertensive drugs. Anesth Prog. 2010;57(1):13–17.
122. Kobzar G, Mardla V, Rätsep I, et al. Platelet activity before and after coronary artery bypass grafting. Platelets. 2006;17:289–291.
123. Lee KW, Blann AD, Lip GY. High pulse pressure and nondipping circadian blood pressure in patients with coronary artery disease: Relationship to thrombogenesis and endothelial damage/dysfunction. Am J Hypertens. 2004;18:104–115.
124. Suleiman MS, Zacharowski K, Angelini GD. Inflammatory response and cardioprotection during open-heart surgery: the importance of anaesthetics. Br J Pharmacol. 2008;153:21–33.
125. Forrest JB, Rehder K, Cahalan MK, et al. Multicenter study of general anesthesia. III. Predictors of severe perioperative adverse outcomes. Anesthesiology. 1992;76:3–15.
126. Mangano DT, Browner WS, Hollenberg M, et al. Association of perioperative myocardial ischemia with cardiac morbidity and mortality in men undergoing noncardiac surgery. The Study of Perioperative Ischemia Research Group. N Engl J Med. 1990;323:1781–1788.
127. Balzer F, Aronson S, Campagna JA, et al. High postoperative blood pressure after cardiac surgery is associated with acute kidney injury and death. J Cardiothorac Vasc Anesth. 2016;30(6):1562–1570.
128. Aronson S, Dyke CM, Stierer KA, et al. The ECLIPSE trials: comparative studies of clevidipine to nitroglycerin, sodium nitroprusside, and nicardipine for acute hypertension treatment in cardiac surgery patients. Anesth Analg. 2008;107(4):1110–1121.
129. Espinosa A, Ripollés-Melchor J, Casans-Francés R, et al; Evidence Anesthesia Review Group. Perioperative use of clevidipine: a systematic review and meta-analysis. PLoS ONE. 2016;11(3):e0150625.
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130. Cruise CJ, Skrobik Y, Webster RE, et al. Intravenous labetalol versus sodium nitroprusside for treatment of hypertension postcoronary bypass surgery. Anesthesiology. 1989;71(6):835–839.
131. Brantley HP, Kiessling JL, Milteer HB Jr, et al. Hyperperfusion syndrome following carotid artery stenting: the largest single-operator series to date. J Invasive Cardiol. 2009;21(1):27–30.
132. Abou-Chebl A, Reginelli J, Bajzer CT, et al. Intensive treatment of hypertension decreases the risk of hyperperfusion and intracerebral hemorrhage following carotid artery stenting. Catheter Cardiovasc Interv. 2007;69(5):690–696.
133. Richards JR, Garber D, Laurin EG, et al. Treatment of cocaine cardiovascular toxicity: a systematic review. Clin Toxicol (Phila). 2016;54(5):345–364.
134. Richards JR, Albertson TE, Derlet RW, et al. Treatment of toxicity from amphetamines, related derivatives, and analogues: a systematic clinical review. Drug Alcohol Depend. 2015;150:1–13.
135. Hofer KE, Degrandi C, Müller DM, et al. Acute toxicity associated with the recreational use of the novel dissociative psychoactive substance methoxphenidine. Clin Toxicol (Phila). 2014;52(10):1288–1291.
136. Liakoni E, Dolder PC, Rentsch K, et al. Acute health problems due to recreational drug use in patients presenting to an urban emergency department in Switzerland. Swiss Med Wkly. 2015;145:w14166.
137. Richards JR, Laurin EG, Tabish N, et al. Acute toxicity from topical cocaine for epistaxis: treatment with labetalol. J Emerg Med. 2016 Sep 29;pii: S0736-4679(16):30636–30639.
138. Rezvani M, Hartfield D. Cocaine toxicity after laryngoscopy in an infant. Can J Clin Pharmacol. 2006;13(2):e232–e235.
139. Samenuk D, Link MS, Homoud MK, et al. Adverse cardiovascular events temporally associated with ma huang, an herbal source of ephedrine. Mayo Clin Proc. 2002;77(1):12–16.
140. McBride BF, Karapanos AK, Krudysz A, et al. Electrocardiographic and hemodynamic effects of a
caffeine: a randomized controlled trial. JAMA. 2004;291(2):216–221.
141. Spindelegger CJ, Papageorgiou K, Grohmann R, et al. Cardiovascular adverse reactions during antidepressant treatment: a drug surveillance report of German-speaking countries between 1993 and 2010. Int J Neuropsychopharmacol. 2014;18(4).
142. Ottenbacher R, Blehm J. An unusual case of licorice-induced hypertensive crisis. S D Med. 2015;68(8):346–347, 349.
143. Baumann BM, Perrone J, Hornig SE, et al. Randomized, double-blind, placebo-controlled trial of diazepam, nitroglycerin, or both for treatment of patients with potential cocaine-associated acute coronary syndromes. Acad Emerg Med. 2000;7(8):878–885.
144. McCord J, Jneid H, Hollander J, et al. Management of cocaine­associated chest pain and myocardial infarction: a scientific
statement from the American heart association acute cardiac care committee of the council on clinical cardiology. Circulation. 2008;117:1897–1907.
145. Lee HM, Ruggoo V, Graudins A. Intrathecal clonidine pump failure causing acute withdrawal syndrome with ‘stress-induced’ cardiomyopathy. J Med Toxicol. 2016;12(1):134–138.
146. Pontén J, Biber B, Bjurö T, et al. Beta-receptor blocker withdrawal. A preoperative problem in general surgery? Acta Anaesthesiol Scand Suppl. 1982;76:32–37.
147. Lefkowitz RJ, Caron MG, Stiles GL. Mechanisms of membrane­receptor regulation. Biochemical, physiological, and clinical insights derived from studies of the adrenergic receptors. N Engl J Med. 1984;310(24):1570–1579.
148. Sarafidis PA, Georgianos PI, Malindretos P, et al. Pharmacological management of hypertensive emergencies and urgencies: focus on newer agents. Expert Opin Investig Drugs. 2012;21(8):1089–1106.
149. Wing LA, Conaglen JV, Meyer-Rochow GY, et al. Paraganglioma in Pregnancy: a case series and review of the literature. J Clin Endocrinol Metab. 2015;100(8):3202–3209.
150. Hakim Y, Forbes A, Khan M, et al. Pheochromocytoma presenting as a mimic of acute coronary syndrome. Acute Med. 2016;15(3):145–148.
151. Sanna GD, Talanas G, Fiore G, et al. Pheochromocytoma presenting as an acute coronary syndrome complicated by acute heart failure: the challenge of a great mimic. J Saudi Heart Assoc. 2016;28(4):278–282.
152. Lenders JW, Duh QY, Eisenhofer G, et al; Endocrine Society. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014;99(6):1915–1942.
153. McMillian WD, Trombley BJ, Charash WE, et al. Phentolamine continuous infusion in a patient with pheochromocytoma. Am J Health Syst Pharm. 2011;68(2):130–134.
154. Scholten A, Cisco RM, Vriens MR, et al. Pheochromocytoma crisis is not a surgical emergency. J Clin Endocrinol Metab. 2013;98(2):581–591.
155. Krassioukov AV, Furlan JC, Fehlings MG. Autonomic dysreflexia in acute spinal cord injury: an under-recognized clinical entity. J Neurotrauma. 2003;20(8):707–716.
156. Canon S, Shera A, Phan NM, et al. Autonomic dysreflexia during urodynamics in children and adolescents with spinal cord injury or severe neurologic disease. J Pediatr Urol. 2015;11(1):32.e1–32.e4.
157. Do D, Sheen VL, Bromfield E. Treatment of paroxysmal sympathetic storm with labetalol. J Neurol Neurosurg Psychiatry. 2000;69(6):832–833.
158. Goddeau RP Jr, Silverman SB, Sims JR. Dexmedetomidine for the treatment of paroxysmal autonomic instability with dystonia. Neurocrit Care. 2007;7(3):217–220.
159. Kern J, Bodek D, Niazi OT, et al. Refractory case of paroxysmal autonomic instability with dystonia syndrome secondary to hypoxia. Chest. 2016;149(2):e39–e40.
28
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Acute Aortic Syndromes: Diagnosis and Management
Peter C. Spittell
OUTLINE
Introduction, 292 Acute Aortic Dissection, 292
Pathogenesis, 292 Predisposing Factors, 293 Classification, 293 Clinical Features, 293 Diagnosis, 294 Management, 297
Penetrating Aortic Ulcer, 298
Pathogenesis, 298 Predisposing Factors, 298
Clinical Features, 298 Laboratory Findings, 298 Diagnosis, 299 Management, 299
Aortic Intramural Hematoma, 300
Pathogenesis, 300 Classification, 300 Clinical Features, 300 Diagnosis, 300 Management, 300
INTRODUCTION
Acute thoracic aortic syndromes comprise a spectrum of medical and surgical emergencies, including acute aortic dissection, penetrating aortic ulcer, and aortic intramural hematoma. All of these conditions are potentially life threatening and warrant prompt diagnosis and emergent management. The clinical presentation of acute aortic syndromes is highly variable, ranging from occult disease to classic clinical presentations. Numerous etiologic factors, acting singly and in combination, have been identified. Diagnosis is possible by noninvasive imaging in the majority of patients, but in some patients complementary noninvasive tests (transesophageal echocardiography [TEE], computed tomography [CT], and magnetic resonance imaging [MRI]) are required for diagnosis. The management of acute aortic syndromes continues to be a therapeutic challenge, while diverse surgical and percutaneous strategies for the treatment of aortic syndromes are continuously evolving. As a result of increasing knowledge and better management strategies in this area, the outcomes of patients treated for acute aortic syndromes have improved. Therefore, awareness of the clinical features of acute aortic syndromes and familiarity with currently available diagnostic techniques is basic to their effective treatment.
ACUTE AORTIC DISSECTION
Acute dissection of the thoracic aorta is one of the most common catastrophic aortic conditions encountered in clinical practice. The incidence of aortic dissection has been reported to be
approximately 2.9 per 100,000 per year. presentations of aortic dissection, in combination with a mortality rate in untreated cases as high as 1% per hour during the first 48 hours after the onset of symptoms, underscore the importance of a high index of suspicion and prompt diagnosis and therapy. Noninvasive testing (TEE, CT, and MRI) allows an accurate diagnosis to be made in the majority of patients. treatment exists so that future improvements in initial and long-term survival in acute aortic dissection depend on increased clinical awareness, rapid noninvasive diagnosis, and the early institution of appropriate medical and/or surgical therapy.
1
The variable clinical
5,6
Effective
2–4
Pathogenesis
Aortic dissection originates at the site of an intimal tear in more than 95% of patients.2 The resultant intimal tear exposes the media to pulsatile aortic flow, creating a second or “false” aortic lumen that then dissects in the outer layer of aortic media, propagating distally and, occasionally, proximally.
Ascending aortic dissections are almost twice as common as descending dissections. Some 50% to 65% of aortic intimal tears originate in the ascending aorta and approximately 20% to 30% of intimal tears originate in the vicinity of the left subclavian
7
ar tery.
Once initiated, the dissection usually extends distally and, occasionally, proximally for a variable distance. As the dissecting process encounters branches of the aorta, it may pass around their origins, extend into their walls, or occlude them.2 Reentry of the dissection through a second, more distal intimal tear may occur, usually in one of the iliac arteries. External rupture of
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Keywords
aorta aortic dissection penetrating aortic ulcer aortic intramural hematoma echocardiography
CHAPTER 28 Acute Aortic Syndromes: Diagnosis and Management 293
Type A (proximal) Type B (distal)
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the dissecting process into the pericardial space is the most common cause of death in aortic dissection. Acute congestive heart failure, usually due to aortic regurgitation, is the second most common cause of death.
Predisposing Factors
Multiple risk factors for aortic dissection have been identified, the most common being advanced age, systemic hypertension, congenital abnormalities of the aortic valve, and heritable dis­orders of connective tissue.
Aortic dissection most frequently affects patients in the fifth to seventh decades of life (mean age, 63 years) and is more common in men (65.3% male).7 In patients younger than 40 years, the incidence between men and women is equal due to the occurrence of aortic dissection in women during pregnancy, with approximately 50% of all aortic dissections occurring during the third trimester of pregnancy.
Hypertension is present in 70% of type B dissections, but only in 25% to 35% of type A dissections.
8
7,9,10
Hypertension may play a role in initiating the intramural hematoma along with having a direct weakening effect on the aortic media.10 The causative role of systemic hypertension is further supported by the finding that coarctation of the aorta predisposes to aortic dissection.
11
Other major risk factors for aortic dissection, especially proximal aortic dissection, are congenitally bicuspid or unicom­missural aortic valves
7,9,10
and the genetically mediated collagen disorders such as Marfan syndrome, Loeys Dietz syndrome, and Ehlers-Danlos syndrome.
7,12
Additional predisposing factors
for acute aortic dissection include preexisting aortic aneu-
7,13
rysm
and a positive family history (in as many as 19% of
patients).
Iatrogenic aortic dissection is an uncommon but potentially serious complication of invasive angiographic procedures and cardiac surgery.
10,14–17
Catheter-induced dissection can originate at any location; the majority are retrograde dissections and tend to decrease in size over time owing to thrombosis of the false lumen, whereas anterograde dissections tend to persist on follow­up. Nearly all iatrogenic aortic dissections can be treated medically with serial clinical examinations and noninvasive testing used to identify those in need of surgical therapy.
15
Cardiac surgical procedures complicated by aortic dissection include those that require cross-clamp or cannulation of the ascending aorta, such as aortic valve replacement and/or coronary artery bypass grafting.
10,18,19
Dissection can arise at the site of ascending aortic cannulation, aortosaphenous vein anastomosis, aortic cross-clamp, or as a result of direct arterial injury. Dissection in association with these procedures usually occurs intraopera­tively and is promptly diagnosed and treated, but chronic dis­section in the postoperative period has been reported.
20
Aortic dissection has also been reported in association with
inflammatory diseases (giant cell aortitis,
10,21
Takayasu aortitis, rheumatoid arthritis, syphilitic aortitis, systemic lupus erythe­matosus,22 Noonan syndrome,23 Turner syndrome,24 fibromuscular dysplasia, annuloaortic ectasia, aortic coarctation, cocaine use,
25,26
methamphetamine use, polycystic kidney disease,27 polyarteritis nodosa,28 trauma,29 and high-intensity weight lifting.
30
Fig. 28.1 Classification of aortic dissection. Type I refers to a
primary tear in the ascending aorta and dissection involving the aortic arch and descending thoracic aorta for a variable distance. Type II refers to dissection involving only the ascending aorta. Type III refers to a primary tear distal to the subclavian artery origin, extending distally for a variable distance. (Modified from DeBakey ME, Henly WS, Cooley DA, et al. Surgical management of dissecting aneurysms of the aorta. J Thorac Cardiovasc Surg. 1965;49:130–149.)
Classification
There are two main anatomic systems—the DeBakey and Daily (Stanford) systems—used to classify aortic dissection. The DeBakey system31 is based on the site of origin of the dissection and recognizes three types of dissection (Fig. 28.1). For clinical purposes, because types I and II have a similar prognosis, the more widely used Stanford system classifies dissections that involve the ascending aorta as type A, and all other dissections as type B.
Dissections are categorized as acute if the diagnosis is made within 2 weeks of symptom onset and as chronic if more than 2 weeks have elapsed. The distinction is important owing to the fact that approximately 65% to 75% of patients with untreated aortic dissection die in the first 2 weeks after the onset of symptoms.
Intimal tear without hematoma is an uncommon variant of aortic dissection characterized by a localized intimal tear exposing the underlying media or adventitial layers to pulsatile aortic flow. There is no progression or separation of the medial layers.
Clinical Features
Owing to variable involvement of the aorta and its branches by the dissecting process, the patient with acute aortic dissection may have clinical manifestations of ischemia of various organ systems, singly or in combination, and symptoms and signs of cardiac disease. The diverse presentations of aortic dissection can make diagnosis difficult, and misdiagnosis commonly occurs. of aortic dissection and in medical and surgical therapy, up to
Type I
32,33
2
2,10
Despite major advances in the noninvasive diagnosis
Type II Type III
34
294 PART IV Noncoronary Diseases: Diagnosis and Management
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55% of patients in reported series die without a correct ante­mortem diagnosis.
1,2,10,35,36
For these reasons, a high index of clinical suspicion for acute aortic dissection in any likely setting is imperative.
The classic presentation of a patient with acute aortic dissec­tion, occurring in more than 70% of patients, is the sudden onset of severe pain, usually beginning in the anterior chest, radiating to the back, and moving distally as the dissection progresses.
1,32,37
Chest pain is significantly more common in patients with type A dissection (79% in type A vs. 63% in type B dissection). In contrast, both back pain and abdominal pain are significantly more common with type B aortic dissection over type A: 64% versus 47% for back pain and 43% versus 22% for abdominal pain.7 Although painless dissection occurs in 14% to 21% of patients, it is relatively uncommon.
2,10,38
Patients with painless aortic dissection tend to be slightly older, have a prior history of diabetes, aortic aneurysm, or prior cardiac surgery, and more often have type A dissection.
Patients with acute aortic dissection often appear to be in shock, although hypertension is present in one-half to two-thirds of cases, especially with type B dissection.7 Hypotension is more common in type A dissection and may result from severe aortic regurgitation and/or rupture of the dissection into the pericardial space with resultant cardiac tamponade or, less commonly, the pleural space or mediastinum.
A cardiac murmur may be present, usually at the cardiac base, and may be systolic, diastolic, or both. A diastolic decrescendo murmur of aortic regurgitation indicates involvement of the ascending aorta and is heard in one-half to two-thirds of patients with type A aortic dissection.7 Congestive heart failure, when present in association with proximal aortic dissection, is most often due to severe aortic regurgitation,
9,32
but cases of congestive heart failure due to rupture of the dissecting process into the right or left atrium or right ventricle have also been reported.
39–41
Myocardial infarction, most commonly inferior infarction, occurs in 5% of patients and is due to compromise of either coronary ostium by a hematoma or intimal flap. Peripheral pulse deficits are noted in 19% to 30% of patients,
3,7,32
more commonly with type A aortic dissection, and are associated with a higher rate of in-hospital complications and mortality.42 Pulse deficits may be transitory owing to oscillation of the intimal flap or distal reentry of the hematoma into the true lumen. Acute lower extremity ischemia, with or without chest pain, as a result of dissection extending into the iliac arteries occurs in 6% to 12%
3,43,44
of patients
and may provide an important clue to the
diagnosis.43 Other cardiovascular findings include a difference in systolic blood pressure between the arms (>20 mm Hg), tachycardia, pericardial friction rub, arterial bruits, pulsus para­doxus, and cardiac tamponade.
Syncope occurs in 5% to 10% of patients with aortic dissection and is an important event, as it is associated with a worse prognosis. Syncope most commonly results from either rupture of the dissecting process into the pericardial space, producing cardiac tamponade or involvement of the brachiocephalic arter-
7,10,32
ies.
Less commonly, rupture occurs into the left pleural space, producing a left hemothorax.10 Neurologic deficits—including cerebrovascular accident, disturbances of consciousness, ischemic
paraparesis, and ischemic peripheral neuropathy—may also
1,7
occur.
Other less frequent findings that occur in association with acute aortic dissection include Horner syndrome, a pulsatile sternoclavicular joint,45 vocal cord paralysis, hemoptysis,46 superior vena cava syndrome,47 upper airway obstruction,48 hematemesis,49 pleural effusion, unilateral pulmonary edema,50 signs of mesenteric or renal infarction, fever,51 and deep venous thrombosis.
As a general rule, aortic dissection should always be considered in the differential diagnosis of a patient with unexplained syncope, stroke, congestive heart failure, acute arterial occlusion, or an abnormal aortic contour on chest radiography, even in the absence of chest pain.
Diagnosis
Although aortic dissection may be suspected from the initial history and physical examination, the correct clinical diagnosis is made in less than 50% of patients. The clinical diagnosis of aortic dissection can be improved upon by utilizing the aortic dissection detection risk score, a clinical tool incorporating high-risk conditions, high-risk pain features, and high-risk examination features that can be used to estimate the pretest probability of disease and rapidly identify high-risk patients, facilitating prompt evaluation and treatment. The aortic dissection detection risk score has a sensitivity of 95.7%.
Although routine blood tests are nonspecific in acute aortic dissection, a D-dimer less than 500 ng/mL is highly predictive for excluding aortic dissection in low-risk patients.53 An aortic dissection detection risk score less than or equal to 1 and D-dimer less than 500 ng/mL accurately ruled out acute aortic dissection with a low failure rate (sensitivity 98.7%).
The most common electrocardiographic abnormality in patients with aortic dissection is left ventricular hypertrophy from chronic systemic hypertension.
3,7
Acute electrocardiographic changes occur in up to 55% of patients and include ST segment depression, T-wave changes, and ST segment elevation, in decreas­ing order of frequency.55 Acute ischemic changes can occur when one or both coronary ostia become obstructed, either by the intimal flap or from external compression by the dissecting hematoma. The electrocardiographic changes of acute pericarditis may be seen if there has been leakage of blood into the pericardial space. Heart block resulting from proximal extension of the hematoma into the area of the atrioventricular node has also been reported.
56
The chief value of the electrocardiogram is in distinguishing aortic dissection from acute myocardial infarction, although the two conditions can coexist.
Chest radiography may be helpful in suggesting the diagnosis of aortic dissection, with abnormalities of the aortic silhouette being the most common finding.
7,57
Additional findings include pleural effusion, mediastinal widening, displacement of intimal calcification greater than 6 mm inside the outer edge of the aortic shadow, and the radiographic findings of congestive heart failure (Fig. 28.2). Nonetheless, it is important to remember that
normal chest radiographic findings (present in 11% to 16% of patients) do not exclude the diagnosis of aortic dissection.
Confirmation of the diagnosis of aortic dissection requires
cardiovascular imaging that demonstrates the dissection flap
52
54
10
7
CHAPTER 28 Acute Aortic Syndromes: Diagnosis and Management 295
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A
Fig. 28.2 Chest radiographs in a patient with aortic dissection. (A) Baseline anteroposterior chest
radiograph before presentation with aortic dissection. (B) Chest radiograph 1 year later when the patient presented with acute aortic dissection. Note the increased diameter of the ascending aorta and aortic arch and new widening of the superior mediastinum.
B
separating a false lumen from the true lumen. Currently available noninvasive imaging modalities that are accurate in the diagnosis of acute aortic dissection include multiplane TEE, CT angiography, and MR angiography.
Transthoracic echocardiography (TTE) can be very useful in some patients with suspected aortic dissection (Video 28.1). When the findings of a dilated aortic root (end-diastolic diameter >42 mm), widening of the aortic walls (16 to 20 mm for the anterior wall and 10 to 13 mm for the posterior wall), and a linear undulating echo representing the intimal flap are present, the positive predictive value for TTE is 100%.
58–60
Advantages of TTE are its portability; real-time diagnosis; ability to identify associated aortic regurgitation; and to assess left ventricular function, regional wall motion abnormalities, pericardial effusion, and cardiac tamponade. The diagnosis of cardiac tamponade in a patient suspected of having aortic dissection deserves special mention. Echocardiographically guided pericardiocentesis
should be avoided in this setting, as the rapid withdrawal of pericardial fluid can result in a prompt improvement in left ventricular systolic function, left ventricular dP/dT, and systolic blood pressure, producing aortic rupture.
61,62
The patient with aortic dissection complicated by cardiac tamponade should be taken emergently to surgery for institution of cardiopulmonary bypass followed by evacuation of blood from the pericardial space. Disadvantages of TTE in the diagnosis of aortic dissection include difficulty in adequately visualizing the descending thoracic
5
and suboptimal echocardiographic windows in patients
aorta with obesity or chronic obstructive pulmonary disease. Overall, the sensitivity and specificity of TTE are inferior to TEE, CT, and MRI in the diagnosis of aortic dissection. Intravenous contrast agents improve the diagnostic accuracy of TTE in patients with aortic dissection, achieving a sensitivity and specificity similar to TEE in type A dissection.63 It should be remembered that a negative TTE does not exclude aortic dissection.
Multiplane TEE has a sensitivity of 99% and a specificity of 98% in the diagnosis of aortic dissection.64 TEE is portable, minimally invasive, and can accurately determine the type and extent of dissection safely in an emergent setting, allowing rapid triage of patients to either surgical or medical therapy
65,66
(Video
28.2). Color flow Doppler imaging significantly improves the sensitivity of TEE by allowing visualization of the intimal flap, dissection entry site, the true and false lumens, presence of thrombus, mechanism and degree of aortic regurgitation, and the proximal coronary arteries66 (see Fig. 28.2). In addition, multiplane TEE provides a comprehensive assessment of left ventricular systolic function, regional wall motion, pericardial effusion, and cardiac tamponade. The overall sensitivity of TEE is comparable with CT,67 MR,
5,68,69
and aortography70 in the diagnosis of aortic dissection. Current multiplane TEE probes have largely overcome impediments in the ascending aorta, although artifacts in this region continue to be a diagnostic challenge.
CT with intravenous iodinated contrast enhancement is an accurate noninvasive screening test in patients with suspected aortic dissection.71 Advantages of CT include ready availability at most hospitals and improved accuracy with spiral (helical) CT and electron beam (ultrafast) or multidetector (multislice) CT.72 CT can reliably demonstrate the intimal flap, pericardial and pleural effusion, associated mediastinal hemorrhage, and involvement of the aortic arch vessels and branches of the abdominal aorta, as well as coronary artery disease5 (Fig. 28.3). Disadvantages of CT include the need for iodinated contrast exposure and nonportability, limiting its use in patients with significant renal insufficiency and in hemodynamically unstable patients, respectively. In addition, the site of entry is rarely identified.
Although less commonly used,
7
MRI is a highly accurate noninvasive technique in the evaluation of patients with suspected aortic dissection.73 MRI is superior to TEE and CT in detecting arch vessel involvement and in identifying the anastomosis in patients managed with surgical therapy and may facilitate comparison of serial studies.
5,68,74
Gated spin-echo MRI accurately demonstrates the entry site and intimal flap75 and may be the optimal method for demonstrating thrombus formation and entry site location within all segments of the aorta76 (Fig. 28.4). The ability to obtain oblique and longitudinal planes of a section makes MRI especially valuable in demonstrating dissection without intimal tear.
5,69,77
Disadvantages of MRI include cost,
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Fig. 28.3 Computed tomographic scan in a patient with type A
aortic dissection. Note the complex intimal flap seen within the distal ascending aorta and descending thoracic aorta (arrows), and differential opacification of the true and false lumens.
Fig. 28.4 Magnetic resonance imaging in the sagittal plane shows
type A aortic dissection with an intimal flap extending into the distal abdominal aorta.
examination time, reduced availability, nonportability, and standard contraindications to MRI.
Of the definitive noninvasive imaging modalities in suspected acute aortic dissection (TEE, CT, MRI), a systematic review of the diagnostic accuracy of these imaging techniques has dem­onstrated a pooled sensitivity (98% to 100%) and specificity (95% to 98%) that is comparable between the three imaging techniques. Therefore the choice of test depends upon which imaging modality is readily available at a particular institution and the hemodynamic stability of the patient.
Fig. 28.5 Aortogram shows a spiraling intimal flap (arrows) in
the ascending aorta and aneurysmal dilation of the ascending aorta.
Aortography, the traditional definitive diagnostic method in aortic dissection, is able to localize the site of origin of the dis­section and delineate the extent of the dissection and circulation to vital organs. Diagnostic aortographic features include opacifica­tion of the false lumen, deformity of the true lumen by the false lumen, dilation of the aorta, narrowing or occlusion of branches of the aorta, and the presence of an intimal flap78 (Fig. 28.5). Disadvantages of aortography include nonportability, invasive technique, exposure to ionizing radiation, the use of intravenous iodinated contrast agents, and an inherent delay in diagnosis. False-negative aortogram results can occur if there is simultaneous and equal opacification of the true and false lumina or if the false channel is very faintly opacified.
79
For these reasons, aor­tography has generally been replaced by noninvasive imaging tests in the diagnosis of acute aortic dissection. However, for patients in whom the suspicion for ascending aortic dissection is very strong but noninvasive imaging is unavailable or incon­clusive, digital subtraction aortography should be performed. Intravascular ultrasound, in combination with standard aorto­graphic technique, greatly improves the accuracy of aortography, can be performed rapidly and safely, and could serve as an accessory diagnostic procedure in selected patients with suspected aortic dissection.
80,81
In view of the increased early mortality of untreated acute aortic dissection, the screening test chosen depends on which test is most readily available at a particular institution and the patient’s hemodynamic status. Noninvasive diagnosis of acute aortic dissection by TEE, CT, or MR, if readily available, is
CHAPTER 28 Acute Aortic Syndromes: Diagnosis and Management 297
Intimal ulcer
hematoma
false aneurysm
rupture
(saccular true aneurysm)
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Atheroma Intima
Media
Adventitia
Plaque ulceration
Intimal deep ulcer
Fig. 28.6 Pathologic consequences of penetrating atherosclerotic ulcer of the aorta. Atheromatous
ulceration that burrows deeply into an atheroma can result in one of four potential outcomes: true saccular aneurysm, medial hematoma, adventitial false aneurysm, or transmural rupture. An intramural (medial) hematoma is the most commonly observed consequence of a penetrating aortic ulcer.
Medial
preferred as it avoids the risks and delays inherent in invasive angiography.
82
Management
Treatment for acute aortic dissection is initiated when the diagnosis is first suspected clinically. After initial patient stabiliza­tion, the diagnosis is pursued with expedited noninvasive imaging (Fig. 28.6). Treatment recommendations (discussed later) are generally in agreement with multidisciplinary cardiovascular guidelines.
The initial treatment objectives in suspected aortic dissection are to control pain and provide anti-impulse therapy to reduce systemic blood pressure, lower the rate of change of pressure development (dP/dT) to decrease aortic shear stress, and limit propagation of the dissection. In hypertensive patients, treatment consists of an intravenous (IV) β-adrenergic blocking agent, often in combination with IV sodium nitroprusside. β-Blockade before nitroprusside therapy is essential because the latter increases the velocity of left ventricular ejection, increases aortic shear stress, and may promote propagation of the dissection. venous labetalol in place of a β-blocker and sodium nitroprusside is an alternative, but it has the potential for hepatotoxicity with long-term therapy.87 In normotensive patients, an IV β-adrenergic blocking agent may be used alone. Intravenous verapamil or diltiazem is an alternative in patients who cannot tolerate β-blockers. To assist in monitoring blood pressure and renal perfusion, an intraarterial cannula and indwelling bladder catheter are advisable. Following initial patient stabilization, expedited noninvasive aortic imaging is pursued (as discussed in the previous section). If the patient continues to require close monitoring and support, an emergency bedside TEE is the test of choice.
Patients with acute type A aortic dissection should undergo emergent surgical repair, unless significant comorbidities that limit survival to 1 year or less are present.
graphy before surgery is not indicated because it does not
83–85
8,88,89
Coronary angio-
86
Intra-
Adventitial
Transmural
improve survival and it results in a delay in surgical interven-
90,91
tion. Dacron is the usual procedure in type A aortic dissection.
Resection of the ascending aorta and replacement with
88,89
The objectives of surgery include excision of the intimal tear, obliteration of the entrance into the false lumen, repair of aortic regurgitation (if present), and restoration of patency to any major arteries occluded by the dissection. With associated aortic regurgitation, resuspension of the valve, if possible, is preferred. Intraoperative TEE can define the severity and mechanisms of aortic regurgitation and can assist the surgeon in identifying patients in whom valve repair is likely to be successful.92 If there is associated annuloaortic ectasia or destruction of the aortic wall, a valved conduit may be used. The coronary arteries are reimplanted or, if they are involved by the dissection, they are reattached using vein bypass grafts anastomosed proximally to the aortic graft and distally to the uninvolved coronary arter-
89,93,94
ies.
If compromise of a branch of the aorta supplying a vital organ is present and is not relieved by closing the false channel, then direct repair or surgical replacement of that branch is indicated. Aortic fenestration may be indicated in patients with severe organ or limb ischemia complicating either acute or chronic aortic dissection. Fenestration, by either surgical or percutaneous techniques, can effectively relieve the ischemia and can be performed safely in chronic aortic dissection.
95,96
Operative mortality for type A dissection at experienced centers varies from 7% to 36%, which is well below the more than 50% mortality with medical therapy.
7,56,97,98
In-hospital mortality is approximately 14% to 27%, patients with cardiogenic shock and those requiring concomitant coronary artery bypass grafting being the highest-risk subgroup.
97,98
There is limited experience with endovascular stent grafts in patients with type A dissection, although preliminary studies suggest that endovascular repair can be performed with minimal adverse effects on aortic valve function and sustained survival.99 Larger studies are needed to confirm the durability of endovascular
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therapy and to more accurately identify those patients most likely to benefit from this approach.
With acute type B (type III) aortic dissection, urgent surgical intervention is reserved for patients who have a complicated course (rupture, acute expansion or impending rupture, or vascular occlusion).
100
Independent predictors of surgical mortality include those 70 years and older, and hypotension or stroke on admission.
101
The optimal treatment for uncomplicated type III aortic dissection is less well defined, but the majority of patients are best treated with medical therapy. mortality for these patients is approximately 10%.
102
Overall in-hospital
103
Long-term
medical therapy emphasizes control of blood pressure, using a β-blocker if possible, and periodic evaluation for evidence of any progression of dissection, patency of the false lumen, progres­sion of aortic diameter to greater than 5 cm, or development of saccular aneurysm.
104–106
The reported long-term survival rate with medical therapy is approximately 60% to 80% at 4 to 5 years and approximately 40% to 45% at 10 years.
103
Survival is best in patients with noncommunicating and retrograde dissec­tions. The proximal descending thoracic aorta is the major site of aneurysm development; an enlarged false lumen in this region predicts poor outcome and subsequent aneurysm development.
107
Furthermore, partial false lumen thrombosis (34% of patients) predicts a significantly worse 3-year mortality rate.
108
An alternate approach is 2 to 3 weeks of pharmacologic therapy followed by surgical repair if the dissection becomes stable and the patient’s general condition does not contraindicate surgery.
109,110
Endo­vascular intervention in type B aortic dissection is being suc­cessfully performed with increasing frequency and may provide an alternative to surgery in highly selected patients in the
111–113
future.
Randomized trials comparing endovascular interven­tion and conventional medical management have not demon­strated a significant difference in overall survival at 2 years, although remodeling with thrombosis of the false lumen and reduction of its diameter was evident in the stent graft group.
114–116
Postoperatively, continuation of β-blockade is essential, if
possible, as hypertension and left ventricular ejection velocity play an important role in the recurrence of aortic dissection. Following hospital dismissal, noninvasive testing is performed at periodic intervals to detect the development of an anastomotic aneurysm or saccular aneurysm, extension of the dissection, patency of the false lumen, or progressive aortic dilation.
117,118
Initial follow-up noninvasive imaging at 3, 6, and 12 months is warranted. Subsequent follow-up is performed every 1 to 2 years if there is no evidence of disease progression. MRI is generally preferred for follow-up (a baseline MRI before hospital discharge should be performed).
PENETRATING AORTIC ULCER
Penetrating aortic ulcer shares several clinical features with aortic dissection, especially type B aortic dissection, but the absence of certain clinical signs favors a diagnosis of penetrating aortic ulcer. Results of noninvasive imaging studies are usually diagnostic, allowing differentiation of penetrating aortic ulcer from typical aortic dissection. two disorders is important in view of the fact that the natural
119,120
Differentiation between the
history of penetrating aortic ulcer is less well defined; therefore, treatment may differ from that currently used for classic aortic dissection.
119,120
Pathogenesis
Penetrating aortic ulcer refers to an atherosclerotic lesion of the thoracic aorta that undergoes ulceration, which penetrates the internal elastic lamina of the thoracic aorta, resulting in formation of one of the following: intramural hematoma within the media of the aortic wall, a true saccular aneurysm, a pseudoaneurysm, or transmural aortic rupture
121
(Video 28.3).
Predisposing Factors
Risk factors for penetrating aortic ulcer are similar to those for aortic dissection, the most common being advanced age, chronic systemic hypertension, and evidence of advanced atherosclerotic disease.
122
In contrast to aortic dissection, men and women are equally affected. Long-standing hypertension is present in the majority of patients and likely contributes to the advanced atherosclerotic disease that is universally evident. More than one-half of the patients with penetrating aortic ulcer have advanced atherosclerotic disease in other locations, including coronary artery disease, peripheral arterial occlusive disease, and cerebrovascular disease. An increased association of penetrating aortic ulcer and abdominal aortic aneurysm, and aneurysms in other locations, has also been reported.
121,123
Clinical Features
The clinical presentation of penetrating aortic ulcer and acute aortic dissection is similar, the most common presentation being an elderly patient with systemic hypertension and the sudden onset of severe pain in the chest, back, and—less commonly— epigastrium. Unlike aortic dissection, the pain is rarely migratory. Since the most common site of penetrating aortic ulcer is in the descending thoracic aorta, a new murmur of aortic regurgitation, pericardial friction rub, and peripheral pulse deficits are not seen. In addition, visceral vessel involvement has not been reported. Neurologic deficits are very rare, but acute lower extremity paraplegia may occur.
121
In a patient with a history compatible with aortic dissection, it is the absence of physical findings that suggests the diagnosis of penetrating aortic ulcer. Asymptomatic penetrating aortic ulcer does occur and is usually incidentally discovered as enlargement of the descending thoracic aorta or a hilar mass on routine chest radiography or on CT done for another indication.
121,123
Laboratory Findings
Routine laboratory studies are nonspecific with penetrating aortic ulcer. Chest radiography is the most helpful of the routine laboratory tests because it is often abnormal. It may demonstrate mediastinal widening, focal or diffuse enlargement of the descending thoracic aorta, a hilar mass, left apical mass, bilateral pleural effusion, or isolated left pleural effusion. However, normal chest radiographic findings do not exclude penetrating aortic ulcer. The most common electrocardiographic abnormality is left ventricular hypertrophy from chronic systemic hypertension.
119,120