Type B Aortic Dissection: Management in a Young Man With Neurologic Sequelae

Transcription

1 C a s e S t u d i e s i n H y p e r t e n s i o n J o e l H a n d l e r, M D, S e c t i o n E d i t o r Type B Aortic Dissection: Management in a Young Man With Neurologic Sequelae Joel Handler, MD 39-year-old man who had never seen a physician noted the sudden onset of central chest A pressure while working on his roof. The pain was severe, radiated to his midback, and was accompanied by shortness of breath. Simultaneously, he developed bilateral leg weakness. In the emergency department, the patient s blood pressure was 169/81 mm Hg, with a heart rate of 76 bpm. Cardiac examination revealed a regular rate and rhythm without gallops, murmurs, or ectopy. Peripheral pulses were present, and there were no abdominal masses or bruits. He was unable to lift either lower extremity, but had normal strength in both upper extremities. The patient did not have long thin extremities or loose joints. Laboratory examination revealed a blood urea nitrogen of 16 mg/dl (normal <19 mg/dl), a creatinine of 0.9 mg/dl (normal mg/dl), a hemoglobin of 15.4 mg/dl (normal g/dl), and a troponin I of 0.01 mg/ml (normal <0.04 mg/ml). Urinalysis showed no cells and was negative for protein. The electrocardiogram revealed minimal nonspecific ST change. The mediastinal contour on the admitting chest radiograph was normal. Computed tomography (CT) scanning in the emergency department showed an intimal flap in the proximal descending aorta just distal to the takeoff of the left subclavian artery with a false lumen extending to both common iliac arteries, consistent with a type B aortic dissection (Figure 1). The patient was given a 10-mg bolus of IV labetalol followed 10 minutes later by a 20-mg bolus and then a 4-mg/min From Kaiser Permanente, Anaheim, CA Address for correspondence: Joel Handler, MD, Kaiser Permanente, 411 Lakeview Avenue, Anaheim, CA joel.handler@kp.org ID: 5146 continuous infusion, achieving systolic pressures of mm Hg and heart rates of bpm. His chest pain resolved after about 12 hours, and he was able to lift each leg weakly off the bed. Oral medications were initiated and, on the third hospital day, he had a blood pressure of 112/68 mm Hg and a heart rate of 64 bpm off all IV medication and receiving hydrochlorothiazide (HCTZ) 25 mg, atenolol 50 mg b.i.d., lisinopril 40 mg, and nifedipine sustainedrelease 30 mg daily. Removal of his Foley catheter led to urinary retention and reinstallation of the catheter. On the evening of the third day, he experienced more substernal chest pressure with interscapular radiation; the systolic pressure rose to 162 mm Hg. A chest radiograph revealed a new small left pleural effusion (Figure 2). On IV labetalol, the pain resolved and a CT scan showed no change in the proximal extent of the aortic dissection. A large aortic dissection flap just distal to the left subclavian artery was clearly visualized on transesophageal echocardiography (TEE). There was no extension of the tear to the ascending aorta, and the aortic valve had normal trileaflet structure. Magnetic resonance angiography (MRA) also did not show more proximal extension of the original aortic tear (Figure 3). Over the next 2 days, the patient was again tapered off of the labetalol drip and had no recurrence of chest discomfort. He was discharged on hospital day 8 on HCTZ 25 mg, atenolol 75 mg b.i.d., lisinopril 40 mg, and terazosin 5 mg daily with a blood pressure of 108/58 mm Hg and a heart rate of 62 bpm. At the time of a 3-week post-hospitalization office visit, the patient was in a wheelchair and unable to ambulate because of continuing bilateral leg weakness and required bladder catheterizations 4 6 times daily for urinary retention. Nine months later, the patient was ambulatory without assistance. VOL. 8 NO. 4 APRIL 2006 THE JOURNAL OF CLINICAL HYPERTENSION 291

2 Figure 1. CT image showing findings of type B aortic dissection. Note intimal flap in descending aorta with some enhancement of the false lumen. The curvilinear focus in the ascending aorta is artifactual. PA=pulmonary artery; AA=ascending aorta; TL=true lumen; FL=false lumen Figure 2. Chest radiograph on hospital day 3 shows development of a new left pleural effusion. DISCUSSION Acute aortic dissection is an uncommon medical emergency that demands early diagnosis and classification to expedite triage to medical vs. surgical management. This case had a rather unusual presentation because of its onset in a 39-year-old. However, the association of acute paraparesis with acute chest pain limited the differential diagnosis. The severity of chest discomfort suggested a differential diagnosis of acute coronary syndrome, pulmonary embolism, and acute pericarditis in addition to aortic dissection. The differential diagnosis of sudden, acute paraparesis includes thromboembolism of a spinal artery, shock, epidural or subdural hematoma at the spinal level, tumor bleeding, rupture of an arteriovenous malformation, multiple myeloma, and transverse myelitis, in addition to aortic dissection with spinal artery involvement. 1 The match-up of these two sets of symptoms made acute aortic dissection a reasonable initial working diagnosis. The three major noninvasive imaging options for making a diagnosis of aortic dissection are contrastenhanced x-ray CT, magnetic resonance imaging (MRI), and TEE. A prospective comparison of these techniques in 110 patients showed slight preference for an MRI (one false negative, 98.3% sensitivity), compared with TEE (one false negative, 97.7% sensitivity), and CT (three false negatives, 93.8% sensitivity), relative to routine aortography in which two false negatives were reported. 2 Specificity comparisons also favored MRI, with one false positive on MRI, four false-positive CT results, and six false positives on TEE (Table I). At the level of the thoracic aorta where detection of the proximal extent of the dissection is the key management indicator, MRI and TEE had comparable sensitivity. More false-positive TEE results were attributed to plaque formation or echo reverberations in an ectatic vessel. 2 Since this study was published in January 1993, newer CT scanners have mostly replaced earlier models and probably offer sensitivity similar to MRI and TEE. Therefore, in most hospitals, CT is the first imaging modality due to availability and speed of testing. This can be followed by a confirmatory MRI if the patient is stable and there is any uncertainty with regard to either the diagnosis or the most proximal location of the tear. Significant renal insufficiency would suggest either MRI or TEE, and hemodynamic instability might indicate a TEE. Transthoracic echocardiography (TTE) can also make a diagnosis of aortic dissection but has suboptimal sensitivity and specificity (Table I). Detection of the most proximal portion of the tear in acute aortic dissection is the key management indicator and is based on an anatomic landmark. Dissection occurring proximal to the take-off of the left subclavian artery is classified as Stanford type A and distal to the left subclavian as type B. There is greater risk associated with type A dissections since more proximal extension can bleed into the pericardium, causing fatal tamponade. In addition, greater shear pressures where the aorta is at its largest diameter (according to LaPlace s law relating internal pressure to vessel diameter) are more likely to cause free rupture of the vessel. Acuity of the dissection is determined by duration: up until 14 days is defined as acute, and past 14 days is classified as chronic, because of patient stabilization and the tailing off of morbidity and mortality with time. 3 A diagnosis of acute Stanford type B aortic dissection was established for this patient on the basis of a CT, but a follow-up TEE and MRI were necessary to confirm the most proximal portion of the tear when the patient had recurrence of pain and 292 THE JOURNAL OF CLINICAL HYPERTENSION VOL. 8 NO. 4 APRIL 2006

3 Table I. Diagnostic Potential of TTE, TEE, CT, and MRI for the Detection of Thoracic Aortic Dissection in the 47 Patients Who Underwent All Four Procedures* LOCATION OF DISSECTION SENSITIVITY (%) SPECIFICITY (%) ACCURACY (%) POSITIVE PREDICTIVE VALUE (%) NEGATIVE PREDICTIVE VALUE (%) Ascending aorta TTE TEE CT ** MRI ** Aortic arch TTE TEE CT MRI Descending aorta TTE TEE CT MRI TTE=transthoracic echocardiogram; TEE=transesophageal echocardiogram; CT=computed tomography; MRI=magnetic resonance imaging; *percentages were calculated on the basis of all assessable findings; **p<0.05 for the comparison with TTE and TEE; p<0.01 for the comparison with TEE, x-ray CT, and MRI. Reprinted with permission from New Engl J Med. 1993;328: elevated blood pressures following transition to oral antihypertensive therapy. Fortunately, there was no evidence that he had converted to a type A dissection. Outcome data from the International Registry of Aortic Dissection (IRAD) 3 suggests that mortality from the type B dissections treated medically vs. surgically is 10.7% compared with 31.4%, and the mortality from type A dissections treated medically compared with surgically is 58.0% vs. 26.0%. These outcome data support the recommendation to triage the acute type A dissections to urgent surgical repair and the acute type Bs to medical management. IRAD, which has collected prospective data from 18 large contributing centers since January 1996, has been a rich source of clinical data. Some demographic, symptomatic, and radiologic presentation data from IRAD are presented in Table II. When a follow-up chest radiograph in this case showed the interval development of a new left pleural effusion, there was concern for vessel leakage and conversion to a type A dissection. The IRAD data report that pleural effusion is an uncommon finding in acute aortic dissection, mostly nonspecific, and as likely to be seen in type A dissection (17.3%) as in type B dissection (21.8%) (Table II). This case showed a completely normal chest radiograph on presentation; the lack of mediastinal widening is seen in approximately 40% of cases (Table II). IRAD has separately analyzed a subgroup of 68 younger patients (younger than 40) with acute aortic Figure 3. Magnetic resonance angiography of the aortic arch and descending aorta showing intimal flap in the proximal to mid-descending aorta (type B dissection). Intercostal arteries show flow. VOL. 8 NO. 4 APRIL 2006 THE JOURNAL OF CLINICAL HYPERTENSION 293

4 Table II. Characteristics of Patients (N=464) With Acute Aortic Dissection TYPE A (N [%])* TYPE B (N [%])* P VALUE CATEGORY N (%)* (N=289) (N=175) TYPE A VS. B Demographics Age (yr) (mean [SD]) 63.1 (14.0) 61.2 (14.1) 66.3 (13.2) <0.001 Male sex 303 (65.3) 182 (63.0) 121 (69.1) 0.18 Patient history Marfan syndrome 22/449 (4.9) 19 (6.7) 3 (1.8) 0.02 Hypertension 326/452 (72.1) 194 (69.3) 132 (76.7) 0.08 Atherosclerosis 140/452 (31.0) 69 (24.4) 71 (42) <0.001 Known aortic aneurysm 73/453 (16.1) 35 (12.4) 238 (2.2) Prior aortic dissection 29/453 (6.4) 11 (3.9) 18 (10.6) Diabetes mellitus 23/451 (5.1) 12 (4.3) 11 (6.6) 0.29 Presenting symptoms Any pain reported 443/464 (95.5) 271 (93.8) 172 (98.3) 0.02 Abrupt onset 379/447 (84.8) 234 (85.4) 145 (83.8) 0.65 Chest pain 331/455 (72.7) 221 (78.9) 110 (62.9) <0.001 Anterior chest pain 262/430 (60.9) 191 (71.0) 71 (44.1) <0.001 Posterior chest pain 149/415 (35.9) 85 (32.8) 64 (41) 0.09 Back pain 240/451 (53.2) 129 (46.6) 111 (63.8) <0.001 Abdominal pain 133/449 (29.6) 60 (21.6) 73 (42.7) <0.001 Quality of pain: sharp 174/270 (64.4) 103 (62) 71 (68.3) NA Quality of pain: tearing or ripping 135/267 (50.6) 78 (49.4) 57 (52.3) NA Radiating 127/449 (28.3) 75 (27.2) 52 (30.1) 0.51 Migrating 74/446 (16.6) 41 (14.9) 33 (19.3) 0.22 Syncope 42/447 (9.4) 35 (12.7) 7 (4.1) Radiography findings (n=427) 427 (100) 256 (88.6) 171 (97.7) No abnormalities noted 53 (12.4) 26 (11.3) 27 (15.8) 0.08 Widened mediastinum 263 (61.6) 169 (62.6) 94 (56) 0.17 Pleural effusion 82 (19.2) 46 (17.3) 36 (21.8) 0.24 *Except where indicated otherwise. Adapted with permission from JAMA. 2000;283: dissection (7% of the total 951 in the registry). 4 Findings included a high prevalence of unique clinical risk factors such as Marfan s syndrome, bicuspid aortic valve, and larger aortic dimensions. It can be seen in Table III that Marfan s syndrome occurred in 50% of patients younger than 40 and was more common than hypertension, which occurred in 34%. Previously undetected hypertension was believed to be the major risk factor for this 39-year-old black patient. Comparing age younger than 40 with age older than 40, mortality rates were the same despite a higher rate of cardiovascular comorbidities in the older group; this phenomenon is not well explained. There was a higher rate of limb ischemia in the younger age range, 18% vs. 10%, respectively (p=0.06), but there was no difference in neurological sequelae (both age groups, 10%). 4 Acute paraparesis in association with aortic dissection may be transient or permanent. Painless transient paralysis of the lower extremities has been reported as a sole manifestation of acute thoracoabdominal aortic dissection. 5 Nine months following the present case presentation, the patient had become ambulatory. Medical management of acute aortic dissection demands rapid blood pressure lowering with agents that also reduce shear pressure and pulsatile load, dp/dt. Target systolic pressure is <120 mm Hg or even <100 mm Hg if tolerated. 6 Primary vasodilators such as nitroprusside should be used only in conjunction with IV β blockade or α/β blockers such as labetalol to reduce the increased shear pressure that might otherwise occur with a pure vasodilator despite blood pressure reduction. Alternative IV β-blocking agents include esmolol and metoprolol. 7 IV fenoldopam, a selective peripheral dopamine-receptor agonist is an alternative vasodilator to nitroprusside, which does not accumulate or have toxic metabolites in patients with renal insufficiency. 8 Hydralazine and minoxidil are generally avoided because, as pure vasodilators, they are more likely to increase pulsatile aortic force. 9 Table IV describes the recommended 294 THE JOURNAL OF CLINICAL HYPERTENSION VOL. 8 NO. 4 APRIL 2006

5 Table III. Baseline Demographics of Patients in the International Registry of Aortic Dissension Based on Age Categories of <40 and 40 Years of Age (n [%])* VARIABLES AGE <40 YR (N=68) AGE 40 YR (N=883) P VALUE Age (yr) (mean ± SD) 30.7± ±11.5 NA Type of dissection Type A 46 (68) 574 (65) NS Type B 22 (32) 309 (35) Male gender 52 (76) 596 (67) NS White race 55 (81) 699 (79) NS Diabetes 0 (0) 38 (4) NS Hypertension 23 (34) 635 (72) < Atherosclerosis 1 (1) 267 (30) < Marfan s syndrome 34 (50) 19 (2) <0.001 Prior aortic valve disease 7 (10) 74 (8) NS Bicuspid aortic valve (n=516) 6 (9) 12 (1) <0.001 Known aortic aneurysm 13 (19) 115 (13) NS Prior aortic dissection 5 (7) 50 (6) NS Prior aortic valve replacement 8 (12) 40 (5) Prior coronary artery bypass grafting 0 (0) 51 (6) 0.04 Peripartum aortic dissection 2 (3) 0 (0) <0.001 Cocaine-related aortic dissection 0 (0) 5 (0.6) NS Other aortic disease 2 (3) 10 (1) NS Iatrogenic aortic dissection 0 (0) 36 (4) NS NA=not applicable; NS=nonsignificant; *except where indicated otherwise. Reprinted with permission from J Am Coll Cardiol. 2004;43: antihypertensive agents and dose ranges for aortic dissection. Transitioning from acute IV to oral antihypertensive therapy is accomplished gradually. The patient first needs to demonstrate complete stability and lack of symptoms for at least 24 hours to allow transition to oral drugs. This case demonstrated a recurrence of symptoms 24 hours following conversion to oral therapy. This exacerbation necessitated urgent imaging reevaluation and a return to IV labetalol. He then tolerated a second transition attempt to oral drugs. REFERENCES 1 Hsu YC, Lin CCK. Paraparesis as the major initial presentation of aortic dissection: report of four cases. Acta Neurol Taiwan. 2004;13: Nienaber CA, Kodolitsch YV, Nicolas V, et al. The diagnosis of thoracic aortic dissection by noninvasive imaging procedures. N Engl J Med. 1993;328: Hagan PG, Nienaber CA, Isselbacher EM, et al. The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. JAMA. 2000;283: Januzzi JL, Isselbacher EM, Fattori R, et al. Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). J Am Coll Cardiol. 2004;43: Joo JB, Cummings AJ. Acute thoracoabdominal aortic dissection presenting as painless, transient paralysis of the lower extremities: a case report. J Emerg Med. 2000;19: Seventh report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure. Hypertension. 2003;42: Table IV. Dosages of IV Antihypertensive Medications Used in Acute Aortic Dissection DRUGS DOSAGE Labetalol* Initial bolus 20 mg, followed by boluses of mg or an infusion starting at 2 mg/min; maximum cumulative dose of 300 mg over 24 h Nitroprusside* 0.5 μg/kg/min; titrate as tolerated to maximum of 2 μg/kg/min Esmolol Loading dose of 500 μg/kg over 1 min, followed by an infusion at μg/ kg/min, which may be increased by 25 μg/kg/min every min until the desired response to a maximum of 300 μg/kg/min Fenoldopam An initial dose of 0.1 μg/kg/min, titrated by increments of μg/kg/min to a maximum of 1.6 μg/kg/min *Most commonly used. Reprinted with permission from Chest. 2000;118: Varon J, Marik PE. The diagnosis and management of hypertensive crises. Chest. 2000;118: Calhoun DA, Oparil S. Treatment of hypertensive crisis. N Engl J Med. 1990;323: Murphy MB, Murray C, Shorten GD. Fenoldopam a selective peripheral dopamine-receptor agonist for the treatment of severe hypertension. N Engl J Med. 2001;345: VOL. 8 NO. 4 APRIL 2006 THE JOURNAL OF CLINICAL HYPERTENSION 295