What is the management guideline for a patient with an aneurysm of the descending aorta accompanied by penetrating aortic ulcer (PAU) and intramural hematoma (IMH)?

Management of Descending Aortic Aneurysm with Concomitant PAU and IMH

In patients with a descending thoracic aortic aneurysm accompanied by both penetrating aortic ulcer (PAU) and intramural hematoma (IMH), immediate aggressive medical therapy with blood pressure control (systolic <120 mmHg) and heart rate control (≤60 bpm) is mandatory, followed by urgent thoracic endovascular aortic repair (TEVAR) given the high-risk combination of pathologies that significantly increases risk of rupture and progression. 1, 2

Initial Medical Management

Hemodynamic Control (Immediate Priority)

• Initiate intravenous beta-blockers immediately to achieve target heart rate ≤60 beats per minute before addressing blood pressure, as vasodilators given before heart rate control can provoke reflex tachycardia that increases aortic wall stress 2
• For patients with contraindications to beta-blockers, use non-dihydropyridine calcium channel blockers as an alternative 2
• Target blood pressure is systolic <120 mmHg and diastolic <80 mmHg 2
• After achieving adequate heart rate control, if systolic blood pressure remains >120 mmHg, add intravenous angiotensin-converting enzyme inhibitors and/or other vasodilators 2
• Provide adequate analgesia as pain control is essential and uncontrolled pain may indicate disease progression 1

Intensive Monitoring

• Admit to intensive care unit for continuous hemodynamic monitoring 3
• Serial clinical assessment for signs of progression including recurrent pain, hemodynamic instability, or malperfusion 1

Risk Stratification and Imaging

High-Risk Features Mandating Intervention

The combination of aneurysm, PAU, and IMH represents a particularly high-risk scenario. The presence of PAU with associated IMH significantly increases the risk of treatment failure and adverse outcomes compared to isolated PAU 4, 5. Specific high-risk imaging features include:

• Maximum PAU width ≥13-20 mm 1
• Maximum PAU depth ≥10 mm 1, 5
• IMH thickness >10 mm 5
• Aortic diameter >40 mm (aneurysmal dilatation) 5
• PAU associated with saccular aneurysm 1
• Increasing pleural effusion 1
• Significant growth of PAU width or depth >5 mm/year 1

Diagnostic Imaging

• Contrast-enhanced computed tomography (CT) is the diagnostic technique of choice with 96% sensitivity for detecting IMH 1, 6, 7
• Perform immediate CT imaging without delay to assess extent of disease and identify high-risk features 1, 7
• Alternative modalities include cardiac magnetic resonance (CMR) or transesophageal echocardiography (TEE) based on availability 1

Definitive Treatment Strategy

Indication for TEVAR

Given the combination of aneurysm with both PAU and IMH in the descending aorta, this represents a complicated type B acute aortic syndrome requiring endovascular intervention 1. The rationale includes:

• Complicated type B PAU with IMH warrants TEVAR as first-line therapy 1, 2
• The presence of aneurysmal dilatation with PAU and IMH creates a particularly unstable substrate with high rupture risk 3, 5
• Patients with PAU and associated IMH have significantly increased risk of TEVAR treatment failure compared to isolated PAU, but this still represents the best treatment option 4
• The coexistence of aneurysm and IMH warrants aggressive surgical approach 3, 5

Timing of Intervention

• Urgent TEVAR should be performed once hemodynamic stability is achieved rather than delayed elective repair 1
• Persistent or recurrent pain despite medical therapy mandates immediate intervention 1, 3, 5
• Evidence of disease progression on repeat imaging (hematoma expansion, periaortic hematoma, intimal disruption) requires urgent treatment 1

Alternative to TEVAR

• Open surgical repair may be considered based on unfavorable anatomy for endovascular repair or patient-specific factors, though TEVAR is preferred for type B disease 1

Critical Pitfalls to Avoid

• Do not initiate vasodilator therapy before achieving heart rate control, as this increases aortic wall stress through reflex tachycardia 2
• Do not pursue conservative medical management alone in the setting of aneurysm with both PAU and IMH, as this combination has malignant potential 3, 5
• Do not delay imaging when clinical progression is suspected, as the combination of these pathologies carries 33-75% rupture risk when complicated 1
• Recognize that the presence of IMH with PAU adversely affects late outcomes and necessitates attentive long-term follow-up even after successful TEVAR 4

Long-Term Management Post-Intervention

Surveillance Imaging

• Follow-up imaging at 1,3,6, and 12 months after intervention, then yearly if stable 1
• Use CT or CMR for surveillance 1

Medical Therapy

• Continue strict blood pressure control indefinitely with target systolic <120 mmHg 2
• Maintain heart rate control with beta-blockers 2
• Initiate moderate- to high-intensity statin therapy with LDL-C goal <55 mg/dL (<1.4 mmol/L) given atherosclerotic disease 2
• Consider low-dose aspirin (75-162 mg/day) to reduce cardiovascular events 2

Monitoring for Complications

• Actuarial freedom from treatment failure is approximately 81.6% at 5 years for PAU treated with TEVAR, with lower rates when IMH is present 4
• Assess for endoleak, stent graft migration, or disease progression in untreated aortic segments 4
• Monitor for late aneurysm formation or dissection progression 1, 3