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b00d2bdb-66e1-41ed-90b4-c52904f4d598 Seth Kligerman, MD, MS
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Cardiac 178f7921-c162-43ee-b7ae-313021635390 4 01/28/25 Intimointimal Intussusception Cardiac, Diagnosis, Aorta, Intimointimal Intussusception Intimointimal Intussusception | STATdx Intimointimal Intussusception DX true
Cardiac
Diagnosis
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Intimointimal Intussusception

title: "Intimointimal Intussusception" docid: "a7004bbb-3699-4ed4-af21-78f51a3685d9" authors:

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  • "Cardiac"
  • "Diagnosis"
  • "Aorta"
  • "Intimointimal Intussusception"

KEY FACTS

  • Terminology

    • Complete circumferential intimal tear in type A > type B aortic dissection
    • Delaminated intima then rolls upon itself

  • Imaging

    • Best visualized with ECG-gated CTA
    • Circumferential intimal tear → unidirectional or bidirectional intimointimal intussusception (IIIS) - Anterograde (43%): Intima superior circumferential intimal tear delaminates upward - Intimal often folded upon itself in aortic arch - Retrograde (56%): Intima inferior to circumferential tear delaminates inferiorly - Intimal may prolapse into LV during diastole - Bidirectional (1%): IIIS both anterograde and retrograde
    • Between delaminated intima that has intussuscepted superiorly &/or inferiorly, portion of aorta appears "normal" but actually lacks intima - Missing flap or naked aorta sign

  • Clinical Issues

    • Diagnosis not prospectively made before surgical intervention in 49% of anterograde IIIS and 16% of retrograde IIIS
    • Anterograde - Chest pain - Neurologic symptoms (62%) - Asymmetric blood pressure (57%)
    • Retrograde - Myocardial infarction (26.5%) - Severe aortic regurgitation
    • Mean age: 54 years
    • M > F
    • Overall mortality: 24%
    • Treatment is surgical repair

TERMINOLOGY

  • Abbreviations

    • Intimointimal intussusception (IIIS)

  • Definitions

    • Complete circumferential intimal tear in type A aortic dissection; delaminated intima then rolls upon itself like tube sock; given this is circumferential tear, intima can intussuscept anterograde (upward) into aortic arch (AA)/descending thoracic aorta (DTA) &/or retrograde (downward) into aortic root and left ventricle (LV)

IMAGING

  • General Features

    • Best diagnostic clue

      - Type A dissection where layers of intimal are folded in on itself within ascending &/or DTA

    • Location

      - Ascending aorta or AA (98%)
- DTA (2%)

  • Radiographic Findings

    • No specific radiographic findings are visible other than findings associated with type A dissection

  • CT Findings

    • Best visualized with ECG-gated CTA
    • Findings of type A dissection in most cases
    • Circumferential intimal tear leads to either unidirectional or bidirectional IIIS - Anterograde (43%): Intima superior to level of circumferential intimal tear delaminates upward into AA and DTA - Intimal is often seen folded upon itself in AA or DTA - Intima may obstruct arch vessels leading to neurologic sequela - Retrograde (56%): Intima inferior to level of circumferential tear delaminates inferiorly into aortic root and may prolapse into LV during diastole - Bidirectional (1%): IIIS both anterograde and retrograde
    • Between delaminated intima that has intussuscepted superiorly &/or inferiorly, there is portion of aorta that appears normal, i.e., free of dissection flap - This portion of aorta lacks intima - Sometimes called missing flap or naked aorta sign
    • Anterograde IIIS can involve entire thoracic aorta and extend into abdominal aorta or into iliac arteries - IIIS occluding superior mesenteric artery (SMA) can lead to mesenteric ischemia

  • MR Findings

    • Few case reports of IIIS visualized with MR
    • Best seen with bright-blood imaging, such as gated SSFP imaging or contrast-enhanced MRA
    • Similar findings to CT with unidirectional or bidirectional intima that has rolled upon itself superiorly &/or inferiorly

  • Echocardiographic Findings

    • May see portion of intimal flap prolapsing into LV - 71% in retrograde IIIS
    • Intimal flap in aortic root
    • Aortic regurgitation; often severe - 84% in retrograde IIIS

  • Imaging Recommendations

    • Best imaging tool

      - ECG-gated CTA

DIFFERENTIAL DIAGNOSIS

  • Type A Dissection Without Intimointimal Intussusception

    • Will not see delaminated intimal folded on itself in aortic lumen

CLINICAL ISSUES

  • Presentation

    • Most common signs/symptoms

      - Anterograde
        - Chest pain
        - Neurologic symptoms (62%)
                  - Cerebrovascular accident
                  - Syncope
        - Asymmetric blood pressure (57%)
                  - Likely due to subclavian artery occlusion
- Retrograde
        - Chest and back pain
        - Dyspnea
        - Myocardial infarction (26.5%)
        - Aortic regurgitation (84%); often severe

  • Demographics

    • Mean age: 54 years
    • M > F

  • Natural History & Prognosis

    • Overall mortality: 24% - Preoperative and postoperative mortality of 14% and 9%, respectively - Similar mortality for anterograde and retrograde IIIS

  • Treatment

    • Diagnosis not prospectively made before surgical intervention in 49% of anterograde IIIS and 16% of retrograde IIIS
    • Aortotomy and surgical repair - Various techniques, including Bentall repair, ascending aortic and arch replacement - Intussuscepted intima may be retracted upward &/or downward and tacked down to aorta

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References

Selected References

  1. Dokollari A et al: Aortic intimo-intimal intussusception in Stanford type A acute aortic dissection. Eur Heart J. 42(34):3410, 2021
  2. Wu ZY et al: Aortic Intimo-intimal intussusception: a pooled analysis of published reports. Ann Vasc Surg. 75:471-8, 2021
  3. Thunberg CA et al: Echocardiographic detection of intimo-intimal intussusception in a patient with acute Stanford type A aortic dissection. Ann Card Anaesth. 18(2):227-30, 2015
  4. Sanders LH et al: Radiological diagnosis and classification of antegrade and retrograde Stanford type A intimal intussusception. Int J Cardiovasc Imaging. 23(5):659-65, 2007
  5. Fan ZM et al: Acute aortic dissection with intimal intussusception: MRI appearances. AJR Am J Roentgenol. 186(3):841-3, 2006

Images

Selected Images

Type A aortic dissection with bidirectional intimointimal intussusception (IIIS) is shown. At the point of circumferential intimal tear, the delaminated intima rolls anterograde into the aortic arch and retrograde into the left ventricle. Type A aortic dissection with bidirectional intimointimal intussusception (IIIS) is shown. At the point of circumferential intimal tear, the delaminated intima rolls anterograde into the aortic arch and retrograde into the left ventricle.

Type A aortic dissection with bidirectional intimointimal intussusception (IIIS) is shown. At the point of circumferential intimal tear, the delaminated intima rolls anterograde into the aortic arch and retrograde into the left ventricle. Type A aortic dissection with bidirectional intimointimal intussusception (IIIS) is shown. At the point of circumferential intimal tear, the delaminated intima rolls anterograde into the aortic arch and retrograde into the left ventricle.

Sagittal oblique CECT shows the anterograde IIIS folded upon itself in the proximal descending thoracic aorta . Intimal calcifications can be seen. Sagittal oblique CECT shows the anterograde IIIS folded upon itself in the proximal descending thoracic aorta . Intimal calcifications can be seen.

Echo image during systole (left) shows a portion of the delaminated intima in the ascending aorta . During diastole (right), there is retrograde IIIS into the left ventricle . Echo image during systole (left) shows a portion of the delaminated intima in the ascending aorta . During diastole (right), there is retrograde IIIS into the left ventricle .

CTA in a 31-year-old man with chest and arm pain shows a type A dissection with a circumferential intimal tear and flap in the aortic root. The anterograde IIIS has lodged into and occluded the left subclavian artery . Only a small portion of intima is in the aortic arch . CTA in a 31-year-old man with chest and arm pain shows a type A dissection with a circumferential intimal tear and flap in the aortic root. The anterograde IIIS has lodged into and occluded the left subclavian artery . Only a small portion of intima is in the aortic arch .

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