Aortic intimo-intimal intussusception: A rare variant of aortic dissection

MN Dilip; A George Koshy; Mathew Iype; K Sunitha Viswanathan

doi:10.4103/jiae.jiae_40_18

INTERESTING CASE REPORT

Year : 2019 | Volume : 3 | Issue : 2 | Page : 104-106

Aortic intimo-intimal intussusception: A rare variant of aortic dissection

MN Dilip, A George Koshy, Mathew Iype, K Sunitha Viswanathan
Department of Cardiology, Government Medical College, Thiruvananthapuram, Kerala, India

Date of Web Publication

29-Aug-2019

Correspondence Address:
M N Dilip
9G, SFS Eternia, NH-47, Koonamthai, Opposite KIMS Hospital, Edapally, Kochi - 682 033, Kerala
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_40_18

Abstract

This report describes a very rare and life-threatening form of Stanford Type A circumferential aortic dissection causing intimo-intimal intussusception. Rapid and accurate diagnosis is possible by transesophageal echocardiography for which the features have been described in this article.

Keywords: Aortic dissection, aortic regurgitation, transesophageal echocardiography

How to cite this article:
Dilip M N, Koshy A G, Iype M, Viswanathan K S. Aortic intimo-intimal intussusception: A rare variant of aortic dissection. J Indian Acad Echocardiogr Cardiovasc Imaging 2019;3:104-6

How to cite this URL:
Dilip M N, Koshy A G, Iype M, Viswanathan K S. Aortic intimo-intimal intussusception: A rare variant of aortic dissection. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2019 [cited 2020 Jul 10];3:104-6. Available from: http://www.jiaecho.org/text.asp?2019/3/2/104/265755

Introduction

Aortic dissection is a catastrophic manifestation of acute aortic syndrome(s). Aortic dissection involving the ascending aorta reportedly has a mortality rate of around 1% per hour. Here we describe a potentially lethal form of aortic dissection as a result of circumferential tear between aortic intima and media.

Clinical Presentation

A 55-year-old man was referred to our institute with a history of right parasternal chest pain for the last 3 days, initially mild to begin with, crescendo in nature. For this, he was treated at a local hospital and pain got temporarily relieved with analgesics. On the next day, the patient again developed similar pain along with acute onset dyspnea followed by orthopnea and diaphoresis for which he was referred to our hospital.

On admission, the patient was in severe distress with a pulse rate of 130/min and a blood pressure of 110/60 mmHg. His jugular venous pressure was elevated just above the root of the neck. On auscultation, the patient had a left ventricular (LV) third heart sound and a grade 2 early diastolic murmur at the aortic area. He had pulmonary congestion with a saturation of 90% at room air. All the peripheral pulses were palpable equally.

Electrocardiogram revealed sinus tachycardia, no apparent ST-T changes, or LV hypertrophy. His chest X-ray taken in the emergency department showed features of pulmonary edema with changes of chronic obstructive pulmonary disease and no cardiomegaly. There was no any history of coronary artery disease, diabetes, or hypertension.

The patient was shifted to the intensive coronary care unit, and an immediate transthoracic echocardiography (echo) was performed. The patient was very restless, but we managed to infer that the patient had severe aortic regurgitation (AR) and a good LV function. A slender hypermobile structure was seen attached to the aortic root in the parasternal long-axis view but could not be further characterized [Figure 1]. Further, in the short-axis view, it appeared as a bicuspid aortic valve with redundant leaflets [Figure 2]. At that point of time, we had a differential diagnosis of ascending aortic dissection versus bicuspid aortic valve with ruptured aortic root abscess.

Figure 1: Slender abnormal structure seen (red arrow). Parasternal long-axis image in transthoracic echocardiography

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Figure 2: Parasternal short-axis image in transthoracic echocardiography

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Hence, we proceeded for urgent transesophageal echo for the patient. Mid-esophageal aortic long-axis view revealed a very short and cylindrical-shaped intimal flap which fell into the left ventricle during diastole causing severe AR [Figure 3] and [Figure 4]. The intimal flap is later ejected back into ascending aorta during ventricular systole [Figure 5] and [Figure 6]. The back-and-forth movement of the cylinder-shaped intima known as the “skipping rope sign” [Video 1] as it prolapses into the LV and thrusts into the aorta during diastole and systole, respectively, is suggestive of retrograde intussusception into the LV outflow tract. Unfortunately, our patient succumbed just while performing the transesophageal echo and could not be revived despite all our efforts. Further imaging of the short axis of the aorta could not be performed in our patient. Aortic short-axis view would have given an excellent image of “barrel in barrel” aorta which is another diagnostic sign of glove-finger intimo-intimal intussusception (III) [Figure 7].

Figure 3: Aortic long-axis transesophageal image of the aortic sinus and ascending aorta in diastole. Note the flap prolapses into the left ventricle

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Figure 4: Aortic long-axis transesophageal image with color flow showing severe aortic regurgitation

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Figure 5: Aortic long-axis transesophageal image of the aortic sinus and ascending aorta in systole. Note the flap is pushed into the ascending aorta

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Figure 6: Pictorial representation of our case of ascending aortic dissection with intimo-intimal intussusception in systole. Note that the flap does not reach up to the arch vessels and hence there was no pulse asymmetry

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Figure 7: Pictorial representation of echocardiographic projection of the ascending aorta in its short axis. Note the circumferential dissection in the ascending aorta

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Discussion

The term aortic III was described by Hufnagel and Conrad ^[1] in 1962 which is a fatal form of Stanford Type A aortic dissection. The term “III” here refers to a circumferential detachment of the intima from the media, forming a loose cylinder inside the aorta. It is a condition that necessitates prompt diagnosis and management. If untreated, the mortality rates have been reported as high as 60% in the first 24 h, 80% within the first 15 days, and 90% within 3 months.^[2]

This syndrome was described over 120 years ago in the German literature by Bostroem in 1887 and Chiari 1909 as inversion of the internal cylinder which involves a circular detachment of the intima to form an intimal “cylinder” inside the aorta with a “windsock” appearance.^[3] In severe cases, the flap prolapses into the aorta which can lead to severe AR or coronary obstruction leading to rapid hemodynamic collapse. In other cases, the flap may swing distally obstructing arch vessels, and cerebral malperfusion may ensue. As in our case, the patient had severe AR, but there was no evidence of compromise in coronary circulation or involvement of arch vessels probably because only aortic sinus and proximal part of the ascending aorta were involved. It is believed that the anomalous occurrence of intimal intussusception after aortic dissection is due to the maintained elastic property of the intima in patients who are most often young or without much risk factors and whose cardiac performance is good.^[4],[5],[6]

As the symptoms overlap substantially with other common illness such as acute coronary syndromes, suspecting Type A aortic dissection may not be easy. However, early recognition and urgent surgical intervention are of utmost importance for a favorable outcome. High index of suspicion for aortic dissection in patients presenting with acute chest pain or features of acute AR or pulse asymmetry along with prompt use of imaging modalities such as echo,^[7] computed tomography (CT) (triple rule out), or magnetic resonance angiography may help in early diagnosis and offer a better chance of survival for the patient. There have been case reports where CT and transthoracic echo failed to detect an intimal flap or a false lumen.^[2],[7] Hence, transesophageal echo is considered as the gold standard in detection of these cases.^[7]

Conlcusion

Although intimal intussusception is a rare complication of aortic dissection, it may have grave implications if unrecognized. As in this case, if suspected, intussusception may be readily identifiable by transesophageal echo examination. In general, patients with this type of tear are critically ill and require an emergency surgery, which carries a very high operative mortality of 40%.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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3.	Yang EH, Kwon MH, Mahajan A, Child JS, Tobis JM, Manthripragada G, et al. Circumferential type A aortic dissection and intimal intussusception of the aorta causing severe aortic regurgitation and obstruction of the left main coronary artery. Echocardiography 2013;30:E81-4.
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