Author + information
- Xun Yuan, MBBS, MMEDa,b,
- Jeffrey Shi Kai Chan, MBChBc,d,
- Andreas Mitsis, MDa and
- Christoph A. Nienaber, MD, PhDa,b,∗ ()
- aCardiology and Aortic Centre, Royal Brompton Hospital and Harefield NHS Foundation Trust, Imperial College London, London, United Kingdom
- bDepartment of Cardiology, National Heart and Lung Institute, Faculty of Medicine, Imperial College London, London, United Kingdom
- cFaculty of Medicine, The Chinese University of Hong Kong, Shatin, Hong Kong
- dDivision of Cardiology, Department of Medicine and Therapeutics, Prince of Wales Hospital, Shatin, Hong Kong
- ↵∗Address for correspondence:
Dr. Christoph A. Nienaber, Cardiology and Aortic Centre, Royal Brompton Hospital and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College London, Sydney Street, London SW3 6NP, United Kingdom.
A 64-year-old man was admitted for crushing chest pain. Computed tomography revealed an aortic intramural hematoma, with progression to a penetrating aortic ulcer on day 7 and aortic dissection on day 30. The patient was treated with strict blood pressure control and thoracic endovascular aortic repair. He was discharged 3 days post-operatively. (Level of Difficulty: Beginner.)
- acute aortic syndrome
- intramural hematoma
- penetrating atherosclerotic ulcer
- type B aortic dissection
History of Presentation
A 64-year-old man presented with a single episode of crushing chest pain at rest. He was hemodynamically stable and afebrile. Cardiovascular and neurological examinations were unremarkable. He had no stigmata of connective tissue disorder.
• Acute aortic syndrome should be included in the differential diagnosis in a patient presenting with chest pain.
• Clinicians should understand the progression of aortic intramural hematoma to penetrating aortic ulcer and aortic dissection.
• Clinicians should understand the need for close surveillance and the principles of management in cases of aortic intramural hematoma.
Past Medical History
The patient had a long-standing history of uncontrolled hypertension and was a long-term smoker. His family history was unremarkable.
Differential diagnoses included acute coronary syndrome, coronary heart disease, and acute aortic syndrome.
Transthoracic echocardiography and coronary angiography were performed to rule out acute coronary syndrome and coronary artery disease. An electrocardiogram-gated computed tomography (CT) scan revealed aortic intramural hematoma (IMH) encompassing the distal aortic arch and the descending aorta with no visible communications, ulcers, or dissection (Figures 1A1 and 1A2).
The patient was admitted for close surveillance and strict blood pressure management with oral valsartan and amlodipine, and his systolic blood pressure was kept between 100 and 110 mm Hg. Despite adequate medical management, a follow-up CT scan on day 7 showed clear progression of the IMH to a penetrating aortic ulcer at the descending aorta, with suspected progression to overt aortic dissection (Figures 1B1 and 1B2). On day 30, a CT scan demonstrated progression of the penetrating aortic ulcer to an overt type B aortic dissection with the formation of a pseudoaneurysm. Nonetheless, the IMH at the distal aortic arch had resolved and resorbed spontaneously (Figures 1C1 and 1C2). The patient remained asymptomatic throughout this course.
With evidence of focal degeneration and a total diameter of 55 mm, thoracic endovascular aortic repair (TEVAR) was carried out using general anesthesia and guidance by transesophageal echocardiography and fluoroscopy (Figures 1D1 and 1D2). Complete sealing of the stent graft was confirmed by digital subtraction angiography and ultrasound examination with Doppler interrogation. The patient was discharged 3 days post-operatively.
A follow-up CT scan at 3 months post-operatively showed a static aortic size with a thrombosed false lumen of the aortic dissection. No endoleak was detected.
IMH is a variation of aortic dissection and is considered a phenotypic form in the spectrum of acute aortic syndromes. With an incidence of 1.2 per 100,000 person-years (1), IMH has been associated with significantly higher cardiovascular risks (2), and it carries an in-hospital or 30-day mortality rate of 4% to 19%, 11% to 24%, and 0% to 6% when treated by medical therapy, open surgery, and TEVAR, respectively (3).
This case report showed that IMH may manifest with nonspecific symptoms, and without any specific physical finding on examination. Therefore, a low clinical threshold of suspicion is required. CT has been recommended in the evaluation of suspected acute aortic syndromes by both the 2010 U.S. guideline and the 2014 European guideline (4,5). Because of the potential of IMH to progress to more serious disease, serial CT has been recommended and was crucial in the management of our patient.
According to the aforementioned guidelines, management of IMH is similar to that of aortic dissection, with an emphasis on analgesia, blood pressure control, and prevention of progression to overt aortic dissection and rupture in patients with type B IMH (4,5). However, this report demonstrated that despite adequate blood pressure control with medical therapy, progression of IMH to aortic dissection may still occur rapidly. As such, we managed the patient with TEVAR, which was recommended by both guidelines.
Such similarities between the management of IMH and aortic dissection, and the progression of IMH to penetrating aortic ulcer and aortic dissection, highlight the overlap between these entities, even though their pathogenesis is incompletely understood. Of note, the differentiation between penetrating atherosclerotic ulcer (PAU) and ulcer-like projection (ULP) has caused confusion in recent decades because of their similar appearance on CT images. However, PAU and ULP have different pathogenesis and course of progression; PAU is usually less malignant, whereas ULP is unrelated to atherosclerosis and is usually a result of IMH progression (6). In our case, the penetrating aortic ulcer that resulted from the IMH was likely a ULP. The presence of ULP has been associated with poorer outcomes, and it further supported our close imaging surveillance of the patient (5).
Aortic intramural hematoma may manifest nonspecifically and progress rapidly to a penetrating aortic ulcer and overt aortic dissection. A low clinical threshold of suspicion is required to detect acute aortic syndromes, and close imaging surveillance is an essential part of management.
Dr. Mitsis has received a training grant from Medtronic. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received July 25, 2019.
- Accepted August 7, 2019.
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