Ann Thorac Surg 2000;70:683-689
© 2000 The Society of Thoracic Surgeons
Collective review
Spontaneous rupture of the thoracic aorta
Hitoshi Yokoyama, MD, PhDa,
Mikio Ohmi, MDa,
Mitsuaki Sadahiro, MDa,
Yoshimi Shoji, MDa,
Koichi Tabayashi, MDb,
Yoshimasa Moizumi, MDa
a Department of Cardiovascular Surgery, Tohoku University School of Medicine, Sendai, Japan
b Department of Cardiovascular Surgery, Sendai Medical Center, Sendai, Japan
Address reprint requests to Dr Yokoyama, Department of Cardiovascular Surgery, Tohoku University School of Medicine, 1–1 Seiryo-cho, Sendai 980–8574, Japan
e-mail: hitoshiy{at}mail.cc.tohoku.ac.jp
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Abstract
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Background. Spontaneous rupture of the thoracic aorta without trauma, aneurysm, or dissection is an extremely rare but catastrophic disorder. Two cases of spontaneous aortic rupture are presented, both treated surgically with satisfactory results.
Methods. A review of the English literature found 16 patients with the diagnosis of spontaneous rupture of the thoracic aorta from 1961 through 1998. Eighteen reported cases, including the 2 cases presented herein, are reviewed.
Results. The representative clinical picture is one of a middle-aged hypertensive patient with acute chest pain and collapse, with imaging modalities demonstrating hemopericardium, hemomediastinum, or hemothorax. According to the reported experiences, aortography was accurate for identifying the rupture site although the findings were sometimes subtle. Misdiagnosis or nonsurgical management resulted in the patient’s death. All 8 patients who did not undergo aortic repair died within 3 weeks after the onset, whereas 9 of 10 patients who underwent surgical aortic repair survived.
Conclusions. For patients with a definitive or possible diagnosis of spontaneous rupture of the thoracic aorta, prompt operation is imperative through an optimal surgical approach to identify and repair the rupture site with appropriate circulatory support.
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Introduction
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Nontraumatic spontaneous rupture of the thoracic aorta without aneurysm or dissection is an extremely rare but fatal event, only 16 cases of which have been reported in the English literature [1–15]. Herein we report 2 cases of spontaneous aortic rupture, both treated by graft replacement with satisfactory results. A collective literature review is also provided to elucidate the clinicopathologic picture of this catastrophic disorder along with considerations of the optimal management for these critically ill patients.
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Case reports
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Patient 1
A 56-year-old man who had been hospitalized for the treatment of pneumonia suddenly collapsed on December 27, 1987. He had no history of hypertension. His chest roentgenogram showed left pleural effusion. Immediately he was transferred to the Sendai Medical Center with clinical diagnosis of acute hemothorax. On his arrival, he was in stable condition with a blood pressure of 120/78 mm Hg and a pulse of 78 beats per minute. An electrocardiogram showed regular sinus rhythm without any evidence of myocardial ischemia. His white blood cell count was 17,000/µL and his hemoglobin was 14.6 g/dL. A chest computed tomography (CT) scan revealed left hemothorax (Fig 1A). Urgent aortography showed no particular findings without any apparent evidence of aortic aneurysm or dissection. At that time his clinical diagnosis was left hemothorax of unknown origin.
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Fig 1. (A) Computed tomography demonstrates left hemothorax without aneurysm or dissection in the thoracic aorta. (B) Aortography demonstrates subtle irregularity on the distal aortic arch (arrows).
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The patient was observed conservatively at bed rest for 4 weeks while the left hemothorax disappeared gradually and his pneumonia resolved. After 2 months, his CT scan showed no left hemothorax; however, repeated aortography showed intimal irregularity on the proximal descending aorta (Fig 1B), which first led us to a diagnosis of spontaneous aortic rupture. He underwent an operation through a left thoracotomy, and the proximal descending aorta was explored on cardiopulmonary bypass. The posterior wall of the aorta showed a shallow intimal defect of 15 x 15 mm, which was considered as the rupture site. The aortic wall showed minimal arteriosclerosis without any aneurysm or dissection. The proximal descending aorta was replaced with a Dacron tube graft. The patient had an uneventful postoperative recovery and has been doing well during 11 years of follow-up.
Patient 2
A 67-year-old man with a history of hypertension presented with sudden chest pain and was transferred to a municipal hospital. His chest roentgenogram showed an enlarged mediastinum (Fig 2A). With a clinical diagnosis of possible acute aortic dissection, he was transferred to Tohoku University Hospital on a continuous infusion of antihypertensive agent. On his arrival, he was in a stable condition with a blood pressure of 120/65 mm Hg and a pulse of 70 beats per minute although he still complained of chest pain. A chest CT revealed massive hematoma in the anterior mediastinum adjacent to the aortic arch (Fig 2B). Two-dimentional transthoracic echocardiography showed a low-echo space around the aortic arch corresponding to the mediastinal hematoma and echo-free space on the wall of distal arch that was interpreted as disrupted aortic wall.
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Fig 2. (A) Chest roentgenogram shows marked mediastinal widening. (B) Contrast-enhanced computed tomography demonstrates massive hematoma in the anterior mediastinum without aneurysm or dissection in the thoracic aorta.
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In an urgent operation through a median sternotomy, a massive hematoma was observed around the distal aortic arch. On cardiopulmonary bypass and selective cerebral perfusion [16] with moderate hypothermia, the aortic arch was opened. A transmural tear (10 x 10 mm) of the aortic arch was found just distal to the ligamentum arteriosus. Neither macroscopic atheromatous plaque, aortic dissection, nor aneurysm was noticed. Total aortic arch replacement with a branched Dacron graft was performed, and surgery was followed by an uneventful postoperative course. The pathologic finding for the resected aortic wall was minimal arteriosclerosis. During 10 months of follow-up, the patient has done well.
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Literature review
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In reviewing the English literature relative to spontaneous rupture of the thoracic aorta, first we searched MEDLINE (1966 to present) using the keywords aorta, rupture, spontaneous, and thoracic. Patients with other etiologies such as aneurysm, aortic dissection, intramural hematoma, penetrating arteriosclerotic ulcer, infection, inflammation, neoplasm, chest trauma, or high-speed accident were excluded. Cases of dilated ascending aorta with aortic regurgitation were also excluded, as was a patient reported in a pathology journal who was found dead without any detailed clinical information [15]. We also referred to cases listed in the recent literature reviews [10, 14] in addition to the database search. The closing date of this report was December 31, 1998.
Clinical presentation
Spontaneous rupture of the thoracic aorta without trauma, aneurysm or dissection is extremely rare. To our best knowledge, only 16 cases have been reported in the English literature since Rodriguez and Rivera [1] reported the first case in 1961. In the 18 cases reviewed, including the present 2 cases (Table 1), mean age was 63 years and ranged from 47 to 79 years. Twelve cases (67%) were male and 6 cases (33%) were female. Thirteen patients (72%) had a history of hypertension, 2 had a long history of rheumatoid arthritis with steroid therapy, and 3 had no associated comorbidity. The majority of the patients had severe acute chest or back pain (78%) associated with shock or collapse (78%). The rupture site was the ascending aorta in 8 patients, the arch in 4, and the descending aorta in 6.
Diagnostic methods
The rupture of the ascending aorta resulted in acute hemopericardium with enlarged cardiac silhouette on chest roentgenogram, and subsequent echocardiography or computed tomography (CT) effectively revealed the cardiac effusion. The rupture of the transverse arch resulted in mediastinal hemorrhage with mediastinal widening on chest roentgenogram. CT was also effective for demonstrating the mediastinal hematoma. In 1 of the cases reviewed (patient 17), two-dimentional echography effectively revealed the rupture site on the distal arch. The rupture of the descending aorta resulted in left hemothorax in most cases along with occasional mediastinal or left pulmonary hemorrhage. Thus chest roentgenogram and CT are less-time consuming, noninvasive modalities that can demonstrate hemorrhage around the thoracic aorta; however, they are not able to identify the rupture site accurately. Aortography was performed in 9 cases (50%), and identified the rupture site in 7 cases. The findings obtained with aortography, however, varied over a wide range, from subtle change to apparent disruption or extravasation of the contrast media. Two cases, including 1 of our series, only showed slight irregularity of the aortic wall. Although the reported experiences demonstrate that aortography is still an effective measure to identify the rupture site, the subtle abnormalities on the aortic wall should not be overlooked.
Although no reports in this review mentioned other imaging modalities such as transesophageal echocardiography (TEE) and magnetic resonance imaging (MRI), these recent devices have been recognized to well demonstrate the discontinuity of the thoracic aortic wall [17]. TEE is now the standard method of assessing the presence and type of acute thoracic aortic dissection and is probably preferable to aortography in many medical centers. TEE can yield high-resolution, real-time axial and longitudinal images of the thoracic aorta except for the upper ascending aorta around the trachea referred to as "blind spot [18]." MRI does not require the iodinated contrast material and provides multiplanar high-resolution images of the thoracic aorta. However its imaging time, incompatibilities with monitoring device and inaccessibility of the patients during examination preclude its use for the patient in an unstable condition. If the patient is awake, alert and stable, MRI is a valuable device for evaluation of the aortic wall abnormalities.
Etiology
The pathologic findings from the ruptured aorta were arteriosclerosis in the majority of the cases (67%). Cystic medial necrosis was observed in 2 cases. In 2 patients with long-term steroid usage the ruptured aortic walls looked thin and fragile as "wet tissue paper" [5]. Thus the leading etiology of the spontaneous aortic rupture is arteriosclerosis with longstanding hypertension. The relationship between aortic arteriosclerosis and spontaneous rupture is speculative. Aortic aneurysm is most commonly associated with arteriosclerotic lesions, with subsequent weakening of the media and expansion of the involved area. Castleman and McNeely [2] speculated that perforation might be due to pressure atrophy of the media by the intimal arteriosclerosis with localized ballooning of the wall, or "mini-aneurysm," before perforation.
Aortic dissections are thought to be the result of cystic medial necrosis with an associated intimal tear or medial hemorrhage leading to false lumen separating the intima and adventitia. Intimal rupture extending into media of the aortic wall is seen in aortic dissection. However, intimal rupture can extend through the aortic adventitia causing spontaneous aortic rupture rather than leading to aortic dissection. Recently penetrating arteriosclerotic ulcer was proposed by Stanson and associates [19] as an atheromatous ulceration that disrupts the intimal elastic lamina and underlying media with accompanying rupture, medial hemorrhage, or pseudoaneurysm formation. Even the aortic wall with minimal arteriosclerosis could have mini-penetrating arteriosclerotic ulcer, which could lead to fatal aortic rupture.
Another aortic pathologic entity in the CT era, intramural hematoma, was recently described as spontaneous localized hemorrhage into the aortic wall in the absence of aortic dissection, intimal tear or penetrating arteriosclerotic ulcer. Intramural hematoma is thought to be caused by primary vasa vasorum hemorrhage within the aortic media or rupture of the arteriosclerotic plaque [20]. The aortic rupture through a small intramural hematoma with ruptured arteriosclerotic plaque could have been interpreted clinically as "spontaneous" aortic rupture. Since sensitive diagnostic imaging methods such as CT, MRI, TEE, and intravascular ultrasonography (IVUS) have been used for patients with signs and symptoms suggesting aortic dissection, the entity of intramural hematoma is likely to be recognized with increasing frequency in the future. Precise histopathologic studies of the affected aorta are necessary for the distinction between spontaneous aortic rupture and aortic rupture due to penetrating arteriosclerotic ulcer or intramural hematoma.
Surgical treatment
Preoperatively, accurate diagnosis of spontaneous rupture of the thoracic aorta was extremely rare. Reported preoperative clinical diagnoses were aortic dissection, acute myocardial infarction, acute hemopericardium, or hemothorax of unknown origin. Misdiagnosis with nonsurgical management likely resulted in the patient’s death. All 8 patients who did not undergo surgery died within 3 weeks of onset. Two died on the day of onset. Eleven patients underwent surgery; however, 1 patient underwent a diagnostic exploration of left thorax and pericardial drainage and died. Pericardial drainage in 3 patients temporarily improved their condition, which was, however, eventually followed by sudden death. Ten patients underwent aortic repair, with 9 patients surviving and 1 death due to postoperative respiratory failure [5]. Seven patients underwent urgent operation and the others on days 3, 7, and 90. The rupture sites of the 10 patients who underwent aortic repair were the ascending aorta in 4 cases, the aortic arch in 3, and the descending aorta in 3. The ascending aorta and aortic arch was approached through a median sternotomy whereas the descending aorta was approached through a left thoracotomy.
If the rupture site is not identified preoperatively with deteriorating patient status, the findings of roentgenography and CT can provide clues. When pericardial effusion or mediastinal hemorrhage is noticed, it is likely that the rupture site is located between the ascending aorta and the aortic arch, which can be easily approached through a median sternotomy. Left hemothorax or pulmonary hemorrhage without mediastinal massive hematoma is highly associated with rupture of the descending aorta, which can be approached through a left thoracotomy. The procedures for aortic repair were suture closure in 4 cases, patch closure in 2, and graft replacement in 4 depending on the size of the aortic tear. We recommend graft replacement as the surgical technique of choice, considering that the adjacent aortic wall around the perforation should have similar histologic vulnerability. Our experience with the first patient (patient 17) who underwent graft replacement with uneventful follow-up for 11 years supports our choice of the procedure for aortic repair, although no other report describes long-term results.
In summary, we reviewed 18 reported cases of spontaneous rupture of the thoracic aorta, including our 2 cases, that were successfully treated with surgical repair. The common clinical picture of this fatal disorder is that of a middle-aged patient presenting with acute chest pain, collapse, and a history of hypertension. Imaging modalities such as chest roentgenography, CT, and transthoracic echocardiography demonstrate hemopericardium, hemomediastinum, or hemothorax. A high degree of suspicion for this catastrophic disorder must be maintained for such a patient presenting with acute hemorrhage around the thoracic aorta without any evidence of aortic aneurysm, dissection, penetrating arteriosclerotic ulcer, or intramural hematoma. Aortography effectively identifies the rupture site in some cases even though the findings are sometimes subtle. Other imaging modalities such as TEE and MRI should be evaluated and compared with the efficacy of aortography. In most cases, prompt aortic repair is imperative for patient survival. Depending on the rupture site, the appropriate surgical approach should be chosen to identify and repair the rupture site. Aortic repair such as simple suture, patch closure, and graft replacement can be performed with circulatory support appropriate for thoracic aortic surgery.
Once again, suspicion of spontaneous aortic rupture and aggressive exploration of the pericardium, mediastinum, or left thorax with appropriate aortic repair seem to provide the only chance for the patient’s survival.
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Addendum
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One additional case report of spontaneous aortic rupture appeared in the Japanese literature (with English abstract) since the closing date of this report (December 31, 1998). Ogata and associates (Jpn J Cardiovasc Surg 1999;28:167–9) reported a 68-year-old woman with a history of hypertension and diabetes who presented in shock with hemoptysis; a ruptured descending aorta into the left lung was successfully replaced with a tube graft.
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Abstract
Introduction
