Acute Aortic Dissection: A Rare but Important Cause of Acute Pericarditis

Quick Takes

  • Acute aortic dissection (AAD) may masquerade as acute pericarditis.
  • Slow leakage or exudate stemming from the dissecting hematoma appeared to have caused pericardial inflammation.
  • AAD is a rare but important cause of acute pericarditis because early and accurate diagnosis is essential for lifesaving surgical management.

Introduction
Acute aortic dissection (AAD) is a life-threatening cardiac emergency, in which serious complications such as cardiac tamponade, coronary malperfusion, aortic regurgitation, and/or shock are frequently observed.1-3 Accurate diagnosis in a timely manner is essential for adequate treatment. However, correct diagnosis of AAD is difficult in some cases due to the wide variety of its clinical manifestations.4-8 Patients with AAD could be misdiagnosed with acute coronary syndrome, pulmonary thromboembolism, gastrointestinal disease(s), and cerebrovascular accidents, among others. We encountered three cases of acute type A AAD that had acute pericarditis-like ECG changes.9 Herein, we share our experience and discuss pericardial involvement in AAD.

Cardiac tamponade
The most common pericardial involvement in type A AAD is cardiac tamponade due to rupture of the adventitia or exudate from the adventitia in the ascending aortic hematoma, which is observed in approximately 20% to 36% of cases of type A AAD.3,10-12 In our previous study, clinical pictures and electrocardiographic (ECG) changes were evaluated in 159 patients with acute type A AAD.13 Cardiac tamponade was the most frequent complication and was present in 32.1% (51/159) of cases, followed by pulse deficit, aortic regurgitation and coronary malperfusion. Acute ECG changes (defined as new shift of ST segment >1.0 mm or new change in the polarity of T waves compared with previous or later ECGs) were observed in 49.7% (79/159) of cases. In contrast, chronic changes, such as left ventricular hypertrophy or bundle branch block, were observed in 36.5% (58/159) of cases, while ECG was normal only in 27% (43/159). Among acute ECG changes, ST elevation was observed in 8.2% (13/159). ST elevation in inferior leads or aVR (with diffuse ST depression) was frequently associated with coronary malperfusion. Diffuse ST elevation consistent with acute pericarditis was evident only in two (1.3%) cases. On the other hand, acute ST depression and T wave inversion were observed in 34% (54/159) and 21.4% (34/159) of cases, respectively. Cases with ST depression and T wave changes had significantly higher incidence of shock (65.2% vs. 28.8%, p<0.001) and cardiac tamponade (51.2% vs. 15.0%, p<0.001) compared with those without changes.13 Significant association with tamponade and acute ST-T changes was also reported by Kosuge et al.14,15 It is assumed that rapid accumulation of blood in the pericardial space due to a leak or a rupture from dissected adventitia may result in mechanical compression of the heart and hypotension causing subendocardial ischemia leading to acute ST depression or T wave changes before causing pericardial inflammation.

Acute pericarditis
The authors recently encountered a patient with acute AAD who presented with ECG changes consistent with acute pericarditis.16 Initially, the patient complained of chest pain and visited the medical clinic. Cardiac enzyme levels were normal and there were no ECG abnormalities. He again visited the emergency department due to persistent tingling sensation in the chest. ECG revealed diffuse ST elevation and PR depression (Case 1 in Table 1, Figure 1).

Table 1: Clinical pictures in three patients with diffuse ST elevation

  Age Sex BP (mmHg)
HR (beats/min)
RR(breaths /min)
Fever
(°C)
Symptoms WBC(/μL)
CRP( mg/dl)
ECG changes CT effusion rupture surgery outcome
Case 1 70' M 130/64
104
32
37.1 chest pain while
carrying heavy objects followed by persistent tingling discomfort for 3 days
12600
16.65 (normal <0.39)
ST elevation
PQ depression (Figure 1)
IMH
localized intimal flap
small amount
light reddish
no yes alive
Case 2 30' M 190/100
 72
 24
37.2 abdominal pain,
right leg numbness,
and right eye blindness
15600
1.72 (normal <0.5)
ST elevation,
mild PQ depression
in V4-6 (Figure 2)
classic small amount
light reddish
no yes alive
Case 3 30' M 140/76
104
18
37.9 chest pain and dental
pain for 4 days
followed by back pain
14600
4.02(normal <0.45)
ST elevation
PQ depression (Figure 3)
classic small amount
light reddish
no yes alive
BP: blood pressure, HR: heart rate, RR: respiratory rate, WBC: white blood cell count, CRP: C-reactive protein, ECG: electrocardiogram, CT: computed tomography, M: Male, IMH: intramural hematoma. Normal range of CRP varies due to the different test kit in different time.

Figure 1

Figure 1
Figure 1. Serial 12-lead electrocardiograms (ECG) and contrast enhanced CT scan in case 1.
A: An ECG obtained 3 days before admission. QRS interval is slightly wide (110 ms) and deep S wave and mild ST elevation in V1-3 suggest left ventricular hypertrophy. These are chronic changes.
B: ECG obtained on admission shows diffuse ST elevation and PR depression (black arrows) consistent with acute pericarditis. A vertical arrow indicates 1 mV, and the paper speed is 25 mm/sec (same for Figures 2 and 3).
C and D: Contrast-enhanced CT exhibiting localized intimal flap (a black arrow in panel D) and intramural hematoma (white arrows) in the ascending aorta. A small amount of pericardial effusion is noted (yellow arrowhead in panel D). Modified from Mukaigawara M, Hirata K, Wake M. JAMA Cardiol 2016;1:229-30, with permission.

Contrast-enhanced computed tomography revealed type A acute AAD and a small amount of pericardial effusion (Figure 1). At the time of emergency surgery, a small amount of light reddish pericardial effusion was noted, which was presumed to be exudate from a hematoma in the ascending aorta; however, there was no gross rupture. This case prompted the authors to review two cases with similar ECG changes described in their previous study.13 ECG data and clinical history are summarized in Table 1 (Cases 2 and 3), and Figures 2 and 3.

Figure 2

Figure 2
Figure 2. Serial 12-lead electrocardiograms (ECG) in Case 2.
A: An ECG obtained 2 years before admission.
B: An ECG obtained at initial presentation.
C: An ECG obtained on day 7.
High voltage in the precordial leads and diffuse ST elevation are noted at initial presentation (B). ST elevation improves on day 7 (C).
Modified from Hirata K, Shimotakahara J, Nakayama I, et al. Acute aortic dissection masquerading as acute pericarditis: a case series. Internal Medicine 2020;59:16, with permission.

Figure 3

Figure 3
Figure 3. Serial 12-lead electrocardiograms (ECG) in Case 3.
A: An ECG obtained at initial presentation shows diffuse ST elevation and PR depression.
B: An ECG obtained on day 3 shows improved ST elevation.
Modified from Hirata K, Shimotakahara J, Nakayama I, et al. Acute aortic dissection masquerading as acute pericarditis: a case series. Internal Medicine 2020;59:16, with permission.

All three patients exhibited a small amount of light reddish pericardial effusion and did not experience rupture into the pericardium or clinical tamponade. Slow leakage or exudate stemming from the dissecting hematoma appeared to have caused inflammation.9

Although the incidence of acute pericarditis in AAD is unclear, it is believed to be rare, with only scattered case reports or case series.16-20 To our knowledge, there has been no description of acute pericarditis or pericarditis-like ECG changes associated with acute AAD in previously published large-scale clinical studies.1,14,15 However, Hirst et al. reported that 4% (22 of 505 autopsied cases) of patients exhibited friction rubs, whereas 6% (11 of 173 cases in which ECG was obtained) exhibited ECG changes consistent with acute pericarditis.21 In our study, as described, 2 of 159 cases (1.3%) exhibited diffuse ST elevation consistent with acute pericarditis.13

Clinical manifestation of acute pericarditis related to AAD is variable. Typical symptoms of acute pericarditis, including pleuritic chest pain and fever, along with ECG changes involving diffuse ST elevation, have been reported in some cases.17,18 However, all three patients described in the present report did not exhibit these typical symptoms, although ECG changes were consistent with acute pericarditis. The underlying mechanism of pericardial effusion was not a rupture of AAD because none of our patients experienced gross rupture of the ascending aorta. The amount of pericardial effusion may increase during the course of disease and lead to clinical tamponade.17-20 In fact, sudden rupture of the aorta into the pericardium may sometimes develop with immediate tamponade.17,18 Therefore, acute pericarditis may be a warning sign of developing tamponade in the ensuing hours or days.17

Constrictive pericarditis
Rarely, constrictive pericarditis may be caused by chronic pericardial inflammation in non-operated cases.18 In addition, constriction may be caused by post-pericardiectomy syndrome after the surgery for AAD.22

Conclusion
The number of cases of acute pericarditis related to AAD is small and pericardial biopsy or detailed evaluation of pericardial effusion are not routinely performed at the time of surgery. As such, the mechanism of pericarditis associated with AAD remains unclear. Recently, AAD has been known to be closely related to systemic and aortic wall inflammation, manifesting as increased levels of cytokine and inflammatory biomarkers including matrix metallopeptidase-9, granulocyte-colony stimulating factor, interleukin-6 and C-reactive protein, among others.23-25 It may be valuable to determine whether exudate from the dissected aortic wall, which is rich in inflammatory cytokines, causes pericardial inflammation and results in acute pericarditis. Nevertheless, further study is needed. For clinicians who care for patients with acute pericarditis, it is important to be aware that AAD is a rare but important cause of acute pericarditis because early and accurate diagnosis is essential for lifesaving surgical management.

References

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Clinical Topics: Acute Coronary Syndromes, Arrhythmias and Clinical EP, Cardiac Surgery, Invasive Cardiovascular Angiography and Intervention, Pericardial Disease, Valvular Heart Disease, ACS and Cardiac Biomarkers, EP Basic Science, Aortic Surgery, Cardiac Surgery and Arrhythmias, Cardiac Surgery and VHD, Interventions and ACS, Interventions and Structural Heart Disease

Keywords: Cardiac Tamponade, C-Reactive Protein, Pericardial Effusion, Pericardiectomy, Interleukin-6, Bundle-Branch Block, Aortic Valve Insufficiency, Hypertrophy, Left Ventricular, Acute Coronary Syndrome, Cytokines, Incidence, Adventitia, Constriction, Pericarditis, Constrictive


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