Case Report

Pericarditis as initial manifestation of proximal aortic dissection in young patients

Abstract

Pericarditis was the primary manifestation of aortic dissection in these 2 young men. Both patients had no phenotypic characteristics of Marfan or Ehlers-Danlos syndrome. These patients had pleuritic chest pain and characteristic electrocardiographic changes consistent with pericarditis. However, timely performed transthoracic echo- cardiograms revealed proximal aortic dissection with hemopericardium noted at surgery in both cases. Although the sensitivity of transthoracic echocardiogram for proximal aortic dissection is approximately 60%, certain findings can alert the physician to the possibility of aortic dissection. Therefore, in young patients with suspected pericarditis, a timely performed transthoracic echocardiogram should include a careful evaluation of the Ascending aorta and arch to rule out this lethal diagnosis.

Proximal aortic dissection rapidly leads to death if not promptly diagnosed and treated. The most common cause of death is intrapericardial rupture leading to cardiac tampo- nade. Rarely, impending rupture may be preceded by signs of pericarditis. Pericardial inflammation may result from slow extravasation of blood into the pericardial space from the false lumen. Because pleuritic chest pain is the most common manifestation of viral pericarditis in young patients, aortic dissection may be dangerously overlooked. We present 2 young patients with proximal aortic dissection with clinical presentation of pericarditis.

A 26-year-old man initially presented to the emergency department (ED) 2 days before admission complaining of acute-onset pleuritic chest pain and Right flank pain. His medical history was remarkable only for nephrolithiasis. He was an active smoker but denied illicit drug use. On physical examination, he was normotensive, had no clinical features of Marfan or Ehlers-Danlos syndrome, and had no evidence of a pericardial rub. He did have mild right flank tenderness. His electrocardiogram (ECG) showed Sinus bradycardia with an apparent early repolarization pattern (Fig. 1A). An Abdominal computed tomography (CT) without contrast revealed small, nonobstructive nephrolithiasis of the right

kidney. The patient was diagnosed with renal colic and was discharged with pain medications.

The patient presented 2 days later with severe pleuritic

chest pain and ECG changes consistent with pericarditis, based on diffuse concave ST elevation and PR depression (Fig. 1B). A transthoracic echocardiogram performed in the ED revealed moderate aortic regurgitation and a severely dilated aortic root with an intimal flap (Fig. 1C-D). No pericardial effusion was seen. An emergent contrast- enhanced CT confirmed proximal aortic dissection extending to the right renal artery. The patient was then taken emergently to surgery for aortic repair.

A complete circumferential aortic tear was noted just above the sinotubular junction upon opening the massively enlarged aorta. This area was excised and replaced with a graft, and the native aortic valve was resuspended. At this point, a 300-mL hemopericardium was drained. The patient’s surgery was uncomplicated, and he made a gradual recovery. Further workup revealed normal urine toxicology, erythrocyte sedimentation rate of 9 mm/h, negativity for antinuclear antibody, and nonreactive rapid plasma reagin. Because of his family history of 2 paternal uncles dying suddenly before the age of 30 years, a diagnosis of familial aortic dilatation and dissection was considered the most probable etiology. Familial testing for aortic root dilatation

was recommended.

A 37-year-old healthy man developed acute onset of pleuritic chest pain at 9 AM on the day of admission. He eventually came to the ED at 10 PM. He had a negative medical history. The patient quit smoking recently and denied any illicit drug use. He had no family history of any heart disease or sudden death.

Initial vital signs revealed sinus tachycardia with normal blood pressure. He had no clinical features of Marfan syndrome. His cardiac examination was notable for a pericardial rub. Chest x-ray revealed cardiomegaly with a normal-sized mediastinum. Electrocardiography revealed typical findings of pericarditis, with diffuse ST elevation and PR depression. The patient was admitted and treated for presumed pericarditis. The next morning, the patient again developed severe chest pain. A transthoracic echocardiogram revealed moderate pericardial effusion, severe dilatation of the proximal aorta with Ascending aortic dissection, and severe aortic regurgitation (Fig. 2A-B).

0735-6757/$ - see front matter (C) 2008

379.e4 Case Report

Fig. 1 Progression of ECG from apparent early repolarization pattern (A) to a more classic pattern of pericarditis with diffuse ST elevation and PR depression (B). Dissection flap (arrow) clearly visible in systole (C) and diastole (D) from the parasternal long-axis view of a transthoracic echocardiogram.

The patient underwent emergency surgery, which revealed large amount of blood in the pericardium. There was a proximal circumferential tear of the aorta extending proximally to the left main and right coronary arteries and distally into the descending aorta. The native aortic valve and root were replaced with a mechanical aortic valve and an aortic graft.

The patient recovered uneventfully after surgery. Pathol- ogy demonstrated myxomatous degeneration of the aortic valve, aortic dissection (Fig. 2C), and cystic medial necrosis of the aorta (Fig. 2D). Evidence of organizing thrombus was apparent, which suggested that aortic dissection had been occurring for several days. The patient failed to follow-up with genetic consultation and other medical visits.

As illustrated in these 2 cases, clinical and ECG findings of pericarditis may be an early warning sign of proximal aortic dissection. Of great concern is that the clinical features of pericarditis may compromise or delay the diagnosis of aortic dissection for several reasons: (1) aortic dissection in young patients without significant medical history is rare; (2) pleuritic chest pain is the most common manifestation of viral pericarditis; and (3) absence of pericardial effusion on transthoracic echocardiography is common but does not exclude pericarditis.

Very few cases of pericarditis preceding aortic dissection have been previously reported [1-5]. However, pericarditis preceding dissection may be more common than clinically

recognized. In a large autopsy series of aortic dissection, 6% of patients had detectable pericarditis [6]. In the 12 reported cases, the diagnosis was established at postmortem in

Fig. 2 Dissection flap (arrow) visible on a parasternal long-axis view (A) and suprasternal view (B) of a transthoracic echocardio- gram. A selected histologic section of the aortic wall (C) revealing dissection of the media (arrow). Histology revealing fragmentation of elastic tissue (D) and presence of mucoid material (arrowheads) diagnostic of cystic medial necrosis.

Case Report 379.e5

9 patients and by aortography in 3 patients. Only 2 of these 3 patients survived to hospital discharge. This high mortality rate (92%) underscores the need for an early diagnosis and emergent aortic repair. Aortography can establish the diagnosis of dissection but is invasive and time-consuming. computed tomographic scanning is also an accurate diagnostic imaging, but it takes time and exposes the patient to lack of monitoring, and pericarditis is not an easily accepted indication for it. As can be seen from these and prior cases, dissection is not high on the differential diagnosis in apparently healthy young subjects.

The 2 cases presented are unique in that the diagnosis of aortic dissection was established solely with a transthoracic echocardiogram, which led to emergent lifesaving surgery. Although the overall sensitivity of transthoracic echocardio-

graphy for detection of proximal aortic dissection is low (~60%), its universal emergent availability and lack of adverse effects make it a very reasonable first choice. An initial transthoracic echocardiogram with Suprasternal notch views performed in the ED with careful scanning of the

ascending aorta and arch to detect aortic dilatation, aortic regurgitation, or an echogenic pericardial effusion suggestive of hemopericardium should alert the clinician to the possibility of aortic dissection (if an aortic intimal flap is not detected) and prompt immediate further testing with transesophageal echocardiography or CT. In this way, patients with presumed pericarditis can be quickly screened for a more lethal diagnosis.

Finally, both patients had no phenotypic characteristics of Marfan or Ehlers-Danlos syndrome. Both patients fit the clinical syndrome of familial aortic dilatation complicated with aortic dissection. This syndrome affects up to 20% of patients without Marfan syndrome with thoracic aortic dissection [7]. This is an autosomal-dominant condition with variable penetrance that occurs at a younger age. In these patients, the aorta may dilated at a faster rate than in those with sporadic or Marfan syndrome. Therefore, screen- ing transthoracic echocardiography and genetic testing is advised for the relatives of patients in whom the diagnosis is suspected or established.

Suchdeep Raj Bains MD Division of Cardiology, University of New Mexico School of Medicine 5-ACC, MSC10 55501

University of New Mexico Albuquerque, NM 87131, USA

Anita Kedia MD

Division of Cardiology, Lovelace Medical Center

5400 Gibson Blvd SE Albuquerque, NM 87108, USA

Carlos A. Roldan MD Division of Cardiology, University of New Mexico School of Medicine Veterans Affairs Medical Center Cardiology 5B-111, 1501 San Pedro Drive SE

Albuquerque, NM 87108, USA E-mail address: [email protected]

doi:10.1016/j.ajem.2007.05.018

References

1. Saner HE, Gobel FL, Nicoloff DM, Edwards JE. Aortic dissection presenting as pericarditis. Chest 1987;91:71-4.
2. Castleman B, McNeely BU. Case records of the Massachusetts General Hospital. Case 10-1969. N Engl J Med 1969;280:550-7.
3. Greenberg DI, Davia JE, Fenoglia J, McAllister HA, Cheitlin MD. Dissecting aortic aneurysm manifesting as acute pericarditis. Arch Intern Med 1979;139:108-9.
4. Weiss S. Dissecting aneurysms of the aorta: two cases with unusual features. N Engl J Med 1938;218:512-7.
5. Morris AL. Pericarditis and impending rupture of aneurysms of the ascending aorta. Can Med Assoc J 1982;126:1190-1.
6. Hirst AE, Johns VJ, Kime SW. Dissecting aneurysm of the aorta; a review of 505 cases. Medicine 1958;37:217-79.
7. Albornoz G, Coady MA, Roberts M, et al. Familial thoracic aortic aneurysms and dissections-incidence, modes of inheritance, and phenotypic patterns. Ann Thorac Surg 2006;82:1400-6.