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Ann Thorac Surg 2009;87:1597-1599. doi:10.1016/j.athoracsur.2008.10.005
© 2009 The Society of Thoracic Surgeons

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Case Reports

Constrictive Pericarditis Presenting as a Calcified Anterior Cardiac Mass

Richard S. Schofield, MDa,c, Steve M. Dorman, Jr, BSd, Juan M. Aranda, Jr, MDa, James A. Hill, MDa, Daniel F. Pauly, MD, PhDa, Charles T. Klodell, MDb,*

a Division of Cardiovascular Medicine, University of Florida, Gainesville, Florida
b Division of Thoracic and Cardiovascular Surgery, University of Florida, Gainesville, Florida
c Department of Veterans Affairs Medical Center, Gainesville, Florida
d Texas A&M Health Science Center College of Medicine, College Station, Texas

Accepted for publication October 3, 2008.

* Address correspondence to Dr Klodell, PO Box 100286 HSC, Gainesville, FL 32610 (Email: klodell{at}surgery.ufl.edu).


    Abstract
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Constrictive pericarditis is an infrequent disorder. We report the case of a 52-year-old man with constrictive pericarditis with an uncommon anterior associated pericardial mass. Upon diagnosis of constrictive pericarditis, the patient underwent pericardectomy and resection of the mass. He is well 6 months postoperatively, with complete resolution of his heart failure symptoms.


    Introduction
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Constrictive pericarditis is rare disorder that causes pericardial thickening and impaired diastolic filling of the heart with associated symptoms of heart failure [1, 2]. Patients often present with generalized symptoms of cardiac failure such as edema of the extremities and hepatic congestion. The most common cause of this disease in underdeveloped areas of the world is either viral or bacterial, and when bacterial, it is likely related to tuberculosis. In the developed world, constrictive pericarditis is often attributed to idiopathic causes, postsurgical issues, trauma, or radiation injury [2].

Presentation of this disease sometimes occurs with calcification of the pericardium. In the United States, calcification of the pericardium in association with constrictive pericarditis has been reported to occur in 5% to 36% of all cases of constrictive pericarditis [2, 3]. We report a case of constrictive pericarditis presenting as a calcified anterior cardiac mass.

A 52-year-old man presented with a 5-year history of worsening nonischemic cardiomyopathy. The patient's left ventricular ejection fraction by left ventricular angiography decreased from 0.45 to 0.20 during this time. Furthermore, he exhibited dyspnea upon exertion, which had progressed significantly during the past year consistent with New York Heart Association (NYHA) class III heart failure. The patient additionally exhibited occasional paroxysmal nocturnal dyspnea and two-pillow orthopnea.

The patient denied any chest pain or history of myocardial infarction. Three cardiac catheterizations had been performed in the last 5 years, all without revealing significant coronary artery disease. At another hospital shortly before presentation, calcification of the inferior pericardium was noted upon left ventriculography and on an abdominal computed tomography (CT) scan.

The patient's medical history was significant for controlled and stable hypertension, as well atrial fibrillation requiring multiple cardioversions. A dual-chamber implantable cardioverter defibrillator had also been placed several months previously for primary prevention of sudden cardiac death. Physical examination revealed mild elevation of jugular venous pressure and mild hepatomegaly without other gross features of heart failure, and no pericardial knock or rub could be heard.

Constrictive pericarditis was suspected due to worsening of symptoms of right heart failure, nonischemic cardiomyopathy, and pericardial calcification on the coronary angiograms; therefore, the patient was further examined by chest CT scan. This scan clearly demonstrated a large spherical mass about 4 cm in diameter in the anterior pericardium, with calcification of the pericardium and partial compression of the right ventricular free wall (Fig 1). At this time, the origin of the mass was unclear. However, the differential diagnosis included constrictive pericarditis and teratoma. A transthoracic echocardiogram also further characterized the mass and revealed again the presence of partial compression of the right ventricular free wall (Fig 2). Cardiac catheterization was performed to better elucidate the hemodynamic effect of suspected constrictive pericarditis on right ventricular filling.


Figure 1
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Fig 1. A computed tomography scan demonstrates a large anterior mass in the pericardium that is compressing the right ventricular free wall.

 

Figure 2
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Fig 2. Echocardiogram demonstrates compression of the right ventricular free wall and an associated mass lesion.

 
With evidence of constriction confirmed, the patient underwent pericardiectomy and resection of the mass. Upon gross examination before removal, the mass was noted to extend from the diaphragm to the top of the atrium, and from the right phrenic nerve to almost the whole length of the pericardium (Fig 3). The anterior pericardium was excised, without the use of cardiopulmonary bypass, from the great vessel origins to the diaphragm and all areas between the right and left phrenic nerves.


Figure 3
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Fig 3. Intraoperative photograph demonstrates the cavity in the large calcified mass in the anterior pericardium.

 
Upon excision of this mass down to the anterior pericardium, the posterior pericardium was elevated and a 6- x 4-cm bar of calcification was removed which was observed to be constrictive. The removed specimens were examined pathologically and were noted to have no evidence of granulomatous inflammation, acute inflammation, or tumor. The removed mass consisted solely of thickened and fibrotic pericardium with nodular calcifications. All pieces removed from the pericardium ranged from rubbery to focally hard in character. The pericardial sac that was removed, excluding the mass, was 0.2 to 0.8 cm thick.

The patient tolerated the surgical resection of the pericardium and the anterior mass well and was discharged 5 days later. Six months postoperatively, the patient is significantly improved (NYHA class I), with normal left ventricular ejection fraction and no cardiac symptoms.


    Comment
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We have described a rare case of calcific constrictive pericarditis with an associated anterior calcific pericardial mass. A very small number of similar cases have been reported, including a patient who had calcified constrictive pericarditis with a calcified blood-filled density compressing the right ventricular inflow tract [4]. The mass in our patient was fully composed of calcified, nodular pericardial tissue rather than blood, and our patient experienced right ventricular free wall compression as the cause for his symptomatology. The findings from our case add to the literature on constrictive pericarditis by emphasizing the variable presentations of this often-occult disease.

Heart failure associated with constrictive pericarditis is a disease that progresses slowly. On average, the sluggish advancement of this disease leads to a 2-year delay in diagnosis from onset [5]. Many nonspecific symptoms are often associated with this disease, including edema of the lower extremities, which makes a quick identification of constrictive pericarditis difficult [6]. Generalized symptoms of right-sided heart failure and decreased cardiac output should lead the clinician to consider constrictive pericarditis in a differential diagnosis [7]. Elevation and equalization of ventricular filling pressures is also often present in patients with constrictive pericarditis [8]. Through observation of dynamic respiratory changes that occur in the heart during cardiac catheterization, the clinician may be able to distinguish constrictive pericarditis from other disorders such as restrictive cardiomyopathy.

In this patient no cause was identified for the calcific constrictive pericarditis with the associated mass on the anterior pericardium. There is often no known etiology for constrictive pericarditis once trauma, postsurgical complications, radiation injury, and tuberculosis have been ruled out. Often there is an occult event that triggers inflammation of the pericardium, leading to fibrosis with calcification and sometimes adherences on the pericardium. This explanation could help clarify why the atypical mass occurred in conjunction with calcification of the pericardium. Pericardectomy is the only established treatment for chronic constrictive pericarditis.


    References
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 Abstract
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 Comment
 References
 

  1. Wang A, Bashore TM. Clinical problem-solving. Undercover and overlooked. N Engl J Med 2004;351:1014-1019.[Medline]
  2. Ling LH, Oh JK, Breen JF, et al. Calcific constrictive pericarditis: is it still with us? Ann Intern Med 2000;132:444-450.[Abstract/Free Full Text]
  3. Oh KY, Shimizu M, Edwards WD, et al. Surgical pathology of the parietal pericardium: a study of 344 cases (1993–1999) Cardiovasc Pathol 2001;10:157-168.[Medline]
  4. Yamauchi T, Masai T, Takeda K, et al. Severely calcified constrictive pericarditis simulating a mediastinal tumor and obstructing the right ventricular inflow tract Ann Thorac Cardiovasc Surg 2007;13:410-412.[Medline]
  5. Schofield RS, Shoemaker SB, Ryerson EG, et al. Left ventricular dysfunction after pericardiectomy for constrictive pericarditis Ann Thorac Surg 2004;77:1449-1451.[Abstract/Free Full Text]
  6. Deterling Jr RA, Humphreys 2nd GH. Factors in the etiology of constrictive pericarditis Circulation 1955;12:30-43.[Abstract/Free Full Text]
  7. Kwan DM, Dhaliwal G, Baudendistel TE. Thinking inside the box J Hosp Med 2008;3:71-76.[Medline]
  8. Bellin DA, Devine PJ. Constrictive pericarditis: a cause of exertion-induced dyspnea in a soldier with a prior sternotomy Mil Med 2007;172:1220-1223.[Medline]




This Article
Right arrow Abstract Freely available
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Charles T. Klodell
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