Pericardial Calcification


Article Author:
Nauman Khalid
Sarah Ahmad


Article Editor:
Evan Shlofmitz


Editors In Chief:
David Wood
Andrew Wilt
Mary Cataletto


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Trevor Nezwek
Radia Jamil
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
6/15/2019 7:58:45 PM

Introduction

The normal pericardium is 1 to 2 mm thick and is comprised of an outer fibrous layer and an inner serous layer (which further subdivides into a visceral layer, or epicardium, and a parietal layer). A potential space that contains approximately 15 to 35 ml of lubrication fluid separates the visceral and parietal layers. The pericardium is a rigid, avascular, fibrous sac and its primary function is minor anchoring, lubrication, preventing distention of cardiac chambers and optimizing diastolic filling.[1] Normally the pericardium lacks any calcium deposits and calcification may be a sign of underlying inflammation or a more sinister etiology. Pericardial calcification alone is generally asymptomatic; however, signs and symptoms can develop due to underlying disease processes such as constrictive pericarditis (CP). However, a significant point to keep in mind is that pericardial calcification may not be present in up to 20% of cases of CP and it may be present in the absence of constrictive physiology. Interestingly, there have been recent reports of the development of CP after cardiac transplantation, an unusual presentation, as the transplanted heart is believed to be free of any pericardial tissue.[2][3]

Etiology

Constrictive pericarditis occurs when a normal thin compliant pericardium gets replaced by a thick, calcified, non-compliant pericardium that interferes with ventricular filling. Varying degrees of pericardial thickening and calcification is present in about 80% of the cases of CP. The etiologic basis of CP in the United States (US) has evolved over the past few decades. Historically tuberculosis had been a major cause of CP in the US and accounted for almost half of the cases, but now it is encountered less frequently. Recent studies have shown major cardiac surgery and 'idiopathic' cases as being the most common etiologies followed by radiation heart disease.[4] Other important etiologies include viral pericarditis, trauma, malignancy, rheumatologic and connective tissue diseases.[5] However, tuberculosis continues to be the most common cause of CP in the developing world.

Epidemiology

For idiopathic and viral constrictive pericarditis an incidence of 0.76 cases per 1000 person-years has been reported in a series of 500 consecutive patients; this contrasts with the connective tissue disease, neoplasms, tuberculosis, and purulent CP in which the incidence was noted to be 4.40, 6.33, 31.65, and 52.74 cases per 1000 person-years, respectively.[6]

History and Physical

Pericardial calcification is generally asymptomatic; symptoms usually develop with cardiac hemodynamic compromise such as in the setting of constrictive pericarditis. Symptoms of right heart failure or low output states such as dyspnea on exertion, orthopnea, bendopnea, and fatigue may develop. Physical examination findings include hepatomegaly, ascites, peripheral edema (usually bilateral), hepatojugular reflux, Kussmaul’s sign, jugular venous distention (with prominent x and y descents), and pulsus paradoxus.

Evaluation

Chest radiography may identify pericardial calcification; however, it suffers from low sensitivity. The laboratory workup may show an elevation of liver enzymes (especially alkaline phosphatase) and creatinine. Computerized tomography (CT) provides excellent anatomic details of the heart and pericardium. The pericardial thickness on the CT scan should be less than 2 mm.[7] Although the pericardial thickness of more than 4 mm on CT scan is predictive of constrictive pericarditis up to 20% of patients with normal pericardial thickness can still develop CP.[8] Cardiac magnetic resonance imaging can be utilized not only to delineate cardiac anatomy and pericardial thickness, it can provide information on ventricular septal motion demonstrating the so-called 'septal bounce' and 'septal shudder' which is a classic finding for CP also noted on transthoracic echocardiogram (TTE).[4]

In a patient with symptoms of right-sided heart failure and suspected constrictive pericarditis, Two-dimensional (2-D) echocardiography and tissue Doppler Imaging are probably the most important tools for diagnosing CP. Two-dimensional echocardiogram demonstrates septal motion abnormalities, enhanced interventricular interdependence, and plethoric inferior vena cava. Tissue Doppler of the mitral annulus reveals increased medial early diastolic velocities (e').[4] In healthy individuals, mitral lateral e' velocity is usually greater than the medial e' velocity, but in CP due to the tethering effect of the lateral annulus to the surrounding calcified pericardium, this velocity becomes lower, a phenomenon called 'annulus reversus' and a hallmark feature of CP.[4] In some cases where the diagnosis is unclear, an invasive hemodynamic assessment with cardiac catheterization is possible. Typically, an equalization of end-diastolic pressures in all four cardiac chambers is noted. Furthermore, with the 'match-up' study, by placing high fidelity catheters in the right and left ventricle and recording pressures simultaneously, discordant respirophasic changes in the ventricular filling patterns are noted (a catheterization equivalent of ventricular interdependence, in which one ventricle fills at the expense of other depending on the respiratory cycle).[9]

Treatment / Management

Pericardial calcification in the absence of symptoms does not require any treatment. Subacute cases of constrictive pericarditis, in which there is underlying inflammation, may respond to anti-inflammatory therapy (such as colchicine, corticosteroids, and non-steroidal anti-inflammatory drugs).[3][10] Surgical pericardiectomy is the gold standard for CP and is potentially curative. 

Differential Diagnosis

Constrictive pericarditis must be ruled out when pericardial calcification is present on imaging modalities. Unusual forms of CP such as transient constriction (which responds to anti-inflammatory therapy) and effusive constrictive pericarditis (developing in patients with cardiac tamponade and develop immediately after pericardiocentesis), or occult constrictive pericarditis (which manifests after a fluid challenge) must be ruled out as well [4]. Other differentials of calcification of pericardium include pericardial scarring as a result of recurrent pericarditis. Additionally, mimickers of CP include restrictive cardiomyopathy (a myocardial disease), and severe tricuspid regurgitation must be ruled out. 

Prognosis

Long-term survival after pericardiectomy for constrictive pericarditis depends on the underlying etiology. In a cohort of 163 patients, idiopathic CP had the best 7-year survival (88%) followed by postsurgical (66%) and post-radiation CP (27%).[11]

Complications

Pericardial calcification in the presence of symptoms should be evaluated further as constrictive pericarditis if present could be potentially curative. Without surgical pericardiectomy, constrictive pericarditis portends a very poor prognosis due to complications associated with heart failure and the low output state. Renal failure, organomegaly, shock, and death are other potential complications. 

Deterrence and Patient Education

Early recognition and diagnosis of pericardial calcification and ruling out constrictive pericarditis is imperative to ensure timely management optimal clinical outcomes. 

Enhancing Healthcare Team Outcomes

Pericardial calcification in the absence of symptoms is a benign condition. However, constrictive pericarditis must be ruled out in such patients. Diagnosis of CP can be challenging and requires a multimodality imaging with a multidisciplinary approach involving invasive and non-invasive cardiologists, radiologists and cardiothoracic surgeons. If missed, CP carries a grave prognosis, and most patients die with medical management alone. Pericardiectomy is potentially curative and carries acceptable mid- and long-term outcomes depending on the etiology. 


  • Image 8518 Not availableImage 8518 Not available
    Image courtesy S Bhimji MD
Attributed To: Image courtesy S Bhimji MD

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Pericardial Calcification - Questions

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A patient is found to have a rim of calcification around the pericardium. He does not give any significant past medical history of any medical problems. Which of the following is the least likely cause of pericardial calcification?



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A 65-year-old female presents with abdominal pain and bloating, peripheral edema, and decreased appetite. Physical examination disclosed peripheral edema. An echocardiogram shows plethoric inferior vena cava and abnormal septal motion. Tissue Doppler imaging of the mitral annulus reveals increased medial early diastolic velocities (e'), as well as calcified and thickened pericardium. Keeping in mind the most likely diagnosis which of the following treatment modalities will not be helpful?



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A 45-year-old man presents with complaints of exertional dyspnea. His past medical history is significant for mediastinal radiation therapy as a teenager for the management of lymphoma. Physical examination is notable for prominent jugular venous distention, enlarged liver span, and mild ascites. A 2 view chest x-ray is normal. Computed tomography of the chest demonstrates thick pericardium. Hemodynamic assessment in the catheterization laboratory with a match up study demonstrates discordance of the right and left ventricular pressures. Keeping in mind the most likely diagnosis, which of the following is is most accurate regarding the pericardial calcification noted on his CT scan?



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Pericardial Calcification - References

References

Miranda WR,Oh JK, Constrictive Pericarditis: A Practical Clinical Approach. Progress in cardiovascular diseases. 2017 Jan - Feb;     [PubMed]
Nguyen T,Phillips C,Movahed A, Incidental findings of pericardial calcification. World journal of clinical cases. 2014 Sep 16;     [PubMed]
Watkins MW,LeWinter MM, Physiologic role of the normal pericardium. Annual review of medicine. 1993;     [PubMed]
Bull RK,Edwards PD,Dixon AK, CT dimensions of the normal pericardium. The British journal of radiology. 1998 Sep;     [PubMed]
Talreja DR,Edwards WD,Danielson GK,Schaff HV,Tajik AJ,Tazelaar HD,Breen JF,Oh JK, Constrictive pericarditis in 26 patients with histologically normal pericardial thickness. Circulation. 2003 Oct 14;     [PubMed]
Talreja DR,Nishimura RA,Oh JK,Holmes DR, Constrictive pericarditis in the modern era: novel criteria for diagnosis in the cardiac catheterization laboratory. Journal of the American College of Cardiology. 2008 Jan 22;     [PubMed]
Haley JH,Tajik AJ,Danielson GK,Schaff HV,Mulvagh SL,Oh JK, Transient constrictive pericarditis: causes and natural history. Journal of the American College of Cardiology. 2004 Jan 21;     [PubMed]
Umer A,Khalid N,Chhabra L,Spodick DH, Role of Pericardiectomy in Postcardiac Transplant Constrictive Pericarditis. The Annals of thoracic surgery. 2015 Dec;     [PubMed]
Umer A,Khalid N,Chhabra L,Memon S,Spodick DH, Constrictive pericarditis complicating cardiac transplantation. Journal of cardiothoracic surgery. 2015 Aug 25;     [PubMed]
Bertog SC,Thambidorai SK,Parakh K,Schoenhagen P,Ozduran V,Houghtaling PL,Lytle BW,Blackstone EH,Lauer MS,Klein AL, Constrictive pericarditis: etiology and cause-specific survival after pericardiectomy. Journal of the American College of Cardiology. 2004 Apr 21;     [PubMed]
Imazio M,Brucato A,Maestroni S,Cumetti D,Belli R,Trinchero R,Adler Y, Risk of constrictive pericarditis after acute pericarditis. Circulation. 2011 Sep 13;     [PubMed]

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