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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 2  |  Issue : 1  |  Page : 27-29

Porcelain constrictive chronic pericarditis: Case report and literature review


Dr. Joe Nwiloh Heart Center, St. Joseph's Hospital, Adazi-Nnukwu, Anambra State, Nigeria

Date of Web Publication12-Dec-2017

Correspondence Address:
Jonathan Nwiloh
Dr. Joe Nwiloh Heart Center, St. Joseph's Hospital, Adazi-Nnukwu, Anambra State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njct.njct_6_17

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  Abstract 

Chronic pericarditis (CP) with bony encasement of the heart resulting in constrictive pathophysiology is relatively rare. We report a 43-year-old female with CP and long-standing right heart failure in New York Heart Association (NYHA) Class IV while on medical management, who was subsequently referred for surgery. Patient had porcelain bony encasement of the heart which was safely resected with the aid of cardiopulmonary bypass (CPB). There was a dramatic reduction in the central venous pressure and subsequent improvement in functional status to NYHA Class I–II postoperatively. While pericardiectomy is generally performed without CPB, it may be a useful adjunct in patients with heavily calcified porcelain pericardium. CPB facilitates more complete pericardial resection leading to lower incidence of residual constrictive symptoms postoperatively in patients with porcelain CP. Although there was no histologic or bacteriologic evidence of tuberculosis (TB), the presumptive etiology was TB given its prevalence in our environment.

Keywords: Bone, chronic pericarditis, constriction


How to cite this article:
Nwiloh J, Ezenwanjiaku V, Etukokwu K, Orakwe O, Ufoegbunam P, Okechukwu U, Adiele K. Porcelain constrictive chronic pericarditis: Case report and literature review. Niger J Cardiovasc Thorac Surg 2017;2:27-9

How to cite this URL:
Nwiloh J, Ezenwanjiaku V, Etukokwu K, Orakwe O, Ufoegbunam P, Okechukwu U, Adiele K. Porcelain constrictive chronic pericarditis: Case report and literature review. Niger J Cardiovasc Thorac Surg [serial online] 2017 [cited 2020 Dec 1];2:27-9. Available from: http://www.nigjourcvtsurg.org/text.asp?2017/2/1/27/220492


  Introduction Top


Pericarditis can be acute, subacute, or chronic and may be associated with fluid accumulation. Chronic pericarditis (CP) defined as persisting for >6 months often leads to fibrosis, scarring, and/or calcification. This sequela may ultimately result in constrictive pathophysiology with impairment of diastolic filling, increase and equalization of venous, and ventricular diastolic pressures which typically manifests as right heart failure. CP with complete bony pericardial calcification encasing the heart, however, is relatively rare and presents a more difficult technical surgical challenge to remove with resultant possible residual constrictive symptoms postoperatively.


  Case Report Top


A 43-year-old female was referred to our center with a 2-year history of shortness of breath, cough, orthopnea, paroxysmal nocturnal dyspnea, abdominal distension, and bilateral leg swelling. The symptoms waxed and waned with medical therapy, until the preceding 3 months when her symptoms became progressively worse with increasing dyspnea and easy fatigability on minimal exertion and at rest. Past medical history was significant only for unspecified liver disease 14 years earlier. Physical examination revealed a cachetic middle-aged female in obvious respiratory distress and in New York Heart Association (NYHA) Class IV. Vital signs were blood pressure 94/60 mmHg, heart rate 107 beats/min, respiratory rate 28/min, SpO2 98%, weight 56 kg, height 163 cm, and body mass index 21.05 kg/m 2. Pertinent findings were elevated jugular venous pressure with Kussmaul's sign, decreased breath sounds at the lung bases, hepatomegaly, ascites, and bilateral 3+ pitting edema up to the knee. Pertinent laboratory results were white blood cell 4.99 × 10, hemoglobin 7.7 g/dl, platelet 610 × 10, sodium 131 mmol/L, potassium 3.0 mmol/L, calcium 2.12 mmol/L, urea 1.73 mmol/L, creatinine 54.8 mmol/L, alkaline phosphatase 17 u/L, albumin 25.7 g/L, international normalized ratio 1.22, prothrombin time 14.7, and activated partial thromboplastin time 71.7. Viral screen with HIV, hepatitis B surface antigen and hepatitis C was negative. Tuberculin skin test was negative. Electrocardiogram showed sinus tachycardia and low voltage tracings with nonspecific ST abnormalities. Chest X-ray showed pericardial calcification [Figure 1]. Chest computed tomography (CT) scan also showed thickened and calcified pericardium circumferentially [Figure 2].
Figure 1: Chest X-ray posteroanterior view.

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Figure 2: Chest computed tomography scan axial view.

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The patient was diagnosed with congestive heart failure from calcific constrictive CP and subsequently taken for surgery.

Operative technique

After median sternotomy and administration of systemic heparin, the aortic arch was cannulated for arterial inflow and venous return through a left common femoral vein cut down with cannula advanced into the right atrium under transesophageal echocardiogram guidance. Partial cardiopulmonary bypass (CPB) was then established to decompress the heart and the surgical procedure performed on a warm beating heart. The overlying pericardium was essentially bone and the only option appeared dividing the anterior surface with an oscillating sternal saw to develop a cleavage plane beneath. However, a small shelving edge of the pericardial reflection between the ascending aorta and main pulmonary artery was identified, which allowed creation of a small opening underneath through which the tips of a Stille-Liston bone cutter was inserted to divide a small segment of the bony pericardium. Beneath the bony pericardium was caseous material loosely adherent to the right ventricular surface, thus allowing a cleavage plane to be carefully developed while cutting the pericardium anteriorly repeatedly in small segments for removal. The pericardium was, however, more densely adherent laterally to the right and left atria requiring more tedious dissections to free. The pericardium was resected from phrenic nerve to phrenic nerve while also freeing both the inferior and superior vena cavae. The densely adherent bony pericardium, however, over the diaphragmatic surface of the heart was left largely intact to reduce the risk of cardiac perforation or injury to coronary arteries.

Postoperative course

Patient had initial low cardiac output and developed severe hyponatremia, hypokalemia, and prerenal azotemia with diuretic therapy which all subsequently improved. The central venous pressure decreased from 28–30 mmHg to 8–10 mmHg range postoperatively, which was accompanied with gradual improvement in dyspnea, ascites, peripheral edema, and exercise tolerance. Antituberculous medications initially started on the presumptive diagnosis of tuberculous CP, was subsequently stopped due to worsening liver function tests, as the histopathology and other tests were all negative for tuberculosis (TB). Patient was later discharged home in stable condition on postoperative day 27 and continues to do well at follow-up visits in NYHA I–II.


  Discussion Top


The pericardial sac consists of visceral and parietal layers which contains about 20–50 ml of fluid that serves as a lubricant for the enclosed heart. While the pericardium plays some role in cardiac filling volumes, it is not crucial for life and can be removed without consequences in patients with pericarditis.[1] Chevers in 1842 first reported CP and described symptoms largely related to compression of the muscle by a sticky material surrounding the heart.[2] Subsequently, constrictive CP pathophysiology was delineated as the dissociation of intracardiac and intrathoracic pressures causing impairment of diastolic filling and cardiac volume by the thickened, scarred, or calcified pericardium.[1] While idiopathic, viral, postcardiotomy, and mediastinal irradiation are the leading causes of CP in the Western world, TB remains the presumed number one etiology in the developing nations.[3] TB pericarditis, however, may be difficult to prove as it often occurs without any evidence of TB elsewhere and the operative specimens are frequently negative.[4] Yangni-Angate et al. in a review of 120 patients from Cote d'Ivoire seen over a 35-year period with CP undergoing pericardiectomy identified TB as the causative agent in 99%.[5] They also observed some pericardial calcifications on chest X-ray in 52.5% of their patients. Pericardial calcification on chest X-ray has been identified as been suggestive of CP and is reportedly seen in 5%–27% of cases.[6] However, definitive diagnosis of CP requires echocardiogram, CT scan, magnetic resonance imaging, or cardiac catheterization. While medical management has a role in management, definitive treatment for symptomatic constrictive CP requires pericardiectomy. Although most cases can be performed without CPB, patients with severe calcific pericardium may require bypass support to reduce intraoperative risks. This is especially so in patients with extensive eggshell or porcelain pericardium encasing the heart as was the case in our patient. Fortunately, this presentation is relatively rare with only a few cases having been reported in the literature. A PubMed and Medline search of the English literature from 1970 to present identified nine cases in eight reports of patients with extensive or eggshell pericardial calcification from CP.[4],[6],[7],[8],[9],[10],[11],[12] Four of the reviews were reported as images in cardiology or cardiothoracic surgery with minimal information. Of the remaining, only two reports mentioned the surgical techniques with one performed using CPB and the other without. In our patient with encasing porcelain bony pericardium requiring a bone cutter, pericardiectomy could not have been safely undertaken without aid of CPB. In this case scenario with concrete bone completely covering the cardiac surface, CPB may only be feasible through peripheral cannulation of the femoral artery and vein. However, in our patient, we were able to find small space for central cannulation through the aortic arch. The use of bypass support enabled a more aggressive removal of the encasing bony pericardium since we had the ability to repair more easily any inadvertent cardiac injury without risk of exsanguination. In addition, the use of CPB by allowing a more extensive and complete removal of the constrictive pericardium may ensure more effective resolution of the constrictive pathophysiology. This translates to a higher probability for complete relief of symptoms, improved functional status, and exercise tolerance postoperatively. An inadequate removal of the constrictive pericardium may result in greater chance for residual symptoms postoperatively. Of the eight case literature reports, the etiology was all idiopathic except in one patient with presumptive TB CP who had been treated for pulmonary TB 27 years earlier.[12] Chronic TB lesions often end up with nonspecific cicatrization leaving no bacteriologic or histologic evidence of their origin.[4] There is, however, compelling evidence that large calcific pericardial deposits are indicative of burnt-out TB pericarditis.[13] As in our patient, there was no evidence of TB, but given the prevalence in our environment, our presumptive diagnosis was TB CP.


  Conclusion Top


Porcelain or eggshell CP though rare can pose significant technical surgical challenges with pericardiectomy. CPB can be a useful adjunct in these cases and in addition to mitigating the surgical risks also allows for a more complete resection of the constrictive bony pericardium.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Hoit BD. Pathophysiology of the pericardium. Prog Cardiovasc Dis 2017;59:341-8.  Back to cited text no. 1
[PUBMED]    
2.
Yetkin U, Kestelli M, Yilik L, Ergunes K, Kanlioglu N, Emrecan B, et al. Recent surgical experience in chronic constrictive pericarditis. Tex Heart Inst J 2003;30:27-30.  Back to cited text no. 2
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3.
Depboylu BC, Mootoosamy P, Vistarini N, Testuz A, El-Hamamsy I, Cikirikcioglu M, et al. Surgical treatment of constrictive pericarditis. Tex Heart Inst J 2017;44:101-6.  Back to cited text no. 3
    
4.
Diethrich EB, Tolls R. Bone around the heart: Presentation of a case of chronic constrictive pericarditis and review of recent surgical literature. Cardiovasc Dis 1977;4:37-48.  Back to cited text no. 4
[PUBMED]    
5.
Yangni-Angate KH, Tanauh Y, Meneas C, Diby F, Adoubi A, Diomande M, et al. Surgical experience on chronic constrictive pericarditis in African setting: Review of 35 years' experience in Cote d'Ivoire. Cardiovasc Diagn Ther 2016;6:S13-9.  Back to cited text no. 5
    
6.
Vijayvergiya R, Vadivelu R, Mahajan S, Rana SS, Singhal M. Eggshell calcification of the heart in constrictive pericarditis. World J Cardiol 2015;7:579-82.  Back to cited text no. 6
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Srinivasan N, Gupta R, Garrison M, Blevins S. Heart in a hard cage: Startling calcific constrictive pericarditis. Congest Heart Fail 2008;14:161-3.  Back to cited text no. 7
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de Luca G, Ponticelli P, Coronella G, Pepino P. Massive and severe calcific constrictive pericarditis. Ann Thorac Surg 2012;94:1738.  Back to cited text no. 8
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9.
Song BG, Kang GH, Park YH, Chun WJ, Oh JH. Heart in an eggshell calcification: Idiopathic calcific constrictive pericarditis. Cardiol Res 2011;2:310-2.  Back to cited text no. 9
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10.
Flecher E, Heautot JF, Abouliatim I, Leguerrier A. Calcified chronic constrictive pericarditis: An egg in the chest. Eur J Cardiothorac Surg 2009;35:174.  Back to cited text no. 10
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Lowenthal MN, Lowenthal A. Hearts of stone: Calcific constrictive pericarditis. Isr Med Assoc J 2000;2:410.  Back to cited text no. 11
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12.
Divakaramenon SM, Krishnan MN, Venugopal K. Calcific constrictive pericarditis (concretio cordis). Heart 2005;91:18.  Back to cited text no. 12
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Roberts WC. Pericardial heart disease: Its morphologic features and its causes. Proc (Bayl Univ Med Cent) 2005;18:38-55.  Back to cited text no. 13
[PUBMED]    


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