Management of Constrictive Pericarditis Due to Calcified Thickened Pericardium of up to 18 mm Thick With Impaired Ventricles

Patient Selection

Constrictive pericarditis results from a stiff pericardium that prevents adequate diastolic filling and can cause diastolic heart failure. The normal thickness of the pericardial is found in up to 18% of constrictive pericarditis cases (1). Myocardial and pericardial cells are both involved in the pathogenesis of pericarditis and constriction as indicated by the patterns of inflammation and fibrosis in the pericardium (2). In a systematic review of recurrent pericarditis, from 1966 to 2006, there were no cases of constrictive pericarditis in the group of patients with recurrent pericarditis and only 1% in idiopathic acute pericarditis (3). Another study showed that constrictive pericarditis was a rare complication of viral or idiopathic acute pericarditis while nonidiopathic aetiologies (especially bacterial) were correlated with an increased risk of constrictive pericarditis (4). Idiopathic subacute pericardial effusion may progress to transient constrictive pericarditis. However, pericardiocentesis and prolonged anti-inflammatory treatment can completely resolve the pericardial syndrome without pericardiectomy (5). Conditions predisposing to calcific pericardial heart disease are Idiopathic, tuberculosis, infection, rheumatic fever, and trauma. Histologic examination of calcified pericardia rarely provides specific diagnoses (6).The class I recommendations with level C evidence for the diagnostic investigations of constrictive pericarditis include chest X-ray, CT scan cardiac MRI, and TTE, while cardiac catheterization is indicated when noninvasive tests fail to provide definite diagnosis of constrictive pericarditis (7). The right and the left ventricle are interdependent as both structures are nested within the pericardium, both have a common septum, and both are encircled with common myocardial fibers (8). The unique features of constrictive pericarditis are ventricular interdependence and dissociation of intrathoracic and intra-cardiac pressures as understood in echocardiography and cardiac catheterization (9). The class I recommendation with level C evidence for the treatment of chronic permanent constrictive pericarditis is pericardiectomy (7). Total pericardiectomy is associated with lower perioperative and late mortality and provides significant long-term superior hemodynamics as compared to partial pericardiectomy (10, 11).

The risk factors of overall mortality in pericardiectomy for constrictive pericarditis included NYHA class III or IV, aetiology of radiation, or post cardiac surgery, and a need for cardiopulmonary bypass (12). The reduced left ventricular ejection fraction and right ventricular dilatation have significant risk of early mortality while the presence of coronary artery disease, chronic obstructive pulmonary disease, and preoperative renal insufficiency has long-term mortality risks (13).

The patient in this video was a 54-year-old man who presented with a six-month history of progressive dyspnea, leg oedema, and abdominal distension. His weight reached up to 102 Kg. He suffered from type II diabetes, was a smoker (38 pack years), and drank alcohol in excess, 40 units per week. He was active in his life and worked as a lorry driver but was unable to work for the last few months. His chest X-ray showed a large right-sided pleural effusion and a calcified pericardium. Later, the pleural effusion was drained with a small chest drain. He underwent further investigations. CT scan showed gross ascites, right-sided pleural effusion, and a calcified pericardium.

Cardiac MRI confirmed extensively calcified and thickened pericardium with the maximum thickness of 18 mm (normal pericardial thickness is 2 mm or less). Both parietal and visceral layers were involved, adhered to the LV lateral and inferolateral walls, and encased the whole heart. It also showed that the left ventricular ejection fraction was 36% while the right ventricular ejection fraction was 43%.

Cardiac MRI demonstrated septal bounce, a sign of ventricular interdependence. This happens in the conditions where an increase in the volume of one ventricle causes a decrease in the volume of the opposite ventricle. Septal bounce is demonstrated by paradoxical interventricular septal movement during early diastole; the septum initially moves towards and then away from the left ventricle as seen in the video clips.

Coronary angiography did not show flow limiting coronary artery disease, however, it did show a rim of calcified pericardium.

The authors discussed this case in their multidisciplinary team meeting with the cardiologists and other cardiac surgeons due to the high risk of mortality (14). The MDT recommended pericardiectomy with informed patient consent.

Furthermore, perioperative TOE showed a globally bright pericardium with spontaneous contrast in the dilated right atrium that indicated RA diastolic collapse and poor forward flow. TOE also demonstrated septal bounce, similar as described in detail with the cardiac MRI. It also showed a thickened right ventricular wall and a small hyperdynamic left ventricle. The left ventricle ejection fraction (EF) was 27.8%, calculated with the Simpson’s biplane method.

Operative Steps

1. Use a preoperative intra-aortic balloon pump (IABP) after the induction of anesthesia to improve left ventricular function as ‘left ventricular contraction is very important for right ventricular developed pressure and volume outflow’ (15, 16).
2. Carefully undertake a midline sternotomy, keeping in mind that the posterior sternum might have adhesions with the pericardium.
3. Gently apply the retractor, opening a bit initially, as opening widely may tear the heart.
4. Cautiously divide the parietal pericardium longitudinally with the curved Metzenbaum scissors; start from the diaphragmatic end and gradually proceed upward.
5. Gently suck and remove the debris underneath the fibrous calcified pericardium.
6. In some areas the fibrous and the serous pericardium are firmly adherent, therefore dissect very carefully.
   1. Hold the edge of the pericardium with the Roberts or Allis forceps; dissect very gently with a very low setting of the diathermy in desiccated mode.
   2. For stiff tissues, use the curved Metzenbaum scissors and dissect very gently.
7. Use the De-Bakey or the Gillies fine-toothed forceps as well as the Watson Cheyne dissector and gently dissect the calcified visceral pericardium from the surface of the ventricle.
8. Gently Peel off the calcified visceral pericardium, bit by bit. Continue the dissection of visceral pericardium with the curved Metzenbaum scissors and the Gillies fine-toothed forceps.
   1. Use the Bailey Aortic Valve Rongeur to peel off the calcium gently.

Attempt to do total pericardiectomy, as it is associated with lower perioperative and late mortality and confers significant long-term advantage by providing superior hemodynamic (10, 11).

• Further dissect and remove the pericardium up to the right phrenic nerve and the left phrenic nerve.
• Dissect the thick calcified pericardium around the superior vena cava and release the constriction.
• Try to dissect and excise the pericardium, from the right to the left phrenic nerve and from the SVC to the IVC, from the right atrium and from the inferior part of the right ventricle adjacent to the diaphragm as far as possible. Leave behind a few islands of the pericardium if the constricting peel is adherent and calcified (7).

In the end, the heart should look free from the constriction.

Tips & Pitfalls

1. Attach the external defibrillator pads before starting the operation as there will be no space inside to use the internal defibrillator if required urgently.
2. Keep the cardiopulmonary bypass system available as stand-by (7).
3. Be very cautious when dissecting near to the right and left phrenic nerves.

Postoperative Management

• The authors kept the intra-aortic balloon pump (IABP) for 48 hours to support left and right ventricular function (15, 16).
• Milrinone and Nor-Adrenaline infusion was continued for four days.
• The patient was discharged to the normal surgical ward on the fifth postoperative day in good clinical status.
• Angiotensin II receptor antagonists, Spironolactone, and Furosemide were started.
• On subsequent days, the patient recovered. Leg oedema and ascites improved. His breathing substantially improved and he was physically active enough to walk outside silently for smoking.
• He was discharged on postoperative day 10 in good condition.

The patient was reviewed by the heart failure nurse on the fourth day of discharge. The patient informed them that he had improved further since he was discharged from the hospital. He walked 15-20 minutes daily without dyspnea. He did not report orthopnea, paroxysmal nocturnal dyspnea, chest pain, dizziness, or palpitations. His legs were less oedematous, while the body weight decreased further by 5.6 Kg, 20 Kg less than the preoperative weight. Oxygen saturation was 100% on air.

Histology

Microscopic:

• This specimen is predominantly comprised of necrotic material with focal dystrophic calcification.
• There is a small focus of viable fibroadipose tissue with attached cardiac muscle and hyalinised collagen.
• Special stains (ZN, ZN-modified and Grocott) are negative, however histology is NOT the best method of detecting micro-organisms.

Acknowledgements:

The authors thank Saad A Khan and Zabreen Ahmed for editing.

References

1. 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;108(15):1852-7. doi: 10.1161/01.CIR.0000087606.18453.FD. Epub 2003 Sep 29. PMID: 14517161.
2. Ramasamy V, Mayosi BM, Sturrock ED, Ntsekhe M. Established and novel pathophysiological mechanisms of pericardial injury and constrictive pericarditis. World J Cardiol. 2018;10(9):87-96. doi:10.4330/wjc.v10.i9.87
3. Imazio M, Brucato A, Adler Y, Brambilla G, Artom G, Cecchi E, Palmieri G, Trinchero R. Prognosis of idiopathic recurrent pericarditis as determined from previously published reports. Am J Cardiol. 2007 Sep 15;100(6):1026-8. doi: 10.1016/j.amjcard.2007.04.047. Epub 2007 Jul 6. PMID: 17826391.
4. 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;124(11):1270-5. doi: 10.1161/CIRCULATIONAHA.111.018580. Epub 2011 Aug 15. PMID: 21844077.
5. Paiardi S, Pellegrino M, Cannata F, Bocciolone M, Voza A. Transitory effusive-constrictive pericarditis. Am J Emerg Med. 2018 Mar;36(3):524.e1-524.e6. doi: 10.1016/j.ajem.2017.11.047. Epub 2017 Nov 21. PMID: 29169889.
6. Roberts WC. Pericardial heart disease: its morphologic features and its causes. Proc (Bayl Univ Med Cent). 2005;18(1):38-55. doi:10.1080/08998280.2005.11928030
7. Yehuda Adler, Philippe Charron, Massimo Imazio, Luigi Badano, Gonzalo Barón-Esquivias, Jan Bogaert, Antonio Brucato, Pascal Gueret, Karin Klingel, Christos Lionis, Bernhard Maisch, Bongani Mayosi, Alain Pavie, Arsen D Ristić, Manel Sabaté Tenas, Petar Seferovic, Karl Swedberg, Witold Tomkowski, ESC Scientific Document Group, 2015 ESC Guidelines for the diagnosis and management of pericardial diseases: The Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology (ESC) Endorsed by: The European Association for Cardio-Thoracic Surgery (EACTS), European Heart Journal, Volume 36, Issue 42, 7 November 2015, Pages 2921–2964, https://doi.org/10.1093/eurheartj/ehv318
8. Robert Naeije, Roberto Badagliacca, The overloaded right heart and ventricular interdependence, Cardiovascular Research, Volume 113, Issue 12, October 2017, Pages 1474–1485, https://doi.org/10.1093/cvr/cvx160
9. Syed FF, Schaff HV, Oh JK. Constrictive pericarditis--a curable diastolic heart failure. Nat Rev Cardiol. 2014 Sep;11(9):530-44. doi: 10.1038/nrcardio.2014.100. Epub 2014 Jul 29. Erratum in: Nat Rev Cardiol. 2015 Dec;12(12):682. PMID: 25072910.
10. Chowdhury UK, Subramaniam GK, Kumar AS, Airan B, Singh R, Talwar S, Seth S, Mishra PK, Pradeep KK, Sathia S, Venugopal P. Pericardiectomy for constrictive pericarditis: a clinical, echocardiographic, and hemodynamic evaluation of two surgical techniques. Ann Thorac Surg. 2006 Feb;81(2):522-9. doi: 10.1016/j.athoracsur.2005.08.009. PMID: 16427843.
11. Cho YH, Schaff HV, Dearani JA, Daly RC, Park SJ, Li Z, Oh JK. Completion pericardiectomy for recurrent constrictive pericarditis: importance of timing of recurrence on late clinical outcome of operation. Ann Thorac Surg. 2012 Apr;93(4):1236-40. doi: 10.1016/j.athoracsur.2012.01.049. Epub 2012 Mar 3. PMID: 22386088.
12. Takashi Murashita, Hartzell V. Schaff, Richard C. Daly, Jae K. Oh, Joseph A. Dearani, John M. Stulak, Katherine S. King, Kevin L. Greason, Experience With Pericardiectomy for Constrictive Pericarditis Over Eight Decades, The Annals of Thoracic Surgery, Volume 104, Issue 3, 2017, Pages 742-750, ISSN 0003-4975. https://doi.org/10.1016/j.athoracsur.2017.05.063.
13. Busch C, Penov K, Amorim PA, Garbade J, Davierwala P, Schuler GC, Rastan AJ, Mohr FW. Risk factors for mortality after pericardiectomy for chronic constrictive pericarditis in a large single-centre cohort. Eur J Cardiothorac Surg. 2015 Dec;48(6):e110-6. doi: 10.1093/ejcts/ezv322. Epub 2015 Sep 15. PMID: 26374871.
14. Landex NL, Ihlemann N, Olsen PS, Gustafsson F. Constrictive pericarditis in a contemporary Danish cohort: aetiology and outcome. Scand Cardiovasc J. 2015 Apr;49(2):101-8. doi: 10.3109/14017431.2015.1014406. Epub 2015 Mar 10. PMID: 25658593.
15. Damiano RJ Jr, La Follette P Jr, Cox JL, Lowe JE, Santamore WP. Significant left ventricular contribution to right ventricular systolic function. Am J Physiol. 1991 Nov;261(5 Pt 2):H1514-24. doi: 10.1152/ajpheart.1991.261.5.H1514. PMID: 1951739.
16. Santamore WP, Lynch PR, Heckman JL, Bove AA, Meier GD. Left ventricular effects on right ventricular developed pressure. J Appl Physiol. 1976 Dec;41(6):925-30. doi: 10.1152/jappl.1976.41.6.925. PMID: 1002647.

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