The fortress becomes a prison: calcified constrictive pericarditis

IMAGES IN RADIOLOGY Robert G. Stern, MD, Section Editor The Fortress Becomes a Prison: Calcified Constrictive Pericarditis María E. Sanhueza, MD,a Rubén Torres, MD,a Paula Segura, MD,b Arturo Villalobos, Medical Student,c Erico Segovia, MDa a c Section of Nephrology, Department of Medicine and bDepartment of Pathology, University of Chile Clinical Hospital, Santiago, Chile; Medical School, University of Chile, Santiago, Chile. PRESENTATION The pericardium, normally so useful in defending the heart from outside invaders, can perform more like a cage when its flexibility is compromised. We present the case of a 50-year-old man who was admitted to the nephrology unit when severe chronic hypotension led to poor hemodynamic tolerance during hemodialysis. He had a 24-year history of kidney disease of unknown etiology. At debut, he presented with pericardial effusion that required urgent initiation of hemodialysis. In 1993, he underwent a kidney transplant. Chronic graft dysfunction evolved, and in 2010, he started hemodialysis again. His medical history included chronic liver damage secondary to venous-occlusive disease induced by azathioprine and mesenteric thrombosis managed with oral anticoagulation. In 2011, he underwent subtotal parathyroidectomy for hyperparathyroidism. who have end-stage renal disease. These calcifications have been associated with other factors, such as hyperphosphatemia, hyperparathyroidism, oral anticoagulation, and chronic inflammation. The main sites of vascular calcification in patients with end-stage renal disease include the medial layer of the arteries and the heart valves. However, calcified pericardium is rare in this population. Pericarditis is frequently an idiopathic disease, but it can also occur after cardiotomy, acute myocardial infarction, radiation, tuberculosis, uremic syndrome, and systemic inflammatory disorders.1 Pericardial inflammation in endstage renal disease is a serious disorder, typically secondary to acute uremic syndrome or dialysis-related disease. Chronic constrictive pericarditis is unusual, and calcified constrictive pericarditis, in which the heart is encased within a rigid calcified pericardium, is even more exceptional.2,3 ASSESSMENT Upon evaluation, the patient had severe dyspnea, permanent jugular distension, severe hypotension, and slow capillary refill. A chest radiograph showed a high-density image outlining the pericardium (Figure 1). Transthoracic echocardiography revealed a thickened pericardium (6 mm) with normal systolic function and diastolic dysfunction. Computed tomography disclosed diffuse calcification and thickening of the pericardium (Figure 2A). DIAGNOSIS The patient was diagnosed with calcified constrictive pericarditis. Vascular calcifications are common among those Funding: None. Conflict of Interest: None. Authorship: All authors had access to the article and a role in writing the manuscript. Requests for reprints should be addressed to María Eugenia Sanhueza, MD, Section of Nephrology, Hospital Clínico Universidad de Chile, Santos Dumont 999, Santiago, Chile. E-mail address: hemofi[email protected] 0002-9343/$ -see front matter Ó 2016 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.amjmed.2015.10.006 Figure 1 A chest radiograph showed a high-density image outlining the pericardium (arrows). 264 The American Journal of Medicine, Vol 129, No 3, March 2016 Figure 2 (A) Computed tomography identified diffuse calcification and thickening of the pericardium (arrowheads). (B) Pericardial tissue (arrowhead) remained after pericardiectomy. MANAGEMENT Figure 3 surgery. This sample of pericardium was removed during Pericardiectomy is the treatment of choice for improving cardiac hemodynamics in patients with constrictive pericarditis. Our patient underwent this procedure because he had severe hemodynamic compromise from calcified constrictive pericarditis (Figures 2B and 3).2,4 A pericardial biopsy showed severely calcified pericardium with no evidence of inflammatory activity (Figure 4). Ziehl-Neelsen staining was negative. After surgery, he remained hospitalized in the intensive care unit for a prolonged period. Nonetheless, he died after multiple organ failure. The extreme degree of calcification identified in this patient is a rare condition. It can be explained by his long history of chronic kidney disease, uremic pericarditis at the initial diagnosis of end-stage renal disease, severe hyperparathyroidism, chronic hyperphosphatemia, and use of oral anticoagulation. His disease was so severe that his pericardium was transformed into a rigid cage, ultimately causing his death. Figure 4 (A) Tissue was treated with nitric acid, 5%. Fibrous and hypocellular tissues with few capillary vessels are visible in this photograph. The darker area (arrow) correlates with the focus of calcification. (B) Here, the tissue has not been treated with nitric acid, 5%. This image shows foci of calcification (arrow) corresponding to a site of tissue disruption caused by the hard consistency of the calcification (hematoxylin and eosin, 40). Sanhueza et al Calcified Pericarditis in a Hemodialysis Patient References 1. Szabó G, Schmack B, Bulut C, et al. Constrictive pericarditis: risks, aetiologies and outcomes after total pericardiectomy: 24 years of experience. Eur J Cardiothorac Surg. 2013;44:1023-1028. 2. Alpert MA, Ravenscraft MD. Pericardial involvement in end-stage renal disease. Am J Med Sci. 2003;325:228-236. 265 3. Park EA, Lee W, Kim KH, Chung JW, Park JH. Rapid progression of pericardial calcification containing a “calcium paste” in a patient with end-stage renal disease. Circulation. 2011;123:e262-e264. 4. Chowdhury UK, Subramaniam GK, Kumar AS, et al. Pericardiectomy for constrictive pericarditis: a clinical, echocardiographic, and hemodynamic evaluation of two surgical techniques. Ann Thorac Surg. 2006;81:522-529.