Rapid Progression of Pericardial Calcification Containing a “Calcium Paste” in a Patient With End-Stage Renal Disease
A 52-year-old man with end-stage renal disease (ESRD) secondary to chronic glomerulonephritis was admitted to our hospital with aggravated exertional dyspnea. He had been maintained on hemodialysis for the past 16 years. One year previously, he had been hospitalized for complicated pleural effusion, which was confirmed as Gram-positive cocci infection, and was treated through percutaneous drainage and antibiotics. Chest computed tomography (CT) taken at the time of initial hospitalization showed bilateral complicated pleural effusion and diffuse pericardial thickening without calcification (Figure 1). During that period, parathyroid hormone markedly increased to 1113 pg/mL (normal range, 10 to 65 pg/mL); hyperphosphatemia was also noted. His hyperparathyroidism was thought to be secondary to ESRD; thus, he underwent subtotal parathyroidectomy 6 months later. Serial plain chest radiographs at the time of pleural infection and at the time of parathyroidectomy showed rapid progression of the smooth and diffuse pericardial calcific opacities within only a 6-month period, and these findings were maintained until this admission (Figure 2). Transthoracic echocardiography revealed diffuse pericardial thickening with septal bouncing and inferior vena cava plethora, suggestive of constrictive pericarditis. ECG-gated cardiac CT showed very homogeneous calcific opacity in the pericardial space without fluid-fluid level and denser linear calcification along portions of the parietal and visceral pericardium (Figure 3). CT also showed that dense calcified plaques were present throughout all 3 coronary arteries. During admission, he suffered sudden cardiac arrest and was supported with extracorporeal membrane oxygenation. Emergency pericardiectomy was done under extracorporeal membrane oxygenation support. On surgery, a creamy whitish paste-like material was drained when the parietal pericardium was opened (Figure 4A). The parietal pericardium was 5 mm thick and heavily calcified (Figure 4B). Although the parietal pericardium was successfully removed, visceral pericardiectomy proved difficult because of the invasion of calcification deep into the myocardium. Extracorporeal membrane oxygenation weaning failed, and the patient died 2 weeks after pericardiectomy.
Although the 3 most identifiable causes of constrictive pericarditis are postcardiac surgery, radiation therapy, and tuberculosis, constrictive pericarditis can occur in ESRD patients, albeit infrequently. Pericardial involvement in ESRD patients manifests most commonly as acute uremic or dialysis pericarditis and less frequently as chronic constrictive pericarditis.1 Dialysis pericarditis is found in up to 5% of patients with ESRD despite otherwise successful renal replacement therapy and biochemical control, and it may be precipitated by viral or bacterial infection.2 We therefore assume that our patient might have been suffering from concurrent pericarditis when he was diagnosed as having infectious pleural effusion 1 year ago. Most cases of constrictive pericarditis are thought to follow an episode of acute pericarditis, with fibrous scarring and fusion of the visceral and parietal pericardium commonly developing during the healing process.2 Interestingly, however, in our patient, calcified visceral and parietal pericardium were not fused; rather, the pericardial space was filled with a calcium-rich pasty content.
The pericarditis of this patient has 2 interesting points: the very rapid progression of pericardial calcification and the filling of the pericardial space with paste-like calcium content. Rapid progression of pericardial calcification and calcified pericardial effusion in our patient can be a manifestation of extraskeletal calcification found in ESRD patients and is thought to be due to secondary hyperparathyroidism. In addition, several findings such as serum phosphate values >8 mg/dL or a calcium-phosphate product >70 mg2/dL2 have been regarded as risk factors for extraskeletal calcification.3 Indeed, our patient's calcium-phosphate product increased to 75 mg2/dL2 at the time of parathyroidectomy. The mechanism of rapid progression of pericardial calcification in our patient can be postulated as follows: Our patient likely developed dialysis-associated pericarditis precipitated by bacterial infection and exhibited rapid pericardial calcification owing to underlying calcium-phosphate metabolic abnormalities. To the best of our knowledge, the feature of a “calcium paste” that we believe resulted from the combination of extraskeletal calcification and effusive pericarditis in this patient with ESRD has not previously been reported.
This case illustrates constrictive pericarditis in a patient with ESRD that showed rapid progression and a “calcium paste” in the pericardial space.
- © 2011 American Heart Association, Inc.