Cardiac disease is the leading cause of death

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1 IMAGING TEACHING CASE Constrictive Pericarditis Masked by CAPD Asif S. Kazmi, MD, 1 Robert B. Canada, MD, 1 Ahmad Munir, MD, 1,2 and Barry M. Wall, MD 1,2 INDEX WORDS: Dialysis pericarditis; constrictive pericarditis; calcific pericarditis; continuous ambulatory peritoneal dialysis. Cardiac disease is the leading cause of death in the end-stage renal disease population. Pericardial diseases, although less common than other types of cardiac disease, continue to contribute to the increased cardiovascular morbidity and mortality in this population. Dialysis pericarditis is a term used to describe the occurrence of clinical features of pericardial disease after clinical stabilization on long-term dialysis therapy (usually 8 weeks after its initiation). Most observational data suggest inadequate dialysis is the major factor in its development. 1-7 Patients with constrictive pericarditis typically present with signs of systemic venous congestion, including hepatomegaly, ascites, and peripheral edema. Deep x and y descent in the jugular venous pulse and Kussmaul sign (jugular venous pressure does not decrease appropriately during inspiration or even increases) may also be present. With more prolonged and severe constrictive pericarditis, symptoms related to decreased cardiac output may occur, including dyspnea, hypotension, dizziness, fatigue, cachexia, and weight loss. 4,8 In this report, we describe a long-term dialysis patient who developed refractory hypotension related to constrictive pericarditis with extensive pericardial calcification. Many of the classic physical findings associated with constrictive pericarditis were absent or masked by ongoing volume removal by means of continuous ambulatory peritoneal dialysis (CAPD). 1-4 We discuss From the 1 University of Tennessee Health Science Center, and 2 Veterans Affairs Medical Center, Memphis, TN. Received September 23, Accepted in revised form May 12, Originally published online as doi: /j.ajkd on August 14, Address correspondence to Barry M. Wall, MD, VAMC 111B, 1030 Jefferson Ave, Memphis TN barry.wall@va.gov 2008 by the National Kidney Foundation, Inc /08/ $34.00/0 doi: /j.ajkd the role of cardiac imaging studies, including computed tomography, magnetic resonance imaging, echocardiography, and right-sided cardiac catheterization, which are central in establishing the diagnosis. CASE REPORT Clinical History A 36-year-old white man with end-stage renal disease requiring dialysis therapy for 16 years was seen in our peritoneal dialysis clinic on a routine visit. His blood pressure during the past 6 to 8 months had been low (80/40 to 90/50 mm Hg) without symptoms. More recently, he had noticed dizziness on walking, but denied chest pain, shortness of breath, or dependent edema. One year before, he had undergone evaluation for possible renal transplantation, at which time he was asymptomatic with blood pressure recorded as 100/60 mm Hg. A chest radiograph showed the presence of pericardial calcification. A transthoracic echocardiogram showed thickened pericardium with evidence of pericardial calcification and abnormal interventricular septal diastolic bounce. Further evaluation was recommended, but the patient refused. On physical examination, the patient was afebrile and in no distress. Heart rate was 98 beats/min, and blood pressure was 84/65 mm Hg while sitting and 95/68 mm Hg on standing with no pulsus paradoxus. Respiratory rate was 16 breaths/min. There was no jugular venous distension and Kussmaul sign was not present. Cardiac apical impulse was not displaced, and normal first and second heart sounds were audible. Lung fields were clear to auscultation. There was no hepatosplenomegaly, and bowel sounds were normal. There was trace dependent pedal edema. The cause of the patient s renal disease was unknown. He had a long history of poorly controlled secondary hyperparathyroidism (intact parathyroid hormone level range, 1,000 to 1,500 pg/ml and calcium-phosphorous product range, 50 to 66 mg 2 /dl 2 during the last 2 years). His prescribed medications included sevelamer, cinacalcet, allopurinol, ferrous sulfate, erythropoietin, omeprazole, and multivitamins. The patient s pharmacy records showed no prior use of hydralazine, minoxidil, alpha-methyldopa, or procainamide. Pertinent laboratory values included serum urea nitrogen, 30 mg/dl (10.7 mmol/l); creatinine, 13 mg/dl (991 mol/ L); calcium, 10.7 mg/dl (2.67 mmol/l); phosphorus, 6.3 mg/dl (2.03 mmol/l); and intact parathyroid hormone, 1,059 pg/ml. Imaging Studies A transthoracic echocardiogram, compared with results from 1 year previously, showed worsening of the abnormal 1016 American Journal of Kidney Diseases, Vol 52, No 5 (November), 2008: pp

2 Constrictive Pericarditis in a CAPD Patient 1017 Figure 1. Echocardiogram shows pericardial thickening and calcification (arrows). Heart chambers are not well visualized, probably because of severely thickened and calcified pericardium. Abbreviations: LV, left ventricle; RV, right ventricle; LA, left atrium; RA, right atrium. interventricular septal diastolic bounce and significantly worsened pericardial thickening and calcification (Fig 1; Movie S1). Ejection fraction was 55%. There was no evidence of cardiac valvular calcification. Computed tomography of the chest showed encasement of the heart in a completely calcified pericardium, giving an eggshell appearance (Fig 2). There were no other calcifications noted in the chest or upper abdomen. Right-sided cardiac catheterization showed the following hemodynamic results. Pulmonary artery mean wedge pressure was 23 mm Hg. Pulmonary artery pressure was 34/22 mm Hg with mean pressure of 26 mm Hg. Right ventricular Figure 2. Computed tomographic scan of the chest, reconstructed sagittal view, shows encasement of the heart in a heavily calcified pericardium.

3 1018 Kazmi et al Figure 3. Right ventricular pressure tracing of the patient shows the characteristic dip and plateau pattern. pressure was 36/18 mm Hg, and right atrial mean pressure was 21 mm Hg. Cardiac output measured by means of thermodilution was 4.0 L/min, and cardiac index was 2.19 L/min/m 2. Right ventricular pressure tracings showed the characteristic dip and plateau of constrictive pericarditis (Fig 3). Right atrial pressure tracings showed the characteristic deep x and y descents of constrictive pericarditis (Fig 4). Clinical Follow-up Pericardiectomy was recommended to the patient, but he refused the procedure and continued peritoneal dialysis therapy. He died at home about 1½ months after these studies. Postmortem examination was not performed. DISCUSSION Imaging studies were critical in clarifying the cause of our patient s hypotension. Computed tomography is highly sensitive and specific in detecting pericardial thickness and calcification. The presence of pericardial thickening or pericardial calcification per se is not diagnostic of constrictive pericarditis. However, cardiac structures may show characteristic morphological features consisting of decreased volume and a narrow tubular configuration of the right ventricle. A sigmoid-shaped ventricular septum or prominent leftward convexity in the septum may also be demonstrable. Systemic venous congestion (particularly in the inferior vena cava), hepatomegaly, and ascites may also be detected. Disadvantages of computed tomography include the use of intravenously administered iodinated contrast media and ionizing radiation. 9 Cardiovascular magnetic resonance (CMR) has developed into an excellent tool for cardiac imaging. CMR has become the diagnostic procedure of choice for the detection of pericardial diseases, including constrictive pericarditis. CMR imaging permits direct visualization of the normal pericardium, which is composed of fibrous tissue and has low CMR signal intensity. The normal pericardium measures approximately 3 mm in thickness. Although pericardial fluid also has low signal intensity, it can be distinguished from pericardium with cine magnetic resonance images, in which it has a bright signal in contrast to the dark line of the pericardium. Myocardial tagging techniques can show tethering of pericardium to the heart during the cardiac cycle. Furthermore, real-time cine imaging allows direct visualization of the effects of respiration on ventricular filling and septal wall configuration. The presence of pericardial effusion can be established and the distinction can be made between a hemorrhagic and nonhemorrhagic effusion. However, gadolinium-based imaging should be avoided in patients with moderate to severe kidney disease (especially dialysis patients) because of the risk of nephrogenic systemic fibrosis Echocardiography is readily available and is typically the first imaging study used in the evaluation of suspected constrictive pericarditis. Two-dimensional echocardiography characteristically shows thickened and immobile pericardium with transmission of heart motion to surrounding organs (tugging). The interventricular septum shows classic displacement in early diastole (septal bounce) (Movie S1). Septal bounce

4 Constrictive Pericarditis in a CAPD Patient 1019 Figure 4. Right atrial pressure tracing of the patient shows the characteristic deep x and y descents. results from the inability of the intrathoracic pressure change during respiration to affect the left side of the heart, as it does the right side of the heart. During inspiration, the pressure difference between the pulmonary veins and left atrium is small, such that flow into the left atrium is decreased. This results in a decrease in left ventricular filling, and because of a constricted pericardium, a shift in the interventricular septum occurs. During expiration, the septum will shift in the opposite direction. The inferior vena cava is plethoric and unresponsive to respiration. The heart has an overall tubular appearance with reduced size of the left and right ventricles. Mild biatrial enlargement may be present (Movies S2, S3, and S4). 12,13 Findings during right-sided cardiac catheterization confirm the presence of constrictive pericarditis. In constrictive pericarditis, the presence of a heavily fibrosed or calcified pericardium restricts diastolic filling of all chambers of the heart and thus reduces the diastolic volume of the heart. The symmetrical constricting effect of the pericardium results in the increase and equilibration of diastolic pressures in all 4 cardiac chambers (as well as pulmonary capillary wedge pressure). Almost all the diastolic filling occurs very rapidly and early in diastole because of increased venous pressure, and very little filling occurs in late diastole, with equilibration of the pressures occurring secondary to noncompliance of the pericardium. This results in the characteristic dip and plateau waveform in ventricular pressure time plots in early and late diastole, respectively 12 (Fig 3). Many of the clinical signs and symptoms associated with constrictive pericarditis were masked in our patient by the control of extracellular fluid volume by means of continuous peritoneal dialysis therapy. It is likely that the intermittent nature of hemodialysis would have resulted in severe hypotension such that volume removal would have been impossible. The presence of dialysate in the peritoneal cavity concealed any possible ascites. Marcu et al 8 also reported a patient on CAPD therapy with constrictive pericarditis who had no edema, jugular venous distention, or hepatosplenomegaly in whom cardiac imaging studies were essential in establishing the diagnosis. ACKNOWLEDGEMENTS Support: None. Financial Disclosure: None. SUPPLEMENTARY MATERIALS Movie S1: Apical 4-chamber view on transthoracic echocardiogram in the patient described in this case. Movie S2: Apical 4-chamber view on transthoracic echocardiogram in a patient showing circumcardiac pericardial effusion. Movie S3: Parasternal long-axis view on transthoracic echocardiogram in a patient delineating pericardium between large pericardial and pleural effusions. Movie S4: Apical 4-chamber view on transthoracic echocardiogram in a patient showing absence of pericardial effusion after successful pericardiocentesis.

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