The American Heart Hospital Journal


For nine years of this standard treatment for heart failure, the patient remained at her New York Heart Association class I functional capacity. She then developed insidious progressive impairment in her exercise tolerance. Her B-type natriuretic peptide (BNP) increased from 71 to 378pg/ml over one year (normal level <100pg/ml). Her chest X-ray revealed a curvilinear calcification adjacent to the left heart border (see Figure 1). Computed tomography of the chest demonstrated a 2–3mm pericardial calcification that extended to both left and right sides and all the way up to the reflection of the pericardium onto the great vessels. A tuberculin skin test was negative. CPX testing showed a significant decline in her exercise


capacity, with peak VO2 15.2ml/kg/min. The patient underwent successful surgical total pericardiectomy (see Figure 2). Histopathologic evaluation of the pericardium revealed calcific fibrous thickening and minimal non- granulomatous lymphoplasmacytic infiltration. After surgery, the patient showed a remarkable improvement in symptoms with an increase in overall functional status. Her BNP decreased to 91pg/ml. Repeat CPX testing showed a dramatically improved peak VO2 of 28.3ml/kg/min.


Discussion


Our patient presented with acute onset of right-sided heart failure with evidence of right atrial and ventricular enlargement seen on imaging studies within two weeks after her flu-like illness. An extensive diagnostic work-up to exclude the possible etiologies of right-sided heart failure, including myocarditis, infiltrative disease, left-to-right intracardiac shunt, pulmonary embolism, pulmonary hypertension, and arrhythmogenic right ventricular dysplasia, turned out to be normal. Endomyocardial biopsy, viral serology, and computed magnetic resonance did not reveal any significant pathology. However, in view of the insensitivity of endomyocardial biopsy sampling,3–5 the patient’s viral prodrome and her symptoms preceding acute onset of heart failure with dilated cardiomyopathy of unknown etiology all suggest the presumable diagnosis of inflammatory heart disease.


Co-existence of myocarditis and pericarditis is frequently observed because these two entities of inflammatory heart disease share common etiologic agents, especially viral infections.2


Table 1: Investigations and Imaging Studies


BLOOD CHEMISTRY AND HEMATOLOGY BUN 12mg/dl (normal 7–23mg/dl) Creatinine 0.7mg/dl (normal 0.5–1.2mg/dl) WBC 9.2x103


/dl (normal 4–10x103 /dl)


Hemoglobin 13.4g/dl (normal 12–15g/dl) Uric acid 3.2mg/dl (normal 3.6–8.3mg/dl) ACE level 26 units/l (normal 12–68 units/l) ESR 2mm/hr (normal <26mm/hr) IMMUNOLOGIC STUDIES


ANA index 0.25 (normal <0.90)


Anticardiolipin antibodies IgG <10 GPL (normal <20 GPL) Anticardiolipin antibodies IgM <10 MPL (normal <20 MPL) Coxsackie B1–B6 Ab acute: <1:8 (normal <1:8) Coxsackie B1–B6 Ab convalescent: <1:8 (normal <1:8) IMAGING STUDIES Chest X-ray: Prominence of the right atrial silhouette and clear lung fields.


2D echocardiogram: Right ventricular enlargement with dilation of intrahepatic vein suggesting right atrial hypertension. No intracardiac shunt and abnormal ventricular wall motion. LVEF 60%. CT chest: Mild pericardial thickening with no significant pulmonary pathology. Pulmonary angiogram: Normal bilateral selective pulmonary arteriogram with no evidence of pulmonary embolism. Cardiovascular magnetic resonance: Marked enlargement of right atrium and ventricle. Right ventricular hypertrophy with distention of persisted left and right superior vena cava. Normal left-sided heart and pericardium.


ACE = angiotensin-converting enzyme; ANA = antinuclear antibody; BUN = blood urea nitrogen; CT = computed tomography; ESR = erythrocyte sedimentation rate; GPL = immunoglobulin G phospholipid unit; Ig = immunoglobulin; LVEF = left ventricular ejection fraction; MPL = immunoglobulin M phospholipid unit; WBC = white blood cell.


Figure 1: Chest Radiograph Demonstrating Curvilinear Calcification Adjacent to the Heart Borders (Arrows)


Case Report


Antipathogen antibodies that cross-react with myocardial epitopes may also cross-react with pericardial epitopes, leading to persistent immune activation and tissue inflammation. Alternatively, it is conceivable that, because of the proximity of the pericardium to the myocardium, inflammatory damage to one may also extend to the another.6


In this patient the initial questionable pericardial knock, square root configuration Winter 2010 Symptomatic Calcific Constrictive Pericarditis After Presumed Resolved Inflammatory Heart Disease 131


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