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The American Journal of Medicine (2006) 119, e1-e4

CLINICAL COMMUNICATIONS TO THE EDITOR Constrictive Pericarditis Masquerading as Chronic Idiopathic Pleural Effusion: Importance of Physical Examination
To the Editor: We describe a patient who was labeled as having idiopathic pleural effusion for several years. A careful physical examination claried the diagnosis and resulted in a curative procedure. 1A) that increased with inspiration (positive Kussmauls sign). A pericardial knock was present at the left lower sternal border. The cardiac impulse was not palpable, and there was no cardiac enlargement on percussion of the chest. There was dullness to percussion over the right lung base with absent breath sounds, hepatomegaly, and lower extremity edema. Chest radiography showed a small rightsided pleural effusion and a normal-sized heart with no evidence of pericardial calcication. The electrocardiogram showed sinus rhythm, low-voltage QRS, and nonspecic T-wave abnormalities. The bedside clinical diagnosis was constrictive pericarditis, with no differential diagnosis (S.H.R.). The diuretic was discontinued. Transthoracic and transesophageal echocardiography/ Doppler showed no pericardial effusion. The pericardium appeared minimally thickened, biatrial enlargement was evident, and the left ventricle (LV) size and function were normal. Signicant respiratory variations in the Doppler signals, obtained across the mitral and tricuspid inows, could not be documented with condence. Computed tomography and magnetic resonance imaging of the heart reconrmed the possible minimal pericardial thickening and showed no calcication or any other pathologic abnormalities. Cardiac catheterization showed near equalization of the right atrial (RA) and pulmonary artery (PA) wedge pressures and of the diastolic right ventricle (RV) and LV pressures (Figures 1A and B). The discordance between the RV and LV systolic pressures could not be convincingly demonstrated. Coronary arteriography showed no coronary arterial obstruction. At surgery, the pericardium had an abnormal brous appearance with diffuse thickening of 5 to 7 mm (Figure 2) and was easily removed. After pericardiectomy, the RV systolic pressure decreased from 42 to 30 mm Hg, and the RA pressure decreased from 20 to 14 mm Hg. The postoperative course was uneventful, and the patient was discharged on the eighth day with no medications. At the 6-month postoperative evaluation, the patient was asymptomatic.

CASE REPORT
A 40-year-old Honduran woman was admitted to the cardiology service in late 2004. For 3 years she had dyspnea on exertion, lower extremity swelling, dry cough, and threepillow orthopnea, but she denied paroxysmal nocturnal dyspnea, and her exercise capacity was very limited (New York Heart Association functional class III/IV). History In January 2002, diabetes mellitus, hypertension, and a right pleural effusion were diagnosed in the patient. Pulmonary function test results showed mixed restrictive-obstructive disease. Bronchoscopy ndings were negative. Computed tomography of the chest showed a right-sided pleural effusion. Pleural biopsy showed chronic inammatory changes, and the pleural uid was a transudate. Four antituberculous medications were prescribed for a presumed diagnosis of tuberculosis, although diagnostic studies were not diagnostic, and the pleural uid was a transudate. In 2003, all of these tests were repeated, and the results were negative. Physical Examination The patient had a paradoxical pulse of 15 to 20 mm Hg and a blood pressure of 110/66 mm Hg. The peaks of the jugular venous pulse (JVP) were higher than the angle of the mandible even when the patient was sitting up. The troughs of the JVP showed prominent x and y descents (Figure
Dr. Rahimtoola is on the Speakers Bureau of Merck and Pzer and has received honoraria from three companies that manufacture prosthetic heart valves. Requests for reprints should be addressed to Shahbudin H. Rahimtoola, MD, University of Southern California, 2025 Zonal Avenue, Los Angeles, CA 90033. E-mail address: rahimtoo@usc.edu.

DISCUSSION
This patient had a diagnosis of chronic idiopathic pleural effusion for 3 years even though the pleural uid was a transudate. The most common cause of a transudate pleural effusion is heart failure.1 Pleural effusions occur in 44% to

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The American Journal of Medicine, Vol 119, No 7, July 2006

Figure 1 A: Right atrial (RA) and pulmonary artery (PA) wedge (indirect left atrial) pressures are elevated. Because of respiratory variation, the PA wedge pressure is higher at times, but the mean RA pressure is 18 mm Hg and the mean pulmonary artery wedge pressure is 20 mm Hg. The x descent is more prominent than the y descent. B: Simultaneous left ventricle (LV) and right ventricle (RV) pressure tracings show that the diastolic pressures are similar (difference is 2-3 mm) and exhibit the ventricular square root sign best seen in the rst and fourth diastolic periods. Diastolic RV and LV pressure are the same as atrial pressures (18-20 mm Hg).

50% of patients with constrictive pericarditis.2,3 Previously, the most common cause of constrictive pericarditis was an infection (especially tuberculosis), but at the present time the common cause is previous cardiac surgery, secondary to therapeutic mediastinal irradiation and idiopathic.4 Pathophysiology and Clinical Signs of Constrictive Pericarditis In constrictive pericarditis, the heart is enclosed in a thickened, rigid pericardium resulting in a restricted and xed

amount of space in the heart (Figure 3). On inspiration the increased venous return to the RA results in an increased ow to the RV, but all of the blood cannot be accommodated in the RA and RV. This has two effects: (1) The JVP is increased and increases further on inspiration (Kussmauls sign),5 and the hepatic venous ow and pressures are also increased. The JVP has a sharp x descent because of accelerated atrial relaxation and a prominent y descent from early rapid, but short, resistance-free lling of the RV (Figure 1). The latter also results in RV pressure having an

Constrictive Pericarditis

e3 Pericardial knock occurs 0.06 to 0.10 seconds after the aortic component of the second heart sound,7 and the third heart sound usually occurs 0.12 to 0.16 seconds after the aortic component of the second heart sound. On occasion the two occur at approximately the same time (0.10-0.12 seconds). The diagnostic nding on echocardiography and Doppler studies is the characteristic respiratory variation in Doppler ow velocities. This includes an increase in the tricuspid E velocity on inspiration (which is also present in the hepatic veins), an increase of more than 25% in the mitral E velocity, and a decrease in the tricuspid and hepatic venous diastolic ow velocity on expiration.6,8 The higher spatial resolution of computed tomography (and its sensitivity to diagnose calcication) and magnetic resonance imaging help in determining the pericardial thickness. Magnetic resonance imaging is said to be able to show a pericardium thicker than 4 mm.9 Moreover, in up to 18% of patients the pericardium may be of normal thickness ( 2 mm).4 LV systolic function is usually normal. Later abnormal LV systolic function results from associated coronary artery disease or calcium spicules from the calcied pericardium that extends into the epicardial layer of the myocardium and results in myocardial damage and necrosis. If the pericardium is not calcied, the surgeon can easily peel off the pericardium from the heart. The denitive therapy is pericardiectomy. Medical treatment is directed to treat the underlying cause. The high lling pressure is needed for rapid early lling of the RV and LV. The RV and LV are lled almost completely in the rst one-third of diastole; thus, the RV and LV stroke volume is more or less xed, and the cardiac output is largely determined by an increase of heart rate.

Figure 2 Abnormally thickened (5-7 mm) pericardium that was removed by pericardiectomy.

early rapid diastolic decrease and increase followed by a at pressure in mid- and late diastole, producing the ventricular square root sign (Figure 1B). The RV systolic pressure is increased, as is RV output. (2) The interventricular septum shifts to the LV. Pulmonary venous ow is reduced from a decreased RV output in the previous expiration. These conditions result in a decrease of LV cavity, LV systolic pressure, and systemic arterial pressure and ow. In expiration, this process is reversed. Pulmonary venous (PV) return is increased because of the increased RV output from the previous inspiration, and the interventricular septum shifts back into the RV. As a result, LV systolic pressure, volume, and stroke volume (LVSV) are increased, and systemic arterial pressure is increased. The RV systolic pressure, volume, and stroke volume (RVSV) are reduced. These pathophysiologic changes explain the observed signs of constrictive pericarditis, namely, Kussmauls sign; paradoxical pulse; echocardiographic/Doppler velocities in the hepatic veins; tricuspid and mitral inow velocities; and discordant changes in systolic pressures in the RV and LV. In diastole, the pressures in both atria and both ventricles are identical or very similar. Thus, by careful examination of the JVP one can know the mean pressures in the whole heart at the patients bedside. The other two conditions in which this last phenomena occurs are cardiac tamponade and large atrial septal defect or single (common) atrium. Changes in venous return to the heart in healthy persons result in a normal decline of arterial pressure of 4 to 6 mm Hg. In pulsus paradoxus, arterial pressure is greater than 10 mm Hg. The term paradoxical is thus a misnomer because it is an exaggeration of the normal variation of blood pressures with respiration. It was described as paradoxical by Kussmaul5 in 1873 because of the discrepancy between heart action and arterial pulse, and partly because the pulse in spite of apparent irregularity shows in reality a regular repeating decrease or disappearance. Pulsus paradoxus occurs in 30% of patients with constrictive pericarditis. Other causes include acute/chronic obstructive lung disease, pulmonary embolus, tense ascites, RV infarction, and circulatory shock.5,6

Figure 3 Diagrammatic illustration of the abnormal hemodynamics. The sizes of the circles represent the increases and decreases of venous return. The sizes of the arrows represent the increases and decreases of ow. The broken arrows indicate retrograde ow. The thick black line outside the heart represents rigid pericardium. SVC superior vena cava; IVC inferior vena cava; HV hepatic vein. Please see text for additional abbreviations.

e4 Attempts to normalize venous pressure and heart rate may result in a disastrous decrease of cardiac output and must be avoided. In summary, constrictive pericarditis is an abnormality with an anatomic construct that has a characteristic pathophysiology and classic physical signs. The abnormalities may be difcult to document or interpret by noninvasive tests, especially if the pericardium is not calcied. Our patient illustrates that imaging modalities should not be a substitute for a thorough physical examination. Mohammed W. Akhter, MD Ismael N. Nuo, MD Shahbudin H. Rahimtoola, MD, DSc (Hon)
The Grifth Center, Division of Cardiovascular Medicine, Department of Medicine, Department of Cardiothoracic Surgery, LACUSC Medical Center, University of Southern California, Keck School of Medicine, Los Angeles, Calif.

The American Journal of Medicine, Vol 119, No 7, July 2006

References
1. Light RW. Pleural effusion. N Engl J Med. 2002;346:1971-1977. 2. Wychulis AR, Connolly DC, McGoon DC. Surgical treatment of pericarditis. J Thorac Cardiovasc Surg. 1971;62:608-617. 3. Bertog SC, Thambidorai SK, Parakh K, et al. Constrictive pericarditis: etiology and cause-specic survival after pericardiectomy. J Am Coll Cardiol. 2004;43:1445-1452. 4. Talreja DR, Edwards WD, Danielson GK, et al. Constrictive pericarditis in 26 patients with histologically normal pericardial thickness. Circulation. 2003;108:1852-1857. ber Schwieligie Mediastino-Pericardits und den Para5. Kussmaul A. U doxenpuls. B Klin Wschr. 1873;10:433. Cited in: Fowler NO. The paradoxical pulse (pulsus paradoxus). In: The Pericardium in Health and Disease. New York: Futura Publishing Company, Inc.; 1985:235245. 6. Goldstein JA. Cardiac tamponade, constrictive pericarditis, and restrictive cardiomyopathy. Curr Probl Cardiol. 2004;29:503-567. 7. Tyberg TI, Goodyer AVN, Langou RA. Genesis of pericardial knock in constrictive pericarditis. Am J Cardiol. 1980;46:570-575. 8. Oh JK, Hatle LK, Seward JB, et al. Diagnostic role of Doppler echocardiography in constrictive pericarditis. J Am Coll Cardiol. 1994;23: 154-162. 9. Wang ZJ, Reddy GP, Gotway MB, et al. CT and MR imaging of pericardial disease. Radiographics. 2003;23:S167-S180.

doi:10.1016/j.amjmed.2005.09.011

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