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BILATERAL PLEURAL EFFUSIONS: A RARE PRESENTATION OF CONSTRICTIVE PERICARDITIS Manesh Kumar Gangwani1, Salman Bin Mahmood1, Javaid Ahmed Khan2. 1. Department of Medicine, The Aga Khan University 2. Professor, Department of Medicine, The Aga Khan University ABSTRACT Constrictive pericarditis is a rare presentation. We need a very high index of clinical suspicion to diagnose the disease. It most commonly presents secondary to Tuberculosis in developing world and post radiation in developed world. Classically it presents with symptoms of heart failure and as pericardial thickening or calcification on imaging studies. In hospital settings, constrictive pericarditis is not usually considered as a differential in patients presenting with pleural effusions. Literature suggests, in cases of constrictive pericarditis, associated pleural effusions could be left sided. Here we present two unusual presentations of cases with bilateral pleural effusions. We suggest that whenever dealing with cases of bilateral pleural effusion, etiology of constrictive pericarditis should be considered. INTRODUCTION Constrictive Pericarditis is a rare disease that can be a challenge to diagnose unless a high index of suspicion is maintained.1 Classically it presents with symptoms of heart failure and as pericardial thickening or calcification on imaging studies. However, initial diagnostic studies remain non-specific in a significant proportion of cases. Chest Radiographs show calcifications in only half the cases with Echocardiogram being sensitive in 37% of cases for pericardial thickening.2 Furthermore, the pericardial thickness may be normal in about 20% of patients. The etiology of this disease has evolved over the years, especially in the Western world, due to an increase in the number of cases secondary to cardiothoracic surgery or previous radiotherapy.3 In the United States only, the incidence of such cases is 800,000 annually. However, in the developing world tuberculosis (TB) remains the leading cause of this presentation.4 Pleural effusion is known to be associated with constrictive pericarditis in up to 55% of cases5 and is usually left-sided at presentation.6 Right sided or bilateral pleural effusions are more characteristic of heart failure. Here we present a case of recurrent unexplained bilateral pleural effusion which eventually was diagnosed to be constrictive pericarditis. CASE 1 A 61 year old male, known asthmatic, presented to our pulmonology clinic with complaints of weight loss for 2 months, and dry cough and dyspnea since 3 months. His history was positive for TB contact. On examination, he had a pulse of 88bpm, respiratory rate 22/min, BP 130/71 and on auscultation had decreased breath sounds on the right side. Radiological imaging revealed large pleural effusion on the right side and narrowing of right main bronchus. An ultrasound guided diagnostic thoracentesis was performed and his pleural fluid analysis revealed a protein content of 4.5 g/dl, glucose of 121 mg/dl, LDH of 158 IU/L and a white blood cell count of 200 cells/mm3, with 80% lymphocytes. A diagnosis of TB versus malignancy was considered and therapeutic thoracentesis was performed and 1450 ml of fluid was drained. AFB smear and cultures were both negative. Four drug anti-tuberculosis treatment (ATT) regimen for six months was still started empirically. Afterwards patient again underwent pleural taps 4 times at another center. One week later patient presented to our emergency with hydropneumothorax. Chest tube insertion with lung re-expansion was performed along with drainage of 1800ml of fluid and another 690 ml was drained on the subsequent day. Patient was later discharged and chest tube was removed in the clinic after resolution of the pneumothorax. One month later patient again presented to the emergency with complaints of shortness of breath along with dry cough. He had a pulse of 90bpm, respiratory rate 20, BP 121/74 and on auscultation had decreased breath sounds on the right side along with pitting edema. Radiological imaging revealed right sided loculated effusion(Figure 1). His pleural fluid analysis revealed a protein content of 3.4 g/dl, glucose of 91 mg/dl, and a white blood cell count of 1300 cells/mm3, with 90% lymphocytes. Another AFB smear performed came out to be negative. Therapeutic thoracentesis was again performed and 1100 ml of fluid was drained. Post tap imaging revealed fibrous bands on right side of chest X-ray. The patient was then discharged with a plan to continue his ATT and to get and echocardiogram as an outpatient investigation. Two-dimensional echocardiogram revealed that both atria were mildly dilated and left ventricular systolic function was preserved with ejection fraction of 55%. There was prominent septal bounce and significant respiratory variation in mitral and tricuspid inflow velocities. Pulmonary vein Doppler showed prominent diastolic flow consistent with increased left atrial pressure. Thickened pericardium was also visible on M-mode. Furthermore, these findings in correlation with the clinical picture, suggested the diagnosis of constrictive pericarditis. The patient was advised a cardiac MRI and subsequent pericardiectomy and referred to cardiothoracic surgery and is awaiting surgery. Figure 1 CASE 2 A 52 year old gentleman, known case of diabetes mellitus and hypertension, presented with dyspnea, bilateral swelling of the feet, non-productive cough and undocumented weight loss. He had a previous history of TB contact. On examination, the patient was vitally stable with BP of 124/84, Pulse 88, Respiratory rate 20/minute and was afebrile. Trachea was central, bilateral basal crepitations on chest auscultation were heard and were more marked on the right side. Bilateral pedal edema was also present. Rest of the systemic examination was unremarkable. Radiological imaging showed bilateral pleural effusion(Figure 2). AFB smears were negative. A suspicion of constrictive pericarditis was raised. Computerized tomography(Figure 3) of the chest showed moderate bilateral pleural effusion, significant pericardial thickening and enhancement with minimal pericardial effusion with pressure effects on the liver with venous congestion and delayed hepatic vein filling. A transthoracic echocardiogram was ordered which showed normal sized cardiac chambers, normal left ventricular systolic function and grade I left ventricular diastolic dysfunction. E/E' was 10, suggestive of normal LV filling pressure. E' at medial mitral annulus was 0.06 m/s and at lateral mitral annulus was also 0.06 m/s. Significant respiratory variation of E wave was noted at mitral valve. Dilated inferior vena cava with loss of inspiratory collapse was also noted. Trace to mild circumferential pericardial effusion with significant pericardial thickening was present. Prominent diastolic flow reversal was noted in hepatic venous flow during expiration. These features again suggested constrictive pericarditis. Patient was acutely managed with diuretics and advised further workup to establish diagnosis. However, patient was lost to follow up. Patient subsequently presented to our emergency after 3 months with exacerbation of his shortness of breath. On examination he was hemodynamically stable but had a respiratory rate of 25 breaths/min. Chest auscultation revealed bilateral basal crepitations. This time his chest xray showed a right sided pleural effusion. A pleurocentesis was done. His pleural fluid analysis revealed protein content of 3.2 g/dl, glucose of 441 mg/dl, LDH of 177 IU/l and a white blood cell of count of 220 cells/mm3, with 55% lymphocytes. AFB smear was negative. Cytology was also negative for any malignancy. Cardiothoracic surgery was taken on board and patient was advised emergent pericardiactomy. However patient preferred to undergo an elective procedure and the cardiology team was involved to optimize his clinical condition with regards to shortness of breath. Later, the patient underwent pericardiectomy. Post operatively the patient dropped blood pressures and required high inotropic support. This was followed by further deterioration of renal functions and he required continuous renal replacement therapy. Due to suspicion of cardiac tamponade he underwent sternotomy again. However no evidence of tamponade was observed and patient was continued to be resuscitated. However his condition further deteriorated and he finally succumbed to his condition. Pericardial histopathology showed dense chronic inflammation and fibrosis compatible with clinical diagnosis of constrictive pericarditis. Figure 2 Figure 3 DISCUSSION A large proportion of the pleural effusion presentations will be due to congestive heart failure, malignancy, pneumonia, or pulmonary emboli. Bilateral pleural effusions can be caused by liver or renal failure, hypothyroidism, hypoalbuminemia and constrictive pericarditis. Pleural effusion occurs in about 50% of patients with constrictive pericarditis7 and several mechanisms have been proposed for its occurrence. Diastolic dysfunction of the left ventricle might cause elevations in the intravascular hydrostatic pressure leading to a transudative pleural effusion. A similar mechanism may cause systemic venous congestion and ascites and this abdominal collection of fluid might later accumulate in the pleural cavity by tracking through defects in the diaphragm. Exudative pleural effusions are primarily inflammatory in etiology, and may result from a variety of infections, post-cardiac-injury syndrome or radiotherapy. TB is a common cause of constrictive pericarditis; other causes include purulent infections, trauma, cardiac surgery, mediastinal irradiation, histoplasmosis, neoplastic disease, acute viral or idiopathic pericarditis, rheumatoid arthritis, systemic lupus erythematosus and chronic renal failure treated by chronic dialysis.8 In both our cases, bilateral pleural effusion due to constrictive pericarditis secondary to tuberculosis must be ruled out due to the endemicity of this infection in our region. Constriction causes a defect in the heart’s diastolic filling due to reduced diastolic compliance resulting in decreased cardiac output. Presenting symptoms in constrictive pericarditis, hence, are very similar to congestive heart failure with peripheral edema, dyspnea, weight-gain, and ascites and pleural effusions. Raised jugular venous pressure may be a prominent physical sign, which would further increase during inspiration. The other important physical sign of constrictive pericarditis is a pericardial knock, a diastolic sound that occurs 0.05–0.10 seconds after the aortic component of the second heart sound, and usually before the third heart sound. Unexplained pleural effusion, especially in conjunction with a raised jugular venous pressure, presence of Kussmaul’s sign, pedal edema, and ascites, should alert the physician to the possibility of constrictive pericarditis. Constrictive Pericarditis has very specific findings on various investigative profiles. Echocardiography shows respiratory variation in Doppler flow velocities with characteristic changes in flow on inspiration and expiration across valves. Similarly, computed tomography or cardiac magnetic resonance imaging can help determine pericardial thickness and assess for pericardial calcification. Left sided pleural effusion are more characteristic of constrictive pericarditis; however, bilateral plural effusions also can be an initial presentation.9 Constrictive pericarditis can be treated conservatively or surgically, however the definitive treatment of constrictive pericarditis is surgical pericardiectomy. Although a small proportion of pleural effusion is attributable to constrictive pericarditis, management principles depend largely on correct diagnosis. We suggest that whenever dealing with cases of bilateral pleural effusion, etiology of constrictive pericarditis should be considered. REFERENCES: 1. 2. 3. 4. 5. 6. 7. 8. 9. Welch TD, Oh JK. Constrictive pericarditis: old disease, new approaches. Curr Cardiol Rep. 2015 Apr;17(4):20. Farkas JD, Amery S, Napier MB, Alderisio WG, Almond JG, Hellwitz FJ. 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