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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.
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