Case presentation:
A 36 years-old Iranian male patient was presented to our emergency
department (Fatemeh-Zahra hospital of Sari University of Medical
Sciences, Sari, Mazandaran, Iran.) with pleuritic chest pain, dyspnea,
fever, and chills. His first symptoms had been pleuritic chest pain,
dyspnea, and fatigue which began about one month earlier and had been
progressively worsened in the last few days. The patient described the
chest pain to be sharp, radiate to the left shoulder, worse in supine
position, and alleviated while leaning forward. He reported exertional
dyspnea with a III to IV function class of the New York heart
association (NYHA) classification [6]. He described the fever to
have an intermittent pattern and was first detected 10 days before
hospital admission. He had no rash, alopecia, or oral ulcers. He did not
complain of arthralgia or morning stiffness. Our patient had been
visited by a pulmonologist before hospital admission, and was first
prescribed with bronchodilators which did not relief his symptoms.
Therefore, a chest computed tomography scan (CT scan) was performed and
revealed enlarged cardiac silhouette, severe pericardial effusion, and
mild to moderate left-sided pleural effusion, for which he was referred
to our institution for pericardiocentesis and further investigations.
He had a history of a psoriatic-like rash on his posterior neck about a
month ago which resolved with topical treatment with no recurrence. He
did not mention any history of recent viral or bacterial infection. His
family history was negative for mycobacterium tuberculosis infection or
any autoimmune diseases.
In physical examination, the patient looked slim (i.e., height: 173 cm,
weight: 60 kg, and body mass index at 20.04). He was febrile (i.e., body
temperature at 39.5 °C), had a pulse rate of 82 beats per minute, blood
pressure of 70/110 mmHg, respiratory rate of 14 breaths per minute, and
elevated jugular venous pressure (JVP). Heart sounds were muffled and
pericardial friction rub was heard. Respiratory sounds were decreased at
the base of the left hemithorax. There was bilateral distal lower limb
pitting edema up to the distal part of his shins which was graded as 1+
(i.e., rebounded in 2-3 seconds after applying pressure). Pulsus
paradoxus was not present. There was not any rash, alopecia, or oral
ulcers. In electrocardiogram (ECG), generalized low voltages were seen.
Transthoracic echocardiography (TTE) showed large pericardial effusion,
more than 4cm in diameter, with loculation and septation, fulfilled with
fibrin strands (i.e., bread and butter pattern). Increased pericardial
thickening, more than 1cm thickness, with constrictive physiology were
observed. No pericardial calcification was seen (Figure 1). There was
septal bounce with abnormal double motion of interventricular septum in
diastole (Figure 2). Septal shift was seen in apical 4 chamber view with
bulging of the interventricular septum with inspiration toward the left
ventricle (LV), causing underfilled LV and bulging of interventricular
septum toward the right ventricle (RV) at LV filling with expiration,
all happening reversely (Figure 3) (Supplementary video 1). Doppler
study showed significant respiratory variations of tricuspid and mitral
valve. The tricuspid valve inflow E velocity was increased more than
50% with inspiration, and the mitral valve inflow E velocity was
increased more than 40% with expiration. Tissue doppler imaging showed
exaggerated E’ velocity in septum (Figure 4 (A)) and annulus reversus
(i.e., decreased E’ lateral velocity compared to E’ septum velocity)
(Figure 4 (B)). TTE study in subcostal view demonstrated pericardial
thickening (Figure 5 (A)) (supplementary video 2) and dilated inferior
vena cava (IVC) without respiratory collapse (Figure 5 (B)).