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)).