Case of a Weeping Pericardium
A 26-year-old male, with no significant past medical history, consulted to a community-based hospital for a 4-week history of shortness of breath and anasarca. The progressive dyspnea (New York Heart Association classification III/IV) prompted him to go to his local emergency room. He denied any chest discomfort, infectious symptoms, or recent travel. Except for a selective serotonin reuptake inhibitor (SSRI), he was not taking any regular medication. He denied alcohol or illicit drugs use and his vaccination status was up to date.
A computed tomography (CT) with contrast administration was initially performed to investigate the dyspnea and revealed bilateral segmental pulmonary emboli with a mildly dilated main pulmonary artery (measured at 31 mm) and some indirect signs of right heart failure (reflux of contrast in the inferior vena cava (IVC) and presence of moderate ascites). In addition, a moderate circumferential pericardial effusion (measured at most 15-18 mm) and bilateral pleural effusions were noted.
A transthoracic echocardiogram (TTE) showed a reduced left ventricular ejection fraction (LVEF) at 40%, with some regional wall motion abnormalities, and a severely hypokinetic right ventricle (RV). A paradoxical septal motion was reported and the IVC was plethoric with very little respiratory variation. Surprisingly, the RV was not dilated, and the estimated PA pressure was normal (24 mmHg, with a right atrial pressure 15 mmHg). A small circumferential pericardial effusion (at most 10 mm) was noted, with no hemodynamic impact.
Considering these findings, the patient was started on anticoagulation for the pulmonary embolism, diuretics, and was transferred to our quaternary care center for the rest of the of the investigation.
On arrival to our institution, the patient was found to be in sinus tachycardia (heart rate of 110 beats per minute), hemodynamically stable and afebrile. His saturation was 96%, not requiring any oxygen therapy. Electrocardiogram showed low voltages and nonspecific ST changes (Figure 1). On physical examination, the cardiopulmonary exam revealed distant heart sounds (no pericardial friction rub), an elevated jugular venous pressure (JVP) and decreased air entry bilateral with dullness on percussion. The abdomen was distended from the ascites seen on previous CT.
Initial laboratory work-up at our institution revealed a mild anemia hemoglobin 13 g/dL (normal 13-17 g/dL) with normal white blood cell and platelet count, an abnormal liver profile consisting of a bilirubin total 1.71 mg/dL (normal 0.3 – 1.0 mg/dL), a low albumin (3.2 g/dL) and a mildly elevated international normalized ratio (INR) 1.36 (normal value <1.1). Both alanine aminotransferase (ALT) and aspartate aminotransferase (AST) were normal. High sensitivity troponins, creatinine, lactates, and thyroid stimulating hormone (TSH) were all within the normal limits. Finally, a mild elevation of the inflammatory markers was noted with a C-reactive protein (CRP) 3.02 mg/L (normal < 0.8 mg/dL).
A cardiac magnetic resonance imaging (CMR) was subsequently performed and revealed an extensive pericardial effusion with constrictive physiology (inspiratory septal shift toward the LV). Both LV and RV systolic function were decreased (respectively 38% and 32%). Pericardial edema (on T2-weighted short-tau inversion recovery sequence) and circumferential pericardial enhancement on late gadolinium enhancement (LGE) images were both appreciated on this examination (Figure 2).
A repeat TTE, to assess specifically for constriction, showed a large circumferential effusion (measured at most 31.2 mm), a plethoric IVC, a tricuspid and mitral inflow respirophasic variation and a septal shift, all compatible with constrictive physiology (Figure 3).
Considering these findings, the patient was started on prednisone (0.25 mg/kg/day) and colchicine and a pericardiocentesis under ultrasound guidance was performed. About 200 ml of serosanguinous pericardial fluid was retrieved and sent for analysis. Cytology showed mixed neutrophils, lymphocytes, and occasional macrophages (consistent with acute and chronic inflammation). Bacterial, fungal, tuberculosis cultures and viral PCR were all negative.
Post pericardial drainage, a right and left heart catheterization confirmed the diagnosis of constrictive pericarditis by demonstrating an equalization of the end-diastolic pressure between the right-sided and left-sided chambers. The typical prominent Y descent (W-sign) was also appreciated on the right atrial pressure curve.
The patient was discharged with a slow tapering of the corticosteroids as an outpatient and a 3-month course of colchicine. Despite the normalization of the inflammatory markers, the patient experienced persistent symptoms limiting his activities. After 1 month of treatment, a repeat CMR was performed and demonstrated persistent pericardial enhancement on LGE sequences and extensive pericardial adhesions on real-time cine images, with no recurrence of pericardial effusion (Figure 4). A repeat TTE was also performed and showed persistent signs of constriction such as septal shift and expiratory diastolic flow reversal in the hepatic vein (Figure 4).
What would be the most likely diagnosis?