We report the case of a 48 years-old male patient with a past medical history of obesity and moderated alcohol habits. He denied smoking or illicit drugs consumption. His father died at the age of 63 years-old due to end-stage liver disease. No other relevant family disorders or cardiovascular diseases were reported.
This patient had a first hospital admission at the age of 41 years old, when he presented with predominant pleural effusion and ascites. He reported a history of progressively worsening abdominal distention and shortness of breath in the prior 2 months. The patient denied previous known infections, traveling abroad or lifestyle changes. No other cardiovascular symptoms, as chest pain or loss of consciousness were referred. The patient´s physical examination was notable for diffuse edema most prominent at the abdomen and diminished breath sounds with dullness to chest percussion.
Attending to this clinical scenario in a healthy young patient, he underwent an in-depth etiological investigation. Admission electrocardiogram showed sinus rhythm with no acute or dynamic changes. Initial work-up diagnosis included a wide range of analytic, serology and immunology tests. Hemogram, metabolic panel, liver enzymes and other biochemical markers were within normal limits, except a raised C reactive protein of 6 mg/dL (normal range less than 0.3 mg/dL). Serum protein (4.6 g/dL) and albumin (2.3 g/dL) levels were both low. 24-hours urinalysis was also normal. Further serological and molecular testing looking for an infectious etiology yielded negative results. The following microorganisms were searched: Bartonella spp., Coxiella burnetii, Brucella spp., Borrelia spp, Chlamydia spp. and Mycobacterium tuberculosis spp.
Specific immunological tests in-association with auto-immune diseases were performed. The antinuclear antibody immunofluorescence assay was positive at a titer of 1:300 and subsequent analysis was positive for antibodies anti-SS-A/Ro.
Imaging work-up included chest X-ray revealed a bilateral pleural effusion. An abdominal computed tomography for additional clarification revealed hepatomegaly with diffuse heterogeneity, splenomegaly and a diffuse subcutaneous cellular edema. A TTE was performed and it was reported as having no significant pathological findings.
Analysis of pleural and ascitic fluid revealed a transudative effusion and cytological analysis excluded the presence of malignant cells. It was also decided to perform a pleural biopsy that revealed nonspecific reactive inflammatory changes, with no evidence of pleural thickness or fibrosis. Despite of absence of sicca symptoms, but in face of the presence of anti-Ro antibodies, it was decided to perform a Schirmer test that was positive with a wetting of less than 3 mm on each eye.
At that time, attending to exams results and after exclusion of other rheumatologic conditions and sicca-related syndromes, the suspicion was of connective tissue disease, particularly primary Sjögren’s Syndrome (pSS). An empirical corticotherapy regiment was initiated and patient was discharged symptom-free.
The patient remained clinically stable until 2-years later, when he was readmitted in the hospital again due to dyspnea and abdominal distension. Hepatosplenomegaly and pleural effusion were detected after an initial imaging workup. Hepatic peliosis was diagnosed on liver biopsy and considered secondary to glucocorticoid therapy. A new thoracic CT scan was performed, and it revealed localized pericardial thickening and a thin circumferential pericardial effusion, however a new TTE was not performed. As the remaining diagnostic tests were unremarkable, diuretic therapy was adjusted to symptom-relief. The patient kept medical surveillance on Rheumatology consultant, due to the clinical suspicion of pSS and remained stable with posterior withdrawn of corticotherapy.
The patient was admitted again, around 4 years later, with relapse of dyspnea, orthopnea and ascites. Physical exam was notable for jugular venous distension, crackles on lung bases auscultation, ascites and bilateral leg edema. Chest X-ray upon admission revealed an important right pleural effusion with areas of passive atelectasis of the adjacent normal lung. Electrocardiogram showed atrial fibrillation rhythm with controlled ventricular rate (HF 86 bpm) and T wave flattening in all precordial leads. At this time a new TTE was performed showing left and right atrium enlargement, small ventricular cavities with mild left and right ventricular systolic dysfunction. It was also notable for increased respiratory phasic septal shift as well as mitral and tricuspid flow variations. The additional presence of mitral annulus reversus and annulus paradoxus, inferior vena cava plethora and of a thickened echogenic pericardium with calcifications and a small pericardial effusion raised the clinical suspicion of constrictive pericarditis. (Fig. 1). A new thoracic CT scan was performed, showing a thickened pericardium with extensive calcified pericardial plaques (Fig. 2). No evidence of masses or lymphadenopathies suggestive of malignancy. Subsequent cardiac catheterization confirmed the diagnosis by demonstrating elevated and equal diastolic pressures in both ventricles, a “square root” sign during diastole and an elevated systolic area index.
In face of this imaging features, the diagnosis of CP was confirmed. Due to the documented extensive calcifications it was assumed a chronic CP, so the patient was proposed and accepted for cardiac surgery with pericardiectomy. During the surgery, complex calcified adherences were evident around both ventricles and only a partial pericardiectomy was performed. The histologic tissue analysis revealed areas of hyaline fibrosis and calcification and a scanty mononuclear inflammatory infiltrate, suggestive of chronic pericarditis (Fig. 3). No evidence of granulomas, epithelial or lymphoid neoplastic tissue.
Since then, the patient referred clinical improvement with dyspnea and orthopnea relief, despite maintaining signs of peripheral congestion amenable with diuretic therapy. Imaging reevaluation with a TTE performed 3 months later still shows the same constrictive physiology signs that were present before surgical intervention.