A 67-year-old woman was admitted to our cardiology department for dyspnea. Her medical history included hypertension, atrial fibrillation, diabetes mellitus, and Degos disease, and she had been taking low-dose aspirin for 20 years.
At the time of diagnosis, she exhibited cutaneous signs, and a histopathological examination displayed perivascular lymphocytic infiltration with distinct mucin deposition. These lesions were associated with Degos disease [1, 2]. No systemic symptoms were observed. Three years ago, gastrointestinal endoscopy revealed a small intestinal lesion, which was suspected as a systemic manifestation of Degos disease [5].
On admission, her blood pressure was 110/62 mmHg, and her heart rate was 99 beats/min with atrial fibrillation. Physical examination revealed liver enlargement, jugular vein distension with Kussmaul’s sign, and limb edema. Chest radiography revealed bilateral pleural effusion and calcification of the pericardium. Computed tomography revealed bilateral pleural effusion and pericardial effusion with marked calcification of the pericardium (Fig. 1).
Cardiac catheterization revealed equal right and left ventricular end-diastolic pressures and square root signs (Fig. 2). No coronary artery stenosis was observed. Echocardiography revealed pericardial thickening, pericardial effusion, ventricular septal paradoxical motion, septal bounce, and a normal left ventricular ejection fraction. The cutaneous signs were similar to those observed 20 years ago. Endoscopy revealed the same findings 3 years previously [5].
Despite optimal medical treatment, her heart failure did not improve, and the patient became catecholamine-dependent. Therefore, surgical pericardiectomy was performed.
During the operation, the pericardium was markedly thickened and calcified. The pericardium was incised, and 200 ml of bloody fluid was suctioned. Inside the pericardial sac, there were adhesions with some calcification (Fig. 3A), partly infiltrating the myocardium (Fig. 3B). The thickened pericardium was then thoroughly resected.
The central venous pressure decreased from 30 to 16 mm Hg, and the cardiac diastolic capacity improved.
Histopathological examination of the pericardium revealed a high degree of fibrosis, vitrification, and calcification of the pericardium. Lymphocytic infiltration was observed around the pericardial vessels (Fig. 4A, B).
The postoperative course was uneventful. The patient was extubated on day 1, discharged from the intensive care unit on day 2, and discharged on day 18. After surgery, the patient received aspirin, furosemide, spironolactone, bisoprolol, and perindopril erbumine treatment for 4 years. Her heart failure has not worsened.