Case Presentation:
An 84-year-old female with a history of ET, factor V Leiden, hypertension and bilateral lower extremity venous insufficiency complicated by multiple ulcers presented to the emergency department (ED) after a fall secondary to a syncopal episode. She was ambulating in her apartment at the nursing home when she lost consciousness. In the preceding weeks, the patient had been experiencing intermittent, pleuritic chest pain, palpitations, in addition to resting and exertional shortness of breath. She denied orthopnea, paroxysmal nocturnal dyspnea, cough, hemoptysis, fever, unintentional weight loss, recent viral illness or sick contacts, tuberculosis exposure, malignancies, and autoimmune disease. Further history revealed that the patient’s pleuritic chest pain, palpitations and shortness of breath started after the patient’s hematologist switched to anagrelide from hydroxyurea because it was likely impeding the healing of her venous ulcers. The dose of anagrelide was gradually increased to 1 mg twice daily, worsening the patient’s aforementioned symptoms. Her other medications included candesartan 16 mg daily and warfarin 5 mg daily.
Initial vital signs consisted of blood pressure 137/81 mm Hg, heart rate 76 beats per minute, respiratory rate 18 respirations per minute, oxygen saturation of 100% on room air, and temperature 97.6° F (36.4° C). Her physical examination was only notable for 3+ bilateral lower extremity edema and multiple venous stasis ulcers, with evidence of JVD and crackles. Laboratory findings included white blood cell count 34.01 x 10³/mcL, hemoglobin 9 g/dL, platelet count 916 10³/mcL, potassium 5.4 mmol/L, troponin 0.039 ng/mL, and NT-pro B-type natriuretic peptide 982 pg/mL. Her admission chest radiograph was unremarkable. However, electrocardiogram (ECG) showed new-onset atrial fibrillation with rapid ventricular response (Figure 1A) which resolved with administration of 500 mL of intravenous normal saline. A bedside echocardiogram showed a large pericardial effusion. The patient was admitted to the cardiac care unit for further care. TTE confirmed the large pericardial effusion and revealed tamponade physiology including abnormal respirophasic variation, free wall inversion and early diastolic right ventricular collapse (Figure 1B-C).
The patient remained hemodynamically stable without any clinical signs of cardiac tamponade. Her oxygen requirement consistently ranged from 2 L nasal cannula to being in room air throughout her hospitalization. Malignancy, tuberculosis and autoimmune workup was negative. As a result, in the absence of common causes, the patient’s pericardial effusion was attributed to anagrelide and the medication was therefore stopped after consulting hematology. Given the high risk of tamponade, pericardiocentesis was offered and later attempted, but proved to be challenging due to increased pericardial clotting and anatomical variance with high risk for RV puncture. Right heart catheterization ruled out constrictive and restrictive patterns, and showed elevated left and right pressures. She continued to have mild, intermittent shortness of breath as well as pleuritic chest pain, both of which were symptomatically managed with nebulized ipratropium bromide/albuterol, supplemental oxygen and pain control as needed. Repeat TTE showed a mildly improved, but persistent pericardial effusion, without tamponade physiology. Given the likelihood of re-accumulation, a pericardial window was offered, but after multiple multidisciplinary discussions concerning her goals of care and previous attempt, the patient declined and decided to be discharged back to her nursing home.