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.