A 56-year-old female with past medical history of heart failure with preserved ejection fraction, obesity treated with Roux-en-Y gastric bypass surgery, type 2 diabetes mellitus, recent COVID-19 related massive pulmonary embolism (PE) 2 months prior, presented with chief complaints of dyspnea on exertion. Dyspnea prevented her from doing activities of anything more than minimal exertion (NYHA class 2). She also noted generalized weakness, and bilateral lower extremity edema for one month. In addition, she complained of sharp nonradiating retrosternal chest pain that worsened with inspiration. Her home cardiac medications included twice daily furosemide and rivaroxaban, with which she reported compliance. She denied orthopnea, paroxysmal nocturnal dyspnea, and cough. This was her third admission in a month for similar complaints. The initial onset of her symptoms was one month after her recent massive PE that had occurred two months prior to the current hospitalization, in the setting of COVID-19 infection. She had required systemic thrombolysis after multiple failed attempts at catheter-directed thrombolysis due to difficulty engaging the pulmonary artery. Despite multiple hospitalizations and aggressive diuresis, she had had progressive worsening of her dyspnea.
Her vitals at presentation were blood pressure 90/67 mm Hg, heart rate 108 beats per minute, temperature 99  F, respiratory rate 20 breaths/minute, and oxygen saturation of 96% on room air. Physical examination was suggestive of right-sided heart failure with elevated jugular venous pressure, 3+ bilateral lower extremity pitting edema and clear lungs. Her BMI was 30 kg/m2. Cardiac auscultation revealed normal S1 and S2 with no rubs, murmurs, or gallops. Her initial labs (Table 1) were significant for mild transaminitis and an elevated brain natriuretic peptide. Her creatinine was also elevated at 1.57 mg/dL (baseline 0.81-1.00 mg/dL). Electrocardiogram (EKG) showed normal sinus rhythm at 98 beats per minute and nonspecific ST-T wave changes in inferior and lateral leads, which were similar to her prior EKGs.
Initial Computed Tomography (CT) scan of the chest with contrast obtained in the emergency department showed interval resolution of pulmonary embolism but revealed bilateral pleural effusions and trace pericardial effusion. She was admitted with a diagnosis of acute on chronic heart failure with preserved ejection fraction and intravenous diuresis was initiated. A transthoracic echocardiogram (TTE) was performed which was technically a very difficult study due to patient's body habitus but revealed a low-normal left ventricular ejection fraction (LVEF) of 50% and no regional wall motion abnormalities. Patient did not respond to diuretic therapy and developed hypotension requiring midodrine. Cardiology was consulted who reviewed the TTE images more closely and noticed respirophasic abnormal septal motion concerning for constrictive physiology. The patient underwent right and left heart catheterization, which showed a mean right atrial pressure of 19.5 mm Hg with a prominent y descent. The right ventricular pressure was 35/19 mm Hg. The pulmonary artery pressure was 30/19 mm Hg with a mean of 24 mm Hg. The mean wedge pressure was 20 mm Hg and the left ventricular end-diastolic pressure was 24 mm Hg. In addition, there was an equilibration of diastolic pressures across all cardiac chambers, square root sign (dip and plateau pattern of right ventricular and left ventricular diastolic tracing), and discordant respiratory variation (Fig. 1). These hemodynamic findings were all consistent with CP. Cardiac catheterization also showed a low cardiac index of 1.09 based on thermal dilution and 1.6 based on Fick's principle. Thus, she was transferred to the cardiovascular intensive care unit for intravenous inotropes and vasopressors.
Patient underwent a transesophageal echocardiogram (TEE) and a coronary angiogram in anticipation of a pericardiectomy procedure. The TEE (Fig. 2) showed an LVEF of 55% with respirophasic abnormal septal motion, bi-atrial dilation, significant respiratory variation of the mitral and tricuspid valve inflow, and increased echodensity of the pericardium with no pericardial effusion, all of which again pointed towards constrictive physiology. Coronary angiogram did not reveal any obstructive coronary artery disease.
To establish the cause of her CP, we obtained more history and reviewed her chart in greater detail. She denied any cardiac surgery, prior chest irradiation, travel to tuberculosis endemic areas, any symptoms concerning for autoimmune disease, or use of any medications associated with a risk for developing CP. There were also no concerns for malignancy as she did not have any suggestive symptoms and was up to date on her cancer screenings. However, she was found to have a moderate pericardial effusion on a CT scan of her chest one month after systemic thrombolysis for her massive PE, which was noted to be only trace on CT scan at this admission (Fig. 3). We hypothesized that she developed a hemorrhagic pericardial effusion from the systemic thrombolysis versus the multiple failed attempts at catheter-directed thrombolysis for her massive pulmonary embolism, which led to constrictive pericarditis.
A gadolinium enhanced Cardiac Magnetic Resonance Imaging (CMRI, Fig. 4) was performed to rule out restrictive cardiomyopathy that showed mild diffuse thickening of up to 4 mm and subtle enhancement of the pericardium with a positive septal bounce. This confirmed CP as the diagnosis.
She underwent pericardiectomy which resulted in significant improvement of her symptoms, hemodynamics, kidney function and liver enzymes. Her pericardial biopsy revealed marked fibrosis, mild acute and chronic inflammation with hemosiderinladen macrophages, and no malignancy (Figs. 3 & 5). This confirmed our suspicion of hemorrhagic pericardial effusion being the etiology of her constrictive pericarditis. The patient was weaned off of all blood pressure support measures and was subsequently discharged to cardiac rehab.