A 61-year-old man with past medical history of coronary artery disease status postplacement of a bare-metal stent in the ramus branch of the left circumflex artery several years ago had end-stage renal disease secondary to long-term vesicoureteral reflux disease. He had a living donor renal transplantation performed 6 years ago that had remained stable on antirejection therapy with tacrolimus and mycophenolate mofetil. He also had a patent left brachiocephalic AV fistula that was placed for hemodialysis 3 years before his renal transplant.
He was referred to our cardiology clinic with progressive shortness of breath and bilateral lower extremity edema of 4 months duration. An echocardiogram showed moderate pericardial effusion, dilated right and left atria (Figure 1), and severe tricuspid regurgitation with a right ventricular systolic pressure of 41 mm Hg. Autoimmune panel, liver and thyroid function tests, tuberculosis testing, uric acid, and blood urea nitrogen levels were all normal. His glomerular filtration rate was 61 mL/min/1.73 m2. Tacrolimus level was 8.2 ng/mL (expected range 6-15 ng/mL). Furosemide was started with partial symptomatic improvement, but the effusion continued to recur despite diuretic therapy. Tacrolimus was suspected as a potential cause of his pericardial effusion and was replaced by cyclosporine. However, off tacrolimus, his symptoms continued, and his glomerular filtration ratio (GFR) worsened from 61 mL/min/1.73 m2 just before stopping the medication to 40 mL/min/1.73 m2 almost a month later. Thus, tacrolimus had to be reinstated to prevent rejection of the transplanted kidney.
Because of the persistence of symptoms with right heart failure and pericardial effusion despite aggressive diuretic therapy, he underwent a pericardial window and was placed on steroid tapering therapy as advised by nephrology. The pericardial fluid analysis revealed a transudate that was negative for malignancy. The anatomic pathologic results on the pericardial tissue sample showed only reactive mesothelial changes (Figure 2). His symptoms improved significantly but recurred 2 months later.
Cardiac magnetic resonance (CMR) imaging showed moderate pericardial effusion with flattening of the interventricular septum during inspiration, small bilateral pleural effusion, ascites, and normal myocardium (Figure 3). Left and right cardiac catheterization showed a cardiac output of 7.45 L/min, equalization of left ventricular and right ventricular diastolic pressures. The pressure waveform showed prominent Y descent with pronounced right ventricular rapid filling wave the "square root sign" (Figure 4). Coronary angiography showed total occlusion of nondominant right coronary artery with grade 2 left to right collaterals and a patent stent in the ramus branch.
The CMR and cardiac catheterization results suggested the presence of effusive-constrictive pericarditis, and the patient was then referred to cardiac surgery for possible total pericardiectomy. However, before proceeding with pericardiectomy, per the recommendation of the cardiac surgeon, closure of the patient's patent AV fistula was performed. To our surprise, shortly after AV fistula closure, the patient's symptoms completely subsided. An echocardiogram showed a small amount of pericardial effusion and mild tricuspid regurgitation. A repeat cardiac catheterization revealed resolution of the constrictive physiology with normalization of right and left heart hemodynamics (Figure 5; Table 1). He has remained asymptomatic during follow-up approximately 1 year later.