Late Effusive-constrictive Pericarditis after Lung Transplantation

A 65-year-old man with persistent atrial fibrillation and stage II chronic kidney disease presented with new onset of dyspnea on exertion, abdominal distention, and lower-extremity edema 4 years after bilateral sequential lung transplantation. He had been maintained on a triple-drug immunosuppressive regimen of tacrolimus, mycophenolate mofetil, and prednisone. A panel of reactive antibodies, cytomegalovirus serology, computed tomographic (CT) pulmonary angiogram, and pulmonary function testing were unremarkable. Echocardiography demonstrated a pericardial effusion with early tamponade that required pericardiocentesis. Pericardial fluid analysis was negative. A follow-up echocardiogram 2 weeks later demonstrated normal left ventricular systolic function, thickening of the pericardium, and abnormal interventricular septal motion (Video). A chest CT scan confirmed thickening of the pericardium (Figure A). Hemodynamic evaluation noted equalization of right ventricular and left ventricular diastolic pressure (Figure B). Pericardiectomy was performed via median sternotomy. Dense pericardial adhesions and a thick pericardial membrane were encountered intraoperatively. The histopathological specimen demonstrated fibroconnective tissue with chronic nonspecific inflammatory changes (Figure C). Postoperatively, hemodynamic instability and septicemia resulted in anuric acute kidney injury. The patient elected not to pursue hemodialysis and focus on comfort care only.

Symptoms of dyspnea after lung transplantation are not uncommon. They are most concerning for allograft rejection, infection, pulmonary embolism, or airway or vascular complications. Pericardial constriction results from progressive pericardial fibrosis and manifests with dyspnea and right heart failure. Its incidence is 2% to 3% after cardiothoracic surgery. Constriction is exceedingly rare after lung transplantation, possibly due to the chronic use of corticosteroids as part of the immunosuppressive regimen.^{,  ,  ,}  In our institutional experience, of 736 lung transplant recipients, only 3 (0.4%) have been diagnosed with constrictive pericarditis: one with infectious pericarditis, one with uremic pericarditis, and the patient described in this report. The definitive treatment is pericardiectomy. Surgical mortality from pericardiectomy increases in the presence of intractable right heart failure and comorbid conditions. It is particularly high in elderly and debilitated patients. Early diagnosis of constriction is important to reduce morbidity and mortality. Pericardial constriction should be considered in symptomatic lung transplant recipients irrespective of time elapsed after surgery.