Winning Abstracts from the 2012 Medical Student Abstract Competition: Pulmonary Hypertension 15 Years Following Aspergillus Empyema: Case Report and Review of the Literature
Author: 2nd Lt. James D. Covelli, Uniformed Services University of the Health Sciences F. Edward Hébert School of Medicine, Class of 2013
Introduction: Pulmonary arterial hypertension (PH) is a devastating condition that results in small vessel fibrosis and destruction. There are numerous potential etiologies, and treatment of PH is based on the World Health Organization (WHO) Classification System. Group 5 of the WHO Classification System includes PH with unclear multifactorial etiologies such as hematologic disorders, metabolic disease and sarcoidosis.
Case Presentation: We report a case of a 61-year-old Caucasian male with a history of Aspergillus empyema in 1997 requiring thoracotomy with decortications, and long-term therapy with itraconazole. Evaluation for immunosuppressive disorders was negative, and he had otherwise been healthy prior to the infection. He presented approximately 12 years later with new onset slowly progressive dyspnea, cough and orthopnea. He had evidence of left heart failure at that time and was treated aggressively with diuretics and afterload reduction. Tricuspid regurgitation was seen on echocardiogram (ECHO) with no evidence of valvular pathology or systolic or diastolic dysfunction. At initial cardiac catheterization, severe pulmonary hypertension with a mean pulmonary artery pressure of 63 mmHg (normal 15-25 mmHg) without evidence of obstructive coronary artery disease was present. He demonstrated vasodilator responsiveness with inhaled nitric oxide. Following aggressive diuresis, ECHO demonstrated markedly abnormal right heart function, with preserved left ventricular function. Computed tomography imaging revealed marked pulmonary artery enlargement, mediastinal lymphadenopathy and multiple dense calcifications predominantly in the left hilar and subcarinal regions, associated with marked decreased size of the left chest cavity. Pulmonary function testing demonstrated severe restriction. Laboratory testing revealed an increased ESR of 34 mm/hr and C-reactive protein elevated at 2.99 mg/dL, but otherwise, there was no evidence of an autoimmune disorder. With the addition of Sildenafil and inhaled prostacyclin to his diuretic regimen, his functional capacity has improved along with improvement in perceived dyspnea.
Discussion: Pulmonary hypertension following fungal empyema has not been previously reported, but it has been described secondary to fibrosing mediastinitis as a complication of sarcoidosis. We postulate our patient developed a similar process secondary to aspergillus empyema. Clinicians should be aware that patients with fungal empyema may be at increased long-term risk for pulmonary hypertension secondary to a process similar to fibrosing mediastinitis, and this may be successfully treated with conventional pulmonary hypertension therapies.