Disorders of the Pleura |

Unilateral Fibrothorax Resulting in Severe Pulmonary Hypertension and Cor Pulmonale FREE TO VIEW

Mediha Ibrahim, MD; Ajsza Matela, MD; Shilpa Desouza, MD; Joseph Mathew, MD
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Winthrop University Hospital, Mineola, NY

Chest. 2014;146(4_MeetingAbstracts):469A. doi:10.1378/chest.1993408
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SESSION TITLE: Pleural Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Dense fibrous tissue may form over the pleural surface for a variety of reasons. Pleural fibrosis usually follows intense inflammation of the pleura; however, the mechanisms following the inflammatory process leading up to fibrosis are unclear.

CASE PRESENTATION: A 60-year-old Liberian woman presented to our institution complaining of dyspnea and leg edema for 3 months. She had a history of pneumothorax from prior trauma 20 years ago, however did not have any procedural interventions at the time. Vitals signs were temperature 97.2F, blood pressure 95/71mmHg, pulse 104/min, respiratory rate 24/min and oxygen saturation 87% on ambient air. Physical findings included jugular venous distension, diminished breath sounds on the right, loud P2, and 3+ lower extremity edema. CT pulmonary angiogram showed a large right thoracic mass compressing the entire right lung, destruction of right-sided posterior ribs, and peripheral filling defects in the main pulmonary artery and right main pulmonary artery. Echocardiography and right heart cardiac catherization were consistent with severe pulmonary hypertension and right heart failure. Rheumatological lab panel and T-spot tests were negative. She was aggressively diuresed and also initiated on rivaroxaban; she refused IV prostacyclin therapy. She was discharged home however progressively deteriorated and expired 3 months later.

DISCUSSION: Fibrothorax commonly develops as a complication of prior pleural injury such as empyema, hemothorax, tuberculosis, collagen vascular diseases, uremia and drug reactions. The diagnosis is made from radiographic findings plus a history of a predisposing cause. Our patient presented with extensive pleuroparenchymal disease, likely as a result of incomplete evacuation of a hemo/pneumothorax. Decortiction can been attempted to treat fibrothorax however success depends on the condition of the underlying lung. Severe disease can lead to chronic venous stasis and chronic venous thromboembolism. Pulmonary hypertension and cor pulmonale likely occurred in this patient due to mechanical compression of the pulmonary circulationasand also chronic thromboembolism.

CONCLUSIONS: A thorough understanding of the etiologies and presentations of fibrothorax and early treatment can prevent its development and complications.

Reference #1: Azoulay E. et al. Eur Respir J 1999;14:971-973.

Reference #2: Copley SJ et al. Eur Respir J 1999;14:971-973.

DISCLOSURE: The following authors have nothing to disclose: Mediha Ibrahim, Ajsza Matela, Shilpa Desouza, Joseph Mathew

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