View Abstract

A6727 - Massive Hemoptysis in a Patient with Marfan’s Syndrome
Author Block: J. Centeno1, P. Trivedi2, A. Iftikhar3, L. Sanso4; 1New York Presbyterian Queens, Flushing, NY, United States, 2New York Presbyterian Queens, Dix Hills, NY, United States, 3New York Presbyterian Queens, Roslyn, NY, United States, 4New York Presbyterian Queens, New York, NY, United States.
Introduction: Massive hemoptysis represents a life-threatening emergency as blood may flood the tracheobronchial tree resulting in asphyxiation and respiratory arrest. Marfan’s syndrome is known to cause collagen disease but its effect on the pulmonary system, especially intra-pulmonary histologic features, remains largely unknown. Case: 42 year old man with Marfan’s syndrome, aortic valve replacement on Coumadin presented with hemoptysis and chest pain. He was found to be hypoxemic requiring endotracheal intubation with copious bright red blood suctioned from endotracheal tube. CT scan revealed material in the trachea and bilateral mainstem bronchi along with bilateral groundglass opacities. INR was elevated to 6 but other labs were unremarkable. He was treated with vitamin K and FFP transfusion and positioned left side down. He remained hypoxemic and was difficult to ventilate with high airway pressures. Bronchoscopy revealed large amount of clot in the trachea and entire tracheobronchial tree which was cleared. Active bright red pulsatile blood was noted in the left lower lobe. No other airway revealed active hemorrhage. Hemostasis was achieved with cold saline and topical phenylephrine. An attempt was made at left bronchial artery embolization but the bronchial artery was unable to be cannulated. Bleeding recurred and a 5 F fogarty balloon was placed under bronchoscopic guidance for airway occlusion with success. Patient subsequently underwent open thoracotomy with left lower lobectomy for control of bleeding. He has made a full recovery. Discussion: Marfan’s syndrome is an autosomal dominant connective tissue disorder affecting the FBN1 gene on chromosome 15. This encodes Fibrillin-1, the main component of microfibrils, which contribute to mechanical stability, tissue development and homeostasis. Tissues with type I collagen are most affected. Many patients with Marfan’s have underlying pulmonary pathology that may go unrecognized. Histopathology from our patient revealed intraparenchymal and intrabronchial hemorrhage and multifocal medial attenuation of the pulmonary arteries. There were multiple foci with significant thinning of arterial walls associated with complete loss of elastic fibers. Marfan’s syndrome has been associated with an array of pulmonary pathology including widespread or patchy cystic changes, emphysema, spontaneous pneumothorax, focal pneumonia or bronchiectasis, bullae, congenital pulmonary malformations, and apical fibrosis. The specific pulmonary histologic changes that occur in Marfan’s, however, have not been adequately characterized in any microscopic detail. It is important to be able to differentiate Marfan-related pulmonary changes from those attributable to other pathologic processes in order to better approach management of a patient like ours.