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A7047 - Unexpected Cause of Acute Severe Headache in a Patient on Chronic Rivaroxaban for Chronic Thromboembolic Pulmonary Hypertension
Author Block: M. M. Khan1, J. M. Elwing2; 1Pulmonary and Critical Care, University of Cincinnati, Cincinnati, OH, United States, 2Pulmonary and Critical care, University of Cincinnati, Cincinnati, OH, United States.
Ventriculo-atrial (VA) shunts are an independent risk factor for developing chronic thromboembolic pulmonary hypertension (CTEPH). Anticoagulation remains a mainstay for medical management of CTEPH. We present a case of acute VA shunt malfunction due a thrombus at the distal end with brief interruption of systemic anticoagulation for a planned procedure.
A 46 year old caucasian female with a past medical history significant for myelomeningocele with a ventriculo-atrial shunt placed in infancy, chronic osteomyelitis of the right foot, severe OSA and CTEPH diagnosed about a year ago, on Riociquat and Rivaroxaban, was scheduled to get a routine repeat right heart catheterization with plans to hold Rivaroxaban for 3 days pre-procedure. She presented to the hospital with severe headaches on the second day of anticoagulation interruption. Work up revealed +1 optic nerve swelling bilaterally and ventricular enlargement on CT head, concerning for shunt malfunction. No Intracranial hemorrhage or sinus venous thrombosis seen but there was chronic occlusion of her right jugular vein near the site of catheter entry and an acute appearing density adjacent the inferior aspect of the shunt catheter likely representing a thrombus. No imaging evidence of shunt disruption or kinking seen, but her shunt terminated in the superior vena cava (SVC). There was no intra-cardiac thrombus on ECHO. She remained on systemic anticoagulation. Her symptoms were medically managed initially but sub-optimally with concerns for removing a very old shunt and distal thrombus. She developed pseudomonal bacteremia and subsequently underwent staged VA shunt externalization with conversion to a ventriculo-peritoneal shunt. Her headaches improved significantly and she continues to do well from a clinical standpoint. Given her on going osteomyelitis she is not a candidate for surgical options for her CTEPH at the current time and continues on medical therapy.
VA shunts are known for thrombotic complications including vascular, cardiac and pulmonary thrombi, which can lead to clinically significant pulmonary HTN in a small number of patients (0.3% to 8%) with a median time of 16.5 years from shunt placement. Various hypothesis have been put forth for the thromboembolic events including endothelial surface damage, foreign body and development of fibrinous coating, possible cerebral thromboplastin activating coagulation cascade or shunt infection, with an increased risk with catheters in SVC as compared to atrium. Our case is unique due to acute shunt malfunction caused by a distal thrombus formation with brief anticoagulation disruption in a patient with CTEPH likely secondary to VA shunt.