Aortic Valve Replacement in Severe Factor V Deficiency and Inhibitor: Diagnostic and Management Challenges

Alexa I Bello,Eric Salazar,Kirk Heyne,Joseph Varon

doi:10.7759/cureus.5918

Abstract

Factor V (FV) deficiency (F5D) is a rare hematological disorder with a variable spectrum of bleeding manifestations. Until now, no specific protocols for the management of these patients have been established. However, available literature suggests that perioperative infusion of fresh frozen plasma (FFP) may help maintain FV levels to prevent bleeding. We present the case of a 64-year-old man with previously undiagnosed severe FV deficiency and mild FV inhibitor, who underwent aortic valve replacement with no bleeding complications.

Highlights

Factor V (FV) deficiency (F5D), known as Owren’s disease or parahemophilia, is a rare autosomal recessive, hematological disorder, with an incidence of one case per one million people [1,2,3,4]
We report a case of F5D, with superimposed mild FV inhibitor, who underwent aortic valve replacement (AVR) with minimal fresh frozen plasma (FFP) infusions and no abnormal bleeding events
A 64-year-old South Asian gentleman, born out of a non-consanguineous marriage, with a history of aortic stenosis (AS) without coronary artery disease (CAD), presented with dyspnea on exertion to his primary care clinician. He was evaluated for potential AVR by minimally invasive cardiac surgery (MICS)

Summary

Introduction

Factor V (FV) deficiency (F5D), known as Owren’s disease or parahemophilia, is a rare autosomal recessive, hematological disorder, with an incidence of one case per one million people [1,2,3,4]. F5D patients may present with clinical manifestations ranging from mucosal bleeding to life-threatening hemorrhagic episodes [1]. Laboratory screening on these patients typically reveals prolonged prothrombin time (PT), activated partial thromboplastin time (aPTT), and a normal thrombin time (TT) [1]. We report a case of F5D, with superimposed mild FV inhibitor, who underwent aortic valve replacement (AVR) with minimal FFP infusions and no abnormal bleeding events. A 64-year-old South Asian gentleman, born out of a non-consanguineous marriage, with a history of aortic stenosis (AS) without coronary artery disease (CAD), presented with dyspnea on exertion to his primary care clinician He was evaluated for potential AVR by minimally invasive cardiac surgery (MICS). The patient was discharged on day seven, and on six months follow-up, he had no evidence of bleeding

Discussion

Conclusions

Disclosures