Eisenmenger Syndrome Complicated by Hemorrhagic Hereditary Telangiectasia: Unique Intersection of Hemorrhage, Cyanosis, and Thrombosis
Abstract
Background: Eisenmenger syndrome is an intracardiac, right-to-left shunt reversal due to a congenital defect. It can rarely co-occur alongside other noncardiac congenital abnormalities.
Case summary: A patient with known Eisenmenger syndrome secondary to a large unrepaired ventricular septal defect with concurrent genetically confirmed hereditary hemorrhagic telangiectasia (HHT) presented with acute gastrointestinal bleed. Multiple subspecialists managed the patient's unique medical challenges of ongoing hemorrhage, cyanosis, and risk for thrombosis. Diagnostic work-up included imaging to visualize arteriovenous malformations and right heart catheterization. Medical management included administration of bevacizumab, macitentan, sildenafil, midodrine, pantoprazole, and iron.
Discussion: This case highlights the unique hematologic pathophysiology and hemodynamic challenges of both Eisenmenger syndrome and HHT.
Take-home messages: Patients with Eisenmenger syndrome and HHT are at risk for both cyanosis and bleeding-albeit by different pathophysiological mechanisms. These factors lead to complex, overlapping scenarios that can worsen both conditions in a patient dually afflicted.
How strong is the evidence?
Evidence Hub reads this as a case report, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
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