Nikola Grubor: In Situ Pulmonary Arterial Thrombosis Mimicking CTEPH in Eisenmenger
Nikola Grubor, Radiology Resident at University Clinical Centre of Serbia, shared a post on LinkedIn:
“Jovana Tumanov, Jelena Cavić, and I (with special help from Jelica Vukmirović) have another complex case of in situ pulmonary arterial thrombosis due to longstanding Eisenmenger physiology presenting with hemoptysis, which we have submitted to Radiopaedia.
At first sight, the CT appearances mimic chronic thromboembolic pulmonary hypertension (СТЕРН), which also increases pulmonary vascular resistance, causing progressive right heart strain; recognition of the VSD seals the diagnosis.
A key hematological complication in these patients is the development of an acquired von Willebrand syndrome, characterized by the loss of high-molecular-weight von Willebrand factor multimers due to shear stress.
This loss of HMW multimers contributes to the bleeding diathesis, which may include mucosal bleeding and hemoptysis, as in this patient.”
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