Principal Diagnosis for Chest Pain with Polycythemia Vera
The principal diagnosis should be the acute coronary syndrome or thrombotic event causing the chest pain, not the underlying polycythemia vera, because the life-threatening thrombotic complication determines immediate management priorities and mortality risk. 1, 2
Clinical Reasoning for Diagnosis Prioritization
Why the Thrombotic Event Takes Priority
Polycythemia vera creates a hypercoagulable state with widespread activation of coagulation proteins, reduced physiologic anticoagulants, and decreased fibrinolytic activity, making thrombosis the most life-threatening complication that directly impacts mortality. 3, 4
Arterial thrombosis occurs in 16% of PV patients at or before diagnosis, with acute myocardial infarction being a common presentation of chest pain in this population. 2
The chest pain represents the acute, life-threatening manifestation requiring immediate intervention (such as cardiac catheterization, thrombolysis, or anticoagulation), while PV is the underlying chronic condition that predisposed to the event. 1, 5
Diagnostic Approach to Chest Pain in PV Patients
Immediately evaluate for acute coronary syndrome with ECG and cardiac biomarkers, as PV patients can develop sequential multi-vessel occlusion and rapidly progressive thrombosis. 1
Consider atypical chest discomfort as a microvascular disturbance from PV, which represents a non-life-threatening manifestation that can be controlled with low-dose aspirin, but this diagnosis should only be made after excluding acute thrombotic events. 3
Recognize that PV patients may present with continuous thrombus reformation even after initial intervention, requiring aggressive anticoagulation strategies beyond standard dual antiplatelet therapy. 1
Critical Pitfalls in Diagnosis Assignment
Do not list PV as the principal diagnosis when an acute thrombotic complication is present, as this misrepresents the immediate threat to life and may result in inadequate treatment intensity. 1, 2
Be aware that young patients without traditional cardiac risk factors presenting with ACS should prompt immediate investigation for hypercoagulable states including PV, especially if hematocrit is elevated or blood appears viscous. 1, 5
Recognize that erythromelalgia (painful burning sensation of extremities with erythema) can mimic cardiac chest pain but represents platelet-mediated microvascular occlusion rather than coronary thrombosis, and responds promptly to aspirin within hours. 3
Documentation and Coding Implications
The principal diagnosis should reflect the condition that required the most resource utilization and posed the greatest immediate threat: acute myocardial infarction, unstable angina, or pulmonary embolism would be principal, with PV listed as a secondary diagnosis explaining the etiology. 1, 2
If chest pain is determined to be microvascular disturbance without acute thrombosis, then "chest pain due to polycythemia vera" or "microvascular symptoms of polycythemia vera" would be appropriate, but only after life-threatening causes are excluded. 3