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Eisenmenger Syndrome - Haemoptysis — EECC MCQ

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HardCongenital Heart Disease (Adult)Eisenmenger Syndrome - HaemoptysisEECC

A 22-year-old man with a large perimembranous ventricular septal defect and Eisenmenger syndrome (resting SpO₂ 82%, haematocrit 0.68) presents with haemoptysis. His haemoglobin is 220 g/L. What is the most important immediate investigation?

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Correct answer: D — CT pulmonary angiography to exclude pulmonary artery thrombus or ruptured bronchial collateral

Haemoptysis in Eisenmenger syndrome can be caused by: (1) pulmonary artery thrombosis (in situ, common due to sluggish flow and erythrocytosis), (2) rupture of bronchial collateral vessels, (3) pulmonary infarction, or (4) coagulopathy from secondary erythrocytosis. CT pulmonary angiography is the first-line investigation to identify the cause. Therapeutic phlebotomy should only be performed for hyperviscosity symptoms (not based on haematocrit alone) and can worsen iron deficiency, which paradoxically increases stroke risk. VSD closure is absolutely contraindicated in established Eisenmenger syndrome as the shunt serves as a pressure relief valve for the RV. Anticoagulation decision is complex and not first-line for acute haemoptysis.

Reference: ESC (2020): Guidelines for the Management of Adult Congenital Heart Disease; ESC (2022): PH Guidelines