UNREPAIRED EBSTEIN ANOMALY IN ADULT WOMAN: CHALLENGES AND STRATEGIES IN LONG-TERM CONSERVATIVE MANAGEMENT – A CASE REPORT

Abstract

Ebstein anomaly is a rare congenital malformation of the tricuspid valve and right ventricle with a highly variable clinical course, and some patients survive into adulthood without surgical repair. We report a 42-year-old woman from a rural area in whom a congenital heart condition was suspected in childhood but not confirmed until her thirties. She presented with progressive exertional dyspnoea and easy fatigability. Electrocardiography showed sinus ryhtym with delta wave and short PR interval, and she later had an episode of supraventricular tachycardia that converted spontaneously. Chest radiography revealed marked cardiomegaly (cardiothoracic ratio 73%). Serial transthoracic echocardiography from 2023 to 2026 demonstrated apical displacement of the septal tricuspid leaflet by 35 mm (≈23 mm/m²) with atrialisation of the right ventricle, severe tricuspid regurgitation, right heart dilatation, and a large secundum atrial septal defect with left-to-right shunting, with preserved biventricular systolic function. Despite treatment with diuretics, a mineralocorticoid receptor antagonist, a beta-blocker, an angiotensin receptor blocker, and sildenafil, she was admitted twice for heart failure. Surgical repair, electrophysiological study, and right heart catheterisation were indicated but unavailable at our hospital, and the patient declined referral to a tertiary centre. This case shows that unrepaired Ebstein anomaly may be tolerated for decades despite severe anatomy. When definitive treatment is not accessible, long-term conservative management should focus on heart failure control, safe management of arrhythmias in the presence of pre-excitation, and structured clinical and echocardiographic surveillance with clear triggers for renewed referral.