Key result
Conservative medical therapy controls symptoms in severe unrepaired Ebstein anomaly despite recurrent heart failure admissions.
Why the study?
Ebstein anomaly is a rare congenital malformation with a highly variable clinical course, and patients surviving into adulthood without surgical repair present challenges in long-term conservative management when definitive treatment is unavailable.
Case Report (n=1)
No
This case illustrates that while severe unrepaired Ebstein anomaly with a large atrial septal defect may be tolerated into adulthood, medical management alone is insufficient to prevent recurrent heart failure decompensations once symptoms and arrhythmias develop, necessitating structured surveillance when surgical referral is declined or unavailable.
May support symptom control with medical therapy in select unrepaired Ebstein adults; leaves open optimal surgical timing.
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.
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Dewi et al. (2026) conducted a case report in Unrepaired Ebstein anomaly with large secundum atrial septal defect, Wolff-Parkinson-White pre-excitation, and severe tricuspid regurgitation (n=1). Conservative medical therapy (furosemide, spironolactone, bisoprolol, candesartan, and sildenafil) was evaluated on Heart failure hospitalizations and clinical progression. Conservative medical therapy controlled symptoms in a 42-year-old woman with severe unrepaired Ebstein anomaly (35 mm leaflet displacement, LVEF 76%), though two heart failure admissions occurred.
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