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Atrial Septal Defect (ASD) is one of the most frequently congenital heart diseases in adults and it is often asymptomatic until adulthood. We report a case of a 90-year-old woman admitted to hospital with dyspnea and orthopnea insidiously progressing over the preceding 5 years and becoming severe with dyspnea on minimal activities, orthopnea and paroxysmal nocturnal dyspnea, in the last 2 weeks. The transthoracic echocardiogram revealed an atrial septal defect ostium secundum type, with left-to-right shunt, moderate to severe tricuspid insufficiency, severe pulmonary hypertension (72 mmHg) and preserved biventricular function. With diuretic therapy optimization the patient showed symptomatic improvement. This present case represents and unusual and very late presentation of an atrial septal defect ostium secundum type, which is usually diagnosed at the mild adult age. Our patient lived symptom-free for over 80 years.