The pulmonary valve and the pulmonary artery

A 65-year-old female was admitted to our hospital complaining of a progressive dyspnoea. On physical examination, there was a mild diastolic murmur over the pulmonary area and the electrocardiogram was normal. Two-dimensional echocardiography showed an aneurysmatic dilation of the pulmonary artery trunk (diameters of 59 mm × 55 mm) and of its branches (35–38 mm each one), and a pulmonary anomalous valve with a moderate incompetence. Cardiac magnetic resonance confirmed that the pulmonary valve had four cups of similar size (asterisk) with a non-significant pulmonary insufficiency, and a great aneurysm of the pulmonary artery (arrow). Using a cardiac catheterism, a significant pulmonary hypertension was ruled out, and there was not a concomitant major pathology, therefore an idiopathic origin of the aneurysm was considered. The quadricuspid pulmonary valve is a rare congenital anomaly which is almost always discovered at post-mortem studies (from 1 in 400 to 2000 autopsies). Usually, it is an isolated anomaly, remains asymptomatic, and its echocardiography diagnosis is very difficult; for these reasons, its diagnosis is exceptional in live people.