Severe aortic complications in a patient with variant Shone's complex and bicuspid aortic valve

Description

A 47-year-old man presented with new onset exertional dyspnoea. He received a surgical repair of coarctation of the aorta (COA) at the age of 21 years and aortic root grafting with mechanical aortic valve replacement for type A aortic dissection and bicuspid aortic valve (BAV) at the age of 28 years. He had been otherwise healthy and did not follow-up with a cardiologist or surgeon after his last surgery. An echocardiogram (Echo) revealed normal left ventricular size and wall thickness, with an ejection fraction of 45%–50%. There was a parachute mitral valve (MV) (figure 1), with only one severely underdeveloped lateral papillary muscle. The mechanical aortic valve was functioning normally. There was a massively dilated distal aortic arch and proximal descending aorta with maximum dimension of up to 65 mm (figure 2). There was no Doppler evidence of recurrent COA. The CT angiogram confirmed the giant aneurysm,...

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