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

BAV has been increasingly reported in patients with Shone's complex. Since the first case series published in 1963, subsequent multiple case series reported BAV as an associated feature of Shone's complex. 4 Our case emphasises that the association of BAV with Shone's complex should not be neglected since BAV is a disease of both the valve and the aorta. The associated aortopathy may cause severe life-threatening complications as shown in our case. [...]for patients with variant Shone's complex and BAV, cardiac imaging of the thoracic aorta should be performed periodically for surveillance. Learning points Shone's anomaly is a very rare congenital cardiac malformation characterised by four serial obstructive lesions of the left side of the heart, namely (1) supravalvular mitral membrane, (2) parachute mitral valve, (3) muscular or membranous subaortic stenosis and (4) coarctation of the aorta.