Bicuspid Aortic Valve

Clinical History

A 64 year old woman presented with a story of chest pain for 5 months, associated with breathlessness and wheezing for 4 months. On examination she was dyspnoeic, with an expiratory wheeze, left-sided crepitations and signs of a right pleural effusion. Pulse rate and blood pressure were normal. There was a precordial systolic murmur and a heaving apex beat in the 5th left intercostal space 10 cm. from the midline. There was no peripheral oedema. The patient died 4 days after admission.

Pathology

The heart has been opened to display the left ventricle and associated valves. The aortic valve has 2 cusps instead of the usual three. The valves are otherwise normal apart from patchy slight thickening. The aortic origins of the left and right coronary arteries are widely patent, as is the left circumflex coronary artery, seen cut transversely in the atrio-ventricular groove at the right hand edge of the specimen. There is dense pericardial fibrosis and adhesions (see back of specimen). (The cause of this is not apparent from the history). At autopsy there was ascites, a small shrunken cirrhotic liver, bilateral pleural effusions, much larger on the right, and right pulmonary collapse. The cause of death was cirrhosis of the liver. The bicuspid aortic valve was an incidental finding.

Note

Bicuspid aortic valve is a congenital anomaly which predisposes to the development of calcific aortic stenosis, usually in the 5th to 7th decade. Calcific aortic stenosis occurs at a later age when it involves previously normal aortic valves.