Congenital defect of the left pericardium with sick sinus syndrome

Abstract

years. 5 Aortic stenosis is rare, bu t approximately 50% of patients with quadricuspid aortic valve have aortic regurgitation. 5 Two-dimensional transthoracic echocardiography has become the diagnostic test of choice, because the four cusps and their relative sizes can easily be seen. 4, 5 In addition, Doppler echocardiography is an excellent modality for assessing the degree of aortic regurgitat ion and its progression. 4 However, because of technical factors such as suboptimal echocardiographic windows or extensive aortic calcification, transthoracic echocardiography may occasionally be limited in visualizing the aortic valve. Transesophageal echocardiography overcomes many of the imaging l imitat ions of transthoracic echocardiography and can clearly delineate the aortic valve morphologic characteristics. 6 In our case the exact cause of the severe aortic regurgitat ion was not seen on transthoracic echocardiography. Transesophageal echocardiography was performed to assess the mitral valve, and the quadricuspid aortic valve was discovered incidentally. To the best of our knowledge this is the first case of quadricuspid aortic valve diagnosed exclusively by transesophageal echocardiography in the English language literature. In conclusion, a quadricuspid aortic valve is a rare congenital abnormali ty tha t usual ly can be diagnosed by transthoracic twodimensional echocardiography. A high degree of suspicion is required to make the diagnosis, because this abnormality can easily be overlooked. On occasion the transthoracic echocardiogram cannot show the quadricuspid na tu re of the aortic valve, and transesophageal echocardiography mus t be performed. REFERENCES