C  2009, the Authors Journal compilation C  2009, Wiley Periodicals, Inc. DOI: 10.1111/j.1540-8175.2009.00948.x Transesophageal Real Time Three-Dimensional Echocardiography in Assessment of Partial Atrioventricular Septal Defect Radoslaw Piatkowski, M.D., ∗ Anna Budaj-Fidecka, M.D., ∗ Piotr Scislo, M.D., Ph.D., ∗ Janusz Kochanowski, M.D., Ph.D., ∗ Mateusz ´ Spiewak, M.D.,† Grzegorz Opolski, M.D., Ph.D. ∗ ∗ Department of Cardiology, Medical University of Warsaw, Poland; and †First Department of Coronary Artery Disease, Cardiac Magnetic Resonance Unit, Institute of Cardiology, Warsaw, Poland We present a practical application of real time three-dimensional transesophageal echocardiography in a 67-year-old male patient with congenital heart disease. (ECHOCARDIOGRAPHY, Volume 26, October 2009) congenital heart defects, atrial septal defects, ASD, 3D TEE, mitral cleft Case Report A 67-year-old man presented with a 6-month history of declining exercise capacity (NYHA II). Clinical examination revealed HR approxi- mately 80/min., BP 130/70 mmHg, pansystolic murmur at the apex and at the left sternal bor- der, and features of pulmonary stasis in the pul- monary circulation. The initial electrocardio- gram showed atrial fibrillation, leftward QRS axis, left anterior hemiblock, and “incomplete” right bundle-branch block. Chest x-ray revealed cardiomegaly due to right heart and left atrial dilation, and evidence of increased pulmonary vascularity. The patient had a normal coronary angiography. Transthoracic two-dimensional echocardiography (2D TTE) showed the right and left ventricles as well as left atrium en- largement. TTE also revealed a defect in the most inferior portion of the atrial septum (os- tium primum defect—ASD 1) with a significant left to right shunt (Qp/Qs = 3.9:1). (Fig. 1). Both atrioventricular (AV) valves were local- ized at the same level. In three-dimensional (3D) TTE there was a cleft of the anterior mi- tral valve (Fig. 2). Transesophageal 2D echocar- diography (2D TEE) excluded LVOT obstruc- tion and showed a large ventricular septal Address for correspondence and reprint requests: Radoslaw Piatkowski, M.D., First Department of Cardiology, Medical University of Warsaw, Poland, Central University Hospital, 1a Banacha Street, 02-097 Warsaw, Poland. Fax: +48 22 599 19 57; E-mail: radekp1@wp.pl aneurysm (Fig. 3). Color Doppler demonstrated significant eccentric mitral and tricuspid regur- gitations with mild pulmonary hypertension. Three-dimensional real time transesophageal echocardiography (3D TEE) revealed an atrial septal defect with the largest diameter of 30 × 12 mm, as well as a cleft mitral valve (Fig. 4 and Video clip 1; Fig. 5 and Video clip 2). Color Doppler 3D TEE demonstrated two large eccen- tric jets of mitral regurgitation (Fig. 6) and two areas without correct leaflet coaptation. After the 3D TEE, the patient was referred for sur- gical correction (repair of the cleft mitral valve, closure of the interatrial communication using a pericardium patch, and repair of the inter- ventricular septum aneurysm with a synthetic patch). Discussion The ostium primum defect is within the spec- trum of AV septal defects. 1 In a patient with ASD 1, there is a common AV junction with two separate valve orifices, formed by fusion of the superior and inferior bridging leaflets. 2 Significant mitral regurgitation due to the cleft mitral valve is observed in many patients. As- sociated defects include disproportion of out- let and inlet dimensions of the left ventri- cle, left ventricular outflow tract elongation, and malformations of the AV septum (muscu- lar and membranous). 3 Symptomatic patients with ASD 1 require a closure of the intera- trial defect and repair of the left AV valve. 1–4 1092 ECHOCARDIOGRAPHY: A Jrnl. of CV Ultrasound & Allied Tech. Vol. 26, No. 9, 2009