Is It a Residual Ventricular Septal Defect?

Abstract

A 1-YEAR-OLD BOY (weight, 7.4 kg; height, 67 cm), with Trisomy 21 and a diagnosis of complete atrioventricular septal defect with tetralogy of Fallot, presented to the author's institution for intracardiac repair. Transthoracic echocardiography reported an ostium primum atrial septal defect, a nonrestrictive inlet-type ventricular septal defect, and anterosuperior deviation of the infundibular septum with a severe right ventricular outflow tract obstruction (peak pressure gradient of 75 mmHg). Intraoperative transesophageal echocardiography confirmed the findings (Fig. 1 and 2; Videos 1-3). After completing the repair and separating from cardiopulmonary bypass, a color Doppler blood-flow map in the mid-esophageal aortic short-axis view demonstrated 2 jets (each about 2 mm in diameter) into the right ventricle in systole, with the lower of the 2 jets also persisting during diastole (Fig 3; Video 4). The color Doppler blood-flow map during deep transgastric right ventricular outflow tract interrogation also demonstrated the jet into the right ventricle throughout the cardiac cycle (Fig 4; Video 5). Continuous-wave Doppler interrogation of the lower jet revealed biphasic flow toward the right ventricle (Fig 5; Video 6). What is the diagnosis? Fig. 2Transgastric long-axis right ventricular outflow tract view with Doppler blood-flow map by transesophageal echocardiography showing the turbulence in the right ventricular outflow tract. View Large Image Figure Viewer Download Hi-res image Fig. 3Postoperative aortic short-axis transesophageal echocardiography views showing (A) 2 jets in systole and (B) 1 lower jet during diastole as correlated with the electrocardiogram. View Large Image Figure Viewer Download Hi-res image Fig. 4Postoperative long-axis right ventricular outflow tract view showing 1 jet in diastole as coordinated with the electrocardiogram. View Large Image Figure Viewer Download Hi-res image Fig. 5Postoperative long-axis right ventricular outflow tract view showing the interrogation of the jet opening into the right ventricle with the continuous wave Doppler signal. View Large Image Figure Viewer Download Hi-res image