Two-Dimensional and Real-Time Three-Dimensional Echocardiographic Fetal Diagnosis of Aorto-Ventricular Tunnel
A 22-gestational-week-old female fetus was referred to our prenatal echocardiographic unit to evaluate her left ventricular dilatation and dysfunction. An aorto-ventricular tunnel was visualized (Figure 1a and 1b), causing marked left ventricular diastolic overload, overt fetal heart failure, hydrops, and polidramnios. The mother was informed about the possibility of spontaneous abortion or premature delivery, and the pregnancy was closely monitored with serial fetal echographic and echocardiographic evaluations. At 33 weeks of gestational age (GA), the signs of fetal heart decompensation were still present, but the fetus’ vitality and growth were satisfactory. A real-time 3D (RT3-D) echocardiographic evaluation was performed, which allowed the precise visualization of the site and dimension of the aorto-ventricular tunnel (Figure 2a).
At 35 weeks of GA, a programmed cesarean section was performed, and the mother gave birth to a 2.2-kg vital girl. APGAR score was 4/1 7/5 8/10. The neonate was intubated and ventilated in the operating room and transferred to the neonatal intensive care unit. At 2 days of age, surgical intervention was performed. At the surgical inspection, the aorto-ventricular tunnel location and dimension were superimposed on the RT3D visualization (Figure 2b). The postoperative course was characterized by low cardiac output syndrome and difficulty in achieving respiratory autonomy. The neonate was extubated on the 7th postoperative week and discharged home on the 9th postoperative week in good general condition and weighing 2.5 kg. The predischarge echocardiographic evaluation (Figure 3) showed marked hypertrophy of the left ventricle, good ventricular function, and dilation of the ascending aorta and aortic arch.
As far as we are aware, this is the first report in the literature of a fetal diagnosis of an aorto-ventricular tunnel.