Circulation. 2005;111:e367-e368
doi: 10.1161/CIRCULATIONAHA.104.475277
(Circulation. 2005;111:e367-e368.)
© 2005 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Two-Dimensional and Real-Time Three-Dimensional Echocardiographic Fetal Diagnosis of Aorto-Ventricular Tunnel
R. Biffanti, MD;
E. Reffo, MD;
S.P. Sanders, MD;
N. Maschietto, MD;
G. Stellin, MD;
O. Milanesi, MD
From the Pediatric Department (R.B., E.R., N.M., O.M.) and the Pediatric Cardiac Surgery Unit (G.S.), University Hospital of Padua, Italy, and the Medical-Surgical Department of Pediatric Cardiology, "Bambin Gesù Hospital" (S.P.S.), Rome, Italy.
Correspondence to Dr O. Milanesi, Via Guistiniani 3, Padua, Italy. E-mail ornella.milanesi{at}unipd.it
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).
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Figure 1. a, 2D view of the fetal heart shows distortion of the aortic valve by the Ao-LV tunnel. The tunnel courses through the right ventricular outflow tract from the ascending aorta to the left ventricular outflow tract (arrow). b, Color Doppler confirms diastolic runoff from the ascending aorta to the LV at this level (arrow). Ao indicates ascending aorta; LV, left ventricle.
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Figure 2. a, Fetal 3D echocardiographic view of the Ao-LV tunnel (black arrow). b, Surgical view of the Ao-LV tunnel (black arrow). Note the exact correspondence between the fetal 3D imaging and the gross surgical anatomy. RA indicates right atrium; RAA, right atrial appendage; AoV, aortic valve.
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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.
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Figure 3. Parasternal long-axis view of the left ventricle before discharge. Note the hypertrophy of the wall of the LV resulting from acute volume reduction at surgery. In addition, the LV end of the tunnel remains visible in the LV outflow (arrow) because only the aortic end was closed at surgery. LA indicates left atrium; LV, left ventricle; Ao, ascending aorta.
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As far as we are aware, this is the first report in the literature of a fetal diagnosis of an aorto-ventricular tunnel.
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Issue Highlights
Circulation 2005 111: 2711.
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