The echocardiographic diagnosis of totally anomalous pulmonary venous connection in the fetus.
BACKGROUND
Infants with isolated totally anomalous pulmonary venous return often present severely decompensated, such that they are at high risk for surgical repair. On the other hand, if surgical repair can be safely accomplished, the outlook is usually good. Thus prenatal diagnosis would be expected to improve the prognosis for the affected child.


OBJECTIVE
To describe the features of isolated totally anomalous pulmonary venous drainage in the fetus.


METHODS
Four fetuses with isolated totally anomalous pulmonary venous connection were identified and the echocardiographic images reviewed. Measurements of the atrial and ventricular chambers and both great arteries were made and compared with normal values.


METHODS
Referral centre for fetal echocardiography.


RESULTS
There were two cases of drainage to the coronary sinus, one to the right superior vena cava, and one to the inferior vena cava. Right heart dilatation relative to left heart structures was a feature of two cases early on, and became evident in some ratios late in pregnancy in the remaining two.


CONCLUSIONS
Ventricular and great arterial disproportion in the fetus can indicate a diagnosis of totally anomalous pulmonary venous connection above the diaphragm. However, in the presence of an atrial septal defect or with infradiaphragmatic drainage, right heart dilatation may not occur until late in pregnancy. The diagnosis of totally anomalous pulmonary venous drainage in fetal life can only be reliably excluded by direct examination of pulmonary venous blood flow entering the left atrium on colour or pulsed flow mapping.
Allan
L D
LD
Department of Pediatric Cardiology, Division of Pediatrics, New York Presbyterian Hospital, 3959 Broadway, New York, NY 10032, USA. la48@columbia.edu
Sharland
G K
GK
D018618:Echocardiography, Doppler, Color; D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging; D005318:Fetal Heart / Q000000981:diagnostic imaging*; D005865:Gestational Age; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging*; D006801:Humans; D011247:Pregnancy; D011667:Pulmonary Veins / Q000002:abnormalities*; D016216:Ultrasonography, Prenatal