In utero diagnosis of obstructed supracardiac total anomalous pulmonary venous connection in a patient with right atrial isomerism and asplenia

Abstract

We describe a rare case of right atrial isomerism, levocardia, right-side stomach, obstructed supracardiac total anomalous pulmonary venous connection, double outlet right ventricle with complete atrioventricular septal defect and absent spleen. From the pulmonary venous confluence behind the atrium an ascending as well as a descending vertical vein communicated with the systemic venous system in the supracardiac as well as the infracardiac position. The pulsed and color Doppler examination of the individual pulmonary veins as well as of the vertical vein helped in making the diagnosis of obstructed total anomalous pulmonary venous connection. The diagnosis was made by fetal echocardiographic examination at 22 weeks of gestation and confirmed on postnatal echocardiography, cardiac catheterization, and at surgery.

Changes in pulmonary venous Doppler parameters in fetal cardiac defects

“…Also of interest are the pulmonary venous findings in the two fetuses with infradiaphragmatic total anomalous pulmonary venous return (TAPVD) (Group 5) and obstruction showing no pulsatile flow pattern (Figure 3), confirming other observations41–43. Due to the absence of a direct connection between the left atrium and the pulmonary veins, pulsatile flow was absent.…”

Retour veineux pulmonaire total isolé : y a-t-il des clés pour le dépistage prénatal ?

“…En ré gion Haute Normandie, le taux de diagnostic anté natal des cardiopathies congé nitales significatives est supé rieur à 83 % depuis 2009, avec un taux de dé pistage des transpositions des gros vaisseaux supé rieur à 80 % et des anomalies de la crosse aortique d'environ 65 %. Dans notre expé rience, comme dans les observations de la litté rature, les anomalies des veines pulmonaires n'é taient dé pisté e, jusqu'en 2009, qu'à l'occasion de l'expertise d'un cardiopathie complexe, en particulier dans les hé té rotaxies [1,2]. Elles sont donc visibles sur une é chographie orienté e mais é chappent à l'examen de dé pistage dans une population à bas risque.…”

Prenatal Diagnosis of Partial Anomalous Pulmonary Venous Connection by Detection of Dilatation of Superior Vena cava in Hypoplastic Left Heart

“…In the current literature little data exists about fetal anomalous pulmonary vein connections and almost none about fetal partial anomalous venous connections [1][2][3][4][5], first because they are rare and secondly they are very difficult to detect prenatally.…”

“…The other authors presented only single case reports of TAPVC in the fetus [2][3][4][5]. Partial anomalous pulmonary vein connections (PAPVC) involving one or more of the pulmonary veins can be extremely difficult to identify prenatally.…”