Prenatal Diagnosis of Total Anomalous Pulmonary Venous Connection with Asplenia

Abstract

The prenatal diagnosis of 2 fetuses with total anomalous pulmonary venous connection was made by color Doppler echocardiography at 31 and 38 weeks of gestation, respectively. Both had asplenic hearts and their diagnoses were confirmed by postnatal echocardiography, magnetic resonance imaging, and angiography. The antenatal diagnosis of total anomalous pulmonary venous connection in right isomerism could provide an optimal postnatal medical and surgical management. Moreover, monitoring of the velocity profile of the pulmonary venous flow is beneficial for decreasing the risk of sudden collapse by pulmonary venous obstruction throughout the perinatal period.

Prenatal diagnosis of total anomalous pulmonary venous connection to the portal vein associated with right atrial isomerism

“…However, the positive identification of a normal pulmonary venous connection to the left atrium is difficult. The diagnosis has subsequently been based on pulsed Doppler examination of pulmonary vein blood flow6, 7. In TAPVC, there is a low‐velocity, mildly pulsatile flow pattern in the pulmonary veins.…”

Hearts with isomerism of the right atrial appendages – one of the worst forms of disease in 2005

“…In the experience reported by Sharland and Cook, 82 slightly more than half of families chose to terminate their pregnancy once the diagnosis was made for isomerism. Surveys of the literature also reveal termination of a significant number of pregnancies following the diagnosis of complex forms of atrioventricular septal defect, 87 right-sided heart, 88 or totally anomalous pulmonary venous connections, 82,[89][90][91] all conditions known to be associated with isomerism. In a contemporary review of 178 cases diagnosed with heterotaxy syndromes at the Toronto Hospital for Sick Children between 1990 and 2003, 6 right isomerism was found in 75 cases (42%), with a prenatal diagnosis having been made in 33 of these.…”

A Simple Rule for Prenatal Diagnosis of Total Anomalous Pulmonary Venous Return

“…Based on previous reports, several clues for prenatal diagnosis of TAPVR have been proposed, including the confluence of the pulmonary veins, the presence of vertical veins, a prominent superior vena cava (SVC), and a post-LA space index. Although several case reports and case series have been published, 2,[4][5][6][7][8][9][10][11][12][13] the number of cases based on such series is still too small to draw a valid conclusion; a larger number of cases are needed to increase the sample size in the literature to gain power for future analyses. Unlike most previous retrospective series, we conducted this prospective study to devise a simple rule for fetal diagnosis of TAPVR in consecutive cases of prenatal diagnosis of TAPVR with postnatal or postabortal confirmation with the intention of enabling general sonographers to diagnose the condition, thus increasing the number of accurate prenatal diagnoses.…”