Prenatal diagnosis of total anomalous pulmonary venous connection: 2D and 3D echocardiographic findings

Abstract

Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart disease that accounts for less than 1% of all congenital heart diseases. TAPVC is characterized by the absence of pulmonary venous drainage into the left atrium. It is underdiagnosed in utero, with prenatal detection rates of less than 1.4%. The prenatal diagnosis of TAPVC affects the postnatal outcome, particularly in obstructive forms (critical TAPVC), since planned delivery and perinatal management are mandatory. Thus, identifying the ultrasound key markers of TAPVC is important for the prenatal diagnosis and perinatal assistance. The ventricular size discrepancy (right ventricle > left ventricle) can be a useful marker. Furthermore, the increased retroatrial distance between the left atrium and the aorta could be a diagnostic marker for prenatal TAPVC, especially when the left atrium is small. Three‐ (3D) and four‐dimensional ultrasonography may improve the prenatal diagnosis of TAPVC. This study reviews the two (2D) and 3D ultrasonographic markers used in the antenatal diagnosis of TAPVC, with a focus on the tools that can be used by sonographers, obstetricians, and fetal medicine specialists to improve the prenatal diagnosis of TAPVC, and when to refer the case to a fetal cardiac specialist.

The heart in congenital diaphragmatic hernia: Knowns, unknowns, and future priorities

“…This in turn leads to reduced pulmonary venous return, LV filling and growth. In support of this flow-based mechanism reduced LV dimensions are similarly observed in fetuses with anomalous pulmonary venous drainage (23). This mechanism would in theory have greatest impact later in gestation when, in the normal fetus, increases in fetal pulmonary blood flow and LV size are usually observed.…”

Successful surgical repair in an older adult with supracardiac total anomalous pulmonary venous connection: A case report

“…TAPVC is a critical CHD accounting for <1% of all CHDs ( 7 ). TAPVC is characterized by the absence of direct connection between the pulmonary venous and the LA.…”

Mixed type total abnormal pulmonary venous connection: Prenatal diagnosis features in a case

“…The main supportive findings in the diagnosis of TAPVC are right ventricular dominance, ventricular asymmetry, small posterior wall of LA, which is smooth, rounded, and hyper‐echogenic, absence of normal pulmonary venous flow, presence of a confluent vein, and prolongation of the distance between the left atrium and the descending aorta (Picture 2). The presence of a confluent vein (twig sign) posterior to the left atrium in TAPVC cases has been presented as one of the most important findings 1,2 . It has been shown that 80%–90% of pulmonary venous return anomalies occur in atrial septal defects of the superior vena cava sinus type 3 …”

“…The presence of a confluent vein (twig sign) posterior to the left atrium in TAPVC cases has been presented as one of the most important findings. 1,2 It has been shown that 80%-90% of pulmonary venous return anomalies occur in atrial septal defects of the superior vena cava sinus type. 3 In our case, all findings suggestive of TAPVC were found in the four-chamber section.…”