Pregnancy is considered to be high-risk for individuals with cyanotic heart diseases such as unrepaired Total Anomalous Pulmonary Venous Return or with pulmonary vein stenosis after repair and is not recommended for individuals with severe forms. There may be a danger of the formation of blood clots, causing strokes in extreme cases, as a woman's blood coagulates more easily during pregnancy. Pulmonary vein stenosis can result in severe pulmonary hypertension. This may lead to further complications that pose risks to both mother and child.

If the patient chooses to proceed with pregnancy, careful monitoring by health professionals is essential. Minimal activity and bed rest are usually recommended, and treatment with anticoagulants and oxygen therapy may be prescribed. Cesarean section may be selected as the safest form of delivery.

This defect can be inherited as a dominant trait in many families with, theoretically, 50% of a patient's offspring having the anomaly, though variation in its expression reduces the incidence to somewhat less than this.

Anyone with congenital heart disease, repaired or non-repaired, should consult with their cardiologist prior to becoming pregnant to review the risks.