Acardia

Synonyms

1

Overview

Acardia is a very rare, serious malformation that occurs almost exclusively in monozygous twins (twins developing from a single egg). This condition results from artery to artery connections in the placenta causing a physically normal fetus to circulate blood for both itself and a severely malformed fetus whose heart regresses or is overtaken by the pump twin's heart.

Symptoms

• No heart

Causes

Acardia is the most extreme form of twin-twin transfusion syndrome. Twin-twin transfusion syndrome is a pregnancy complication in which twins abnormally share blood flow from the umbilical artery of one twin to the umbilical vein of the other. This abnormal connection can cause serious complications including loss of the pregnancy. In acardiac twin pregnancies, blood vessels abnormally connect between the twins in the placenta. The placenta is the important interface of blood vessels between a mother and baby through which babies receive nutrients and oxygen. This abnormal connection forces the twin with stronger blood flow to pump blood for both, straining the heart of this "pump" twin. This abnormal connection causes the malformed twin to receive blood directly from the pump twin before this blood gathers new oxygen. The poorly deoxygenated blood from the normal twin as well as the pressure deficiency as a result of trying to serve both infants may be the cause of the other twin's malformations.

Diagnosis

The diagnosis of acardiac twins or TRAP sequence is suggested by the presence of a monochorionic (single placenta) twin pregnancy in which one twin (the pump twin) appears structurally normal (no ultrasound findings consistent with birth defects), while the other twin (the acardiac/TRAP twin) has multiple profound birth defects (as listed in the background section above) which are not compatible with life.

The diagnosis is confirmed with the use of combined pulsed and color Doppler ultrasound studies. This method allows for the documentation of the arterial blood flow perfusing the acardiac/TRAP twin in a retrograde fashion, thus securing the diagnosis.

Once the diagnosis is established, further ultrasound studies must be performed to assess whether that individual pregnancy is in the high-risk category for pregnancy loss.

Treatment

• Surgery

Resources

• NIH