Management of Fetus Diagnosed with Common Arterial Trunk and Tetralogy of Fallot with Pulmonary Atresia
Prenatal Management
- Screening for 22q11.2 deletion syndrome should be offered to all pregnant women with fetuses diagnosed with conotruncal abnormalities like TOF and truncus arteriosus, as recommended by the American Heart Association 1, 2
- Genetic consultation should be arranged if 22q11.2 deletion or other genetic syndrome is identified to provide appropriate counseling, according to the American College of Cardiology 3
- Regular fetal echocardiographic monitoring should be performed by specialists with expertise in congenital heart disease to assess progression and plan for delivery, as suggested by the European Society of Cardiology 3, 4
- Fetal echocardiography should be performed at least every 2-4 weeks to monitor cardiac function and development, as recommended by the American Heart Association 3
Delivery Planning
- Delivery should be planned at a tertiary care center with expertise in neonatal cardiac care and immediate access to pediatric cardiac surgery, as recommended by the American College of Cardiology 3
- A multidisciplinary team including maternal-fetal medicine specialists, pediatric cardiologists, cardiac surgeons, and neonatologists should coordinate care, according to the American Heart Association 5
- Cesarean delivery is not routinely indicated based solely on the cardiac diagnosis and should follow standard obstetric indications, as suggested by the American College of Obstetricians and Gynecologists 5
Postnatal Management
- Immediate postnatal assessment should include evaluation of oxygenation status, cardiac function, and confirmation of diagnosis, as recommended by the European Society of Cardiology 6
- For TOF with pulmonary atresia, management is determined by pulmonary artery anatomy, source of pulmonary blood flow, and presence of associated anomalies, according to the European Heart Journal 4
Parental Counseling
- Parents should be counseled about the complexity of the cardiac defect and need for multiple surgeries, potential association with genetic syndromes, long-term outcomes, and quality of life expectations, as recommended by the American Heart Association 2, 4
- Parents should also be informed about the risk of recurrence in future pregnancies, which is 4-6% without 22q11 deletion and 50% with 22q11 deletion, according to the American College of Cardiology 5
- Fetal echocardiography should be offered in future pregnancies, as recommended by the American Heart Association 5