**9. Follow-up and special considerations**

Patients with ccTGA require outpatient follow-up every 1-2 years by a pediatric or adult congenital cardiologist. Symptomatology, ventricular function, and valvar insufficiency should further guide frequency of follow-up. It is recommended an ECG be performed at each visit to monitor for AV block with periodic consideration of Holter monitor. Cardiopulmonary exercise testing is performed to assess overall function as well as response to medical or surgical therapy. RV function in the unrepaired or physiologically repaired ccTGA patient must be closely monitored with serial echocardiography even in asymptomatic patients (Bos et al., 2006). Cardiac MRI with cine data used to quantify RV volume, mass, and ejection fraction is the best modality to serially quantify RV function, and should be performed every 3-5 years.

#### **9.1 Pregnancy**

Pregnancy in the ccTGA patient is generally well tolerated except in the presence of maternal NYHA class III-IV symptoms, moderate or severe AV valve regurgitation, or poor ventricular function (EF<40%). Evaluation of pregnancy outcome in 22 women with ccTGA revealed 50 live births in 60 total pregnancies (83%). However, the rate of miscarriage in the ccTGA mothers was higher than the general population (Connolly et al., 1999). A recent cohort of patients by Gelson and colleagues (2011) revealed high maternal and neonatal morbidity in women with systemic right ventricles with a significant number of babies born small for gestational age. Although cyanosis in women with ccTGA has been shown to be a risk factor for miscarriage, the women in the cohort of Gelson et al. were normally saturated (Gelson et al., 2011; Thierrien et al. 1999). The risk of congenital heart defects in the offspring of mothers with ccTGA has not been defined.

### **9.2 Heart transplant**

Patients for which heart transplantation may be considered are those with end-stage RV failure, significant LV dysfunction and pulmonary valve abnormalities precluding successful DS operation, or uncontrollable arrhythmia (Duncan & Mee, 2005). For patients undergoing surgical intervention, poor preoperative EF of the systemic ventricle has been shown to predict the eventual need for transplantation (Beauchesne et al., 2002).
