Body growth is severely impaired in Fontan patients. A close interstage follow-up between the first surgery and the BCPA must be targeted at optimizing nutritional support to counter the important growth retardation occurring before the BCPA. The better catch-up growth at the TCPC when the BCPA is performed earlier in life supports the current trend to perform the BCPA at a younger age. Heart failure treatment after a Fontan completion is independently associated with decreased late somatic development.

In a 15-year's experience with transatrial-transpulmonary correction of tetralogy of Fallot, a valve- and infundibulum-sparing approach has been advanced by lowering the age for elective repair. This change has been performed without compromising immediate clinical outcome, despite an increased early re-operation rate for residual obstruction. However, longer follow-up will disclose whether this approach is protective against progressive and late RV dysfunction.

No abstract

Patients < or =15 kg requiring ASD closure form a special population with high incidence of associated anomalies and medical conditions. For this particular set of patients, percutaneous closure is a valid alternative. Asymptomatic or mildly symptomatic children should be treated at a later age.

This study shows that the outcome of palliation with the MBTS is importantly affected by the occurrence of a shunt-related complication, whose circulatory effect is even more dismal in single ventricle hearts. Since an increased shunt size-to-weight ratio additionally compromises the shunt-dependent survival, it emphasizes that the choice of the shunt with regard to size as well as surgical approach remains critical.

Congenital heart disease is the most frequent form of congenital anomaly in newborn infants and accounts for more than a quarter of all serious congenital afflictions worldwide. A genetic etiology is identified in <20 % of cases of congenital heart defects, and in most cases the etiology remains a mystery. In the context of the health burden caused by congenital heart disease, the contribution of non-inherited risk factors is important especially if it turns out to be caused by a drug which can be avoided during pregnancy. We sought to determine whether maternal dydrogesterone treatment in early pregnancy is associated with congenital heart disease in the infant. We conducted a retrospective case-control study of birth defects and associated risk factors. Data were obtained and compared between 202 children born with congenital heart disease and a control group consisting of 200 children. All children were born in the period of 2010-2013. Dydrogesterone exposure was defined as any reported use during the first trimester of pregnancy. Exclusion criteria included stillbirths, children with chromosomal abnormalities and infants of mothers with chronic medical illnesses, e.g., diabetes. Binary logistic regression analyses were used to analyze the data and attempt to identify a causal relationship between drug exposure and congenital heart disease. Mothers of children born with congenital heart disease received more dydrogesterone during first trimester of pregnancy than mothers of children in the control group [adjusted odds ratio 2.71; (95 % CI 1.54-4.24); P = 0.001]. We identified a positive association between dydrogesterone usage during early pregnancy and congenital heart disease in the offspring. Nevertheless, further studies are needed to confirm these results.

The initially dilated aortic root in tetralogy of Fallot normalises in size at the level of the annulus and sinotubular junction within 7 years after early repair. This process seems delayed at the level of the aortic sinuses, although the indexed root diameter shows significant regression over time. These results suggest that early repair of ToF abrogates the enlargement of the aortic root, validating one aspect of the need for tetralogy correction at a young age.

In prepubertal TS girls, stiffness of the ascending aorta is increased in patients with a BAV and TAV while dilation of the ascending aorta is more frequent in BAV. This suggests an intrinsic aortic wall abnormality making all TS patients at increased risk for severe aortic complications although the risk is the highest for TS with BAV.