Obesity is common in children with congenital and acquired heart disease. Pediatric cardiologists demonstrate inadequate communication regarding this problem to referring practitioners. Healthy-lifestyle counseling and routine exercise in children with heart disease may be underemphasized.

Objective To evaluate the extent and determinants of missed prenatal detection of congenital heart disease (CHD) in a population-based setting. (OR, 4.3 (95% CI,), presence of extracardiac defects (OR, 2.7 (95% CI,) and ultrasound location i.e. high risk clinic vs clinic (OR, 2.1 (95% CI, Methods )). Defects that would be expected to have an abnormal outflow-tract view were missed more often (64%) than were those that would be expected to have an abnormal four-chamber view (42%). ConclusionThe majority of CHD cases over the 10-year study period were missed prenatally and detection rates did not increase materially during that time. The failure to detect CHD prenatally was related to encounter characteristics, specifically involving screening ultrasound examinations, which may be targeted for improvement.

There is a low incidence of aortic complications during pregnancy in women with Marfan syndrome and an aortic diameter <4.5 cm. However, pregnancy does increase the risk of aortic complications in the long-term in this group of patients.

Objectives We sought to investigate the impact of 22q11.2 deletion on perioperative outcome in tetralogy of Fallot. Methods We conducted a retrospective review of patients with tetralogy of Fallot who underwent complete surgical reconstruction at The Children’s Hospital of Philadelphia between 1995 and 2006. Inclusion criteria included diagnosis of tetralogy of Fallot and known genotype. Fisher’s exact and Mann Whitney tests were used for categorical and continuous variables, respectively. Regression analysis was used to determine whether deletion status predicts outcome. Results We studied 208 subjects with tetralogy of Fallot, 164 (79%) without, and 44 (20%) with a 22q11.2 deletion (22q11.2DS). There were no differences in sex, race, gestational age, age at diagnosis, admission weight, and duration of mechanical ventilation. Presenting anatomy, survival, complications and re-operations were also comparable between patients with and without 22q11.2DS. Those with 22q11.2DS had more aortopulmonary shunts preceding complete surgical repair (21% vs. 7%, p= 0.02). This association was present after adjustment for presenting anatomy (stenosis, atresia, or absence of pulmonary valve and common atrioventricular canal) and surgical era. In addition, those with 22q11.2DS had longer cardiopulmonary bypass time (84 vs. 72 minutes, p=0.02), and duration of intensive care (6 days vs. 4 days, p=0.007). Conclusions Genotype affects early operative outcomes in tetralogy of Fallot resulting, in particular, in longer duration of intensive care. Future studies are required to determine factors contributing to such differences in this susceptible population.