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Considerable variation exists in interstage outpatient care after the Norwood procedure in the care domains of discharge communication, nutrition, and home surveillance. Standardizing care around evidence-based practices may improve the outcomes for these very high-risk children.

Objective To evaluate temporal trends in prenatal diagnosis of transposition of the great arteries with intact ventricular septum (TGA/IVS) and its impact on neonatal morbidity and mortality. Methods Newborns with TGA/IVS referred for surgical management to our center over a 20-year period (1992 – 2011) were included. The study time was divided into 5 four-year periods, and the primary outcome was rate of prenatal diagnosis. Secondary outcomes included neonatal pre-operative status and perioperative survival. Results Of the 340 patients, 81 (24%) had a prenatal diagnosis. Prenatal diagnosis increased over the study period from 6% to 41% (p<0.001). Prenatally diagnosed patients underwent a balloon atrial septostomy (BAS) earlier than postnatally diagnosed patients (0 vs. 1 day, p<0.001) and fewer required mechanical ventilation (56% vs. 69%, p=0.03). There were no statistically significant differences in pre-operative acidosis (16% vs. 26%, p=0.1) and need for preoperative ECMO (2% vs. 3%, p=1.0). There was also no significant mortality difference (1 pre-operative and no post-operative deaths among prenatally diagnosed patients, as compared to 4 pre-operative and 6 post-operative deaths among postnatally diagnosed patients). Conclusion The prenatal detection rate of TGA/IVS has improved but still remains below 50%, suggesting the need for strategies to increase detection rates. The mortality rate was not statistically different between pre- and postnatally diagnosed patients; however, there were significant pre-operative differences with regard to earlier BAS and less mechanical ventilation. Ongoing study is required to elucidate whether prenatal diagnosis confers long-term benefit.

Fetal aortic valvuloplasty (FAV) has shown promise in averting progression of mid-gestation aortic stenosis (AS) to hypoplastic left heart syndrome in a subset of patients. Patients who achieve biventricular circulation after FAV frequently have left ventricular (LV) diastolic dysfunction (DD). This study evaluates DD in fetuses with AS by comparing echocardiographic indices of LV diastolic function in fetuses undergoing FAV (n=20) to controls (n=40) and evaluates for LV factors associated with DD in FAV patients. We also compared pre- and post-FAV DD variables (n=16). Median gestational age (24 weeks, range 18–29 weeks) and fetal heart rate were similar between FAV and controls. Compared to controls, FAV patients had universally abnormal LV diastolic parameters including fused mitral inflow E and A waves (p=0.008), higher E velocity(p<0.001), shorter mitral inflow time (p=0.001), lower LV lateral annulus E′ (p<0.001), septal E′ (p=0.003) and higher E/E′ (p<0.001) than controls. FAV patients had abnormal right ventricular mechanics with higher tricuspid inflow E velocity (p<0.001), and shorter tricuspid inflow time (p=0.03). Worse LV diastolic function (lower LV E′) was associated with higher endocardial fibroelastosis (EFE) grade (r=0.74, p<0.001), large LV volume (r=0.55, p=0.013) and sphericity (r=0.58, P=0.009) and with lower LV pressure by mitral regurgitation jet (r=−0.68, p<0.001). Post-FAV, fewer patients had fused mitral inflow E and A than pre-FAV (p=0.05) and septal E′ was higher (=0.04). In conclusion, fetuses with mid-gestation AS have evidence of marked DD. Worse DD is associated with larger, more spherical LV, with more extensive EFE and lower LV pressure.

Surgical repair of TGA performed in the developing world is associated with an early survival of 85%. Type of surgical repair and age at operation varied considerably, but no associations with mortality were identified. In contrast, poor nutrition and small surgical volume were most strongly associated with mortality. Multicenter collaborative quality improvement efforts may benefit patients with TGA in the developing world.

We describe a novel 4-week-long boot camp designed to expose incoming paediatric cardiology fellows to the broad spectrum of knowledge and skills required for the practice of paediatric cardiology. The experience increased trainee confidence and sense of preparedness to begin fellowship-related responsibilities. Given that highly interactive activities were rated most highly, boot camps in paediatric cardiology should strongly emphasise these elements.

Objective Fetal echocardiography (FE) provides detailed information about cardiac structure and function in-utero. Limited information is available regarding normal findings late in pregnancy. We therefore sought to identify and describe common cardiac findings in late gestation. Methods Fetuses with structurally normal hearts were identified in mid-gestation within a subset of pregnant women in a prospective study. Atrioventricular valves (AV), right and left atrium (RA/LA), aortic isthmus (AoI) and ductus arteriosus (DA) dimensions and flow abnormalities, aneurysm of septum primum, and presence/grade of tricuspid regurgitation (TR) were assessed throughout pregnancy. Linear and logistic regression analyses were used to characterize change in quantitative and qualitative FE parameters by gestational age. Results 40 fetuses between 24 and 38 weeks gestational age (GA) were studied. Each had a FE study completed before and after 34 weeks gestation that were compared. Tricuspid/mitral valve and RA/LA ratios increased with GA (p<0.001). More frequently noted after 34 weeks GA were tapering of the DA (2.5% vs. 32%), prominent AoI diastolic flow (5% vs. 67%), prominent DA diastolic flow (2.5% vs. 25%), trivial/mild TR (35% vs. 80%), and aneurysm of septum primum (37% vs 80%). These findings all increased linearly with GA (p<0.001). Conclusion AV valve and RA/LA disproportion, mild DA tapering, prominent AoI and DA diastolic flow, trivial/mild TR, and aneurysm of septum primum are frequently identified after 34 weeks GA. This suggests that these FE findings in isolation are likely normal and are a result of the physiologic alterations that occur late in the third trimester.

Heterotaxy (HTX) is a rare condition of abnormal thoraco-abdominal organ arrangement across the left–right axis of the body. The pathogenesis of HTX includes a derangement of the complex signaling at the left–right organizer early in embryogenesis involving motile and non-motile cilia. It can be inherited as a single-gene disorder, a phenotypic feature of a known genetic syndrome or without any clear genetic etiology. Most patients with HTX have complex cardiovascular malformations requiring surgical intervention. Surgical risks are relatively high due to several serious comorbidities often seen in patients with HTX. Asplenia or functional hyposplenism significantly increase the risk for sepsis and therefore require antimicrobial prophylaxis and immediate medical attention with fever. Intestinal rotation abnormalities are common among patients with HTX, although volvulus is rare and surgical correction carries substantial risk. While routine screening for intestinal malrotation is not recommended, providers and families should promptly address symptoms concerning for volvulus and biliary atresia, another serious morbidity more common among patients with HTX. Many patients with HTX have chronic lung disease and should be screened for primary ciliary dyskinesia, a condition of respiratory cilia impairment leading to bronchiectasis. Mental health and neurodevelopmental conditions need to be carefully considered among this population of patients living with a substantial medical burden. Optimal care of children with HTX requires a cohesive team of primary care providers and experienced subspecialists collaborating to provide compassionate, standardized and evidence-based care. In this statement, subspecialty experts experienced in HTX care and research collaborated to provide expert- and evidence-based suggestions addressing the numerous medical issues affecting children living with HTX.