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Over recent years, thanks to remarkable advances in pediatric cardiology, cardiac surgery and catheter interventions, survival of children with congenital heart disease has significantly increased with the majority of patients surviving into adulthood. Therefore, the prevalence of adult patients with congenital heart disease has dramatically increased, as well as the need for specific and dedicated programs. Acute heart failure, infective endocarditis and arrhythmias represent the most common causes of visit in the emergency department in this population. Our task force aimed at guiding physicians taking care of this peculiar cohort of patients in the emergency department.

Background Fluid challenge may help in the differential diagnosis between pre- and postcapillary pulmonary hypertension (PH). However, the test is still in need of standardization and better defined clinical relevance. Methods Two hundred twelve patients referred for PH underwent a right-sided heart catheterization with measurements before and after rapid infusion of 7mL/kg of saline. PH was defined as mean pulmonary artery pressure≥ 25mmHg, and postcapillary PH was defined as pulmonary artery wedge pressure (PAWP) > 15mmHg. An increase in PAWP≥ 18mmHg was considered diagnostic for postcapillary PH. At baseline, 66 patients received a diagnosis of no PH; 22, of postcapillary PH; and 124, of precapillary PH (mostly pulmonary arterial hypertension). Results After fluid challenge, five of 66 patients with no PH (8%) and eight of 124 with precapillary PH (6%) had the diagnosis reclassified as postcapillary PH. Fluid challenge was associated with an increase in PAWP by 7 ± 2mmHg in postcapillary PH and 3 ± 1mmHg in both precapillary PH and no-PH groups. Between-group differences were significant, but there was overlap. There were no adverse events related to fluid challenge. Prediction bands calculated from quadratic fits of the PAWP responses in pooled control subjects with no PH and patients with precapillary PH helped confirm 18mmHg as the cutoff for diagnosing postcapillary PH. Conclusions Fluid challenge with 7mL/kg saline increases PAWP, more in postcapillary than in precapillary PH or in control subjects with no PH. A cutoff value of 18mmHg allows reclassification of 6%to 8%of patients with precapillary PH or normal hemodynamic characteristics at baseline.

Percutaneous closure is widely recognized as the first therapeutic option in the majority of cases of secundum atrial septal defect (ASD) because of its high effectiveness and safety. Nonetheless, with the progressive increase of implanted devices and follow-up duration, several adverse events, some of them potentially life-threatening, have been reported. We report the case of an asymptomatic aortic erosion that occurred 13 years after the procedure. The main feature of our case is the very late occurrence of a life-threatening asymptomatic complication of ASD percutaneous closure, which should prompt lifelong surveillance in this population.