Aims. To outline the etiology, clinical course, short-term survival to discharge and neurological outcome of infants (<1 yr) with new cardiac diagnoses presenting to a pediatric intensive care (PICU) unit with acute cardiac compromise.Methods. Retrospective search of a computerized database and medical case notes for all acute cardiac admissions to PICU from June 2001 to 2006. Pre-existing hospital-based patients with new cardiac diagnoses were excluded.Results. Seventy patients were identified, 38 (54%) of whom were male. There were six main subgroups: obstructive left heart lesions (n = 20), transposition of the great arteries (TGA) (n = 9), total anomalous pulmonary venous drainage (TAPVD) (n = 7), dilated cardiomyopathy (n = 11), arrhythmia (n = 12), and others (n = 11). Fifty-nine patients (84%) were external referrals to our center. The median age at presentation was 13.5 days (0-272) with median duration of symptoms of 1 day (0-21). The median base deficit at presentation was -7.6 mEq/L (-43 to +4.2). Fifty-three patients (76%) required respiratory support with a median duration of ventilation of 4 days (1-49). Fifty-six patients (80%) required inotropic support. The median PICU stay was 7 days (1-64) with a median total hospital stay of 16 days (1-71). Six patients (9%) died prior to discharge. Of the survivors 7 (11%) had seizure activity or evolving clinical neurological abnormalities.Conclusions. Cardiovascular compromise due to previously unrecognized congenital or acquired heart disease is associated with clinically significant morbidity and mortality. Longer term follow-up is required to evaluate the initial effect of poor cardiac output and hypoxia on long-term neurodevelopmental outcome.

AIMS:To determine the efficacy of the implantable loop recorder (ILR) in patients with congenital heart disease. METHODS AND RESULTS:Retrospective data analyses of all patients with congenital heart disease undergoing ILR implantation in a congenital cardiac centre. Of 78 ILRs implanted between July 2000 and October 2007, 22 (15 male) patients had congenital heart disease. The median age at implantation was 15.5 years (7.0-46.5). Six patients had neurodevelopmental delay. Indications for implantation were syncope (n = 15), palpitations (n = 4), and pre-syncope (n = 3). Median implantation time was 19 months (1-30). Explantation data were available in 16 patients (73%). Implantable loop recorders were explanted following establishment of positive diagnosis (n = 3), negative diagnosis (n = 7), and at the end of battery life (n = 4). One patient died with the ILR in situ. One device eroded after 2 months without further complication. Following ILR interrogation, diagnosed arrhythmias included ventricular tachycardia (n = 2) and sinus node dysfunction (n = 1). On the basis of these findings, two patients had intracardiac defibrillators and one patient had a dual-chamber pacemaker implanted. CONCLUSION:Implantable loop recorders lead to a positive or negative arrhythmic diagnosis following explantation in 71% of patients with congenital heart disease. This is particularly beneficial in patients with neurodevelopmental delay where accurate history is often limited.