Oral medication can be superior to IM injections for sedating children with congenital heart disease; however, the safety of all medications remains an issue.

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Our study demonstrates that patients SCD on LTE have cardiac dysfunction based on elevated LVMPI. This may be a reflection of the global severity of disease. Our findings merit further investigation with serial monitoring of LVMPI on a larger number of patients with SCD.

The purpose of the study was to assess the feasibility and safety of symptom-limited cardiopulmonary stress testing (CPST) in children with sickle cell disease (SCD), who are on long-term erythrocytapheresis. Maximal symptom-limited CPST was performed in 16 children with SCD who were maintained on long-term erythrocytapheresis and the exercise response in this patient cohort was compared with those of a healthy control population. All patients completed the CPST without any complications. Twelve patients with SCD had significant reduction in aerobic capacity [peak oxygen consumption (VO2) <80% predicted]. No patient demonstrated evidence of myocardial ischemia. In summary, symptom-limited CPST can be performed safely in a subgroup of children with SCD and can yield valuable clinical information.

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Our objective is to describe our approach to the management of patients with single-ventricle physiology and restrictive tunnel patent foramen ovale (TPFO) with unfavorable atrial septal morphology. We describe a series of five patients with single-ventricle physiology and restrictive TPFO and our experience with radiofrequency perforation (RFP), static balloon atrial septostomy (BAS), and stent implantation to create an alternative pathway for left atrial decompression. Between July 4, 2006, and July 10, 2007, five patients with single-ventricle physiology and restrictive TPFO were brought to the cardiac catheterization laboratory for decompression of a hypertensive left atrium. Four of five patients underwent RFP followed by static BAS and stent implantation across the newly created atrial communication. One patient had a stent placed across an existing TPFO. Unfortunately, stable stent position was not achieved in this case, and the patient required open atrial septectomy. In patients with single-ventricle physiology and a restrictive TPFO associated with left atrial hypertension, stent placement across the existing defect can result in unstable stent position. Using a RFP wire to create a new defect in the septum primum allows stable stent deployment across the atrial septum and achieves left atrial decompression.