Risk Factors for Acquisition of Extended-spectrum Beta-lactamase and AmpC Resistant Gram-negative Bacteria in Critically Ill Infants With Congenital Heart Disease.

In a cohort of 257 infants with congenital heart disease admitted to the pediatric intensive care unit, 22 infants had positive cultures for extended-spectrum beta-lactamase or AmpC Gram-negative bacteria. These infants had longer exposure to broad-spectrum antibiotics, greater support with invasive devices and longer intensive care and hospital lengths of stay.

Comparative Effectiveness of Intranasal Dexmedetomidine-Midazolam versus Oral Chloral Hydrate Targeting Moderate Sedation during Pediatric Transthoracic Echocardiograms.

Objective To compare efficacy and safety of two moderate sedation regimens for transthoracic echocardiography (TTE): intranasal dexmedetomidine-midazolam (DM) versus oral chloral hydrate (CH) syrup. Method This was a retrospective cohort of 93 children under 4 years of age receiving moderate sedation with either DM or CH for TTE from January 2011 through December 2014. Measurements and Main Results Forty-nine patients received oral CH and 44 received the intranasal combination of DM. The demographics between groups were similar except the DM patients were slightly older and heavier (each p < 0.05). Failure rate between groups did not reach statistical significance (CH 14.3% vs. DM 6.8%; p = 0.324). Total sedation to discharge time was similar between groups (CH 89.4 minutes vs. DM 89.6 minute; p = 0.97). Cardiopulmonary data did reveal a significantly lower heart rate (101.9 vs. 91.7; p < 0.001) and respiratory rate (23.4 vs. 21.0, p = 0.03) in the DM group, but no difference in blood pressure measurements or echo determined shortening fraction. Conclusion These data support the use of intranasal DM as a safe and efficacious method of moderate sedation for children undergoing TTE.

Methylenetetrahydrofolate Reductase C677T: Hypoplastic Left Heart and Thrombosis.

The incidence of congenital heart defects is higher in infants with mutation of methylenetetrahydrofolate reductase (MTHFR) gene. The MTHFR C677T gene decreases the bioavailability of folate and increases plasma homocysteine, a risk factor for thrombosis. There have been no reported cases in the literature on the clinical implications of this procoagulable state in the setting of cyanotic heart disease, which itself has prothrombotic predisposition. Two patients with hypoplastic left heart syndrome developed postoperative thrombotic complications, both were homozygous for MTHFR C677T. We present these cases and highlight the implications of MTHFR mutation in the management of complex congenital heart disease.