Appropriateness and Cost-Effectiveness of Echocardiograms Ordered by Pediatric Cardiologists and Primary Care Providers for Syncope.

The pediatric appropriate use criteria (AUC) were applied to transthoracic echocardiograms (TTE) ordered by primary care providers (PCPs) and pediatric cardiologists for the diagnosis of syncope to compare appropriateness ratings and cost-effectiveness. Included were patients ≤18 years of age from October 2016 to October 2018 with syncope who underwent initial outpatient pediatric TTE ordered by a PCP or were seen in Pediatric Cardiology clinic. Ordering rate of TTE by pediatric cardiologists, AUC classification, and TTE findings were obtained. PCPs ordered significantly more TTEs than pediatric cardiologists for "rarely appropriate" indications (61.5% vs 7.5%, P < .001). Cardiologists ordered TTEs at 17.2% of visits. Using appropriateness as a marker of effect, with the incremental cost-effectiveness ratio, it was more cost-effective ($543.33 per patient) to refer to a pediatric cardiologist than to order the TTE alone. This suggests that improved PCP education of the AUC and appropriate indications of TTEs for syncope may improve cost-effectiveness when using order appropriateness as a marker of effectiveness.

Successful childbirth in a Fontan with protein losing enteropathy.

As survival rates and functional status of the adult single ventricle population have grown, some may become pregnant inadvertently or against our advice. The outcomes are often poor, being worse for the fetus/baby rather than the mother with a Fontan circuit. No reports of a successful delivery of a healthy baby to a Fontan mother with protein losing enteropathy were found in the literature. We present one such case.

Evaluation of the Tolerability of Intermittent Intravenous Sildenafil in Pediatric Patients With Pulmonary Hypertension.

OBJECTIVES: The primary purpose of this study was to determine the tolerability of intermittent intravenous (IV) sildenafil for the treatment of pulmonary hypertension in pediatric patients. Secondary objectives were to evaluate parameters related to efficacy. METHODS: This was a retrospective chart review from January 2013 to August 2014 of pediatric patients under age 18 years treated with intermittent doses of IV sildenafil for pulmonary hypertension. Patients were excluded if they were over age 18 years or received sildenafil for other indications. Measures collected to assess tolerability include blood pressure and heart rate before and after the administration of IV sildenafil, as well as adverse events. RESULTS: Thirty-seven patients (21 females and 16 males) were identified meeting inclusion criteria, and 21 (56.8%) were on oral sildenafil prior to the initial IV dose. The mean decrease in blood pressure after the first dose of IV sildenafil was 7.16/2.74 mmHg. The decrease in systolic blood pressure was statistically significant. During the study period, 5 patients experienced medication related adverse events, primarily hypotension. Despite this, none of the patients had the medication discontinued due to these events. For secondary objectives, a statistically significant difference was not found between other clinical measures before and after intermittent IV sildenafil dosing. CONCLUSIONS: Sildenafil, when administered as intermittent IV doses, was tolerated by the majority of patients evaluated in this study. For pediatric patients with pulmonary hypertension in whom enteral or continuous IV sildenafil cannot be administered, intermittent IV sildenafil may be considered as an alternative administration option.

Aortitis in a child with Abiotrophia defectiva endocarditis.

Abiotrophia defectiva, one of several nutritionally variant Streptococcus species, is an uncommon but important cause of endocarditis in children. We describe an unusual case complicated by extensive aortitis with pits in the ascending aorta and the proximal aortic arch.