Prenatally diagnosed coronary artery abnormalities in hypoplastic left heart syndrome are associated with a higher probability of heart transplantation.

OBJECTIVE: Coronary artery abnormalities (CA) occur in patients with hypoplastic left heart syndrome (HLHS) and may be associated with higher mortality and heart transplantation (HT). We aimed to determine whether fetuses with HLHS and prenatal CA have a higher risk of death or HT. METHODS: We performed a retrospective review of fetal echocardiograms with HLHS from 2011 to 2018. We excluded fetuses with ventricular septal defects, elective termination, death in utero, planned postnatal non-intervention, or absent follow-up data. Presence or absence of CA was determined by review of serial fetal echocardiograms. Survival analysis was used to evaluate the relationship between prenatal CA and death or HT. RESULTS: Of 86 patients with fetal HLHS, 11 had prenatal diagnosis of CA. Of these, six required HT and five died (one after undergoing HT); only one remains alive without HT. Of those without prenatal CA (n = 75), 25 died and 7 underwent HT. Patients with prenatal diagnosis of HLHS and CA had a significantly increased likelihood of death or HT (p-value <0.05). CONCLUSION: Prenatal diagnosis of CA in our cohort of patients with HLHS was associated with increased risk of death or HT. These data have significance for prenatal counseling and postnatal management.

Tetralogy of Fallot Associated With Right Aortic Arch and Isolated Left Brachiocephalic Artery.

A newborn presented with tetralogy of Fallot (TOF), right aortic arch (RAA), and isolated left brachiocephalic artery. The RAA supplied the right common carotid artery, right vertebral artery, and right subclavian artery, in that order. The left common carotid and left subclavian arteries were in continuity with no aortic origin. Ultrasound demonstrated retrograde flow in the left vertebral artery supplying antegrade flow to the diminutive left subclavian artery (ie, "steal phenomenon"). The patient underwent repair of TOF without intervention on the left common carotid or left subclavian arteries and is being followed conservatively.

Total Parenteral Nutrition Standardization and Electronic Ordering to Reduce Errors: a Quality Improvement Initiative.

INTRODUCTION: Total parenteral nutrition (TPN) provides vital intravenous nutrition for patients who cannot tolerate enteral nutrition but is susceptible to medical errors due to its formulation, ordering, and administrative complexities. At Johns Hopkins All Children's Hospital, 22% of TPN orders required clarification of errors and averaged 10 minutes per order for error correction by pharmacists. Quality improvement methodology improved patient safety by standardizing TPN formulations and incorporating TPN ordering processes into the electronic medical record. METHODS: A multidisciplinary group of providers developed standardized TPN solutions for neonatal and pediatric patients. Inclusion, exclusion, and discontinuation criteria were defined. The primary outcome measure was reducing TPN ordering error rate, and secondary outcomes were improving TPN ordering and processing time along with reducing blood draws. Through multiple plan-do-study-act cycles, we standardized TPN solutions, incorporated them in the electronic medical record, monitored blood draws, and evaluated resource efficiency. Data were analyzed using chi-square tests of independence and t tests for 2 independent samples. RESULTS: The TPN ordering error rate significantly decreased from baseline of 22% to 3.2% over the final quarter of the study period, χ2 (1, N = 2,467) = 89.13, P < 0.001. Order processing time fell from 10 to 5 minutes by project end. The average number of blood draws decreased significantly from 6.2 (SD = 3.12) blood draws to 4.3 (SD = 2.13) in the last quarter of the study, t (506) = 5.97, P < 0.001. CONCLUSIONS: Standardizing TPN and transitioning to electronic ordering effectively and significantly reduced ordering errors and processing time. It also substantially improved resource efficiency by reducing the number of blood draws.

Role of myofibril-inducing RNA in cardiac TnT expression in developing Mexican axolotl.

The Mexican axolotl, Ambystoma mexicanum, has been a useful animal model to study heart development and cardiac myofibrillogenesis. A naturally-occurring recessive mutant, gene "c", for cardiac non-function in the Mexican axolotl causes a failure of myofibrillogenesis due to a lack of tropomyosin expression in homozygous mutant (c/c) embryonic hearts. Myofibril-inducing RNA (MIR) rescues mutant hearts in vitro by promoting tropomyosin expression and myofibril formation thereafter. We have studied the effect of MIR on the expression of various isoforms of cardiac troponin T (cTnT), a component of the thin filament that binds with tropomyosin. Four alternatively spliced cTnT isoforms have been characterized from developing axolotl heart. The expression of various cTnT isoforms in normal, mutant, and mutant hearts corrected with MIR, is evaluated by real-time RT-PCR using isoform specific primer pairs; MIR affects the total transcription as well as the splicing of the cTnT in axolotl heart.