Congenital long QT syndrome (LQTS) is a group of heritable conditions that are associated with cardiac repolarization abnormalities characterized by QT prolongation on electrocardiogram and the risk of life-threatening arrhythmias. The prenatal detection of LQTS presents significant challenges for clinicians, and a multidisciplinary approach is required for optimal prenatal and postnatal management. In this comprehensive literature review, we describe strategies for the fetal diagnosis of LQTS with variable initial presentation, genetic testing in suspected fetal LQTS, the utility of fetal magnetocardiography as an additional diagnostic tool, prenatal management, and postnatal treatment. We focus on a multidisciplinary team approach including fetal cardiology, adult and pediatric electrophysiology, neonatology, maternal–fetal medicine, and genetic counselors, all playing vital roles in the comprehensive prenatal management and orchestration of postnatal treatment to optimize neonatal outcomes.

Congenital complete heart block (CCHB) is associated with high intrauterine and post-natal mortality. Prenatal detection and management, as well as appropriate delivery planning, may improve the outcomes in CCHB. We describe a rare case of CCHB that initially presented with fetal ascites and high-grade second-degree heart block noted on fetal echocardiography. The mother was noted to be positive for anti-SSA antibodies, and treatment with maternal steroids was started in an effort to reverse the fetal cardiac conduction abnormality. However, the fetal cardiac rhythm progressed to complete heart block by the follow up evaluation and the fetus had a continual declination of heart rate throughout the pregnancy to a low fetal heart rate of 25 beats per minute (bpm). This case demonstrates the lowest fetal ventricular rate documented in the literature and illustrates a severe presentation of a rare disease process. An overview of the existing knowledge related to etiology, prenatal evaluation with fetal echocardiography and fetal magnetocardiography, prenatal management, and delivery planning in fetuses with prenatally detected CCHB is included.

Fetal cardiology has evolved over the last 40 years and changed the timing of diagnosis and counseling of congenital heart disease, decision-making, planning for treatment at birth, and predicting future surgery from the postnatal to the prenatal period. Ethical issues in fetal cardiology transect multiple aspects of biomedical ethics including improvement in prenatal detection and diagnostic capabilities, access to equitable comprehensive care that preserves a pregnant person’s right to make decisions, access to all reproductive options, informed consent, complexity in shared decision-making, and appropriate use of fetal cardiac interventions. This paper first reviews the literature and then provides an ethical analysis of accurate and timely diagnosis, equitable delivery of care, prenatal counseling and shared decision-making, and innovation through in utero intervention.

Background Pediatric echocardiograms are performed daily on inpatient hospital units, predominantly in the Cardiac Intensive Care Unit. Understanding the precise study indication from the ordering providers is critical for completing imaging. Given the volume of echocardiograms ordered daily and staffing shortages, timely completion of echocardiograms is important for maintaining efficiency and productivity. The aim of this study was to identify areas to improve efficiency using quality improvement methodologies. Methods The SMART aim was to decrease the total echo completion time by 10% within 6 months. Using a fishbone diagram, limitations in the ordering system and inadequate ordering provider knowledge were identified as areas for improvement. Primary process and outcome metrics included total echo completion time, pre-echocardiogram buffer time, and order documentation of pertinent clinical history. PDSA 1 implemented order updates to provide increased specificity in orders with a picklist of common reasons for inpatient echocardiograms. PDSA 2 implemented a physician alert for STAT studies to reduce the number of inappropriate orders. PDSA 3 implemented additional order updates and staff education. Results Total echo completion time decreased with each cycle for total 25% reduction. This correlated with a decrease in the pre-echocardiogram buffer time of 56% without any significant changes in the number of images obtained per study or the daily inpatient volume. The documentation of pertinent clinical history increased by 18% and maintained at 99% appropriate documentation for the final two PDSA cycles. Pre and post intervention surveys indicated an overall improvement in the ordering process for both the inpatient ordering providers and echocardiography laboratory staff. Conclusions Echocardiography laboratory efficiency significantly improved throughout the course of this project without negative effects on the ordering providers or echocardiography laboratory staff. Our project demonstrates the ability to affect clinically relevant improvements utilizing simple workflow process changes in the pediatric echocardiography laboratory.