Evaluation of a Focused Cardiac Ultrasound Protocol in a Pediatric Emergency Department.

OBJECTIVES: The objective of this study was to evaluate the implementation of a focused cardiac ultrasound (FoCUS) protocol in a pediatric emergency department (PED). METHODS: We conducted a cross-sectional, observational, quality improvement project in a PED of an urban tertiary care children's hospital. A FoCUS protocol was collaboratively developed by pediatric cardiology and pediatric emergency medicine. This included a reference document with definitions, indications, image acquisition guidelines, and interpretation expectations. We measured physician-sonographer performance against pediatric cardiologist interpretation of stored cine clips as our reference standard. Focused cardiac ultrasound interpretation was dichotomized for the presence or absence of pericardial effusion, depressed left ventricular function, and chamber size abnormalities. Run charts were used to compare the number FoCUS performed each month and the quality of captured cine clips with those from the previous year. RESULTS: Ninety-two FoCUSs were performed by 34 different physician-sonographers from January to December 2016. The prevalence of FoCUS abnormalities was 18.5%. For pericardial effusion, sensitivity was 100% (95% confidence interval [CI], 48%-100%) and specificity was 99% (95% CI, 94%-100%). For depressed function, sensitivity was 100% (95% CI, 54%-100%) and specificity was 99% (95% CI, 94%-100%). For chamber size abnormalities, sensitivity was 100% (95% CI, 54%-100%) and specificity was 95% (95% CI, 89%-99%). The median number of monthly FoCUS increased from 1 (preprotocol) to 5 (postprotocol), and the median rate of adequate studies increased from 0% to 55%. CONCLUSIONS: We report the collaborative development and successful implementation of a PED FoCUS protocol. Physician-sonographer interpretation of FoCUS yielded acceptable results. Improvements in FoCUS utilization and cine clip adequacy were observed.

Fetal Echocardiographic Measures to Improve the Prenatal Diagnosis of Coarctation of the Aorta.

The objective of this study is to identify fetal echocardiographic measures that predict postnatal coarctation of the aorta (CoA). A retrospective review of patients from 2013 to 2017 identified 13 cases of prenatal diagnosis of CoA confirmed postnatally and 14 cases of prenatal diagnosis of CoA with normal arches postnatally. There were 30 controls. Measurements were made and indices applied on all available longitudinal fetal echocardiograms for each patient. Linear mixed effects models were used to examine the between-group differences in the trajectories of the measurements. Significant differences were seen in the true CoA group for the following: smaller distal transverse arch diameter to distance between the left common carotid and left subclavian arteries (DT/LCA-LSCA) index (p = 0.04), smaller distal transverse arch diameter (p = 0.005), and longer brachiocephalic to left common carotid artery (LCA) (p = 0.004) and LCA-left subclavian artery (LSCA) distances (p < 0.0001). Additionally, the LCA/DT index trend appears to differentiate false positives from true coarctations (p < 0.03). The fetal echocardiographic DT/LCA-LSCA index, brachiocephalic-LCA distance and LCA-LSCA distance are significant predictors of postnatal coarctation. The LCA/DT index trend over time may differentiate which of those patients with prenatal concern for coarctation are more likely to develop coarctation postnatally. The use of fetal echocardiographic measures may improve prenatal detection and predication of postnatal coarctation.

Modified Starnes procedure in a neonate with severe tricuspid regurgitation.

We report a modification of the Starnes technique for palliating severe tricuspid regurgitation associated with a dysplastic right ventricle in a neonate, using a fenestrated pericardial patch allowing for unidirectional flow. The patient eventually underwent a successful Glenn shunt construction with a persistent reduction in right ventricle size at 1 year follow-up.

Is the rate of congenital heart defects detected by fetal echocardiography among pregnancies conceived by in vitro fertilization really increased?: a case-historical control study.

OBJECTIVE: We investigated the prenatal prevalence of congenital heart defects (CHDs) among in vitro fertilization (IVF) pregnancies at a referral program in the United States. METHODS: Study patients were referred for fetal echocardiography between April 1, 2006, and May 1, 2009, due to IVF. An IVF pregnancy was defined as a patient who conceived with IVF with or without intracytoplasmic sperm injection. Congenital heart defect odds relative to historical data were calculated by standard methods. P < .05 was considered statistically significant. RESULTS: During the study period, we performed fetal echocardiography on 749 consecutive IVF pregnancies. Overall, the frequency of CHDs was 1.1% (95% confidence interval, 0.3%-1.8%) per pregnancy. Compared to earlier historical population data, IVF pregnancies had a significantly higher risk of CHDs (odds ratios, 7.3 [3.6-14.7] and 2.9 [1.4-5.9], respectively). However, compared to more contemporary population data, there was no difference in the CHD risk between IVF gestations and naturally conceived pregnancies. Further analysis indicated that IVF twin pregnancies were as much as 12.5 (4.6-33.5) times as likely to have CHDs compared to a general population. CONCLUSIONS: In this study population, the frequency of CHDs in IVF pregnancies was higher than early historical population data; however, it was similar to that of a more contemporary general population. Further analysis showed that this increase was mainly driven by IVF twin gestations. Previous reports of increased CHD risk in pregnancies conceived via IVF may have been due, in part, to an increased frequency of higher-order pregnancies seen among these patients.

Prenatal course of isolated muscular ventricular septal defects diagnosed only by color Doppler sonography: single-institution experience.

OBJECTIVE: Counseling patients with an isolated ventricular septal defect (i-VSD) is clinically important because with high-resolution ultrasound equipment, more small muscular VSDs are now being diagnosed. The prevalence of these lesions is not yet completely described, and the frequency with which muscular VSDs resolve in utero has also not been extensively reported. METHODS: We investigated the perinatal course of isolated muscular VSDs diagnosed only on color Doppler examinations and followed between January 1, 2005, and December 31, 2006. A complete evaluation of the fetal heart was performed by gray scale, spectral Doppler, and color Doppler examinations. RESULTS: We performed a total of 2583 fetal echocardiographic examinations on 2410 fetuses during 2318 pregnancies. The study group included 78 twin gestations (3.4%) and 7 triplet gestations (0.3%). There were 16 fetuses with an i-VSD (6.6/1000 fetuses) within the study group. The mean gestational age +/- SD at diagnosis was 23.5 +/- 4.3 weeks. Two of the i-VSDs (12.5%) spontaneously resolved prenatally. One fetus with an i-VSD had trisomy 21 and also had increased nuchal translucency in the first trimester. One i-VSD was diagnosed among 22 fetuses with trisomy 21 examined during the study period. CONCLUSIONS: An i-VSD is a common congenital heart defect. Prenatal resolution of i-VSDs is less frequent than reported in the literature. A larger cohort is needed to provide a better risk estimate for aneuploidy in the presence of an i-VSD.

Prevalence of congenital heart defects in monochorionic/diamniotic twin gestations: a systematic literature review.

OBJECTIVE: Congenital heart defects (CHDs) affect approximately 0.5% of all neonates. Recent literature points to a possible increase in the CHD prevalence among monochorionic/diamniotic (MC/DA) twin gestations. We hypothesized that MC/DA twin pregnancy is a risk factor for CHD. METHODS: A systematic review of all published English literature was conducted on MEDLINE (Ovid and PubMed) from January 2000 through April 2007 using the medical subject heading terms "congenital heart defect" and "monozygotic twins." Four observational studies were included in the final analysis. Published historical data were used for the population background risk of CHD. Relative risk (RR) estimates with 95% confidence intervals (CIs) were calculated by fixed and random effect models. RESULTS: We included a total of 40 fetuses with CHDs among 830 fetuses from MC/DA twin gestations. Compared with the population, CHDs were significantly more prevalent in MC/DA twins regardless of the presence of twin-twin transfusion syndrome (TTTS) (RR, 9.18; 95% CI, 5.51-15.29; P < .001). Monochorionic/diamniotic twin gestations affected by TTTS were more likely to be complicated by CHDs than those that did not have TTTS (RR, 2.78; 95% CI, 1.03-7.52; P = .04). Ventricular septal defects were the most frequent heart defects. Pulmonary stenosis and atrial septal defects were significantly more prevalent in pregnancies complicated with TTTS. CONCLUSIONS: Monochorionic/diamniotic twin gestation appears to be a risk factor for CHDs. Conditions that lead to abnormal placentation may also contribute to abnormal heart development, especially in MC/DA twin pregnancies complicated with TTTS. Fetal echocardiography may be considered for all MC/DA twin gestations because ventricular septal defects and pulmonary stenosis are the most common defects.

Outcomes after a Ladd procedure for intestinal malrotation with heterotaxia.

PURPOSE: Intestinal rotation abnormalities and complex congenital heart disease associated with heterotaxia coexist. Despite the risk for midgut volvulus, performing a Ladd procedure for asymptomatic malrotation with heterotaxia remains to be controversial because the presumed risk for postoperative complications is thought to exceed the benefits of the operation. The purpose of this study was to review the incidence of complications after a Ladd procedure in asymptomatic patients with heterotaxia to guide recommendations for this patient population. METHODS: The medical records of all patients with heterotaxia who underwent a Ladd procedure for asymptomatic malrotation between 1984 and 2004 were reviewed. Type of cardiac disease, postoperative complications, and survival were recorded. RESULTS: Twenty-two patients (9 boys and 13 girls) with heterotaxia underwent an elective Ladd procedure after their medical stabilization or surgical correction or palliation of their cardiac anomaly. Of these patients, 19 were younger than 1 month at the time of the operation. The remaining 3 patients underwent the operation when they were between 2 and 5 months old. Three of the 22 patients (14%) developed postoperative intestinal obstruction: lysis of adhesions was performed in 1 patient; another patient required a staged bowel resection for a closed loop obstruction; and yet another patient had recurrent midgut volvulus 4 years after an incomplete initial Ladd procedure. All patients survived the initial and secondary procedures. Four deaths, all more than 1 month after the surgery, occurred as sequelae of the underlying cardiac anomaly. Length of follow-up ranged from 1 to 17 years. CONCLUSIONS: We report on a 14% risk of postoperative bowel obstruction after an elective Ladd procedure, as compared with a small but significant incidence of midgut volvulus in patients with malrotation in the setting of complex congenital heart disease. Our results support the conclusion that an elective Ladd procedure at a time of relative cardiac stability for selected patients with heterotaxia has an acceptably low morbidity and should be considered to prevent midgut volvulus.

Training pediatric residents to evaluate congenital heart disease in the current era.

Working with young pediatricians to create an exciting educational environment in which to learn cardiology remains a challenge for all of us. Numerous forces impact our efforts, making the process of training residents to evaluate and treat congenital heart disease in the current era a dynamic and, at times, difficult endeavor. This article considers the changes that have occurred in the medical school graduate who chooses pediatrics and subsequently pediatric cardiology as a career; explores the changes in the graduate medical training guidelines, requirements, and restrictions that have been put into place within the last several years; and discusses the seemingly constant advances in scientific understanding and technology that shape our field and move us forward as a discipline.