Prenatal detection of congenital heart disease: Recent experience across the state of Arizona.

OBJECTIVE: To determine the prenatal detection rate (PDR) of congenital heart disease (CHD) in Arizona as well as describe various factors that may influence detection rates. METHODS: This was a retrospective chart review using the Society of Thoracic Surgeons and Phoenix Children's Fetal Cardiology databases. We included all cases of CHD requiring surgery <1 year of age between 2013 and 2018. A total of 1137 patients met the criteria, and various demographic, socioeconomic, and patient outcome data were collected. RESULTS: The overall PDR was 58% with an improving detection rate over the course of our study, with the final year having a PDR of 67%. Over time, PDR improved in urban communities, but this was not seen in rural communities. Rural address, public insurance, and Native American ethnicity were associated with lower PDR. Postnatal outcomes, including Apgars, initial pH, and lactate, did not differ with the presence of a prenatal diagnosis. Diagnoses typically identified with the outflow tract and 3-vessel views on the fetal echocardiogram were less likely to be detected prenatally. CONCLUSIONS: The PDR of CHD continues to improve with evolving technologies and guidelines. We highlight a discrepancy between urban, rural, and Native American populations. Additionally, by supplying descriptors of missed diagnosis and associated echocardiography views, we hope to provide data for future interventions.

Contribution of fetal magnetic resonance imaging in fetuses with congenital heart disease.

BACKGROUND: Increasing evidence supports an association among congenital heart disease (CHD), structural brain lesions on neuroimaging, and increased risk of neurodevelopmental delay and other structural anomalies. Fetal MRI has been found to be effective in demonstrating fetal structural and developmental abnormalities. OBJECTIVE: To determine the contribution of fetal MRI to identifying cardiovascular and non-cardiovascular anomalies in fetuses with CHD compared to prenatal US and fetal echocardiography. MATERIALS AND METHODS: We performed a retrospective study of fetuses with CHD identified by fetal echocardiography. Exams were performed on 1.5-tesla (T) or 3-T magnets using a balanced turbo field echo sequence triggered by an external electrocardiogram simulator with a fixed heart rate of 140 beats per minute (bpm). Fetal echocardiography was performed by pediatric cardiologists and detailed obstetrical US by maternal-fetal medicine specialists prior to referral to MRI. We compared the sensitivity of fetal MRI and fetal echocardiography for the diagnosis of cardiovascular anomalies, as well as the sensitivity of fetal MRI and referral US for the diagnosis of non-cardiac anomalies. We performed statistical analysis using the McNemar test. RESULTS: We identified 121 anomalies in 31 fetuses. Of these, 73 (60.3%) were cardiovascular and 48 (39.7%) involved other organ systems. Fetal echocardiography was more sensitive for diagnosing cardiovascular anomalies compared to fetal MRI, but the difference was not statistically significant (85.9%, 95% confidence interval [CI] 77.8-94.0% vs. 77.5%, 95% CI 67.7-87.2%, respectively; McNemar test 2.29; P=0.13). The sensitivity of fetal MRI was higher for diagnosing extracardiac anomalies when compared to referral US (84.1%, 95% CI 73.3-94.9% vs. 31.8%, 95% CI 18.1-45.6%, respectively; McNemar test 12.9; P<0.001). The additional information provided by fetal MRI changed prognosis, counseling or management for 10/31 fetuses (32.2%), all in the group of 19 fetuses with anomalies in other organs and systems besides CHD. CONCLUSION: Fetal MRI performed in a population of fetuses with CHD provided additional information that altered prognosis, counseling or management in approximately one-third of the fetuses, mainly by identifying previously unknown anomalies in other organs and systems.

Factors Influencing Pediatric Outpatient Transthoracic Echocardiography Utilization Before Appropriate Use Criteria Release: A Multicenter Study.

BACKGROUND: Although pediatric appropriate use criteria (AUC) for outpatient transthoracic echocardiography (TTE) are available, little is known about TTE utilization patterns before their release. The aims of this study were to determine the relation between AUC and TTE utilization and to identify patient and physician factors associated with discordance between the AUC and clinical practice. METHODS: A retrospective review of 3,000 initial outpatient pediatric cardiology encounters at six centers was performed. Investigator-determined indications were classified using AUC definitions. Concordance between AUC and TTE utilization was determined. Multivariate analysis was performed to identify patient and physician factors associated with TTE's being performed for rarely appropriate and TTE's not being performed for appropriate indications. RESULTS: Concordance between AUC and TTE utilization was 88%. TTE was performed for rarely appropriate indications in 9% and was associated with patient age < 3 months, indications of murmur, noninvasive imaging physician subspecialty, and physician volume. No TTE was ordered for appropriate indications in 3% and was associated with indications including prior test result (primarily abnormal electrocardiographic findings), older patients, and physician subspecialty other than generalist or imaging. There was high variability in TTE utilization among centers. CONCLUSIONS: There was a reasonable degree of concordance between AUC and clinical practice before AUC publication. Several patient and physician factors were associated with discordance with the AUC. These findings should be considered in efforts to disseminate the AUC and in the development of future iterations. The causes for variation among centers deserve further exploration.

Tricuspid annular plane systolic excursion as a marker of right ventricular dysfunction in pediatric patients with dilated cardiomyopathy.

BACKGROUND: Right ventricular systolic dysfunction (RVSD) is a predictor of outcomes in dilated cardiomyopathy (DCM) in adults, but little is known in children. Tricuspid annular plane systolic excursion (TAPSE) has emerged as a reliable tool to assess RVSD. We sought to determine the prevalence and prognostic significance of RVSD using TAPSE in children with DCM. METHODS: The first echocardiogram at the time of diagnosis with DCM was analyzed to obtain left ventricular ejection fraction (LVEF) and 2D TAPSE. RVSD was defined as TAPSE age-based z-score ≤-2. The association of a composite primary endpoint including death, mechanical support, or transplantation with RVSD was analyzed using LVEF and TAPSE. RESULTS: Of the 96 patients, RVSD was noted in 84 (88%). During a median follow-up of 17.5 months (IQR 1.4, 76.2), the primary endpoints were reached in 59%. There was a lower LVEF in patients with RVSD (P=.016). Moderate or severe RVSD showed a significant association with the composite primary endpoint (unadjusted OR 2.7 [95% CI: 1.15-6.33], P=.023). Kaplan-Meier analysis showed that the event-free survival was significantly lower in patients with LVEF ≤30% and TAPSE z-score ≤-4. CONCLUSION: Children with DCM have a high prevalence of RVSD based on reduced TAPSE, and those with moderate or severe RVSD have worse clinical outcomes.

Complex Surgical Repair of a Flail Tricuspid Valve After Chest Wall Trauma in a Pediatric Patient.

Traumatic injury to the tricuspid valve can result from severe nonpenetrating chest wall trauma. We present the case of an initially asymptomatic 11-year-old girl who was kicked in the chest by a horse. The trauma resulted in avulsed papillary muscles, ruptured chordae, and right heart failure. She underwent early tricuspid valve reconstruction and annuloplasty. We advocate for routine use of echocardiography to assess for traumatic injury to intracardiac structures. In addition, we believe that early surgical intervention may prevent right-sided heart dysfunction, atrial arrhythmias, and the need for valve replacement.

Fetal Diagnostics and Fetal Intervention.

Advances in ultrasound technology and specialized training have allowed clinicians to diagnose congenital heart disease in utero and counsel families on perinatal outcomes and management strategies, including fetal cardiac interventions and fetal surgery. This article gives a detailed approach to fetal cardiac assessment and provides the reader with accompanying figures and video clips to illustrate unique views and sweeps invaluable to diagnosing congenital heart disease. We demonstrate that using a sequential segmental approach to evaluate cardiac anatomy enables one to decipher the most complex forms of congenital heart disease. Also provided is a review of fetal cardiac intervention and surgery from the fetal cardiologist's perspective.

Contemporary Results of Aortic Coarctation Repair Through Left Thoracotomy.

BACKGROUND: Although surgical results for repair of coarctation of the aorta (CoA) have steadily improved, management of this condition remains controversial. The purposes of this study were to analyze the long-term outcomes of patients undergoing CoA repair through left thoracotomy and to define risk factors for reintervention. METHODS: All patients who were less than 18 years old and who underwent initial repair of CoA through left thoracotomy from 1995 to 2013 at Texas Children's Hospital (Houston, TX) were included. Patients were classified into 3 groups: 143 (42%) neonates (0 to 30 days old), 122 (36%) infants (31 days to 1 year old), and 78 (23%) older children (1 to 18 years old). Univariate and multivariate analyses were performed. RESULTS: A total of 343 patients (129 [38%] girls) with median age of 53 days (interquartile range [IQR],12 days to 9 months) and weight of 4.1 kg (IQR, 3.1 to 8.0) underwent repair with extended end-to-end anastomosis (291 patients [85%]), end-to-end anastomosis (44 patients [13%]), interposition graft (2 patients [0.6%]), or subclavian flap (6 patients [2%]). Concomitant diagnoses included genetic abnormalities (48 patients [14%]), isolated ventricular septal defects (58 patients [17%]), small left-sided structures (53 patients,16%), or other complex congenital heart disease (18 patients [5%]). Perioperative mortality was 1% (n = 4, all neonates). At a median follow-up of 6 years (7 days to 19 years), only 14 (4%) patients required reintervention (10 catheter-based procedures, 6 surgical repairs). A postoperative peak velocity of 2.5 m/s or greater was an independent risk factor for reintervention (odds ratio [OR], 4.0; 95% confidence interval [CI], 1.4 to 11.6). Within the cohort, 95 (33%) patients were hypertensive or remained on cardiac medications a median of 12 years (6 months to 19 years) after the surgical procedure. Development of perioperative hypertension was associated with higher risk of chronic hypertension or cardiac medication dependency (OR, 1.9; 95% CI, 1.1 to 3.3). CONCLUSIONS: CoA repair through left thoracotomy is associated with low rates of morbidity, mortality, and reintervention. Aortic arch obstruction should be completely relieved at the time of surgical intervention to minimize the risk of long-term recoarctation.

Swimming of Xenopus laevis sperm exhibits multiple gears and its duration is extended by egg jelly constituents.

The motility of Xenopus sperm is initiated by the osmotic shock experienced when these cells are ejaculated into low-salinity pond water. Motility is brief and is required for the sperm to penetrate the jelly layers and fertilize the egg. In this study we demonstrate that extracts of egg jelly contain factors that extend the period of sperm motility as well as providing a chemoattractant activity as previously reported. Both activities are partially dependent on extracellular calcium. Time-lapse and video microscopy show that after activation of motility the number of motile sperm decreases rapidly, with a half-time of about 2 min. Addition of 10% v/v egg jelly extract ("egg water") increased the number of motile sperm 2-fold over controls at 20 s and about 4- to 10-fold over controls at 10 min after initiation of motility. Extension of motility lifetime was not mediated by a nonspecific protein or by allurin, the egg-water protein that has chemoattractant activity. The helical path of Xenopus sperm exhibited tight coupling between rotational and forward velocities in egg jelly, but coupling changed rapidly from moment to moment in low-salinity buffer. Our observations suggest that jelly-derived factors regulate both the longevity and directionality of sperm propulsion.