Evaluation of cardiac function in the recipient twin in successfully treated twin-to-twin transfusion syndrome using a novel fetal speckle-tracking analysis.

INTRODUCTION: This study was designed to evaluate ventricular size, shape, and function in recipient twins following laser therapy for twin-twin transfusion syndrome (TTTS), using novel speckle-tracking techniques. METHODS: This retrospective study enrolled patients that underwent fetal laser surgery for TTTS and had fetal echocardiograms (FE) performed pre- and post-operatively (op), with adequate resolution in the 4-chamber view for analysis, using a speckle-tracking software, to compute the size, shape, and function of both the right (RV) and left (LV) ventricles. Values were indexed to published normal values. Pre- and post-laser Z-score values for each of the measurements were compared using the Student's t-test, with significance defined as P < 0.05. RESULTS: Fifteen TTTS candidate pregnancies that underwent laser therapy between 2010 and 2017, with adequate pre- and post-op FE, were selected for the analysis. Post-op FE at 28.5 ± 8.3 days showed a significant decrease in RV base dimension, increased LV base dimension, and improvements in many functional measurements: LV global and free wall strain, LV fractional area change, LV basal-apical fractional change, and LV and RV 24-segment fractional shortening (FS) of the basal segments. CONCLUSIONS: Cardiac remodeling, following laser surgery in TTTS recipient twins, was demonstrated in the basal portion of both the RV and LV with improved biventricular function.

The Fetal 3-Vessel Views: An Illustrative Case-Based Tutorial.

In 2018, the American Institute of Ultrasound in Medicine revised its obstetric Practice Parameter for the second-trimester fetal anatomic survey. The 2018 Practice Parameter recommends incorporation of the 3-vessel view and 3-vessel and trachea view "if technically feasible." Sonographers and other medical providers may require additional training and education to develop greater proficiency in obtaining and interpreting these views. This pictorial essay, including ultrasound images alongside their respective schematic diagrams, provides an up-to-date, practical, and clinically oriented review of the 3-vessel view and 3-vessel and trachea view and their most common presentations in the context of congenital heart disease.

Handshake-free zone in a neonatal intensive care unit: Initial feasibility study.

BACKGROUND: The handshake represents a social custom with special importance in health care settings. However, handshakes can transmit disease and compliance with hand hygiene protocols averages <50%. We hypothesized that a handshake-free zone (HFZ) could be established within our neonatal intensive care unit (NICU) and would be well-received by patient families and their health care providers (HCPs). METHODS: We established an HFZ and conducted a prospective cohort study in the NICU at 2 UCLA Medical Centers. Data collection tools included questionnaires for NICU families and their HCPs. RESULTS: Handshake greetings occurred more frequently before than during the HFZ, as reported by HCPs (P = .0002) and patient families (P = .05). Before the HFZ, physicians were more likely than nurses to shake hands with patient families (P = .001), and believe the handshake was extremely important (P = .002); during the HFZ physicians' behaviors and attitudes shifted toward those of the nurses. All patient families and 66% of HCPs believed the NICU should consider establishing an HFZ. CONCLUSIONS: The HFZ decreased the frequency of handshakes within the NICU. The influence of the HFZ on HCP behavior and attitudes varied with gender and profession. Patient families and most HCPs supported the implementation of an HFZ.

Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association.

BACKGROUND: The goal of this statement is to review available literature and to put forth a scientific statement on the current practice of fetal cardiac medicine, including the diagnosis and management of fetal cardiovascular disease. METHODS AND RESULTS: A writing group appointed by the American Heart Association reviewed the available literature pertaining to topics relevant to fetal cardiac medicine, including the diagnosis of congenital heart disease and arrhythmias, assessment of cardiac function and the cardiovascular system, and available treatment options. The American College of Cardiology/American Heart Association classification of recommendations and level of evidence for practice guidelines were applied to the current practice of fetal cardiac medicine. Recommendations relating to the specifics of fetal diagnosis, including the timing of referral for study, indications for referral, and experience suggested for performance and interpretation of studies, are presented. The components of a fetal echocardiogram are described in detail, including descriptions of the assessment of cardiac anatomy, cardiac function, and rhythm. Complementary modalities for fetal cardiac assessment are reviewed, including the use of advanced ultrasound techniques, fetal magnetic resonance imaging, and fetal magnetocardiography and electrocardiography for rhythm assessment. Models for parental counseling and a discussion of parental stress and depression assessments are reviewed. Available fetal therapies, including medical management for arrhythmias or heart failure and closed or open intervention for diseases affecting the cardiovascular system such as twin-twin transfusion syndrome, lung masses, and vascular tumors, are highlighted. Catheter-based intervention strategies to prevent the progression of disease in utero are also discussed. Recommendations for delivery planning strategies for fetuses with congenital heart disease including models based on classification of disease severity and delivery room treatment will be highlighted. Outcome assessment is reviewed to show the benefit of prenatal diagnosis and management as they affect outcome for babies with congenital heart disease. CONCLUSIONS: Fetal cardiac medicine has evolved considerably over the past 2 decades, predominantly in response to advances in imaging technology and innovations in therapies. The diagnosis of cardiac disease in the fetus is mostly made with ultrasound; however, new technologies, including 3- and 4-dimensional echocardiography, magnetic resonance imaging, and fetal electrocardiography and magnetocardiography, are available. Medical and interventional treatments for select diseases and strategies for delivery room care enable stabilization of high-risk fetuses and contribute to improved outcomes. This statement highlights what is currently known and recommended on the basis of evidence and experience in the rapidly advancing and highly specialized field of fetal cardiac care.

Improved prenatal detection of congenital heart disease in an integrated health care system.

The reported prenatal detection rates (PDRs) for significant congenital heart disease (sCHD) have been suboptimal, even in the current era. Changes in prenatal ultrasound policy and training may lead to improved prenatal detection of sCHD. This study analyzed the results of a policy to assess fetal cardiac outflow tracts shown by screening prenatal ultrasound using the electronic medical record (EMR). During a 6-year period, fetuses and patients younger than 1 year with sCHD were identified. The EMR was used to gather detection and outcome data. As an internal control within the same health care system, the PDR of only the surgical cases was compared with that of a similar group in which documentation of the fetal cardiac outflow tracts was not standard policy. Among 25,666 births, sCHD was identified in 93 fetuses or patients, yielding an incidence of 3.6 per 1,000 births. The PDR was 74.1%. Detection after birth but before discharge was 20.4%, and detection after discharge was 5.4%. A significant improvement in the PDR of sCHD was found when a concerted effort was made to obtain fetal cardiac outflow tract views during pregnancy screening (59.3 vs. 28%). Within an integrated health care system and with the use of an EMR, a PDR of 74% can be obtained, and 94% of sCHD can be detected before discharge. A concerted program that includes documentation of fetal cardiac outflow tracts in the pregnancy screening can result in improved PDR of sCHD.

Prenatal diagnosis of congenital heart disease: impact of mode of delivery on neonatal outcome.

OBJECTIVE: We sought to evaluate the impact of mode of delivery (MOD) on early outcome for neonates diagnosed prenatally with major forms of congenital heart disease (CHD). METHODS: We retrospectively studied infants admitted, over a 2-year period, to a single institution for cardiac intervention. Infants were grouped on the basis of timing of diagnosis (prenatal/postnatal) and MOD--planned (induced labor or planned cesarean delivery) versus spontaneous labor. Multivariate logistic regression was used to evaluate independent predictors for MOD and early outcomes. RESULTS: Of 329 patients, 45% received a prenatal diagnosis of CHD. A prenatal diagnosis of CHD increased the likelihood for planned delivery [odds ratio (OR) 2.6, 95% confidence interval (CI) 1.6 to 4.5, p < 0.001]. Newborns prenatally diagnosed with CHD were more likely to have been delivered between 8 am and 6 pm, Monday through Friday (OR 2.3, 95% CI 1.1 to 4.8, p = 0.019). However, MOD had no statistical impact on Apgar score, duration of pre-operative intubation, and survival to surgery or to discharge. The Risk Adjustment for Congenital Heart Surgery 1 surgical mortality score was the only independent predictor of hospital mortality. CONCLUSIONS: In our experience, although a prenatal diagnosis of CHD decreased the likelihood of spontaneous labor, MOD had no demonstrable impact on neonatal outcome.

Color M-mode sonography for evaluation of fetal arrhythmias.

Fetal arrhythmias can be challenging to diagnose, even with the use of 2-dimensional, M-mode, and spectral Doppler sonography of myocardial or blood flow signals to determine the rate, synchrony, and timing. Color Doppler sonography combined with M-mode echocardiography uses the myocardium and blood flow to provide a robust evaluation of cardiac rhythm. Limited descriptions of color M-mode sonography have been published. This article describes the systematic application of the color M-mode technique using 4 specific clinical case examples and contrasts this technique with more conventional approaches to fetal arrhythmia diagnosis.

Hypoplastic left heart syndrome in patients with Kabuki syndrome.

The association of cardiac defects with Kabuki syndrome has been well described. The majority of these defects are isolated shunt lesions, conotruncal abnormalities, or various forms of arch obstruction. This report describes a series of three patients with hypoplastic left heart syndrome and Kabuki syndrome. The series illustrates the full spectrum of left-sided obstructive lesions and expands the phenotype of cardiac defects associated with Kabuki syndrome.

Prenatal screening for major congenital heart disease: superiority of outflow tracts over the 4-chamber view.

OBJECTIVE: The purpose of this study was to determine the relative importance of the 4-chamber view (4CV) compared with the outflow tract views (OFTVs) in prenatal screening for major congenital heart disease (CHD). METHODS: We prospectively evaluated 200 consecutive infants undergoing cardiac surgery at our institution for major CHD. By reviewing the infants' medical records and conducting bedside interviews with their parents or guardians, we evaluated detection rates both prenatally and postnatally (before and after discharge to home), and we noted any prenatally identifiable risk factors for CHD. For each infant, we determined whether the 4CV or OFTVs would be expected to have been normal or abnormal on routine midgestation screening fetal sonography. RESULTS: A prenatal diagnosis of CHD was made in 65 infants (33%): 30 of 124 low-risk pregnancies (24%) and 35 of 76 high-risk pregnancies (46%). An abnormal screening midgestation 4CV would have been expected in up to 63% of the infants, whereas abnormal midgestation OFTVs would have been expected in up to 91% of the infants. Thus, the potential sensitivity for detecting major CHD was higher with the OFTVs than with the 4CV (91% versus 63%; P < .001). Moreover, the OFTVs were more sensitive than the 4CV for detecting ductal-dependent forms of CHD. Diagnosis after discharge to home occurred in 39 of 135 postnatal diagnoses (29%), including many cases of isolated outflow tract abnormalities requiring early invasive intervention. CONCLUSIONS: Cases of major neonatal CHD with OFTV abnormalities predominate over cases with 4CV abnormalities, particularly among those forms of CHD requiring early invasive intervention.

Pregnancy: maternal and fetal heart disease.

Cardiac disorders complicate less than 1% of all pregnancies. Physiologic changes in pregnancy may mimic heart disease. In order to differentiate these adaptations from pathologic conditions, an in-depth knowledge of cardiovascular physiology is mandatory. A comprehensive history, physical examination, electrocardiogram, chest radiograph, and echocardiogram are sufficient in most cases to confirm the diagnosis. Care of women with cardiac disease begins with preconception counseling. Severe lesions should be taken care of prior to contemplating pregnancy. Management principles for pregnant women are similar to those for the non-pregnant state. A team approach comprised of a maternal fetal medicine specialist, cardiologist, neonatologist, and anesthesiologist is essential to assure optimal outcome for both the mother and the fetus. Although fetal heart disease complicates only a small percentage of pregnancies, congenital heart disease causes more neonatal morbidity and mortality than any other congenital malformation. Unfortunately, screening approaches for fetal heart disease continue to miss a large percentage of cases. This weakness in fetal screening has important clinical implications, because the prenatal detection and diagnosis of congenital heart disease may improve the outcome for many of these fetal patients. In fact, simply the detection of major heart disease prenatally can improve neonatal outcome by avoiding discharge to home of neonates with ductal-dependent congenital heart disease. Fortunately, recent advances in screening techniques, an increased ability to change the prenatal natural history of many forms of fetal heart disease, and an increasing recognition of the importance of a multidisciplinary, team approach to the management of pregnancies complicated with fetal heart disease, together promise to improve the outcome of the fetus with congenital heart disease.

Echocardiographic predictors of outcome in fetuses with pulmonary atresia with intact ventricular septum.

OBJECTIVE: We sought to identify in utero predictors of postnatal outcomes in fetal patients with pulmonary atresia with intact ventricular septum (PAIVS) or critical pulmonary stenosis. BACKGROUND: Although PAIVS or critical pulmonary stenosis can be diagnosed in utero by echocardiography, our ability to predict outcomes is limited. METHODS: Fetal echocardiograms from 28 patients with PAIVS/critical pulmonary stenosis were retrospectively reviewed. Tricuspid valve (TV) annulus, right and left ventricular internal dimensions, and degree of tricuspid regurgitation were recorded. To establish normal fetal values, echocardiograms from healthy patients were analyzed in an identical fashion. RESULTS: Both a fetal TV z score of -4 or less beyond 23 weeks of gestation and a fetal TV annulus of 5 mm or less beyond 30 weeks of gestation were predictive of poor postnatal outcomes. In addition, right:left ventricular length or width less than 0.5 and/or the absence of tricuspid regurgitation were predictive of poor outcome. CONCLUSIONS: TV annulus size, right:left ventricular ratios, and presence of tricuspid regurgitation on fetal echocardiograms may aid in guiding prenatal counseling regarding postnatal outcome in PAIVS.