Evolution and Impact of a Diagnostic Point-of-Care Ultrasound Program in a PICU.

OBJECTIVES: To evaluate the impact of point-of-care ultrasound (POCUS) use on clinicians within a PICU and to assess infrastructural elements of our POCUS program development. DESIGN: Retrospective observational study. SETTING: Large academic, noncardiac PICU in the United States. SUBJECTS: Patients in a PICU who had diagnostic POCUS performed. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Between January 1, 2017, and December 31, 2022, 7201 diagnostic POCUS studies were ordered; 1930 (26.8%) had a quality assurance (QA) record generated in an independent POCUS QA database. The cardiac domain was most frequently imaged (81.0% of ordered studies, 81.2% of reviewed studies). POCUS images changed clinician understanding of pathophysiology in 563 of 1930 cases (29.2%); when this occurred, management was changed in 318 of 563 cases (56.5%). Cardiac POCUS studies altered clinician suspected pathophysiology in 30.1% of cases (472/1568), compared with 21.5% (91/362) in noncardiac studies (p = 0.06). Among cases where POCUS changed clinician understanding, management changed more often following cardiac than noncardiac POCUS (p = 0.02). Clinicians identified a need for cardiology consultation or complete echocardiograms in 294 of 1568 cardiac POCUS studies (18.8%). Orders for POCUS imaging increased by 94.9%, and revenue increased by 159.4%, from initial to final study year. QA database use by both clinicians and reviewers decreased annually as QA processes evolved in the setting of technologic growth and unit expansion. CONCLUSIONS: Diagnostic POCUS imaging in the PICU frequently yields information that alters diagnosis and changes management. As PICU POCUS use increased, QA processes evolved resulting in decreased use of our initial QA database. Modifications to QA processes are likely necessary as clinical contexts change over time.

Left atrial stiffness and strain are novel indices of left ventricular diastolic function in children: validation followed by application in multisystem inflammatory syndrome in children due to COVID-19.

AIMS: We hypothesized left atrial (LA) stiffness may serve as a surrogate marker in children to differentiate elevated pulmonary capillary wedge pressure (PCWP) from normal and help detect diastolic dysfunction in myocardial injury due to multisystem inflammatory syndrome in children (MIS-C). METHODS AND RESULTS: We validated LA stiffness in 76 patients (median age 10.5 years), 33 had normal PCWP (<12 mmHg) and 43 had elevated PCWP (≥12 mmHg). LA stiffness was applied to 42 MIS-C patients [28 with myocardial injury (+) and 14 without myocardial injury (-)], defined by serum biomarkers. The validation group consisted of a group with and without cardiomyopathies, whose PCWP values ranged from normal to severely elevated. Peak LA strain was measured by speckle-tracking and E/e' from apical four chamber views. Noninvasive LA stiffness was calculated as: LAStiffness=E/e'LAPeakStrain (%-1). Patients with elevated PCWP showed significantly elevated LA stiffness [median 0.71%-1 vs. 0.17%-1, P < 0.001]. Elevated PCWP group showed significantly decreased LA strain (median: 15.0% vs. 38.2%, P < 0.001). Receiver operator characteristic (ROC) curve for LA stiffness yielded an area under the curve (AUC) of 0.88 and cutoff value of 0.27%-1. In MIS-C group, ROC curve yielded an AUC of 0.79 and cutoff value of 0.29%-1 for identifying myocardial injury. CONCLUSION: In children with elevated PCWP, LA stiffness was significantly increased. When applied to children with MIS-C, LA stiffness classified myocardial injury accurately. LA stiffness and strain may serve as noninvasive markers of diastolic function in the pediatric population.

Novel Utilization of Ultrasound Enhancing Agents in Complex Congenital Heart Disease Following Superior Cavopulmonary Connection.

BACKGROUND: Children with single-ventricle congenital heart disease typically undergo a superior cavopulmonary connection (SCPC) as the second stage in their surgical palliation. Postoperatively, stenoses of the SCPC and branch pulmonary arteries can occur. If there are clinical concerns and echocardiography is insufficient for diagnosis, patients undergo invasive evaluation with exposure to radiation and anesthesia. The use of ultrasound enhancing agents (UEAs) to improve echocardiographic diagnostic capabilities has not previously been described in this population. METHODS: A single-center, retrospective case review was conducted of children who underwent echocardiography with UEA, following SCPC, from March 1, 2020, to April 15, 2022, at the Children's Hospital of Philadelphia. Twenty-two patients with hypoxemia or concern for obstruction following SCPC underwent UEA echocardiography. Extracted clinical data included patient demographics, echocardiographic images, angiography, surgical and transcatheter interventions, as well as available follow-up data. RESULTS: Six of the 22 UEA echocardiograms demonstrated stenosis or occlusion of either the SCPC or a pulmonary artery. All six patients underwent cardiac catheterization and five required intervention. Angioplasty was performed in each case with one requiring subsequent surgical revision. Sixteen of 22 UEA echocardiograms demonstrated no evidence of stenosis. Ten of these 16 patients improved, while six experienced persistent hypoxemia prompting referral for cardiac catheterization. Angiography confirmed the UEA echocardiographic findings (absence of stenosis) in four of these six patients. There were no adverse reactions related to UEA administration. CONCLUSIONS: Echocardiography with UEAs is a valuable and safe adjunctive imaging modality following SCPC, particularly when two-dimensional and color imaging is limited. This novel application of UEAs in complex patients with congenital heart disease provides an "angiogram-like" image, better delineating vessel walls and improving assessment of postoperative obstruction. As experience with UEAs increases in the congenital heart disease population, there may be opportunities to decrease invasive and costly procedures, while expediting the care of patients in need of intervention.

Longitudinal Assessment of Cardiac Outcomes of Multisystem Inflammatory Syndrome in Children Associated With COVID-19 Infections.

Background In multisystem inflammatory syndrome in children, there is paucity of longitudinal data on cardiac outcomes. We analyzed cardiac outcomes 3 to 4 months after initial presentation using echocardiography and cardiac magnetic resonance imaging. Methods and Results We included 60 controls and 60 cases of multisystem inflammatory syndrome in children. Conventional echocardiograms and deformation parameters were analyzed at 4 time points: (1) acute phase (n=60), (2) subacute phase (n=50; median, 3 days after initial echocardiography), (3) 1-month follow-up (n=39; median, 22 days), and (4) 3- to 4-month follow-up (n=25; median, 91 days). Fourteen consecutive cardiac magnetic resonance imaging studies were reviewed for myocardial edema or fibrosis during subacute (n=5) and follow-up (n=9) stages. In acute phase, myocardial injury was defined as troponin-I level ≥0.09 ng/mL (>3 times normal) or brain-type natriuretic peptide >800 pg/mL. All deformation parameters, including left ventricular global longitudinal strain, peak left atrial strain, longitudinal early diastolic strain rate, and right ventricular free wall strain, recovered quickly within the first week, followed by continued improvement and complete normalization by 3 months. Median time to normalization of both global longitudinal strain and left atrial strain was 6 days (95% CI, 3-9 days). Myocardial injury at presentation (70% of multisystem inflammatory syndrome in children cases) did not affect short-term outcomes. Four patients (7%) had small coronary aneurysms at presentation, all of which resolved. Only 1 of 9 patients had residual edema but no fibrosis by cardiac magnetic resonance imaging. Conclusions Our short-term study suggests that functional recovery and coronary outcomes are good in multisystem inflammatory syndrome in children. Use of sensitive deformation parameters provides further reassurance that there is no persistent subclinical dysfunction after 3 months.

Contrast-enhanced ultrasound in pediatric echocardiography.

The safety and benefits of cardiac contrast-enhanced ultrasound (CEUS) have been demonstrated in children and adolescents for a variety of clinical indications, including congenital heart disease. Cardiac CEUS is performed with US and the intravenous administration of ultrasound contrast agents (UCAs). It improves transthoracic echocardiography, which can be challenging in children and adults with acoustic window limitations (e.g., from obesity) and alterations in chest wall and cardiac geometry (e.g., from prior surgical procedures). Cardiac CEUS is also used to evaluate ischemia in the follow-up of congenital and acquired heart disease. In 2019, the United States Food and Drug Administration (FDA) approved a UCA for pediatric echocardiography. This article focuses on the clinical applications of UCAs in pediatric and adult echocardiography, outlining its diagnostic value, safety and potential for future applications.

Point-of-care ultrasound for the evaluation of venous cannula position in neonatal extracorporeal membrane oxygenation.

OBJECTIVE: To assess the ability of point-of-care ultrasound (POCUS) to identify venous cannula position in neonates on extracorporeal membrane oxygenation (ECMO) and compare with conventional imaging. STUDY DESIGN: Retrospective review of 37 infants on ECMO with 51 POCUS studies between January 2017 and October 2019. Studies were reviewed for identification of venous cannula location and compared with plain radiography and echocardiography. Kappa statistic and predictive values were calculated. RESULTS: Venous cannula tip position was identified in 90% of POCUS studies. Fifty percent of the cannula tips were malpositioned. Plain radiography, the most commonly used method for evaluating tip position, showed poor agreement (57%) with POCUS (kappa 0.13). There was substantial agreement (89%) between echocardiography and POCUS (kappa 0.78). CONCLUSION: This study provides preliminary evidence that POCUS is more accurate than plain radiography for the evaluation of ECMO venous cannula position. Adoption of this practice may prevent potentially catastrophic ECMO complications.

Genotype-phenotype association by echocardiography offers incremental value in patients with Noonan Syndrome with Multiple Lentigines.

BACKGROUND: Noonan Syndrome with Multiple Lentigines (NSML) and Noonan Syndrome (NS) can be difficult to differentiate clinically in early childhood. This study aims to describe characteristics of the ventricular septum that may differentiate NSML from NS. We hypothesize that the shape of the ventricular septum determined by echocardiography correlates with genotype and may distinguish patients with NSML from those with NS. METHODS: We analyzed data from 17 NSML and 67 NS patients. Forty normal and 30 sarcomeric hypertrophic cardiomyopathy (HCM) patients were included as controls. Septal morphology was qualitatively evaluated, and septal angle was measured quantitatively at end diastole. We recorded the presence of a ventricular septal bulge (VSB) and reviewed genetic testing results for each patient. RESULTS: The most important findings were a sigmoid septum (71%) and VSB (71%) in NSML. NSML septal angle was decreased compared to the normal and sarcomeric HCM control groups, respectively (149 ± 13 vs. 177 ± 3, p < 0.001; 149 ± 13 vs. 172 ± 7, p < 0.001). NS septal angle was similar to the controls (176 ± 6 vs. 177 ± 3, p > 0.5; 176 ± 6 vs. 172 ± 7, p > 0.5). NSML-linked pathogenic variants were associated with sigmoid septum and VSB. CONCLUSIONS: These findings provide novel phenotypic evidence to clinicians that may offer incremental diagnostic value in counseling families in ambiguous NSML/NS cases. IMPACT: Characteristics of the ventricular septum are linked to specific gene variants that cause NSML and NS. Sigmoid septum and VSB are associated with NSML. This novel echocardiographic association may help clinicians distinguish NSML from NS in ambiguous cases. Early distinction between the two may be important, as syndrome-specific therapies may become available in the near future. This study may encourage further research into genotype-phenotype associations in other forms of HCM.

Socioeconomic barriers to prenatal diagnosis of critical congenital heart disease.

OBJECTIVE: The study was designed to assess the impact of socioeconomic barriers on the rate of prenatal diagnosis of critical congenital heart disease (CCHD). METHODS: This was a retrospective review of the Medicaid analytic extract (MAX) dataset, a national Medicaid administrative claims database with linked maternal-infant claims, from 2007 to 2012. Infants with CCHD were identified by searching for International Classification of Diseases (ICD) 9 codes and Procedural Coding System (PCS) codes for CCHD within the first 6 months after the delivery date. Multivariate logistic regression was used to evaluate the effect of maternal and socioeconomic factors on the prenatal diagnosis rate. RESULTS: There were 4702 mother-infant dyads included in the analysis. The prenatal diagnosis rate of CCHD was 27.9%. Factors independently associated with odds of prenatal diagnosis of CCHD were presence of maternal diabetes (OR, 2.055; P < .001), ZIP code level median household income (OR, 1.005; P = .015), sonographer labor quotient (OR, 1.804; P = .047), the year of the delivery (OR, 1.155; P < .001), and needing a view other than a 4 chamber or outflow tract view to obtain the diagnosis (OR, 0.383; P < .001). CONCLUSION: Maternal health, diabetes, socioeconomic factors, and access to sonographers impacts prenatal diagnosis of CCHD.

Echocardiographic Findings in Pediatric Multisystem Inflammatory Syndrome Associated With COVID-19 in the United States.

BACKGROUND: Centers from Europe and United States have reported an exceedingly high number of children with a severe inflammatory syndrome in the setting of coronavirus disease 2019, which has been termed multisystem inflammatory syndrome in children (MIS-C). OBJECTIVES: This study aimed to analyze echocardiographic manifestations in MIS-C. METHODS: A total of 28 MIS-C, 20 healthy control subjects and 20 classic Kawasaki disease (KD) patients were retrospectively reviewed. The study reviewed echocardiographic parameters in the acute phase of the MIS-C and KD groups, and during the subacute period in the MIS-C group (interval 5.2 ± 3 days). RESULTS: Only 1 case in the MIS-C group (4%) manifested coronary artery dilatation (z score = 3.15) in the acute phase, showing resolution during early follow-up. Left ventricular (LV) systolic and diastolic function measured by deformation parameters were worse in patients with MIS-C compared with KD. Moreover, MIS-C patients with myocardial injury were more affected than those without myocardial injury with respect to all functional parameters. The strongest parameters to predict myocardial injury in MIS-C were global longitudinal strain, global circumferential strain, peak left atrial strain, and peak longitudinal strain of right ventricular free wall (odds ratios: 1.45 [95% confidence interval (CI): 1.08 to 1.95], 1.39 [95% CI: 1.04 to 1.88], 0.84 [95% CI: 0.73 to 0.96], and 1.59 [95% CI: 1.09 to 2.34], respectively). The preserved LV ejection fraction (EF) group in MIS-C showed diastolic dysfunction. During the subacute period, LVEF returned to normal (median from 54% to 64%; p < 0.001) but diastolic dysfunction persisted. CONCLUSIONS: Unlike classic KD, coronary arteries may be spared in early MIS-C; however, myocardial injury is common. Even preserved EF patients showed subtle changes in myocardial deformation, suggesting subclinical myocardial injury. During an abbreviated follow-up, there was good recovery of systolic function but persistence of diastolic dysfunction and no coronary aneurysms.

Longitudinal changes in echocardiographic measures of ventricular function after Fontan operation.

BACKGROUND: While numerous indices have been developed in an attempt to quantify ventricular function in patients with single ventricle heart disease after Fontan, there are little data on how these parameters change over time. METHODS: A retrospective observational study was performed of individuals who underwent Fontan operation at Children's Hospital of Philadelphia (CHOP) in 2006 and 2007. Measurements of fractional area change (FAC), tricuspid annular planar systolic excursion (TAPSE), myocardial performance index (MPI), systolic to diastolic (s/d) ratio, and myocardial strain and strain rate were made offline. A composite outcome of protein-losing enteropathy (PLE), plastic bronchitis, transplant, or death was created, and change in function was compared between those who did and those who did not meet the outcome. RESULTS: There were 312 echocardiograms from 40 unique patients (75% male, 55% dominant right ventricle). The aggregate mean values for most assessed parameters were worse than what would be expected for a healthy age-matched population. The global longitudinal strain rate increased (worsened) by 0.014 (1/s) per year (P = .02), and the global circumferential strain rate increased (worsened) by 0.011 (1/s) per year (P = .01). There was no difference in the rate of change of ventricular function in the 6 patients who met the composite endpoint vs those who did not. CONCLUSION: This study demonstrates that global longitudinal strain rate and global circumferential strain rate decrease over 10 years following Fontan operation. These measures of ventricular performance may be early signs of cardiac dysfunction that predate more obvious echocardiographic signs of deterioration.

Right Ventricular Strain, Brain Natriuretic Peptide, and Mortality in Congenital Diaphragmatic Hernia.

Rationale: Brain-type natriuretic peptide (BNP) correlates with pulmonary hypertension as demonstrated by echocardiogram in congenital diaphragmatic hernia (CDH); however, its association with right ventricular (RV) function and mortality is unknown.Objectives: To characterize the relationships between echocardiogram-derived RV strain, BNP, and mortality in diaphragmatic hernia.Methods: We performed a single-center retrospective cohort study of infants with CDH and at least one BNP-echocardiogram pair within a 24-hour period. RV global longitudinal strain (GLS) and free-wall strain (FWS) were measured on existing echocardiograms. Associations among strain, BNP, and mortality were tested using mixed-effect linear and logistic regression models. Survival analysis was stratified by BNP and strain abnormalities.Results: There were 220 infants with 460 BNP-echocardiogram pairs obtained preoperatively (n = 237), ≤1 week postoperatively (n = 35), and >1 week postoperatively ("recovery"; n = 188). Strain improved after repair (P < 0.0001 for all periods). Higher BNP level was associated with worse strain in recovery but not before or immediately after operation (estimate [95% confidence interval] for recovery: GLS, 1.03 [0.50-1.57]; P = 0.0003; FWS, 0.62 [0.01-1.22]; P = 0.047). BNP and strain abnormalities were associated with an extracorporeal-membrane oxygenation requirement. Higher BNP level in recovery was associated with greater mortality (odds ratio, 11.2 [1.2-571.3]; P = 0.02). Abnormal strain in recovery had high sensitivity for detection of mortality (100% for GLS; 100% for FWS) but had low specificity for detection of mortality (28% for GLS; 48% for FWS).Conclusions: Persistent RV dysfunction after CDH repair may be detected by a high BNP level and abnormal RV strain.

Three-Dimensional Modeling of Surgically Implanted Stent-Based Valves in the Mitral Position in Children.

PURPOSE: In children with a mitral annulus too small to accommodate traditional prostheses, surgical implantation of stent-based valves is a promising option. However no reliable preoperative methods exist to guide patient selection, device sizing, and positioning. We describe a novel methodology to visualize and quantify device fit in 3-dimensional echocardiogram (3DE)-derived heart models. DESCRIPTION: Heart models were created from existing preoperative 3DEs using custom software. Valve models were virtually implanted into the models, and both device fit and left ventricular outflow tract (LVOT) area were quantified. EVALUATION: The 3DEs of 3 patients who underwent Melody valve placement in the mitral position were retrospectively modeled: 1 with LVOT obstruction, 1 with perivalvar leak, and 1 without complications. In all cases 2-dimensional measurements underestimated 3D annular dimensions, and the patient with clinical LVOT obstruction had the lowest predicted LVOT area-to-aortic area ratio (0.5). CONCLUSIONS: 3DE-based preoperative modeling of surgical implantation of stent-based valves in the mitral position may improve quantification of mitral valve dimensions and inform risk stratification for potential LVOT obstruction.

Prenatal Diagnosis Influences Preoperative Status in Neonates with Congenital Heart Disease: An Analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.

The early postnatal course for a newborn with critical congenital heart disease (CHD) can be negatively impacted if diagnosis is delayed. Despite this, there continues to be inconsistent evidence regarding potential benefits associated with prenatal diagnosis (PND) in neonates who undergo cardiac surgery. The objective of this study was to better define the impact of a PND on pre-operative morbidity by utilizing a large clinical database. Neonates (< 30 days) undergoing heart surgery from 2010 to 2014 and entered in the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) were included. Multivariable logistic regression was used to evaluate the association between PND and a composite measure including nine major pre-operative risk factors. Co-variates were included to adjust for important patient characteristics (e.g., weight-for-age z-score, genetic syndromes, prematurity), case complexity, and center effects. Centers and patients with excess missing data for relevant co-variates were excluded. Included were 12,899 neonates undergoing surgery at 112 centers. Major pre-operative risk factors were present in 34% overall. By univariate analysis, PND was associated with a lower overall prevalence of major pre-operative risk factors. After adjusting for potential confounders, major pre-operative risk factors were less prevalent among neonates with PND compared to neonates without PND (adjusted OR 0.62, 95% CI 0.57-0.68, p < 0.001). A sensitivity analysis excluding neonates with genetic syndromes, non-cardiac anatomic abnormalities, and prematurity demonstrated similar findings (adjusted OR 0.55, 95% CI 0.49-0.61, p < 0.0001). Among neonates with CHD, prenatal diagnosis is associated with significantly lower rates of pre-operative risk factors for cardiac surgery. Further studies are needed to define association of these pre-operative benefits of a PND with longer term clinical outcomes.

The Congenital Heart Surgeon's Society Complete Atrioventricular Septal Defect Cohort: Baseline, Preintervention Echocardiographic Characteristics.

Quantifying unbalance, the threshold for single ventricle palliation vs biventricular repair in patients with unbalanced complete atrioventricular septal defect (AVSD), is challenging. Using a core laboratory review of baseline echocardiograms, we sought to assess the correlations among commonly used measures of unbalance and common atrioventricular valve (AVV) and ventricular sizes. A single reviewer evaluated baseline echocardiograms from an inception cohort of babies age < 1 year with complete AVSD admitted to 1 of 25 Congenital Heart Surgeon's Society institutions. A standardized echo review protocol of 111 quantitative and qualitative measures was used. Descriptive statistics were computed and Pearson correlation coefficients were calculated to assess correlation among unbalance indices with valvar and ventricular dimensions. Two-hundred fifty-seven baseline echocardiograms of infants with complete AVSD were included. Median age at baseline echocardiogram was 11 days (interquartile range 1-79) and mean atrioventricular valve index was 0.45 ± 0.1. Mean right ventricle/left ventricle inflow angle was 90.2 ± 15.6° and median left ventricular inflow index was 0.46 (interquartile range 0.4-0.5). There are weak or moderate correlations between the measures of unbalance. Correlations between the measures of unbalance with common AVV leaflet or ventricular sizes are also weak to moderate, when statistically significant. Measures of unbalance in common clinical use correlate poorly, or not at all, with one another, common AVV, and ventricular dimensions. The concept of "unbalance" is difficult to define using baseline echocardiographic indices. These findings suggest that the indices may describe different morphologic and functional characteristics. Further analysis is necessary to quantify the contributions of unbalance indices to patient outcome.

Medium-Term Outcomes After Implantation of Expanded Polytetrafluoroethylene Valved Conduit.

BACKGROUND: Pulmonary valve replacement is becoming the most frequent congenital heart surgery performed on adolescents and young adults. Numerous surgical options are available, including autologous pericardium, mechanical valves, allografts, and bioprosthetic valves. Each option has limitations with durability and freedom from reintervention for stenosis or insufficiency, particularly in the pediatric population. The purpose of this study was to analyze our uniquely designed, expanded polytetrafluoroethylene (ePTFE) valved conduit (VC) for right ventricular outflow tract reconstruction. METHODS: Beginning in 2012, ePTFE VC were implanted in 26 patients with a median age of 9.8 years (range, 1.0 to 15.9). Bicuspid VC were used in the first 3 patients, and tricuspid VC were used in 23 patients. The ePTFE VC is fashioned from commercially available ePTFE tube graft (16 mm in 1 patient, 20 mm in 7 patients, 24 mm in 18 patients) and 0.1 mm thick ePTFE membrane for the leaflet material. Valve function was assessed by echocardiogram after the implantation. RESULTS: There were no postoperative hospital deaths. Mean follow-up was 2.6 years (range, 4 months to 4.7 years), and there have been no reoperations for the ePTFE VC. There was one reintervention for stenosis at the distal anastomosis. Pulmonary insufficiency was mild or less in 24 patients (92%). The average peak instantaneous pressure gradient between the right ventricle and the pulmonary artery was 22.4 ± 15.1 mm Hg at latest follow-up. CONCLUSIONS: Compared with historical data for other pulmonary valve replacement options, our ePTFE VC shows superior medium-term performance, with less reintervention or significant valve dysfunction.

Counseling Practices for Fetal Hypoplastic Left Heart Syndrome.

While counseling parents of a fetus diagnosed with hypoplastic left heart syndrome (HLHS), pediatric cardiologists play a critical role in shaping a family's expectations for the months and years to come. However, techniques for the most effective counseling practices have not been studied, and significant variation among physicians is likely present. Web-based survey of pediatric cardiologists that perform fetal echocardiography using snowball sampling. 201 physicians responded (61% male, 81% from academic centers, and 95% from the U.S.), with an average experience of 12 years. The majority of respondents (73%) typically received initial referrals for HLHS between 20 and 24 weeks of gestation. Most physicians counsel families alone (54%), while others counsel with a nurse (35%), social worker (12%), and/or maternal-fetal medicine colleague (15%). Termination of pregnancy was discussed by 79% of respondents, although 15% did not know their state's legal limit for termination. While initial counseling sessions routinely described the typical earlier ramifications of HLHS, many long-term sequelae of the disease were not commonly discussed. Content of counseling was affected by region of the country, but not by practice setting, experience, or fetal volume. Respondents identified multiple barriers that limited their counseling practices. Our data suggest that current counseling practices often fail to cover important information. Perceived barriers to a full discourse on long-term sequelae of HLHS are common and may lead to a disconnect between reality and a family's understanding of the natural history of palliated HLHS. Opportunities to improve counseling practices exist, and there may be benefits to gain from more formal training.