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Echocardiographic measurements carry the promise of improving interrater (IR) agreement over subjective assessment. The aim of this study was to assess the effect of implementing a measurement-based protocol on IR agreement and accuracy in reporting of right ventricular (RV) systolic pressure in children. The effect of this reporting protocol on IR agreement in reporting RV dilation, hypertrophy, and systolic function was also evaluated.Five echocardiography readers reported their assessment of RV systolic pressure, dilation, hypertrophy, and systolic function on 40 deidentified echocardiograms using their individual accustomed methods and then using an agreed-upon protocol based solely on RV measurements. IR agreement was assessed using κ statistics. Accuracy of RV systolic pressure ratings was assessed using the McNemar test in comparison with hemodynamic data obtained by cardiac catheterization. The reliability of the RV measurements was assessed using intraclass correlation coefficient (ICC) and coefficient of variation.IR agreement and accuracy of RV systolic pressure assessment improved after using the measurement-based protocol (agreement from 0.39 [95% CI, 0.27-0.5] to 0.62 [95% CI, 0.48-0.76] and accuracy from 18 of 40 to 29 of 40 [P = .03]). IR agreement of RV dilation improved (from 0.36 [95% CI, 0.25-0.48] to 0.63 [95% CI, 0.48-0.79]), while IR agreement of RV hypertrophy (from 0.29 [95% CI, 0.17-0.42] to 0.35 [95% CI, 0.15-0.55]) and RV systolic function (from 0.57 [95% CI, 0.45-0.69] to 0.53 [95% CI, 0.41-0.66]) did not improve. The reliability of the measurements was good (ICC0.8), except for RV free wall thickness (ICC = 0.62, coefficient of variation = 24%) and RV fractional area change (ICC = 0.47, coefficient of variation = 22%), suggesting a possible reason for the lack of improvement in IR agreement of RV hypertrophy and RV systolic function. Heteroscedasticity was observed in the reliability of RV measurements, with the ICC being significantly lower at larger magnitudes for all RV measurements.Standardization of reporting protocols using RV measurements in place of subjective assessment improved IR agreement and accuracy of RV systolic pressure assessment. Reliable measurements (RV systolic pressure and dilation) resulted in improvement in IR agreement while unreliable measurements (RV hypertrophy and systolic function) did not. Special attention to measurements' reliability and heteroscedasticity of reliability is required when designing clinical protocols to decrease IR disagreement as a source of error.

Neonatal Lupus (NLE) is a rare acquired autoimmune disorder caused by transplacental passage of maternal autoantibodies to Sjogren’s Syndrome A or B (SSA-SSB) autoantigens1 which target fetal and neonatal tissues for immune destruction. The cardiac trademark of NLE is autoimmune heart block, which accounts for more than 80% of cases of complete atrioventricular heart-block (AVB) in newborns with a structurally normal heart2. NLE presenting with cardiac alterations not involving rhythm disturbances are described in the literature, but they are rare. Here, we report a case of a neonate with high anti-SSA antibodies who developed severe systemic hypertension and transient ventricular dysfunction in absence of the most common cardiac presentations of NLE: rhythm abnormalities, endocardial fibroelastosis, and dilated cardiomyopathy3. The patient developed severe multiorgan dysfunction syndrome that required prolonged critical care support but fully recovered and was discharged home. We highlight the unusual clinical features of this NLE case and the importance of timely treatment of NLE allowing complete recovery of a critically ill neonate.

There is limited published experience with transitioning pediatric patients from parenteral treprostinil to oral selexipag therapy. In addition, published transitions have typically been protracted, taking several weeks to complete. We present a case series of 3 adolescent patients who were transitioned from parenteral treprostinil to oral selexipag over a 5- to 7-day period. Their clinical courses leading up to the transitions are summarized and their outcomes are described. The 3 patients were successfully rapidly transitioned during an inpatient hospitalization without any observed adverse events or prostacyclin-related side effects. We conclude that when indicated rapid transition of parenteral to oral prostacyclin therapy may be performed safely in adolescents in an inpatient setting.

Rationale Pulmonary hypertension (PH) is relatively common in infants with severe bronchopulmonary dysplasia (BPD), however, hemodynamic data and factors associated with mortality in this patient group are sparsely described in the literature. Objectives To characterize the hemodynamics of former preterm infants with BPD and PH, as measured at cardiac catheterization, and to identify respiratory and cardiovascular predictors of mortality. Methods Single-center, retrospective cohort study, including, 30 patients born at less than 32-week gestational age (GA), who had an oxygen requirement at 36 weeks postmenstrual age and underwent cardiac catheterization between July 2014 and December 2017. Results Median GA at birth was 25 5/7 weeks (interquartile range [IQR], 24 4/7-26 6/7 weeks). Median birth weight was 620 g (IQR, 530-700 g). With a median of 23 months of follow up (IQR, 11-39 months), mortality as of July 2018 was 27% (8 of 30). The alveolar-arterial oxygen gradient as a measure of lung disease did not correlate with mortality (log-rank test P = 0.28). However, indexed pulmonary vascular resistance (PVR) of greater than 3 Woods units × m 2 showed a trend toward increased mortality (log-rank test P = 0.12). Pulmonary vein stenosis was the only predictor significantly associated with mortality (log-rank test P = 0.005). Conclusions In this cohort, the severity of lung disease as assessed by impaired oxygenation at cardiac catheterization did not correlate with mortality. The only factor significantly associated with mortality was the presence of pulmonary vein stenosis on cardiac catheterization, although PVR may also be an important factor.

OBJECTIVE Fetal cerebrovascular resistance is influenced by several factors in the setting of intact autoregulation to allow for normal cerebral blood flow and oxygenation. Maternal hyperoxygenation (MH) allows for acute alterations in fetal physiology and can be a tool to test cerebrovascular reactivity in late-gestation fetuses. In this study, we utilized MH to evaluate cerebrovascular reactivity in fetuses with specific congenital heart disease (CHD). METHODS This was a cross-sectional study of fetuses with complex CHD compared to controls without CHD. CHD cases were grouped according to physiology into: left-sided obstructive lesion (LSOL), right-sided obstructive lesion (RSOL) or dextro-transposition of the great arteries (d-TGA). Subjects underwent MH testing during the third-trimester fetal echocardiogram. The pulsatility index (PI) was calculated for the fetal middle cerebral artery (MCA), umbilical artery (UA) and branch pulmonary artery (PA). The change in PI from baseline to during MH was compared between each CHD group and controls. RESULTS Sixty pregnant women were enrolled (CHD, n = 43; control, n = 17). In the CHD group, there were 27 fetuses with LSOL, seven with RSOL and nine with d-TGA. Mean gestational age was 33.9 (95% CI, 33.6-34.2) weeks. At baseline, MCA-PI Z-score was lowest in the LSOL group (-1.8 (95% CI, -2.4 to -1.2)) compared with the control group (-0.8 (95% CI, -1.3 to -0.3)) (P = 0.01). In response to MH, MCA-PI Z-score increased significantly in the control and d-TGA groups but did not change significantly in the LSOL and RSOL groups. The change in MCA-PI Z-score was significantly higher in the control group than in the LSOL group (0.9 (95% CI, 0.42-1.4) vs 0.12 (95% CI, -0.21 to 0.45); P = 0.03). This difference was more pronounced in the LSOL subgroup with retrograde aortic arch flow. Branch PA-PI decreased significantly in response to MH in all groups, with no difference in the change from baseline to MH between the groups, while UA-PI was unchanged during MH compared with at baseline. CONCLUSIONS The fetal cerebrovascular response to MH varies based on the underlying CHD diagnosis, suggesting that cardiovascular physiology may influence the autoregulatory capacity of the fetal brain. Further studies are needed to determine the clinical implications of these findings on long-term neurodevelopment in these at-risk children. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

* Abbreviations: CVO: : combined ventricular output DVP: : deepest vertical pocket EXIT: : ex utero intrapartum therapy FTC: : Fetal Treatment Center MCA: : middle cerebral artery MRI: : magnetic resonance imaging NTD: : neural tube defect PPROM: : preterm premature rupture of membranes RFA: : radiofrequency ablation SCT: : sacrococcygeal teratoma TFR: : tumor volume–to–fetal weight ratio UCSF: : University of California, San Francisco A large sacrococcygeal teratoma (SCT) was detected on routine 18-week fetal ultrasonography in a 26-year-old, gravida 2, para 0 woman. She had a history of Helicobacter pylori gastric infection and an ovarian cyst, in addition to a previous spontaneous abortion at 15 weeks’ gestation. This pregnancy had been uncomplicated and prenatal laboratory testing was unremarkable. She was evaluated at an outside fetal care center at 21 weeks’ gestation. At that time, the fetal SCT measured 5.9×5.3 cm of mixed solid and cystic components, about 30% to 50% of the fetal size. Given the size of the SCT, with interval growth from initial diagnosis, she was referred to the University of California, San Francisco (UCSF) Fetal Treatment Center (FTC) to discuss the options for fetal surgical intervention. The woman was seen at the UCSF FTC at 23 weeks’ gestation. Imaging at that time included fetal ultrasonography and fetal echocardiography. Ultrasonography (Fig 1) demonstrated a largely exterior SCT with a small, presacral component. The tumor was largely solid with small cystic components and classified as an SCT type 1 lesion. The dimensions were 11.6×10.5×9.6 cm with an estimated volume of 611 mL, about 87% of the fetal size. There was no evidence of polyhydramnios, with the deepest vertical pocket (DVP) at 7.7 cm. Fetal echocardiography showed normal cardiac anatomy with significant dilation of the inferior vena cava and mild cardiomegaly with a cardiothoracic ratio of 0.36. The combined ventricular output (CVO) was significantly elevated at 817 mL/kg per minute (upper limit of normal is 500 mL/kg per minute). Diastolic flow velocity in the umbilical artery (systolic-to-diastolic velocity ratio of 6.2) was low, possibly secondary to a “steal phenomenon” to the tumor. The middle cerebral artery (MCA) diastolic flow velocity was also low, suggestive of cerebral vasodilation to maintain cerebral blood flow (brain-sparing). …

Supplemental material, sj-pdf-1-pul-10.1177_2045894021991446 for Practice patterns of pulmonary hypertension secondary to left heart disease among pediatric pulmonary hypertension providers by Hythem Nawaytou, Jeffrey R. Fineman, Shahin Moledina, Dunbar Ivy, Steven H. Abman and Maria J. Del Cerro in Pulmonary Circulation

To evaluate B-type natriuretic peptide (BNP) as a longitudinal biomarker of clinical outcome in infants with congenital diaphragmatic hernia (CDH).We conducted a retrospective study of 49 infants with CDH, classifying the cohort by respiratory status at 56 days, based on a proposed definition of bronchopulmonary dysplasia for infants ≥32 weeks' gestation: good outcome (alive with no respiratory support) and poor outcome (ongoing respiratory support or death). BNP levels were available at age 1-5 weeks. Longitudinal BNP trends were assessed using mixed-effects modeling. Receiver operating characteristic curves were generated to identify BNP cutoffs maximizing correct outcome classification at each time point. The time to reach BNP cutoff by outcome was assessed using Kaplan-Meier curves for weeks 3-5.Twenty-nine infants (59%) had a poor outcome. Infants with a poor outcome were more likely than those with a good outcome to have liver herniated into the thorax (90% vs 50%; P = .002) and to undergo nonprimary repair (93% vs 35%; P .001). Mixed-effects modeling demonstrated a differing decline in BNP over time by outcome group (P = .003 for interaction). BNP accurately predicted outcome at 3-5 weeks (area under the curve, 0.81-0.82). BNP cutoffs that maximized correct outcome classification decreased over time from 285 pg/mL at 3 weeks to 100 pg/mL at 4 weeks and 48 pg/mL at 5 weeks. Time to reach the cutoffs of 100 pg/mL and 48 pg/mL were longer in the poor outcome group (log-rank P = .006 and .0001, respectively).Elevated BNP accurately predicts poor outcome in infants with CDH at age 3-5 weeks, with declining cutoffs over 3-5 weeks of age.

BackgroundThe clinical utility of echocardiography for the diagnosis of pulmonary vascular disease (PVD) in former preterm infants with bronchopulmonary dysplasia (BPD) is not established. Elevated pulmonary vascular resistance (PVR) rather than pulmonary artery pressure (PAP) is the hallmark of PVD. We evaluated the utility of echocardiography in infants with BPD in diagnosing pulmonary hypertension and PVD (PVR >3 Wood units×m2) assessed by cardiac catheterization.MethodsA retrospective single center study of 29 infants born ≤29weeks of gestational age with BPD who underwent cardiac catheterization and echocardiography was performed. PVD was considered present by echocardiography if the tricuspid valve regurgitation jet peak velocity was >2.9m/sec, post-tricuspid valve shunt systolic flow velocity estimated a right ventricular systolic pressure >35mm Hg, or systolic septal flattening was present. The utility (accuracy, sensitivity, and positive predictive value [PPV]) of echocardiography in the diagnosis of PVD was tested. Subgroup analysis in patients without post-tricuspid valve shunts was performed. Echocardiographic estimations of right ventricular pressure, dimensions, function, and pulmonary flow measurements were evaluated for correlation with PVR.ResultsThe duration between echocardiography and cardiac catheterization was a median of 1day (interquartile range, 1-4days). Accuracy, sensitivity, and PPV of echocardiography in diagnosing PVD were 72%, 90.5%, and 76%, respectively. Accuracy, sensitivity, and PPV increased to 93%, 91.7%, and 100%, respectively, when infants with post-tricuspid valve shunts were excluded. Echocardiography had poor accuracy in estimating the degree of PAP elevation by cardiac catheterization. In infants without post-tricuspid valve shunts, there was moderate to good correlation between indexed PVR and right ventricular myocardial performance index (rho=0.89, P=.005), systolic to diastolic time index (0.84, P&nbsp

Current diagnostic criteria for noncompaction cardiomyopathy (NCC) lack specificity, and the disease lacks prognostic indicators. Reverse apical rotation (RAR) with abnormal rotation of the cardiac apex in the same clockwise direction as the base has been described in adults with NCC. The aim of this study was to test the hypothesis that RAR might differentiate between symptomatic NCC and benign hypertrabeculations and might be associated with ventricular dysfunction.Echocardiograms from 28 children with NCC without cardiac malformations were prospectively compared with those from 29 age-matched normal control subjects. A chart review was performed to identify the patients' histories and clinical characteristics. Speckle-tracking was used to measure longitudinal strain, circumferential strain, and rotation.RAR occurred in 39% of patients with NCC. History of left ventricular (LV) dysfunction or arrhythmia was universal in, but not exclusive to, patients with RAR. Patients with RAR had lower LV longitudinal strain but similar ejection fractions compared with patients without RAR (median, -15.6% [interquartile range, -12.9% to -19.3%] vs -19% [interquartile range, -14.5% to -21.9%], P.01; 53% [interquartile range, 43% to 68%] vs 61% [interquartile range, 58% to 67%], P = .08). Only a pattern of contraction with RAR, early arrest of twisting by mid-systole, and premature untwisting was associated with lower ejection fraction (46%; interquartile range, 43% to 52%; P = .006).RAR is not a sensitive but is a specific indicator of complications in children with NCC. Therefore, RAR may have prognostic rather than diagnostic value. Premature untwisting of the left ventricle during ejection may be an even more worrisome indicator of LV dysfunction.

Background Intraoperative transesophageal echocardiography (iTEE) is used to assess for residual left ventricular outflow tract obstruction (LVOTO) after surgical resection of subaortic membrane causing subaortic stenosis (sub-AS). We aimed to identify the iTEE features associated with recurrence of LVOTO. Methods We conducted a retrospective study of children undergoing sub-AS resection from June 2006 to June 2014. Doppler assessment of the flow velocity and the anatomical features of the left ventricular outflow tract were analyzed from stored echocardiograms. Recurrent LVOTO was defined as an increase in the mean pressure gradient across the left ventricular outflow tract of > 15 mm Hg on the most recent follow-up echocardiogram from the mean pressure gradient on the predischarge echocardiogram or as doubling of the mean pressure gradient to a value ≥20 mm Hg. Results Thirty-five patients were included, with median age at surgery was 8.1 years (range: 0.7-29 years) and median follow-up was 47 months (2-91 months). Ten patients (29%) had recurrent LVOTO, which was associated with a shorter distance between the narrowest diameter of the outflow tract and the aortic valve on iTEE [median 0.59 cm (range 0.39-0.74) vs 0.98 cm (0.75-1.5), P = .03]. No patients with more than mild residual LVOTO on iTEE regressed to mild or no LVOTO on follow-up echocardiograms. Conclusions LVOTO recurrence after sub-AS resection is common, and residual LVOTO remains the same or increases over time. Proximity of the LVOTO to the aortic valve is a risk factor for recurrent LVOTO. These findings may be useful in counseling patients and to guide the frequency of postoperative follow-up.

Graphical abstract, Highlights • Right atrial dissection is a potentially life-threatening complication after ablation. • Hemodynamic instability may occur and can be rapidly progressive. • Agitated saline contrast echocardiography may help diagnose intracardiac dissection.

The current echocardiographic diagnostic criteria for noncompaction cardiomyopathy (NCC) have variable sensitivity and low specificity. Moreover, there are limited data on the use of myocardial deformation imaging for early detection of myocardial dysfunction in children with NCC. We describe left ventricular (LV) deformation patterns in children with NCC, with the goal of identifying a potential diagnostic pattern. We prospectively enrolled 30 children with NCC (47% male; mean age 7.2 years) and 30 age- and gender-matched controls. Extent and severity of non compaction in each segment were evaluated in LV 16-segment model. Regional (base, mid and apex) and segmental (16 segments) longitudinal strain (LS), circumferential strain (CS) and radial strain (RS) were measured using speckle tracking echocardiography. In all patients with NCC, regional and segmental CS and RS at the apex were significantly decreased compared to controls (CS apex: - 19.2 ± 5.4% vs. - 30.2 ± 6.9%, p 0.001/RS apex: 23.5 ± 8.6% vs. 44.1 ± 14.5%, p 0.001). Thirty percent (9/30) of patients had an EF 50%. In these patients, there was additional decrease in CS in basal segments and in LS in basal, mid-cavity and apical segments (CS base: - 16.4 ± 4.7% vs. - 24.6 ± 3.9%, p 0.001/LS (average all LV segments): - 13.9 ± 3.1% vs. - 20.7 ± 4.7%, p 0.001). A cut-off value of CS at the apex of - 24.5% was a strong differentiating feature between patients with NCC and EF 50% and controls (sensitivity: 87%, specificity 79%, AUC 0.88, p 0.001). Children with NCC exhibit a deformation pattern characterized by decreased apical circumferential strain, which may serve as a potential diagnostic tool for NCC. The role of decreased global LV longitudinal and basal circumferential strain should be further evaluated as a potential prognostic tool.

A biomarker is a characteristic that can be used as an indicator of a biological state. A biomarker can be a clinical observation, laboratory test or an imaging parameter. In this review, we discuss the use of biomarkers in differentiating cardiac from noncardiac disease; predicting the prognosis of patients with heart failure, pulmonary hypertension and dilated cardiomyopathy; diagnosing subclinical cardiac involvement in muscular dystrophy and postchemotherapy cancer patients; detecting acute rejection following heart transplantation; diagnosing Kawasaki disease; aiding the management of postoperative cardiac patients; and managing both common (tetralogy of Fallot) and complex (single-ventricle physiology) congenital heart diseases.

Background— Left ventricular (LV) dysfunction after orthotopic heart transplantation (OHT) is multifactorial and can be an indicator of graft rejection or coronary artery vasculopathy. Analysis of rotational mechanics may help in the early diagnosis of ventricular dysfunction. Studies describing the left ventricular rotational strain in children after OHT are lacking. It is important to establish the baseline rotational mechanics in pediatric OHT to pursue further studies in this population. Methods and Results— Rotational strain measured by speckle tracking was compared in 32 children after OHT, with no evidence of active rejection or coronary artery vasculopathy with 35 age-matched normal controls. Twelve OHT patients and 13 controls underwent moderate exercise with pre- and postexercise echocardiography. Torsion, slope of the systolic limb of the torsion–radial displacement loop, and the untwist rate were significantly higher in OHT patients (torsion: median 2.7°/cm [Q1–Q3, 2.3–3.2] versus 2.3°/cm [Q1–Q3, 1.9–2.7]; P =0.03, torsion–radial displacement loop: 2.7°/mm [Q1–Q3, 2.1–3.6] versus 2.0°/mm [Q1–Q3, 1.6–2.7]; P =0.008, indexed peak untwist rate: −21.6°/s/cm [Q1–Q3, −24.3 to −15.7] versus −17.1°/s/cm [Q1–Q3, −19.6 to −13.3]; P =0.01). Contrary to controls, OHT recipients were unable to increase torsion with exercise (OHT: 2.8°/cm [2.7–3.2] versus 3°/cm [2.4–3.5]; P =0.81, controls: 2.2°/cm [2–2.6] versus 3°/cm [2.4–3.7]; P =0.01, pre and post exercise, respectively). The systolic slope of the torsion–radial displacement loop relationship decreased with exercise in most OHT patients. Conclusions— Baseline rotational strain in OHT patients is higher than normal with a blunted response to exercise. The slope of torsion–radial displacement loop, and its response to exercise, may serve as a marker of left ventricular dysfunction in OHT patients.

BackgroundA growing body of evidence indicates that right ventricular dysfunction in patients with palliated hypoplastic left heart syndrome (HLHS) originates in fetal life. In this study, the systolic-to-diastolic time index (SDI) was used to study the presence of ventricular dysfunction in single right ventricles in fetuses with HLHS or evolving HLHS and to assess whether this dysfunction is related to increase preload, myocardial performance, or interventricular interaction.MethodsEchocardiograms from 78 fetuses with HLHS and 10 with evolving HLHS were retrospectively compared with those of 78 normal control fetuses. Fetuses with HLHS were further grouped according to morphology of the left ventricle (LV): not visible (n=35) or visible (n=43). Spectral Doppler signals obtained from right ventricular inflow (blood pool) and tissue Doppler from the tricuspid lateral annulus were analyzed. The SDI was calculated as the ratio of the ejection time plus isovolumic contraction and relaxation times to the diastolic filling time. E/A and E/e' ratios, cardiac output, preload index, and Tei index were also calculated.ResultsFetuses with HLHS demonstrated significantly elevated right ventricular SDI values by both blood pool Doppler and Doppler tissue imaging compared with control subjects (1.89±0.33 vs 1.58±0.29 [P&nbsp