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Objective: Image quality of fetal echocardiography (FE) has improved in the recent era, but few recent studies have reported the accuracy of FE, specifically in single ventricle (SV) congenital heart disease (CHD). This study aimed to assess the ability of FE to correctly predict SV‐CHD postnatal anatomy and physiology in a contemporary cohort. Methods: The contemporary clinical reports of patients with SV‐CHD, in which FE was performed between July 2017 and July 2021, were compared with postnatal echocardiograms from a formal quality assurance program. SV fetuses were grouped by anatomical subtype. Diagnostic errors were designated as major if the error would have caused significant alteration in parental counseling or postnatal management. The remaining errors were classified as minor. Physiological discrepancies, including prostaglandin‐E (PGE) dependency, atrioventricular valve regurgitation (AVVR), pulmonary venous obstruction and restrictive atrial septum (RAS), were assessed by chart review of the postnatal course. Results: A total of 119 subjects were analyzed. SV subtypes in the cohort included hypoplastic left heart syndrome (HLHS) (n = 68), tricuspid atresia (n = 16), double‐inlet left ventricle (n = 12), unbalanced atrioventricular canal (UAVC) (n = 11), heterotaxy (n = 9) and other (n = 3). The rate of major anatomical and physiological errors was low (n = 6 (5.0%)). A higher proportion of minor errors was noted in HLHS and tricuspid atresia, but the differences were not statistically significant. Physiological discrepancies were uncommon, with three major discrepancies, including underestimation of the degree of venous obstruction in one non‐HLHS fetus with total anomalous pulmonary venous return, overestimation of RAS in one HLHS fetus and incorrect prediction of PGE dependency in one case false‐negative for pulmonary blood flow. No discrepancy in degree of AVVR or RAS affected postnatal care. Minor physiological discrepancies included two false‐positive predictions of PGE dependency with one false‐positive for ductal‐dependent systemic flow and one false‐positive for pulmonary blood flow. Conclusions: In this contemporary review of FE at our center, there was high accuracy in describing anatomical and physiological findings in SV‐CHD. Major physiological discrepancies were uncommon but included important cases of false‐negative prediction of PGE dependency and underestimation of obstruction of total anomalous pulmonary venous return. These data can inform more accurate counseling of families with SV‐CHD fetuses and guide diagnostic improvement efforts. © 2024 International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

Objective High cardiac output lesions are associated with an increased risk of fetal death, largely as a result of cardiac failure and hydrops fetalis. The cardiovascular profile score ( CVPS) has been used to characterize cardiovascular wellbeing, and has been linked to fetal outcomes in other conditions. We aimed to test the hypothesis that elevated combined cardiac output ( CCO) in fetuses with high output lesions may be associated with worsening cardiovascular status, as evidenced by a lower CVPS. Methods A retrospective review was performed of fetuses with high cardiac output lesions that underwent echocardiography between July 2006 and November 2010. Diagnoses included sacrococcygeal teratoma, placental chorioangioma and vein of Galen aneurysm. Fetal echocardiographic evaluation included assessment of CVPS, as well as Doppler/two-dimensional estimation of CCO, indexed to estimated fetal weight ( CCOi). The relationship between CCO and CVPS was assessed. Results A total of 35 fetuses were studied: 27 had sacrococcygeal teratoma, seven had chorioangioma and one had vein of Galen aneurysm. There was a significant inverse relationship between mean logCCOi and CVPS (r2 = 0.48, P = 0.008). Of 31 patients with clinical outcome data, 10 experienced either in-utero demise or intervention; 80% of these fetuses had a CVPS of < 8. Conclusions There is an inverse relationship between CCO and CVPS in the fetus with high cardiac output lesions. As a measure of fetal cardiovascular wellbeing in this population, the CVPS may be a useful tool for stratifying risk and for selection for intervention in these fetuses. [ABSTRACT FROM AUTHOR]

Objective: We sought to evaluate amniotic fluid brain natriuretic peptide (BNP) levels as a biomarker of recipient twin (RT) cardiomyopathy (RTCM) in twin-twin transfusion syndrome.Study Design: Amniotic fluid samples were obtained from 157 twin-twin transfusion syndrome RTs and from 6 singletons (controls) from 2007 through 2009. N-terminal prohormone BNP (NT-proBNP) levels were quantified by enzyme-linked immunosorbent assay. RTCM was classified as mild (IIIA), moderate (IIIB), or severe (IIIC) by fetal echocardiography. The relationship between NT-proBNP and RTCM was evaluated using analysis of variance. The ability of NT-proBNP to predict moderate or greater RTCM was evaluated by receiver operating characteristic analysis.Results: There is a significant positive correlation between NT-proBNP levels and worsening RTCM (r = 0.33; P < .001). NT-proBNP thresholds of 569 fmol/mg and 369 fmol/mg had a sensitivity of 70% and 87%, and specificity of 67% and 42%, respectively, in predicting moderate or greater RTCM.Conclusion: This is the first large case series that demonstrates a relationship between NT-proBNP and RTCM. This pathophysiologic insight supports ongoing efforts to develop screening biomarkers. [ABSTRACT FROM AUTHOR]

Studies the effect of ballon aortic valvuloplasty of congenital aortic stenosis in children in regression of left ventricular (LV) mass. Baseline characteristics of patients involved in the study; Calculation of the LV mass from M-mode measurements according to the American Society of Echocardiography convention; Average age at the time of balloon valvuloplasty.

The early and 1-year follow-up of a single United States center using the Amplatzer atrial septal defect closure device is reported. Complete closure was documented in all patients by 1 year after device implantation. [ABSTRACT FROM AUTHOR]

Vascular rings are increasingly identified on fetal echocardiography. The purpose of this study is to analyze clinical outcomes and patterns of diagnostic testing in fetuses with vascular rings diagnosed by echocardiography. A retrospective cohort study was performed of fetuses with postnatally confirmed vascular rings from 2017 to 2022. Clinical outcomes included type and timing of symptoms, and timing of surgical intervention. Freedom from symptoms and/or surgery was assessed by Kaplan-Meier analysis. Frequency of genetic and diagnostic testing (barium esophagogram, CT/MRI angiogram, and bronchoscopy) was also assessed. Overall, 46 patients were evaluated (91% with a right aortic arch/left ductus and 4% with a double aortic arch). A vascular ring was isolated in 59%, associated with structural heart lesions in 33%, and associated with noncardiac anomalies in 8%. Prenatal diagnoses increased over time. Symptoms developed in 24% (11/46); 82% (9/11) had respiratory and 45% (5/11) had gastroesophageal complaints. Surgery was performed in 17% (11/46). Symptoms presented bimodally, prior to 100 or after 400 days of life. There was no difference in the type of symptoms for early (< 100 days) or late (> 400 days) presenters. Symptomatic patients received more diagnostic testing. Genetic testing was obtained in 46% and positive in 33%, with 22q11 deletion and Trisomy 21 being identified. Prenatal diagnoses of vascular rings increased over time, with subjects developing symptoms bimodally in early or late infancy. The frequency of genetic testing was suboptimal given the prevalence of genetic abnormalities seen in this population., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective:  Risk stratification of fetuses diagnosed with congenital heart disease (CHD) helps provide a delivery plan and prepare families and medical teams on expected course in the delivery room. Our aim was to assess the accuracy of echocardiographically determined risk-stratification assignments in predicting postnatal cardiac outcomes beyond the delivery room., Study Design:  This was a retrospective study at a single center evaluating all fetuses with CHD who were risk-stratified by echocardiographically determined level of care (LOC) assignment, ranging from 1a (lowest risk) to 4 (highest risk). All data were collected from January 1, 2017, to November 1, 2021. Outcomes included any unexpected cardiac interventions and neonatal clinical outcomes including in-hospital mortality, the need for prostaglandins or inotropes, and defined critical illness. These outcomes were assessed for each LOC assignment by Fisher's exact test., Results:  Out of 817 patients assigned a LOC, a total of 747 fetuses were included in our final cohort with a separate subanalysis of 70 fetuses diagnosed with coarctation of the aorta. The sensitivity and specificity were high for all LOC levels in predicting delivery room needs (93-100%). Higher LOC levels (3-4) had a lower positive predictive value (66-67%) indicating a high false-positive rate. Subjects with higher LOC assignments had a greater frequency of critical illness, hospital mortality, need for inotropes, need for neonatal surgical or catheterization interventions, and need for prostaglandins ( p  < 0.001 for all outcomes). A post-hoc analysis reviewing LOC assignments revealed a greater tendency to over-assign LOC at higher assignments (19% for LOC 3 and 4) compared to lower assignments (4% for LOC 1 and 2)., Conclusion:  Risk stratification based on fetal echocardiography can predict neonatal clinical outcomes and acuity of postnatal management needs. However, there is greater variability in expected clinical events and an expected degree of false positives for those with higher LOC assignments., Key Points: · Risk stratification utilizing fetal echocardiography can be used to predict neonatal needs.. · Complex heart disease has lower positive predictive value in predicting postnatal clinical needs.. · There is a tendency to over-assign risk of acute hemodynamic instability for complex heart disease.. · False positives are expected when planning high-risk deliveries to avoid compromising situations.., Competing Interests: None declared., (Thieme. All rights reserved.)

Study Objectives: The purpose of this study is to examine the prevalence of pulmonary hypertension (PHTN) in children with obstructive sleep apnea (OSA) using echocardiographic (ECHO) parameters and to examine ECHO findings as they relate to severity of OSA., Methods: A retrospective cohort study of patients with OSA undergoing polysomnogram and ECHO within 30 days of each other, between January 1, 2015, and December 31, 2020, was performed, excluding cardiac disease. ECHO evidence of PHTN was defined as ≥ 2 of the following: tricuspid regurgitation velocity > 3.0 m/s, pulmonary acceleration/ejection time ratio < 0.3, left ventricular eccentricity index > 1.5, and right ventricular dysfunction or abnormal geometry. ECHO parameters were compared to OSA severity using obstructive apnea-hypopnea index, percent of time with oxygen saturation < 90%, and percent of time with end-tidal carbon dioxide > 50 mmHg. Odds ratios were calculated for each comorbidity to evaluate for risk factors., Results: Of 509 patients, 4.3% were found to have echocardiographic evidence of PHTN. Neither obstructive apnea-hypopnea index severity nor worsening hypoxemia or hypercarbia correlated with worsening ECHO parameters. Comorbidities including bronchopulmonary dysplasia (odds ratio, 5.22, 2.01-13.53), prematurity (odds ratio, 3.10, 1.28-7.47), and autism (odds ratio, 3.69, 1.01-13.49) were associated with increased odds of PHTN., Conclusions: Significant echocardiographic evidence of PHTN was seen in 4.3% of children with OSA. ECHO findings of PHTN did not correlate with polysomnogram parameters. Comorbidities, particularly bronchopulmonary dysplasia, prematurity, and autism, appear to be a risk factor for developing PHTN in patients with OSA., Citation: Milligan I, Shaw AK, Leu R, Kanaan U, Michelfelder E. Prevalence of pulmonary hypertension in obstructive sleep apnea and its relation to disease severity. J Clin Sleep Med . 2024;20(5):675-680., (© 2024 American Academy of Sleep Medicine.)

Although most congenital heart defects (CHDs) are asymptomatic at birth, certain CHD lesions are at significant risk of severe hemodynamic instability and death if emergent cardiac interventions are not performed in a timely fashion. Therefore, accurate identification of at-risk fetuses and appropriate delivery resource planning according to the degree of anticipated hemodynamic instability is crucial. Fetal echocardiography has increased prenatal CHD detection in recent years due to advancements in ultrasound techniques and improved obstetrical cardiac screening protocols, enabling the prediction of newborns' hemodynamic status. This assessment can guide multidisciplinary resource planning for postnatal care, including selection of delivery site, delivery room management, and transport to a cardiac center based on CHD risk severity. This review will discuss fetal cardiovascular physiology and the circulatory changes that occur at the time of and immediately following birth, outline fetal echocardiographic findings used to risk-stratify newborns with CHDs, and outline principles for neonatal resuscitation and initial transitional care in neonates with these complex CHD lesions.

Background Fetal diagnosis of congenitally corrected transposition of the great arteries (ccTGA) has been increasingly reported; however, predictors of clinical outcomes remain underexplored. We undertook a multicenter, retrospective study to investigate natural history, associated anomalies, and outcomes of fetal ccTGA. Methods and Results Fetuses with ccTGA diagnosed from January 2004 to July 2020 within 20 North American programs were included. Fetuses with severe ventricular hypoplasia thought to definitively preclude biventricular repair were excluded. We included 205 fetuses diagnosed with ccTGA at a median gestational age of 23 (interquartile range, 21-27) weeks. Genetic abnormalities were found in 5.9% tested, with extracardiac anomalies in 6.3%. Associated cardiac defects were diagnosed in 161 (78.5%), with atrioventricular block in 23 (11.3%). On serial fetal echocardiogram, 39% demonstrated a functional or anatomic change, most commonly increased tricuspid regurgitation (6.7%) or pulmonary outflow obstruction (11.1%). Of 194 fetuses with follow-up, 26 were terminated, 3 experienced fetal death (2 with atrioventricular block), and 165 were live-born. Of 158 with postnatal data (median follow-up 3.7 years), 10 (6.6%) had death/transplant before 1 year. On univariable analysis, fetal factors associated with fetal death or death/transplant by 1 year included ≥ mild tricuspid regurgitation, pulmonary atresia, aortic obstruction, fetal arrhythmia, and worsening hemodynamics on serial fetal echocardiogram (defined as worse right ventricular function, tricuspid regurgitation, or effusion). Conclusions Associated cardiac lesions and arrhythmias are common in fetal ccTGA, and functional changes commonly occur through gestation. Worse outcomes are associated with fetal tricuspid regurgitation (≥mild), any arrhythmia, pulmonary atresia, aortic obstruction, and worsening hemodynamics on serial echocardiograms. These findings can inform prenatal counseling and perinatal management planning.

Background: Elevated left ventricular outflow tract (LVOT) gradients during exercise can occur in patients with hypertrophic cardiomyopathy (HCM) as well as in athletes and normal controls. The authors' staged exercise protocol calls for imaging at rest and during each stage of exercise to evaluate the mechanism of LVOT obstruction at each stage. They investigated whether this staged approach helps differentiate HCM from athletes and normal controls., Methods: They reviewed pediatric exercise stress echocardiograms completed between January 2009 and October 2017 at their center and identified those with gene-positive HCM, athlete's heart, and normal controls. Children with inducible obstruction (those with no LVOT gradient at rest who developed a LVOT peak gradient > 25 mm Hg during exercise) were included. LVOT peak gradient, velocity time integral, acceleration time, and deceleration time were measured at rest, submaximal stages, and peak exercise., Results: Compared with athletes, HCM patients had significantly higher LVOT peak gradients at rest (P = .019), stage 1 of exercise (P = .002), and peak exercise (P = .051), as well as a significantly higher change in LVOT peak gradient from rest to stage 1 (P = .016) and from rest to peak (P = .038). The acceleration time/deceleration time ratio of the LVOT Doppler was significantly lower in HCM patients compared with normal controls at peak exercise., Conclusions: The HCM patients who develop elevated LVOT gradients at peak exercise typically manifest early obstruction in the submaximal stages of exercise, which helps to differentiate them from athletes and normal controls.

Opinion Statement: Two-dimensional (2D) and Doppler echocardiography has become both an essential component in the assessment of anatomic anomalies of the fetal heart and an important tool in the assessment of fetal cardiac function. Various techniques for assessing fetal cardiovascular function exist and can be used to characterize systolic and diastolic ventricular function and estimate fetal cardiac output. These techniques can be utilized to assess fetuses with cardiovascular compromise due to a number of cardiac and non-cardiac conditions, including twin-twin transfusion syndrome, high-cardiac output lesions such as fetal tumors and vascular malformations, and primary fetal cardiomyopathy. As many of these conditions can now be treated by a variety of fetal therapeutic techniques, it is now important to be able to understand and identify fetal cardiac dysfunction. Characterization of fetal cardiac dysfunction now has the potential to provide clinicians with the means to better select patients for intervention, predict outcome, and perform follow-up following fetal interventional procedures.

Changes in ventricular geometry are often seen in patients with right ventricular hypertension secondary to pulmonary hypertension (PH). Progressive systolic bowing of the inter-ventricular septum occurs with increasing right ventricular pressure (RVp) and can be quantified with the left ventricular end-systolic eccentricity index (LVEI). Only limited data exist in children to evaluate the relationship between the LVEI and invasive RVp. We sought to assess the correlation between the LVEI and an invasively measured peak systolic RVp to aortic pressure (pAo) ratio. Medical records of patients undergoing echocardiography within 30 days of right and left heart catheterization for evaluation of PH between February 2009 and March 2014 were retrospectively reviewed. Forty-six studies in 29 subjects (median age 3.8 years, 46 % female), with a median time from echocardiogram to catheterization of -1.0 days, were included for analysis. The mean LVEI was 1.6 ± 0.5, and mean RVp/pAo ratio was 0.68 ± 0.26. There was a significant positive correlation (r = 0.76, p < 0.001) between LVEI and RVp/pAo ratio. ROC analysis demonstrated an area under the curve = 0.91 for prediction of RVp/pAo >0.50 by the LVEI. An LVEI >1.48 had a sensitivity of 76 % and specificity of 100 % in predicting RVp/pAo >0.50, while an LVEI >1.24 had a sensitivity of 88 % and specificity of 83 %. Echocardiographically derived LVEI is strongly correlated with invasively determined RVp/pAo ratio. In combination with other noninvasive measures of RVp, LVEI may help minimize the need for invasive patient evaluation.

Background: According to the American Society of Echocardiography, coronary artery (CA) imaging is recommended in pediatric examinations to identify CA anomalies. A review of the authors' center's echocardiographic studies revealed that CA images were often nondiagnostic. The aim of this study was to utilize quality improvement methodology to increase the percentage of first-time pediatric studies with definitive CA identification from a baseline of 45% to a goal of at least 75% in 9 months., Methods: A scoring system was developed to characterize the completeness of CA imaging. One point was scored for demonstration of each of the following: right CA origin by two-dimensional imaging, right CA origin by color flow Doppler imaging, left CA origin by two-dimensional imaging, and left CA origin by color flow Doppler imaging. A score of 4 was considered to represent definitive imaging. A baseline was obtained on 100 first-time echocardiograms with normal findings. During the intervention, 10 randomly selected first-time studies with normal findings were scored weekly for assessment of CA imaging. Interventions were focused on the following domains: excellence in image quality, shared ownership, transparency, and effective communication. Key interventions included labeling CA images, requiring two-dimensional and color Doppler images, optimization of settings, and elimination of macros for CA reporting., Results: The percentage of definitive CA identification increased from 45% to 82.5% over 4 months and was sustained for 7 months. Accurate reporting of incomplete CA imaging increased from 17% to 77.5%., Conclusions: Improved pediatric CA imaging and reporting were achieved through the implementation of key interventions., (Copyright © 2016 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Objectives: Elective deliveries in fetal congenital heart disease (CHD) attempt to balance fetal and neonatal risk with the goal of optimizing overall outcome. However, the magnitude of the risk for intrauterine fetal demise (IUFD) is unclear. This study aimed to (1) determine the rate of IUFD and (2) identify fetal risk factors associated with IUFD., Methods: Retrospective review of pregnancies complicated by CHD between 1998 and 2010. Data were collected regarding pregnancy outcome, extracardiac anomalies (ECA), genetic and cardiac diagnoses, severity of valve regurgitation, gestational age at birth and birth weight. Fisher's exact test and odds ratios were used to compare outcomes between groups., Results: A total of 501 pregnancies analyzed resulted in 445 live births, 22 IUFD, 16 terminations and 18 unknown outcomes. Amongst IUFD, 27% had a genetic diagnosis, 50% had an ECA and 27% had severe valve regurgitation. IUFD odds increased threefold with ECA and sevenfold with severe valve regurgitation. IUFD occurred in 1.2% without risk factors., Conclusions: IUFD in fetuses with CHD is associated with ECA, genetic syndromes and severe valve regurgitation. In absence of these fetal characteristics, the occurrence of IUFD is low, although it remains higher than in fetuses without CHD., (© 2015 John Wiley & Sons, Ltd.)

Objectives: Twin-twin transfusion syndrome is a serious complication in 10-20% of monochorionic diamniotic (or higher) twin gestations. Cardiac changes in the "recipient" twin in twin-twin transfusion can include anatomic and functional abnormalities of the right ventricular outflow tract, which have only been described in several small series. It was the aim of this study to evaluate the incidence and outcome of right ventricular outflow tract abnormalities in a large cohort of twin-twin transfusion cases seen at a single referral center., Methods: A retrospective review of all cases of twin-twin transfusion syndrome seen in our center between 2004 and 2010 was performed. Fetal echocardiographic findings were reviewed, and the presence of right ventricular outflow tract abnormalities--including functional/anatomic pulmonary atresia, pulmonary stenosis, or isolated pulmonary insufficiency--was noted. Impact of selective fetoscopic laser therapy on right ventricular outflow tract abnormality and postnatal outcomes with regard to survival and residual right ventricular outflow tract abnormality were ascertained by review of medical records and telephone interview., Results: Right ventricular outflow tract abnormalities were observed in 53/610 (8.7%) of recipient twins but in no donor twins. Pulmonary atresia (24/53, 45%) was most common, followed by pulmonary stenosis (16/53, 30%) and isolated pulmonary insufficiency (13/53, 25%). Fetal therapy resulted in acute improvements in 10/42 recipient twins following selective fetoscopic laser therapy, but persistent right ventricular outflow tract abnormality was noted in 33% of recipient twins surviving to birth., Conclusions: Right ventricular outflow tract abnormalities impact ∼ 9% of recipient twins in twin-twin transfusion syndrome, many of whom will have persistent abnormalities despite otherwise successful therapy. These data justify efforts to increase awareness and monitoring of the cardiac sequelae of twin-twin transfusion syndrome in these high-risk pregnancies., (© 2014 Wiley Periodicals, Inc.)

Objective: To identify clinical factors associated with pulmonary hypertension (PH) and mortality in patients with congenital diaphragmatic hernia (CDH)., Study Design: A prospective cohort of neonates with a diaphragm defect identified at 1 of 7 collaborating medical centers was studied. Echocardiograms were performed at 1 month and 3 months of age and analyzed at a central core by 2 cardiologists independently. Degree of PH and survival were tested for association with clinical variables using Fischer exact test, χ(2), and regression analysis., Results: Two hundred twenty patients met inclusion criteria. Worse PH measured at 1 month of life was associated with higher mortality. Other factors associated with mortality were need for extracorporeal membrane oxygenation, patients inborn at the treating center, and patients with a prenatal diagnosis of CDH. Interestingly, patients with right sided CDH did not have worse outcomes., Conclusions: Severity of PH is associated with mortality in CDH. Other factors associated with mortality were birth weight, gestational age at birth, inborn status, and need for extracorporeal membrane oxygenation., (Copyright © 2013 Mosby, Inc. All rights reserved.)

Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect that results in death if not treated shortly after birth. In approximately 60 to 70% of cases, HLHS can be detected prenatally and generally is well tolerated due to the presence of the foramen ovale and ductus arteriosus, which allow for blood to bypass the left side of the heart but still provide adequate blood flow to the systemic circulation. A rare case of HLHS involving a fetus with tricuspid valve stenosis, abnormal venous Doppler findings, and hydrops is reported.

Background: Twin-twin transfusion syndrome (TTTS) complicates 10% to 15% of monochorionic twin pregnancies. Cardiovascular changes of variable severity, such as ventricular hypertrophy, atrioventricular valve regurgitation, and systolic dysfunction, occur predominantly in recipient twins (RTs). It was the purpose of this study to perform a detailed assessment of ventricular geometry and diastolic function between controls, donor twins (DTs), and RTs., Methods: In this prospective, case-control study, two-dimensional, pulsed-wave, and Doppler tissue imaging were used to evaluate biventricular geometry and diastolic function in controls, DTs, and RTs. RTs were divided into two groups, severe and mild, on the basis of evidence of high central venous pressure. Specific variables evaluated included relative wall thickness, mitral valve and tricuspid valve E/A velocities, diastolic filling time corrected for heart rate, isovolumic relaxation time, and early (E') and late (A') diastolic myocardial velocities., Results: A total of 120 fetuses (39 TTTS twin pairs and 42 controls) were compared. Increases in relative wall thickness and isovolumic relaxation time were seen in the mild group. In the severe group, further increases in relative wall thickness and isovolumic relaxation time as well as decreased diastolic filling time corrected for heart rate were accompanied by the appearance of a monophasic Doppler inflow profile and elevations in the E/E' ratio, consistent with elevated ventricular filling pressures., Conclusions: Concentric hypertrophy is observed in RTs affected by TTTS and is associated with impaired ventricular relaxation and shortened filling time. In severe cases, further decreases in diastolic filling time and Doppler signs of elevated ventricular filling pressures are present., (Copyright © 2011 American Society of Echocardiography. Published by Mosby, Inc. All rights reserved.)

Objective: Up to 20% of fetuses with critical left heart obstructive lesions have highly restrictive or intact atrial septae. Although this condition is generally tolerated in utero, severe hypoxemia requiring emergency atrial septostomy often develops in newborns with restrictive atrial septum. We have reported that a pulmonary venous Doppler forward/reverse time-velocity integral ratio less than 5 is highly predictive of the need for emergency atrial septostomy. We reviewed our subsequent experience using fetal pulmonary venous Doppler patterns to identify and manage fetuses with critical left heart obstruction and suspected restrictive atrial septum., Methods: A retrospective review of neonates with a prenatal diagnosis of critical left heart obstruction was performed. Fetal restrictive atrial septum was defined as a small/absent interatrial shunt on 2-dimensional imaging and a mean forward/reverse time-velocity integral ratio of 5 or less. Available serial pulmonary venous Doppler data were reviewed. The primary outcome was postnatal confirmation of restrictive atrial septum or severe left atrial hypertension., Results: Eight of 39 infants had a forward/reverse time-velocity integral ratio of 5 or less. A restrictive atrial septum was confirmed postnatally in 6 of 8 infants. Overall, a forward/reverse time-velocity integral ratio of 5 or less had a sensitivity of 100% and specificity of 94% for emergency atrial septostomy. Lowering the cutoff value to 3 or less would have eliminated false-positive diagnoses in the current series. Serial data demonstrated that late second trimester values did not change in later gestation with respect to either threshold in 30 of 32 fetuses., Conclusions: In the fetus with critical left heart obstruction, a threshold forward/reverse time-velocity integral ratio of 3 or less optimizes specificity for predicting emergency atrial septostomy. Most late second trimester values will not change over time with regard to threshold levels., (Copyright © 2011 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: The purpose of this study was to evaluate the effect of maternal nifedipine on fetal survival when started 24-48 hours before selective fetoscopic laser photocoagulation (SFLP)., Study Design: We conducted a case control study of consecutive cases of twin-twin transfusion syndrome (TTTS) in which TTTS cardiomyopathy was treated with maternal nifedipine 24-48 hours before SFLP, compared with gestational age and stage-matched control cases. The primary outcome was recipient and donor survival., Results: One hundred forty-one cases of TTTS were treated with nifedipine, and 152 gestational age- and stage-matched control cases were analyzed. There was a significant increase in overall fetal survival in nifedipine-treated cases compared with control cases (237/284 [83%] vs 232/308 [75%]; P = .015). There is an increase in survival of recipients who were treated with nifedipine in stage IIIA (100% vs 81%; P = .021) and IIIB (93% vs 71%; P = .014); however, there was no difference in donor survival., Conclusion: Maternal nifedipine is associated with improved recipient survival in TTTS that undergoes SFLP. This is the first study to suggest a benefit of adjunctive maternal medical therapy in patients with TTTS who undergo SFLP., (Copyright © 2010 Mosby, Inc. All rights reserved.)

Hypoplastic left heart syndrome (HLHS) with intact atrial septum (IAS) is a severe congenital cardiac malformation with little possibility of postnatal survival, unless an intra-atrial connection is created promptly after delivery. The timing of that intervention is often dictated by logistic restrictions, such as the proximity of the delivery room to the cardiac catheterization laboratory. This report details the use of a hybrid cardiac catheterization suite to expedite the delivery, transfer, and initial treatment of a fetus with HLHS/IAS. The mother underwent cesarean delivery in one hybrid room, and the newborn infant was transferred directly into an adjoining room where successful atrial septal stenting was performed without delay via a percutaneous, transhepatic approach., (Copyright 2008 Wiley-Liss, Inc.)

Objective: This study evaluated the acute effects of selective fetoscopic laser photocoagulation (SFLP) on recipient cardiovascular function in a twin-twin transfusion syndrome (TTTS) pregnancy., Study Design: This was a retrospective chart review of echocardiographic data in TTTS including right (RV) and left (LV) ventricular Doppler myocardial performance index (MPI); LV and RV end diastolic wall thickness; and umbilical artery (UA), vein (UV), and ductus venosus (DV) Dopplers. The primary outcome measures were improved MPI defined as greater than 10% interval decrease in left and/or right MPI. Data were analyzed by Student t test and Fisher's exact test., Results: Sixty-five patients met inclusion criteria. SFLP results in a significant improvement in UV and DV Doppler and an increase in both RV and LV wall thickness. A 10% or greater improvement in recipient LV MPI after SFLP is associated with improved recipient survival as compared with unimproved LV MPI (100% vs 86.1%, P = .05)., Conclusion: Improved recipient myocardial performance index after SFLP is associated with improvement in recipient survival.

Background: Mechanisms leading to left ventricular hypoplasia and endocardial fibroelastosis in the fetus remain unknown. Prevailing theory is that obstruction to blood flow through the left ventricle leads to elevated end-diastolic pressures, compromised myocardial perfusion, and endocardial ischemia. Fetal interventions are now being performed, based on the presumption that they would prevent such pathogenic mechanisms., Methods: Forty first-trimester fetal sheep (mean gestational age, 53 days) were studied. Severe fetal left ventricular outflow obstruction was created by banding the ascending aorta in 25 fetuses; 15 control fetuses underwent "sham" surgery with thoracotomy. Serial fetal echocardiography was used to assess left ventricular growth and fetal hemodynamics. Findings were correlated to morphologic and histopathologic changes, and intracardiac pressure measurements obtained from fetal cardiac catheterization., Results: Surviving banded fetuses (n = 13) had one of two phenotypes: compensatory left ventricular hypertrophy (n = 7) or noncompensatory left ventricular dilatation (n = 6) with hydrops and severe left ventricular dysfunction. All fetuses had elevated left ventricular end-diastolic pressures (mean, 21 mm Hg; range, 14 to 28 mm Hg), which correlated to the gradient across the ascending aorta (mean, 41 mm Hg; range, 28 to 73 mm Hg). In vivo echocardiography findings were incongruous with those at autopsy, and demonstrated preservation of left ventricular growth indices in all fetuses. Endocardial fibroelastosis and myocardial fibrosis were not observed in any banded fetus., Conclusions: While early gestational obstruction to flow can compromise left ventricular function in the fetus, it does not retard normal growth. Similarly, an elevated left ventricular end-diastolic pressure is not sufficient to cause myocardial fibrosis or endocardial fibroelastosis in the fetus.

Reverse orientation of the ductus arteriosus (RDA), defined as an inferior angle at the aortic junction of <90 degrees , is associated with pulmonary atresia; however, the significance of RDA in nonatretic pulmonary outflow tract obstruction (POTO) is unknown. The purpose of this study was to evaluate the relation between ductal morphology and the need for early intervention. Ductal morphology was retrospectively reviewed in 76 neonatal cases of POTO. Patients were stratified to those with conotruncal (CT) or primary pulmonary valve (PV) abnormalities. The prevalence of RDA was 40 of 68 (59%) and was higher in patients with CT defects (28 of 30, 93%) compared with PV abnormalities (12 of 38, 32%; chi-square = 26.4, p <0.0001). Early intervention was required in 29 of 40 patients with RDA (73%) compared with only 8 of 28 patients with normal ductal morphology (31%; chi-square = 12.8, p <0.001). The proportion of patients with RDA who required early intervention was greater in PV abnormalities (12 of 12, 100%) compared with CT defects (17 of 28, 61%; chi-square = 6.5, p <0.02). Importantly, after excluding patients with pulmonary atresia, these analyses were unchanged. In patients with nonatretic POTO, a threshold inferior angle of 65 degrees was predictive of the need for early intervention. RDA in patients with PV abnormalities, or CT defects and an inferior angle <65 degrees , is a specific indicator for early intervention. These findings demonstrate an association between RDA in the context of atretic and nonatretic POTO and the need for early intervention. The need for early intervention should be considered when RDA is identified in this population.

Circumferential and noncircumferential myofiber contraction may have varying impact on systolic and diastolic function. The purpose of this study was to determine the relation of circumferential, longitudinal, and oblique fiber shortening to early diastolic filling in children. Twenty-five patients (8.1 +/- 5.6 years of age; 12 boys and 13 girls) with normal echocardiograms and no heart disease had prospective echocardiographic evaluation of circumferential (shortening fraction, fractional area change), longitudinal (left ventricular axial shortening), combined circumferential and longitudinal (left ventricular ejection fraction), oblique (left ventricular systolic twist [LVST]) shortening, and early diastolic filling. Mean LVST was 16 +/- 8 degrees. There was no relation between early diastolic filling indexes and indexes of circumferential or longitudinal shortening. However, there was a significant inverse relation between heart rate-corrected E-wave acceleration time and LVST (r = 0.63, P <.001). Oblique fiber shortening affects early diastolic filling in children. Describing the functional role of noncircumferential left ventricular myofibers may improve our understanding of global left ventricular function.

The purpose of this study was to describe the unique echocardiographic findings associated with deployment of the Amplatzer atrial septal defect (ASD) device. Thirty-five patients (2 to 40 years old; 23 female and 12 male patients) underwent echocardiography during attempted ASD closure with the Amplatzer device. Transesophageal and transthoracic echocardiograms were performed during the placement and follow-up of the device, respectively. In 5 patients, the device was not deployed because of transesophageal echocardiography (TEE) findings (an exceedingly large defect in 3 patients, partial obstruction of the upper right pulmonary vein by the device in 1, and complex atrial septal anatomy in 1). In the remaining 30 patients, after deployment but before release, the device distorted the atrial septum from the normal vertical orientation to an oblique transverse orientation. Excessive septal distortion (i.e., > or =90 degrees in 1 patient) was associated with device embolization upon release. In other patients, TEE also identified mild splaying of the device on the aortic wall, mild abutment of the device upon the mitral valve, and temporary partial obstruction of pulmonary vein flow. Color Doppler revealed residual shunts in 21 of 29 patients immediately after release, but in none of 15 patients at 1-year follow-up. Transesophageal echocardiography is essential to ensure proper Amplatzer device placement. Distortion of the atrial septum and Amplatzer device orientation occur before release but resolve on release from the delivery cable. Small residual shunts are common early, but they resolve in 6 to 12 months.

To evaluate exposure to ionizing radiation during Amplatzer device occlusion, a prospective study was performed to measure surface entrance radiation dose by thermoluminescent dosimetry (TLD). Between June 1998 and April 1999, dosimetry was carried out on 12 patients with Amplatzer device occlusion of atrial septal defects (n = 10) or Fontan fenestration (n =) and 12 age-matched patients who underwent diagnostic catherization. TLD chips were placed at the posterior (PA) and right lateral (LA) chest wall as well as the thyroid (TH) and gonadal (GN) regions. The Amplatzer group had a median age of 6.4 yr (2.4-12.4 yr) and a median weight of 23.7 kg (15.6-28.9 kg), which were similar (p = NS) to those of the control group, who had a median age of 7.9 yr (3.3-16.2 yr) and a median weight of 29.9 kg (10.6-58.0 kg). Device placement was successful in 11 of 12 patients; one device was removed owing to partial obstruction of the right-upper pulmonary vein. Fluoroscopy times were also similar in the Amplatzer group (23.5 +/- 2.1 min) and the control group (16.4 +/- 3.1 min; P = NS). The measured surface entrance doses of the Amplatzer group was similar (p = NS) to those of the control group in all four regions: PA (4.96 +/- 1.88 vs. 6.07 +/- 2.16 cGy), LA (5.22 +/- 1.68 vs. 3.13 +/- 1.25 cGy), TH (0.92 +/- 0.14 vs. 0.69 +/- 0.09 cGy), and GN (0.20 +/- 0.00 vs. 0.22 +/- 0.01cGy). Fluoroscopy times and measured surface entrance doses of ionizing radiation in patients undergoing Amplatzer device occlusion are similar to those in patients undergoing routine diagnostic catheterization.

Background: Patients with univentricular hearts and ventriculoarterial discordance with potentially obstructed systemic blood flow continue to pose difficult management problems. The goals of neonatal palliative operations are to control pulmonary blood flow while avoiding pulmonary artery distortion, to relieve systemic outflow tract obstruction, and to avoid heart block., Methods: Between January 1987 and December 1996, 38 patients with either tricuspid atresia or a double-inlet left ventricle and ventriculoarterial discordance underwent a modified Norwood procedure. Their mean age was 15 days, and their mean weight was 3.4 kg. Aortic arch anomalies were present in 92% of the patients. Morbidity and mortality statistics, intraoperative data, and postoperative echocardiograms were reviewed., Results: There were 3 early deaths (7.8%) and 5 late deaths (13.1%). The actuarial survival rates at 1 month, 1 year, and 5 years were 89%, 82%, and 71%, respectively. Follow-up was complete in all children at a mean interval of 30 +/- 9 months. None of the patients had significant neoaortic valve insufficiency, and 1 patient required therapy for residual aortic arch obstruction. Nine patients (30% of the survivors) have undergone the hemi-Fontan procedure, and 18 patients (60%) successfully have undergone the Fontan procedure., Conclusions: In this patient population, we recommend the modified Norwood procedure as the neonatal palliative treatment of choice. It can be performed with acceptable early morbidity and mortality, and it improves suitability for the Fontan procedure. It reliably relieves all levels of systemic outflow tract obstruction, controls pulmonary blood flow, and avoids heart block.

Doppler estimation of RV dP/dt correlates well with micromanometer catheter-measured values in children with hypoplastic left heart syndrome. Doppler estimation of RV dP/dt is a method of quantifying RV systolic function independent of geometric assumptions, and may be a valuable method for longitudinal analysis of RV function.