Prenatal vs postnatal diagnosis of 22q11.2 deletion syndrome: cardiac and noncardiac outcomes through 1 year of age.

BACKGROUND: The 22q11.2 deletion syndrome is the most common microdeletion syndrome and is frequently associated with congenital heart disease. Prenatal diagnosis of 22q11.2 deletion syndrome is increasingly offered. It is unknown whether there is a clinical benefit to prenatal detection as compared with postnatal diagnosis. OBJECTIVE: This study aimed to determine differences in perinatal and infant outcomes between patients with prenatal and postnatal diagnosis of 22q11.2 deletion syndrome. STUDY DESIGN: This was a retrospective cohort study across multiple international centers (30 sites, 4 continents) from 2006 to 2019. Participants were fetuses, neonates, or infants with a genetic diagnosis of 22q11.2 deletion syndrome by 1 year of age with or without congenital heart disease; those with prenatal diagnosis or suspicion (suggestive ultrasound findings and/or high-risk cell-free fetal DNA screen for 22q11.2 deletion syndrome with postnatal confirmation) were compared with those with postnatal diagnosis. Perinatal management, cardiac and noncardiac morbidity, and mortality by 1 year were assessed. Outcomes were adjusted for presence of critical congenital heart disease, gestational age at birth, and site. RESULTS: A total of 625 fetuses, neonates, or infants with 22q11.2 deletion syndrome (53.4% male) were included: 259 fetuses were prenatally diagnosed (156 [60.2%] were live-born) and 122 neonates were prenatally suspected with postnatal confirmation, whereas 244 infants were postnatally diagnosed. In the live-born cohort (n=522), 1-year mortality was 5.9%, which did not differ between groups but differed by the presence of critical congenital heart disease (hazard ratio, 4.18; 95% confidence interval, 1.56-11.18; P<.001) and gestational age at birth (hazard ratio, 0.78 per week; 95% confidence interval, 0.69-0.89; P<.001). Adjusting for critical congenital heart disease and gestational age at birth, the prenatal cohort was less likely to deliver at a local community hospital (5.1% vs 38.2%; odds ratio, 0.11; 95% confidence interval, 0.06-0.23; P<.001), experience neonatal cardiac decompensation (1.3% vs 5.0%; odds ratio, 0.11; 95% confidence interval, 0.03-0.49; P=.004), or have failure to thrive by 1 year (43.4% vs 50.3%; odds ratio, 0.58; 95% confidence interval, 0.36-0.91; P=.019). CONCLUSION: Prenatal detection of 22q11.2 deletion syndrome was associated with improved delivery management and less cardiac and noncardiac morbidity, but not mortality, compared with postnatal detection.

Cardiac problems in the fetus: a review for pediatric providers.

PURPOSE OF REVIEW: The aim of this study was to provide pediatric providers with a review of the diagnosis and management of fetal cardiac disease in the current era. RECENT FINDINGS: Prenatal detection of congenital heart disease (CHD) has improved but is still imperfect. In experienced hands, fetal echocardiography can detect severe CHD as early as the first trimester and a majority of more subtle conditions in the second and third trimesters. Beyond detection, a prenatal diagnosis allows for lesion-specific counseling for families as well as for development of a multidisciplinary perinatal management plan, which may involve in-utero treatment. Given the diversity of cardiac diagnoses and the rarity of some, collaborative multicenter fetal cardiac research has gained momentum in recent years. SUMMARY: Accurate diagnosis of fetal cardiac disease allows for appropriate counseling, pregnancy and delivery planning, and optimization of immediate neonatal care. There is potential for improving fetal CHD detection rates. Fetal interventions are available for certain conditions, and fetal and pediatric cardiac centers have developed management plans specific to the expected postnatal physiology.

Fetal Echocardiographic Variables Associated with Pre-Surgical Mortality in Truncus Arteriosus: A Pilot Study.

Truncus arteriosus (TA) is a rare congenital heart defect that can be prenatally detected by fetal echocardiography. However, prognostication and prenatal counseling focus primarily on surgical outcomes due to limited fetal and neonatal pre-surgical mortality data. We aimed to describe the incidence and identify predictors of pre-surgical mortality in prenatally detected TA. This two-center, retrospective cohort study included fetuses diagnosed with TA between 01/2010 and 04/2020. The primary outcome was pre-surgical mortality, defined by fetal or neonatal pre-surgical death or primary listing for transplantation prior to discharge. Univariable regression modeling, Chi-square tests, and t tests assessed for associations between prenatal clinical, demographic, and fetal echocardiographic (fetal-echo) variables and pre-surgical mortality. Of 23 pregnancies with prenatal diagnosis of TA, 4 (17%) were terminated. Of the remaining 19, pre-surgical mortality occurred in 4 (26%), including 2 (11%) fetal deaths and 2 (11%) neonatal pre-surgical deaths. No transplantation listings. Of liveborn fetuses (n = 17), 15 (88%) underwent a neonatal surgery, and 1 (6%) required ECMO. As compared to the survivors, the pre-surgical mortality group had a higher likelihood of having left ventricular dysfunction (0% vs. 40%; p = 0.01), right ventricular dysfunction (0% vs. 60%; p = 0.002), cardiovascular profile score < 7 (0% vs. 40%; p = 0.01), skin edema (0% vs. 40%; p = 0.01), and abnormal umbilical venous (UV) Doppler (0% vs. 60%; p = 0.002). The presence of truncal valve regurgitation or stenosis neared significance. In this cohort with prenatally diagnosed TA, there is significant pre-surgical mortality, including fetal death and neonatal pre-surgical death. Termination rate is also high. Fetal-echo variables associated with pre-surgical mortality in this cohort include ventricular dysfunction, low CVP, skin edema, and abnormal UV Doppler. Knowledge about prenatal risk factors for pre-surgical mortality may guide parental counseling and postnatal planning in prenatally diagnosed TA.

Current State of Fetal Heart Disease Counseling and Training: Room for Improvement? : Endorsed by the Fetal Heart Society.

We sought to describe the fellowship experiences and current practice habits of pediatric cardiologists who counsel patients with fetal heart disease (FHD) and to identify fellowship experiences related to FHD counseling perceived as valuable by respondents as well as opportunities for improvement. A cross-sectional survey of attending pediatric cardiologists who care for patients with FHD was performed. The respondents' demographics, fellowship experiences related to FHD counseling, reflections on fellowship training, and current practice habits were collected. The Fetal Heart Society endorsed this survey. There were 164 survey responses. 56% of respondents did not have 4th-year subspecialty training in fetal cardiology. Observing and performing FHD counseling were the most commonly used methods of training, with the highest perceived effectiveness. The number of counseling sessions observed and performed correlated moderately with confidence in FHD counseling skills at fellowship graduation. Extracardiac pathology and neurodevelopment were the least frequently addressed topics in fellowship training and in current practice. Fewer than 50% of respondents received formal education and feedback in counseling techniques during fellowship training. A significant proportion of practicing pediatric cardiologists provide FHD counseling with only standard categorical training. This highlights the potential importance of expanding FHD counseling education into categorical fellowship curricula. We suggest increasing opportunities for fellows to perform FHD counseling and receive feedback as this is a valued and beneficial experience during training. A formalized curriculum including extracardiac pathology and neurodevelopment and the use of evidence-based workshops in counseling techniques may address identified gaps in fellowship education.

High Output Cardiovascular Physiology and Outcomes in Fetal Diagnosis of Vein of Galen Malformation.

Vein of Galen aneurysmal malformation (VGAM) is a rare anomaly associated with poor outcomes from high output cardiac failure and neurologic complications. Studies addressing fetal cardiovascular status and outcomes in this population are limited. A single-center retrospective review was conducted on patients with a prenatal diagnosis of VGAM who underwent a fetal echocardiogram between January 2015 and July 2019. Fetal echocardiographic data, brain magnetic resonance imaging (MRI) findings and outcomes were collected. Nine fetuses [median gestational age at echocardiogram 34 (1.1) weeks] were included. All patients had superior vena cava dilation and reversal of diastolic flow in the transverse aortic arch. Median cardiothoracic (CT) ratio was 0.39 (0.09). Right ventricular (RV) and left ventricular (LV) dysfunction was present in 66% and 11% fetuses, respectively. Four out of five patients that underwent postnatal endovascular neurosurgical interventions at our center were alive at follow-up (mean 2.7 years). Of the non-survivors (n = 5), 3 received comfort care because of severe brain damage and died in the neonatal period. Non-survivors more commonly had > mild tricuspid regurgitation (TR) (40% vs. 25%) and > mild RV dilation (60% vs. 25%). Combined cardiac index (CCI) was higher in non-survivors when compared to survivors (672.7 vs. 530.2 ml/kg/min, p = 0.016). Fetuses with significant parenchymal damage on brain MRI tended to have a higher CCI than those without (979.8 vs. 605.0 ml/kg/min, p = 0.047). RV dysfunction, TR and elevated CCI are more commonly seen in non-survivors with VGAM. A higher CCI is seen in those deemed untreatable due to significant parenchymal volume loss. Future multicenter studies are needed to assess for prenatal prediction of outcomes in this high-risk population.

Cardiac Manifestations of Noonan Syndrome.

Noonan syndrome NS, a RASopathy, is commonly seen in association with cardiovascular abnormalities, with structural defects and/or cardiomyopathy present in 80-90-% of cases. Though a wide spectrum of cardiac pathology has been reported, pulmonary stenosis is the most common structural abnormality and more likely to be seen in PTPN11 mutations. Hypertrophic cardiomyopathy is the second most common and is more often associated with RAF1 mutations. Cardiac disease tends to be more progressive in infants and children with NS and therefore close cardiology follow-up is indicated. In general, the earlier the presentation, the more severe the phenotype and worse the long term prognosis. As genotype phenotype associations are being better understood, the mechanisms for development of cardiomyopathy are also becoming elucidated, raising the possibility of medical therapies targeted at the involved pathway.

Abnormalities in Left Ventricular Rotation Are Inherent in Young Children with Repaired Tetralogy of Fallot and Are Independent of Right Ventricular Dilation.

Left ventricular (LV) dysfunction is a risk factor for adverse outcomes in older children and adults with repaired Tetralogy of Fallot (rToF). Pulmonary regurgitation (PR), right ventricular (RV) dilation, and dysfunction have been shown to result in abnormal LV myocardial mechanics and dysfunction. The aim of our study was to evaluate LV rotational mechanics, especially apical rotation in young children with rToF with and without RV dilation. This is a retrospective, single center study in 28 asymptomatic young children with rToF (16 with RV dilation; 12 without RV dilation); 29 age-matched normal controls. RV and LV systolic and diastolic function was studied using conventional two-dimensional echocardiography (2DE) and speckle tracking echocardiography (STE). Rotational mechanics studied included basal and apical rotation (BR, AR), peak twist (calculated by difference between the apical and basal rotation), twist rate (TR), and untwist rate (UnTR). The mean age of the cohort was 4.7 years (± 2.3). Abnormal AR, BR, TR, and UnTR were noted in patients with rToF. The abnormalities were significant in magnitude as well as the direction of rotation; more pronounced in the absence of RV dilation. LV systolic and diastolic dysfunction as evidenced by abnormal AR and degree of untwist is inherent in rToF and not associated with RV dilation in rToF children. Abnormal BR may reflect a lack of maturation to adult type of rotational mechanics. Further longitudinal studies are required to study the progression of these abnormalities and their correlation with clinical outcomes.

Malattached septum primum and deficient septal rim predict unsuccessful transcatheter closure of atrial communications.

OBJECTIVES: Assess impact of malattached septum primum (MASP) on transcatheter closure of interatrial communications. BACKGROUND: Large defect size and deficient rims have been described as predictors for complications and unsuccessful device placement in closure of interatrial communications. MASP is an underappreciated morphologic atrial septal variation whose prevalence in isolated atrial communications and its influence on percutaneous device closure has not been systematically assessed. METHODS: We retrospectively evaluated echocardiographic data of 328 patients scheduled for percutaneous secundum atrial septal defect (ASD) and patent foramen ovale (PFO) closure between January 2006 and January 2013. In ASD patients, defect size was measured by balloon stretch diameter while the length of the surrounding rims was measured in standard transthoracic and transesophageal views. Furthermore, the distance between septum primum and septum secundum as it attaches to the left atrial roof was evaluated in both, ASD and PFO patients. Septal anatomy of patients with procedural failure was compared to the anatomy of patients in whom the procedure was successful. RESULTS: Transcatheter defect closure was successful in 131 (92.3%) of 142 ASD patients (32.3 years ± 24.7 years) and in all 186 PFO patients (49.6 years ± 16.2 years). Thirteen (4%) patients were found to have MASP (1.6 mm-13 mm). Smaller retroaortic rim, inferior rim, and the presence of a MASP were independently associated with procedural failure in ASD patients (P = 0.02, P = 0.03, and P = 0.003, respectively). CONCLUSION: Smaller retroaortic and inferior rims, as well as MASP are independent risk factors for unsuccessful transcatheter ASD closure.

Subpulmonary stenosis assessed in midtrimester fetuses with tetralogy of Fallot: a novel method for predicting postnatal clinical outcome.

This study aimed to determine whether quantification of subpulmonary stenosis (SPS) in tetralogy of Fallot (TOF) in the second-trimester fetus can predict postnatal clinical outcome measured by pulmonary valve size and/or timing or type of intervention. The study retrospectively identified fetuses with TOF from 1998 to 2010 diagnosed at 26 weeks gestation or earlier. The data evaluated included pre- and postnatal pulmonary valve z-scores (PVZ). To quantify fetal SPS, the authors created a novel index, the SPS/DAO ratio, a ratio of the minimum infundibular diameter to the descending aorta diameter (DAO). Multiple linear regression was used to predict postnatal PVZ from prenatally determined parameters, including SPS/DAO. Fetal parameters were analyzed by logistic regression for association with postnatal outcomes, namely, timing of surgery (<1 month), used as a surrogate for severity, and type of surgery [transannular patch (TAP) vs valve sparing surgery]. A total of 23 fetuses met the inclusion criteria. The mean gestational age was 21.8 ± 1.9 weeks (range, 16.6-25.4 weeks). There was excellent correlation between predicted and measured PVZ (r = 0.82; p < 0.0001) using the following derived equation: -3.68 + (0.91 × prenatal PVZ) - (4.44 × SPS/DAO) - 3.19 (prenatal PVZ × SPS/DAO). An SPS/DAO value lower than 0.5 had 100 % sensitivity and 56 % specificity for repair before the age of 1 month, and a value lower than 0.47 had 100 % sensitivity and 75 % specificity for TAP repair. Prenatal PVZ and the SPS/DAO ratio at 26 weeks gestation or earlier can reliably predict postnatal PVZ in fetuses with TOF. Quantification of SPS with the SPS/DAO ratio identifies patients who may require early intervention secondary to disease severity and may predict the type of repair, thereby influencing prenatal counseling.

Streptococcus pneumoniae purulent pericarditis in a neonate.

Purulent bacterial pericarditis is an uncommon infection that manifests during childhood, and in the post-antibiotic era Streptococcus pneumoniae is an unusual cause.We report a case of purulent bacterial pericarditis in a neonate caused by Streptococcus pneumoniae serotype 7F. Although cases of bacterial pericarditis caused by Streptococcus pneumoniae as a causative agent have been reported, their combination in a neonate is unique and this is, to our knowledge, the first case of this combination in the newborn period.

Opportunities to enhance parental well-being during prenatal counseling for congenital heart disease.

Prenatal diagnosis of congenital heart disease (CHD) can be a life-altering and traumatic event for expectant parents. Parental anxiety, depression, and traumatic stress are common following a prenatal cardiac diagnosis and if untreated, symptoms often persist long-term. During prenatal counseling, parents must try to manage psychological distress, navigate uncertainty, process complex medical information, and make high-stakes medical decisions for their unborn child and their family. Physicians must deliver the diagnosis, describe the expected perinatal management plan, discuss short and long-term prognoses and introduce elements of uncertainty that may exist for the particular diagnosis. Physican training in these important skills is highly variable and many in our field acknowledge the need for improved guidance on best practices for counseling and supporting parents during pregnancy and early parenthood after prenatal diagnosis, while also sustaining physicians' own emotional well-being. We describe these challenges and the opportunities that exist to improve the current state of prenatal counseling in CHD.

Outcomes of mitral regurgitation associated with large ventricular septal defect and a normal mitral valve apparatus: does intact atrial septum have an impact?

The hemodynamics of isolated large ventricular septal defect with an intact atrial septum results in greater mitral valve (MV) annular dilation and an increased incidence of mitral regurgitation (MR). MR will resolve in most patients after surgical VSD closure alone without MV repair.

Diagnostic Accuracy of Fetal Echocardiography in Congenital Heart Disease.

BACKGROUND: The accuracy of fetal echocardiography (FE) is not well defined, and reporting of diagnostic discrepancies (DDs) is not standardized. The authors applied a categorization scheme developed by the American College of Cardiology Quality Metric Working Group and applied it to FE. METHODS: A retrospective single-center study was conducted of prenatally diagnosed major structural congenital heart disease, defined as expected need for intervention within the first year of life. DDs between pre- and postnatal findings were identified and categorized. Minor DDs had no clinical impact, moderate DDs had impact without harm, and severe DDs resulted in adverse events. Multivariate regression analysis was used to determine factors associated with discrepancy. RESULTS: From December 2008 to September 2017, 17,096 fetal echocardiograms were obtained, among which 222 fetuses with a median gestational age at first FE of 24 weeks were included. There were 30 DDs (13.5%), of which the majority were false negatives (56.7%). Most were minor or moderate in severity, with one severe DD. The majority were possibly preventable (90%), with the most common contributing factor being technical limitations (43.3%). The most common anatomic segment involved was the ventricular septum (23%), primarily missed septal defects. Comparing cases with DDs versus those without, those with DDs were more likely to have high anatomic complexity (16.7% vs 3.6%, P = .01), maternal comorbidities (40.0% vs 22.1%, P = .03), and a younger maternal age (median, 27 vs 30 years, P = .02). They were also more likely to have later gestation at initial FE (median, 29.5 vs 24 weeks, P = .003), to have fewer total fetal echocardiograms (median, 2 vs 3, P = .002), and to have a fellow as the initial sonographer (36.7% vs 16.7%, P = .03). There were no significant differences in maternal race/ethnicity, fetal comorbidities, and interpreting physician experience between cases with DDs and those without. On multivariate analysis, variables associated with DD included high anatomic complexity, maternal comorbidities, and fellow as initial imager. A greater number of fetal echocardiograms was associated with reduced DD. CONCLUSIONS: FE had a DD rate of 13.5%, mostly minor and moderate in severity. Factors associated with DD included high anatomic complexity, maternal comorbidities, fellow as the initial sonographer, and fewer fetal echocardiograms. Strategies to reduce DD could include a regular secondary review and repeat FE, particularly when anatomic complexity is high.

Screening for rejection in symptomatic pediatric heart transplant recipients: the sensitivity of BNP.

As the pediatric OHT population expands, there is increasing demand for convenient, yet sensitive screening techniques to identify children with acute rejection when they present to acute care facilities. In children, symptoms of acute rejection or other causes of graft dysfunction are often non-specific and can mimic other childhood illnesses. The aim of this study was to assess the utility of BNP as a biomarker to assist providers in clinical decision-making when evaluating symptomatic pediatric heart transplant patients. One hundred twenty-two urgent care and emergency room visits from 53 symptomatic pediatric OHT patients were retrospectively reviewed to evaluate the relationship between BNP levels, symptoms, and clinical diagnosis at these visits. An ROC curve was generated to determine the accuracy of BNP as a screening tool for acute rejection in this patient population. In this group of patients, a BNP value of >700 pg/mL was 100% sensitive and 92% specific for detecting allograft acute rejection (NPV of 100%). We concluded that BNP is a highly sensitive screening test for acute rejection in symptomatic pediatric heart transplant patients.

Impact of changing indications and increased utilization of fetal echocardiography on prenatal detection of congenital heart disease.

BACKGROUND: Antenatal diagnosis of congenital heart defects (CHD) can impact outcomes in neonates with severe CHD. Obstetric screening guidelines and the indications for fetal echocardiography (FE) have evolved in an attempt to improve the early prenatal detection of CHD. Analyzing yield for specific indications will help clinicians better stratify at-risk pregnancies. METHODS: Retrospective cohort study of all FE performed between 2000 and 2010 at a single tertiary care academic medical center in New York City. A total of 9878 FE met inclusion criteria for our study. In cases of multiple gestations (MG), each fetus was counted as a separate study. RESULTS: The number of new diagnosis of fetal CHD by FEs increased 200%. There was a statistically significant increase in those referred for suspected CHD, increased nuchal translucency (NT), MG, and suboptimal imaging (P < .001). The indication of "suboptimal imaging" (SO) not only accounted for 5.23% of all referrals from 2000 to 2002, compared to 22.26% of all referrals from 2008 to 2010 (P < .0001), but also had the lowest yield for diagnoses of CHD (P < .02). CONCLUSIONS: Over the past decade, there has been an increase in utilization of FE with a proportional increase in prenatally diagnosed CHD. For indications such as suspected CHD, NT and MG increases in referrals have led to a proportionate increase in fetal diagnosis of CHD. SO as an indication has the lowest yield of fetal diagnosis of CHD. Antenatal detection of CHD may be improved by a change in obstetric imaging protocols to ensure appropriate referrals.

Utility of Multimodality Imaging in the Morphologic Characterization of Anomalous Aortic Origin of a Coronary Artery.

BACKGROUND: Anomalous aortic origin of a coronary artery from the wrong Sinus of Valsalva (AAOCA) is a rare congenital anomaly and is associated with sudden cardiac death. Morphologic features considered to be "high risk" are significant luminal narrowing, acute coronary angulation at its origin, intramural course, and long interarterial course. A consistent approach for characterization of these features is lacking. METHODS: A retrospective single-center review of all patients diagnosed with AAOCA using echocardiogram and computed tomography (CT)/magnetic resonance imaging (MRI) studies was performed. Twenty-nine patients were identified (25 using CT and 4 using MRI) with subsequent three-dimensional data sets. The MRI data sets lacked adequate resolution and were excluded. Twenty-five patients (median age 15.1, range 10-39.5 years, 72% male) were further analyzed using echocardiogram and CT. Morphologic assessment focused on luminal stenosis, coronary angulation, and interarterial length. Additional morphologic features focusing on cross-sectional area and degree of ellipticity were also assessed. RESULTS: Echocardiography tended to yield smaller measurements compared to CT and had poor interobserver reproducibility for measurements pertaining to the narrowest proximal and distal coronary segments. Computed tomography showed good inter-/intraobserver reproducibility for the same. Agreement between both modalities for coronary angulation at its origin was excellent. There was good agreement for measurements of interarterial length between echocardiography and CT, but echocardiography had superior reproducibility. Assessment of luminal cross-sectional area and elliptical shape by CT had excellent inter-/intraobserver reproducibility. CONCLUSION: The combination of echocardiography and CT characterizes morphologic features of anomalous origin of the coronary artery more reliably than either modality alone.

Prevalence and outcomes of coronary artery ectasia associated with isolated congenital coronary artery fistula.

Isolated congenital coronary artery fistula (CAF) is rare and varies with respect to size and hemodynamic significance. The prevalence of coronary artery ectasia in association with isolated congenital CAF, regardless of size, and after closure of large fistulae has not been systematically evaluated in the literature. This study aimed to characterize the demographic and echocardiographic differences between patients with large and small fistulae and to describe outcomes with respect to coronary ectasia in those who underwent closure. This is a retrospective review of an echocardiographic database that identified patients coded for CAF (1995 to 2012) and excluded those associated with complex cardiac disease and/or coronary anomalies and cardiomyopathy. Small fistulae were noted to arise mostly from the left anterior descending artery, drain into the pulmonary artery, and have a very low incidence of ectasia (n = 3 of 92), with a mean coronary artery diameter z score in these 3 patients of 3.45 ± 1.15. Larger fistulae had a female predominance, with most originating from the right coronary artery and draining into the right atrium; among the 12 patients who underwent procedural closure of large CAF, all feeding coronary arteries remained ectatic after closure, with a mean coronary artery diameter z score of 9.54 ± 5.66 after a total mean follow-up time of 3.95 ± 4.07 years. In conclusion, the occurrence of coronary dilatation justifies long-term follow-up irrespective of fistula size and successful closure of large CAF.

Normal values for left ventricular volume in infants and young children by the echocardiographic subxiphoid five-sixth area by length (bullet) method.

BACKGROUND: Left ventricular (LV) end-diastolic volume (LVEDV) can be estimated by the formula (5/6) x area x length, or the "bullet" method. The aim of this study was to determine the range of normal LVEDV values in infants and young children (aged 0-3 years) by the subxiphoid bullet method. METHODS: Echocardiograms from 100 normal subjects aged ≤ 3 years were retrospectively analyzed. Subjects with systemic disease, abnormal body size, cardiovascular disease, or nondiagnostic subxiphoid images were excluded. Measurements of LV short-axis cross-sectional diastolic area at the midventricular level and LV length were made offline from subxiphoid images. LVEDV was indexed to body surface area (BSA) to the powers of 1.0 and 1.38. Relationships between indexing methods, age, and gender were explored. RESULTS: The median age was 0.98 years (range, 0-2.9 years), the median weight was 9.5 kg (range, 3.1-16.0 kg), and the median BSA was 0.45 m(2) (range, 0.21-0.66 m(2)). The mean LVEDV/BSA(1.38) was 70.4 ± 9.1 mL/m(2.6), with an excellent correlation between LVEDV and BSA(1.38) (r = 0.96, P < .01). There was no residual relationship between LVEDV/BSA(1.38) and BSA (r = 0.06, P = NS) and no significant relationship between LVEDV/BSA(1.38) and age (r = 0.10, P = NS) or LVEDV/BSA(1.38) and gender. CONCLUSIONS: The normal range for LVEDV by the subxiphoid echocardiographic bullet method is reported for newborns, infants, and young children. LVEDV should be indexed to BSA(1.38), which is consistent with the known relationship between LV size and body size. In children aged ≤ 3 years, these data can be used to calculate Z scores for LVEDV by the subxiphoid bullet technique independent of age or gender.