Right Ventricular Global Longitudinal Strain in Fetuses with Hypoplastic Left Heart Syndrome Does Not Differ Between Those With and Without Genetic Conditions.

The presence of a genetic condition is a risk factor for increased mortality in hypoplastic left heart syndrome (HLHS). Speckle tracking strain analysis in interstage echocardiograms have shown promise in identifying patients with HLHS at increased risk of mortality. We hypothesized that fetuses with a genetic condition and HLHS have impaired right ventricular global longitudinal strain compared with fetuses with HLHS and no evident genetic condition. We performed a retrospective analysis of 60 patients diagnosed in fetal life with HLHS from 11/2015 to 11/2019. We evaluated presenting echocardiograms and calculated right ventricular global longitudinal strain (RV GLS) and fractional area of change (FAC) using post-processing software. We first compared RV GLS and FAC between those with genetic conditions to those without. We examined the secondary outcome of mortality among those with and without genetic conditions and among HLHS subgroups. Of the 60 patients with available genetic testing, 11 (18%) had an identified genetic condition. Neither RV GLS nor FAC was significantly different between patients with and without genetic conditions. There was no difference in RV GLS or FAC among HLHS phenotype or those who died or survived as infants. However, patients with a genetic syndrome had increased neonatal and overall mortality. In this cohort, RV GLS did not differ between those with and without a genetic diagnosis, among HLHS phenotypes, or between those surviving and dying as infants. Further analysis of strain throughout gestation and after birth could provide insight into the developing heart in fetuses with HLHS.

Fetal endoscopic tracheal occlusion and pulmonary hypertension in moderate congenital diaphragmatic hernia.

OBJECTIVE: To study the role of fetal endoscopic tracheal occlusion (FETO) on resolution of pulmonary hypertension (PH) in fetuses with isolated moderate left-sided diaphragmatic hernia (CDH). METHODS: This retrospective study included fetuses with CDH evaluated between February 2004 and July 2017. Using the tracheal occlusion to accelerate lung growth (TOTAL) trial definition, we classified fetuses into moderate left CDH if O/E-LHR (observed/expected-lung head ratio) was 25-34.9% regardless of liver position or O/E-LHR of 35-44.9% if liver was in the chest. Postnatal echocardiograms were used to diagnose PH. Logistic regression analyses were performed to determine the relationship of FETO with study outcomes. RESULTS: Of 184 cases with no other major anomalies, 30 (16%) met criteria. There were nine FETO and 21 non-FETO cases. By hospital discharge, a higher proportion of infants in the FETO group had resolution of PH (87.5 (7/8) vs. 40% (8/20); p=.013). FETO was associated with adjusted odds ratio of 17.3 (95% CI: 1.75-171; p=.015) to resolve PH by hospital discharge. No significant differences were noted in need for ECMO or survival to discharge between groups. CONCLUSIONS: Infants with moderate left-sided CDH according to O/E-LHR, FETO is associated with resolution of PH by the time of hospital discharge.

Left ventricular strain and left atrial strain are impaired during hemodialysis in children.

We aimed to investigate intradialytic changes in ventricular and atrial function using speckle tracking echocardiography (STE) in pediatric hemodialysis (HD). Children with HD vintage > 3 months were enrolled, and echocardiography was performed prior to, during, and after HD. STE was analyzed using GE EchoPAC. Left ventricular (LV) global longitudinal strain (GLS), strain rate (Sr), and mechanical dispersion index (MDI) were calculated as the average from 3 apical views; diastolic strain (Ds) and Sr from 4-chamber tracing; left atrial strain (LAS) and Sr from the 4- and 2-chamber views. A total of 15 patients were enrolled at a median age of 12 years (IQR 8, 16) and median HD vintage of 13 months (IQR 9, 25). GLS worsened during HD (- 15.8 ± 2.2% vs - 19.9 ± 1.9%, p < 0.001). Post-HD GLS was associated with BP decrease (coefficient = 0.62, p = 0.01). LV MDI and systolic Sr did not change. LV Ds progressively worsened (- 8.4% (- 9.2, - 8.0) vs - 11.9% (- 13.4, - 10.3), p < 0.001). LAS changes at mid-HD returned to baseline post-HD. Ds, DSr, LAS, LASr were not associated with BV removal or BP decrease (p > 0.1). In conclusions, intradialytic LV strain and LAS changes consistent with subclinical systolic and diastolic dysfunction were observed during HD in children. Changes in Ds, DSr, LAS, and LASr were not associated with BP change or BV removal and may be related to the disease progression. Longitudinal study using these novel indices may unfold the effect of these subclinical changes on long-term cardiovascular health in children requiring chronic HD.

Chronic Maternal Hyperoxygenation and Effect on Cerebral and Placental Vasoregulation and Neurodevelopment in Fetuses with Left Heart Hypoplasia.

INTRODUCTION: In a pilot study of chronic maternal hyperoxygenation (CMH) in left heart hypoplasia (LHH), we sought to determine effect estimates of CMH on head size, vascular resistance indices, and neurodevelopment compared to controls. MATERIAL AND METHODS: Nine gravidae meeting the inclusion criteria (fetal LHH, ≥25.9 weeks' gestation, and ≥10% increase in percent aortic flow after acute hyperoxygenation) were prospectively enrolled. Controls were 9 contemporary gravidae with fetal LHH without CMH. Brain growth and Doppler-derived estimates of fetal cerebrovascular and placental resistance were blindly evaluated and compared using longitudinal regression. Postnatal anthropomorphic and neurodevelopmental assessments were compared. RESULTS: There was no difference in baseline fetal measures between groups. There was significantly slower biparietal diameter (BPD) growth in the CMH group (z-score change -0.03 ± 0.02 vs. +0.09 ± 0.05 units/week, p = 0.02). At 6 months postnatal age, the mean head circumference z-score in the CMH group was smaller than that of controls (-0.20 ± 0.58 vs. +0.85 ± 1.11, p = 0.048). There were no differences in neurodevelopmental testing at 6 and 12 months. DISCUSSION: In this pilot study, relatively diminished fetal BPD growth and smaller infant head circumference z-scores at 6 months were noted with in utero CMH exposure.

Fetal left-sided cardiac structural dimensions in left-sided congenital diaphragmatic hernia - association with severity and impact on postnatal outcomes.

OBJECTIVES: Fetuses with congenital diaphragmatic hernia (CDH) demonstrate varying degrees of left heart hypoplasia. Our study assesses the relationship between fetal left-sided cardiac structural dimensions, lung size, percentage liver herniation, lung-to-head ratio, postnatal left-sided cardiac structural dimensions, and postnatal outcomes. METHODS: We performed a retrospective cohort study of fetuses with left-sided CDH who had prenatal echocardiographic, ultrasound, and magnetic resonance imaging examinations at our institution between January 2007 and March 2015. Postnatal outcomes assessed include use of inhaled nitric oxide (iNO), use of extracorporeal membrane oxygenation, and death. RESULTS: Fifty-two fetuses with isolated left-sided CDH were included. Multivariate logistic regression models indicated that smaller fetal aortic valve z-score was associated with postnatal use of iNO (p = 0.03). Fetal mitral valve z-score correlated with lung-to-head ratio (p = 0.04), postnatal mitral valve z-score correlated with percent liver herniation (p = 0.03), and postnatal left ventricular end-diastolic dimension z-score correlated with liver herniation <20% (p = 0.04). CONCLUSION: We identified associations between smaller fetal left-sided cardiac structural dimensions and classic CDH indices. Smaller aortic valve z-score was associated with iNO use; however, left heart dimensions showed no association with extracorporeal membrane oxygenation or mortality. Further study into the impact of left-sided hypoplasia on outcomes in CDH is worthy of evaluation in a larger, prospective study. © 2017 John Wiley & Sons, Ltd.

Left ventricular rotational mechanics in early infancy: Normal reference ranges and reproducibility of peak values and time to peak values.

INTRODUCTION: Left ventricular cardiac twist and torsion values have been described in premature and term neonates, but not in early infancy. Early and late peak untwist rates and time to peak (TTP) values have not been described in infants. METHODS: 53 term infants were enrolled prospectively. The following parameters were obtained by two blinded observers at 1-2months postnatal age: peak twist and torsion (twist indexed to LV length), peak twist rate and torsion rate, TTP twist, early peak untwist rate, TTP early untwist rate, late peak untwist rate, TTP late untwist rate. Reproducibility was assessed using intraclass correlation and Bland Altman analysis. RESULTS: Intraclass correlation was ≥0.87 for all peak rotational mechanics values. Measures of TTP values had intraclass correlation (ICC) values ≤0.77, with TTP twist rate demonstrating the lowest ICC (0.69). The only measure which demonstrated significant bias was TTP twist rate. Peak twist demonstrated modest correlation (R=0.52, p<0.001) with global circumferential strain, and no correlation with ejection fraction, global longitudinal strain, or left ventricular myocardial performance index. CONCLUSIONS: Measurements of rotational mechanics and timing to peak values have acceptable reproducibility. Peak twist, twist rate, and early untwist rate values in early infancy are similar to those reported in premature neonates, and higher than those reported in older children. Twist indexed to LV length (torsion) is lower in early infancy than in premature neonates, but higher than in term neonates.

Late outcomes in children with Shone's complex: a single-centre, 20-year experience.

OBJECTIVE: Shone's syndrome is a complex consisting of mitral valve stenosis in addition to left ventricle outflow obstruction. There are a few studies evaluating the long-term outcomes in this population. We sought to determine the long-term outcomes in our paediatric population with Shone's syndrome and the factors associated with left heart growth. METHODS: All patients diagnosed with Shone's syndrome with biventricular circulation treated between 1978 and 2010 were reviewed. Baseline echocardiograms and data from catheterisations were also reviewed. Number of interventions (surgical+transcatheter), incidence of mitral valve replacement, and incidence of heart transplantation were tracked. Survival of the population and left heart structural growth were also reviewed. RESULTS: A total of 121 patients with Shone's syndrome presented at a median age of 28 days (0-17.3 years) and were followed-up for 7.2 years (0.01-35.5 years). These patients underwent 258 interventions during the study period, and the presence of coarctation was associated with repeat left heart interventions. The 10-year, transplant-free survival was 86%. Presence of pulmonary hypertension was associated with mortality. Left heart structural growth was seen for mitral and aortic valve annuli and left ventricular end-diastolic dimension over time. CONCLUSIONS: Shone's syndrome patients undergo a number of left heart interventions. Coarctation of the aorta is associated with an increased likelihood for repeat interventions. Survival appears to be more favourable than expected. Significant left heart growth will occur in the population. Pulmonary hypertension is associated with an increased risk of mortality.

Estimated combined cardiac output and laser therapy for twin-twin transfusion syndrome.

INTRODUCTION: Twin-twin transfusion syndrome (TTTS) is the major cause for fetal demise in monochorionic diamniotic pregnancies. Estimated combined cardiac output (CCO) has not been studied in this setting. The primary aims of this study were to compare CCO in twin donor and recipient fetuses and assess differences in CCO before and after laser coagulation therapy. A secondary aim was to evaluate the relationship of CCO with perinatal mortality, defined as intrauterine or neonatal mortality (≤30 days of age) in fetuses that underwent laser coagulation therapy. METHODS: We performed a retrospective review of fetuses with TTTS who received echocardiograms prior to laser therapy. Prelaser echocardiograms were performed in 66 fetuses, of which 45 (21 donor and 24 recipient) survived to the postlaser echocardiogram. RESULTS: Donor CCO increased from a mean of 487±137 mL/min/kg prelaser to 592±128 mL/min/kg postlaser (P=.025). There was no significant change in mean recipient CCO from prelaser 551±165 mL/min/kg to postlaser 575±112 mL/min/kg (P=.564). In recipient fetuses, higher prelaser CCO correlated with increasing Quintero stage (P=.010) and perinatal mortality (P=.003). Thirty-day postnatal outcome data were available for 38 fetuses, of whom 34 (89%) survived. Amongst survivors, 25 (74%) experienced an increase in CCO, while 9 (26%) experienced a decrease in CCO (P=.010). Donor fetuses had higher CCO after laser therapy. CONCLUSIONS: Higher prelaser CCO in recipient fetuses may indicate more advanced disease. Fetuses who exhibit a decrease in CCO after therapy laser may be at higher risk for perinatal mortality.

Echocardiographic Parameters and Outcomes in Primary Fetal Cardiomyopathy.

OBJECTIVES: Primary fetal cardiomyopathy is a rare entity, with a poor prognosis. We sought to describe its echocardiographic characteristics and outcomes. METHODS: We performed a 12-year retrospective review of fetuses with primary cardiomyopathy. RESULTS: Of more than 6000 fetuses evaluated, 25 met criteria for primary cardiomyopathy, and 18 had sufficient echocardiographic and pregnancy outcome data for inclusion. At echocardiography, the median gestational age was 29.6 weeks (range, 21.0-36.4 weeks); median cardiovascular profile score was 6 (range, 1-9); median right ventricular Tei index was 0.52 (range, 0.32-0.94); and median left ventricular Tei index was 0.40 (range, 0.15-0.88). Two had fetal demise, and 16 survived to delivery. The median cardiovascular profile score in those with fetal demise was 3.0 and in those who survived to delivery was 6.5 (range, 3-9; P = .14). The median right ventricular Tei index in those with fetal demise was 0.39 and in those surviving to delivery was 0.53 (range, 0.38-0.94; P = .49). The median left ventricular Tei index in those with fetal demise was 0.29 and in those surviving to delivery was 0.42 (range, 0.15-0.88; P = .50). Sixty-day survival was available in 11 of 16 fetuses. In addition to the 2 with fetal demise, 4 had postnatal demise, and 5 were alive at a median follow-up of 39 months. Hydrops (P = .01), skin edema (P = .01), and mild or greater mitral regurgitation (P = .02) were associated with fetal or postnatal demise, with a trend toward an association between moderate or greater tricuspid regurgitation (P = .07) and fetal or postnatal demise. CONCLUSIONS: Hydrops and atrioventricular valvar regurgitation are ominous signs in primary fetal cardiomyopathy. Although other commonly used methods for assessing cardiovascular performance may help in diagnosing primary cardiomyopathy, these data suggest limited predictive value.

Fetal and Neonatal Diastolic Myocardial Strain Rate: Normal Reference Ranges and Reproducibility in a Prospective, Longitudinal Cohort of Pregnancies.

BACKGROUND: Normative fetal diastolic strain rate values have not been comprehensively reported. The aims of this study were to report normative data for diastolic strain rate parameters across gestation and upon delivery, determine the effect of advancing gestational age on these measures, and compare interobserver variability at multiple gestational age time points. METHODS: Sixty gravid women were enrolled before 20 weeks' gestation. The following measures were obtained by two blinded observers at five time points across gestation and at 4 to 8 weeks' postnatal age: global left ventricular circumferential strain rate peak E and A waves, global left and right ventricular longitudinal strain rate peak E and A waves, and mitral and tricuspid valve E/A ratios. Reproducibility was assessed using intraclass correlation and Bland-Altman analysis. Least square means analysis was used to evaluate for changes across gestational age. RESULTS: Left ventricular longitudinal and circumferential diastolic strain rate values decreased across gestation, while right ventricular longitudinal values remained stable. With delivery, left ventricular values remained fairly stable, while right ventricular values increased. Intraclass correlations for diastolic strain rate values were 0.68 to 0.94 at ≥24 weeks' gestation and 0.25 to 0.82 for values at 20 to 21 weeks' gestation. Intraclass correlations ranged from 0.49 to 0.90 for mitral valve and tricuspid valve E/A ratios across gestation. CONCLUSIONS: Fetal measures of diastolic strain rate have acceptable reproducibility after 24 weeks' gestation. The described changes across gestation likely reflect intrinsic myocardial adaptation to loading conditions. These reference ranges can be used to assess effects of various disease states on fetal myocardial deformation.

A genome-wide association study of congenital cardiovascular left-sided lesions shows association with a locus on chromosome 20.

Congenital heart defects involving left-sided lesions (LSLs) are relatively common birth defects with substantial morbidity and mortality. Previous studies have suggested a high heritability with a complex genetic architecture, such that only a few LSL loci have been identified. We performed a genome-wide case-control association study to address the role of common variants using a discovery cohort of 778 cases and 2756 controls. We identified a genome-wide significant association mapping to a 200 kb region on chromosome 20q11 [P= 1.72 × 10-8 for rs3746446; imputed Single Nucleotide Polymorphism (SNP) rs6088703 P= 3.01 × 10-9, odds ratio (OR)= 1.6 for both]. This result was supported by transmission disequilibrium analyses using a subset of 541 case families (lowest P in region= 4.51 × 10-5, OR= 1.5). Replication in a cohort of 367 LSL cases and 5159 controls showed nominal association (P= 0.03 for rs3746446) resulting in P= 9.49 × 10-9 for rs3746446 upon meta-analysis of the combined cohorts. In addition, a group of seven SNPs on chromosome 1q21.3 met threshold for suggestive association (lowest P= 9.35 × 10-7 for rs12045807). Both regions include genes involved in cardiac development-MYH7B/miR499A on chromosome 20 and CTSK, CTSS and ARNT on chromosome 1. Genome-wide heritability analysis using case-control genotyped SNPs suggested that the mean heritability of LSLs attributable to common variants is moderately high ([Formula: see text] range= 0.26-0.34) and consistent with previous assertions. These results provide evidence for the role of common variation in LSLs, proffer new genes as potential biological candidates, and give further insight to the complex genetic architecture of congenital heart disease.

Longitudinal Changes and Interobserver Variability of Systolic Myocardial Deformation Values in a Prospective Cohort of Healthy Fetuses across Gestation and after Delivery.

BACKGROUND: Normative data for fetal myocardial deformation values have not been comprehensively described in a longitudinal cohort. The effect of gestational age on these values and on interobserver variability require further investigation. METHODS: Sixty gravid women were prospectively enrolled before 20 weeks' gestation. The following measures were obtained by two blinded observers at five time points across gestation and also at 4 to 8 weeks' postnatal age: global circumferential strain and strain rate, global longitudinal left ventricular strain and strain rate, global longitudinal right ventricular strain and strain rate, and left and right ventricular myocardial performance indices. Optimal myocardial visualization and frame rate (≥100 frames/sec) were ensured. RESULTS: For gestational age groups ≥24 weeks, intraclass correlation coefficients between observers were >0.70 for all measures and >0.85 for the majority of measures of myocardial deformation. At 20 to 21 weeks' gestation, intraclass correlation coefficients were 0.35 to 0.52 for longitudinal measures and 0.74 to 0.82 for circumferential measures. Myocardial performance index intraclass correlation coefficients were <0.80 at all time points and <0.70 for most time points. Global longitudinal left ventricular strain and global circumferential strain values remained stable across gestational age groups. Global longitudinal right ventricular strain values remained stable across gestation and increased after birth. Global circumferential strain rate, global longitudinal left ventricular strain rate, and global longitudinal right ventricular strain rate decreased from 20 to 21 weeks' gestation to the remainder of gestation and then remained stable until delivery. Upon delivery, global circumferential strain rate and global longitudinal left ventricular strain rate decreased, and global longitudinal right ventricular strain rate increased. CONCLUSIONS: Interobserver variability of fetal strain and strain rate measured at ≥24 weeks' gestation was lower in comparison with values obtained at 20 to 21 weeks' gestation and lower in comparison with left ventricular and right ventricular myocardial performance indices using the described protocol. Gestational changes in fetal myocardial deformation values likely reflect changes in preload and/or afterload on the fetal heart.

5, 4, 3, 2, 1: embryologic variants of pentalogy of Cantrell.

BACKGROUND: The purpose of this study was to evaluate our experience with pentalogy of Cantrell and the various embryologic variants. MATERIALS AND METHODS: Patient charts and diagnostic imaging studies of all fetuses evaluated at Texas Children's Fetal Center for pentalogy of Cantrell between April 2004 and June 2014 were reviewed retrospectively. Data collected from patient charts included demographic information, clinical presentation, fetal and postnatal imaging findings, operative treatment, pathologic evaluation, and outcomes. RESULTS: There were 10 patients who presented with embryologic variants of pentalogy of Cantrell over a 6-y period. Two cases displayed the full range of embryologic defects observed, and eight cases exhibited variants of the classic pentalogy. Sternal and pericardial defects were each present in 40% of patients. Additional anomalies present included pulmonary hypoplasia, pulmonary artery stenosis, and chromosomal abnormalities. Four patients presented with diaphragmatic defects but no defect in the pericardium, and one patient presented with a defective pericardium but no associated diaphragmatic defect, suggesting highly specific losses of somatic mesoderm during embryologic development. One patient was lost to follow-up, and a second patient underwent termination of pregnancy. Five of the remaining eight patients survived, one of which had the full range of embryologic defects and now attends preschool but requires speech and occupational therapy. The remaining surviving patients have developed without serious sequelae. CONCLUSIONS: This report highlights the spectrum of anomalies observed in the pentalogy of Cantrell and demonstrates that these fetuses can survive but with substantial morbidity.

Giant omphaloceles: surgical management and perinatal outcomes.

BACKGROUND: The purpose of this study was to describe the current management and outcomes of infants with omphalocele. METHODS: The medical records of all patients treated for omphalocele at a large children's hospital from January, 2003-February, 2014 were reviewed. Patients were classified as having an isolated omphalocele or omphalocele with minor or major associated anomalies. Prenatal data collected included fetal magnetic resonance imaging-based observed-to-expected total fetal lung volumes. Giant omphalocele (GO) was defined as >50% of liver in the omphalocele sac. RESULTS: Of 95 patients, 59 presented prenatally and had comprehensive fetal center evaluation. Of 82 live-born infants, 21 had chromosomal and 25 had major associated anomalies. No live-born baby with an isolated defect (n = 19) died, whereas mortality was 41% and 17% for those with major and minor anomalies, respectively (P = 0.006). Infants with major anomalies had significantly longer median length of intubation (36 versus 0 versus 0 d; P = 0.04) and hospital stay (157 versus 28.5 versus 18 d; P < 0.001) compared with those with minor or no anomalies. Of 40 infants with GO, the majority (85%) were managed surgically by delayed closure with a median age at repair of 10 mo (range, 3.4-23.6 mo). Six-month survival was 80%. None of the delayed repair patients required a later operative revision, whereas 2 of 5 with early repair did. CONCLUSIONS: The presence of associated anomalies is the strongest predictor of morbidity and mortality in fetuses or neonates with omphalocele. In patients with GO, delayed closure is associated with good outcomes, but larger, prospective studies comparing delayed to early closure are needed to determine the optimal timing of repair.

Fetal cardiac tamponade in a case of right-side congenital diaphragmatic hernia.

BACKGROUND: Intrafetal fluid collection is a rare ultrasound finding in fetuses with right-side congenital diaphragmatic hernia. CASE: Our patient had a fetus with a large right-side congenital diaphragmatic hernia with a significant amount of the fetal liver herniated into the chest. At 31 weeks of gestation, the fetus had significant ascites and high-pressure intrathoracic fluid accumulation, hydrops fetalis, deviation of the mediastinum, and tamponade-like physiology that compromised cardiac function. Ultrasound-guided fetal thoracoamniotic and peritoneal amniotic shunt placement markedly improved the fetal cardiac function and resolved the hydrops. The patient delivered at 34 weeks of gestation because of preterm labor and the neonate had an uncomplicated diaphragmatic hernia repair 3 days after delivery. CONCLUSION: Fetal thoracoamniotic shunting in selected cases of congenital diaphragmatic hernia with hydrops may improve the prognosis.

EXIT-to-resection for fetuses with large lung masses and persistent mediastinal compression near birth.

PURPOSE: To identify prenatal diagnostic features that will help select fetuses with lung masses (LM) who may benefit from ex-utero intrapartum treatment (EXIT procedure) as the preferred mode of delivery. METHODS: The CCAM-volume ratio (CVR), fetal treatment, and outcomes of all fetuses with LM evaluated between 2001 and 2011 were reviewed retrospectively. Fetuses with hydrops or CVR>1.6 were classified as high risk. Indications for fetal interventions included hydrops and heart failure, and indication for EXIT-to-resection was the finding of persistent mediastinal compression (PMC) near birth. RESULTS: Of 110 fetuses evaluated for LM, 78 were classified as low-risk. No fetus in this group had PMC near birth and none required perinatal treatment. Of 32 high-risk fetuses, 8 developed heart failure of which 4 survived (3 following fetal surgery). Nine high-risk fetuses with no PMC near birth were asymptomatic postnatally and treated electively. Sixteen high-risk fetuses had PMC near birth. All 9 babies with PMC treated with EXIT-to-resection did well with discharge at a median of 10 days post-operatively. All 7 fetuses treated without an EXIT developed respiratory distress following birth requiring an urgent operation; 2 died. CONCLUSION: The EXIT-to-resection procedure is a favorable delivery approach for those fetuses with large LM and PMC near birth.

Defining hydrops and indications for open fetal surgery for fetuses with lung masses and vascular tumors.

PURPOSE: The aim of this study was to identify the most accurate prenatal predictors of outcomes and need for fetal surgery for fetuses with high-risk lung masses and vascular tumors. METHODS: The records of all fetuses with high-risk lung mass (congenital cystic adenomatoid malformation-volume ratio > 1.6 or findings of hydrops) and vascular tumor evaluated between July 2001 and March 2011 were reviewed retrospectively. Hydrops was defined as accumulation of fluid in 2 or more compartments. RESULTS: Of fetuses with high-risk lung mass, hydrops was identified in 46% (11/24). Fetuses with hydrops and an abnormal echocardiogram (n = 8) demonstrated poor survival without fetal surgery (13%) compared with 100% survival in fetuses with hydrops and a normal echocardiogram (n = 3; P = .02). Of 21 fetuses with vascular tumor (11 sacrococcygeal and 8 cervical teratomas; 2 hemangioendotheliomas), hydrops was identified in 29% and an abnormal echocardiogram in 57%. All fetuses with hydrops had an abnormal echocardiogram and either died (n = 5) or required fetal surgery (n = 1). However, all fetuses with abnormal echocardiograms alone (n = 7) survived without fetal intervention. CONCLUSIONS: For fetuses with lung mass, an abnormal echocardiogram in the setting of hydrops is the best predictor of mortality and need for fetal surgery. For fetuses with vascular tumor, hydrops in the setting of high-output physiology best predicts demise and need for fetal surgery.