Prognostic value of echocardiographic parameters in congenital diaphragmatic hernia: a systematic review and meta-analysis.

BACKGROUND: Prognostication of mortality and decision to offer extracorporeal membrane oxygenation (ECMO) treatment in infants with congenital diaphragmatic hernia (CDH) can inform clinical management. OBJECTIVE: To summarise the prognostic value of echocardiography in infants with CDH. METHODS: Electronic databases Ovid MEDLINE, Embase, Scopus, CINAHL, the Cochrane Library and conference proceedings up to July 2022 were searched. Studies evaluating the prognostic performance of echocardiographic parameters in newborn infants were included. Risk of bias and applicability were assessed using the Quality Assessment of Prognostic Studies tool. We used a random-effect model for meta-analysis to compute mean differences (MDs) for continuous outcomes and relative risk (RR) for binary outcomes with 95% CIs. Our primary outcome was mortality; secondary outcomes were need for ECMO, duration of ventilation, length of stay, and need for oxygen and/or inhaled nitric oxide. RESULTS: Twenty-six studies were included that were of acceptable methodological quality. Increased diameters of the right and left pulmonary arteries at birth (mm), MD 0.95 (95% CI 0.45 and 1.46) and MD 0.79 (95% CI 0.58 to 0.99), respectively) were associated with survival. Left ventricular (LV) dysfunction, RR 2.40, (95% CI 1.98 to 2.91), right ventricular (RV) dysfunction, RR 1.83 (95% CI 1.29 to 2.60) and severe pulmonary hypertension (PH), RR 1.69, (95% CI 1.53 to 1.86) were associated with mortality. Left and RV dysfunctions, RR 3.30 (95% CI 2.19 to 4.98) and RR 2.16 (95% CI 1.85 to 2.52), respectively, significantly predicted decision to offer ECMO treatment. Limitations are lack of consensus on what parameter is optimal and standardisation of echo assessments. CONCLUSIONS: LV and RV dysfunctions, PH and pulmonary artery diameter are useful prognostic factors among patients with CDH.

Inter-twin differences in fetal echocardiographic findings are associated with decreased dual twin survival in twin-twin transfusion syndrome.

OBJECTIVE: To determine if inter-twin differences in fetal echocardiographic findings are associated with fetal survival in monochorionic pregnancies complicated by twin-to-twin transfusion syndrome (TTTS). METHODS: This study included women who underwent laser surgery for TTTS between 2012 and 2018 at a single institution. Echocardiographic cardiac parameters in the donor and recipient twins were compared using Z-scores and regression analyses (adjusted for confounding variables) to determine whether any measurable inter-twin differences were associated with neonatal survival at birth. Results are expressed as (Odds ratio [95% confidence interval], p-value). RESULTS: Fetal echocardiography and delivery information was available in 124 TTTS cases. Dual live-birth occurred in 72% and at least one live-birth was seen in 89% of cases.Sixty-four percent (51/79) of recipient twins had evidence of cardiac dysfunction compared to 10% (8/79) of the donor twins (p < .01). In the logistic regression, inter-twin differences in left ventricle short axis dimension (0.62[0.44-0.87], p < .01), aortic valve diameter (0.67[0.45-0.99], p = .047), peak systolic velocity across the pulmonary artery (PA-PSV) (0.09[0.01-0.53], p < .01) and mitral valve diameter (0.56[0.38-0.84], p < .01) were associated with lower dual twin survival at birth. CONCLUSION: Inter-twin differences in left cardiac geometry and function are associated with decreased survival at delivery in TTTS.

Umbilical Artery Doppler Patterns and Right Ventricular Outflow Abnormalities in Twin-Twin Transfusion Syndrome.

OBJECTIVES: To evaluate the association of abnormal Doppler velocimetric patterns in the umbilical arteries (UAs) and right ventricular outflow tract abnormalities (RVOTAs) in twin- twin transfusion syndrome (TTTS) cases. METHODS: This retrospective study involved women who had laser surgery for TTTS between January 2012 and May 2018 at a single institution. The prevalence of an RVOTA in either twin was compared among TTTS cases in which both twins had positive end-diastolic flow (EDF) in the UA and those in which either twin had intermittent or persistent absent/reversed UA EDF. Nonparametric tests were used for comparisons. Logistic regression was performed to identify variables associated with an RVOTA in either twin, adjusted for moderate or severe tricuspid regurgitation, right ventricular hypertrophy, right ventricular systolic or diastolic dysfunction, the Quintero stage, and other confounders. P < .05 was considered significant. RESULTS: A total of 126 consecutive TTTS cases were included. Right ventricular outflow tract abnormalities were seen in 8.7% (11of 126) of cases, all in recipient twins. Significant differences in the rate of RVOTAs in the recipient twin were seen between TTTS cases with intermittent absent/reversed UA EDF and those with positive UA EDF (26.9% [7 of 26] versus 3.7% [3 of 82]; P = .002]. However, no significant differences were noted among the other study groups. Intermittent absent/reversed UA EDF was associated with a significantly increased risk for an RVOTA (adjusted odds ratio, 20.6 [95% confidence interval, 3.1-138]; P = .002) after adjusting for confounders. CONCLUSIONS: Intermittent changes in vascular impedance to UA flow may contribute to the pathogenesis of acquired right-sided cardiac lesions in the recipient twin affected with TTTS.

The impact of fetal endoscopic tracheal occlusion in isolated left-sided congenital diaphragmatic hernia on left-sided cardiac dimensions.

OBJECTIVES: Fetal endoscopic tracheal occlusion (FETO) is offered to fetuses with congenital diaphragmatic hernia (CDH) and severe lung hypoplasia to promote lung growth and may secondarily affect left heart growth. The effects of FETO on left heart hypoplasia (LHH) are not described post-CDH repair. METHODS: A retrospective analysis was performed for fetuses with left-sided CDH who underwent FETO and severity-matched controls from 2007 to 2016 at our institution. Echocardiographic, ultrasound, and MRI data were reviewed. Left heart dimensions were assessed prenatally and postnatally. Primary clinical outcome evaluated was death. RESULTS: Twelve FETO patients and 18 controls were identified. Fetal LHH was noted in both groups and worsened after FETO. Postnatal mitral valve dimensions were larger in the FETO group pre-CDH repair (P = .03). Post-CDH repair, mitral valve and left ventricular dimensions were not significantly different between groups (P = .79 and P = .63 respectively) while FETO aortic valve dimensions were smaller (P = .04). Extracorporeal membrane oxygenation use was lower in the FETO group. No associations were found between left heart dimensions and outcomes. CONCLUSION: Although increased lung growth was seen after FETO, fetal LHH persisted with relative normalization seen post-repair. Persistent LHH post-FETO could be secondary to a small contribution of pulmonary venous return to the fetal left heart and increased intrathoracic pressures post-FETO.

Predictive value of oxygenation index for outcomes in left-sided congenital diaphragmatic hernia.

BACKGROUND & OBJECTIVES: Congenital Diaphragmatic Hernia (CDH) is associated with significant morbidity and mortality. This study compares the efficacy of the highest oxygenation index in the first 48 h (HiOI) versus current prenatal indices to predict survival and morbidity. METHODS: Medical records of 50 prenatally diagnosed, isolated, left-sided CDH patients treated from January 2011 to April 2016 were reviewed. Data abstracted included HiOI, lung to head ratio (LHR), observed to expected total fetal lung volume (O/E TFLV), percent liver herniation (%LH), 6 month survival, respiratory support at discharge, ventilator days and length of stay. Data were analyzed using parametric and nonparametric tests and regression analyses as appropriate. RESULTS: HiOI was associated with significantly increased LOS (p<0.001), respiratory support at discharge (p<0.001), greater ventilator days (p=0.001) and higher odds of death (p=0.004) with risk of death increasing by 5% for every one-unit increase in OI. HiOI was statistically a better predictor of LOS than O/E TFLV (p=0.007) and %LH (p=0.02). CONCLUSIONS: In isolated, left-sided CDH patients, HiOI is associated with higher mortality, greater length of stay, more ventilator days and increased respiratory support at discharge. HiOI is a better predictor of length of stay than O/E TFLV and %LH. TYPE OF STUDY: Retrospective Study LEVEL OF EVIDENCE: II.

Isolated coarctation of the aorta in the fetus: A diagnostic challenge.

Isolated coarctation of the aorta (CoA) is estimated by the Centers for Disease Control and Prevention to account for 4%-6% of all congenital heart disease (CHD) in the United States, with a reported prevalence of ~4 per 10 000 live births. Prenatal recognition of coarctation is important as it may improve neonatal survival and reduce morbidity. However, despite advances in imaging and the trend toward detailed aortic arch assessment as part of a comprehensive fetal echocardiogram, isolated CoA may still elude prenatal detection, with potentially lethal consequences if the diagnosis is not suspected and the patent ductus arteriosus (PDA) closes spontaneously in postnatal life. The purpose of this review is to outline the methods of antenatal aortic arch evaluation in the current era, discuss "red flags" that raise the suspicion for CoA, including associated anomalies and serve as a repository of the most up to date information regarding its diagnosis in utero and its perinatal management. Other aortic arch abnormalities, such as interrupted aortic arch, or CoA associated with complex single ventricles, are not included in this review.

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.

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.

Longitudinal myocardial deformation is selectively decreased after pediatric cardiac transplantation: a comparison of children 1 year after transplantation with normal subjects using velocity vector imaging.

The transplanted heart experiences numerous hemodynamic changes during and after cardiac transplantation. This study sought to evaluate the left ventricular myocardial mechanics in the pediatric heart transplant population using Velocity Vector Imaging (VVI). This study retrospectively evaluated 28 heart transplant recipients by echocardiography 12 months after transplantation. Echocardiograms from 28 age- and gender-matched subjects were used as a control group. Peak global longitudinal and circumferential left ventricular strain, systolic strain rate, and diastolic strain rate were obtained. Student's t tests were used to assess differences between the two groups (defined as p ≤ 0.05). The peak global left ventricular longitudinal strain was lower in the transplant group (17.21%) than in the control group (22.14%). The transplant and control groups did not differ significantly in terms of their peak global circumferential strain (20.28% vs. 20.79%, respectively). Similar results were observed for longitudinal and circumferential systolic and diastolic strain rates. The transplant patients showed statistically significant reductions in all peak global longitudinal measures compared with those of the control subjects. Circumferential myocardial deformation appears to be preserved in transplant recipients. This could suggest evidence of ischemia given the known myocardial fiber arrangement of longitudinal fibers toward the endocardial surface, which is also more distal in the coronary arterioles.