Fetal magnetic resonance imaging, ultrasound, and echocardiography findings in twin reversed arterial perfusion sequence.

BACKGROUND: Twin reversed arterial perfusion (TRAP) sequence is a rare complication of monochorionic multiple gestation pregnancies, in which the pump twin provides hemodynamic support to a nonviable co-twin (acardius). Fetal magnetic resonance imaging (MRI) is used to detect pump twin abnormalities, particularly brain ischemia, prior to fetal intervention to interrupt umbilical blood flow to the acardius. OBJECTIVE: To summarize the imaging findings of TRAP sequence pregnancies in a large series. MATERIALS AND METHODS: A single-center retrospective review was performed of all TRAP sequence pregnancies referred for fetal MRI (2004-2021). Fetal MRI, ultrasound, and echocardiography data were collected. RESULTS: Eighty-eight TRAP sequence pregnancies with MRI were included (mean gestational age, 19.8±2.8 weeks). Demise of the pump twin was noted in two pregnancies at the time of MRI. By MRI, 12% (10/86) of live pump twins had abnormalities, including 3% (3/86) with brain abnormalities and 9% (8/86) with extra-cranial abnormalities. By echocardiography, 7% (6/86) of pump twins had structural cardiac abnormalities. Three acardius morphological subtypes were identified by MRI: acephalus (55%, 48/88), anceps (39%, 34/88), and amorphous (7%, 6/88). The mean ultrasound acardius to pump twin ratio A/P ratio, calculated for each twin pair as the ratio of the acardius trunk (and head, if present) plus limb volume to the pump twin estimated fetal weight) differed among the three acardius subtypes (P=.03). The mean A/P ratio moderately correlated with pump twin cardiothoracic ratio and combined cardiac output (Pearson's r=0.45 and 0.48, respectively, both P<.001). CONCLUSION: Fetal MRI of TRAP sequence pregnancies found anomalies in a substantial number of pump twins. The three acardius subtypes differed in A/P ratio, which moderately correlated with the pump twin cardiothoracic ratio and combined cardiac output.

Tricuspid atresia with restrictive foramen ovale: A rare combination with implications on fetal growth.

Restrictive atrial communication is rarely reported in tricuspid atresia but when present it can lead to important morbidity. We describe two fetuses with tricuspid atresia with restrictive foramen ovale who were found to have fetal growth failure. Fetal echocardiography detected a restrictive atrial communication by flow acceleration on color Doppler and significant right atrial dilation in one patient; the atrial septum was not well interrogated in the other patient. Restrictive foramen ovale in tricuspid atresia may be associated with fetal growth failure. Color Doppler interrogation of the atrial septum on fetal echocardiogram may help identify this condition prenatally.

Dosing and efficacy of intranasal dexmedetomidine sedation for pediatric transthoracic echocardiography: a retrospective study.

PURPOSE: We designed this retrospective observational study on the use of α2-agonist dexmedetomidine to determine the optimum intranasal dose to achieve sedation for pediatric transthoracic echocardiography and to identify any dose-related adverse effects. METHODS: Outpatient children aged three months to three years with diverse diagnoses of congenital heart disease, including cyanotic cardiac defects, underwent transthoracic echocardiography under dexmedetomidine sedation. Aerosolized intranasal dexmedetomidine was administered with initial doses ranging from 1-3 µg·kg(-1). A rescue dose of 1 µg·kg(-1) was administered if adequate sedation was not achieved within 45 min following the first dose. The primary study outcome was the achievement of adequate sedation to allow transthoracic echocardiography (TTE) scanning, including subxiphoid and suprasternal probe manipulation. RESULTS: Sedation with intranasal dexmedetomidine for transthoracic echocardiography was successful in 62 of the 63 (98%) patients studied, with an intranasal rescue dose required in 13 (21%) patients. Intranasal doses of dexmedetomidine 2.5-3.0 µg·kg(-1) were required for tolerating TTE probe placement, including subxiphoid and suprasternal manipulation, with minimal response and a 90% success rate. Excluding patients who required a second dose of dexmedetomidine, the mean (standard deviation) time from administration to achieving such sedation (onset time) was 26 (8) min for low-dose (1-2 µg·kg(-1)) dexmedetomidine and 28 (8) min for moderate-dose (2.5-3.0 µg·kg(-1)) dexmedetomidine (P = 0.33). Time from administration of low-dose dexmedetomidine to discharge, including TTE scan time, was 80 (14) min, and it increased with moderate-dose dexmedetomidine to 91 (22) min (P = 0.05). Mild to moderate bradycardia and hypotension were observed, but no interventions were required. CONCLUSION: We found that aerosolized intranasal dexmedetomidine offers satisfactory conditions for TTE in children three months to three years of age with an optimal dose of 2.5-3.0 µg·kg(-1)administered under the supervision of a pediatric cardiac anesthesiologist.

Microcephaly is associated with early adverse neurologic outcomes in hypoplastic left heart syndrome.

BACKGROUND: Hypoplastic left heart syndrome (HLHS) is associated with significant mortality and morbidity. Fetal head growth abnormalities have been identified in a subset of HLHS fetuses, but it is unclear whether specific patterns of maladaptive growth affect clinical outcomes. We hypothesized that poor fetal head growth is associated with an increased frequency of adverse clinical outcomes. METHODS: We retrospectively examined a cohort of HLHS patients from midgestation to 1 y of age. Fetal and birth anthropometric measurements were analyzed using the Olsen standard, and clinical outcomes were obtained. RESULTS: A total of 104 HLHS patients were identified over a 12-y period; fetal data were available in 38 cases. HLHS neonates demonstrated a high incidence of microcephaly (12%), small head size (27%), and poor head growth (32%). All-cause mortality was 31% at 30 d and 43% at 1 y. Neurologic outcomes were observed in 12% of patients and were significantly increased with microcephaly (43 vs. 4%; P = 0.02). The average length of hospital stay following stage I palliation was 33.4 ± 33 d, correcting for early death. CONCLUSION: In term nonsyndromic HLHS, fetal and neonatal microcephaly are associated with early adverse neurologic outcomes but not mortality.

Somatic growth trajectory in the fetus with hypoplastic left heart syndrome.

BACKGROUND: Fetal growth abnormalities in hypoplastic left heart syndrome (HLHS) have been documented primarily by birth measurements. Fetal growth trajectory has not been described. We hypothesized that fetal growth trajectory declines across late gestation in this population. METHODS: Infants with a prenatal diagnosis of HLHS and no history of prematurity or a genetic syndrome were identified. Fetal ultrasound measurements and birth anthropometrics were obtained from clinical records. z-Scores for estimated fetal weight (EFWz) and birth weight (BWTz) were compared. BWTz for three neonatal standards were compared. RESULTS: Paired fetal and neonatal data were identified in 33 cases of HLHS. Mean gestational age at ultrasound and birth were 27 and 38 wk, respectively. BWTz was lower than EFWz by a mean of 0.82 (SD: 0.72, P < 0.0001), with 64% of subjects demonstrating a decrease in z-score of >0.5. Umbilical artery (UA) Doppler found no evidence of significant placental insufficiency. Modest differences in BWTz were seen across BWT standards in this cohort. CONCLUSION: The majority of fetuses with HLHS demonstrate decreased growth velocity during later pregnancy, suggesting growth abnormalities manifest in utero. The potential relationship to future clinical outcomes warrants further study.

Neonatal Depression Is Associated With 1-Year Mortality in Critical Congenital Heart Disease.

Background Low 5-minute Apgar scores (AS) are predictive of term and preterm neonatal mortality but have not been well studied in the critical congenital heart disease (CCHD) population. We analyzed US national vital statistics data to evaluate the association between neonatal depression (AS 0-3) and 1-year mortality in CCHD. Methods and Results We performed a retrospective cohort study using 2014 to 2018 Centers for Disease Control and Prevention cohort-linked birth certificate and infant death records. Five-minute AS were categorized as ≤3, 4 to 6, or ≥7. We calculated birth rates and associated mortality rates by AS group in infants with and without CCHD. Multivariable logistic regression analyzed neonatal, maternal, and pregnancy-related risk factors for neonatal depression and 1-year mortality. Of 11 642 neonates with CCHD (0.06% of all births), the 5.8% with AS 0 to 3 accounted for 23.3% of all 1-year CCHD mortality, with 69.9% of deaths occurring within 1 month of life. Gestational age at birth, growth restriction, extracardiac defects, race, and low maternal education were associated with an increased odds of AS 0 to 3 in neonates with CCHD relative to those with AS 7 to 10 on multivariable analysis. AS 0 to 3 was associated with 1-year CCHD mortality after adjusting for these factors, prenatal care, and delivery location (adjusted odds ratio, 14.57 [95% CI, 11.73-18.10]). Conclusions The AS is a routine clinical measure providing important prognostic information in CCHD. These findings suggest that prenatal and perinatal factors, beyond those included in current risk stratification tools, are important for CCHD outcomes. Multidisciplinary collaboration to understand the pathophysiology underlying neonatal depression may help identify interventions to improve CCHD mortality rates.

The low incidence of clinically significant heart disease in school-age children following COVID-19. / Baja incidencia de cardiopatía clínicamente significativa en niños en edad escolar tras padecer COVID-19.

The burden of clinically-apparent cardiac injury secondary to coronavirus disease 2019 (COVID-19) in school-age children is unclear. Over 12 months at a large academic pediatric hospital in the Midwestern portion of the United States, there were 1481 COVID-19 positive non-hospitalized otherwise healthy schoolaged children with 195 having cardiac testing performed. While incidental findings occurred, no definitive COVID-19 related cardiac pathology was discovered. Additionally, only ~3% of children had acute cardiac symptoms necessitating evaluation by pediatric cardiology. School-age children who were not hospitalized for COVID-19 have a very low risk of having clinically significant cardiac damage and are more likely to discover incidental findings.

No es clara la carga de morbimortalidad de la lesión cardíaca clínicamente evidente secundaria a la enfermedad por coronavirus de 2019 (COVID-19) en los niños en edad escolar. A lo largo de 12 meses, en un importante hospital pediátrico académico en la región del medio oeste de Estados Unidos, hubo 1481 casos de COVID-19 sin hospitalización en niños en edad escolar por lo demás sanos, en quienes se hicieron 195 pruebas cardíacas. Si bien aparecieron hallazgos fortuitos, no se descubrió ninguna patología cardíaca relacionada con la COVID-19. Además, ~3 % de los niños solamente tuvieron síntomas cardíacos agudos que requirieron una evaluación por el área de cardiología pediátrica. Los niños que no fueron hospitalizados por COVID-19 tienen un riesgo muy bajo de desarrollar daño cardíaco clínicamente significativo y son más propensos a presentar hallazgos fortuitos.

Severe Maternal Morbidity in Pregnancies Complicated by Fetal Congenital Heart Disease.

Background: Maternal risk factors for fetal congenital heart disease (CHD) may also be associated with delivery complications in the mother. Objectives: This study aimed to determine the prevalence of and risk factors for severe maternal morbidity (SMM) and maternal hospital transfer in pregnancies complicated by fetal CHD. Methods: A population-based retrospective cohort study utilizing linked Ohio birth certificates and birth defect data for all live births from 2011 to 2015 was performed. The primary outcome was composite SMM. Secondary outcome was maternal hospital transfer prior to delivery. Pregnancies with isolated fetal CHD were compared to pregnancies with no fetal anomalies and isolated fetal cleft lip/palate (CLP). Results: A total of 682,929 mothers with live births were included. Of these, 5,844 (0.85%) mothers had fetal CHD, and 963 (0.14%) had fetal CLP. SMM in pregnancies with fetal CHD was higher than that in those with no anomalies (3.6% vs 1.9%, P < 0.001) or CLP (3.6% vs 1.9%, P = 0.006). After adjusting for known risk factors, fetal CHD remained independently associated with SMM when compared to no fetal anomalies (adjusted relative risk [adjRR]: 1.81, 95% CI: 1.58-2.08) and CLP (adjRR: 1.81, 95% CI: 1.12-2.92). Maternal hospital transfer occurred more frequently in fetal CHD cases vs for those without fetal anomalies with an increased adjusted risk (adjRR: 3.65, 95% CI: 3.14-4.25). Conclusions: Pregnancies with isolated fetal CHD have increased risk of SMM and maternal hospital transfer after adjusting for known risk factors. This may inform delivery planning for mothers with fetal CHD. Understanding the biological mechanisms may provide insight into other adverse perinatal outcomes in this population.

Partnering With Stakeholders to Inform the Co-Design of a Psychosocial Intervention for Prenatally Diagnosed Congenital Heart Disease.

Input from diverse stakeholders is critical to the process of designing healthcare interventions. This study applied a novel mixed-methods, stakeholder-engaged approach to co-design a psychosocial intervention for mothers expecting a baby with congenital heart disease (CHD) and their partners to promote family wellbeing. The research team included parents and clinicians from 8 health systems. Participants were 41 diverse parents of children with prenatally diagnosed CHD across the 8 health systems. Qualitative data were collected through online crowdsourcing and quantitative data were collected through electronic surveys to inform intervention co-design. Phases of intervention co-design were: (I) Engage stakeholders in selection of intervention goals/outcomes; (II) Engage stakeholders in selection of intervention elements; (III) Obtain stakeholder input to increase intervention uptake/utility; (IV) Obtain stakeholder input on aspects of intervention design; and (V) Obtain stakeholder input on selection of outcome measures. Parent participants anticipated the resulting intervention, HEARTPrep, would be acceptable, useful, and feasible for parents expecting a baby with CHD. This model of intervention co-design could be used for the development of healthcare interventions across chronic diseases.

Prenatal heart block screening in mothers with SSA/SSB autoantibodies: Targeted screening protocol is a cost-effective strategy.

OBJECTIVE: Maternal anti-Ro/SSA and anti-La/SSB antibodies can lead to fetal complete heart block (CHB). Current guidelines recommend weekly echocardiographic screening between 16 and 28 weeks gestation. Given the cost of screening and the rarity of conduction abnormalities in fetuses of mothers with low anti-Ro levels (<50 U/mL), we sought to identify a strategy that optimizes resource utilization. DESIGN: Decision analysis cost-utility modeling was performed for three screening paradigms: "standard screening" (SS) in which mid-gestation mothers are screened weekly, "limited screening" (LS) in which fetal echocardiograms are avoided unless the fetus develops bradycardia, and "targeted screening by maternal antibody level" (TS) in which only high anti-Ro values warrant weekly screening. A systematic review of existing literature and institutional cost data were used to define model inputs. RESULTS: The average cost of LS, TS, and SS was $8566, $11 038, and $23 279, respectively. SS was cost-ineffective with an incremental cost-effectiveness ratio (ICER) of $322 756 while TS was cost-effective with an ICER of $43 445. CONCLUSION: While the efficacy of fetal intervention for first or second degree AV block remains unclear, this analysis supports utilizing antibody levels to stratify this population for optimized surveillance for CHB. SS is cost-ineffective and results in resource overutilization.

Baja incidencia de cardiopatía clínicamente significativa en niños en edad escolar tras padecer COVID-19 / The low incidence of clinically significant heart disease in school-age children following COVID-19

No es clara la carga de morbimortalidad de la lesión cardíaca clínicamente evidente secundaria a la enfermedad por coronavirus de 2019 (COVID-19) en los niños en edad escolar. A lo largo de 12 meses, en un importante hospital pediátrico académico en la región del medio oeste de Estados Unidos, hubo 1481 casos de COVID-19 sin hospitalización en niños en edad escolar por lo demás sanos, en quienes se hicieron 195 pruebas cardíacas. Si bien aparecieron hallazgos fortuitos, no se descubrió ninguna patología cardíaca relacionada con la COVID-19. Además, ~3 % de los niños solamente tuvieron síntomas cardíacos agudos que requirieron una evaluación por el área de cardiología pediátrica. Los niños que no fueron hospitalizados por COVID-19 tienen un riesgo muy bajo de desarrollar daño cardíaco clínicamente significativo y son más propensos a presentar hallazgos fortuitos.

The burden of clinically-apparent cardiac injury secondary to coronavirus disease 2019 (COVID-19) in school-age children is unclear. Over 12 months at a large academic pediatric hospital in the Midwestern portion of the United States, there were 1481 COVID-19 positive non-hospitalized otherwise healthy schoolaged children with 195 having cardiac testing performed. While incidental findings occurred, no definitive COVID-19 related cardiac pathology was discovered. Additionally, only ~3 % of children had acute cardiac symptoms necessitating evaluation by pediatric cardiology. School-age children who were not hospitalized for COVID-19 have a very low risk of having clinically significant cardiac damage and are more likely to discover incidental findings.

First-Line Antiarrhythmic Transplacental Treatment for Fetal Tachyarrhythmia: A Systematic Review and Meta-Analysis.

BACKGROUND: There is no consensus on the most effective and best tolerated first-line antiarrhythmic treatment for fetal tachyarrhythmia. The purpose of this systematic review and meta-analysis was to compare the efficacy, safety, and fetal-maternal tolerance of first-line monotherapies for fetal supraventricular tachycardia and atrial flutter. METHODS AND RESULTS: A comprehensive search of several databases was conducted through January 2017. Only studies that made a direct comparison between first-line treatments of fetal tachyarrhythmia were included. Outcomes of interest were termination of fetal tachyarrhythmia, fetal demise, and maternal complications. Ten studies met inclusion criteria, with 537 patients. Overall, 291 patients were treated with digoxin, 137 with flecainide, 102 with sotalol, and 7 with amiodarone. Digoxin achieved a lower rate of supraventricular tachycardia termination compared with flecainide (odds ratio [OR]: 0.773; 95% confidence interval [CI], 0.605-0.987; I2=34%). In fetuses with hydrops fetalis, digoxin had lower rates of tachycardia termination compared with flecainide (OR: 0.412; 95% CI, 0.268-0.632; I2=0%). There was no significant difference in the incidence of maternal side effects between digoxin and flecainide groups (OR: 1.134; 95% CI, 0.129-9.935; I2=80.79%). The incidence of maternal side effects was higher in patients treated with digoxin compared with sotalol (OR: 3.148; 95% CI, 1.468-6.751; I2=0%). There was no difference in fetal demise between flecainide and digoxin (OR: 0.767; 95% CI, 0.140-4.197; I2=44%). CONCLUSIONS: Flecainide may be more effective treatment than digoxin as a first-line treatment for fetal supraventricular tachycardia.

Associations Between Day of Admission and Day of Surgery on Outcome and Resource Utilization in Infants With Hypoplastic Left Heart Syndrome Who Underwent Stage I Palliation (from the Single Ventricle Reconstruction Trial).

Newborns with hypoplastic left heart syndrome and other single right ventricular variants require substantial health care resources. Weekend acute care has been associated with worse outcomes and increased resource use in other populations but has not been studied in patients with single ventricle. Subjects of the Single Ventricle Reconstruction trial were classified by whether they had a weekend admission and by day of the week of Norwood procedure. The primary outcome was hospital length of stay (LOS); secondary outcomes included transplant-free survival, intensive care unit (ICU) LOS, and days of mechanical ventilation. The Student's t test with log transformation and the Wilcoxon rank-sum test were used to analyze associations. Admission day was categorized for 533 of 549 subjects (13% weekend). The day of the Norwood was Thursday/Friday in 39%. There was no difference in median hospital LOS, transplant-free survival, ICU LOS, or days ventilated for weekend versus non-weekend admissions. Day of the Norwood procedure was not associated with a difference in hospital LOS, transplant-free survival, ICU LOS, or days ventilated. Prenatally diagnosed infants born on the weekend had lower mean birth weight, younger gestational age, and were more likely to be intubated but did not have a difference in measured outcomes. In conclusion, in this cohort of patients with single right ventricle, neither weekend admission nor end-of-the-week Norwood procedure was associated with increased use of hospital resources or poorer outcomes. We speculate that the complex postoperative course following the Norwood procedure outweighs any impact that day of admission or operation may have on these outcomes.