79 research outputs found

    Ross-Konno and Endocardial Fibroelastosis Resection After Hybrid Stage I Palliation in Infancy: Successful Staged Left-Ventricular Rehabilitation and Conversion to Biventricular Circulation After Fetal Diagnosis of Aortic Stenosis

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    We report a patient who presented during fetal life with severe aortic stenosis, left-ventricular dysfunction, and endocardial fibroelastosis (evolving hypoplastic left heart syndrome). Management involved in utero and postnatal balloon aortic valvuloplasty for partial relief of obstruction and early postnatal hybrid stage I palliation until recovery of left-ventricular systolic function had occurred. The infant subsequently had successful conversion to a biventricular circulation by combining resection of endocardial fibroelastosis with single-stage Ross-Konno, aortic arch reconstruction, hybrid takedown, and pulmonary artery reconstruction

    Neonatal Survival After Serial Amnioinfusions for Bilateral Renal Agenesis: The Renal Anhydramnios Fetal Therapy Trial

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    IMPORTANCE: Early anhydramnios during pregnancy, resulting from fetal bilateral renal agenesis, causes lethal pulmonary hypoplasia in neonates. Restoring amniotic fluid via serial amnioinfusions may promote lung development, enabling survival. OBJECTIVE: To assess neonatal outcomes of serial amnioinfusions initiated before 26 weeks\u27 gestation to mitigate lethal pulmonary hypoplasia. DESIGN, SETTING, AND PARTICIPANTS: Prospective, nonrandomized clinical trial conducted at 9 US fetal therapy centers between December 2018 and July 2022. Outcomes are reported for 21 maternal-fetal pairs with confirmed anhydramnios due to isolated fetal bilateral renal agenesis without other identified congenital anomalies. EXPOSURE: Enrolled participants initiated ultrasound-guided percutaneous amnioinfusions of isotonic fluid before 26 weeks\u27 gestation, with frequency of infusions individualized to maintain normal amniotic fluid levels for gestational age. MAIN OUTCOMES AND MEASURES: The primary end point was postnatal infant survival to 14 days of life or longer with dialysis access placement. RESULTS: The trial was stopped early based on an interim analysis of 18 maternal-fetal pairs given concern about neonatal morbidity and mortality beyond the primary end point despite demonstration of the efficacy of the intervention. There were 17 live births (94%), with a median gestational age at delivery of 32 weeks, 4 days (IQR, 32-34 weeks). All participants delivered prior to 37 weeks\u27 gestation. The primary outcome was achieved in 14 (82%) of 17 live-born infants (95% CI, 44%-99%). Factors associated with survival to the primary outcome included a higher number of amnioinfusions (P = .01), gestational age greater than 32 weeks (P = .005), and higher birth weight (P = .03). Only 6 (35%) of the 17 neonates born alive survived to hospital discharge while receiving peritoneal dialysis at a median age of 24 weeks of life (range, 12-32 weeks). CONCLUSIONS AND RELEVANCE: Serial amnioinfusions mitigated lethal pulmonary hypoplasia but were associated with preterm delivery. The lower rate of survival to discharge highlights the additional mortality burden independent of lung function. Additional long-term data are needed to fully characterize the outcomes in surviving neonates and assess the morbidity and mortality burden

    Prenatal findings in total anomalous pulmonary venous return: a diagnostic road map starts with obstetric screening views.

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    ObjectivesOptimal perinatal management of total anomalous pulmonary venous return (TAPVR) involves timely identification followed by surgical correction. Antenatal diagnosis, however, has long been a challenge. We aimed to identify consistent prenatal sonographic features in this condition in a large cohort in whom the diagnosis was made antenatally and confirmed postnatally.MethodsWe conducted a systematic retrospective review of the 2-dimensional and Doppler sonographic features that had helped make the diagnosis of TAPVR at our institution from 2001 to 2012.ResultsTwenty-six patients had prenatal diagnosis of TAPVR (mean gestational age, 24.1 weeks). Four of the fetuses with a prenatal diagnosis represented isolated cases of TAPVR; 22 had heterotaxy syndrome, additional cardiac abnormalities, or both. Prenatally diagnosed abnormal pulmonary venous connections were supracardiac (type I) in 18 cases, cardiac (type II) in 1, and infradiaphragmatic (type III) in 7. Lack of a visible connection of the pulmonary veins to the atrium (100%) and the presence of a visible venous confluence on axial 4-chamber views (96%) were the most consistent findings. Cardiac asymmetry and the presence of additional vertical venous channels on 3-vessel or axial abdominal views were also noted but less consistently. Abnormal pulmonary venous spectral Doppler findings were present in 25 of the 26 fetuses.ConclusionsThe diagnosis of TAPVR can be suspected on standard axial views included in second-trimester obstetric screening examinations of the fetal heart and confirmed on fetal echocardiography with the use of pulsed wave Doppler imaging. Clues recognizable on obstetric sonographic screening have the potential to contribute to increasing the diagnostic yield for prenatal detection of TAPVR

    Impact of congenital heart disease on outcomes among pediatric patients hospitalized for COVID-19 infection

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    Abstract Background COVID-19 infection is generally regarded as an acute self-limiting illness in children, but it can cause significant morbidity and mortality in both healthy and high-risk children. There are limited data on the outcomes of children with congenital heart disease (CHD) and COVID-19. This study aimed to examine the risks of mortality, in-hospital cardiovascular and non-cardiovascular complications in this patient population. Methods We analyzed data from hospitalized pediatric patients from 2020 using the nationally representative National Inpatient Sample (NIS). Children hospitalized for COVID-19 were included, and weighted data were used to compare in-hospital mortality and morbidities between children with and without CHD. Results Out of 36,690 children admitted with a diagnosis of COVID-19 infection(ICD-10 code:U07.1 and B97.29) during calendar year 2020, 1240 (3.4%) had CHD. The risk of mortality in children with CHD was not significantly higher than those without CHD(1.2% vs. 0.8%, p = 0.50), with adjusted OR (aOR) of 1.7 (95% CI: 0.6–5.3). Tachyarrhythmias and heart block were more likely in CHD children with an aOR of 4.2 (95% CI: 1.8–9.9) and aOR of 5.0 (95% CI: 2.4–10.8), respectively. Similarly, respiratory failure [aOR = 2.0 (1.5–2.8)], respiratory failure requiring non-invasive mechanical ventilation [aOR = 2.7 (1.4–5.2)] and invasive mechanical ventilation [aOR = 2.6 (1.6-4.0)], and acute kidney injury [aOR = 3.4 (2.2–5.4)] were all significantly higher among patients with CHD. Median length of hospital stay in children with CHD was longer than those without CHD [5 days (IQR: 2–11) vs. 3 days (IQR: 2–5), p = < 0.001]. Conclusions Children with CHD hospitalized with COVID-19 infection were at increased risk of serious cardiovascular and non-cardiovascular adverse clinical outcomes. They also had increased length of hospital stay and utilization of healthcare resources
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