Impact of fetal haemodynamics on surgical and neurodevelopmental outcomes in patients with Ebstein anomaly and tricuspid valve dysplasia

2022 ◽  
pp. 1-12
Author(s):  
Min Bao ◽  
Edgar Jaeggi ◽  
Liqun Sun ◽  
Fu-Tsuen Lee ◽  
Renee Sananes ◽  
...  
Keyword(s):  
Tricuspid Valve ◽  
Motor Skills ◽  
Umbilical Vein ◽  
Brain Mri ◽  
Ascending Aorta ◽  
Neurodevelopmental Outcomes ◽  
Ebstein Anomaly ◽  
Descending Aorta ◽  
Neurodevelopmental Delay

Abstract Objectives: To evaluate the impact of fetal haemodynamics on surgical and neurodevelopmental outcomes in severe Ebstein anomaly and tricuspid valve dysplasia. Methods: Thirty-four fetuses with Ebstein anomaly/tricuspid valve dysplasia were referred from 2013 to 2019 for fetal echocardiography and clinical management. Nineteen fetuses with Ebstein anomaly/tricuspid valve dysplasia and 30 controls underwent cardiovascular magnetic resonance to quantify the fetal blood flow and to calculate cerebral oxygen delivery (cDO2) and consumption (cVO2). The 3D steady-state free precession acquisition was used to measure fetal brain volume. Surgical outcome, brain MRI, and neurodevelopmental follow-up were reviewed. Results: Twenty-six fetuses were live born (76%) and survival (65%) at a mean follow-up of 4 years. Nine fetuses had a brain MRI before discharge, and all had clinically silent injuries and volume loss. At 18 months, five single-ventricle patients had a neurodevelopmental delay in cognition and language (mean percentile: 11th), with gross-motor skills more affected than fine-motor skills (mean percentiles: 4th and 34th). Fetuses with Ebstein anomaly/tricuspid valve dysplasia had smaller brains, lower combined ventricular output, ascending aorta, superior caval vien and umbilical vein flows, lower oxygen saturation in ascending aorta and superior caval vien, lower cDO2 and cVO2 (p < 0.05). Superior caval vien/combined ventricular output and descending aorta/combined ventricular output ratios were lower in fetuses with circular shunt (p < 0.05). Fetuses requiring the Starnes procedure tended to have smaller brains, lower combined ventricular output, superior caval vien, descending aorta, and umbilical vein flows. Conclusions: All patients with Ebstein anomaly/tricuspid valve dysplasia are at high risk of neurodevelopmental delay and warrant follow-up. Fetal cardiovascular magnetic resonance revealed impaired brain growth with diminished cerebral blood flow and cDO2, the extenting dependent on the severity of the haemodynamic compromise.

Neurodevelopmental Outcomes after Neonatal Mechanical Ventilation

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Eman Ahmed Zaky ◽  
Hebatallah AM Shaaban ◽  
Mohamed OA Dawoud ◽  
Kareem SEF Madbouly ◽  
Shaymaa M Deifalla
Keyword(s):  
Mechanical Ventilation ◽  
Rating Scale ◽  
Neurodevelopmental Outcomes ◽  
Neurodevelopmental Delay ◽  
Increased Risk ◽  
Study Results ◽  
Extremely Preterm ◽  
Extremely Preterm Infants ◽  
Significant Difference

Abstract Background A majority of extremely preterm infants are treated with mechanical ventilation, which is associated with an increased risk for future development of chronic lung disease, neonatal brain damage, and neurodevelopmental impairments. Objectives The aim of the current study was to evaluate the current and follow up neurodevelopmental status of an Egyptian sample of newly and previously discharged mechanically ventilated infants following them up for a period of 6 months for the earlier group and a year for the latter. Patients and Methods The current study was designed to be a descriptive study with retrospective (50 infants) and prospective (50 infants) domains. It was carried out on 100 neonates who were recruited from the Pediatric Neonatology Clinic, Children's Hospital and Neonatal Intensive Care Unit, Ain Shams University using clinical evaluation, Bayley Scales of Infant Development, and Childhood Autism Rating Scale (CARS).. Results There was statistically significant negative correlation between CARS score and Bayley Scale by using Composite Score (Cognitive, Language and Motor) on first assessment and follow up in the Prospective group while no correlation was found in the retrospective group. The study results showed that there was no statistical significant difference between two groups as regards gestational age, gender, residency, consanguinity, maternal disease, maturity, mode of delivery, respiratory distress, duration of stay in NICU, duration on mechanical ventilation, weight on admission, audiometry and fundus examination (P &gt; 0.05). Conclusion Using a mechanical ventilator in the neonatal period for a prolonged duration increased the risk for ASD and neurodevelopmental delay. Future studies on large samples are recommended from multicenters to confirm the validity of such findings, Bayley scale is a predicative for neurodevelopmental delay in neonates with long duration stay at NICU especially preterms with low birth weight.

scholarly journals The Management and Outcome of Higher Order Multifetal Pregnancies: Obstetric, Neonatal and Follow-up Data

Twin Research ◽  
2001 ◽  
Vol 4 (1) ◽  
pp. 4-11
Author(s):  
Mark H. Yudin ◽  
Elizabeth V. Asztalos ◽  
Ann Jefferies ◽  
Jon F.R. Barrett
Keyword(s):  
Statistical Analysis ◽  
Motor Skills ◽  
Gestational Age ◽  
Neurodevelopmental Outcome ◽  
Higher Order ◽  
Neurodevelopmental Outcomes ◽  
Significant Difference ◽  
Before And After

AbstractThe objective of this study was to describe current obstetric, neonatal, and long-term neurodevelopmental outcomes of higher order multifetal gestations (≥ 3 fetuses) in the 1990s. We also intended to identify a target gestational age at which neonatal and neurodevelopmental morbidities are low. Records from all multifetal pregnancies (≥ 3 viable fetuses ≥ 20 weeks gestation) delivered at the two perinatal centers in Toronto, Ontario, Canada during the study period (January 1, 1990–December 31, 1996) were reviewed. Data were collected on obstetric, neonatal, and long-term neurodevelopmental outcomes. Follow up data were gathered regarding the presence of a severe deficit in four categories (vision, hearing, cognition, and motor skills). Statistical analysis was performed to determine a gestational age at which a significant decrease in deficit occurred. During the study period 165 multifetal pregnancies were delivered. This resulted in 511 fetuses, of which 496 were live births. Of these 496 infants, 453 survived to discharge. Follow up data were obtained on 332 (73.3 per cent) infants. Infant survival increased with gestational age, and was approximately 90 per cent or greater at 26 weeks or more. Of all infants followed, the proportion of those without deficit increased with increasing gestational age, such that the per cent without deficit was 96.9 at 31 weeks or greater. Of all infants followed, 301 (90.7 per cent) had no deficit. Statistical analysis revealed a significant difference in long-term neurodevelopmental outcome between infants born before and after 28 weeks gestation. The incidence of a major deficit was 44.1 per cent for those born earlier than and 5.4 per cent for those born later than this gestational age (p = 0.001). In our cohort, survival figures were high. Even in lower gestational groupings, survival was high, but not without serious concerns about severe morbidity. This information is useful when counseling parents of higher order multifetal pregnancies.

Neurotoxicant Exposures

2019 ◽  
pp. 212-246
Author(s):  
Roberta F. White ◽  
Caitlin Brand ◽  
Birgit Claus Henn ◽  
Patricia A. Janulewicz
Keyword(s):  
Brain Damage ◽  
Motor Skills ◽  
Scientific Evidence ◽  
Clinical Manifestations ◽  
Neurodevelopmental Outcomes ◽  
The Central Nervous System ◽  
Cns Dysfunction ◽  
Cns Effects ◽  
Neuropsychological Findings

This chapter reviews the neuropsychological consequences of exposure to neurotoxicants, which are defined as chemicals to which individuals are exposed in the environment that adversely affect the central nervous system (CNS). The scientific evidence linking exposures to these chemicals and CNS dysfunction is reviewed. The clinical manifestations of neurotoxicant-induced brain damage are discussed, and a system for diagnosing the effects of acute and chronic exposures is outlined. Clinical neuropsychological approaches to assessing the effects of exposure on cognition, motor skills, mood, and neuropsychiatric and neurodevelopmental outcomes are considered. Differential diagnosis of positive neuropsychological findings, recovery from the behavioral effects of neurointoxication, and treatment and follow-up recommendations are discussed. Finally, the chapter summarizes the research and clinical challenges to understanding the CNS effects of exposures to neurotoxicants. Many chemicals are neurotoxic, and different chemicals affect the CNS in differing, highly specific ways. Thus, each section on neurotoxicant-induced brain damage begins by discussing issues relating to neurotoxicants in general, followed by a detailed discussion of lead effects on the CNS. Lead was chosen because exposure to it throughout the lifespan has been studied extensively. Tables listing the research studies that have been published on this topic through 2017 are provided. These tables summarize research methodology, populations studied, assessment of lead exposure, outcomes analyzed, and results. Separate tables outline the neuropsychological correlates that can be observed in adulthood when the exposure occurs in adulthood, in childhood, and across the lifespan.

scholarly journals Time-Resolved Three-Dimensional Contrast-Enhanced Magnetic Resonance Angiography in Patients with Chronic Expanding and Stable Aortic Dissections

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Michael Trojan ◽  
Fabian Rengier ◽  
Drosos Kotelis ◽  
Matthias Müller-Eschner ◽  
Sasan Partovi ◽  
...  
Keyword(s):  
Three Dimensional ◽  
False Lumen ◽  
Ascending Aorta ◽  
Stable Group ◽  
Time Resolved ◽  
Descending Aorta ◽  
Peak Intensity ◽  
Contrast Enhanced ◽  
Aortic Dissections

Objective. To prospectively evaluate our hypothesis that three-dimensional time-resolved contrast-enhanced magnetic resonance angiography (TR-MRA) is able to detect hemodynamic alterations in patients with chronic expanding aortic dissection compared to stable aortic dissections. Materials and Methods. 20 patients with chronic or residual aortic dissection in the descending aorta and patent false lumen underwent TR-MRA of the aorta at 1.5 T and repeated follow-up imaging (mean follow-up 5.4 years). 7 patients showed chronic aortic expansion and 13 patients had stable aortic diameters. Regions of interest were placed in the nondissected ascending aorta and the false lumen of the descending aorta at the level of the diaphragm (FL-diaphragm level) resulting in respective time-intensity curves. Results. For the FL-diaphragm level, time-to-peak intensity and full width at half maximum were significantly shorter in the expansion group compared to the stable group (p=0.027 and p=0.003), and upward and downward slopes of time-intensity curves were significantly steeper (p=0.015 and p=0.005). The delay of peak intensity in the FL-diaphragm level compared to the nondissected ascending aorta was significantly shorter in the expansion group compared to the stable group (p=0.01). Conclusions. 3D TR-MRA detects significant alterations of hemodynamics within the patent false lumen of chronic expanding aortic dissections compared to stable aortic dissections.

Abstract 19334: Management and Outcomes of Acute Retrograde Type A Aortic Dissection: Insights From the International Registry of Acute Aortic Dissection

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Foeke J Nauta ◽  
Joon Bum Kim ◽  
Himanshu J Patel ◽  
Mark D Peterson ◽  
Hans-Henning Eckstein ◽  
...  
Keyword(s):  
Aortic Dissection ◽  
Acute Aortic Dissection ◽  
Innominate Artery ◽  
Type A ◽  
Ascending Aorta ◽  
Early Mortality ◽  
Long Term Results ◽  
Descending Aorta ◽  
International Registry

Background: Presentations and outcomes of acute aortic dissection (AD) with an entry tear in the ascending aorta may differ from retrograde dissection with an entry tear in the descending aorta. However, guidelines recommend urgent surgical repair for both entities. Methods and Results: All patients with AD enrolled in the International Registry of Acute Aortic Dissection (IRAD) between 1996 and 2014 were analyzed. We identified 99 patients (67 men; 63.2±14.0 years) with an entry tear in the descending aorta and retrograde extension into the arch or ascending aorta. Overall, independent predictors of retrograde type A AD were increasing age(OR 1.0; 95% CI, 1.0 to 1.0; P=0.004), history of cocaine abuse (OR 4.9; 95% CI, 1.7 to 13.6; P=0.003), back pain at presentation (OR 2.1; 95% CI, 1.3 to 3.3; P=0.002), and non-white race (OR 0.4; 95% CI, 0.2 to 0.6; P<0.001). During initial hospitalization, 44 of these patients were managed medically (MED), 33 with open surgery(SURG) and 22with endovascular therapy (ENDO). Patients in the SURG group presented with larger ascending aortic diameters than MED and ENDO patients (P=0.04). The majority of the MED (72.7%) and ENDO (86.4%) patients had AD extension confined to zone 1 (proximal arch, P<0.001), whereas most of the SURG patients (71.8%) presented with AD extension into zone 0 (proximal to the innominate artery, P<0.001). Early (30-day or in-hospital) mortality rates of the MED, SURG and ENDO groups were 9.1%, 18.2%, and 13.6%, respectively (P=0.51), with 5-year survival of 86.7%, 80.0%, and 90.9%, respectively (mean follow-up, 3.3 years, log rank P=0.67). A trend of favorable early mortality was observed in patients with retrograde extension till zone 1 (8.6%) versus into zone 0 (18.6%, P=0.14).Early mortality of patients with retrograde type A AD (12.9%) was lower than those with type A AD (20.0%, P=0.001), while 5-year survival was similar (86.8% and 89.5%, respectively, mean follow-up, 3.0 years, log rank P=0.96). Conclusion: There is a subset of patients with acute retrograde type A AD who can be managed non-operatively with acceptable short and long-term results. This implies that a selective approach may be reasonable, particularly among those with proximal extension limited to the arch distal to the innominate artery.

Thoracic Endovascular Aortic Repair for Retrograde Type A Intramural Hematoma With Intimal Disruption in the Descending Aorta

2021 ◽  
pp. 152660282110612
Author(s):  
Yingliang Wang ◽  
Songlin Song ◽  
Chen Zhou ◽  
Wenying Zhu ◽  
Jiacheng Liu ◽  
...  
Keyword(s):  
Adverse Events ◽  
Intramural Hematoma ◽  
Type A ◽  
Ascending Aorta ◽  
Thoracic Endovascular Aortic Repair ◽  
Endovascular Aortic Repair ◽  
Descending Aorta ◽  
Aortic Repair ◽  
Intimal Tear

Purpose: To evaluate the safety and efficacy of thoracic endovascular aortic repair (TEVAR) for retrograde type A intramural hematoma (IMH) with intimal disruption in the descending aorta and report our endovascular therapeutic experience. Materials and Methods: From January 2014 to October 2020, a total of 24 consecutive patients with retrograde type A IMH with intimal disruption (intimal tear or ulcer-like projection) in the descending aorta underwent TEVAR. The demographics, clinical characteristics, treatment details, imaging information, and follow-up results were reviewed. Results: Among all patients with retrograde type A IMH, 13 (54.2%) patients presented with ulcer-like projection and 11 (45.8%) with intimal tear (aortic dissection) in the descending aorta. Successful TEVAR was achieved in all patients. There was no 30-day mortality. During a mean follow-up of 37.5 months, 1 patient (4.2%) developed permanent paralysis, 1 patient (4.2%) underwent reintervention due to the expansion of the aorta distal to the stent resulting from the enlargement of distal intimal tear at the 2 month follow up, and no other adverse events were observed. The latest computed tomographic angiography images showed that the maximum diameter of the ascending aorta and descending aorta significantly decreased after TEVAR (both p<0.001), and the IMH/false lumen in the ascending aorta and the descending thoracic aorta were completely absorbed. Conclusion: Thoracic endovascular aortic repair for selected patients with retrograde type A intramural hematoma that presented with intimal disruption in the descending aorta is feasible and efficient, but close surveillance is needed to manage aortic-related adverse events.

Relationship of cognitive function, quality of life (QOL), and neuroimaging in primary CNS lymphoma (PCNSL) survivors.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 2040-2040 ◽  
Author(s):  
Nancy Diane Doolittle ◽  
Agnieszka Korfel ◽  
Meredith A. Lubow ◽  
Elisabeth Schorb ◽  
Uwe S. Schlegel ◽  
...  
Keyword(s):  
Executive Function ◽  
Motor Skills ◽  
Verbal Memory ◽  
Brain Mri ◽  
Primary Cns Lymphoma ◽  
Cns Lymphoma ◽  
Linear Measurements ◽  
Delayed Treatment ◽  
The Mean

2040 Background: Delayed treatment-related neurotoxicity in PCNSL is a significant problem since improved treatments have increased survival. The study purpose is to describe and correlate neuropsychologic (NP), QOL and neuroimaging outcomes as neurotoxicity indicators. Methods: Four centers in Germany and U.S. prospectively evaluated PCNSL patients (pts) in complete remission (CR) for 2 yrs or more, treated as shown in the Table. NP tests evaluated attention/executive function, verbal memory, motor skills, and QOL (Correa, Ann Oncol 2007). Brain MRI was obtained; the size of T2 abnormalities was determined using two perpendicular linear measurements where hyperintensities were largest and the sum of the measurements was calculated. Differences in total T2 among treatments were compared using analysis of variance; correlations between total T2 and NP or QOL results were assessed using Pearson’s correlation coefficient (r). Results: From Feb 2009 to Feb 2011, 80 pts were evaluated (43 male; median age, 59; median KPS, 80). Median follow-up from diagnosis to evaluation was 5.5 yrs. Total T2 abnormalities were significantly different among treatments (p = 0.0006). The mean area of total T2 in pts treated with WBRT was significantly higher and more than twice the mean of any of the other 3 treatments. Total T2 abnormalities were negatively associated with NP results ie. attention/executive function, r = -0.38 (p = 0.0006), verbal memory, r = -0.23 (p = 0.042), motor skills, r = -0.28 (p = 0.016), composite score, r = -0.34 (p = 0.002); and functional/global QOL (higher total T2 associated with lower QOL). Conclusions: This large PCNSL series in long-term (LT) CR reveals higher total T2 abnormalities in pts treated with WBRT, which are associated with poorer cognitive performance and lower QOL at LT follow-up. Enhanced chemotherapy results in exciting LT survival and function. [Table: see text]

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