Anaesthetic Management of a Neonate with Ebstein’s Anomaly Undergoing a Meningomyelocele Surgery: A Case Report

Ebstein’s Anomaly (EA) is a congenital heart defect characterised by the downward displacement of the posterior and septal leaflets of the tricuspid valve toward the right ventricular apex. Hereby the authors discuss a 16-day-old female neonate with EA who underwent surgery for a ruptured meningocel...

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Main Authors: Prakriti, Monika Yadav, Vaishali Gupta, Komal Yadav, Shikha Garg
Format: Article
Language:English
Published: JCDR Research and Publications Private Limited 2025-01-01
Series:Journal of Clinical and Diagnostic Research
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Online Access:https://www.jcdr.net/articles/PDF/20548/73352_CE(Ra1)__F(Sh)_QC(PS_SHU)_PF1(JY_IS_SS)_PFA_NC(IS)_PN(IS).pdf
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author Prakriti
Monika Yadav
Vaishali Gupta
Komal Yadav
Shikha Garg
author_facet Prakriti
Monika Yadav
Vaishali Gupta
Komal Yadav
Shikha Garg
author_sort Prakriti
collection DOAJ
description Ebstein’s Anomaly (EA) is a congenital heart defect characterised by the downward displacement of the posterior and septal leaflets of the tricuspid valve toward the right ventricular apex. Hereby the authors discuss a 16-day-old female neonate with EA who underwent surgery for a ruptured meningocele. The baby, born at full term, had an Appearance, Pulse, Grimace, Activity and Respiration (APGAR) score of 7 at one minute and 9 at five minutes. Upon further observation, she showed multiple episodes of breath-holding and cyanosis. On examination, an enlarged head and swelling on the lumbar spine led to an ultrasound that confirmed hydrocephalus and meningocele. An Electrocardiogram (ECG) showed supraventricular tachycardia, and a chest X-ray revealed cardiomegaly. The diagnosis of EA was confirmed by two-dimensional (2D) echocardiography. General anaesthesia with inhalational induction was planned. Perioperative considerations for this patient included right-to-left shunting with the possibility of paradoxical emboli, prolonged onset time for intravenous drugs due to pooling and recirculation of blood in the right atrium, increased Pulmonary Vascular Resistance (PVR), perioperative pulmonary hypertension, right and left ventricular dysfunction, and arrhythmias. Key cardiovascular goals for this patient included maintaining normal sinus rhythm, ensuring adequate preload while avoiding significant changes in Systemic Vascular Resistance (SVR) or PVR, avoiding medications and factors that depress myocardial function, and promptly identifying haemodynamic instability or inadequate tissue perfusion with appropriate corrections. The surgery for excision and repair of the meningocele was uneventful, and the neonate was sent to the Neonatal Intensive Care Unit (NICU) for further monitoring.
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institution Kabale University
issn 2249-782X
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publishDate 2025-01-01
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spelling doaj-art-336e145a6389479bb7dfc1ee827670f22025-01-28T11:25:53ZengJCDR Research and Publications Private LimitedJournal of Clinical and Diagnostic Research2249-782X0973-709X2025-01-011901101210.7860/JCDR/2025/73352.20548Anaesthetic Management of a Neonate with Ebstein’s Anomaly Undergoing a Meningomyelocele Surgery: A Case Report Prakriti0Monika Yadav1Vaishali Gupta2Komal Yadav3Shikha Garg4Senior Resident, Department of Anaesthesia, Pt. B.D. Sharma, PGIMS, Rohtak, Haryana, India.Senior Resident, Department of Anaesthesia, Pt. B.D. Sharma, PGIMS, Rohtak, Haryana, IndiaJunior Resident, Department of Anaesthesia, Pt. B.D. Sharma, PGIMS, Rohtak, Haryana, India.Junior Resident, Department of Anaesthesia, Pt. B.D. Sharma, PGIMS, Rohtak, Haryana, IndiaJunior Resident, Department of Anaesthesia, Pt. B.D. Sharma, PGIMS, Rohtak, Haryana, India.Ebstein’s Anomaly (EA) is a congenital heart defect characterised by the downward displacement of the posterior and septal leaflets of the tricuspid valve toward the right ventricular apex. Hereby the authors discuss a 16-day-old female neonate with EA who underwent surgery for a ruptured meningocele. The baby, born at full term, had an Appearance, Pulse, Grimace, Activity and Respiration (APGAR) score of 7 at one minute and 9 at five minutes. Upon further observation, she showed multiple episodes of breath-holding and cyanosis. On examination, an enlarged head and swelling on the lumbar spine led to an ultrasound that confirmed hydrocephalus and meningocele. An Electrocardiogram (ECG) showed supraventricular tachycardia, and a chest X-ray revealed cardiomegaly. The diagnosis of EA was confirmed by two-dimensional (2D) echocardiography. General anaesthesia with inhalational induction was planned. Perioperative considerations for this patient included right-to-left shunting with the possibility of paradoxical emboli, prolonged onset time for intravenous drugs due to pooling and recirculation of blood in the right atrium, increased Pulmonary Vascular Resistance (PVR), perioperative pulmonary hypertension, right and left ventricular dysfunction, and arrhythmias. Key cardiovascular goals for this patient included maintaining normal sinus rhythm, ensuring adequate preload while avoiding significant changes in Systemic Vascular Resistance (SVR) or PVR, avoiding medications and factors that depress myocardial function, and promptly identifying haemodynamic instability or inadequate tissue perfusion with appropriate corrections. The surgery for excision and repair of the meningocele was uneventful, and the neonate was sent to the Neonatal Intensive Care Unit (NICU) for further monitoring.https://www.jcdr.net/articles/PDF/20548/73352_CE(Ra1)__F(Sh)_QC(PS_SHU)_PF1(JY_IS_SS)_PFA_NC(IS)_PN(IS).pdfcardiomegalymeningocelepaediatric anaesthesiatricuspid regurgitation
spellingShingle Prakriti
Monika Yadav
Vaishali Gupta
Komal Yadav
Shikha Garg
Anaesthetic Management of a Neonate with Ebstein’s Anomaly Undergoing a Meningomyelocele Surgery: A Case Report
Journal of Clinical and Diagnostic Research
cardiomegaly
meningocele
paediatric anaesthesia
tricuspid regurgitation
title Anaesthetic Management of a Neonate with Ebstein’s Anomaly Undergoing a Meningomyelocele Surgery: A Case Report
title_full Anaesthetic Management of a Neonate with Ebstein’s Anomaly Undergoing a Meningomyelocele Surgery: A Case Report
title_fullStr Anaesthetic Management of a Neonate with Ebstein’s Anomaly Undergoing a Meningomyelocele Surgery: A Case Report
title_full_unstemmed Anaesthetic Management of a Neonate with Ebstein’s Anomaly Undergoing a Meningomyelocele Surgery: A Case Report
title_short Anaesthetic Management of a Neonate with Ebstein’s Anomaly Undergoing a Meningomyelocele Surgery: A Case Report
title_sort anaesthetic management of a neonate with ebstein s anomaly undergoing a meningomyelocele surgery a case report
topic cardiomegaly
meningocele
paediatric anaesthesia
tricuspid regurgitation
url https://www.jcdr.net/articles/PDF/20548/73352_CE(Ra1)__F(Sh)_QC(PS_SHU)_PF1(JY_IS_SS)_PFA_NC(IS)_PN(IS).pdf
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AT vaishaligupta anaestheticmanagementofaneonatewithebsteinsanomalyundergoingameningomyelocelesurgeryacasereport
AT komalyadav anaestheticmanagementofaneonatewithebsteinsanomalyundergoingameningomyelocelesurgeryacasereport
AT shikhagarg anaestheticmanagementofaneonatewithebsteinsanomalyundergoingameningomyelocelesurgeryacasereport