scholarly journals Targeted Neonatal Echocardiography-Guided Therapy in Vein of Galen Aneurysmal Malformation: A Report of Two Cases with a Review of Physiology and Approach to Management

2019 ◽  
Vol 09 (02) ◽  
pp. e172-e176 ◽  
Author(s):  
R. E. Giesinger ◽  
Y. N. Elsayed ◽  
M. P. Castaldo ◽  
P. J. McNamara
Keyword(s):  
Ductus Arteriosus ◽  
Endovascular Embolization ◽  
Vein Of Galen ◽  
Vein Of Galen Malformation ◽  
Systemic Blood ◽  
Systemic Blood Flow ◽  
Ventricular Performance ◽  
Right Ventricular Performance ◽  
Neonatal Echocardiography ◽  
Guided Therapy

AbstractVein of Galen malformation results in predictable changes in physiology which exist on a continuum. Severe pulmonary hypertension may present as hypoxemia; however, excessive reduction in pulmonary vascular resistance may precipitate progressive pulmonary overcirculation and impaired systemic blood flow. Right ventricular performance and the patency and direction of the ductus arteriosus may play a crucial role in postductal organ perfusion. Physiological stabilization may be complex and variable over time. The utilization of targeted neonatal echocardiography to guide treatment decisions may improve the ability to provide therapy tailored to the specific disease pathophysiology and monitor serially as conditions change. An enhanced approach to physiological stabilization may reduce the risk of unexpected decompensation and allow for thoughtful, controlled endovascular embolization in appropriate candidates.

A cardiopulmonary bypass strategy to support a patient with vein of Galen malformation

Perfusion ◽  
2021 ◽  
pp. 026765912110015
Author(s):  
Alex Robertson ◽  
Nagarajan Muthialu ◽  
Mike Broadhead
Keyword(s):  
Cardiopulmonary Bypass ◽  
Surgical Repair ◽  
Circulatory Arrest ◽  
Ductus Arteriosus ◽  
Venous Drainage ◽  
Deep Hypothermic Circulatory Arrest ◽  
Vein Of Galen ◽  
Vein Of Galen Malformation ◽  
Neurological Sequelae ◽  
Ph Stat

We present a dissection of the patent ductus arteriosus and pulmonary artery for surgical repair utilising cardiopulmonary bypass in the setting of vein of Galen malformation. Several strategies were employed to attenuate the cerebral shunt including pH-stat, high cardiac index, restrictive venous drainage, continuous ventilation and deep hypothermic circulatory arrest. The patient recovered from surgery with no apparent neurological sequelae.

Fistulous-type vein of Galen malformation phantom model for endovascular training and research

2016 ◽  
Vol 9 (9) ◽  
pp. 880-886
Author(s):  
Dan Meila ◽  
Katharina Melber ◽  
Dominik Grieb ◽  
Collin Jacobs ◽  
Heinrich Lanfermann ◽  
...  
Keyword(s):  
Treatment Options ◽  
Endovascular Embolization ◽  
High Flow ◽  
Vein Of Galen ◽  
Vein Of Galen Malformation ◽  
Phantom Model ◽  
Before And After ◽  
And Training

IntroductionVein of Galen malformation (VGM), a high-flow intracranial arteriovenous shunt, is among the most severe neurovascular diseases in childhood. In many cases untreated children die or survive only severely disabled. Endovascular embolization is the preferred treatment.ObjectiveTo develop a simple fistulous-type VGM phantom model for teaching and training of different endovascular treatment methods and to investigate new treatment options and devices.MethodsAn experimental in vitro pulsatile phantom model was developed imitating a high-flow fistulous-type VGM, which is typical, especially in the neonatal phase. Pressure measurements at different arterial sites were performed before and after closure of the VGM. Closure of the VGM was achieved by coiling using a combined microcatheter-based transvenous and transarterial approach called ‘kissing microcatheter technique’.ResultsThe behaviour of the phantom model in vitro under fluoroscopy and under angiographic runs was extremely similar to that in in vivo conditions in children. The results showed that intra-arterial pressures changed and increased statistically significantly at all measurement sites after embolization, as in human arteriovenous malformation. We also demonstrated different and complementary visualizations of hemodynamics and angioarchitecture by antegrade and retrograde microcatheter injections.ConclusionsOur phantom model behaves like a typical fistulous-type VGM and can be used in vitro for teaching and training and for further research. It offers a new and better understanding of hemodynamics and angioarchitecture in the endovascular management of VGM.

Maximal exercise capacity and oxygen consumption of lambs with an aortopulmonary left-to-right shunt

1990 ◽  
Vol 69 (4) ◽  
pp. 1479-1485 ◽  
Author(s):  
J. W. Gratama ◽  
J. J. Meuzelaar ◽  
M. Dalinghaus ◽  
J. H. Koers ◽  
A. J. Werre ◽  
...  
Keyword(s):  
Oxygen Consumption ◽  
Exercise Capacity ◽  
Maximal Exercise ◽  
Left Ventricular ◽  
Peak Oxygen Consumption ◽  
Systemic Blood ◽  
Systemic Blood Flow ◽  
Ventricular Performance ◽  
Left Ventricular Output ◽  
Maximal Exercise Capacity

We determined maximal exercise capacity and measured hemodynamics in 10 6-wk-old lambs with an aortopulmonary left-to-right shunt [S, 57 +/- 11%, (SD)] and in 9 control lambs (C) during a graded treadmill test 8 days after surgery. Maximal exercise capacity (3.7 +/- 0.2 km/h and 10 +/- 5% inclination vs. 4.0 +/- 0.9 km/h and 15 +/- 0% inclination, P less than 0.02) and peak oxygen consumption (25 +/- 7 vs. 34 +/- 8 ml O2.min-1.kg-1, P less than 0.02) were both lower in the shunt than in the control lambs. This was due to a lower maximal systemic blood flow in the shunt lambs (271 +/- 38 vs. 359 +/- 71 ml.min-1.kg-1, P less than 0.01). Despite their high maximal left ventricular output, which was higher than in the control lambs (448 +/- 87 vs. 359 +/- 71 ml.min-1.kg-1, P less than 0.05), the left-to-right shunt could not be compensated for during maximal exercise because of a decreased reserve in heart rate (S: 183 +/- 22 to 277 +/- 38 beats/min; C: 136 +/- 25 to 287 +/- 29 beats/min) and in left ventricular stroke volume (S: 1.8 +/- 0.3 to 1.6 +/- 0.4 ml/kg; C: 1.0 +/- 0.3 to 1.3 +/- 0.2 ml/kg). We conclude that exercise capacity of shunt lambs is lower than that of control lambs, despite a good left ventricular performance, because a part of the reserves for increasing the left ventricular output is already utilized at rest.

scholarly journals Vein of Galen malformation

2009 ◽  
Vol 27 (5) ◽  
pp. E8 ◽  
Author(s):  
Stanley Hoang ◽  
Omar Choudhri ◽  
Michael Edwards ◽  
Raphael Guzman
Keyword(s):  
Poor Prognosis ◽  
Pediatric Population ◽  
Vascular Lesion ◽  
Endovascular Embolization ◽  
Vein Of Galen ◽  
Vein Of Galen Malformation ◽  
Developmental Mechanisms

A vein of Galen malformation is a rare intracranial vascular lesion affecting the pediatric population. Its poor prognosis has been significantly improved with the development of endovascular embolization. In this paper the authors review the developmental mechanisms, clinical pathophysiology, and the available data on the management and outcome of the disease.

Efficacy of very low-dose prostaglandin E1 in duct-dependent congenital heart disease

2013 ◽  
Vol 25 (1) ◽  
pp. 56-62 ◽  
Author(s):  
Ilker K. Yucel ◽  
Ayhan Cevik ◽  
Mustafa O. Bulut ◽  
Reyhan Dedeoğlu ◽  
İbrahim H. Demir ◽  
...  
Keyword(s):  
Blood Flow ◽  
Treatment Duration ◽  
Pulmonary Blood Flow ◽  
Prostaglandin E1 ◽  
Low Dose ◽  
Ductus Arteriosus ◽  
Systemic Blood ◽  
Systemic Blood Flow ◽  
Group 2 ◽  
Group 1

AbstractAimThe present study aims to define the lowest effective prostaglandin E1 dose in patients with inadequacy of pulmonary blood flow and/or intracardiac blood mixing and those with inadequate systemic blood flow.MethodsPatients with inadequacy of both pulmonary blood flow and/or blood mixing (Group 1) and those with inadequate systemic blood flow (Group 2) were retrospectively evaluated in two separate groups with regard to the prostaglandin E1 starting dose given in the referring facility, the lowest and the highest dose administered in our centre, treatment duration, adverse effects, and administered treatment.ResultsNo difference between the groups could be detected with respect to sex or birth weight (p=0.95 and 0.42, respectively). Group 1 and Group 2 were statistically similar in aspect of prostaglandin treatment duration (9.73±0.81 days versus 11.6±1.05 days, p=0.064). When compared with Group 2, the initial, maintenance and lowest efficient doses of prostaglandin E1 treatment were significantly lower and the titrated dose of prostaglandin E1 was significantly higher in Group 1 (p=0.001 for each).ConclusionOur findings indicate that the infusion of prostaglandin at a very low dose (0.003–0.005 mcg/kg/minute) is sufficient to maintain the patency of the ductus arteriosus. A higher dose of prostaglandin E1 may be necessary in patients with inadequate systemic blood flow.

Outcomes following prenatal diagnosis of hypoplastic left heart syndrome

2002 ◽  
Vol 13 (3) ◽  
pp. 169-180 ◽  
Author(s):  
David A Somerset ◽  
Katherine J Barber ◽  
Mark D Kilby
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Cardiac Disease ◽  
Neonatal Death ◽  
Ductus Arteriosus ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Systemic Blood ◽  
Systemic Blood Flow ◽  
Congenital Cardiac Disease ◽  
Left Heart Syndrome

Hypoplastic Left Heart Syndrome (HLHS) was first described fifty years ago. It is characterised by underdevelopment of the left ventricle with associated aortic and mitral valve hypoplasia or atresia, and varying degrees of hypoplasia of the aortic arch (Figure 1). In utero a physiological right to left shunt of oxygenated blood through the ductus arteriosus bypasses the obstruction and allows normal fetal growth. Closure of the ductus arteriosus in the post-natal period interrupts systemic blood flow, resulting in rapid deterioration and death. Untreated it is a universally fatal condition, leading to neonatal death within the first few days or weeks of life. Although HLHS affects only one baby in 10,000 and accounts for less than 10% of all congenital heart disease, HLHS is responsible for 25% of all deaths due to congenital cardiac disease occurring within the first week of life.

scholarly journals Large vein of Galen malformation associated with an apical ventricular septal defect and a patent ductus arteriosus in an infant: A case report

1997 ◽  
Vol 2 (2) ◽  
pp. 28-30
Author(s):  
P. Szkup ◽  
A. M. Cilliers
Keyword(s):  
Ventricular Septal Defect ◽  
Patent Ductus Arteriosus ◽  
Septal Defect ◽  
Transarterial Embolization ◽  
Ductus Arteriosus ◽  
Male Infant ◽  
Vein Of Galen ◽  
Vein Of Galen Malformation ◽  
Large Vein ◽  
Patent Ductus

A four month old male infant who had a transarterial embolization of a large vein of Galen malformation attempted is reported. The infant presented in cardiac failure and a hydrocephalus. In addition the rare association of an apical ventricular septal defect and a patent ductus arteriosus with pulmonary hypertension was diagnosed. A staged transarterial coil embolisation using microcoils was commenced. The first attempt was uneventful. This was followed by the unfortunate demise of the patient from an intercurrent nosocomial pneumonia.

Effect of Positive End-Expiratory Pressure on Ductal Shunting and Systemic Blood Flow in Preterm Infants with Patent Ductus Arteriosus

Neonatology ◽  
2014 ◽  
Vol 105 (1) ◽  
pp. 9-13 ◽  
Author(s):  
Maria Florencia Fajardo ◽  
Nelson Claure ◽  
Sethuraman Swaminathan ◽  
Sumbal Sattar ◽  
Amelia Vasquez ◽  
...  
Keyword(s):  
Blood Flow ◽  
Patent Ductus Arteriosus ◽  
Preterm Infants ◽  
Ductus Arteriosus ◽  
Positive End Expiratory Pressure ◽  
Systemic Blood ◽  
Systemic Blood Flow ◽  
Patent Ductus

scholarly journals Neurocognitive improvements following endovascular repair of vein of Galen malformation in a child

2015 ◽  
Vol 15 (2) ◽  
pp. 197-202 ◽  
Author(s):  
Robyn A. Howarth ◽  
Andrew Reisner ◽  
Joshua J. Chern ◽  
Laura L. Hayes ◽  
Thomas G. Burns ◽  
...  
Keyword(s):  
Successful Treatment ◽  
Endovascular Repair ◽  
Endovascular Embolization ◽  
Initial Presentation ◽  
Cognitive Deterioration ◽  
Vein Of Galen ◽  
Neurocognitive Deficits ◽  
Vein Of Galen Malformation ◽  
Neurocognitive Profile

Cognitive regression is a well-described presentation of vein of Galen aneurysmal malformations (VGAMs) in childhood. However, it remains unclear whether successful treatment of the malformation can reverse cognitive regression. Here, the authors present the case of a 5-year-old girl with a VGAM that was treated with staged endovascular embolization procedures. Comprehensive neurocognitive assessments were completed before intervention and approximately 6 years after initial presentation. There were significant age-matched improvements in this child's neurocognitive profile over this period. The authors believe that timely and successful treatment of VGAM in children may not only stabilize the associated cognitive deterioration but, in some cases, may ameliorate these deficits. Details of this case and a discussion of neurocognitive deficits related to VGAM are presented.

Sign in / Sign up

Export Citation Format

Share Document