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

AJP Rep 2019; 09: e172-e176 DOI: 10.1055/s-0039-1688765Vein 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. [...] Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals: Table of contents  |  Abstract  |  open access Full text
Source: American Journal of Perinatology Reports - Category: Perinatology & Neonatology Authors: Tags: Case Report Source Type: research

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CONCLUSIONS: Sildenafil in a dose of 25 mg three times daily increases HR, reduces BP and reduces arterial stiffness in pregnancies complicated by FGR. These changes are modest, consistent with the anticipated vasodilatory effect and their clinical impact on the mother and baby, in both the short- and long-term, remains uncertain. This article is protected by copyright. All rights reserved. PMID: 31432556 [PubMed - as supplied by publisher]
Source: The Ultrasound Review of Obstetrics and Gynecology - Category: Radiology Authors: Tags: Ultrasound Obstet Gynecol Source Type: research
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Source: American Journal of Physiology. Heart and Circulatory Physiology - Category: Physiology Authors: Tags: Am J Physiol Heart Circ Physiol Source Type: research
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Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: Clinical Source Type: research
AbstractMagnetic resonance imaging (MRI) plays an increasingly important role in the non-invasive evaluation of the pulmonary vasculature. MR angiographic (MRA) techniques provide morphological information, while MR perfusion techniques provide functional information of the pulmonary vasculature. Contrast-enhanced MRA can be performed at high spatial resolution using 3D T1-weighted spoiled gradient echo sequence or at high temporal resolution using time-resolved techniques. Non-contrast MRA can be performed using 3D steady state free precession, double inversion fast spin echo, time of flight or phase contrast sequences. M...
Source: The International Journal of Cardiovascular Imaging - Category: Radiology Source Type: research
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Source: Current Respiratory Care Reports - Category: Respiratory Medicine Source Type: research
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Source: Journal of Vascular and Interventional Radiology : JVIR - Category: Radiology Authors: Tags: Images in IR Source Type: research
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Source: Anasthesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie : AINS - Category: Intensive Care Authors: Tags: Anasthesiol Intensivmed Notfallmed Schmerzther Source Type: research
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Source: Frontiers in Endocrinology - Category: Endocrinology Source Type: research
Right ventricular (RV) dysfunction secondary to pulmonary hypertension is responsible for heart failure and death in many clinical settings, including congenital heart disease, chronic thromboembolic pulmonary hypertension, left-sided heart failure, and pulmonary artery hypertension.1 Increase in RV afterload leads to a series of complex changes that initially result in compensatory hypertrophy and increased contractility, followed by RV dilatation and failure.2,3 The mechanisms responsible for these changes include ventriculoarterial uncoupling, myocardial ischemia secondary to decreased perfusion pressure and capillary r...
Source: The Journal of Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: Commentary Source Type: research
CONCLUSION: There is preclinical evidence that maternally administered sildenafil prevents the vascular changes that lead to PPH in CDH newborns. The phase I/IIb clinical study together with the p-PBPK model will define the maternal dose needed for a therapeutic effect in the foetus. Foetal safety will be investigated both in the clinical study and in the sheep. The final step will be a multicentre, randomized, placebo-controlled trial. PMID: 30894101 [PubMed - as supplied by publisher]
Source: Current Pharmaceutical Design - Category: Drugs & Pharmacology Authors: Tags: Curr Pharm Des Source Type: research
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