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

Abstract

Vein 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.

Keywords

vein of Galen malformation - targeted neonatal echocardiography - hemodynamics

• References

• 1 Lakshminrusimha S, Wiseman D, Black SM. , et al. The role of nitric oxide synthase-derived reactive oxygen species in the altered relaxation of pulmonary arteries from lambs with increased pulmonary blood flow. Am J Physiol Heart Circ Physiol 2007; 293 (03) H1491-H1497
• 2 McSweeney N, Brew S, Bhate S, Cox T, Roebuck DJ, Ganesan V. Management and outcome of vein of Galen malformation. Arch Dis Child 2010; 95 (11) 903-909
• 3 Frawley GP, Dargaville PA, Mitchell PJ, Tress BM, Loughnan P. Clinical course and medical management of neonates with severe cardiac failure related to vein of Galen malformation. Arch Dis Child Fetal Neonatal Ed 2002; 87 (02) F144-F149
• 4 Finan E, Sehgal A, Khuffash AE, McNamara PJ. Targeted neonatal echocardiography services: need for standardized training and quality assurance. J Ultrasound Med 2014; 33 (10) 1833-1841
• 5 Amer R, Kalash R, Seshia MM, Elsayed YN. The impact of integrated evaluation of hemodynamics on management of preterm infants with late-onset compromised systemic circulation. Am J Perinatol 2017; 34 (10) 1011-1019
• 6 Harabor A, Soraisham AS. Utility of targeted neonatal echocardiography in the management of neonatal illness. J Ultrasound Med 2015; 34 (07) 1259-1263
• 7 Evans N. Echocardiography on neonatal intensive care units in Australia and New Zealand. J Paediatr Child Health 2000; 36 (02) 169-171
• 8 Papadhima I, Louis D, Purna J. , et al. Targeted neonatal echocardiography (TNE) consult service in a large tertiary perinatal center in Canada. J Perinatol 2018; 38 (08) 1039-1045
• 9 Dahdah NS, Alesseh H, Dahms B, Saker F. Severe pulmonary hypertensive vascular disease in two newborns with aneurysmal vein of Galen. Pediatr Cardiol 2001; 22 (06) 538-541
• 10 Reddy VM, Meyrick B, Wong J. , et al. In utero placement of aortopulmonary shunts. A model of postnatal pulmonary hypertension with increased pulmonary blood flow in lambs. Circulation 1995; 92 (03) 606-613
• 11 Reddy VM, Wong J, Liddicoat JR, Johengen M, Chang R, Fineman JR. Altered endothelium-dependent responses in lambs with pulmonary hypertension and increased pulmonary blood flow. Am J Physiol 1996; 271 (2 Pt 2): H562-H570
• 12 Reller MD, Morton MJ, Reid DL, Thornburg KL. Fetal lamb ventricles respond differently to filling and arterial pressures and to in utero ventilation. Pediatr Res 1987; 22 (06) 621-626
• 13 Altin SE, Schulze PC. Metabolism of the right ventricle and the response to hypertrophy and failure. Prog Cardiovasc Dis 2012; 55 (02) 229-233
• 14 Ruffolo Jr RR. The pharmacology of dobutamine. Am J Med Sci 1987; 294 (04) 244-248
• 15 Barrington KJ, Finer NN, Chan WK. A blind, randomized comparison of the circulatory effects of dopamine and epinephrine infusions in the newborn piglet during normoxia and hypoxia. Crit Care Med 1995; 23 (04) 740-748
• 16 Godfrey ME, Tworetzky W, Morash D, Friedman KG. Cardiac findings in the fetus with cerebral arteriovenous malformation are associated with adverse outcome. Fetal Diagn Ther 2017; 41 (02) 108-114