Vein of galen aneurysm as an extracardiac cause of severe heart failure in the neonatal period

Abstract

Extracardiac arteriovenous malformations are considered to be an extremely rare cause of severe heart failure in the neonatal period. Intracranial arteriovenous malformation associated with the large-vein aneurysm of Galen (VGA) is the most common pathology among these malformations, due to impaired development of the embryonic precursor — Markovskiy's medial prosenecephalic vein. Presence of this congenital pathology significantly complicates the diagnostic search, since clinical manifestations are characteristic only for heart failure.
Materials and methods. A clinical case of a newborn girl with VGA, who was admitted to the neonatal intensive care unit of the Regional Children's Hospital with signs of severe heart failure in January 2019. On the third day after birth, the child developed cyanosis (O2 saturation was 50%). There fore, congenital heart disease was suspected.
Results. On examination in the neonatal intensive care unit: cyanosis (50% saturation), dyspnea (respiratory rate 50–60 / min), tachycardia (heart rate 140–160 bpm) and significant swelling and pulsation of the neck veins, large fontanelle 3×3 cm in size, at the level of the bones of the skull, auscultation of the fontanel was not performed. Pulsation was determined on the femoral arteries, and the liver was enlarged (+4 cm) on palpation. During auscultation — pansystolic murmur across left lower sternal border 4/6 by Levine scale. Cardiomegaly (CTI>80%) with enhanced pulmonary pattern and hepatomegaly where revealed on the roentgenogram. During an echocardiographic examination, the child was diagnosed with dilated right heart chambers with tricuspid insufficiency, patent foramen ovale and a dilated innominate vein. After excluding the diagnosis of critical heart disease, neurosonography was carried out and a diagnosis was made — arteriovenous malformation — Vein of Galen aneurysm, which was confirmed on computed tomography.
Conclusions. Aneurysm of the vein of Galen is an extremely rare pathology, however, the swelling and pulsation of the cervical veins during the examination and the presence of severe cardiovascular insufficiency in newborns in the absence of congenital heart disease requires children's cardiologists to suspect and exclude arteriovenous malformation of the cerebral vessels. Endovascular treatment of VGA is considered the most effective, despite the high postoperative mortality.
The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee (LEC) of all institutions.