Perinatology.  2019 Jun;30(2):99-104. 10.14734/PN.2019.30.2.99.

Pulmonary Hypertension in Newborn Infants with Vein of Galen Malformation

• Song WS
• Oh MY
• Cho JY
• Kim CY
• Suh DC
• Jung E
• Kim EA
• Kim KS
• Lee BS

• KMID: 2453045
• DOI: http://doi.org/10.14734/PN.2019.30.2.99

Abstract

OBJECTIVE
Vein of Galen malformation (VGM) is a rare cerebrovascular malformation. Children with VGM presenting in newborn period demonstrate various clinical courses from normal development to severe neurologic impairment or death. The aim of our study was to describe the characteristics of pulmonary hypertension and its treatment response in newborn infants with VGM.
METHODS
We retrospectively reviewed medical records of newborn infants with VGM who were antenatal diagnosed and treated with endovascular embolization in a single tertiary center. Clinical characteristics including echocardiographic data were described.
RESULTS
A total of six patients were diagnosed with VGM. Endovascular embolization was performed early postnatal days (median, 6.5 days) in four patients who required cardiorespiratory support. Echocardiography revealed pulmonary hypertension in the four cases and two of them received targeted therapy against pulmonary hypertension including inhaled nitric oxide and other pulmonary vasodilators. Incomplete occlusion of VGM failed to reverse the pulmonary hypertension. Mortality occurred in two patients with severe pulmonary hypertension in whom incomplete occlusion for VGM lesion. The cause of death was multi-organ failure secondary to right heart failure.
CONCLUSION
Pulmonary hypertension was a common manifestation in newborn case with symptomatic VGM. The efficacy of early embolization and management for pulmonary hypertension in the newborn cases with persistent pulmonary hypertension of the newborn requires further large studies.

Keyword

Vein of Galen malformations; Heart failure; Pulmonary hypertension; Embolization

MeSH Terms

Cause of Death
Cerebral Veins*
Child
Echocardiography
Heart Failure
Humans
Hypertension, Pulmonary*
Infant, Newborn*
Medical Records
Mortality
Nitric Oxide
Retrospective Studies
Vasodilator Agents
Vein of Galen Malformations
Veins*
Nitric Oxide
Vasodilator Agents

Figure

• 

  Fig. 1. Representative images of mural type vein of Galen malformation in patients in whom complete (A-D; case 4) or incomplete (E-H; case 6) endovascular embolization was performed. Pre-treatment brain imaging (T2 weighted MRI; A, E) and post-treatment brain imaging (T1 weighted MRI; B, F) and pre-treatment (C, G) and post-treatment (D, H) angiography with microcatheter approach to the vascular lesion was shown. MRI, magnetic resonance imaging.

• 

  Fig. 2. Representative chest radiographs and echocardiography images of the patients with severe pulmonary hypertension (case 5). Chest radiographs immediately after birth (A) and at 2 days after initial embolization (B) demonstrate severe cardiomegaly. A pre-embolization echocardiography shows a right to left shunt through patent ductus arteriosus (C), tricuspid valve regurgitation (D) and interventricular septum flattening (E). Pulmonary hypertension and right heart failure persisted despite endovascular embolization of vein of Galen malformation, ductus ligation and aggressive pulmonary hypertensive medications including inhaled nitric oxide and other pulmonary vasodilators. A post embolization echocardiography shows enlarged right atrium (F), right to left shunt through atrial septal defect (G), tricuspid valve regurgitation (H).

Reference

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