Jai Khurana, Darren B. Orbach, Kimberlee Gauvreau, Shane A. Collins, Joseph B. Tella, Pankai B. Agrawal, Helen A. Christou, Mary P. Mullen
Boston Children’s Hospital, Boston. Harvard Medical School. Brigham & Women’s Hospital.
United States
Journal of Pediatrics
J Pediatr 2023;
DOI: 10.1016/j.jpeds.2023.113404
Abstract
Objective: To assess the extent and resolution of pulmonary hypertension (PH), cardiovascular factors, and echocardiographic findings associated with mortality in infants and children with vein of Galen malformation (VOGM).
Study design: We performed a retrospective review of 49 consecutive children with VOGM admitted to Boston Children’s Hospital (BCH) from 2007 to 2020. Patient characteristics, echocardiographic data, and hospital course were analyzed for two cohorts based on age at BCH presentation: Group 1 (age < 60 days) or Group 2 (age > 60 days).
Results: Overall hospital survival was 35/49 (28.6%); 13/26 (50%) in Group 1 and 22/23 (96%) in Group 2 (p<0.001). High output PH (p=0.01), cardiomegaly (p=0.011), intubation (p=0.019) and dopamine use (p=0.01) were significantly more common in Group 1 than Group 2. Among Group 1 patients, congestive heart failure (CHF) (p=0.015), intubation (p<0.001), use of inhaled nitric oxide (iNO) (p=0.015) or prostaglandin E1 (PGE1) (p=0.030), suprasystemic PH (p=0.003), and right-sided dilation were significantly associated with mortality, while in contrast, left ventricular volume and function, structural congenital heart disease, and supraventricular tachycardia (SVT) were not associated. iNO achieved no clinical benefit in 9 of 11 treated patients. Resolution of PH was associated with overall survival (p<0.001).
Conclusions: VOGM remains associated with substantial mortality among infants presenting <60 days of life owing to factors associated with high output PH. Resolution of PH is an indicator associated with survival and a surrogate endpoint for benchmarking outcomes.
Category
Class I. Pulmonary Hypertension Associated with Cardiovascular Heart Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
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