Pulmonary Hypertension in Infants and Children with Vein of Galen Malformation and Association with Clinical Outcomes
The Journal of Pediatrics(2023)
摘要
Objective To assess the extent and resolution of pulmonary hypertension (PH), cardiovascular factors, and echo -cardiographic 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 from 2007 to 2020. Patient characteristics, echocardiographic data, and hospital course were analyzed for 2 cohorts based on age at presentation to Boston Children's Hospital: group 1 (age <= 60 days) or group 2 (age >60 days).Results Overall hospital survival was 35 of 49 (71.4%); 13 of 26 (50%) in group 1 and 22 of 23 (96%) in group 2 (P < .001). High-output PH (P = .01), cardiomegaly (P = .011), intubation (P = .019), and dopamine use (P = .01) were significantly more common in group 1 than group 2. Among patients in group 1, congestive heart failure (P = .015), intu-bation (P < .001), use of inhaled nitric oxide (P = .015) or prostaglandin E1 (P = .030), suprasystemic PH (P = .003), and right-sided dilation were significantly associated with mortality; in contrast, left ventricular volume and function, struc-tural congenital heart disease, and supraventricular tachycardia were not associated. Inhaled nitric oxide achieved no clinical benefit in 9 of 11 treated patients. Resolution of PH was associated with overall survival (P < .001).Conclusions VOGM remains associated with substantial mortality among infants presenting at <= 60 days of life owing to factors associated with high output PH. Resolution of PH is an indicator associated with survival and a surrogate end point for benchmarking outcomes. (J Pediatr 2023;258:113404).
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关键词
galen malformation,pulmonary hypertension,vein,infants
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