Arin L Madenci1, Joseph T Church2, Robert J Gajarski3, Kathryn Marchetti4, Edwin J Klein4, Megan A Coughlin5, Jeannie Kreutzmann6, Marjorie Treadwell7, Maria Ladino-Torres8, George B Mychaliska6. 1. Department of Surgery, Brigham and Women's Hospital, Boston, Massachusetts, United States. 2. Department of Surgery, University of Michigan Health System, Ann Arbor, Michigan, United States. 3. Department of Pediatric Cardiology, Nationwide Children's Hospital, Columbus, Ohio, United States. 4. Department of Education, University of Michigan Medical School, Ann Arbor, Michigan, United States. 5. Department of Surgery, Henry Ford Health System, Detroit, Michigan, United States. 6. Section of Pediatric Surgery, Department of Surgery, University of Michigan Medical School and C.S. Mott Children's Hospital, Ann Arbor, Michigan, United States. 7. Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of Michigan Medical School and C.S. Mott Children's Hospital, Ann Arbor, Michigan, United States. 8. Section of Pediatric Radiology, Department of Radiology, University of Michigan Medical School and C.S. Mott Children's Hospital, Ann Arbor, Michigan, United States.
Abstract
PURPOSE: The relationship between pulmonary hypoplasia and pulmonary arterial hypertension (PHTN) in patients with congenital diaphragmatic hernia (CDH) remains ill-defined. We hypothesized that prenatal estimates of lung size would directly correlate with PHTN severity. METHODS: Infants with isolated CDH (born 2004-2015) at a single institution were included. Estimates of lung size included observed-to-expected LHR (o:eLHR) and %-predicted lung volumes (PPLV = observed/predicted volumes). The primary outcome was severity of PHTN (grade 0-3) on echocardiography performed between day of life 3 and 30. RESULTS: Among 62 patients included, there was 32% mortality and 65% ECMO utilization. PPLV (odds ratio [OR] = 0.94 per 1 grade in PHTN severity, 95% confidence interval [CI] = 0.89-0.98, p < 0.01) and o:eLHR (OR = 0.97, 95% CI = 0.94-0.99, p < 0.01) were significantly associated with PHTN grade. Among patients on ECMO, PPLV (OR = 0.92, 95% CI = 0.84-0.99, p = 0.03) and o:eLHR (OR = 0.95, 95% CI = 0.92-0.99, p = 0.01) were more strongly associated with PHTN grade. PPLV and o:eLHR were significantly associated with the use of inhaled nitric oxide (iNO) (OR = 0.90, 95% CI = 0.83-0.98, p = 0.01 and OR = 0.94, 95% CI = 0.91-0.98, p < 0.01, respectively) and epoprostenol (OR = 0.91, 95% CI = 0.84-0.99, p = 0.02 and OR = 0.93, 95% CI = 0.89-0.98, p < 0.01, respectively). CONCLUSION: Among infants with isolated CDH, PPLV, and o:eLHR were significantly associated with PHTN severity, especially among patients requiring ECMO. Prenatal lung size may help predict postnatal PHTN and associated therapies. Georg Thieme Verlag KG Stuttgart · New York.
PURPOSE: The relationship between pulmonary hypoplasia and pulmonary arterial hypertension (PHTN) in patients with congenital diaphragmatic hernia (CDH) remains ill-defined. We hypothesized that prenatal estimates of lung size would directly correlate with PHTN severity. METHODS:Infants with isolated CDH (born 2004-2015) at a single institution were included. Estimates of lung size included observed-to-expected LHR (o:eLHR) and %-predicted lung volumes (PPLV = observed/predicted volumes). The primary outcome was severity of PHTN (grade 0-3) on echocardiography performed between day of life 3 and 30. RESULTS: Among 62 patients included, there was 32% mortality and 65% ECMO utilization. PPLV (odds ratio [OR] = 0.94 per 1 grade in PHTN severity, 95% confidence interval [CI] = 0.89-0.98, p < 0.01) and o:eLHR (OR = 0.97, 95% CI = 0.94-0.99, p < 0.01) were significantly associated with PHTN grade. Among patients on ECMO, PPLV (OR = 0.92, 95% CI = 0.84-0.99, p = 0.03) and o:eLHR (OR = 0.95, 95% CI = 0.92-0.99, p = 0.01) were more strongly associated with PHTN grade. PPLV and o:eLHR were significantly associated with the use of inhaled nitric oxide (iNO) (OR = 0.90, 95% CI = 0.83-0.98, p = 0.01 and OR = 0.94, 95% CI = 0.91-0.98, p < 0.01, respectively) and epoprostenol (OR = 0.91, 95% CI = 0.84-0.99, p = 0.02 and OR = 0.93, 95% CI = 0.89-0.98, p < 0.01, respectively). CONCLUSION: Among infants with isolated CDH, PPLV, and o:eLHR were significantly associated with PHTN severity, especially among patients requiring ECMO. Prenatal lung size may help predict postnatal PHTN and associated therapies. Georg Thieme Verlag KG Stuttgart · New York.
Authors: D S Walsh; A M Hubbard; O O Olutoye; L J Howell; T M Crombleholme; A W Flake; M P Johnson; N S Adzick Journal: Am J Obstet Gynecol Date: 2000-11 Impact factor: 8.661
Authors: Barbara R Pober; Angela Lin; Meaghan Russell; Kate G Ackerman; Sharmila Chakravorty; Bernarda Strauss; Marie Noel Westgate; Jay Wilson; Patricia K Donahoe; Lewis B Holmes Journal: Am J Med Genet A Date: 2005-10-01 Impact factor: 2.802
Authors: Kevin P Lally; Pamela A Lally; Robert E Lasky; Dick Tibboel; Tom Jaksic; Jay M Wilson; Bjorn Frenckner; Krista P Van Meurs; Desmond J Bohn; Carl F Davis; Ronald B Hirschl Journal: Pediatrics Date: 2007-09 Impact factor: 7.124