Leslie A Lusk1, Katherine C Wai2, Anita J Moon-Grady3, Amaya M Basta4, Roy Filly4, Roberta L Keller5. 1. Division of Neonatology, Department of Pediatrics, Benioff Children's Hospital, University of California, San Francisco, CA. Electronic address: Leslie.Lusk@ucsf.edu. 2. School of Medicine, University of California, San Francisco, CA. 3. Fetal Treatment Center, University of California, San Francisco, CA. 4. Department of Radiology and Biomedical Imaging, Medical Center, University of California, San Francisco, CA. 5. Division of Neonatology, Department of Pediatrics, Benioff Children's Hospital, University of California, San Francisco, CA; Fetal Treatment Center, University of California, San Francisco, CA.
Abstract
OBJECTIVE: Congenital diaphragmatic hernia (CDH) results in morbidity and death from lung hypoplasia and persistent pulmonary hypertension (PH). We sought to define the relationship between fetal ultrasound markers of severity in CDH and the time to resolution of neonatal PH. STUDY DESIGN: We conducted a retrospective study of fetuses with an antenatal ultrasound scan and left-sided CDH cared for at the University of California San Francisco (2002-2012). Fetal liver position was classified on ultrasound scan as abdominal (entire liver within the abdomen) or thoracic (any portion of the liver within the thorax). Fetal stomach position was classified from least to most aberrant: abdominal, anterior left chest, mid-posterior left chest, or retrocardiac (right chest). Lung-to-head ratio (LHR) was determined from available scans at 20-29 weeks of gestational age (GA). Routine neonatal echocardiograms were performed weekly for up to 6 weeks or until PH resolved or until discharge. PH was assessed by echocardiogram with the use of a hierarchy of ductus arteriosus level shunt, interventricular septal position, and tricuspid regurgitant jet velocity. Days to PH-free survival was defined as the age at which pulmonary artery pressure was estimated to be <2/3 systemic blood pressure. Cox proportional hazards models adjusted for GA at birth, era of birth, fetal surgery, and GA at ultrasound scan (LHR model only), with censoring at 100 days. RESULTS: Of 118 patients, the following fetal markers were available: LHR (n = 53), liver position (n = 112), and stomach position (n = 80). Fewer infants experienced resolved PH if they had LHR <1 (P = .006), thoracic liver position (P = .001), or more aberrant stomach position (P < .001). There was also a decreased rate of resolution of PH in infants with LHR <1 (hazard ratio, 0.30; P = .007), thoracic liver position (hazard ratio, 0.38; P < .001), and more aberrant stomach position (hazard ratios, 0.28 [P = .002]; 0.1 [P < .001]; and 0.07 [P < .001]). CONCLUSION: Fetal ultrasound markers of CDH severity are predictive not only of death but also of significant morbidity. LHR <1, thoracic liver, and aberrant stomach position are associated with delayed time to resolution of PH in infants with CDH and may be used to identify fetuses at high risk of persistent PH.
OBJECTIVE:Congenital diaphragmatic hernia (CDH) results in morbidity and death from lung hypoplasia and persistent pulmonary hypertension (PH). We sought to define the relationship between fetal ultrasound markers of severity in CDH and the time to resolution of neonatal PH. STUDY DESIGN: We conducted a retrospective study of fetuses with an antenatal ultrasound scan and left-sided CDH cared for at the University of California San Francisco (2002-2012). Fetal liver position was classified on ultrasound scan as abdominal (entire liver within the abdomen) or thoracic (any portion of the liver within the thorax). Fetal stomach position was classified from least to most aberrant: abdominal, anterior left chest, mid-posterior left chest, or retrocardiac (right chest). Lung-to-head ratio (LHR) was determined from available scans at 20-29 weeks of gestational age (GA). Routine neonatal echocardiograms were performed weekly for up to 6 weeks or until PH resolved or until discharge. PH was assessed by echocardiogram with the use of a hierarchy of ductus arteriosus level shunt, interventricular septal position, and tricuspid regurgitant jet velocity. Days to PH-free survival was defined as the age at which pulmonary artery pressure was estimated to be <2/3 systemic blood pressure. Cox proportional hazards models adjusted for GA at birth, era of birth, fetal surgery, and GA at ultrasound scan (LHR model only), with censoring at 100 days. RESULTS: Of 118 patients, the following fetal markers were available: LHR (n = 53), liver position (n = 112), and stomach position (n = 80). Fewer infants experienced resolved PH if they had LHR <1 (P = .006), thoracic liver position (P = .001), or more aberrant stomach position (P < .001). There was also a decreased rate of resolution of PH in infants with LHR <1 (hazard ratio, 0.30; P = .007), thoracic liver position (hazard ratio, 0.38; P < .001), and more aberrant stomach position (hazard ratios, 0.28 [P = .002]; 0.1 [P < .001]; and 0.07 [P < .001]). CONCLUSION: Fetal ultrasound markers of CDH severity are predictive not only of death but also of significant morbidity. LHR <1, thoracic liver, and aberrant stomach position are associated with delayed time to resolution of PH in infants with CDH and may be used to identify fetuses at high risk of persistent PH.
Authors: Anthony O Odibo; Tasnim Najaf; Akshaya Vachharajani; Barbara Warner; Amit Mathur; Brad W Warner Journal: Prenat Diagn Date: 2010-06 Impact factor: 3.050
Authors: J Jani; K H Nicolaides; R L Keller; A Benachi; C F A Peralta; R Favre; O Moreno; D Tibboel; S Lipitz; A Eggink; P Vaast; K Allegaert; M Harrison; J Deprest Journal: Ultrasound Obstet Gynecol Date: 2007-07 Impact factor: 7.299
Authors: Holly L Hedrick; Enrico Danzer; Aziz M Merchant; Michael W Bebbington; Huaqing Zhao; Alan W Flake; Mark P Johnson; Kenneth W Liechty; Lori J Howell; R Douglas Wilson; N Scott Adzick Journal: Am J Obstet Gynecol Date: 2007-10 Impact factor: 8.661
Authors: J C Jani; A Benachi; K H Nicolaides; K Allegaert; E Gratacós; R Mazkereth; J Matis; D Tibboel; A Van Heijst; L Storme; V Rousseau; A Greenough; J A Deprest Journal: Ultrasound Obstet Gynecol Date: 2009-01 Impact factor: 7.299
Authors: R Cruz-Martinez; F Figueras; O Moreno-Alvarez; J M Martinez; O Gomez; E Hernandez-Andrade; E Gratacos Journal: Ultrasound Obstet Gynecol Date: 2010-07 Impact factor: 7.299
Authors: Roberta L Keller; Theresa A Tacy; Karen Hendricks-Munoz; Jie Xu; Anita J Moon-Grady; John Neuhaus; Phillip Moore; Kerilyn K Nobuhara; Sam Hawgood; Jeffrey R Fineman Journal: Am J Respir Crit Care Med Date: 2010-04-22 Impact factor: 21.405
Authors: Jeffrey D Sperling; Teresa N Sparks; Victoria K Berger; Jody A Farrell; Kristen Gosnell; Roberta L Keller; Mary E Norton; Juan M Gonzalez Journal: Am J Perinatol Date: 2018-01-05 Impact factor: 1.862
Authors: Candace C Style; Oluyinka O Olutoye; Mariatu A Verla; Keila N Lopez; Adam M Vogel; Patricio E Lau; Stephanie M Cruz; Jimmy Espinoza; Caraciolo J Fernandes; Sundeep G Keswani; Timothy C Lee Journal: J Pediatr Surg Date: 2019-02-20 Impact factor: 2.545
Authors: Michaël Levy; Nolwenn Le Sache; Mostafa Mokhtari; Guy Fagherazzi; Gaelle Cuzon; Benjamin Bueno; Virginie Fouquet; Alexandra Benachi; Sergio Eleni Dit Trolli; Pierre Tissieres Journal: Ann Intensive Care Date: 2017-03-21 Impact factor: 6.925
Authors: Jan A Deprest; Alexandra Benachi; Eduard Gratacos; Kypros H Nicolaides; Christoph Berg; Nicola Persico; Michael Belfort; Glenn J Gardener; Yves Ville; Anthony Johnson; Francesco Morini; Mirosław Wielgoś; Ben Van Calster; Philip L J DeKoninck Journal: N Engl J Med Date: 2021-06-08 Impact factor: 176.079
Authors: Katinka Weller; Nina C J Peters; Joost van Rosmalen; Suzan C M Cochius-Den Otter; Philip L J DeKoninck; Rene M H Wijnen; Titia E Cohen-Overbeek; Alex J Eggink Journal: Prenat Diagn Date: 2021-07-28 Impact factor: 3.242