Duy T Dao1, Ali Kamran2, Jay M Wilson3, Catherine A Sheils4, Virginia S Kharasch5, Mary P Mullen6, Samuel E Rice-Townsend2, Jill M Zalieckas2, Donna Morash2, Mollie Studley2, Steven J Staffa7, David Zurakowski7, Ronald E Becker8, Charles J Smithers9, Terry L Buchmiller10. 1. Department of Surgery, Boston Children's Hospital, Boston, MA; Vascular Biology Program, Boston Children's Hospital, Boston, MA. 2. Department of Surgery, Boston Children's Hospital, Boston, MA. 3. Department of Pediatric Surgery, McGovern Medical School at UTHealth, Children's Memorial Hermann Hospital, Houston, TX. 4. Division of Respiratory Diseases, Boston Children's Hospital, Boston, MA. 5. Department of Pediatrics, Franciscan Children's Hospital, Brighton, MA. 6. Department of Cardiology, Boston Children's Hospital, Boston, MA. 7. Department of Surgery, Boston Children's Hospital, Boston, MA; Department of Anesthesiology, Boston Children's Hospital, Boston, MA. 8. Division of Developmental Medicine, Boston Children's Hospital, Boston, MA. 9. Department of Surgery, Johns Hopkins All Children's Hospital, St. Petersburg, FL. 10. Department of Surgery, Boston Children's Hospital, Boston, MA. Electronic address: terry.buchmiller@childrens.harvard.edu.
Abstract
OBJECTIVE: To determine the natural history of pulmonary function for survivors of congenital diaphragmatic hernia (CDH). STUDY DESIGN: This was a retrospective cohort study of survivors of CDH born during 1991-2016 and followed at our institution. A generalized linear model was fitted to assess the longitudinal trends of ventilation (V), perfusion (Q), and V/Q mismatch. The association between V/Q ratio and body mass index percentile as well as functional status was also assessed with a generalized linear model. RESULTS: During the study period, 212 patients had at least one V/Q study. The average ipsilateral V/Q of the cohort increased over time (P < .01), an effect driven by progressive reduction in relative perfusion (P = .012). A higher V/Q ratio was correlated with lower body mass index percentile (P < .001) and higher probability of poor functional status (New York Heart Association class III or IV) (P = .045). CONCLUSIONS: In this cohort of survivors of CDH with more severe disease characteristics, V/Q mismatch worsens over time, primarily because of progressive perfusion deficit of the ipsilateral side. V/Q scans may be useful in identifying patients with CDH who are at risk for poor growth and functional status.
OBJECTIVE: To determine the natural history of pulmonary function for survivors of congenital diaphragmatic hernia (CDH). STUDY DESIGN: This was a retrospective cohort study of survivors of CDH born during 1991-2016 and followed at our institution. A generalized linear model was fitted to assess the longitudinal trends of ventilation (V), perfusion (Q), and V/Q mismatch. The association between V/Q ratio and body mass index percentile as well as functional status was also assessed with a generalized linear model. RESULTS: During the study period, 212 patients had at least one V/Q study. The average ipsilateral V/Q of the cohort increased over time (P < .01), an effect driven by progressive reduction in relative perfusion (P = .012). A higher V/Q ratio was correlated with lower body mass index percentile (P < .001) and higher probability of poor functional status (New York Heart Association class III or IV) (P = .045). CONCLUSIONS: In this cohort of survivors of CDH with more severe disease characteristics, V/Q mismatch worsens over time, primarily because of progressive perfusion deficit of the ipsilateral side. V/Q scans may be useful in identifying patients with CDH who are at risk for poor growth and functional status.
Authors: Duy T Dao; Lystra P Hayden; Terry L Buchmiller; Virginia S Kharasch; Ali Kamran; Charles J Smithers; Samuel E Rice-Townsend; Jill M Zalieckas; Ronald Becker; Donna Morash; Mollie Studley; Jay M Wilson; Catherine A Sheils Journal: J Pediatr Date: 2019-11-06 Impact factor: 4.406