دورية أكاديمية
أعرض في EDS

Congenital Diaphragmatic Hernia Patients with Left Heart Hypoplasia and Left Ventricular Dysfunction Have Highest Odds of Mortality.

التفاصيل البيبلوغرافية
العنوان: Congenital Diaphragmatic Hernia Patients with Left Heart Hypoplasia and Left Ventricular Dysfunction Have Highest Odds of Mortality.
المؤلفون: Fraga MV; Division of Neonatology, The Children's Hospital of Philadelphia, Philadelphia, PA; Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania. Philadelphia, PA. Electronic address: fragam@chop.edu., Hedrick HL; Department of Pediatric General Thoracic and Fetal Surgery, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania Philadelphia, Philadelphia, PA., Rintoul NE; Division of Neonatology, The Children's Hospital of Philadelphia, Philadelphia, PA; Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania. Philadelphia, PA., Wang Y; Division of Cardiology, The Children's Hospital of Philadelphia, Pennsylvania, Philadelphia, PA., Ash D; Division of Cardiology, The Children's Hospital of Philadelphia, Pennsylvania, Philadelphia, PA., Flohr SJ; Center for Fetal Diagnosis and Treatment, Department of Pediatric General Thoracic and Fetal Surgery, Children's Hospital of Philadelphia, Philadelphia, PA., Mathew L; Center for Fetal Diagnosis and Treatment, Department of Pediatric General Thoracic and Fetal Surgery, Children's Hospital of Philadelphia, Philadelphia, PA., Reynolds T; Center for Fetal Diagnosis and Treatment, Department of Pediatric General Thoracic and Fetal Surgery, Children's Hospital of Philadelphia, Philadelphia, PA., Engelman JL; Center for Fetal Diagnosis and Treatment, Department of Pediatric General Thoracic and Fetal Surgery, Children's Hospital of Philadelphia, Philadelphia, PA., Avitabile CM; Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania. Philadelphia, PA; Division of Cardiology, The Children's Hospital of Philadelphia, Pennsylvania, Philadelphia, PA.
المصدر: The Journal of pediatrics [J Pediatr] 2024 Aug; Vol. 271, pp. 114061. Date of Electronic Publication: 2024 Apr 16.
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: Mosby Country of Publication: United States NLM ID: 0375410 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1097-6833 (Electronic) Linking ISSN: 00223476 NLM ISO Abbreviation: J Pediatr Subsets: MEDLINE
أسماء مطبوعة: Original Publication: St. Louis, MO : Mosby
مواضيع طبية MeSH: Hernias, Diaphragmatic, Congenital*/complications , Hernias, Diaphragmatic, Congenital*/mortality , Hernias, Diaphragmatic, Congenital*/therapy , Extracorporeal Membrane Oxygenation* , Ventricular Dysfunction, Left*/mortality, Humans ; Male ; Female ; Retrospective Studies ; Infant, Newborn ; Infant ; Echocardiography ; Severity of Illness Index
مستخلص: Objectives: To describe the scope of left ventricular (LV) dysfunction and left heart hypoplasia (LHH) in infants with congenital diaphragmatic hernia (CDH), to determine associations with CDH severity, and to evaluate the odds of extracorporeal membrane oxygenation (ECMO) and death with categories of left heart disease.
Study Design: Demographic and clinical variables were collected from a single-center, retrospective cohort of patients with CDH from January 2017 through May 2022. Quantitative measures of LV function and LHH were prospectively performed on initial echocardiograms. LHH was defined as ≥2 of the following: z score ≤ -2 of any left heart structure or LV end-diastolic volume <3 mL. LV dysfunction was defined as shortening fraction <28%, ejection fraction <60%, or global longitudinal strain <20%. The exposure was operationalized as a 4-group categorical variable (LV dysfunction +/-, LHH +/-). Logistic regression models evaluated associations with ECMO and death, adjusting for CDH severity.
Results: One hundred eight-two patients (80.8% left CDH, 63.2% liver herniation, 23.6% ECMO, 12.1% mortality) were included. Twenty percent demonstrated normal LV function and no LHH (LV dysfunction-/LHH-), 37% normal LV function with LHH (LV dysfunction-/LHH+), 14% LV dysfunction without LHH (LV dysfunction+/LHH-), and 28% both LV dysfunction and LHH (LV dysfunction+/LHH+). There was a dose-response effect between increasing severity of left heart disease, ECMO use, and mortality. LV dysfunction+/LHH + infants had the highest odds of ECMO use and death, after adjustment for CDH severity [OR (95% CI); 1.76 (1.20, 2.62) for ECMO, 2.76 (1.63, 5.17) for death].
Conclusions: In our large single-center cohort, patients with CDH with LV dysfunction+/LHH + had the highest risk of ECMO use and death.
Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest.
(Copyright © 2024 Elsevier Inc. All rights reserved.)
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معلومات مُعتمدة: K23 HL150337 United States HL NHLBI NIH HHS
فهرسة مساهمة: Keywords: congenital diaphragmatic hernia; left heart hypoplasia; left ventricular dysfunction; pulmonary arterial hypertension
تواريخ الأحداث: Date Created: 20240418 Date Completed: 20240711 Latest Revision: 20240714
رمز التحديث: 20240714
مُعرف محوري في PubMed: PMC11239301
DOI: 10.1016/j.jpeds.2024.114061
PMID: 38636784
قاعدة البيانات: MEDLINE
الوصف
تدمد:1097-6833
DOI:10.1016/j.jpeds.2024.114061