Journal article
Congenital diaphragmatic hernia-associated pulmonary hypertension
Seminars in pediatric surgery, Vol.33(4), 151437
07/2024
DOI: 10.1016/j.sempedsurg.2024.151437
Abstract
Congenital diaphragmatic hernia (CDH) is characterized by a developmental insult which compromises cardiopulmonary embryology and results in a diaphragmatic defect, allowing abdominal organs to herniate into the hemithorax. Among the significant pathophysiologic components of this condition is pulmonary hypertension (PH), alongside pulmonary hypoplasia and cardiac dysfunction. Fetal pulmonary vascular development coincides with lung development, with the pulmonary vasculature evolving alongside lung maturation. However, in CDH, this embryologic development is impaired which, in conjunction with external compression, stifle pulmonary vascular maturation, leading to reduced lung density, increased muscularization of the pulmonary vasculature, abnormal vascular responsiveness, and altered molecular signaling, all contributing to pulmonary arterial hypertension. Understanding CDH-associated PH (CDH-PH) is crucial for development of novel approaches and effective management due to its significant impact on morbidity and mortality. Antenatal and postnatal diagnostic methods aid in CDH risk stratification and, specifically, pulmonary hypertension, including fetal imaging and gas exchange assessments. Management strategies include lung protective ventilation, fluid optimization, pharmacotherapies including pulmonary vasodilators and hemodynamic support, and extracorporeal life support (ECLS) for refractory cases. Longitudinal re-evaluation is an important consideration due to the complexity and dynamic nature of CDH cardiopulmonary physiology. Emerging therapies such as fetal endoscopic tracheal occlusion and pharmacological interventions targeting key CDH pathophysiological mechanisms show promise but require further investigation. The complexity of CDH-PH underscores the importance of a multidisciplinary approach for optimal patient care and improved outcomes.
Details
- Title: Subtitle
- Congenital diaphragmatic hernia-associated pulmonary hypertension
- Creators
- Kylie I. Holden - The University of Texas Health Science Center at HoustonNatalie E. Rintoul - University of PennsylvaniaPatrick J. McNamara - University of IowaMatthew T. Harting - The University of Texas Health Science Center at Houston
- Resource Type
- Journal article
- Publication Details
- Seminars in pediatric surgery, Vol.33(4), 151437
- Publisher
- Elsevier Inc
- DOI
- 10.1016/j.sempedsurg.2024.151437
- ISSN
- 1055-8586
- eISSN
- 1532-9453
- Language
- English
- Electronic publication date
- 07/2024
- Academic Unit
- Internal Medicine; Stead Family Department of Pediatrics; Neonatology
- Record Identifier
- 9984651159602771
Metrics
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