Front Pediatr
. 2024 Mar 25:12:1356157.
doi: 10.3389/fped.2024.1356157. eCollection 2024. https://pubmed.ncbi.nlm.nih.gov/38590769/
Diagnosis, management and long term cardiovascular outcomes of phenotypic profiles in pulmonary hypertension associated with congenital diaphragmatic hernia
Tejasvi Chaudhari 1 2, Nadia Schmidt Sotomayor 1 2, Rajesh Maheshwari 3 4
Affiliations expand
- PMID: 38590769
- PMCID: PMC10999638
- DOI: 10.3389/fped.2024.1356157
Abstract
Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm resulting in herniation of viscera into the chest. This condition is characterized by pulmonary hypoplasia, pulmonary hypertension (PH) and cardiac ventricular dysfunction. PH is a key component of the pathophysiology of CDH in neonates and contributes to morbidity and mortality. Traditionally, PH associated with CDH (CDH-PH) is thought to be secondary to increased pulmonary arterial resistance and vasoreactivity resulting from pulmonary hypoplasia. Additionally, there is increasing recognition of associated left ventricular hypoplasia, dysfunction and elevated end diastolic pressure resulting in pulmonary venous hypertension in infants with CDH. Thus, hemodynamic management of these infants is complex and cautious use of pulmonary vasodilators such as inhaled nitric oxide (iNO) is warranted. We aim to provide an overview of different phenotypic profiles of CDH associated PH and potential management options based on current evidence and pathophysiology.
Keywords: atrial shunt; cardiac output; congenital diaphragmatic hernia; ductal shunt; left ventricular hypoplasia; pulmonary hypertension; pulmonary hypoplasia; ventricular dysfunction.
© 2024 Chaudhari, Schmidt Sotomayor and Maheshwari.
