Leonce Mwizerwa, Raphael Joye, Vladimir L. Cousin, Iliona Malaspinas, Maya Bouhabib, Hugues Lucron, Maurice Beghetti, Julie Wacker
Geneva University Hospitals and University of Geneva. AP-HP Paris-Saclay University and Bicêtre Hospital.
Switzerland and France
Translational Pediatrics
Trans Pediatr 2025; 14: 2374-2380
DOI: 10.21037/tp-2025-264
Abstract
Background: Prenatal closure of the ductus arteriosus (DA) is an uncommon but potentially severe condition that can result in persistent pulmonary hypertension of the newborn (PPHN), right ventricular (RV) dysfunction, and even fetal demise. While often associated with maternal exposure to non-steroidal anti-inflammatory drugs (NSAIDs), idiopathic cases are also reported. The pathophysiology involves disruption of prostaglandin-mediated DA patency, leading to increased pulmonary vascular resistance (PVR) and RV afterload, with subsequent postnatal hemodynamic compromise. Given the rarity of this entity, management strategies are based primarily on case series and expert opinion.
Case description: We report two full-term newborns diagnosed with idiopathic prenatal closure of the DA, both presenting with severe persistent PPHN. Case 1 was a female neonate born at 39+5 weeks with oligohydramnios and cardiac rhythm abnormalities. Echocardiography confirmed DA closure, RV dilatation, and RV dysfunction. She was successfully treated with high-flow nasal cannula oxygen, inhaled nitric oxide (iNO), milrinone, and oral sildenafil, with complete recovery by 5 months. Case 2 was a male neonate delivered at 40+1 weeks after fetal echocardiography revealed DA closure and RV hypertrophy. Postnatal management with continuous positive airway pressure alone led to rapid improvement, and he was discharged on day 9 without medication. Both infants demonstrated normal echocardiographic findings and neurodevelopment at one year.
Conclusions: Prenatal closure of the DA has various clinical manifestations, leading to neonatal pulmonary hypertension. It should be suspected in cases of RV hypertrophy, tricuspid valve dysplasia with regurgitation on prenatal echocardiography, or postnatal hypoxemia without significant underlying pulmonary or cardiac associated diseases. Early induction of labor is recommended, while postnatal management aims to reduce PVR and optimize cardiac output.
Category
Class I. Persistent Pulmonary Hypertension of the Newborn
Medical Therapy. Efficacy or Lack of Efficacy
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes