New Insights: P.I.G in Preterm Infants With Isolated PDA and Severe Pulmonary Hypertension

Nadya Ben Fadel ¹, Elham Almoli ², Joseph de Nanassy ³

⁰Department of Pediatrics, Division of Neonatology, Faculty of Medicine, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, Ontario, Canada.

Case Reports in Pediatrics +

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December 9, 2025

View abstract on PubMed

Summary

A premature infant with persistent patent ductus arteriosus (PDA) developed severe pulmonary hypertension (PHT). A lung biopsy revealed pulmonary interstitial glycogenosis (PIG), a rare finding in such cases.

Area Of Science

Neonatology
Pediatric Cardiology
Pulmonology

Background

Persistent patent ductus arteriosus (PDA) can lead to severe pulmonary hypertension (PHT) and respiratory failure in premature infants.
Pulmonary interstitial glycogenosis (PIG) is a rare condition characterized by interstitial thickening and immature interstitial cells with glycogen accumulation.
Previous reports on PIG associated with PHT primarily involved full-term infants or those with other congenital heart defects.

Purpose Of The Study

To report a case of pulmonary interstitial glycogenosis (PIG) diagnosed in a premature infant with isolated persistent patent ductus arteriosus (PDA) and severe pulmonary hypertension (PHT).
To highlight the diagnostic utility of lung biopsy in identifying PIG in this specific clinical context.

Main Methods

A premature infant with persistent PDA, severe PHT, and respiratory failure underwent PDA ligation.
A lung biopsy was performed during the surgical procedure.
Histopathological examination of the lung biopsy was conducted to identify characteristic features of PIG.

Main Results

The lung biopsy revealed interstitial thickening and immature interstitial cells with abundant cytoplasmic glycogen, consistent with PIG.
No evidence of maturational arrest, infection, alveolar proteinosis, or alveolar capillary dysplasia was found.
This represents the first reported case of PIG diagnosis in a premature infant with isolated PDA.

Conclusions

This case expands the understanding of pulmonary interstitial glycogenosis (PIG) by demonstrating its occurrence in a premature infant with isolated persistent patent ductus arteriosus (PDA) and severe pulmonary hypertension (PHT).
Lung biopsy is a valuable diagnostic tool for PIG in premature infants presenting with PDA and PHT, even without other congenital heart conditions.

Keywords:

patent ductus arteriosus (PDA) preterm pulmonary hypertension (PHT) pulmonary interstitial glycogenolysis (P.I.G)