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Congenital heart disease-associated pulmonary dysplasia and its underlying mechanisms

De-Bao Li1, Xiu-Xia Xu2, Yu-Qing Hu3

  • 1Department of Thoracic and Cardiovascular Surgery, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai, People's Republic of China.

American Journal of Physiology. Lung Cellular and Molecular Physiology
|December 6, 2022
PubMed

Insights

Reduced pulmonary blood flow (RPF) in congenital heart disease (CHD) causes pulmonary dysplasia, impairing lung development and exercise capacity. Immunosuppressants may offer a therapeutic approach for this condition.

Area of Science:

  • Pulmonary Medicine
  • Pediatric Cardiology
  • Developmental Biology

Background:

  • Exercise capacity is crucial for survival in congenital heart disease (CHD).
  • Reduced pulmonary blood flow (RPF) in CHD significantly decreases exercise capacity, but the mechanism is unknown.
  • Pulmonary dysplasia is characterized by impaired alveolarization and vascularization.

Purpose of the Study:

  • To investigate the mechanism underlying pulmonary dysplasia in congenital heart disease with reduced pulmonary blood flow.
  • To explore the therapeutic potential of immunosuppressants in CHD-associated pulmonary dysplasia.

Main Methods:

  • Analysis of human RPF lung samples from tetralogy of Fallot patients.
  • Pulmonary artery banding in piglet and rat models to induce RPF.
  • RNA sequencing, flow cytometry, and cytokine detection to assess molecular changes.
  • Evaluation of cyclosporine A treatment for its effects on pulmonary dysplasia.

Main Results:

  • RPF led to impaired alveolarization and vascularization, smaller lungs, cyanosis, and reduced alveolar type 2 cells in animal models.
  • RNA sequencing revealed suppressed metabolism and migration, alongside heightened immune response in RPF lungs.
  • Cyclosporine A treatment improved pulmonary dysplasia and enhanced Wnt signaling pathway expression.

Conclusions:

  • Reduced pulmonary blood flow causes pulmonary dysplasia, contributing to reduced exercise capacity in CHD patients.
  • Immune response activation is implicated in the pathogenesis of RPF-induced pulmonary dysplasia.
  • Immunosuppressants demonstrate therapeutic potential for CHD-associated pulmonary dysplasia.

Keywords:
alveolicongenital heart diseasehumanlungpulmonary dysplasia

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