[Pulmonary vascular remodeling in congenital cardiovascular abnormalities: an eternal topic]

Area of Science:

• Oncology
• Cardiology
• Pharmacology

Context:

• Pulmonary arterial hypertension (PAH) is a severe complication of congenital heart defects with left-to-right shunts.
• Pulmonary vascular remodeling (PVR) is a critical factor in PAH pathogenesis.
• Current therapies focus on managing PVR, but new approaches are needed.

Purpose:

• To review emerging research on PVR from oncology and anti-tumor pharmacy.
• To explore the potential of the "ATM signal turning point hypothesis" in understanding PAH.
• To introduce novel lung-targeted drug delivery systems for PVR therapy.

Summary:

• The "ATM signal turning point hypothesis" from DNA damage response research suggests tumor-like vascular smooth muscle cell proliferation underlies obstructive PAH.
• A new lung-targeted drug delivery system utilizes low-concentration anti-tumor drugs to inhibit angiogenesis without cellular toxicity.
• These novel approaches offer potential new avenues for PVR therapy.

Impact:

• This review bridges oncology and PAH research, offering new perspectives on PVR.
• The "ATM signal turning point hypothesis" provides a novel framework for understanding PAH.
• Targeted drug delivery systems may lead to more effective and less toxic treatments for children with PAH.