[Primary pulmonary hypertension in children].

Area of Science:

• Pediatric Cardiology
• Pulmonology
• Critical Care Medicine

Background:

• Primary pulmonary hypertension (PPH) is a severe, progressive, and often fatal clinical syndrome characterized by elevated pulmonary artery pressure.
• PPH is notoriously resistant to conventional therapies, posing significant challenges in pediatric patient management.

Observation:

• This case series details three pediatric patients diagnosed with PPH.
• Two patients experienced rapid deterioration and mortality shortly after diagnosis, with one succumbing during cardiac catheterization.
• The third patient demonstrated a positive initial response to a combination of calcium channel blockers, diuretics, and nocturnal oxygen therapy.

Findings:

• The third patient exhibited improved quality of life and hemodynamic parameters for approximately 2.5 years following treatment initiation.
• Despite initial benefits, her condition eventually worsened, leading to death three years post-diagnosis.
• Non-invasive investigations like pulse oximetry, echocardiography, and exercise testing are deemed sufficient for initiating and monitoring PPH therapy.

Implications:

• Early identification and intervention using non-invasive methods are crucial for managing pediatric PPH.
• Calcium channel blockers may offer a therapeutic benefit for a subset of PPH patients exhibiting vasodilatory responses.
• Further research into effective long-term treatments for pediatric PPH is warranted given its poor prognosis.