Which Functional Classification Scale is Optimal for Children with Pulmonary Hypertension (PAH)?

Area of Science:

• Pediatric Cardiology
• Pulmonary Hypertension Research
• Clinical Assessment Tools

Background:

• The World Health Organization Functional Class (WHO-FC) scale, used for adults, is a prognostic factor in pulmonary hypertension.
• Limited data exist on the Panama-FC scale's utility in pediatric pulmonary hypertension.
• Assessing functional status in children with pulmonary hypertension requires validated tools.

Purpose of the Study:

• To compare the WHO-FC and Panama-FC scales in pediatric pulmonary hypertension.
• To evaluate the Panama-FC scale's usefulness in different clinical scenarios.
• To examine the reliability of a Panama-FC questionnaire for patient assessment.

Main Methods:

• Analyzed 26 functional status assessments from 19 pediatric pulmonary arterial hypertension patients.
• Utilized WHO-FC, Panama-FC (interview), and Panama-FC (questionnaire) independently.
• Correlated scale results with 6-minute walking distance (6MWD), NTproBNP, and echocardiographic parameters (TAPSE, RV/LV ratio).

Main Results:

• Panama-FC interview and questionnaire results were consistent.
• Both WHO-FC and Panama-FC accurately reflected disease progression via NTproBNP, 6MWD, TAPSE, and RV/LV ratio.
• Discrepancies in risk stratification were noted between scales; Panama-FC identified some patients in higher risk categories.
• Functional status changes correlated with NTproBNP levels post-treatment intensification, but not 6MWD.
• Echocardiographic parameters changed significantly in some patients, aligning with functional class changes.

Conclusions:

• Both WHO-FC and Panama-FC scales effectively reflect disease advancement in pediatric pulmonary hypertension.
• The Panama-FC questionnaire simplifies the scale's application.
• Panama-FC is advantageous for long-term follow-up, while WHO-FC is more suitable for short-term treatment monitoring.