Diagnostic evaluation of paediatric pulmonary hypertension in current clinical practice

Area of Science:

• Pediatric Cardiology
• Pulmonary Hypertension Research
• Clinical Practice Guidelines

Background:

• Current pediatric pulmonary hypertension (PH) diagnostic algorithms exist, but their clinical application remains unclear.
• The Tracking Outcomes and Practice in Paediatric Pulmonary hypertension (TOPP) registry provides insights into current diagnostic workup.
• Evaluation of diagnostic practices and complications of heart catheterization (HC) in pediatric PH is crucial.

Purpose of the Study:

• To describe the diagnostic workup for suspected PH in the TOPP registry.
• To analyze the frequency and types of complications associated with heart catheterization in pediatric PH patients.
• To assess adherence to current diagnostic guidelines in pediatric PH.

Main Methods:

• Analysis of 456 patients enrolled in the TOPP registry until February 2010 for diagnostic workup.
• Review of 908 heart catheterizations (HCs) reported until February 2012 to evaluate complications.
• Assessment of noninvasive test utilization (ECG, echocardiograms, chest radiographs) and HC-related adverse events.

Main Results:

• ECGs (94%), echocardiograms (96%), and chest radiographs (89%) were the most frequently used noninvasive tests for suspected PH.
• No patient had all three noninvasive tests normal, suggesting their combined utility in ruling out PH.
• Complications were reported in 5.9% of HCs, with five deaths attributed to HC, potentially indicating a higher rate than in adult studies.

Conclusions:

• Most children in the TOPP registry did not receive the full diagnostic workup recommended for adults.
• The high utilization of noninvasive tests suggests their importance, while their combined normal results may rule out PH.
• Further data is needed to establish the appropriate risk-benefit ratio for HC in pediatric PH, given observed complication rates.