Assessment of Right Heart Functions in Children with Mild Cystic Fibrosis

Area of Science:

• Pediatric Cardiology
• Pulmonology
• Medical Imaging

Background:

• Cystic Fibrosis (CF) is a genetic disorder primarily affecting the lungs, leading to hypoxemia and potential right ventricle (RV) dysfunction.
• Progressive lung damage in CF can increase pulmonary artery systolic pressure (PASP), impacting RV anatomy and function.
• Early detection of RV alterations is crucial for managing CF progression.

Purpose of the Study:

• To investigate echocardiographic alterations in PASP, RV anatomy, and RV function in children with mild CF.
• To compare echocardiographic findings between children with mild CF and healthy controls.
• To evaluate the utility of novel echocardiographic techniques in assessing RV status in pediatric CF patients.

Main Methods:

• Conventional echocardiography was used to assess RV anatomy, systolic, and diastolic functions.
• New echocardiographic modalities, including pulmonary artery acceleration time (PAAT) and right ventricular ejection time (RVET) ratio (PAAT/RVET), were employed to estimate PASP.
• Echocardiographic data from 30 mild CF patients were compared with 30 healthy children.

Main Results:

• Pediatric CF patients exhibited significant differences in RV anatomy and function compared to controls.
• Novel echocardiographic parameters indicated elevated PASP in mild CF children compared to controls and published data (p<0.001).
• Variability in conventional parameters necessitated the exploration of new measurement techniques.

Conclusions:

• Elevated PASP and RV dysfunction, potentially leading to Cor pulmonale, can manifest early in children with mild CF.
• Routine echocardiography should incorporate advanced modalities like PAAT/RVET for comprehensive RV evaluation in mild CF.
• Early identification and monitoring of RV changes using novel echocardiography are recommended for children with mild CF.