Persistent left ventricular disease in clinically "cured" primary endocardial fibroelastosis.

Area of Science:

• Cardiology
• Pediatric Cardiology
• Cardiovascular Research

Background:

• Primary endocardial fibroelastosis (PEF) is a rare condition characterized by fibrosis and elastin proliferation in the endocardium.
• Dilated forms of PEF can lead to congestive heart failure, particularly in infants and children.
• Successful treatment of heart failure symptoms in PEF may not equate to complete disease resolution.

Purpose of the Study:

• To assess the long-term cardiac status of patients with dilated primary endocardial fibroelastosis after initial treatment success.
• To investigate the persistence or regression of cardiac abnormalities, including left ventricular function and pressures, following clinical recovery.
• To evaluate the utility of electrocardiography in monitoring disease status in treated PEF patients.

Main Methods:

• Serial cardiac catheterization was performed on eight patients with dilated PEF who had shown resolution of congestive heart failure.
• Echocardiography and electrocardiography were used to assess cardiac structure and function before and after treatment.
• Left ventricular dimensions, ejection fraction, and end-diastolic pressure were key parameters evaluated during catheterization.

Main Results:

• Despite clinical improvement and symptom resolution for at least three years, repeat cardiac catheterization revealed persistent left ventricular dilatation and reduced ejection fraction in six of eight patients.
• Elevated left ventricular end-diastolic pressure was observed in most patients, even those with improved electrocardiographic findings.
• Electrocardiographic changes showed variable responses, with regression in four, persistence in three, and progression in one, indicating limited correlation with underlying cardiac status.

Conclusions:

• Clinical "cure" of congestive heart failure in primary endocardial fibroelastosis does not signify complete resolution of cardiac pathology.
• Persistent subclinical cardiac abnormalities, including ventricular dysfunction and elevated filling pressures, may predispose patients to sudden death or late clinical deterioration.
• Electrocardiography has limited value in assessing the true extent of disease or predicting outcomes in treated PEF patients, underscoring the importance of advanced cardiac imaging and hemodynamic assessment.