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Persistent left ventricular disease in clinically "cured" primary endocardial fibroelastosis
Insights
Primary endocardial fibroelastosis treatment can resolve congestive heart failure, but cardiac abnormalities often persist. This suggests "cure" is incomplete, potentially explaining late deterioration in patients with this heart condition.
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.
Abstract:
We studied by serial cardiac catheterisation eight patients with the dilated form of primary endocardial fibroelastosis in whom congestive heart failure disappeared with treatment. All remained without symptoms for at least three years before recatheterization. Four patients showed regression of the abnormal electrocardiographic findings, three showed persistence, and one showed progression of electrocardiographic left ventricular overload pattern. On first cardiac catheterisation all patients had a dilated left ventricle with a mean ejection fraction of 0.36. In six of the patients repeat cardiac catheterisation showed left ventricular dilatation with a diminished ejection fraction (mean 0.32). Left ventricular end-diastole pressure was raised (12 to 28 mmHg, mean 19 mmHg). In this group were included the three patients with persistence and one with progression of the abnormal electrocardiographic findings, and two of the four patients with regression of these findings. The highest left ventricular end-diastolic pressure was found in a patient in whom the abnormal electrocardiographic findings almost reverted to normal. In the two remaining patients with reversion of the electrocardiographic abnormalities repeat cardiac catheterisation showed nothing abnormal. Our findings indicate that "cure" in primary endocardial fibroelastosis is incomplete. These findings may be the cause of sudden death or late clinical deterioration in some reported patients with "cured" primary endocardial fibroelastosis. The electrocardiogram is of little value in assessing these processes.