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Hemodynamic and angiocardiographic observations in familial amyloidosis with polyneuropahy
Insights
Cardiac amyloidosis in familial amyloidosis with polyneuropathy patients often shows impaired diastolic function, not typically congestive heart failure. Hemodynamic changes suggest restrictive cardiomyopathy and potential outflow tract obstruction.
Area of Science:
- Cardiology
- Genetics
- Neurology
Background:
- Familial amyloidosis with polyneuropathy (FAP) can affect the heart.
- Cardiac involvement in FAP, known as cardiac amyloidosis, is often associated with congestive heart failure.
Purpose of the Study:
- To investigate the hemodynamic patterns in patients with familial amyloidosis with polyneuropathy.
- To characterize cardiac function and identify potential obstructions in these patients.
Main Methods:
- Heart catheterization was performed on eleven patients.
- Right and left ventriculography and selective coronary angiography were conducted on eight patients.
Main Results:
- Congestive heart failure signs were present in only one patient.
- Systolic function was relatively preserved.
- Hemodynamic data indicated restrictive cardiomyopathy in several patients.
- Six patients exhibited signs of ventricular outflow tract obstruction.
- Impaired diastolic function was a key finding.
Conclusions:
- The hemodynamic profile in cardiac amyloidosis associated with FAP is not uniform.
- Diastolic dysfunction is a significant finding, alongside potential outflow tract obstruction.
- Findings challenge the typical presentation of congestive heart failure in cardiac amyloidosis.
Abstract:
Eleven patients with familial amyloidosis with polyneuropathy were studied by heart catheterization; eight of them also by right and left ventriculography and selective coronary angiography. Signs of congestive heart failure, often reported as typical of cardiac amyloidosis, were found in only one patient. The systolic function was relatively unimpaired, but in several patients hemodynamic data suggested restrictive cardiomyopathy. Six patients showed signs of right or left ventricular outflow tract obstruction. Thus, no uniform hemodynamic pattern could be identified, but the changes found indicate impaired diastolic function.