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Syncope from dynamic left ventricular outflow tract obstruction simulating hypertrophic cardiomyopathy in a patient
José-Luis E Velazquez-Ceceña1, David L Lubell, Nagapradeep Nagajothi
1Department of Cardiology, Mount Sinai Hospital Medical Center and the Chicago Medical School, California Avenue at 15th Street, Chicago, IL 60608, USA. joseluis_velazquez@hotmail.com
Insights
In cardiac amyloidosis patients resembling hypertrophic cardiomyopathy, dynamic left ventricular outflow tract (LVOT) obstruction can cause syncope. This study highlights LVOT obstruction as a key cause of fainting in these complex cases.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Nephrology
Background:
- Dynamic left ventricular outflow tract (LVOT) obstruction is a hallmark of hypertrophic cardiomyopathy.
- Cardiac amyloidosis can mimic hypertrophic cardiomyopathy with asymmetric hypertrophy.
- Syncope is frequent in cardiac amyloidosis but often attributed to other causes.
Observation:
- A patient with primary AL-type amyloidosis presented with hypertrophic cardiomyopathy-like features and LVOT obstruction.
- The patient experienced syncope attributed to dynamic LVOT obstruction.
- Provocation of dynamic LVOT obstruction reproduced presyncope symptoms.
Findings:
- Immunocyte-derived cardiac amyloidosis can cause dynamic LVOT obstruction.
- Dynamic LVOT obstruction was identified as the cause of syncope in this patient.
- Echocardiography revealed hypertrophic cardiomyopathy-like changes and LVOT obstruction.
Implications:
- Dynamic LVOT obstruction should be considered in syncope evaluation for cardiac amyloidosis patients.
- This finding expands the differential diagnosis for syncope in cardiac amyloidosis.
- Understanding this mechanism can improve diagnostic accuracy and patient management.
Abstract:
Dynamic left ventricular outflow tract (LVOT) obstruction is seen classically in hypertrophic cardiomyopathy. Cardiac amyloidosis can present with asymmetric hypertrophy that resembles hypertrophic cardiomyopathy, and, in some cases, with dynamic LVOT obstruction. The occurrence of syncope in such patients is not uncommon. The syncope is usually thought to be related to mechanisms other than LVOT obstruction, such as arrhythmias, conduction disturbances, orthostatic hypotension, or vasovagal effects associated with neuropathy.Herein, we report the case of a patient who had immunocyte-derived (primary AL-type) cardiac amyloidosis with the echocardiographic appearance of hypertrophic cardiomyopathy and evidence of LVOT obstruction that caused syncope. We were able to provoke and identify dynamic LVOT obstruction that produced presyncopal symptoms similar to those that typically occur in such patients spontaneously. Dynamic LVOT obstruction as a cause of syncope should be considered in patients who have cardiac amyloidosis and echocardiographic evidence of hypertrophic cardiomyopathy.
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