Skill Checkup: An Electrical Conundrum

Xiaoxiao Qian, MD; Raghav Gattani, MD; Chirag M. Sandesara, MD


June 11, 2021

In the clinical scenario of syncope, conduction system abnormality (first-degree AV block), heart failure, and significantly decreased GLS with apical sparing on echocardiography, cardiac amyloidosis should be considered. However, the clinical presentation, ECG changes, and echocardiographic findings can be nonspecific, and it is important to consider alternative etiologies.

Patients with sarcoidosis can also present with heart failure, conduction system abnormalities, low voltage QRS on ECG, and decreased GLS on echocardiography. However, the apical-sparing strain pattern is a key differentiator between amyloidosis and sarcoidosis.

Patients with uncontrolled hypertension, which this patient has, can have severe concentric left ventricular hypertrophy on echocardiography that mimics infiltrative cardiomyopathy. However, the ECG in ventricular hypertrophy demonstrates increased-voltage QRS complexes, not low-voltage complexes as in this patient's case. The presence of low-voltage QRS complexes in the setting of significantly increased left ventricular wall thickness is suggestive of an infiltrative process. Conduction system disease in amyloidosis is usually caused by infiltration into the atrioventricular nodal tissue and His-Purkinje system; however, sinus node disease has also been observed.

Syncope, presyncope, dizziness, angina, palpitations, shortness of breath, and fatigue are clinical symptoms of hypertrophic cardiomyopathy. Sudden death can also occur. Echocardiography in patients with hypertrophic cardiomyopathy can demonstrate left ventricular and septal hypertrophy, left atrial enlargement, decreased left ventricular chamber size, and decreased mid-aortic flow. This patient's clinical picture and echocardiographic findings are not consistent with hypertrophic cardiomyopathy.


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