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A 78-year-old man with a history of bilateral carpal tunnel syndrome was admitted to our hospital with congestive heart failure. On physical examination a systolic ejection murmur was documented. B-type natriuretic peptide and Troponin I were elevated (8342 pg/ml and 0,2 ng/dl respectively).
Electrocardiogram showed a first-degree atrioventricular block and a pseudo-infarction pattern in V1-V2. Echocardiography documented severe biventricular thickening with a mild left ventricular dysfunction, mild pericardial effusion, a restrictive filling pattern, and a calcific aortic stenosis (AS) with a low-flow, low gradient pattern. Noncontrast CT quantitation of aortic valve calcium burden of 2150 AU confirmed AS severity. LV global longitudinal strain was markedly reduced (−10,5%), with a “cherry on the top” pattern (Fig. 1A). These findings were consistent with infiltrative cardiomyopathy. A Tc-99m-DPD scintigraphy showed strong cardiac uptake, grade 3 (Fig. 1B). After being discussed in a Heart Team session, the patient underwent a transcatheter aortic valve implantation (TAVI). During the procedure he suffered cardiac arrest and died of cardiogenic shock 24 h later.
1. What is the diagnosis?
Transthyretin cardiac amyloidosis.
Autopsy findings were consistent with transthyretin cardiac amyloidosis (CA). Macroscopic examination demonstrated a severe biventricular (right ventricle, Fig. 1C; left ventricle, Fig. 1D) and biatrial thickening (Fig. 1E). A biologic prosthetic aortic CoreValve and the native aortic valve after the procedure are shown in Fig. 1F and Fig. 1G, respectively). Congo Red staining revealed extracellular amyloid deposition in atria and ventricles (Fig. 1H) and immunofixation confirmed transthyretin deposition.
The association of AS and of CA is not uncommon in the elderly. In a prospective study of 151 patients [
], the CA prevalence of patients with severe AS undergoing TAVI was 16%. It was associated with a low-flow low-gradient phenotype and mildly reduced ejection fraction.
The therapeutic management in patients with AS and CA is challenging. TAVI procedure is often preferred to surgery, but it has been hypothesized that may be futile and associated with procedural complications, as in this case. However, in recent studies, TAVI implantation significantly improved outcome in this group of patients [