Assessment of clinical characteristics of cardiac amyloidosis as a potential underlying etiology in patients diagnosed with heart failure with preserved ejection fraction

Abstract

Background: Heart failure with preserved ejection fraction (HFpEF) is heterogeneous clinical syndrome. Transthyretin cardiac amyloidosis (CA) is an underdiagnosed cause of HFpEF. Red flags are extremely useful for suspecting CA. Aims:We aimed to evaluate the frequency of cardiac and extracardiac manifestations of CA in HFpEF patients based on red flags.Methods: Baseline characteristics of 85 patients were recorded during admission. Electrocardiogram and echocardiography were performed. All patients were examined for red flags. Cardiac scintigraphy was performed in 85 patients. Results:The mean (standard deviation [SD]) age of the study group was 67.9 (9.8) years, and 52 (61.2%) patients were female. At least 1 red flag was observed in 67% of HFpEF patients. Only 4 of the patients had more than 3 red flags. The mean number of red flags in a patient with HFpEF was 1.3. Extracardiac clinical red flags were observed in only 9 (10.5%) patients. Cardiac clinical red flags were extremely rare. An electrocardiographic red flag was detected in 2 out of 10 patients and an echocardiographic red flag in 4 out of 10 patients with HFpEF. Scintigraphy showed that 17.6% of all patients have had a grade 2 or 3 cardiac uptake. The patients with wild-type transthyretin CA had twice as many red flags as those without. Conclusion:The results of the study showed that patients diagnosed with HFpEF had an average of 1.3 red flags suggestive of CA. In real life, extracardiac red flags are rare, while electrocardiographic and echocardiographic red flags are more common in patients with HFpEF.

Current barriers and recommendations on the diagnosis of transthyretin amyloid cardiomyopathy: a Delphi study

“…A recent prospective, observational, single-center study from Turkey that aimed to evaluate the frequency of cardiac and extracardiac manifestations of CA in patients with heart failure with HFpEF based on red flags also confirms the necessity of timely expert center referrals as the study reported that patients diagnosed with HFpEF had an average of 1.3 red flags suggestive of CA (D-14). The same study also reported that patients with ATTR-CA had twice as many red flags as those without (2.46 vs. 1.04), and ATTR-CA diagnosis was more common in patients with 2 or more red flags ( 14 ).…”

“…Another multicenter, national, observational study examined 886 patients who applied to the cardiology clinics in 22 centers managed to identify 15 (1.69%) patients with CA who were diagnosed by endomyocardial biopsy (13). A prospective, observational, single-center study from Turkey also reported 15 patients (17.6%) with a positive specific scintigraphy result, confirming the presence of ATTR-CA among 85 patients with heart failure with preserved ejection fraction (HFpEF) (14).…”

The Most Predictive Red Flags for Suspecting Cardiac Amyloidosis in Patients with Heart Failure with Preserved Ejection Fraction

“…A previously published study with a smaller cohort of HFpEF patients reported that echocardiographic red flags were detected in six out of every ten patients with TTR-CA. 25 Among these red flags, unexplained left ventricular hypertrophy (LVH) is one of the most important findings, as it raises the clinician's suspicion of TTR-CA during the echocardiographic evaluation of a patient with HFpEF. Previous studies have reported that the prevalence of TTR-CA in patients with LVH ranges between 13% and 19%.…”

“…Previous studies have reported that the prevalence of TTR-CA in patients with LVH ranges between 13% and 19%. [25][26][27][28] While LVH is recognized as an important finding, a recent study found that the prevalence of CA in HFpEF patients without LVH was 5.2%. 24 In contrast, our study did not obtain any grade 2 or 3 99mTc-PYP scintigraphy results in HFpEF patients lacking LVH, and none of these patients were diagnosed with TTR-CA.…”

Clinical features, etiology and survival in patients with restrictive cardiomyopathy: A single center experience

“…As LVEF remains normal or slightly decreased in RCM patients for a long time [2], it is important to include amyloidosis [22] and na-RCM in differential diagnosis of HF with preserved ejection fraction. Although LVEF is commonly used in clinical practice, it is less suitable for assessing prognosis in RCM than RV function.…”