Page 78 - Read Online

Canepa et al. Vessel Plus 2022;6:30 https://dx.doi.org/10.20517/2574-1209.2021.106 Page 5 of 13

shown that HF patients treated by non-cardiologists were older, more often female, with more
comorbidities, and with HFpEF [37-42] . Although these findings need to be confirmed in larger multinational
consecutive HF populations, they should be carefully considered in light of the high ATTR-CA prevalence
in HFpEF discussed above. Awareness and knowledge of CA (and particularly of ATTR-CA) is rapidly
expanding within the cardiology community, whereas it should be increasingly shared with internal
medicine physicians and physicians from other specialties, who are apparently caring for the vast majority
of the HFpEF population. This is of utmost importance when one also considers that no therapeutic
[44]
solutions are currently available for patients with HFpEF , with the exception of sodium-glucose co-
transporter 2 inhibitors . The discovery of a specific and potentially treatable etiology of HFpEF such as in
[45]
the case of ATTR-CA may have a significant impact on the management and prognosis of the affected
patient. In relation to this, it has also been argued that the inclusion of patients with a missed diagnosis of
CA in HFpEF studies might have significantly hindered the results of some recent HFpEF clinical trials and
[46]
contributed to the failure of some HFpEF treatments . Thus, a multistep screening strategy is today
suggested in HFpEF patients, recommending those with red flags for CA to undergo both a light-chain
assay and a bone scintigraphy to exclude for the presence of CA. These further advancements in knowledge
should be spread within the whole community of physicians caring for the HFpEF population today. At
present, cardiologists should always be involved in the management of these patients, particularly in the in-
patient setting , offering rapid consultation and specific diagnostic and therapeutic directives.
[42]
Aortic valve stenosis
AS is the most common primary valve disease leading to surgery or catheter intervention in Europe and
North America, with a growing prevalence due to the aging population. It causes hemodynamic overload of
the left ventricle and secondary LVH. Particularly in older subjects, AS may be underdiagnosed, as doctors
and patients explain their declining exercise capacity with age [47,48] and under-treated due to the increased
surgical risk with aging. However, the advent of percutaneous intervention has significantly contributed to
increasing the number of potential candidates to valve replacement therapy, whose efficacy vs. standard
[49]
surgical procedure has also been recently demonstrated in lower-risk groups . This expanding population
of AS patients coming earlier to diagnosis and treatment could be the focus of a global effort aiming at
excluding potential overlapping causes of LVH and particularly CA. Characteristics of AS candidates to
valve replacement considered at risk of CA have been identified and include patients with low-voltage
patterns and conduction abnormalities at electrocardiography and those with low-flow low-gradient
phenotype and restrictive filling transmitral patterns at echocardiography . Echocardiography remains the
[50]
fundamental examination for the diagnosis of LVH, which represents a primary trigger of ATTR-CA
suspicion. The diagnostic yield of echocardiography could be significantly improved today by increasing the
awareness of secondary causes of LVH among cardiologists performing the test. Thus, the
echocardiography laboratory is a key place for investments aimed at raising the suspicion of ATTR-CA and
increasing the number of early-stage ATTR-CA diagnoses.

HCM
Once considered a disease of the young, with sudden death in athletes being its most ominous clinical
manifestation, HCM is now recognized as a more common condition, which is increasingly diagnosed in
older middle-aged adults [51,52] . The most recent data from the international SHaRe registry (Sarcomeric
Human Cardiomyopathy Registry) including over 7000 HCM patients diagnosed between 1961 and 2019 at
six American and five non-American HCM referral centers show that the mean age at HCM diagnosis has
been steadily increasing in the years, and it is nowadays an average of 52 years . The rate of diagnoses > 60
[53]
years increased from 9.2% before 2000 to 31.8% after 2010, and the prevalence of patients diagnosed at > 70
years reached 10.7% after 2010. In addition, this greater representation of older patients was associated with
[53]
a more sporadic form of disease, milder phenotypes, and more frequent genotype-negative status . When