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Porcari et al. Vessel Plus 2022;6:33 https://dx.doi.org/10.20517/2574-1209.2021.134 Page 3 of 14

Table 1. Knowledge and uncertainty in the management of cardiac amyloidosis

Knowledge Uncertainties
AF Rate Improves HF symptoms; What is the prognostic impact of rate control compared to rhythm
control Drugs commonly used can be poorly tolerated and have a narrow therapeutic window; control?
Low-dose BB is generally safe and well-tolerated; Is rate control the strategy of choice?
Digoxin should be avoided; Which drugs should be used?
AV node ablation with biventricular PM implantation might be an option when other strategies When is rate preferred to rhythm control?
fail
Rhythm Exclude cardiac thrombosis before cardioversion; Does it improve HF symptoms?
control AF recurrence is frequent; Does maintaining SR have a prognostic impact?
Amiodarone is the agent of choice. Ibutilide and propafenone might be used under close Are markers of atrial remodeling commonly used in HF reliable for
monitoring of the QT interval and renal function; predicting the risk of AF recurrence after cardioversion?
DCCV has significant risk of complications; When should catheter ablation be performed?
Catheter ablation might be an option, but data are scarce and controversial Should PV isolation be associated with other ablation targets?
Thromboembolism & In AF Anticoagulation is recommended regardless of the CHA DS -VASc score; Which is the first-line anticoagulant in CA without other indications?
2
2
Anticoagulation Bleeding risk is frequently increased in CA (especially AL) and should be carefully assessed Are DOACs as effective as warfarin?
before initiation of anticoagulation Should LAA closure be considered in patients with prohibitive bleeding
risks?
In SR Advanced atrial dysfunction promotes embolic events; Should anticoagulation be used in SR?
CHA DS -VASc score ≥ 3 is associated with increased thromboembolic risk Should cardiac thrombosis be excluded in all patients?
2 2
How should we identify the best candidates?
Are transmitral A wave < 20 cm/s or LAA velocities < 40 cm/s useful?
CAD CAD should be regularly assessed and promoting factors should be treated as in patients Should PCI be preferred to surgery?
without CA; How shoulw we manage anti-thrombotic therapy?
Treatment of significant stenosis of the main epicardial coronaries confers a more benign How can we predict the risk of low cardiac syndrome after CABG?
outcome; How can we predict the impact of revascularization on symptoms and
Low cardiac output syndrome is frequent after coronary artery bypass surgery; prognosis?
Coronary revascularization might not ameliorate symptoms due to microvascular dysfunction How can microvascular dysfunction be relieved?
Treatment of AS Accurate characterization of AS is fundamental; When should we treat AS?
Untreated severe AS confers worse prognosis; Should LF-LG AV be regularly treated?
The prognostic benefit of treating LF-LG AS is debated; How should we monitor disease progression?
TAVI seems to improve the outcome of ATTR-CA patients compared to medical management; How can we predict the feasibility of TAVI and the risk of
LVEF to monitor disease progression is an inaccurate parameter in heavily hypertrophied complications?
hearts Should dual antiplatelet therapy follow TAVI?
Should patients with CAD and AS be treated with PCI and TAVI or
CABG and AVR?
SCD and ICD Ventricular arrhythmias are common in CA; Which parameters should guide the decision to implant an ICD?
No survival benefit is demonstrated following ICD implantation in CA; Could patients with biventricular systolic dysfunction benefit from ICD
AL-CA exhibits a high frequency of appropriate ICD shocks; placement?
ICD implantation for secondary prevention can be considered in AL or hereditary ATTR-CA How can we predict the risk of SCD with shockable rhythm to guide
(Class IIa, level of evidence C); prophylactic ICD implantation?
The role of prophylactic ICD remains controversial, but it might be considered as bridge to How should we select patients for secondary prevention of SCD?
transplant or in ischemic heart disease fulfilling indications for implantation Do ATTR and AL deserve different strategies for SCD prevention?
CRT LBBB is frequent in ATTR-CA at diagnosis; Is there a prognostic benefit of CRT in CA?
Decline in LVEF is a late phenomenon in CA; Is CRT effective in CA patients fulfilling indications according to latest