Table 2 Overview of included studies

Spinelli et al. [9]

Italy

Genetically proven FD with normal left ventricular ejection fraction

eGFR, LVMI

Cardiac events (defined as cardiac death, malignant ventricular tachycardia, atrial fibrillation and severe cardiac failure)

Graziani et al. [6, 15]

Italy

Genetically confirmed FD patients belonging to 20 different families

eGFR, LVMI

Composite measure of major events: cardiac failure, atrial fibrillation, stroke, progression to dialysis or eGFR decline

Hanneman et al. [8, 16]

Canada

Patients aged ≥ 18 years with gene-positive FD

LVMI

Cardiac failure, cardiac death

Feriozzi et al. [12, 18]

USA

Male and female patients aged ≥ 18 years with FD treated with agalsidase alfa

eGFR, LVMI

Myocardial infarction/cardiac failure, renal failure

Lenders et al. [10]

Germany

Classical or late-onset clinical phenotype form of FD including FD-typical signs and symptoms (patients with genetic variants of unknown significance and polymorphisms were not included)

eGFR

Myocardial infarction, ESRD

Siegenthaler et al. [13, 17]

Switzerland

Genetically confirmed FD patients

eGFR, LVMI

Composite measure including: cardiac failure, cerebrovascular events or death

Patel et al. [11]

Multiple

Previously untreated FD patients

eGFR

Mortality: cardiac events, stroke

Arends et al. [5, 14]

Netherlands, UK, and Germany

Adults with a definite FD diagnosis who were treatment-naïve and subsequently treated with either agalsidase alfa or agalsidase beta for ≥ 9 months

eGFR, LVMI

Clinical events: Renal event, cardiac arrest, cerebral events