Table 2.

Frequency of manifestations related to ventricular hypertrophy, fibrosis and dysfunction in FD.

Cardiac Manifestations	Frequencies	References
LVH	43% in males Mean age of onset 39 ± 10 years 26% in females Mean age of onset 50 ± 11 years	Mehta et al. [31]
	76.9% in patients ≥ 75 years	Lidove et al. [33]
	In late-onset phenotype with predominant cardiac involvement 73.1% of males Mean age at diagnosis of 57 ± 10 years 19.0% of females Mean age at diagnosis of 73 ± 8 years	Azevedo et al. [5]
LGE	50% of patients	Moon et al. [48]
	In late-onset phenotype with predominant cardiac involvement 21.4% of patients	Azevedo et al. [5]
LV diastolic dysfunction	63% of patients with LGE	Niemann et al. [77]
	In late-onset phenotype with predominant cardiac involvement 29.2% of patients 69.4% of patients with LVH	Azevedo et al. [4]
LV systolic dysfunction (reduced EF)	6.7% of patients	Wu et al. [80]
Latent LVOT obstruction	43% of patients	Calcagnino et al. [90]
RV hypertrophy	31–71% of patients	Niemann et al. [92] Graziani et al. [93]
Heart Failure (or Dyspnoea)	19.4% in untreated males 19.7% in untreated females	Linhart et al. [34]
	34.6% in patients ≥ 75 years	Lidove et al. [33]
	Severe heart Failure (NYHA class ≥III) in 10% Annual incidence of severe heart failure: 1.62 per 100 person-years	Patel et al. [100]
	In late-onset phenotype with predominant cardiac involvement 32.9% of males Mean survival free from heart failure: 64 ± 1 years 14.8% of females Mean survival free from heart failure: 76 ± 2 years	Azevedo et al. [5]

EF, ejection fraction; LGE, late gadolinium enhancement; LV, left ventricular; LVH, left ventricular hypertrophy; LVOT, left ventricular outflow tract; NYHA, New York Heart Association; RV, right ventricular.