Table 3.

Frequency of dysrhythmias and cardiac conduction disorders in FD.

Cardiac Manifestations	Frequencies	References
Palpitations	15.3% in untreated males 21.3% in untreated females	Linhart et al. [34]
Syncope	5.6% in untreated males 2.4% in untreated females	Linhart et al. [34]
Short PR interval	14% of patients	Namdar et al. [108]
Bradycardia	72% of patients	Lobo et al. [112]
Bradyarrhythmic events	23% of patients	Di et al. [114]
PR interval > 200 ms	3% of patients	O’Mahony et al. [111]
QRS duration > 120 ms	9% of patients	O’Mahony et al. [111]
Right bundle branch block	In late-onset phenotype with predominant cardiac involvement 38.8% of males Mean age at diagnosis of 61 ± 6 years 8.5% of females Mean age at diagnosis of 76 ± 9 years	Azevedo et al. [5]
Left anterior fascicular block	In late-onset phenotype with predominant cardiac involvement 45.5% of males Mean age at diagnosis of 60 ± 7 years 10.3% of females Mean age at diagnosis of 72 ± 14 years	Azevedo et al. [5]
Bifascicular block	In late-onset phenotype with predominant cardiac involvement 25.3% of males Mean age at diagnosis of 62 ± 6 years 5.7% of females Mean age at diagnosis of 78 ± 6 years	Azevedo et al. [5]
Complete AV block	In late-onset phenotype with predominant cardiac involvement 12.7% of males Mean age at diagnosis of 60 ± 7 years 1.6% of females Mean age at diagnosis of 79 ± 6 years	Azevedo et al. [5]
Atrial Fibrillation	3% of patients	O’Mahony et al. [111]
	3.9% of patients had persistent atrial fibrillation 13.3% of patients had paroxysmal atrial fibrillation	Shah et al. [98]
	Annual incidence: 1 per 100 person-years	Patel et al. [100]
	In late-onset phenotype with predominant cardiac involvement 7.6% of males Mean age at diagnosis of 67 ± 11 years 2.4% of females Mean age at diagnosis of 77 ± 3 years	Azevedo et al. [5]
Atrial Flutter	In late-onset phenotype with predominant cardiac involvement 2.5% of males 0.8% of females	Azevedo et al. [5]
Ventricular Arrhythmias	13% in males 20% in females	Pinderski et al. [116]
	Non-sustained VT in 8.3% of patients during 1.9 years of follow-up (n = 78)	Shah et al. [98]
	Non-sustained VT in 21% of patients Sustained VT in 1% of patients (Median follow-up of 36 months)	Hanneman et al. [117]
	Systematic review of the literature VT in 15.3% of the patients	Baig et al. [67]
	In late-onset phenotype with predominant cardiac involvement Non-sustained VT in 14.1% of males Mean age at diagnosis of 57 ± 8 years Non-sustained VT in 5.6% of females Mean age at diagnosis of 70 ± 6 years	Azevedo et al. [5]
SCD	SCD in 1 patient during 1.9 years of follow-up (n = 78)	Shah et al. [98]
	VF in 1 patient in the setting of multiorgan failure during mean follow-up of 53 months (n = 19)	Acharya et al. [125]
	Systematic review of the literature 62% of cardiac deaths are sudden Incidence of SCD events: 0.34–1.4% per annum	Baig et al. [67]
Cardiac device	Annual incidence: 1.9 per person-years	Sené et al. [123]
	Annual incidence of devices for the treatment of bradycardia (pacemaker/ICD): 1.07 per person-years	Patel et al. [100]
Pacemaker	12.5% of patients	Sené et al. [123]
	5-year incidence: 8%	O’Mahony [111]
	In late-onset phenotype with predominant cardiac involvement 12.7% in males Mean survival free from pacemaker: 71 ± 2 2.4% in females Mean survival free from pacemaker: 86 ± 1	Azevedo et al. [5]
ICD	4.2% of patients	Sené et al. [123]
	In late-onset phenotype with predominant cardiac involvement 1.3% in males 0.8% in females	Azevedo et al. [5]

AV, atrioventricular; ICD, implantable cardioverter-defibrillator; SCD, sudden cardiac death; VF, ventricular fibrillation; VT, ventricular tachycardia.