Table 2.

Literature review of similar reports

Study	Year of publication	Population	RV affection	Conduction system disease	Arrhythmias	Diagnosis and follow-up
Ibsen et al. [11]	1985	Three members affected (one female and two males) 33 normal family members screened	Dilated RV (two patients), impaired LV function (one patient)	+	Atrial arrhythmias	Heart failure first, then arrhythmia and heart block; postmortem biopsy carried out for proband showed RV hypertrophy with fiber attenuation, fibrosis of replacement type, and fatty infiltration
Graber et al. [12]	1986	One family (history of 214 members and six generations was obtained); proband and other 9 members were affected	Biventricular failure	+	Atrial and ventricular arrhythmias	LV affection and ventricular arrhythmias (this could be an early report of LMNA mutation)
Caglar et al. [13]	1993	One patient	Isolated RV cardiomyopathy	+ Transient	+ Transient	Transient atrial flutter with heart block; patient refused pacing and was lost to follow-up
Blondheim et al. [14]	2000	Two sisters	+ Idiopathic RA dilatation and RV enlargement	+ One had complete heart block and VVI pacemaker	–	Presentation with heart block at younger age
Doi et al.[15]	2003	Two sisters	+ Isolated RV failure	+	–	Diagnosed as Fabry disease by endomyocardial biopsy, absence of extracardiac affection, LV hypertrophy diagnosed by echocardiography, restrictive pathology by catheterization
Biočić et al. [16]	2010	Brother and sister	Idiopathic RA enlargement, progressive RV dilatation at follow-up with normal LV function in first patient; his sister showed normal RV function and mild impairment of LV systolic function	+ Complete heart block (first patient)	+ Atrial fibrillation	SCD of the first patient (no biopsy) after short follow-up
Vakil et al. [17]	2014	One female patient	+ Isolated RV failure	+ (Complete heart block)	+ Atrial flutter	Diagnosed by pathology after transplantation, as isolated RV sarcoidosis
Figuero et al. [18]	2015	One male	Isolated RV cardiomyopathy	–	–	CMR (no scars, no ARVC), TEE: no shunts, right heart catheterization (RHC) and pulmonary angiography (PA) (excludes pulmonary hypertension (PH)); follow-up for 2 years: the patient is asymptomatic, and RV still dilated