Rapid and effective response of the R222Q SCN5A to quinidine treatment in a patient with Purkinje-related ventricular arrhythmia and familial dilated cardiomyopathy: a case report

Table 1 Clinical characteristics of living affected family members

Subject	III:1	IV:1
Mutation SCN5A	p.R222Q	p.R222Q, p.H558R
Age at onset/now (years)	25/57	7/31
Sex	Male	Male
Symptoms	Palpitations, presyncope	Palpitations/SCD -successfully resuscitated
NYHA functional class	III	IV- > 23 year old - HTx
LVdD (mm)/LVEF (%)	68/20	84/10-15
Arrhythmia	MPVCs/nsVT/IVR	MPVCs/nsVT/VT/VF/AF
Conduction disorders	AVB I^o, RBBB	No
ICD	Yes	Yes /explantation after HTx
Amiodarone	Yes - > hyperthyroidism	Yes - > unsuccessful
Quinidine treatment
Quinidine	Yes	No
Symptoms before/after	Yes/no	–
NYHA functional class	III- > II	–
LVdD (mm) before/after	68/62	–
LVEF (%) before/after	20/35	–
Arrhythmia during 24 h before/after	65,000 MPVCs/ ~ 4000 MPVCs	–

SCD sudden cardiac death, HTx heart transplantation, AVB I^o first degree of atrio–ventricular block, RBBB right bundle branch block, IVR idioventricular rhythm, MPVCs multifocal premature ventricular contractions, LVdD left ventricle enddiastolic diameter, LVEF left ventricle ejection fraction, VT ventricular tachycardia, ns non-sustained, VF ventricular fibrillation, AF atrial fibrillation, ICD implantable cardioverter–defibrillator

ISSN: 1471-2350