Cardiol Res

Cardiology Research, ISSN 1923-2829 print, 1923-2837 online, Open Access

Article copyright, the authors; Journal compilation copyright, Cardiol Res and Elmer Press Inc

Journal website https://www.cardiologyres.org

Original Article

Volume 9, Number 4, August 2018, pages 204-214

Appropriate and Inappropriate Implantable Cardioverter Defibrillators Therapies in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia Patients

Figure 1. ECG of a patient with ARVC/D showing the presence of an epsilon wave (electric potentials after the end of the QRS complex) (red arrows) and T-wave inversion in V1 - V5.

Figure 2. ECG in a patient with ARVC/D showing ventricular tachycardia with a left bundle branch block (LBBB) morphology and superior axis suggesting a RV inferior wall origin.

Figure 3. Parasternal long axis (a) and apical four-chamber (b) echocardiographic views showing RV dilatation [13].

Figure 4. (a) Axial cine SSFP (steady-state free precession) MRI showing significant RV dilatation and (b) black-blood-prepared HASTE (half-Fourier acquired single-shot turbo spin echo) axial slice MRI at level of RVOT showing RVOT dilatation [13].

Figure 6. Causes of inappropriate implantable cardioverter defibrillator (ICD) therapy. AF: atrial fibrillation; EMI: elecromanganic interference; ST: sinus tachycardia; SVT: supraventricular tachycardia.

**Table 1.** Clinical Characteristics of the Patients
Patient characteristics	ICD primary prevention N (%)	ICD secondary prevention N (%)	P value
Male (%)	6 (85.7 %)	14 (93.3%)	0.563
Female (%)	1 (14.3 %)	1 (6.7%)	0.563
Clinical presentation
Palpitations	5 (71.4%)	14 (93.3%)	0.163
Syncope	1 (14.3 %)	2 (13.3%)	0.952
Shortness of breath	2 (28.6%)	2 (13.3%)	0.388
Chest pain	1 (14.3 %)	2 (13.3%)	0.952
Dizziness	1 (14.3 %)	2 (13.3%)	0.952
Ventricular tachycardia	0 (0.0%)	12 (80%)	0.000
Sudden cardiac death	0 (0.0%)	3 (20.0%)	0.203
Medications
Amiodarone	0 (0.0%)	3 (20.0%)	0.203
Sotalol	1 (14.0%)	6 (40.0%)	0.228
Beta blockers	4 (57.1%)	7 (46.7%)	0.647
Angiotensin-converting enzyme inhibitors (ACE inhibitors)	2 (28.6%)	7 (46.7%)	0.421
Spironolactone	0 (0.0%)	2 (13.3%)	0.311
Implantable cardioverter defibrillator (ICD) therapy
Single chamber ICD	3 (42.9%)	10 (66.7%)	0.296
Appropriate ICD therapy	1 (14.0%)	10 (66.7%)	0.022
Inappropriate ICD therapy	1 (14.0%)	4 (26.7%)	0.519
Genetic results
Positive	3 (13.6%)	7 (31.8%)	0.672
Negative	2 (9.1%)	2 (9.1%)
Not performed	2 (9.1%)	6 (27.3%)

Table 1. Clinical Characteristics of the Patients

Patient characteristics

ICD primary prevention N (%)

ICD secondary prevention N (%)

P value

Male (%)

6 (85.7 %)

14 (93.3%)

0.563

Female (%)

1 (14.3 %)

1 (6.7%)

0.563

Clinical presentation

Palpitations

5 (71.4%)

14 (93.3%)

0.163

Syncope

1 (14.3 %)

2 (13.3%)

0.952

Shortness of breath

2 (28.6%)

2 (13.3%)

0.388

Chest pain

1 (14.3 %)

2 (13.3%)

0.952

Dizziness

1 (14.3 %)

2 (13.3%)

0.952

Ventricular tachycardia

0 (0.0%)

12 (80%)

0.000

Sudden cardiac death

0 (0.0%)

3 (20.0%)

0.203

Medications

Amiodarone

0 (0.0%)

3 (20.0%)

0.203

Sotalol

1 (14.0%)

6 (40.0%)

0.228

Beta blockers

4 (57.1%)

7 (46.7%)

0.647

Angiotensin-converting enzyme inhibitors (ACE inhibitors)

2 (28.6%)

7 (46.7%)

0.421

Spironolactone

0 (0.0%)

2 (13.3%)

0.311

Implantable cardioverter defibrillator (ICD) therapy

Single chamber ICD

3 (42.9%)

10 (66.7%)

0.296

Appropriate ICD therapy

1 (14.0%)

10 (66.7%)

0.022

Inappropriate ICD therapy

1 (14.0%)

4 (26.7%)

0.519

Genetic results

Positive

3 (13.6%)

7 (31.8%)

0.672

Negative

2 (9.1%)

Not performed

2 (9.1%)

6 (27.3%)

**Table 2.** Summary of ECG Changes
Patients	Epsilon wave (M)*	T-wave inversion in V1-3 or beyond (M)*	T-wave inversion in V1-2 or V4, 5 or 6 (m)*	RBBB + T-wave inversion in V1-4 (m)*	Terminal activation duration of QRS in V1, 2 or 3 ≥ 55 ms (m)*
*M indicates major, and m indicates minor diagnostic criterion based on 2010 ARVC/D Task Force Criteria [4]. RBBB: right bundle-branch block. -: absent; +: present.
1	+	+	-	-	+
2	-	+	-	-	-
3	-	+	-	-	-
4	-	+	-	-	-
5	+	+	-	-	-
6	-	+	-	-	-
7	-	+	-	-	-
8	+	+	-	-	-
9	-	-	-	-	-
10	+	+	-	-	-
11	-	+	-	-	-
12	-	-	-	-	-
13	-	-	+	-	+
14	-	-	-	-	-
15	+	+	-	-	+
16	-	-	+	-	+
17	-	+	-	-	-
18	-	+	-	-	+
19	+	+	-	-	-
20	-	+	-	-	-
21	-	-	-	-	-
22	-	-	-	-	+

Table 2. Summary of ECG Changes

Patients

Epsilon wave (M)*

T-wave inversion in V1-3 or beyond (M)*

T-wave inversion in V1-2 or V4, 5 or 6 (m)*

RBBB + T-wave inversion in V1-4 (m)*

Terminal activation duration of QRS in V1, 2 or 3 ≥ 55 ms (m)*

*M indicates major, and m indicates minor diagnostic criterion based on 2010 ARVC/D Task Force Criteria [4]. RBBB: right bundle-branch block. -: absent; +: present.

**Table 3.** Summary of Echocardiogram Changes
Patients no.	LVEF	RV systolic function	PLAX RVOT (corrected for body size (PLAX/BSA))	PSAX RVOT (corrected for body size (PSAX/BSA))	Other abnormalities
BSA: body surface area; LVEF: Left ventricular ejection fraction; mm: millimeters; m²: square meter; PLAX: parasternal long-axis view; PSAX: parasternal short-axis view; PR: pulmonic valvular regurgitation; RV: right ventricle; RVOT: RV outflow tract; SPAP: systolic pulmonary artery pressure; TR: tricuspid regurgitation. For the 2D echo major and minor criteria values, see reference [4].
1	> 55%	Mildly reduced	43 mm (21.5 mm/m²)	46 mm (23 mm/m²)
2	45-50% (47.5%)	Moderately to severely reduced	51 mm (24.3 m/m²)	48 mm (29 mm/m²)	RV apical trabeculations and small aneurysms
3	50-55% (52.5%)	Mildly to moderately reduced	33 mm (17.3 mm/m²)	32 mm (16.8 mm/m²)	RV wall thinning, multiple microaneurysms and hyertrabeculations. Mild TR
4	> 55%	Moderately reduced	34 mm (16 mm/m²)	41 mm (19 mm/m²)	RV thinned and akinetic wall, multiple microaneurysms and prominent trabeculations Moderate TR
5	> 55%	Mildly reduced	42 mm (23.3 mm/m²)	45 mm (25 mm/m²)	RV regional hypokinesia Mild TR
6	> 50%	Severely reduced	34 (22.7 mm/m²)	36 mm (24 mm/m²)	Severe TR
7	> 55%	Mildly reduced	28 mm (14 mm/m²)	31 mm (15.5 mm/m²)	RV thin wall with multiple microaneurysms in the apex and RVOT prominent, disarrayed trabeculations and dyskinetic anterior wall of RVOT
8	50-55%	Moderately to severely reduced	30 mm (15.8 mm/m²)	35 mm (18.4 mm/m²)	Thin and fibrosed RV wall with prominent trabeculations Moderate TR SPAP 35 - 40 mm Hg.
9	> 55%	Normal	20 mm (10 mm/m²)	24 mm (12 mm/m²)
10	30-35%	Severely reduced	41 mm (34.2 mm/m²)	40 mm (33.3 mm/m²)	Hypertrabeculated RV Severe TR Mild-to-moderate PR
11	45 %	Severely reduced	33 mm (19.4 mm/m²)	33 mm (19.4 mm/m²)	Thinning of the RV free wall
12	> 55%	Normal	30 mm (18.7 mm/m²)	34 mm (21.25 mm/m²)
13	> 55%	Low normal	29 mm (15.26 mm/m²)	28 mm (14.7 mm/m²)	Thinning of the apical RV wall
14	51%	Normal	23 mm (11.5 mm/m²)	30 mm (15 mm/m²)	RV systolic pressure 30 - 40 mm Hg
15	44%	Normal	32 mm (20 mm/m²)	39 mm (24.4 mm/m²)
16	45%	Normal	29 mm (16 mm/m²)	31 mm (17 mm/m²)	RV systolic pressure 30- 35 mmHg
17	> 55%	Normal	28 mm (18.7 mm/m²)	30 mm (20 mm/m²)
18	> 55%	Moderately to severely reduced	36 mm (24 mm/m²)	36 mm (24 mm/m²)	Mild TR
19	> 55%	Mildly reduced	23 mm (13.5 mm/m²)	28 mm (16.5 mm/m²)
20	< 25%	Severely reduced	47 mm (29.4 mm/m²)	48 mm (30 mm/m²)	Markedly trabeculated RV
21	> 55%	Normal	27 mm (14 mm/m²)	24 mm (12.6 mm/m²)
22	> 55%	Normal	21 mm (17.5 mm/m²)	15 mm (12.5 mm/m²)

Table 3. Summary of Echocardiogram Changes

Patients no.

LVEF

RV systolic function

PLAX RVOT (corrected for body size (PLAX/BSA))

PSAX RVOT (corrected for body size (PSAX/BSA))

Other abnormalities

BSA: body surface area; LVEF: Left ventricular ejection fraction; mm: millimeters; m²: square meter; PLAX: parasternal long-axis view; PSAX: parasternal short-axis view; PR: pulmonic valvular regurgitation; RV: right ventricle; RVOT: RV outflow tract; SPAP: systolic pulmonary artery pressure; TR: tricuspid regurgitation. For the 2D echo major and minor criteria values, see reference [4].

> 55%

Mildly reduced

43 mm (21.5 mm/m²)

46 mm (23 mm/m²)

45-50% (47.5%)

Moderately to severely reduced

51 mm (24.3 m/m²)

48 mm (29 mm/m²)

RV apical trabeculations and small aneurysms

50-55% (52.5%)

Mildly to moderately reduced

33 mm (17.3 mm/m²)

32 mm (16.8 mm/m²)

RV wall thinning, multiple microaneurysms and hyertrabeculations.
Mild TR

> 55%

Moderately reduced

34 mm (16 mm/m²)

41 mm (19 mm/m²)

RV thinned and akinetic wall, multiple microaneurysms and prominent trabeculations
Moderate TR

> 55%

Mildly reduced

42 mm (23.3 mm/m²)

45 mm (25 mm/m²)

RV regional hypokinesia
Mild TR

> 50%

Severely reduced

34 (22.7 mm/m²)

36 mm (24 mm/m²)

Severe TR

> 55%

Mildly reduced

28 mm (14 mm/m²)

31 mm (15.5 mm/m²)

RV thin wall with multiple microaneurysms in the apex and RVOT prominent, disarrayed trabeculations and dyskinetic anterior wall of RVOT

50-55%

Moderately to severely reduced

30 mm (15.8 mm/m²)

35 mm (18.4 mm/m²)

Thin and fibrosed RV wall with prominent trabeculations
Moderate TR
SPAP 35 - 40 mm Hg.

> 55%

Normal

20 mm (10 mm/m²)

24 mm (12 mm/m²)

30-35%

Severely reduced

41 mm (34.2 mm/m²)

40 mm (33.3 mm/m²)

Hypertrabeculated RV
Severe TR
Mild-to-moderate PR

45 %

Severely reduced

33 mm (19.4 mm/m²)

Thinning of the RV free wall

> 55%

Normal

30 mm (18.7 mm/m²)

34 mm (21.25 mm/m²)

> 55%

Low normal

29 mm (15.26 mm/m²)

28 mm (14.7 mm/m²)

Thinning of the apical RV wall

51%

Normal

23 mm (11.5 mm/m²)

30 mm (15 mm/m²)

RV systolic pressure 30 - 40 mm Hg

44%

Normal

32 mm (20 mm/m²)

39 mm (24.4 mm/m²)

45%

Normal

29 mm (16 mm/m²)

31 mm (17 mm/m²)

RV systolic pressure 30- 35 mmHg

> 55%

Normal

28 mm (18.7 mm/m²)

30 mm (20 mm/m²)

> 55%

Moderately to severely reduced

36 mm (24 mm/m²)

Mild TR

> 55%

Mildly reduced

23 mm (13.5 mm/m²)

28 mm (16.5 mm/m²)

< 25%

Severely reduced

47 mm (29.4 mm/m²)

48 mm (30 mm/m²)

Markedly trabeculated RV

> 55%

Normal

27 mm (14 mm/m²)

24 mm (12.6 mm/m²)

> 55%

Normal

21 mm (17.5 mm/m²)

15 mm (12.5 mm/m²)

**Table 4.** Summary of Cardiac Magnetic Resonance Imaging (MRI) Changes
Study no./gender	Left ventricle (LV)	Right ventricle (RV)
*Between practices values corrected for body size (value/body surface area (BSA)). EF: ejection fraction; EDV: end-diastolic volume; ESV: end-systolic volume mm millimeters; m²: square meter, TR: tricuspid valve. For the cardiac MRI major and minor criteria values, see reference [4].
1/M	Normal with EF 55%	EDV 151 mL (79.5 mL/m²)* ESV 83 mL (43.7 mL/m²) RVEF 45% Moderately to severely dilated with dyskinetic motion in the free wall and basal aneurysm T1-weighted images show area of severe thinning on the free wall of right ventricle
2/M	Normal EDV 153 mL ESV 56 mL EF 63%	EDV 178 mL (93.7 mL/m²) ESV 121 mL (63.7 mL/m²) EF 32% Moderate dilatation with generalized hypokinesis. There are localized RV areas of micro-aneurysms, mainly at the RVOT and the RV apex Prominent papillary muscle and the trabeculation
3/M	EF > 55%	EDV 100 mL (50 mL/m²) ESV 48 mL (24 mL/m²) EF 52% Thinned of the free wall with a small area of severe thinning, nears the RV apex with dyskinesis and diastolic bulging fulfilling the criterion of aneurysm
4/F	Normal systolic function	EDV 249 mL (207 mL/m²) ESV 212 mL (176.7 mL/m²) EF 15% Stroke volume 36.2 mL Severely dilated and hypertrabiculated Very thinned out RV especially apical regions with evidence of microaneurysm and diastolic bulge and segmental dilatation of RV The RVOT is dilated with hypokinesia. Evidence of at least mild-to-moderate TR regurgitation
5/F	EDV 83 mL ESV 41 mL EF 50% Mild global hypokinesis and akinesis of the distal septum	EDV 120 mL (70.6 mL/m²) RV ESV 83 mL (48.8 mL/m²) EF 31% Stroke volume 37 mL Severely dilated with severe global hypokinesis and dyskinesis of the RV apex and severe thinning noted in both cine and dark blood imaging Delayed myocardial enhancement demonstrates severe thinning of the free wall of the right ventricle
6/M	EDV 99 mL ESV 39 mL EF 60% Stroke volume 59 mL	EDV 81 mL (50.6 mL/m²) ESV 52 mL (32.4 mL/m²) RVEF 36% Mildly-to-moderately dilated with dyskinesia and akinesis of the free wall adjacent to the right ventricular apex T1, T2 and delayed gadolinium enhancement demonstrate two focal aneurysms in the free wall of RV
7/M	Normal EF 58%	EDV 171 mL (90 mL/m²) ESV 93 mL (49 mL/m²) EF 49% Stroke volume 76 mL( 40 mL/m²) Focal dyskinesia in the RVOT Small patent ductus arteriosus measuring about 5 mm. Main pulmonary artery (MPA) not enlarged measuring 26 mm
8/M	Normal EF 57%	EDV 87 mL (43.5 mL/m²) ESV 47 mL (23.5 mL/m²) EF 46% Mildly dilated with mild global hypokinesis Focal aneurysm noted in RV free wall or RVOT
9/M	Normal LV	Moderately dilated RV and reduced RV function (CMRI was performed in another cardiac center and full report no available)
10/M	EDV 110.5 mL (65 mL/m²) ESV 49.3 mL (29 mL/m²) EF 54%	EDV187.5 mL (105 mL/m²) ESV 100 mL(59 mL/m²) EF 44%
11/M	EF 45-50%	Quantification was not apparent at this point due to the poor ECG gating Dilated at the RVOT, there is evidence of macroaneurysm with small microaneurysm noted at the RV apex with a large macroaneurysm noted at the RVOT and RV free wall with dyskinesis Overall RV systolic function appeared to be mildly reduced
12/M	EDV 80 mL ESV 25 mL EF 68%	EDV 74 mL ESV 26 mL EF 64% Late gadolinium enhancement in mid myocardium at the septum

Table 4. Summary of Cardiac Magnetic Resonance Imaging (MRI) Changes

Study no./gender

Left ventricle (LV)

Right ventricle (RV)

*Between practices values corrected for body size (value/body surface area (BSA)). EF: ejection fraction; EDV: end-diastolic volume; ESV: end-systolic volume mm millimeters; m²: square meter, TR: tricuspid valve. For the cardiac MRI major and minor criteria values, see reference [4].

1/M

Normal with EF 55%

EDV 151 mL (79.5 mL/m²)*
ESV 83 mL (43.7 mL/m²)
RVEF 45%
Moderately to severely dilated with dyskinetic motion in the free wall and basal aneurysm
T1-weighted images show area of severe thinning on the free wall of right ventricle

2/M

Normal
EDV 153 mL
ESV 56 mL
EF 63%

EDV 178 mL (93.7 mL/m²)
ESV 121 mL (63.7 mL/m²)
EF 32%
Moderate dilatation with generalized hypokinesis.
There are localized RV areas of micro-aneurysms, mainly at the RVOT and the RV apex
Prominent papillary muscle and the trabeculation

3/M

EF > 55%

EDV 100 mL (50 mL/m²)
ESV 48 mL (24 mL/m²)
EF 52%
Thinned of the free wall with a small area of severe thinning, nears the RV apex with dyskinesis and diastolic bulging fulfilling the criterion of aneurysm

4/F

Normal systolic function

EDV 249 mL (207 mL/m²)
ESV 212 mL (176.7 mL/m²)
EF 15%
Stroke volume 36.2 mL
Severely dilated and hypertrabiculated
Very thinned out RV especially apical regions with evidence of microaneurysm and diastolic bulge and segmental dilatation of RV
The RVOT is dilated with hypokinesia. Evidence of at least mild-to-moderate TR regurgitation

5/F

EDV 83 mL
ESV 41 mL
EF 50%
Mild global hypokinesis and akinesis of the distal septum

EDV 120 mL (70.6 mL/m²)
RV ESV 83 mL (48.8 mL/m²)
EF 31%
Stroke volume 37 mL
Severely dilated with severe global hypokinesis and dyskinesis of the RV apex and severe thinning noted in both cine and dark blood imaging
Delayed myocardial enhancement demonstrates severe thinning of the free wall of the right ventricle

6/M

EDV 99 mL
ESV 39 mL
EF 60%
Stroke volume 59 mL

EDV 81 mL (50.6 mL/m²)
ESV 52 mL (32.4 mL/m²)
RVEF 36%
Mildly-to-moderately dilated with dyskinesia and akinesis of the free wall adjacent to the right ventricular apex
T1, T2 and delayed gadolinium enhancement demonstrate two focal aneurysms in the free wall of RV

7/M

Normal EF 58%

EDV 171 mL (90 mL/m²)
ESV 93 mL (49 mL/m²)
EF 49%
Stroke volume 76 mL( 40 mL/m²)
Focal dyskinesia in the RVOT
Small patent ductus arteriosus measuring about 5 mm. Main pulmonary artery (MPA) not enlarged measuring 26 mm

8/M

Normal EF 57%

EDV 87 mL (43.5 mL/m²)
ESV 47 mL (23.5 mL/m²)
EF 46%
Mildly dilated with mild global hypokinesis
Focal aneurysm noted in RV free wall or RVOT

9/M

Normal LV

Moderately dilated RV and reduced RV function (CMRI was performed in another cardiac center and full report no available)

10/M

EDV 110.5 mL (65 mL/m²)
ESV 49.3 mL (29 mL/m²)
EF 54%

EDV187.5 mL (105 mL/m²)
ESV 100 mL(59 mL/m²)
EF 44%

11/M

EF 45-50%

Quantification was not apparent at this point due to the poor ECG gating
Dilated at the RVOT, there is evidence of macroaneurysm with small microaneurysm noted at the RV apex with a large macroaneurysm noted at the RVOT and RV free wall with dyskinesis
Overall RV systolic function appeared to be mildly reduced

12/M

EDV 80 mL
ESV 25 mL
EF 68%

EDV 74 mL
ESV 26 mL
EF 64%
Late gadolinium enhancement in mid myocardium at the septum

**Table 5.** Comparison Between Patients With Appropriate and Inappropriate Implantable Cardioverter Defibrillator (ICD) Therapy
Patient characteristics	Appropriate therapy N (%), 11 (50%)	Inappropriate therapy N (%), 3 (13.6%)	P value
ACE: angiotensin-converting enzyme; ICD: implantable cardioverter defibrillator.
Male (%)	10 (90.9 %)	3 (100%)	0.814
Female (%)	1 (9.1 %)	0 (0.0%)	0.814
Clinical presentation
Palpitations	10 (90.9 %)	3 (100%)	0.462
Syncope	1 (9.1 %)	1 (33.3%)	0.552
Shortness of breath	2 (18.2%)	1 (33.3%)	0.727
Chest pain	2 (18.2%)	1 (33.3%)	0.295
Dizziness	1(9.1 %)	1(33.3%)	0.552
Ventricular tachycardia	8 (72.7 %)	1 (33.3%)	0.229
Sudden cardiac death	3(27.3%)	0 (0.0%)	0.176
Medications
Amiodarone	2 (18.2%)	0 (0.0%)	0.713
Sotalol	2 (18.2%)	0 (0.0%)	0.713
Beta blockers	4 (36.4%)	1 (33.3%)	0.870
ACE inhibitors	6 (54.5%)	2 (66.7%)	0.630
Spironolactone	6 (54.5%)	1 (33.3%)	0.416
ICD type
Single ICD	2 (18.2%)	0 (0.0%)	0.333
Dual ICD	9 (81.8%)	3 (100%)
Genetic results
Positive	6 (54.5%)	2 (66.7%)	0.323
Negative	1 (9.1 %)	1 (33.3%)	0.516
Not performed	4 (36.4%)	0 (0.0%)	0.308
Prevention type
Primary	1 (9.1 %)	1 (33.3%)	0.048
Secondary	10 (90.9%)	2 (66.7%)

Table 5. Comparison Between Patients With Appropriate and Inappropriate Implantable Cardioverter Defibrillator (ICD) Therapy

Patient characteristics

Appropriate therapy
N (%), 11 (50%)

Inappropriate therapy
N (%), 3 (13.6%)

P value

ACE: angiotensin-converting enzyme; ICD: implantable cardioverter defibrillator.

Male (%)

10 (90.9 %)

3 (100%)

0.814

Female (%)

1 (9.1 %)

0 (0.0%)

0.814

Clinical presentation

Palpitations

10 (90.9 %)

3 (100%)

0.462

Syncope

1 (9.1 %)

1 (33.3%)

0.552

Shortness of breath

2 (18.2%)

1 (33.3%)

0.727

Chest pain

2 (18.2%)

1 (33.3%)

0.295

Dizziness

1(9.1 %)

1(33.3%)

0.552

Ventricular tachycardia

8 (72.7 %)

1 (33.3%)

0.229

Sudden cardiac death

3(27.3%)

0 (0.0%)

0.176

Medications

Amiodarone

2 (18.2%)

0 (0.0%)

0.713

Sotalol

2 (18.2%)

0 (0.0%)

0.713

Beta blockers

4 (36.4%)

1 (33.3%)

0.870

ACE inhibitors

6 (54.5%)

2 (66.7%)

0.630

Spironolactone

6 (54.5%)

1 (33.3%)

0.416

ICD type

Single ICD

2 (18.2%)

0 (0.0%)

0.333

Dual ICD

9 (81.8%)

3 (100%)

Genetic results

Positive

6 (54.5%)

2 (66.7%)

0.323

Negative

1 (9.1 %)

1 (33.3%)

0.516

Not performed

4 (36.4%)

0 (0.0%)

0.308

Prevention type

Primary

1 (9.1 %)

1 (33.3%)

0.048

Secondary

10 (90.9%)

2 (66.7%)