Rhythm disorders in isolated left ventricular noncompaction

Figures & data

Figure 1. Echocardiographic appearance of IVNC. End-diastolic still frame (close-up view of LV apex) of a 37-year-old woman showing the typical echocardiographic features of IVNC with a thin epicardial and a thickened endocardial layer with prominent deep recesses. Blood flow from the ventricular cavity into the recesses is visualized with color Doppler. Reprinted from Steffel et al. (12), Electrocardiographic characteristics at initial diagnosis in patients with isolated left ventricular noncompaction, American Journal of Cardiology 2009;104:984–89, Reprinted from American Journal of Cardiology, volume 104, Steffel J, Kobza R, Oechslin E, Jenni R, Duru F, Electrocardiographic characteristics at initial diagnosis in patients with isolated left ventricular noncompaction, pages 984–89, 2009 (12), Copyright (2009), with permission from Elsevier.

Table I. Electrocardiographic findings in patients with IVNC. Number of patients and (%) are shown.

	Shoki et al., 2010 (14)	Steffel et al., 2009 (12)	Stanton et al., 2009 (13)	Aras et al., 2006 (16)	Lofiego et al., 2007 (15)	Murphy et al., 2005 (6)	Oechslin et al., 2000 (4)
Number of patients	187	74	27	67	65	45	34
Year of diagnosis	2000–2007	1995–2008	2001–2006	2001–2005	1991–2004	1992–2002	1984–1998
Rhythm
Normal ECG	46 (24.6)	10 (13)	2 (7.4)	10 (15)	8 (12)	4 (9)	2 (6)
Sinus rhythm		74 (95)
Atrial fibrillation/flutter	7 (3.7)	3 (4)	2 (7.4)	8 (12)		3 (7)	9 (26)
Heart rate (bpm), mean (±SD)		76 (±18)
Atrial activation
PR interval (ms), mean (±SD)		167 (±32)
P mitrale		20 (26)
P pulmonale		12 (15)
AV block first degree		12 (15)		2 (3)
AV block third degree	6 (3.2)	2 (3)		1 (1)
WPW pattern	5 (2.7)	2 (3)		0 (0)			0 (0)
Premature atrial contractions	6 (3.2)	4 (5.1)
Ventricular depolarization
QRS duration (ms), mean (±SD)		109 (±31)	114 (±28.5)
Right bundle branch block	24 (12.8)	2 (3)	2 (7.4)	3 (4)			4 (12)
Left bundle branch block	4 (2.1)	15 (19)	6 (22.2)	14 (21)	21 (32)	13 (29)	15 (44)
Intraventricular conduction disorder	5 (2.7)	7 (9)		7 (11)
Incomplete right bundle branch block		2 (3)
Left anterior fascicular block		3 (4)
Left posterior fascicular block		0 (0)
Left ventricular hypertrophy	14 (7.4)	30 (38)		17 (25)
Right ventricular hypertrophy		5 (6)
Premature ventricular contractions	15 (8.0)	9 (11.5)
Brugada-like pattern	6 (3.2)
Ventricular repolarization
Pathologic repolarization		15 (19.2)					12 (35)
ST elevation	33 (17.6)	8 (10)
ST depression	6 (3.2)	40 (51)				4 (9)
T wave abnormalities	27 (14.4)	32 (41)				7 (16)
QTc duration (ms), mean (±SD)		455 (±52)	449 (±52.4)
QTc prolongation		40 (52)
Specials	Japanese adults	Swiss adults		Turkish adults		British patients, age 13–83
	Jenni criteria	Jenni criteria		Jenni criteria		Chin or Jenni criteria

Table II. Frequency of ventricular arrhythmias in IVNC.

	Kobza et al., 2010 (32)	Aras et al., 2006 (16)	Lofiego et al., 2007 (15)	Murphy et al., 2005 (6)	Oechslin et al., 2000 (4)
Number of patients	30	67	65	45	34
Year of diagnosis	1997–2008	2001–2005	1991–2004	1992–2002	1984–1998
Ventricular arrhythmias	11 (37%)
Non-sustained VT		13/42 (31%)		9 (20%)	11 (32%)
Sustained VT		1	4 (6%)		3 (9%)
Sudden death			3 (5%)
Method of assessment	ICD memory	Holter, ECG		Holter
Follow-up (months)	40 ± 34	30	46 ± 44	46 (6–179)	44 ± 40

Table III. EP findings in 24 patients with IVNC. Data from Steffel et al., Europace 2009 (10).

Spontaneous rhythm at time of EPS, n (%):
Sinus rhythm	22 (92)
with pre-excitation	2 (8)
Atrial fibrillation, third-degree AV block, junctional escape rhythm	1 (4)
Junctional escape rhythm, third-degree AV block, VAT paced	1 (4)
AV conduction, n (%):
Normal AV node physiology	19 (79)
Third-degree AV block	2 (8)
Dual AV node physiology	1 (4)
Prolonged AV conduction	1 (4)
Supranormal AV conduction	1 (4)
Inducible ventricular tachycardia (VT):	9 (38)
Non-sustained VT	5 (21)
Sustained polymorphic VT / VF	2 (8)
Sustained monomorphic VT	2 (8)
Inducible supraventricular tachycardia:	7 (29)
AVRT (orthodromic)	2 (8)
Atrial fibrillation	2 (8)
Atrial flutter	1 (4)
AVNRT	1 (4)
Non-specific atrial tachycardia	1 (4)
Interventions:
Accessory pathway ablation (WPW)	2 (8%)
Isthmus ablation (atrial flutter)	1 (4%)
Slow-pathway modification (AVNRT)	1 (4%)

AVNRT = AV node reentry tachycardia; AVRT = atrioventricular reentry tachycardia; VT = ventricular tachycardia; VF = ventricular fibrillation; EPS = electrophysiological study; WPW = Wolf Parkinson White; AV = atrioventricular.

Figure 2. Proposed algorithm for arrhythmic risk stratification in IVNC.

EF = ejection fraction; VT = ventricular tachycardia; VF = ventricular fibrillation; SCD = sudden cardiac death; EDVI = end-diastolic volume index; ICD = implantable cardioverter defibrillator; EP = electrophysiological.

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