Left Ventricular Noncompaction in Adults: Imaging and Clinical Findings in 63 Patients

Se Jin Cho; Dong Hyun Yang; Joon-Won Kang; Tae-Hwan Lim

doi:10.3348/jksr.2018.78.3.170

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Cho, Yang, Kang, and Lim: Left Ventricular Noncompaction in Adults: Imaging and Clinical Findings in 63 Patients

Original Article

J Korean Soc Radiol 2018;78(3):170-178.

Published online: 20 January 2018

DOI: https://doi.org/10.3348/jksr.2018.78.3.170

Left Ventricular Noncompaction in Adults: Imaging and Clinical Findings in 63 Patients

Se Jin Cho, MD, Dong Hyun Yang, MD^∗, Joon-Won Kang, MD, Tae-Hwan Lim, MD

Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea

^∗Corresponding author: Dong Hyun Yang, MD Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea. Tel. 82-2-3010-5820 Fax. 82-2-2045-4127 E-mail: donghyun.yang@gmail.com

Received 11 April 2017 Revised 10 August 2017 Accepted 1 October 2017

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

To describe imaging and clinical findings for a left ventricular noncompaction (LVNC) in the adult.

Materials and Methods

From 2000 to 2014, 63 patients were diagnosed with LVNC by echocardiography, computed tomography, and magnetic resonance imaging at our hospital. Baseline characteristics, clinical manifestations, combined cardiac or systemic anomalies, and imaging findings were reviewed. We made a comparison between the isolated and combined disease groups.

Results

Among 63 patients with LVNC, 32 (51%) patients did not have combined cardiac anomalies (isolated disease group). The mean age at the initial diagnosis was higher in the isolated than in the combined disease group (54.2 years vs. 40.2 years, p < 0.001). The combined disease group presented symptoms more frequently at initial diagnosis than the isolated disease group (94% vs. 75%, p = 0.082). Heart failure symptoms were the most common adverse events (60.3% in all patients). Thromboembolic events developed in 20 patients, and were more frequent in the combined disease group than in the isolated disease group (39% vs. 26%, p = 0.279). The most common cardiac abnormality was dilated cardiomyopathy (n = 15, 24%). There was no significant difference in the mean noncompacted/compacted ratios between both of the disease groups.

Conclusion

Isolated and combined LVNC disease groups showed differences in age at diagnosis and clinical manifestations. The clinical and imaging findings may be helpful to better understand LVNC.

Keywords: Index terms Cardiomyopathies Magnetic Resonance Multidetector Computed Tomography Echocardiography

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Fig. 1.

63 years old male with typical image findings of left ventricular noncompaction. Long and short axis view on echocardiography at end systolic phase of a 63 years old male hospitalized for dyspnea (A, B). Note that extensive trabeculations, two inner compacted (A, marking with bidirectional arrow end) and outer noncompacted layer (A, marking with bidirectional circle end) with different echogenicity, increased the non-compacted layer thickness over the compacted layer thickness ratio, and visible low echogenic inter-trabecular recess (B, arrow). The CT (C) and MRI (D) images at end diastolic phase of a 34 years old female with prominent inner noncompacted layer, especially in mid to apical wall of left ventricle.

Fig. 2.

31 years old female with LVNC: correlation between the autopsy specimen and image findings. The gross morphology of autopsy specimen (A), TTE finding (B), and MRI findings (C, D) of a 31years old female LVNC patient who underwent heart transplantation. There was delayed myocardial enhancement at mid anterior, mid septal, and mid inferior wall on MRI examination (D). LVNC = left ventricular noncompaction

Table 1.

Baseline Characteristics of Left Ventricular Noncompaction Patients

	Total (n = 63)	Isolated (n = 32)	Combined (n = 31)	p-Value
Age	47.4 ± 16.0	54.3 ± 12.2	40.3 ± 16.6	0
Sex
Male, n (%)	43 (68)	24 (75)	19 (61)	0.243
Female	20 (32)	8 (25)	12 (39)	0.243
Chief complaint at diagnosis
CHF symptom	38 (60)	15 (47)	23 (74)	0.027
Incidental	10 (16)	8 (25)	2 (7)	0.082
Chest pain	8 (13)	8 (25)	0 (0)	0.005
Known heart disease	2 (3)	0 (0)	2 (7)	0.238
Systolic BP	117.7 ± 20.0	122.4 ± 21.0	112.8 ± 18.0	0.055
Diastolic BP	72.6 ± 12.1	75.7 ± 14.2	69.4 ± 8.5	0.037
Hypertension	14 (22)	11 (36)	3 (11)	0.031
Heart rate	77.7 ± 19.4	74.4 ± 19.2	81.2 ± 19.2	0.170
Chest pain	35 (56)	16 (52)	19 (69)	0.440
Shock	17 (27)	3 (10)	10 (32)	0.059
DM	6 (10)	6 (24)	0 (0)	0.024
Smoke	16 (25)	12 (40)	5 (17)	0.084
Combined NMD	3 (5)	2 (6)	1 (3)
Thromboembolic event	20 (32)	8 (26)	12 (39)	0.279
Significant CAD	11 (18)	8 (26)	3 (11)	0.182
Ischemic heart disease	8 (13)	7 (23)	1 (3)	0.053
Cardiac intervention or OP	15 (24)	1 (3)	14 (45)
Implantation for HR control	11 (18)	5 (16)	6 (19)	1
Heart transplantation	4 (6)	1 (3)	3 (11)	0.355
ECG
LBBB	13 (21)	9 (28)	4 (13)	0.222
Normal sinus rhythm	11 (18)	8 (25)	3 (11)	0.062
Atrial fibrillation	10 (16)	2 (6)	8 (26)	0.082
Paced rhythm	5 (8)	3 (9)	2 (7)	0.668
RBBB	4 (6)	1 (3)	3 (11)	0.355
Ventricular arrhythmia	4 (6)	2 (6)	2 (7)	0.238
Sinus tachycardia	3 (5)	0 (0)	3 (11)	0.113
WPW syndrome	1 (2)	0 (0)	1 (3)	0.492
Others	12 (19)	7 (23)	5 (17)
Cardiac function
LVEF < 50%	44 (72)	23 (71)	21 (72)
Initial LVEF, %	36.9 ± 17.5	37.8 ± 16.4	35.7 ± 18.8	0.642
Initial LV ESV index, mL/m²	74.5 ± 49.8	68.1 ± 42.7	81.5 ± 56.6	0.299
Initial LV EDV index, mL/m²	110.1 ± 53.5	104.8 ± 47.5	115.9 ± 59.7	0.426
Initial LN mass index	188.1 ± 277.8	148.0 ± 50.5	234.4 ± 403.4	0.250

Table 1.

Baseline Characteristics of Left Ventricular Noncompaction Patients (continued)

	Total (n = 63)	Isolated (n = 32)	Combined (n = 31)	p-Value
Wall motion
Normal	12 (19)	8 (26)	4 (15)	0.672
Hypokinetic	40 (64)	20 (65)	20 (74)	0.721
Akinetic	5 (8)	3 (10)	2 (27)	1
Dyskinetic	0 (0)	0 (0)	0 (0)
Paradoxic	1 (2)	0 (0)	1 (4)	0.344
Death	4 (6)	4 (13)	0 (0)
Combined heart abnormality			31 (49)
Dilated cardiomyopathy			15 (24)
Kawasaki disease			2 (3)
Hypertrophic cardiomyopathy			1 (2)
ARVD			1 (2)
Congenital heart disease			12 (19)
Image modality
Only TTE	34 (54)	22 (69)	12 (39)
Only CT	6 (10)	1 (3)	5 (16)
Only MR	3 (10)	3 (9)	0 (0)
TTE and CT	4 (13)	1 (3)	3 (5)
TTE and MRI	8 (26)	4 (13)	4 (6)
CT and MRI	3 (10)	3 (9)	0 (0)
ALL	5 (16)	3 (9)	2 (3)

Continuous variables are presented as mean ± standard deviation. Categorical variables are expressed number (percentage). ARVD = arrhythmogenic right ventricular dysplasia, BP = blood pressure, CAD = coronary artery disease, CHF = congestive heart failure, DM = diabetes mellitus, ECG = echocardiography, EDV = end diastolic volume, EF = ejection fraction, ESV = end systolic volume, HR = heart rate, LBBB = left bundle branch block, LV = left ventricle, LVEF = left ventricular ejection fraction, NMD = neuromuscular disease, OP = operation, RBBB = right bundle branch block, TTE = transthoracic echocardiography, WPW syndrome = Wolff-Parkinson-White syndrome

Table 2.

NC/C Ratio on Each Three Image Modality

	Compacted Layer	Noncompacted Layer	NC/C Ratio
TTE
Total (n = 51)	5.9 ± 1.2	16.4 ± 4.2	2.8 ± 0.6
Isolated (n = 31)	6.0 ± 1.2	16.7 ± 4.3	2.8 ± 1.7
Combined (n = 20)	5.8 ± 1.2	15.9 ± 4.1	2.8 ± 0.3
CT
Total (n = 17)	6.4 ± 2.1	16.6 ± 3.5	2.8 ± 0.7
Isolated (n = 6)	8 ± 1.9	16.4 ± 3.3	2.2 ± 0.4
Combined (n = 12)	5.6 ± 1.6	17.2 ± 4.3	3 ± 0.7
MRI
Total (n = 18)	5.9 ± 1.6	16.2 ± 3.3	2.9 ± 0.8
Isolated (n = 6)	7 ± 1.4	16.5 ± 3.6	2.6 ± 0.6
Combined (n = 13)	5.3 ± 1.4	16 ± 3.3	3.1 ± 0.8

Parameters are shown in millimeters, presented as mean ± standard deviation. NC/C ratio = the ratio of noncompacted layer thickness over the compacted layer thickness, TTE = transthoracic echocardiography

Table 3.

Inter-Modality Difference of NC/C between Three Different Image Modality in All Groups

	TTE vs. CT (n = 9)			TTE vs. MRI (n = 13)			CT vs. MRI (n = 8)
	TTE	CT	p-Value	TTE	MRI	p-Value	CT	MRI	p-Value
Compacted layer	5.6 ± 1.2	6.9 ± 2.3	0.147	5.6 ± 1.3	6.1 ± 1.7	0.370	6.5 ± 2.5	5.6 ± 1.8	0.433
Noncompacted layer	14.3 ± 3.0	17.7 ± 4.5	0.083	15.8 ± 4.6	16.3 ± 2.9	0.440	16.8 ± 3.5	15 ± 2.2	0.247
NC/C ratio	2.6 ± 0.4	2.7 ± 0.9	0.737	2.7 ± 0.3	2.9 ± 0.7	0.592	2.8 ± 0.9	2.8 ± 1.9	0.980

Parameters are shown in millimeters, presented as mean ± standard deviation. For determining the p-value, Independent t-test was used. NC/C ratio = the ratio of noncompacted layer thickness over the compacted layer thickness, TTE = transthoracic echocardiography

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