Intra-cardiac distribution of late gadolinium enhancement in cardiac sarcoidosis and dilated cardiomyopathy

Table 1 Clinical cardiac findings in Diagnostic Standard and Guideline for Sarcoidosis-2015-Japanese Society of Sarcoidosis and Other Granulomatous Disorders

(1) More than two of five major findings are satisfied

(2) One of five major findings and more than two of three minor findings are satisfied

Major findings

Advanced atrioventricular block (including complete atrioventricular block) or sustained ventricular tachycardia

Basal thinning of the interventricular septum or morphological ventricular abnormality (ventricular aneurysm, wall thinning of other ventricular region, wall thickening)

Impaired left ventricular contraction (LVEF < 50%) or regionally abnormal wall motion

Abnormal cardiac uptake in gallium-67 citrate scintigraphy or fluorine-18 fluorodeoxyglucose PET

Late myocardial enhancement in gadolinium enhanced magnetic resonance imaging

Minor findings

Non-sustained ventricular tachycardia, multifocal or frequent premature ventricular contractions, bundle branch block, axis deviation, or abnormal Q wave in electrocardiography

Defect on myocardial perfusion scintigraphy

Endomyocardial biopsy: Interstitial fibrosis or monocyte infiltration over moderate grade

LVEF: Left ventricular ejection fraction; PET: Positron emission tomography.

Table 2 Clinical features and magnetic resonance imaging parameters in patients with cardiac sarcoidosis and with dilated cardiomyopathy

	CS	DCM	P values
Number	14	30
Sex (M/F)	M4/F10	M23/F7	0.001
Age (yr)	59.8 ± 13.5	69.2 ± 12.6	0.03
Syncope n (%)	2 (14.3)	6 (20.0)	0.65
Palpitation n (%)	7 (50.0)	17 (56.7)	0.74
NYHA (I/II/III/IV)	8/5/1/0 (57.1%/35.7%/ 7.1%/0%)	8/11/6/5 (26.7%/36.7%/ 20%/16.7%)	0.08
ECG findings
PQ duration	188.4 ± 26.0	188.1 ± 40.9	0.91
1st/2nd AVB	7/1 (50.0%/7.1%)	7/0 (23.3%/0%)	0.14
QRS duration	118.6 ± 22.9	128.4 ± 36.3	0.18
Abnormal Q waves n (%)	6 (42.9)	3 (10.0)	0.09
RBBB/LBBB	3/5 (21.4%/35.7%)	2/15 (6.7%/50%)	0.57
VTs n (%)	7 (50.0)	15 (50.0)	0.74
Medications n (%)
Corticosteroids	7 (50.0)	0 (0)	< 0.001
ACEI/ARB	9 (64.3)	20 (66.7)	0.73
β blockers	7 (50.0)	23 (76.7)	0.07
AADs	4 (28.6)	14 (46.7)	0.51
Diuretics	7 (50.0)	18 (60.0)	0.32
MRI
LVEDVI (mL/m2)	107.0 ± 45.8	135.5 ± 43.4	0.08
LVESVI (mL/m2)	74.2 ± 44.5	106.3 ± 42.1	0.04
LVMI (g/m2)	60.1 ± 24.9	67.1 ± 28.9	0.34
LVEF (%)	33.9 ± 11.0	22.8 ± 10.0	0.003
LE segment number	8.6 ± 4.6	5.3 ± 3.1	0.04

The categorical variables were expressed as number and percentage (%) and compared by χ² test. The continuous variables were expressed as means ± SD and examined by unpaired t test. CS: Cardiac sarcoidosis; DCM: Dilated cardiomyopathy; M/F: Male/female; NYHA: New York Heart Association; ARB: Angiotensin receptor blockers; ACEI: Angiotensin converting enzyme inhibitors; AVB: Atrioventricular block; AAD: Anti-arrhythmic drugs; MRI: Magnetic resonance imaging; LVEDVI and LVESVI: Left ventricular end-diastolic and end-systolic volume indices; LVEF: LV ejection fraction; LE: Late gadolinium enhancement; L/RBBB: Left/right bundle branch blocks; LVMI: LV mass index; VT: Ventricular tachycardia.

Table 3 Diagnostic value of characteristic late gadolinium enhancement distribution patterns to differentially diagnose cardiac sarcoidosis from dilated cardiomyopathy

LE patterns	Sensitivity (%)	Specificity (%)	PPV (%)	NPV (%)
Striated	85.7	3.3	29.3	33.3
Nodular	57.1	96.7	88.9	82.9
Circumferential	35.7	96.7	83.3	76.3
Subepi + subend	50.0	96.7	87.5	80.6

PPV and NPV: Positive and negative predictive values; Sub-epi + sub-end: Subepicardial and subendocardial distribution with spared midwall; LE: Late gadolinium enhancement.

Table 4 Reports for patterns of late gadolinium enhancement distribution and clinical relevance of late gadolinium enhancement in cardiac sarcoidosis

Ref.	Patients	LE distribution		Clinical relevance
		Intra-cardiac	Intra-mural
Smedema et al[8]	12 CS	Mostly basal and lateral LV wall	Any	Diagnostic
Matoh et al[13]	5 sCS	Mid ventricular septum	Midwall to subepicardial	Correlations between LE area and LVEDV, LVESV and LVEF
Ichinose et al[10]	10 CS	Any, but mostly basal LV wall	Any, but mainly subepicardial	Correlations between sum of LE score and BNP, LVEF, LVEDV
Manis et al[26]	11 CS	Ventricular septum	Patchy	Diagnostic
Patel et al[5]	21sCS	Any, but mainly basal ventricular septum, rarely RV wall	CAD; subendo-cardial non-CAD; mid wall, subepi-cardial, patchy	Higher rate of adverse events and cardiac death
Watanabe et al[27]	19 CS	NA	Subepicardial, transmural	Correlations between total LE segments, and reduced LV function and duration of extra-cardiac lesions
Greulich et al[28]	39 sCS	Any, but mainly ventricular septum (RV side)	Patchy, intramural to transmural	Higher Hazard ratio for MACE than other clinical parameters
Yang et al[29]	6 sCS	Ventricular septum, LV free wall, papillary muscle	Patchy	Decreased T2 (inactive phase)
Pöyjönen et al[30]	8 CS	Basal ventricular septum	Multifocal	Diagnostic
Tezuka et al[25]	9 sCS and 4 iCS	Any, but mainly anterior ventricular septum	Any, but mainly subepicardial	No difference between sCS and iCS in LE distribution and clinical features

BNP: Serum brain natriuretic peptide level; CAD: Coronary arterial disease type; CS: Cardiac sarcoidosis; iCS: Isolated CS; LV/RV: Left/right ventricles; LVEDV/ESV: LV end-diastolic/systolic volume; LVEF: LV ejection fractionl; MACE: Major adverse cardiac events; NA: Not available; sCS: Cardiac involvement of systemic sarcoidosis.