Monomorphic Ventricular Tachycardia and Mediastinal ...Monomorphic Ventricular Tachycardia and...
Transcript of Monomorphic Ventricular Tachycardia and Mediastinal ...Monomorphic Ventricular Tachycardia and...
Journal of the American College of Cardiology Vol. 58, No. 1, 2011© 2011 by the American College of Cardiology Foundation ISSN 0735-1097/$36.00
Monomorphic Ventricular Tachycardia andMediastinal Adenopathy Due to Granulomatous Infiltrationin Patients With Preserved Ventricular Function
Ajit Thachil, MD, DM,* Johann Christopher, MD, DNB,* B. K. S. Sastry, MD, DM,*Kavitha Nallapa Reddy, DNB, DRM,† Vijaya K. Tourani, MD,* Ashfaq Hassan, MD,*Bhupathiraju Soma Raju, MD, DM,* Calambur Narasimhan, MD, DM*
Hyderabad, India
Objectives This report characterizes a syndrome of granulomatous infiltration presenting as sustained monomorphic ven-tricular tachycardia (SMVT) with mediastinal adenopathy in patients with preserved ventricular function.
Background Unlike truly idiopathic ventricular tachycardia, SMVT due to granulomatous infiltration responds poorly to radio-frequency ablation and has a poor prognosis.
Methods Patients without obstructive coronary artery disease and with normal ventricular function presenting with SMVTother than posterior fascicular morphology were evaluated. Computed chest tomograms, cardiac magneticresonance imaging, and 18-fluorodeoxyglucose positron emission tomographic scans (18FDG PET-CT) wereperformed. Significant lymph nodes were evaluated for tuberculosis and sarcoidosis. Initial treatment includedantiarrhythmic drugs � radiofrequency ablation. Additionally, patients with evidence of tuberculosis receivedanti-tuberculosis therapy; the rest were treated as sarcoidosis.
Results Mediastinal adenopathy with mid-myocardial scar and/or focal myocardial inflammation was observed in 14patients; lymph nodes revealed noncaseating granulomas in all. Evidence of tuberculosis was present in 79%.During follow-up (median duration 25 months), SMVT recurred despite initial treatment in 92%. Addition ofdisease-specific therapy abolished further recurrences in 64% of them. Decrease in SMVT correlated with resolu-tion of myocardial inflammation on serial 18FDG PET-CTs. Appropriate therapies occurred in 67% of patients re-ceiving implantable cardioverter-defibrillators.
Conclusions A subset of patients with SMVT with preserved ventricular function has a syndrome of arrhythmogenic myocardi-tis with granulomatous mediastinal adenopathy due to myocardial tuberculosis or cardiac sarcoidosis. This entityis optimally managed with a combination of disease-specific therapy and antiarrhythmic measures. (J Am CollCardiol 2011;58:48–55) © 2011 by the American College of Cardiology Foundation
Published by Elsevier Inc. doi:10.1016/j.jacc.2011.02.044
thot
Sustained monomorphic ventricular tachycardia (SMVT)with preserved ejection fraction (EF) typically has a benigncourse and responds well to antiarrhythmic drugs (AADs)and radiofrequency ablation (RFA) (1). We describe theclinical features, diagnostic and therapeutic strategies, andfollow-up results of a cohort of patients who presented withSMVT with preserved EF who were found to have tuber-culosis or sarcoidosis on evaluation; this patient subsetresponded poorly to AADs and RFA.
From the *Care Hospital, Banjara Hills, Hyderabad, India; and the †ApolloGleneagles PET-CT Center, Jubilee Hills, Hyderabad, India. The authors havereported that they have no relationships to disclose.
oManuscript received August 31, 2010; revised manuscript received January 31,
2011, accepted February 15, 2011.
Methods
Patient population. Between January 2008 and June 2009,of the 29 patients referred to our center with SMVT and thefeatures listed in Table 1, 19 consented to further evalua-tion. Sustained monomorphic ventricular tachycardia withgranulomatous mediastinal lymphadenopathy was diag-nosed in 11 of them (58%). This report describes the courseof these 11 patients and an additional 3 patients diagnosedbetween May 2006 and December 2007.Diagnostic evaluation. Figure 1 summarizes the diagnos-ic and therapeutic strategy adopted in patients suspected ofaving SMVT due to granulomatous infiltration. The sitef the index ventricular tachycardia (VT) was localized onhe basis of 12-lead electrocardiography as interpreted by 2
bservers. Three or more episodes of sustained VT/day or aua
MpmsEssrt
(atpaemGgfo2ad(fi
tsa
8tsFadIlfptSomiawmTcii
49JACC Vol. 58, No. 1, 2011 Thachil et al.June 28, 2011:48–55 VT in Granulomatous Disease
single episode lasting more than 1 h despite treatment wasdefined as a VT storm (2).
IMAGING. All patients underwent contrast-enhanced 64-slice computed tomograms (Siemens, Erlangen, Germany).Delayed gadolinium enhancement-cardiac magnetic reso-nance imaging (DE-CMR) was done with a 1.5-T scanner(Siemens) in the 11 patients without prior implanteddevices. “Late” enhancement denoting myocardial scar wassought as previously described (3). A resting positronemission tomographic study after administration of 18-fluorodeoxyglucose in conjunction with a computed tomo-gram (18FDG PET-CT) and a 13N-ammonia cardiac per-fusion scan was performed on 12 patients, including the 3who did not undergo a DE-CMR, with a 16-slice scanner(Siemens) as per a standard protocol (4). High 18FDGptake was interpreted as inflammation; matched perfusionnd metabolism defects were interpreted as scar.
ETIOLOGICAL EVALUATION. The tuberculin skin test for.tuberculosis was performed with 5 tuberculin units of
urified protein derivative. Horizontal induration of �10m at 48 h was considered positive. Lymph nodes were
ampled in all, as per the described protocol (Fig. 1).ndomyocardial biopsy was performed in 4. All tissue
amples were subjected to histopathology, Gram’s stain, andtaining and culture for mycobacteria and fungi. A deoxy-ibonucleic acid polymerase chain reaction (PCR) detectinghe IS6110 sequence of M.tuberculosis was performed on 9
of the tissue samples.Treatment. INITIAL THERAPY. All patients received AADsbeta-blockers and amiodarone) as initial therapy, withdditional intravenous lidocaine/oral Mexiletine being usedo control breakthrough episodes of arrhythmia. RFA waserformed in 5 as part of initial therapy, predominantlymong the earlier patients. Mapping was performed with anlectroanatomic system (CARTO, Biosense Webster, Dia-ond Bar, California); a Stockert RF generator (StockertmBh, Freiburg, Germany) and an 8-F 3.5-mm tip irri-
ated catheter (NAVISTAR, Biosense Webster) were usedor ablation. Successful RFA was defined as noninducibilityf SMVT on ventricular stimulation from 2 sites includingextrastimuli; stimulation was performed without drugs
nd during continuous infusion of isoprenaline to a maximumose of 6 �g/min. Implantable cardioverter-defibrillatorsICDs) were recommended in all; however, owing tonancial constraints, only 9 received ICDs.
DISEASE-SPECIFIC THERAPY. Disease-specific therapy for tu-berculosis or sarcoidosis was added to initial therapy in allpatients as per the protocol in Figure 1. Individualized titrationof disease-specific therapy was guided by clinical response andserial 18FDG PET-CTs. Treatment duration ranged from 12o 30 months. A 3-month period was allowed for disease-pecific therapy to take effect, after which its effect was
nalyzed. Specific therapy was delayed due to late diagnosis inof the initial patients; this helpedo assess the impact of disease-pecific therapy.ollow-up. Patients were evalu-ted at 3 monthly intervals anduring symptomatic recurrence.nterrogation of ICD and 12-ead electrocardiograms were per-ormed at each evaluation. Oneatient was lost to follow-up af-er 1 month.tatistical methods. Continu-us variables were reported asean � SD or medians (with
nterquartile range), as appropri-te. Significance of differencesas estimated with a Wilcoxonatched-pairs signed-rank test.he Pearson’s test was used to
orrelate post-treatment changen VT frequency with resolution ofnflammation on 18FDG PET-
CT. The patient who was lost tofollow-up was excluded from alloutcome analyses.
Results
Baseline characteristics. Baseline and follow-up featuresare summarized in Table 2. The mean age at presentationwas 44 � 11 years; mean left ventricular EF was 58 � 6%.None of the patients had symptoms of extracardiac disease.No patient had atrioventricular conduction block. Medianfollow-up was 25 months (interquartile range: 15 to 58months).VT morphology. All of the patients presented withSMVT. Morphologies of the index and subsequent VTs didnot conform to any particular pattern (Table 2). Themajority of the recurrent VTs (78%, or 7 of 9 recurrenceswhere 12-lead electrocardiograms were available) had adifferent morphology, as compared with the index VT.VT recurrence. Sustained monomorphic ventricular tachy-cardia recurred at a median follow-up of 4 months (range0.6 to 9 months) in 12 of 13 patients. Eleven of thesepatients had recurrence of VT before effective disease-
Features of Patients Evaluated for Monomorphic VTWith Mediastinal Adenopathy Due to GranulomatousInfiltrationTable 1
Features of Patients Evaluated for Monomorphic VTWith Mediastinal Adenopathy Due toGranulomatous Infiltration
Normal biventricular systolic function (ejection fraction �50%)
Absence of obstructive coronary artery disease
Absence of known ion channelopathies causing VT
Absence of transient, reversible causes of VT like electrolyte abnormalities
�1 episode of sustained monomorphic VT of any morphology other than typicalleft ventricular posteroseptal fascicular morphology, defined as VT of rightbundle branch block morphology, superior axis, and QRS duration �140 ms
Abbreviationsand Acronyms
18FDG PET-CT � 18-fluorodeoxyglucose positronemission tomography withcomputed tomogram
AAD � antiarrhythmic drug
ATT � anti-tuberculosistherapy
CS � cardiac sarcoidosis
DE-CMR � delayedgadolinium-enhancementcardiac magneticresonance imaging
EF � ejection fraction
ICD � implantablecardioverter-defibrillator
PCR � polymerase chainreaction
RFA � radiofrequencyablation
SMVT � sustainedmonomorphic ventriculartachycardia
VT � ventriculartachycardia
VT � ventricular tachycardia.
50 Thachil et al. JACC Vol. 58, No. 1, 2011VT in Granulomatous Disease June 28, 2011:48–55
Figure 1 Diagnostic and Therapeutic Strategy in Patients Suspected of Having SMVT Due to Granulomatous Disease
AFB � acid fast bacilli; CAD � coronary artery disease; DE-CMR � delayed gadolinium enhancement-cardiac magnetic resonance imaging; DNA-PCR � DNA-based poly-merase chain reaction; 18FDG PET-CT � 18fluorodeoxyglucose positron emission tomography with computed tomogram; FNAC � fine needle aspiration cytology; HRCT �
contrast-enhanced high resolution computed tomogram of the chest; ICD � implantable cardioverter-defibrillator; LV � left ventricular; MMVT � monomorphic ventriculartachycardia; PCR � polymerase chain reaction; RFA � radiofrequency ablation; SMVT � sustained monomorphic ventricular tachycardia; USG � ultrasonography;VATS � video-assisted thoracoscopic surgery; VT � ventricular tachycardia.
duspmEwiaasHoc(nc
Ttppw4nEmpomc(Rt
51JACC Vol. 58, No. 1, 2011 Thachil et al.June 28, 2011:48–55 VT in Granulomatous Disease
specific therapy. Overall, there were 117 recurrences during58.2 patient years of follow-up, including 18 VT storms.Myocardial scarring and inflammation. Myocardial scar-ring or focal myocardial hypermetabolism was present in all(Fig. 2). DE-CMR revealed mid-myocardial scars in 9 of 11patients (82%) (Fig. 3A); overall, when defined by eitherDE-CMR or single-photon emission computed tomography–PET, scar was present in 10 (71%). 18FDG PET-CT revealedmyocardial inflammation in 11 of 12 (92%) (Fig. 3B). The onlypatient without myocardial uptake on 18FDG PET-CT had aiffuse matched perfusion-metabolism defect; etiological eval-ation was delayed by several years in this patient. Myocardialcar in the absence of inflammation was the cause of VT in thisatient. In contrast, 2 patients with normal DE-CMRs hadyocardial inflammation in the absence of scar.xtracardiac involvement. 18FDG avid mediastinal nodesere present in all, including the patient without myocardial
nflammation. Six patients had additional extramediastinaldenopathy (Table 2). Computed tomography scan showedsymptomatic pulmonary parenchymal abnormalities con-istent with sarcoidosis in 2 patients.
istopathologic analysis. Multiple noncaseating epitheli-id granulomas consistent with either sarcoidosis or tuber-ulosis were present in 13 of the 14 lymph node samples1 sample was inadequate). Endomyocardial biopsy wasormal in 3 patients and showed nongranulomatous, non-
Figure 2 Sites of Myocardial Scar and/or Inflammation Among
Scar was defined as delayed gadolinium enhancement on cardiac magnetic resonaemission tomographic computed tomography scan (PET) metabolism defect. Increascar and/or focal myocardial inflammation. *Matched metabolism - perfusion defeventricle; LV-AW � anterior wall of the left ventricle; LV-IW � inferior wall of the leventricle; RV � right ventricle; RVOT � right ventricular outflow tract; sl. no. � ser
aseating myocardial necrosis in the fourth.
uberculosis as an underlying etiology. Skin test foruberculosis was positive in 11 of 14 patients (79%). Threeatients showed an exuberant, necrotic local reaction to theurified tubercular protein derivative. Lymph node biopsyas positive for M.tuberculosis deoxyribonucleic acid PCR inof 9 patients. M.tuberculosis was cultured from the lymph
ode in 2 patients (Table 2).lectrophysiological study and RFA. Sustained mono-orphic ventricular tachycardia was inducible in 4 of 5
atients. Two morphologies of SMVT were inducible in 2f them. Induced VTs were consistent with a re-entrantechanism in 2 and focal mechanism in 2; the mechanism
ould not be determined in 2 of the VTs. Low voltage areasbipolar voltage �1.5 mV) on substrate map and sites ofFA corresponded to the sites of scar in DE-CMR or to
he site of inflammation in 18FDG PET-CT (Table 3).Response to treatment. RFA was successful in 3 of the 5patients who received it as part of initial therapy. Beforeeffective disease-specific therapy, VT recurred in 100% of thepatients who received AADs with RFA as initial therapy andin 75% of patients who received AADs only. Among the 11patients who experienced recurrence while receiving initialtherapy, disease-specific therapy controlled the VT in 9 (82%).This included 7 patients (64%) in whom further VT wasabolished and 2 others (18%) in whom VT frequency de-creased. Overall, disease-specific therapy reduced the VT
nts With Granulomatous Myocarditis
aging (MRI) or matched 13N-ammonia perfusion/18fluorodeoxyglucose positron8FDG uptake on PET-CT indicated inflammation. All patients had mid-myocardialgestive of scar was present on PET. IVS � interventricular septum; LV � leftricle; LV-LW � lateral wall of the left ventricle; LV-PW � posterior wall of the left
ber.
Patie
nce imsed 1
ct sugft vential num
frequency from 6.5 VTs/patient-year to 0.6 VTs/patient-year
rtIda
D
TlR
ptDcbe
52 Thachil et al. JACC Vol. 58, No. 1, 2011VT in Granulomatous Disease June 28, 2011:48–55
(p � 0.016) (Fig. 4); 16 VT storms occurred before effectivedisease-specific therapy, compared with 2 after. Reduction inVT burden correlated with resolution of myocardial inflam-mation on post-treatment 18FDG PET-CTs (r � 0.87). Thisesponse was observed among all disease-specific therapy pro-ocols (Fig. 5). Appropriate ICD therapies occurred in 6 of 9CD recipients (67%). One patient who was diagnosed lateeveloped biventricular dysfunction before etiological diagnosisnd succumbed to progressive heart failure.
iscussion
his report describes a cohort of patients wherein a granu-omatous disease presented as SMVT with preserved EF.
Figure 3 Demonstrations of Myocardial Scar and Inflammation
(A) Demonstration of myocardial scar. Delayed gadolinium enhanced cardiac magncharacteristic mid-myocardial scar (arrows) in the interventricular septum. Owing t(B) Demonstration of myocardial inflammation. The 18fluorodeoxyglucose (FDG) poA showing myocardial hypermetabolism in the anterior and lateral walls, basal sep
ecurrent VT of morphology different from the index
resentation should arouse suspicion of an underlying infil-rative disorder among such patients.
iagnosis of cardiac sarcoidosis. Some of our patientsannot be diagnosed to have cardiac sarcoidosis (CS) on theasis of the Japanese Ministry of Health criteria (5,6). How-ver, patchy mid-myocardial scar, focal intense myocardial
18FDG uptake, noncaseating granulomas in lymph nodes, andthe observed treatment response are all consistent with CS(4,6). We recommend a protocol of routine contrast-enhancedchest computed tomography scans followed by sampling of theinvolved nodes as the preferred strategy for histological diag-nosis. Over-reliance on endomyocardial biopsies might have
esonance imaging (from patient number 7 in Table 2 and Figure 2) showing theatchy nature, the scar does not enhance as intensely as an ischemic scar.emission tomographic computed tomography scan of the same patient as innd a mediastinal lymph node.
etic ro its psitrontum, a
underdiagnosed this entity in the past (7).
ccScitftpcoaTwraAAVmISse
line
and
Follo
w-U
pFe
atur
esof
Pat
ient
sW
ith
SM
VT
Due
toSar
coid
osis
/Tu
berc
ulos
isab
le2
Bas
elin
ean
dFo
llow
-Up
Feat
ures
ofP
atie
nts
Wit
hSM
VT
Due
toSar
coid
osis
/Tu
berc
ulos
is
atie
nter
ial#
Age
(yrs
)Sex
LVEF
(%)
Tota
lFo
llow
-Up
(mon
ths)
Inde
xVT
Mor
phol
ogy
M/C
/S/A
/A
bd
Tube
rcul
inSki
nTe
stM
.tb
PC
RA
FBC
ultu
reD
iagn
osis
Dis
ease
-Spe
cific
Trea
tmen
tA
ntia
rrhy
thm
icTr
eatm
ent
Sit
esof
Ade
nopa
thy
Lym
phN
odes
Wit
hH
igh
18FD
GU
ptak
eB
iops
ied
Nod
e
15
7F
62
79
LB,R
IM
MM
NN
AN
CS
PD
N/M
txA
AD
�R
FA2
46
F6
01
84
LB,R
SM
,SM
,SS
PN
AN
CS/T
BA
TT�
PD
NA
AD
�IC
D3
21
F5
01
5R
B,R
SM
,C,A
MM
PN
NCS/T
BA
TT�
PD
N/M
txA
AD
46
2M
52
58
LB,L
SM
MM
NN
NCS
PD
N/M
txA
AD
�IC
D5
42
M6
02
1LB
,LI
MM
MP
NA
NCS/T
BA
TT�
PD
N/M
txA
AD
�IC
D6
32
F6
31
8R
B,R
IM
,S,A
,Abd
M,S
,Abd
SP
NP
TBA
TTA
AD
75
4M
60
12
LB,R
IM
,SM
,SS
PP
NCS/T
BA
TT�
PD
NA
AD
�R
FA�
ICD
83
5M
51
37
LB,R
IM
NA
MP
NA
NCS/T
BA
TT�
PD
N/M
txA
AD
�R
FA�
ICD
94
8M
55
16
3R
B,L
SM
MM
PN
AN
CS/T
BA
TT�
PD
NA
AD
�IC
D1
04
6M
50
11
RB
,RI
M,A
M,A
AP
PP
TBA
TTA
AD
�R
FA�
ICD
11
41
M7
32
9LB
,LI
MM
MN
NN
CS
PD
N/M
txA
AD
12
40
F6
15
7R
B,R
IM
MM
PP
NCS/T
BA
TT�
PD
N/M
txA
AD
�R
FA�
ICD
13
55
M5
81
NA
M,C
,SN
AM
PN
NCS/T
BA
TT�
PD
N/M
txA
AD
14
38
M6
01
5LB
,LI
MM
MP
PN
CS/T
BA
TT�
PD
NA
AD
�IC
D
G�
18flu
orod
eoxy
gluc
ose;
A�
axill
ary
lym
phad
enop
athy
;A
AD
�an
tiarr
hyth
mic
drug
s;A
bd�
abdo
min
ally
mph
aden
opat
hy;
AFB
�ac
idfa
stba
cilli
;A
TT�
anti-
tube
rcul
osis
ther
apy;
C�
cerv
ical
lym
phad
enop
athy
;CS
�ca
rdia
csa
rcoi
dosi
s;IC
D�
impl
anta
ble
iove
rter
-defi
brill
ator
;IVS
�in
terv
entr
icul
arse
ptum
;LB
�le
ftbu
ndle
;LI�
left
infe
rior
axis
;LS
�le
ftsu
perior
axis
;LVA
�le
ftve
ntricu
larap
ex;L
VEF
�le
ftve
ntricu
larej
ectio
nfr
actio
n;LV
-LW
�le
ftve
ntricu
larla
tera
lwal
l;M
�m
edia
stin
ally
mph
aden
opat
hy;M
.tbP
CR
�
mer
ase
chai
nre
actio
nto
dete
ctM
ycob
ater
ium
tube
rcul
osis
;Mtx
�m
etho
trex
ate;
N�
nega
tive;
NA
�no
tav
aila
ble;
P�
posi
tive;
PD
N�
pred
niso
lone
;RB
�righ
tbu
ndle
;RI�
righ
tin
ferior
axis
;RFA
�ra
diof
requ
ency
abla
tion;
RS
�righ
tsu
perior
axis
;RVA
�righ
tricu
lar
apex
;RVO
T�
righ
tve
ntricu
lar
outfl
owtr
act;
S�
supr
acla
vicu
lar
lym
phad
enop
athy
;TB
�tu
berc
ulos
is;V
T�
vent
ricu
lar
tach
ycar
dia.
53JACC Vol. 58, No. 1, 2011 Thachil et al.June 28, 2011:48–55 VT in Granulomatous Disease
Unlike Banba et al. (8), who described VT as a latemanifestation of CS, we found that both the early inflam-matory and late scar phases of this disease could causeSMVT, as evidenced by: 1) myocardial inflammation with-out scar in 2 patients, and scar without inflammationcausing SMVT in another; and 2) resolution of inflamma-tion on 18FDG PET-CT correlating with reduction in VTburden.CS versus myocardial tuberculosis. Isolation of M.tuber-ulosis in 2 of our patients and their response to ATTonfirms isolated myocardial tuberculosis presenting asMVT, a rare but described entity (9). Whether M.tuber-
ulosis PCR positivity in sarcoid granulomas has an etiolog-cal implication is a matter of debate (10). Tuberculin skinest positivity has been used to differentiate tuberculosisrom sarcoidosis; whether this observation can be extendedo isolated CS is unexplored (11). From a therapeuticerspective, administering immunosuppressants withoutoncomitant ATT to such patients could result in flare-upf tuberculosis. This underscores the importance of evalu-tion for tuberculosis among these patients.reatment considerations. Unlike usual forms of SMVTith preserved EF, those due to granulomatous myocarditis
esponded poorly to RFA and had a 100% recurrence rate,finding similar to that reported by Koplan et al. (12).ppropriate therapies occurred in 67% of ICD recipients.ddition of disease-specific therapy significantly decreasedT burden and nearly eliminated VT storms. We recom-end a combination of disease-specific therapy, AADs, and
CD rather than RFA as the first line of treatment forMVT with preserved EF due to granulomatous
Figure 4 Change in VT Frequency After Addition of Disease-Specific Therapy to Antiarrhythmic Drugs/RFA
In the 13 patients available to follow-up, the effect of disease-specific therapywas measured starting from 3 months after its initiation. Mean follow-upbefore effective disease-specific therapy was 37 � 53 months and after effec-tive therapy was 16 � 13 months. Effective disease-specific therapy caused asignificant (p � 0.05) decrease in ventricular tachycardia (VT) burden. RFA �
radiofrequency ablation.
myocarditis.Ba T P S
18FD
card
poly
vent
emission tomographic computed tomography scan; IVS � interventricular septum; IW � inferior wall of the left ventricle; LV � left ventricle; LW � lateral wall of the left ventricle; RFA � radiofrequencyablation; RVOT � right ventricular outflow tract.
54 Thachil et al. JACC Vol. 58, No. 1, 2011VT in Granulomatous Disease June 28, 2011:48–55
Figure 5 Pre- and Post-Treatment 18FDG PET-CT Scans Showing Resolution of Inflammation With Treatment
(A) 18Fluorodeoxyglucose positron emission tomographic computed tomography scan (18FDG PET-CT) images showing high myocardial and mediastinal node (whitearrows) uptake at baseline which resolved after treatment with anti-tuberculosis therapy in a patient with a positive M. tuberculosis culture from the mediastinal node.(B) 18FDG PET-CT images showing high myocardial, mediastinal, and splenic (white arrows) uptake at baseline which resolved after treatment with corticosteriods andmethotrexate in a patient with a negative acid fast bacilli stain, culture, M. tuberculosis DNA-polymerase chain reaction, and a negative tuberculin skin test.
EPS/RFA Findings of Patients Undergoing RFA, Correlated With DE-CMR and 18FDG PET-CT FindingsTable 3 EPS/RFA Findings of Patients Undergoing RFA, Correlated With DE-CMR and 18FDG PET-CT Findings
Patient Serial #
1 7 8 10 12
Site of scar in DE-CMR None IVS IVS, AW, IW, LW IVS, RVOT RVOT
Site of inflammation in 18FDG PET-CT IVS IVS, AW, LW Not done IVS, LW AW, IW, LW, LV apex
Site of low voltage None IVS IVS LW RVOT
Site of RFA IVS/RVOT septum IVS IVS LW RVOT
Patient serial # correspond to Table 2 and Figure 2.AW � anterior wall of the left ventricle; DE-CMR � delayed gadolinium enhanced cardiac magnetic resonance imaging; EPS � electrophysiological study; 18FDG PET-CT � 18fluorodeoxyglucose positron
1
1
1
55JACC Vol. 58, No. 1, 2011 Thachil et al.June 28, 2011:48–55 VT in Granulomatous Disease
Study limitations. This is a predominantly retrospectiveanalysis performed on a small number of patients from anarea endemic to tuberculosis. The report highlights theeffect of specific therapy in myocardial tuberculosis anddescribes only a limited number of patients with CS.Etiological factors might vary in different areas; however,work-up for various granulomatous disorders is warranted inthis entity. Larger, prospective studies are required to clarifythe relative diagnostic importance of the various findings wehave observed.
ConclusionsA subset of patients with SMVT and preserved EF hasunderlying granulomatous inflammation, due to CS or myo-cardial tuberculosis. Mediastinal adenopathy with mid-myocardial scar and/or focal myocardial hypermetabolismcharacterize this subset. Histopathology of the involved nodesreveals granulomas. Recurrent arrhythmia is frequent in them,despite AADs and RFA. Disease-specific therapy tailored tothe underlying disease can reduce and often abolish recurrentVT. Late recurrences of VT seem to justify ICD insertion.
AcknowledgmentsThe authors wish to thank Professor Hein J. Wellens(University of Maastricht, Maastricht, the Netherlands),Professor S. Jaishankar (CARE Hospitals, Hyderabad,India), and Dr. Andre d’Avila (Mount Sinai Hospital,New York, New York) for reviewing this report.
Reprint requests and correspondence: Dr. Calambur Narasimhan,Cardiac Arrhythmia Services, CARE Hospital, Road Number 1,Banjara Hills, Hyderabad, Andhra Pradesh 500034, India. E-mail:
[email protected].m
REFERENCES
1. Latif S, Dixit S, Callans DJ. Ventricular arrhythmias in normal hearts.Cardiol Clin 2008;26:367–80.
2. Aliot EM, Stevenson WG, Almendral-Garrote JM, et al. EHRA/HRS expert consensus on catheter ablation of ventricular arrhythmias.Europace 2009;11:771–817.
3. Kim RJ, Shah DJ, Judd RM. How we perform delayed enhancementimaging. J Cardiovasc Magn Reson 2003;5:505–14.
4. Okumura W, Iwasaki T, Toyama T, et al. Usefulness of fasting18F-FDG PET in identification of cardiac sarcoidosis. J Nucl Med2004;45:1989–98.
5. Hiraga H, Hiroe M, Iwai K. [Guideline for Diagnosis of CardiacSarcoidosis: Study Report on Diffuse Pulmonary Diseases]. Tokyo,Japan: The Japanese Ministry of Health and Welfare, 1993;23–4 [inJapanese].
6. Soejima K, Yada H. The work-up and management of patients withapparent or subclinical cardiac sarcoidosis: with emphasis on theassociated heart rhythm abnormalities. J Cardiovasc Electrophysiol2009;20:578–83.
7. Uusimaa P, Ylitalo K, Anttonen O, et al. Ventricular tachyarrhythmiaas a primary presentation of sarcoidosis. Europace 2008;10:760–6.
8. Banba K, Kusano KF, Nakamura K, et al. Relationship betweenarrhythmogenesis and disease activity in cardiac sarcoidosis. HeartRhythm 2007;4:1292–9.
9. Khurana R, Shalhoub J, Verma A, et al. Tubercular myocarditispresenting with ventricular tachycardia. Nat Clin Pract CardiovascMed 2008;5:169–74.
0. Gupta D, Agarwal R, Aggarwal AN, Jindal SK. Molecular evidencefor the role of mycobacteria in sarcoidosis: a meta-analysis. Eur RespirJ 2007;30:508–16.
1. Gupta D, Chetty M, Kumar N, Aggarwal AN, Jindal SK. Anergy totuberculin in sarcoidosis is not influenced by high prevalence oftuberculin sensitivity in the population. Sarcoidosis Vasc Diffuse LungDis 2003;20:40–5.
2. Koplan BA, Soejima K, Baughman K, Epstein LM, Stevenson WG.Refractory ventricular tachycardia secondary to cardiac sarcoid: elec-trophysiologic characteristics, mapping, and ablation. Heart Rhythm2006;3:924–9.
Key Words: cardiac sarcoidosis y granulomatous myocarditis y
yocardial tuberculosis y ventricular tachycardia.