Echocardiography in Chagas Heart Disease

Harry Acquatella

Circulation. 2007;115:1124-1131
doi: 10.1161/CIRCULATIONAHA.106.627323
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 Echocardiography in Chagas Heart Disease
Harry Acquatella, MD

BackgroundChagas heart disease is a frequent cause of morbidity and mortality in Latin America. Echocardiography
provides useful diagnostic and prognostic information and is an important tool in the management of patients with
Chagas disease.
Methods and ResultsA search for relevant publications was obtained from MEDLINE, LILACS, and SCIELO sources.
Acute Chagas myocarditis is a rare disorder in which pericardial effusion is frequent. Echocardiography may exclude
pericardial tamponade in case of heart failure. Chronic Chagas cardiomyopathy evolves for several decades after the
infection. Epidemiological history, positive serology, and suggestive clinical and ECG abnormalities establish the
diagnosis. About three quarters of chronic Chagas cardiomyopathy subjects remain asymptomatic with normal
(indeterminate form) or abnormal ECGs. Early Doppler abnormalities includes prolongation of isovolumic contraction
and relaxation times. Systolic function frequently is normal, but dysfunction may be elicited by stress tests. Half or more
of symptomatic patients have a left ventricular apical aneurysm and other segmental contractile abnormalities similar
to those seen in coronary heart disease. The dilated nonsegmental form is indistinguishable from dilated cardiomyop-
athy. Results from univariate and multivariate Cox survival analyses indicate that impaired systolic function and
increased ventricular dimensions have significant value in predicting cardiac morbidity and mortality. Cardiac
ultrasound commonly is used in the follow-up of patients and in the assessment of various therapeutic modalities.
ConclusionsEchocardiographic and Doppler techniques provide useful structural and functional information in the
detection of early myocardial damage, risk assessment of prognosis, disease progression, and management of patients
with Chagas disease. (Circulation. 2007;115:1124-1131.)
Key Words: cardiomyopathy Chagas disease echocardiography, Doppler echocardiography
heart failure survival

C hagas disease remains a public health problem in several

countries of Latin America. Prophylactic control pro-
grams have diminished substantially the number of infected
Chagas disease. Other aspects of epidemiology and patho-
physiology are briefly considered.

individuals from 16 to 18 million in the early 1990s to 11 Acute Chagas Disease

million in a recent estimation, but 100 million persons still
continue to be at risk of acquiring the infection.1 The
diagnosis of Chagas disease requires an epidemiological
history and 2 positive serologic tests. Cardiac damage is
suspected by 1 of the following ECG findings: right
bundle-branch block, left anterior fascicular block, AV
blocks, multiform ventricular beats, sinus bradycardia, abnor-
mal ST-T segment, and abnormal T and Q waves.27 The
decreasing prevalence of the disease in some endemic areas
or migration of infected subjects to urban nonendemic places
within or outside their original country may result in misdi-
agnosis of a cardiomyopathy of different origin.8,9 Two-
dimensional and Doppler echocardiography provides addi-
tional valuable information on cardiac structure and function
that complements information provided by ECG. This review
is focused primarily on the utility of cardiac ultrasound in the
diagnosis, classification, and detection of early myocardial
damage and the prognostic assessment of patients with
Chagas disease usually is acquired during childhood.1,3,4,7
Infected forms of Trypanosoma cruzi present in the feces of
reduvidae insects penetrate the skin or conjunctiva of people
living in poor rural housing. The disease has an acute phase
and a chronic phase. Acute Chagas disease may appear as a
nonspecific febrile illness lasting for 2 to 8 weeks, becom-
ing clinically manifest in 1% of infected subjects.3,4 Acute
chagasic myocarditis is infrequent, appearing in only 1% to
5% of those having the acute phase (1 to 5 of every 10 000
infected subjects).4
The largest published echocardiographic series on acute
Chagas disease includes 58 subjects. 10 Abnormal
2-dimensional echocardiograms were present in 52%, and
pericardial effusion was seen in 42%. In 10 of 12 patients
with heart failure (HF), the effusion was moderate to severe.
Of note, mean left ventricular ejection fraction (LVEF) was
normal (63%). Apical or anterior dyskinesis was found in
21%, and only 6% had LV dilation. Five patients died, 4 of

Received March 15, 2006; accepted August 8, 2006.

From the Faculty of Medicine Universidad Central de Venezuela, Hospital Universitario and Centro Medico, Caracas, Venezuela.
Correspondence to Dr Harry Acquatella, 275 Centro Medico, San Bernardino, Caracas 1011, Venezuela. E-mail hacquatella@cantv.net
2007 American Heart Association, Inc.
Circulation is available at http://www.circulationaha.org DOI: 10.1161/CIRCULATIONAHA.106.627323

1124
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 Acquatella
Figure 1. Four-chamber apical view echocardiogram of a
woman with a 1-month history of acute Chagas myocarditis
with pericardial effusion and tamponade. Chamber size and sys-
tolic function were normal. Notice the compression of the right
atrial (RA) wall. LA indicates left atria; PE, pericardial effusion.
them in HF. In 2 other reports,11,12 pericardial effusion was
present in 7 of 8 patients, 3 of whom were in tamponade.
Figure 1 shows a patient with this complication who im-
proved substantially after pericardiocentesis. These findings
demonstrate the need to perform echocardiograms to rule out
a rapidly treatable cause of HF (pericardial effusion) and to
evaluate systolic dysfunction during the acute phase. More
studies are necessary.
Chronic Chagas Disease
In most patients, the initial infection occurs unnoticed,
passing to the chronic stage 1 to several decades later. On
histology, diffuse and patchy chronic myocarditis, intersti-
tial mononuclear cell infiltrates, and myocardial fiber
destruction with fibrotic replacement are commonly pres-
ent3,4,1316 (Figure 2). Most initial autopsy studies per-
formed in subjects dying at the end stage of the disease
reported grossly enlarged hearts.3,4,14,16 A pioneering nec-
ropsy work17 pointed out the high rate of LV chagasic
apical aneurysm found in these patients. This result was
confirmed by others studies,14,18 including a large series of
1078 autopsies19 in which apical aneurysms were found in
half of cases, more frequently in male than in female
patients, and at similar proportions in all age groups.
Eighty-two percent were found at the LV apex, 9% at the
right ventricular (RV) apex, and 9% at both. Other series
had rates ranging from 30% to 92%.3,14,17,18 Additional LV
segmental lesions such as at the posteroinferior walls
ranged from 21%14 to 33%3 (Figure 2).
Chronic Chagas cardiomyopathy (CCM) may be detected
with or without symptoms. Most investigators combine clin-
ical and ECG findings, cardiomegaly, and systolic dysfunc-
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Echocardiography in Chagas Disease 1125
Figure 2. Echocardiogram and postmortem specimen from a
patient who had HF secondary to CCM. A, M-mode long-axis,
slow-sweep echocardiogram showing the relatively preserved
systolic septal (S) motion and thickening and a thin, noncontrac-
tile posterior wall (PW). B, Long-axis autopsy section of the
heart. Notice the LV apical and posteroapical fibrotic thinning
with relative septal sparing. The coronary arteries were normal.
Reproduced from Acquatella et al25, with permission of the pub-
lisher. Copyright 1980, the American Heart Association.
tion into 4 groups of progressive heart damage7,20 23 (Table
1). The recent American College of Cardiology/American
Heart Association staging24 of disease progression into A
(high risk of HF without structural heart disease), B (struc-
tural heart disease without HF), C (structural heart disease
with prior or present HF), and D (refractory HF) is incorpo-
rated into Table 1. Asymptomatic subjects comprise about
three quarters of seropositive persons. Those with a normal
ECG are referred as being in the indeterminate phase of the
disease (stage A). The appearance of ECG abnormalities
implies disease progression (stage B). By definition, no
cardiomegaly is present, and LV systolic function is normal.
Fewer than 10% may have LV segmental wall motion
abnormalities. Symptomatic patients with mild to moderate
cardiac damage are in New York Heart Association (NYHA)
functional class II or III (stage C) and may present with
arrhythmias, embolism, sudden death, and reversible HF.20 22
The ECG is abnormal in almost all. The heart is dilated, and
LV systolic and diastolic functions are usually abnormal.
About half of these patients may have LV apical and other
segmental wall abnormalities.21,2529 Mitral and tricuspid
valve regurgitation may be present. Survival at 10 years is
75% to 85%.21,22,30 Those with HF in NYHA functional
class IV (stage D),13,21,22,30 may, if untreated, have a very high
1126 Circulation March 6, 2007

TABLE 1. Clinical, ECG, and Echocardiographic Findings in CCM

Asymptomatic CCM Symptomatic CCM
NYHA class I IIIII IV
Stage A B C D
5
ECG Normal Abnormal Abnormal
RBBB, % 918 3440
LAFB, % 915 2339
PVCs, % 39 1275
AVB 12, % 25 914
Heart size Normal Increased
LV aneurysm, % 1.68.6 4764
LV posterobasal lesion, % 5.322 1630
LV ejection fraction Normal Decreased
LV diastolic function* NL NL, PR PR, PN, RR RI
Survival at 5 y, % 98 85 30
RBBB indicates right bundle-branch block; LAFB, left anterior fascicular block; PVCs, premature
ventricular contractions; AVB 12, first- and second-degree AV block; NL, normal; PR, prolonged
relaxation; PN, pseudonormal; RR, restrictive reversible; and RI, restrictive irreversible. Percentages
represent the range of means from different series.
*All may have prolonged isovolumic times.

mortality (50%) at 2 years. In all groups, coronary angiogra- ellipsoid shape even in patients with relatively preserved LV
phy, when performed, is normal or shows nonsignificant volumes. This finding may suggest that early segmental lesions
lesions. may progress to diffuse hypokinesis and increased LV end-dia-
stolic dimensions as found in advanced disease.
Echocardiographic and Doppler Findings
Anatomic Abnormalities
In asymptomatic subjects, it may be difficult to differentiate
precisely a normal thin apical segment from an early scar. This
difficulty may explain the different rates of apical abnormalities
published in various reports.26 29,3134 LV cavity opacification
with ultrasound contrast and harmonic imaging may help in
difficult cases.31 The aneurysm may range from as small as a
hollow punch to large, indistinguishable from a myocardial
infarction.
The mean prevalence of LV aneurysm from different
2-dimensional echocardiographic series was 8.5% (range, 1.6%
to 8.6%)26,28,31 among 920 asymptomatic individuals or patients
with mild cardiac damage and 55% (range, 47% to 64%)26 29 in
242 patients with moderate to severe cardiac impairment (Table
1 and Figure 3). In a recent work that included 1053 subjects,34
it was less frequent, 2% in subjects with a normal ECG and 24%
in those with an abnormal ECG. In logistic regression analysis,
the LV apical aneurysm was an independent predictor of mural
thrombus.34 In another work, patients with aneurysms had a
significant association with thrombus and stroke during a mean
follow-up of 2 years.29
Segmental LV contractile abnormalities of other walls also
may be detected. The most common is at the posteroinferior
wall, with a mean prevalence of 20% (range, 5.3% to
22%)26,28,31,35 in 1164 asymptomatic subjects or patients with
mild heart damage and of 23% (range, 16% to 30%)26 28 in
280 patients having symptoms or with HF (Figure 2).
Endocardial curvature and global shape evaluated by Fourier Figure 3. Two-dimensional 4-chamber echocardiogram apical
analysis of LV 2-dimensional echocardiograms36 disclosed a view of a patient with CCM with an LV apical aneurysm (AA).
There is a distinct limit between the normal contractile myocar-
significantly decreased apical curvature with a more spherical dium and the dyskinetic apex (arrows). LVEF was moderately
LV, resulting in a disruption of the optimal global prolate- decreased (40%).

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 Acquatella
Systolic Function
Subjects presenting with the indeterminate form almost
invariably have a normal global systolic function,31,33,3739
but some may have abnormal echocardiograms.31 Among
505 subjects with normal ECGs and a mean normal LVEF
of 67%, 13% had segmental lesions and 0.8% had systolic
dysfunction. In contrast, of 257 persons with abnormal
ECGs and a mean LVEF of 68%, 33% had segmental
lesions and 8% had systolic dysfunction. Both groups had
normal biventricular end-diastolic and end-systolic dimen-
sions.31 In patients with abnormal ECGs, global systolic
function has prognostic implications. A cohort of 538
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Echocardiography in Chagas Disease 1127
Figure 4. Doppler findings of the patient in
Figure 3 (tracings are aligned with the first
QRS complex). A, Mitral valve pulsed-
wave Doppler displays a decreased E/A
ratio of 0.64 and a prolonged E decelera-
tion time of 295 ms, consistent with a dia-
stolic pattern of prolonged relaxation.
Mitral regurgitation was mild. B, TDI at the
septal mitral annular level shows pro-
longed isovolumic contraction time (ICT)
and relaxation time (IRT) of 130 and 155
ms, respectively. S, E, and A peak veloc-
ities are decreased. Note the prominent
apical-directed IRT wave (white arrow on
C), probably related to earlier relaxation of
the aneurysm. C, Color Doppler M-mode
velocity propagation of the LV inflow tract.
A diminished slope of the color edge in
early diastole (white line) represents a
lower velocity of blood flow toward the
aneurysmal apex, consistent with loss of
apical suction.
patients39 grouped in 4 stages of disease progression (A to
D)24 had markedly dissimilar 5-year survival rates of 98%,
91%, 45%, and 13% for those with a normal LVEF, mildly
decreased LVEF, reversible HF, or irreversible HF,
respectively.
With tissue Doppler imaging (TDI),40 myocardial velocity
systolic shortening and diastolic lengthening (E and A)
during the cardiac cycle can be estimated (Figure 4). With
TDI, subjects with normal ECGs and 2-dimensional echocar-
diograms have shown prolonged LV41,42 and RV43 isovolu-
mic contraction times as signs of early contractile abnormal-
ities. Thus, a normal ECG does not rule out myocardial
1128 Circulation March 6, 2007

TABLE 2. Results of Echocardiographic Survival Analysis

Events, %

Patients, Mean Follow-Up, Mortality, Mortality Predictors*

Author n mo % SD HF EM (P0.05)
Ianni et al38 159 99 0 0 0 0
Viotti et al31 849 118 0.6 LVSD, LVEF, group
Salles et al57,58 738 58 8 74 22 4 LVSD, QTd
34
Xavier et al 1053 66 10 57 22 4 LVEF
Bestetti et al59 56 24 28 69 31 LVEF
Rodriguez-Salas et al32 283 48 38 37 44 6 Group, FS, EPSS
Mady et al60 104 30 48 64 36 LVEF, VO2max
SD indicates sudden death; EM, embolism; LVSD, left ventricular systolic dimension; Group, clinical group of each
series; QTd, ECG QT-interval dispersion; FS, fractional shortening; and EPSS, M-mode echocardiographic E-point
septal separation. Decimals were rounded.
*Multivariate analysis.

abnormalities, the clinical significance of which is currently The combination of pulsed Doppler of mitral valve inflow,
unknown. pulmonary veins, and mitral annular TDI velocities currently
In patients with normal EF, systolic dysfunction may is used to classify patients into 4 groups of worsening
become apparent under pharmacological stress. An impair- diastolic function: abnormal relaxation, pseudonormal, re-
ment for chronotropic, global, and regional contractile re- versible, and nonreversible restrictive filling.40
sponses has been seen during standard dobutamine stress In a group of 169 CCM subjects, diastolic dysfunction was
echocardiography.44 Some patients show a biphasic response found in 20% of the patients.26 There was a strong
(initially augmentation followed by hypocontractility at correlation between worsening diastolic function and in-
higher dobutamine doses) predominantly at the LV posteroin- creased left atrial and ventricular dimensions and decreased
ferior wall segments, suggestive of viable but dysfunctional LVEF. A reduced TDI septal E wave of 11 cm/s and a septal
myocardium. The possible mechanisms are complex and may E/E ratio of 7.2 were highly sensitive and moderately
include -adrenergic dysfunction, endothelial dysfunction, specific and had a high negative predictive value for detecting
ischemia, and structural myocardial damage.44 Others have any kind of diastolic dysfunction (Figure 4). Another group of
shown a lower slope of the end-systolic pressure dimension 89 patients52 were classified according to the presence of
relation during phenylephrine infusion,45 suggestive of a normal or pseudonormal ventricular filling pattern. Patients
reduction in LV contractility in patients with the indetermi-
nate form, mild cardiac involvement, or the digestive form of
the disease.45
RV systolic impairment may be the only abnormality
detected by biventricular radionuclide angiography.46,47 In
contrast, in a recent group of 74 patients studied by
2-dimensional and Doppler echocardiography, RV dysfunc-
tion, when present, was secondary to the severity of LV
damage and high levels of pulmonary pressure rather than
primary RV depressed function.48 These apparently discor-
dant results may be due to the different techniques used.

Diastolic Function
The chronic myocarditic damage3,13,14,18,19,49 may impair
ventricular relaxation and diastolic filling. Diastolic abnor-
malities usually precede systolic dysfunction.50,51 Reduced
LV compliance leads to an increase in left atrial pressure,
changes in transmitral and pulmonary venous flow velocities,
and prolongation of systolic and diastolic time intervals.
Early works using simultaneous M-mode echocardiographic,
phonocardiographic, and apex cardiographic tracings50,51 had
already shown that significant prolongation of the isovolumic
relaxation and ventricular filling times was present in asymp-
tomatic subjects with normal systolic function and in symp-
tomatic patients.
Figure 5. Inverse significant correlation between LVEF and mor-
tality among 3138 patients from 6 echocardiographic series. The
first letter indicates the author initial; the second, the group. I
indicates Ianni et al38; V, Viotti et al,31 groups 0, 1, and 2; S,
Salles et al,57,58 groups Y (survived) and N (not survived); B,
Bestetti et al,59 groups Y (survived) and N (not survived); R,
Rodriguez-Salas et al,32 groups A (asymptomatic) and S (symp-
tomatic); and X, Xavier et al,34 groups Y and N (apical aneu-
rysm, yes or no). See text and Table 2 for other details.

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 Acquatella
with pseudonormal filling pattern had statistically signifi-
cantly larger LV dimensions, higher LV wall motion score,
and lower LVEF. TDI was able to differentiate CCM patients
with normal LV diastolic function from those with a pseudo-
normal filling pattern and increased LV filling pressures.
Other Echocardiographic Doppler Techniques
In color M-mode, early inflow jet velocities across the mitral
valve as it moves toward the apex display a slope represent-
ing the blood flow velocity propagation40 (Figure 4). A group
of CCM patients had decreased velocity propagation consis-
tent with relaxation abnormalities, which was more pro-
nounced in those with LV apical aneurysm. LVEF and
pulsed-wave Doppler patterns were not significantly different
between CCM patients and a control group.53
The myocardial performance index (Tei index) is a global
function index combining the time duration of systole and
diastole by pulsed-wave Doppler.54 A study found abnor-
mally high values of LV myocardial performance index
(0.32) in one third of asymptomatic subjects, in all of
symptomatic patients, and in 2% of the control subjects.55
ECG abnormalities in the asymptomatic chagasic patients
were associated with abnormal myocardial performance in-
dex, but not in the control subjects with the same ECG
changes. Symptomatic patients had markedly increased RV
and LV myocardial performance index values, suggestive of
severe myocardial dysfunction.
Doppler-derived myocardial strain also has been studied in
patients with Chagas disease.56 CCM patients had lower
radial and longitudinal values compared with normal control
subjects. However, strain was not able to differentiate be-
tween normal control subjects and subjects with the indeter-
minate form of the disease. This technique was able to
quantify subtle segmental contractile dysfunction not de-
tected visually.
Echocardiographic Prognostic Variables
Echocardiographic series of chronic CCM patients were
selected on the basis of having survival analysis performed by
the Cox multivariate method (Table 2 and Figure 5). The
populations included subjects with a broad expression of
disease severity ranging from none31,38 to none to se-
vere31,32,34,57,58 to severe symptoms.59,60
No mortality occurred in 2 series of asymptomatic subjects
with normal ECG at entry. In 1 series of 159 subjects, there
were no events,38 LVEF remained normal in all subjects, and
ECG was unchanged in nearly 80%. In the other series of 505
subjects, 8% had events.31 In the same work, among 257
asymptomatic subjects with an initially abnormal ECG,
mortality was 1%, and 26% had clinical events. In 87 patients
with abnormal ECGs31 and cardiomegaly but no HF, 52% had
clinical events with 14% mortality. On multivariate analysis,
change in clinical group, LV systolic dimension, and LVEF
were predictors of mortality. Chagas-related ECG abnormal-
ities, LV diastolic and systolic dimensions, and LVEF were
predictors of events.31
Four series included mixed populations, from asymptom-
atic subjects to patients with HF. One consisted of 738
patients.57,58 QT-interval dispersion and LV end-systolic
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Echocardiography in Chagas Disease 1129
dimension were the strongest independent predictors of all
end points. In another work of 1053 subjects classified
according to whether an LV apical aneurysm was present,34
LVEF remained the only significant predictor of mortality.
Apical aneurysm was not significant when adjusted for
LVEF. In a study of 283 patients32 grouped as asymptomatic
and symptomatic, significant mortality risk factors were
initial NYHA class, E-point septal separation, and M-mode
echo fractional shortening. Finally, 2 series had patients with
severe HF. One collected 56 persons in NYHA functional
classes III and IV.59 The only significant risk factor was
LVEF. In a series of 104 male patients60 in NYHA functional
classes II, III, and IV, statistically significant differences
between survivors and nonsurvivors on multivariate analysis
were LVEF and maximal O2 uptake (VO2max). Thus, systolic
dysfunction and increasing heart size emerged as significant
predictors of morbidity and mortality (Figure 5).
Applications in the Management of Patients
Echocardiography is used in the routine clinical evaluation of
CCM patients to assess disease progression, in survival
studies31,32,34,38,57 60 to rule out the presence of intraventric-
ular thrombus,29,34 in the follow-up of pacemakers devices61
and implanted automatic cardioverter-defibrillators,62 and to
monitor response to vasodilator therapy in patients with HF
(enalapril)63 or to antiparasitic medications (benznidazole),64
among others. The last study showed in a long-term
follow-up that the only independent predictors of deteriorat-
ing clinical status were a lower echocardiographic LVEF and
a higher LV end-diastolic diameter.
Study Limitations
The echocardiographic and Doppler findings of CCM are
nonspecific and may be similar to those observed in other
cardiomyopathies (ischemic and nonischemic). A need exists
for comparative studies to estimate the sensitivity and spec-
ificity of such abnormalities. The prognostic value of associ-
ated mitral regurgitation or RV dysfunction is unknown.
LVEF should be preferentially estimated by 2-dimensional
instead of M-mode echocardiography because the latter does
not evaluate the frequently abnormal ventricular apex of these
patients.
Conclusions
In patients with acute chagasic myocarditis, echocardiograms
should be performed almost routinely, especially if HF is
present, to exclude pericardial effusion and tamponade. In the
chronic phase, subjects with a normal ECG have a good
long-term prognosis; most have normal global systolic func-
tion, but contractile and diastolic abnormalities may be found,
although the significance of these findings in disease progres-
sion is unknown. Ventricular involvement in CCM symptom-
atic individuals may range from an isolated small LV apical
aneurysm to a globally dilated heart without segmental scars.
A chagasic origin should be considered in the differential
diagnosis of a cardiomyopathy patient with a positive epide-
miological history and serology, suggestive ECG abnormal-
ities, and apical aneurysm. Depressed LVEF and increased
LV internal dimensions emerged in different survival studies
1130 Circulation March 6, 2007
as significant risk factors associated with increasing morbid-
ity and mortality.
Acknowledgment
We thank Dr Leonardo Rodriguez (Cleveland Clinic) for his help in
editing the manuscript.
Disclosures
None.
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