Case

Reports

Atypical Transient StressInduced Cardiomyopathies

with an Inverted
Takotsubo Pattern
in Sepsis and in the Postpartal State

Sahng Lee, MD
Kyung Jin Lee, MD
Hyeon Soo Yoon, MD
Ki-Woon Kang, MD
Young Sook Lee, MD
Jun Wan Lee, MD

Several cases of inverted Takotsubo cardiomyopathya variant form with hyperdynamic

left ventricular apex and akinesia of the left ventricular base and mid-portionhave been
reported recently, especially in association with cerebrovascular accidents and catecholamine cardiomyopathies. Herein, we describe 2 cases of inverted Takotsubo cardiomyopathy: one that occurred in a middle-aged woman who had a septic condition, and another
in a young woman who was in the postpartal state. Such cases have not been reported
previously. (Tex Heart Inst J 2010;37(1):88-91)

S
Key words: Cardiomyopathies/physiopathology/
therapy/ultrasonography;
coronary angiography;
echocardiography; heart
ventricles/physiopathology/
radiography; postpartum
period; sepsis; ventricular
dysfunction/diagnosis/
physiopathology; ventricular
dysfunction, left
From: Departments of
Internal Medicine (Drs.
Kang, K.J. Lee, S. Lee, Y.S.
Lee, and Yoon) and Cardiothoracic Surgery (Dr. J.W.
Lee), Eulji University School
of Medicine, Daejeon 302799, Republic of Korea
Address for reprints:
Kyung Jin Lee, MD,
Department of Internal
Medicine, Eulji University
Hospital, 1306 Dunsandong, Seo-gu, Daejeon
302-799, Republic of Korea
E-mail:
lee.kjlee@gmail.com
2010 by the Texas Heart
Institute, Houston

88

tress-induced cardiomyopathy is characterized by a transient abnormality of

left ventricular (LV) apical wall motion, electrocardiographic changes, and
minimal cardiac enzyme release. The condition mimics acute coronary syndrome in patients who have no angiographic stenosis upon coronary angiography.
Recently, atypical stress-induced cardiomyopathies without involvement of the LV
apex have been reported.1 Most of the cases were transient midventricular ballooning
syndrome with midventricular akinesia and normal wall motion of the LV base and
apex,1-3 and some were the inverted Takotsubo pattern cardiomyopathy that is characterized by a hyperdynamic LV apex and akinesia of the LV base and mid-portion.4-6
Here, we describe 2 cases of inverted Takotsubo cardiomyopathy, one of which occurred in a middle-aged woman with a septic condition and one in a young woman
who was in the postpartal state.

Case Reports
Patient 1

In June 2007, a 41-year-old woman was referred to us by the general surgery department at our institution because she suddenly developed hemodynamic instability with
blood pressure of 70/40 mmHg and heart rate of 120 beats/min. Her medical history included hospitalization for 6 months after a jejunostomy with bowel resection,
and reoperation because of a metastatic myometrial sarcoma. The patients condition
was stable during that time; she was receiving total parenteral nutrition via the subclavian root, along with anticancer treatment. Now, upon physical examination, she
was semicomatose and febrile, with a body temperature of 38.5 C. She was intubated, and an arterial blood-gas analysis gave the following values: pH, 7.3; partial pressure of oxygen, 176 mmHg; carbon dioxide pressure, 49 mmHg; bicarbonate, 25.7
mEq/L; and fraction of inspired oxygen, 0.8. Clinical and laboratory findings suggested a septic condition with multiorgan damage: white blood cell count, 24,330/
mm3; hemoglobin, 11.6 g/dL; platelet count, 22,000/mm3; C-reactive protein, 16.56
mg/dL; alanine aminotransferase, 365 U/L; aspartate aminotransferase, 137 U/L;
total bilirubin, 11.6 mg/dL; and serum creatinine, 1.5 mg/dL. N-terminal pro-brain
natriuretic peptide was elevated to 3,500 pg/mL, and cardiac enzymes peaked at
creatine kinaseMB fraction, 19.47 ng/mL (reference range, 07 ng/mL) and troponin T, 0.393 ng/mL (reference range, <0.01 ng/mL). Chest radiography showed mild
cardiomegaly and pulmonary congestion with pleural effusion. Electrocardiography
showed sinus tachycardia with T inversion in leads V4 through V6 and QT prolonga-

Atypical Cardiomyopathies with Inverted Takotsubo Pattern

Volume 37, Number 1, 2010

tion. Echocardiography revealed severe LV systolic dysfunction with akinesia of the LV base and mid-portion,
together with hypercontractility of the apex (Figs. 1A
and 1B). Coronary angiography on the same day revealed that both coronary arteries were intact (Figs. 1C
and 1D). Left ventriculography showed akinesia of the
LV base and mid-portion except for the apex (Figs. 1E
and 1F). Because of the patients hemodynamic instability, intra-aortic balloon pumping was begun, and
medical treatment that included inotropic agents and
antibiotics was started. She responded quickly to the
treatment, and intra-aortic balloon pumping was discontinued 2 days later. Follow-up echocardiography 1
week later indicated complete recovery of LV systolic function.
Patient 2

In July 2007, a 30-year-old woman with no history of

cardiac disease was referred to us from the obstetrics department at our institution because of chest discomfort
and dyspnea (New York Heart Association functional
class III), 5 days after a cesarean delivery. Her symptoms
had developed 1 day after delivery and had progressed
even after treatment with diuretics. On physical examination, she was afebrile, with blood pressure of 130/80
mmHg, heart rate of 90 beats/min, and respiration rate

of 24 breaths/min. The heart sounds were regular, with

an S3 and a holosystolic murmur (grade 4/6) in the mitral area. Laboratory findings included a white blood
cell count of 7,800/mm3, a hemoglobin level of 11 g/
dL, and a platelet count of 236,000/mm3. Cardiac enzymes peaked at creatine kinaseMB fraction, 7.12 ng/
mL (reference range, 07 ng/mL) and troponin T, 0.12
ng/mL (reference range, <0.01 ng/mL). Chest radiography showed cardiomegaly and pleural effusion in
both lungs. Electrocardiography revealed T-wave inversions in leads I, aVL, V1, and V2. Echocardiography
showed moderate LV systolic function (LV ejection fraction, 0.35 by the modified Simpson method) with severe hypokinesia of the LV base and mid-portion, and
hypercontractility of the LV apex (Figs. 2A and 2B).
Coronary angiography performed on the same day revealed that both coronary arteries were intact (Figs. 2C
and 2D), and left ventriculography showed severe global hypokinesia except for the LV apex (Figs. 2E and 2F).
The patient received standard heart-failure treatment,
comprising diuretics and angiotensin-converting enzyme inhibitors. Although she responded well to treatment, follow-up echocardiography 1 week later revealed
no significant improvement in LV systolic function.
Two weeks later, echocardiography showed complete
recovery of LV systolic and diastolic function.

Fig. 1 Patient 1. Echocardiography shows severe left ventricular systolic dysfunction with akinesia of the left ventricular base and midportion, and hypercontractility of the apex (A and B). Coronary angiography shows that the C) left and D) right coronary arteries are
intact. Left ventriculography reveals global akinesia except for the left ventricular apex (E and F).

Texas Heart Institute Journal

Atypical Cardiomyopathies with Inverted Takotsubo Pattern

89

Fig. 2 Patient 2. Echocardiography shows severe hypokinesia of the left ventricular base and mid-portion, and hypercontractility of the
left ventricular apex (A and B). Coronary angiography shows that the C) left and D) right coronary arteries are intact. Left ventriculography reveals severe global hypokinesia except in the left ventricular apex (E and F).

Discussion
Stress-induced cardiomyopathy, also called transient
LV apical ballooning or Takotsubo cardiomyopathy, is
a clinical entity that was first described in Japan.7 It is
characterized by a transient abnormality of LV apical
wall motion (which gives the heart the appearance of a
Japanese octopus trap or takotsubo), electrocardiographic changes, and minimal cardiac enzyme release, and the
condition mimics acute coronary syndrome in patients
who have no angiographic stenosis upon coronary angiography. The underlying pathogenesis remains incompletely understood, although some possible mechanisms
have been suggested.8 One such mechanism is myocardial ischemia due to microvascular spasm. Increased sympathetic activity is another possibility, because exposure
to internal or external stresses is confirmed in most cases.
Sympathetic activity is also implicated in the neurogenic
stunned myocardium during acute cerebrovascular accident and in catecholamine cardiomyopathy during the
endocrine crisis of pheochromocytoma.
Atypical stress-induced cardiomyopathies without involvement of the LV apex have been reported recently.1
Most of the cases were instances of transient midventricular ballooning syndrome with midventricular akinesia and normal wall motion of the LV base and apex,
90

although some cases displayed the inverted Takotsubo

pattern of cardiomyopathy, which is characterized by
a hyperdynamic LV apex and akinesia of the LV base
and mid-portion. Inverted Takotsubo cardiomyopathies have been reported in patients who experienced
an acute cerebrovascular accident,4 pheochromocytoma,5,6 paraglioma,9 acute pancreatitis,10 amphetamine
use,11 or shoulder surgery.12 Most patients experienced a
cerebrovascular accident or pheochromocytoma, which
explains the state of catecholamine excess. Inverted Takotsubo cardiomyopathy triggered by a septic condition
or parturition has not been reported. Our reports are
unique in that the cardiomyopathies presented during
sepsis in a middle-aged woman and after parturition in
a young woman.
It remains unclear why the LV apex is selectively vulnerable and subsequently forms a balloon in typical Takotsubo cardiomyopathy. Several anatomic and physiologic factors might contribute to LV apical wall-motion
abnormalities, including the lack of a 3-layered myocardial structure at the LV apex and the easy loss of elasticity of the LV apex after excessive expansion.8 Although
myocardial responsiveness to adrenergic stimulation increases in the apical myocardium, the norepinephrine
content is lower in the apex than in the base; the human
heart has a heterogeneous nerve-distribution pattern.13

Atypical Cardiomyopathies with Inverted Takotsubo Pattern

Volume 37, Number 1, 2010

However, the apex may notin all patientsbe a structure more vulnerable to catecholamine excess than is the
mid-ventricle or the base, and the observation of inverted Takotsubo cardiomyopathy in some individuals suggests this possibility.

7.
8.

References
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