The International Journal of Cardiovascular Imaging

, Volume 26, Supplement 2, pp 345–351

A case of transient left ventricular apical ballooning syndrome in a child: clinical features and imaging findings

Authors

  • So Young Lee
    • Department of PediatricsSeoul National University Bundang Hospital
  • Se Eun Lee
    • Department of PediatricsSeoul National University Bundang Hospital
  • Jong Woon Choi
    • Department of PediatricsBundang General Hospital, Daejin Medical Center
  • Sang Il Choi
    • Department of RadiologySeoul National University Bundang Hospital
  • Eun Ju Chun
    • Department of RadiologySeoul National University Bundang Hospital
    • Department of PediatricsSeoul National University Bundang Hospital
Original Paper

DOI: 10.1007/s10554-010-9685-1

Cite this article as:
Lee, S.Y., Lee, S.E., Choi, J.W. et al. Int J Cardiovasc Imaging (2010) 26: 345. doi:10.1007/s10554-010-9685-1

Abstract

Transient left ventricular apical ballooning syndrome (TLVABS) is an acute cardiac disease that is characterized by transient left ventricular systolic dysfunction involving the apical region. The symptoms and electrocardiographic changes of TLVABS mimic those observed in acute myocardial infarction while obstructive coronary arterial lesions are not seen in patients with TLVABS. TLVABS usually occurs in elderly women after physical or emotional stress. However, it is very rare in children and so it not well known to pediatricians. Accordingly, TLVABS in children can be misdiagnosed as myocarditis or cardiomyopathy. We report here on a case of child who showed the typical findings of TLVABS in association with pericarditis. He presented with dyspnea and pericardial effusion, which required pericardiocentesis. After pericardiocentesis, he showed the typical echocardiographic and electrocardiographic findings of TLVABS. The MRI findings at 14 days after the initial symptoms showed normal coronary arteries and normal left ventricular function without any wall motion abnormalities. In addition, no delayed hyper enhancement was found on delayed-enhanced (DE)-MRI. We also reviewed the other reported cases of TLVABS in patients who were under the age of 40.

Keywords

Takotsubo cardiomyopathyPericarditisChild

Introduction

Transient left ventricular apical ballooning syndrome (TLVABS) is also called takotsubo cardiomyopathy or stress-induced cardiomyopathy, and this is an acute cardiac disease of an unknown etiology. It is characterized by transient left ventricular systolic dysfunction, i.e., ballooning of the left ventricular apex in the systolic phase due to apical akinesia. The disease was first described by Dote et al. [2] in 1991 and it was named after the Japanese octopus trap, called “takotsubo”, due to the shape of the left ventricle in the systolic phase [6].

The clinical features of TLVABS are similar to those of anterior acute myocardial infarction except that coronary angiography fails to detect a significant obstructive lesion. Moreover, in almost all patients with TLVABS, the left ventricular wall motion becomes normal within weeks [6].

TLVABS typically occurs in elderly women after physical or emotional stress [9], but a few cases involving younger patients have been reported [4, 5, 9]. In this report, we describe a case that initially presented with pericarditis and in the ensuing days the patient exhibited the typical manifestations of TLVABS. We also review the published cases of TLVABS in patients under the age of 40.

Case report

A 14-year-old boy visited the hospital due to exertional dyspnea and general weakness that had developed seven days prior to his admission. An intermittent fever and a non-productive cough had begun 4 days prior to admission. The patient had not suffered any chest or epigastric pain. His past medical history and family history were not remarkable. On physical examination, he looked pale and acutely ill, but he was not cyanotic or dyspneic. His skin was cold, but not mottled. His vital signs were as follows: the blood pressure was 120/65 mmHg, the pulse rate was 111/min, the respiratory rate was 22/min and the body temperature was 36.9°C. The precordium was rather quiet. A grade 2/6 systolic murmur was audible at the apex and the heart sounds were decreased. The breath sounds of the left lower lung were decreased, but they were not associated with crackling or wheezing. The liver was palpable 2 cm below the right costal margin. The peripheral pulses were thought to be slightly weak. Any friction rub was not noted before pericardiocentesis, but this was audible over the precordial region after pericardiocentesis.

The initial creatine kinase level, the MB fraction of creatine kinase and the cardiac troponin I level were within the normal ranges. However, the B-type natriuretic peptide level was slightly elevated at 214.5 pg/ml (normal range 0–88 pg/ml), and this level reached the peak level of 8,552 pg/ml at the 4th day in the hospital. It gradually improved over the following 3 months and then it finally normalized. The C-reactive protein level was also elevated on admission (9.06 mg/dL). The initial chest radiograph showed moderate cardiomegaly (cardiothoracic ratio: 0.74), mild pulmonary congestion and bilateral pleural effusion (Fig. 1) while the electrocardiography indicated a regular sinus rhythm with low QRS voltage. In addition, flat T waves in the limb leads and left precordial leads were observed (Fig. 2). The initial echocardiography revealed a large amount of pericardial effusion and diastolic collapse of the right ventricular anterior wall, which prompted us to perform emergency pericardiocentesis. The left ventricular wall motion was not properly assessed. Laboratory analyses of the pericardial fluid showed findings consistent with tuberculous pericarditis (RBC > 1,000/mm3, WBC: 6,300/mm3 [segmented neutrophils: 2%, lymphocytes: 88%], protein: 4720.7 mg/dL, adenosine deaminase: 110 IU/L).
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Fig. 1

Serial chest radiographs taken on days 1, 6 and 13 after admission. Severe cardiomegaly, pulmonary congestion and bilateral pleural effusion were initially noted, but these features gradually resolved over the ensuing 2 weeks

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Fig. 2

Serial electrocardiographs obtained on days 1, 3, 11, 17 and 80 after admission. The first electrocardiography revealed low voltage, flat T waves and tachycardia. On the third day of admission, only sinus tachycardia and low voltage remained. QS waves in V1–V4 and T wave inversion in the left precordial leads were noted. The RS progression in the precordial leads gradually improved during the second week after admission. The T wave inversion was the last symptom to improve, but normal T waves were found upon testing 80 days after admission

Despite pericardiocentesis, the patient’s condition remained unchanged. On the follow-up echocardiography a few hours after the pericardiocentesis, there was merely a small amount of pericardial fluid without diastolic collapse of the right ventricular wall. However, the echocardiography showed the typical features of takotsubo cardiomyopathy: akinesia at the apex and hyperkinesia at the base of the left ventricle (Fig. 3). For the following 3 or 4 days, he took a turn for the worse. He suffered from hypotension, intermittent fever, persistent tachycardia and severe general weakness, but his condition finally improved and he was discharged 2 weeks after admission.
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Fig. 3

The first echocardiography performed on the day of admission showed the typical findings of TLVAVS. Left panels: M-mode of the left ventricular apical portion, middle panels: M-mode of the left ventricular basal portion; right panel: 2-D pictures of the parasternal long axis view of the left ventricle. The upper panels show the diastolic phase while the lower panels show the systolic phase. The apical portion of the left ventricle is barely contracted whereas the basal portion is normally contracted

Serial chest radiographs revealed gradual resolution of pleural fluid, pulmonary congestion and pneumonic infiltration (Fig. 1). Follow-up electrocardiography during the first week of admission showed low voltage, QS progression through V2–V4 and T wave inversion in the left precordial leads. The RS progression and T wave inversion of the precordial leads gradually improved and this became normal 3 months after the patient presented at the hospital (Fig. 2). The echocardiographic abnormalities also gradually resolved over the next 3 weeks after admission. Cardiac magnetic resonance (MR) images were taken at 14 days after admission. The MRI study was performed using a 1.5-T cardiac MR imager (Intera CV release 10; Philips Medical Systems, Best, Netherlands) with a 5-channel cardiac coil around the chest. Breath-hold T2-weighted images were obtained using the electrocardiogram-triggered, turbo spin-echo sequence with the following parameters: echocardiographic time: 101 ms, flip angle: α 15°, field of view: 32–37 cm, slice thickness: 8 mm, matrix: 256 × 256. We also obtained the cine images using a segmented steady-state free precession pulse sequence (repetition time: 3 ms, echocardiographic time: 1.5 ms, flip angle: α 60°, field of view: 32–37 cm, slice thickness: 8 mm, no gap, matrix: 256 × 256). Whole-heart coronary MR angiography was obtained while the patient was free breathing with the use of a 3-dimensional, segmented steady-state free precession sequence with T2 preparation and radial k-space sampling (repetition time = 4.6 ms, echo time = 2.3 ms, flip angle = 90°, excitations per cardiac cycle = 20–50, SENSE factor = 2.0, a navigator gating (ED note: grated?) window of ± 2.5 mm, no drift correction, field of view = 280 × 280 × 120 mm, acquisition matrices = 256 × 256 × 80, reconstruction matrices = 512 × 512 × 160). After the acquisition of the coronary MR angiography, adenosine was infused at a dose of 0.14 mg/kg/min for up to 6 min. Within the last minute of infusion, the adenosine stress MR perfusion scan was performed to visualize the 3 short-axis geometries using 40 dynamic acquisitions. During the inspiratory phase of the second breath, a bolus of gadodiamide (Omniscan®; GE Healthcare, USA) was injected, with using a power injector (Spectris; Medrad, Indianola, PA), into an antecubital vein at a dose of 0.1 mmol per kilogram of body weight and an injection rate of 4 ml/s and this was followed by a 20 mL-saline flush. The stress perfusion MR images were obtained with a gradient-echo sequence by using SR-SSFP (2,744/1,372 ms: TR/TE, a 50° flip angle, a 256 × 256 matrix, an 8–10 mm section thickness, a 10–20 mm intersection gap). After 15 min, an identical MR perfusion scan at rest was continued to allow adequate clearance of the first bolus of the contrast agent. The delayed-enhancement imaging was performed 10 min after the rest perfusion imaging by using an inversion recovery fast gradient echocardiographic pulse sequence (repetition time: 4.6 ms, echocardiographic time: 1.5 ms, flip angle: α 15°, field of view: 32–37 cm, slice thickness: 8 mm, matrix: 256 × 256, inversion recovery time: 200–280 ms). We acquired 8–10 contiguous short-axis slices of the heart to cover the entire left heart and we acquired the images in the 2-chamber and 4-chamber views.

MRI showed normal coronary arteries and normal left ventricular function without any wall motion abnormalities. In addition, no delayed hyper enhancement was found on delayed-enhanced (DE)-MRI. The MR perfusion images at rest and stress showed normal perfusion, as is shown in Fig. 4. Coronary angiography was not performed.
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Fig. 4

MR perfusion imaging at rest (left panel) and at stress (right panel) at 14 days after admission. Any areas of decreased perfusion were not detected

Discussion

Transient left ventricular apical ballooning syndrome is an acute, self-limited cardiac disease that was first described in Japan and it has subsequently been recognized in other countries as well [14, 712]. Although there are many case reports of adult TLVABS, TLVABS is rare in children and so it is not well known to pediatricians. As such, the pediatric cases may have been missed or misdiagnosed as myocarditis.

This case had the typical clinical features of TLVABS such as the typical electrocardiographic and echocardiographic findings and very high levels of B-type natriuretic peptide. The clinical course was also compatible with TLVABS. The coronary arteries and myocardial perfusion, as assessed by MRI at 14 days, were normal. Thus the diagnosis of TLVAS seems highly likely despite the lack of coronary angiography.

However, it is not clear whether the original disease was TLVABS in association with pericarditis or TLVABS was induced by the pericardiocentesis. The initial echocardiography before pericardiocentesis did not properly assess the left ventricular wall motion.

The differential diagnosis of this case should include myopericarditis or dilated cardiomyopathy. However, myocarditis can be excluded because widespread ST elevation was not present, nor were the cardiac enzymes elevated. TLVABS in children can be misdiagnosed as dilated cardiomyopathy. Careful analysis of the left ventricular wall motion, detection of the typical electrocardiography changes, the presence of very high B-type natriuretic peptide levels and the rapidly and spontaneously resolving clinical course may help in making a correct diagnosis of TLVABS.

In a meta-analysis of 28 papers that encompassed 563 patients, 90.7% of the patients were women and the mean age ranged from 62 to 76 years [6]. TLVABS seems to very rarely occur in children. However, MEDLINE searches of the English medical literature using the terms of “TLVABS”, “takotsubo cardiomyopathy”, “broken heart syndrome” or “stress cardiomyopathy” revealed 12 cases of TLVABS patients under the age of 40. These cases are described in Table 1. Of these, there were only three cases with patients who were under the age of 18. The clinical manifestations of the above 12 cases with patients under the age of 40 were more or less the same as those of the older ones and females also predominated, as at least nine of the 12 cases were female (the gender of one case was not recorded). The most common presenting symptom was chest pain, followed by hypotension or collapse. Dyspnea did not seem to be as prevalent in the younger cases as that in the older cases. The laboratory findings and electrocardiographic changes of the younger cases were almost identical to those of the older cases.
Table 1

The reported cases of takotsubo cardiomyopathy in patients under the age of 40 years

Reference

Gender

Age

Triggering event

Underlying disease

Presenting symptoms

ECG findings

Cardiac enzymes

Catecholamine levels

Outcome

Maruyama et al. [4]

F

2

Withdrawal of buprenorphine and midazolam

VSD, s/p PDA division and PA banding, encephalopathy, pneumonia

Tachycardia, hypotension

ST elevation and T wave inversion in I, II, V4–6

Normal

NR

Recovered

Tsuchihashi et al. [11]

NR

10

NR

NR

NR

NR

NR

NR

NR

Ohwada et al. [5]

F

17

Hypoglycemia

Anorexia nervosa

Hypoglycemic coma

T wave inversion in II, III, aVF, V3–6

Elevated CK

Elevated norepinephrine

Recovered

Sasaki et al. [8]

F

22

Torsade de pointes

Long QT syndrome

Chest pain, vomiting, syncope

Torsade de pointes, QT prolongation, T wave inversion in II, aVL, V2–6

Normal

NR

Recovered

Wittstein et al. [13]

F

27

Tragic news

No

Chest pain, hypotension

NR

NR

NR

NR

Wittstein et al. [13]

F

32

Mother’s death

No

Chest pain

NR

NR

NR

NR

Jeon et al. [3]

F

29

Postpartum state

No

Chest pain, collapse

VF, ST elevation in II, III, aVF, V1–6

Elevated CK-MB and troponin-T

Normal

Recovered

Vultaggio et al. [12]

F

31

Anaphylactic reaction

No

Dyspnea, vomiting, hypotension

T wave inversion in I, aVL, V5–6

Normal

NR

Recovered

Denney et al. [1]

M

32

Torsade de pointes

Asthma, migraine, obesity

Chest pain

Torsade de pointes, VF, QT prolongation, T wave inversion in V2–6

Normal

NR

Recovered

Sharkey et al. [9]

F

32

Serious argument

Hypertension

Chest pain

ST elevation and T wave inversion

Normal

NR

Recovered

Takizawa et al. [10]

M

32

No

Pheochromocytoma

Chest pain

ST depression in III, aVF, V1

Normal

Elevated epinephrine, norepinephrine and dopamine

Recovered after adrenalectomy

Ramakrishna et al. [7]

F

35

Hepatectomy

Polycystic liver disease

VT, hypotension

ST elevation and T wave inversion in I, aVL

Elevated troponin-T

NR

Recovered

Abbreviations: NR not recorded, PA pulmonary artery, PDA persistent ductus arteriosus, VF ventricular fibrillation, VSD ventricular septal defect, VT ventricular tachycardia (Although the reference 7 includes three cases, the one confirmed case was included here)

Several studies have demonstrated that patients with takotsubo cardiomyopathy show no delayed hyper enhancement and transmural high signal intensity on the T2-weighted MR image involving the mid-anterior wall and apical segments and that match the distribution of hypokinesis [14, 15]. Interestingly, Joshi et al. [16] reported that patients who had follow-up MR imaging at 2–3 weeks showed normalization of the wall motion abnormalities associated with a significant reduction in the T2 signal intensity. That finding is consistent with the MRI result of this current case. Therefore, we suggest that T2-weigthed MR imaging may be a valuable approach for patients who are suspected of suffering with Takotsubo cardiomyopathy in the acute phase. In addition, cardiac MR imaging can be indicated with using DE-MR imaging for excluding acute myocardial infarction that mimics Takotsubo cardiomyopathy.

In summary, we report here on a case of TLVABS in a Korean child. To the best of our knowledge, this is the first case of TLVABS associated with pericarditis or pericardiocentesis. While TLVABS appears to rarely occur in children, it may not be as rare as was previously thought. We also reviewed the published cases of TLVABS in patients under the age of 40.

Copyright information

© Springer Science+Business Media, B.V. 2010