Takotsubo Syndrome with Complete Heart Block and Cardiogenic Shock

Madhu Shukla; Jagdish Chander Mohan

doi:10.4103/jiae.jiae_3_23

INTERESTING CASE REPORT

Year : 2023 | Volume : 7 | Issue : 1 | Page : 59-61

Takotsubo Syndrome with Complete Heart Block and Cardiogenic Shock

Madhu Shukla, Jagdish Chander Mohan
Department of Cardiology, Institute of Heart and Vascular Diseases, Jaipur Golden Hospital, Rohini, Delhi, India

Date of Submission	10-Jan-2023
Date of Decision	20-Jan-2023
Date of Acceptance	26-Jan-2023
Date of Web Publication	30-Mar-2023

Correspondence Address:
Prof. Jagdish Chander Mohan
A51, Hauz Khas, New Delhi - 110 016
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_3_23

Abstract

This report describes a 62-year-old postmenopausal female patient who presented with cardiogenic shock, wide QRS complete heart block (CHB), left ventricular apical ballooning with severe systolic dysfunction, and normal coronary angiogram. She underwent permanent pacemaker implantation and showed a disappearance of apical dyskinesis and significant improvement in left ventricular function over the next 1 week. CHB was persistent at follow-up. Relevant literature with regard to this unique combination is discussed.

Keywords: Cardiogenic shock, complete heart block, Takotsubo syndrome

How to cite this article:
Shukla M, Mohan JC. Takotsubo Syndrome with Complete Heart Block and Cardiogenic Shock. J Indian Acad Echocardiogr Cardiovasc Imaging 2023;7:59-61

How to cite this URL:
Shukla M, Mohan JC. Takotsubo Syndrome with Complete Heart Block and Cardiogenic Shock. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2023 [cited 2023 Sep 27];7:59-61. Available from: https://jiaecho.org/text.asp?2023/7/1/59/373010

Introduction

Takotsubo syndrome (TTS) is a relatively benign entity resembling acute coronary syndrome with some definite markers of higher in-hospital mortality such as hemodynamic compromise and serious rhythm disorders. Complete heart block (CHB) with TTS has been reported to occur in 0.7%–2.2% of cases.^[1] A nationwide survey from the United States involving 56,431 TTS patients reported CHB prevalence of 0.7% with twice higher mortality and 55% rate of permanent pacemaker implantation which steadily climbed over the period of survey.^[2] A recent report debates the causal relationship between the two entities and proposes CHB as one of the physical triggers for TTS.^[3] Herein, we report a 62-year-old female who presented in cardiogenic shock, CHB, marked left ventricular (LV) apical ballooning, and severe systolic dysfunction that largely recovered over a period of 7 days. A permanent pacemaker was implanted on the 4^th day of presentation. Follow-up at 3 months showed no LV apical asynergy, mild systolic dysfunction, and reduced longitudinal strain but persistent CHB requiring 100% right ventricular pacing.

Case Report

This 62-year-old postmenopausal average-built nondiabetic female patient presented with acute dyspnea, extreme fatigue, sweating, and presyncope to the emergency department. Physical examination revealed a pulse rate of 35 beats/min, poor peripheral circulation, cold extremities, and supine blood pressure of 70/50 mmHg. There were no cardiac murmurs and jugular venous pressure was not elevated. A 12-lead electrocardiogram showed wide QRS (left bundle branch-like) CHB with an atrial rate of 82/min and ventricular rate of 35 beats/min. She underwent a quick two-dimensional echocardiography followed by temporary pacemaker implantation and resuscitation with intravenous fluids and low-dose noradrenaline. Her blood pressure remained 90/70 mmHg with oliguria and then slowly recovered over the next 3 days. Her admission troponin level was 0.9 ng/mL and the brain natriuretic peptide level was 1650 pg/mL. High-sensitivity C-reactive protein and D-dimer were not measured. 2D echocardiography at admission showed apical ballooning with dyskinesis and LV ejection fraction of 24% [Figure 1] and [Video 1]. Continuous wave Doppler interrogation of the mitral inflow showed intermittent diastolic and early systolic mitral regurgitation [Figure 2]. Acoustic speckle tracking showed holosystolic lengthening of the apical segments suggestive of dyskinesis [Figure 3] and the global longitudinal strain of the LV was -8.1%. Selective coronary angiography performed on day 4 of admission showed no abnormality [Figure 4]. As CHB persisted, the patient underwent dual-chamber rate-responsive pacemaker implantation on the 4^th day of admission. A repeat echocardiography on the 8^th day of admission showed disappearance of apical dyskinesis with an LV ejection fraction of 52% [Figure 5] and [Video 2]. The LV global longitudinal strain improved from − 8.1% to − 13.7% [Figure 6].

Figure 1: Left ventricular apical ballooning (shown by yellow arrows) seen in the systolic frames in the apical four-chamber view (panel A) and the long-axis view (panel B) at presentation. LA: Left atrium, LV: Left ventricle

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Figure 2: Mitral inflow pulsed-wave spectral Doppler recorded in the apical four-chamber view. The rhythm electrocardiogram at the bottom shows wide QRS rhythm with complete heart block. The arrows point to diastolic mitral regurgitation contributing to hemodynamic compromise

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Figure 3: Acoustic Speckle tracking showing systolic dyskinesis of the apical segments. The blue segments show segmental longitudinal lengthening

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Figure 4: Normal Coronary angiogram. Panel A shows left coronary angiogram in right anterior view and panel B shows right coronary angiogram in left anterior oblique view

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Figure 5: Apical four-chamber views in end-systole showing apical ballooning (panel A) and its disappearance after 3 days (panel B) pointed by yellow arrows. LA: Left atrium, LV: Left ventricle

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Figure 6: Left ventricular global longitudinal strain at presentation (panel A) and on day 4 (panel B). Global longitudinal strain increased from -8.1% to -13.7% and the apical segments recovered significantly

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[Additional file 1]

Video 1: Apical 4-chamber view at presentation. Note typical apical ballooning.

[Additional file 2]

Video 2: Apical 4-chamber view showing amelioration of apical dyskinesis on day 4.

The patient was discharged on 5^th day of admission. A follow-up at 3 months after pacemaker implantation showed nearly 100% right ventricular pacing (atrium sensed and ventricular paced). Repeat echocardiography revealed mild LV global hypokinesis (ejection fraction 46% and global longitudinal strain of -12%) possibly related to right ventricular pacing-induced dyssynchrony.

Discussion

Of late, sporadic cases of combined TTS and CHB have been reported in the literature.^{[4],[5],[6],[7],[8],[9],[10]} TTS is complicated by CHB in 0.5%–0.8% of cases in population-based data.^[2] Myocardial edema in TTS has been proposed to be the pathogenetic mechanism for the occurrence of CHB.^[8] Despite this potentially reversible causative mechanism, current literature reports permanent pacemaker implantation in over 75% of these cases indicating ambiguity in the therapeutic approach.^[3] There are no guidelines from expert groups^[9] and we also do not know the exact frequency and time course of restoration of atrioventricular conduction. Authors of a previous case discussion^[3] had raised three pertinent questions in this regard:

Is CHB a physiological stressor that triggers TTS?
Does TTS cause CHB?
Is the association coincidental and there is no causation?

A recent report shows TTS occurring in a patient with preexisting CHB of 1-year duration, probably the latter acting as a trigger.^[10] Another report mentions the restoration of intrinsic rhythm with improvement in segmental wall motion abnormality in such a case indicating a causal relationship.^[11] Our patient having persistent CHB at 3 months may suggest coincidence. It is difficult to tilt to one or the other side while answering the above three questions unless we have systematic prospective data from multiple centers or registries.

Conclusion

This case describes CHB and cardiogenic shock presenting in the context of TTS. The need for early permanent pacemakers in all such cases is debatable. Our patient had persistent CHB at 3-month follow-up justifying this approach in hindsight.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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