Clinical Review & Education

JAMA Cardiology Clinical Challenge

Tombstone Pattern Electrocardiogram in a Young Woman

Yun Chen, MD; Ping Ge, MD; Xiao-Feng Zhuang, MD, PhD

Figure 1. Initial electrocardiogram demonstrating sinus tachycardia with

ST-segment elevations of 1.0 mm in lead I; 2.0 mm in leads II, III, and the
augmented vector foot (aVF); and 2.0 to 5.0 mm in the precordial leads.
Widened QRS complexes and low-voltage complexes in the limb leads were
observed. aVR indicates augmented vector right.

A woman in her mid-20s with no relevant medical history presented to the emergency de-
partment with a 2-day history of fever, chest pain, and exertional dyspnea. An initial elec- WHAT WOULD YOU DO NEXT?
trocardiogram (ECG) showed sinus tachycardia, QRS widening (130 milliseconds), low-
voltage complexes in the limb leads, and tombstonelike convex ST-segment elevation (STE) A. Emergent coronary angiography
in most leads, particularly V1 through V6 (Figure 1). Transient second-degree atrioventric-
ular block was observed on ambulatory ECG monitoring. Her blood pressure was 81/50 mm
B. Cardiac magnetic resonance
Hg. The N-terminal pro–brain-type natriuretic peptide was 10 099 pg/mL (to convert to ng/L,
imaging
multiply by 1; reference value, <150) and the troponin I level was 22 ng/mL (to convert to
μg/L, multiply by 1; reference value, <0.016). No obstructive stenosis was seen on coro-
nary computed tomography angiography. Transthoracic echocardiography revealed sym- C. Myocardial positron emission
metric left ventricular (LV) wall thickening (septal thickness, 16 mm) and reduced LV sys- tomography
tolic function with an ejection fraction of 35%.
D. Endomyocardial biopsy

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Diagnosis V3 (Figure 2B). Concomitantly, repeat ECG revealed that both LV wall
Fulminant myocarditis thickness and LV ejection fraction were restored to the normal range.
Subsequent cardiac magnetic imaging showed no late-gadolinium
What to Do Next enhancement.
D. Endomyocardial biopsy FM represents the most acute and life-threatening manifesta-
tion of myocarditis, characterized by sudden onset and rapid clini-
Discussion cal deterioration, with left and/or right ventricular dysfunction, re-
The diagnosis of fulminant myocarditis (FM) was confirmed subse- fractory ventricular arrhythmias, and the need for pharmacological
quently by endomyocardial biopsy, which demonstrated extensive and/or mechanical circulatory support.1 In this patient, tombstone-
and diffuse lymphocytic infiltration, severe interstitial edema, and like STE in extensive leads was indicative of severe myocardial in-
slightly myocyte necrosis (Figure 2A). Due to cardiogenic shock, an jury. This is more commonly seen in ST-elevation myocardial infarc-
intra-aortic balloon pump was implemented soon after admission, tion, but a diagnostic hallmark in FM is that there is no ST-segment
combined with glucocorticoids and intravenous immunoglobulins. depression in the reciprocal leads as would be seen in ST-elevation
On the sixth day after admission, the intra-aortic balloon pump was myocardial infarction. Additionally, ST segments are elevated in lead
removed, and a repeat ECG showed that the STE had decreased augmented vector right in FM, while they are usually depressed in
nearly to baseline, with diffuse T-wave inversion and Q waves in V1 to acute pericarditis.2 Lead augmented vector right has also been char-

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 Clinical Review & Education JAMA Cardiology Clinical Challenge

A Myocardial biopsy B ECG results

Figure 2. Myocardial biopsy and

Hospitalization 5y Hospitalization 5y
day 6 after discharge day 6 after discharge electrocardiogram (ECG) results.
A, The pathologic findings included
I V1 extensive and diffuse lymphocytic
infiltration, severe interstitial edema,
and mild myocyte necrosis. B, On day
II V2 6 following admission, ST-segment
elevation had decreased nearly to
III V3 baseline, with T-wave inversion in
most leads and Q waves in V1 to V3.
The 5-year follow-up ECG showed
aVR V4 sinus rhythm with left anterior
fascicular block; flat T waves in leads
II, III, and augmented vector foot
aVL V5
(AVF); and poor R-wave progression
in leads V1 to V3. aVL indicates
aVF V6 augmented vector left;
aVR, augmented vector right.

acterized in pericarditis or myocarditis by elevation of the PR inter- tis, which ruled out giant cell myocarditis and eosinophilic myocar-
val. However, this change is often subtle in FM.2 ditis and helped to guide an appropriately immunosuppressive
The increased LV wall thickness in FM is due to the intense in- treatment regimen. After effective immunotherapy, the myocar-
flammatory response seen on endomyocardial biopsy. Interstitial dial thickness recovered, the relevant ECG indicators improved, and
edema contributes not only to the wall thickening, but also to the cardiac magnetic resonance imaging also suggested resolution of the
decreased ventricular contractility in this disorder.3 Therefore, the myocardial edema.
low-voltage complexes, QRS widening, and transient second-
degree atrioventricular block in this patient were considered re- Patient Outcome
lated to myocardial edema and inflammation, in line with the myo- At 5-year follow-up after discharge, the patient felt well, and echo-
cardial pathology. A QRS interval greater than 120 milliseconds on cardiography revealed normal LV size, wall thickness, and ejection
ECG emerged as an adverse prognostic factor associated with the fraction. The ECG demonstrated sinus rhythm with left anterior fas-
long-term outcome of patients with FM.4 The patient was con- cicular blocks; flat T waves in leads II, III, and augmented vector foot,
firmed on endomyocardial biopsy to have lymphocytic myocardi- and poor R-wave progression in leads V1 to V3 (Figure 2B).

ARTICLE INFORMATION Published Online: August 21, 2024. characteristics in patients with fulminant
Author Affiliations: Department of Cardiology, the doi:10.1001/jamacardio.2024.2537 myocarditis. J Arrhythm. 2022;38(5):763-771.
First Branch, the First Affiliated Hospital of Conflict of Interest Disclosures: None reported. doi:10.1002/joa3.12751
Chongqing Medical University, Chongqing, China Additional Contributions: We thank the patient for 3. Felker GM, Boehmer JP, Hruban RH, et al.
(Chen, Ge); Heart Failure Care Unit, Heart Failure granting permission to publish this information. Echocardiographic findings in fulminant and acute
Center, Fuwai Hospital, Chinese Academy of myocarditis. J Am Coll Cardiol. 2000;36(1):227-232.
Medical Sciences, National Center for REFERENCES doi:10.1016/S0735-1097(00)00690-2
Cardiovascular Diseases, Beijing, China (Zhuang). 4. Ammirati E, Veronese G, Brambatti M, et al.
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Corresponding Author: Xiao-Feng Zhuang, MD, Fulminant myocarditis: haracteristics, treatment, Fulminant versus acute nonfulminant myocarditis in
PhD, Heart Failure Care Unit, Heart Failure Center, and outcomes. Anatol J Cardiol. 2018;19(4):279-286. patients with left ventricular systolic dysfunction.
Fuwai Hospital, Chinese Academy of Medical doi:10.14744/AnatolJCardiol.2017.8170 J Am Coll Cardiol. 2019;74(3):299-311. doi:10.1016/
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Diseases, 167 Beilishi Rd, Xicheng District, Beijing 2. Itoh T, Kobayashi T, Oshikiri Y, Arakawa Y, Satoh
100037, China ([email protected]). M, Morino Y. Clinical and electrocardiographic

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