Two cases of juvenile hypertrophic cardiomyopathy presenting with marked bi-atrial enlargement on standard 12-lead electrocardiogram

doi:10.4236/ojim.2012.21007

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Open Journal of Internal Medicine, 2012, 2, 31-33 OJIM

http://dx.doi.org/10.4236/ojim.2012.21007 Published Online March 2012 (http://www.SciRP.org/journal/ojim/)

Two cases of juvenile hypertrophic cardiomyopathy

presenting with marked bi-atrial enlargement on

standard 12-lead electrocardiogram*

Hajime Kataoka

Division of Internal Medicine, Nishida Hospital, Oita, Japan

Email: [email protected]

Received 19 September 2011; revised 8 November 2011; accepted 18 November 2011

ABSTRACT

This report describes two cases of juvenile hypertro-

phic cardiomyopathy (HCM) in which prominent

bi-atrial enlargement was observed on standard 12-

lead electrocardiogram, suggesting inherent predis-

position to extreme enlargement and/or hypertrophy

may exist in atrial myocardium in juvenile HCM.

Comparative study between juvenile and adult HCM

patients using a large sample size is required to con-

firm this hypothesis.

Keywords: Hypertrophic Cardiomyopathy;

Electrocardiogram; P-Wave; Atrial Enlargement

1. INTRODUCTION

Previous reports [1,2] indicate that an inverse relation

exists between left ventricular hypertrophy and age, and

suggest that left ventricular hypertrophy often progresses

in juvenile hypertrophic cardiomyopathy (HCM). Pro-

gression of atrial enlargement in relation to age has not

yet been examined. Here, this report describes two cases

of juvenile HCM in which prominent bi-atrial enlarge-

ment was observed on 12-lead electrocardiograms (ECG).

In this report, the ECGs were interpreted following the

criteria described by Savage et al. [3]; notably, the left

atrial enlargement was diagnosed if the product of the

depth and duration of the negative portion of the P-wave

in lead V1 was greater than −0.03 mV-sec [4]. The right

atrial enlargement was diagnosed if there were peaked

P-waves in leads II and III or V1,2 ≥ 2.5 mV in amplitude.

2. CASE PRESENTATION

Case No. 1. A 19-year-old female had been diagnosed as

HCH since the age of 11 years and was taking verapamil.

Follow-up clinical, chest x-ray, standard 12-lead ECG

and echo-Doppler studies were obtained periodically.

Gradual bi-atrial enlargement was observed on serial

standard 12-lead ECGs. In parallel with this, her New

York Heart Association functional class deteriorated

from class I at initial visits to class III at recent visits.

The 12-lead ECG taken at the age of 19 years (Figure 1)

showed a regular sinus rhythm with prominent right

(amplitude of the P initial force V2 lead = 0.5 mV) and

left atrial (Morris index of the P terminal force V1 = −0.4

mm-sec) enlargement, incomplete right bundle branch

block and ST-T changes. Echo-Doppler study taken

around this period disclosed asymmetric septal hyper-

trophy (20 mm) and hypertrophic right ventricular free

wall (10 mm). Left cavity size was normal (end-diastolic

*Disclosures: The author has no conflicts of interest to disclose. Figure 1. Standard 12-lead ECG of Case No. 1.

OPEN ACCESS

H. Kataoka / Open Journal of Internal Medicine 2 (2012) 31-33

volume of 83 cc), but the ejection fraction was slightly

depressed (51%). Moderate enlargement of both atria and

mild tricuspid regurgitation were noted. Rest thallium-

201 myocardial scintigram showed marked right ven-

tricular hypertrophy and visualization of the right atrial

appendage. Addition of diuretics slightly improved her

clinical condition.

Case No. 2. An 18-year-old male had been diagnosed

as HCM since the age of 5 years. At the age of 13 years,

he developed complete atrio-ventricular block and un-

derwent permanent VVI pacemaker therapy. Serial stan-

dard 12-lead ECGs before (a) and after pacemaker ther-

apy (b and c) are shown in Figure 2. At the 3-year fol-

low-up, 12-lead ECG demonstrated gradual enlargement

of the right (amplitude of the P initial force V1 lead =

0.35 mV) and left atrial (Morris index of the P terminal

force V1 = −0.4 mm-sec) enlargement (Figure 2(b)). In

parallel with P-wave abnormalities, progressive heart

failure developed and he was admitted twice during this

follow-up period. Echo-Doppler study during the conva-

lescent phase demonstrated asymmetric septal hypertro-

phy (16 mm) with a slightly dilated (end-diastolic vol-

ume of 118 cc) and moderately hypokinetic (ejection

fraction of 45%) left ventricle. Both the left and right

atria were moderately enlarged. Slight mitral regurgita-

tion and moderate tricuspid regurgitation were detected

on Doppler echocardiography. Rest thallium-201 myo-

cardial imaging showed a moderate degree of isotopic

uptake in the right ventricle. Medical therapy was not

sufficient to improve his clinical condition. Because

atrio-ventricular uncoupling due to VVI pacing was con-

sidered to be a major contributory factor to congestive

heart failure, VVI pacing was switched to dual-chamber

pacing, resulting in an improved clinical condition and

decrease of the bilateral atrial enlargement on 12-lead

ECG (Figur e 2(c)).

3. DISCUSSION

No previous report has specifically addressed the relation

between atrial abnormalities and age in HCM patients.

This case presentation of two juvenile HCM patients

seems to be important because it was suggested that,

beside from the hemodynamic overloading on the right

and left atria as the main cause, such ECG findings of

prominent atrial enlargement may be induced by factor

responsible for the body growth and development char-

acteristics of childhood and adolescence. Previous re-

ports in the literature on adult HCM patients [3,5-8] have

not described HCM patients with prominent atrial enlar-

gement on ECG as in the cases reported here. In con-

secutive 24 adult HCM patients (age, 36 - 80 years; 16

males) that the author has experienced (Table 1), left

atrial enlargement defined by Morris index [4] was fre-

quent, but the magnitude of the P-wave abnormality was

minimal when compared to the two juvenile HCM pa-

tients described in this report. It may be that adult HCM

patients usually develop atrial fibrillation [9,10] before

reaching such prominent bi-atrial enlargement on ECG.

Comparative study between juvenile and adult HCM

patients using a large sample size is required to confirm

the possibility of inherent predisposition to extreme atrial

enlargement/hypertrophy causing prominent P-waves on

ECGs in juvenile HCM patients.

Table 1. Summary of ECG findings in 24 adult HCM patients.

Sinus rhythm without history

of heart failure (N = 12)

Paroxismal atrial fibrillation and/or

history of heart failure* (N = 12) Total (N = 24)

Left atrial enlargement 1 (8%) 6 (50%) 7 (29%)

Morris index (mV-sec) −0.12 −0.07 ± 0.03 (−0.04 - −0.12) −0.08 ± 0.03 (−0.04 - −0.12)

Amplitude (mV) −1.5 −1.17 ± 0.41 (−1 - −2) −1.21 ± 0.39 (−1 - −2)

Duration (sec) 0.08 0.06 ± 0.02 (0.04 - 0.08) 0.06 ± 0.02 (0.04 - 0.08)

Right atrial enlargement 0 0 0

Repolarization abnormalities (ST-T) 10 (83%) 11 (92%) 21 (88%)

Left ventricular hypertrophy 7 (58%) 5 (42%) 12 (50%)

Right ventricular hypertrophy 2 (17%) 0 2 (8%)

Abnormal Q waves 1 (8%) 3 (33%) 5 (21%)

Conduction disturbance

Left bundle branch block 0 2 (17%) 2 (8%)

Left bundle branch block 1 (8%) 2 (17%) 3 (13%)

* = ECG recording during sinus rhythm.

H. Kataoka / Open Journal of Internal Medicine 2 (2012) 31-33 33

Figure 2. Standard 12-lead ECG of Case No. 2.

4. CONCLUSION

Based on the ECG findings, this case report suggested

that inherent predisposition to extreme enlargement and/

or hypertrophy may exist in atrial myocardium in juve-

nile HCM patients.

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