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JCHS-IQ-02-2022 High Risk of Sudden Cardiac Death Managed by Cardiac Implantable Electronic Device

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Vol 7(2) (2022) 56-57 | jchs-medicine.uitm.edu.my | eISSN 0127-984X https://doi.org/10.24191/jchs.v7i2.12917

56

Case Presentation

A 28-year-old lady with background medical history of type 2 diabetes mellitus since 2019 (HbA1c: 6.6%) presented with recurrent episodes of syncope associated with palpitations. Her father had passed away at 36 years of age as a result of sudden death and the brother had similar heart condition. She had no symptoms of heart failure. Clinically well and euthyroid with blood pressure of 132/76 mmHg, pulse rate 82 beats per minute and oxygen saturation of 98%.

Cardiovascular and neurological examination were unremarkable. Serum electrolytes were within the normal range.

Transthoracic echocardiogram revealed Left Ventricular Ejection Fraction of 60% and maximum left ventricle wall thickness of 22mm. Coronary angiogram revealed 30% disease at mid left anterior descending artery, mild disease at both left circumflex and right coronary artery.

Figure 1 Cardiac device seen on chest X-ray

JCHS-IQ-02-2022

High Risk of Sudden Cardiac Death Managed by Cardiac Implantable Electronic Device

Huzairi Sani

1,2,3

, Ahmad Bakhtiar

1,2

, Muhammad Hanis

1,2

, Khairul Shafiq

1,2

1 Cardiology Unit, Department of Internal Medicine, Faculty of Medicine, Universiti Teknologi MARA(UiTM), Sungai Buloh, Selangor, Malaysia 2 Cardiac Vascular and Lung Research Institute (CaVaLRI), Pusat Perubatan Universiti Teknologi MARA (UiTM), Sungai Buloh, Selangor, Malaysia

3 Integrative Pharmacogenomics Institute (iPROMISE), UiTM Puncak Alam Campus, Puncak Alam, Selangor, Malaysia

(2)

Vol 7(2) (2022) 56-57 | jchs-medicine.uitm.edu.my | eISSN 0127-984X https://doi.org/10.24191/jchs.v7i2.12917

57 Question:

What is seen on this chest X-ray?

A. Biventricular pacemaker

B. Cardiac resynchronization therapy defibrillator (CRT-D) C. Dual chamber implantable cardioverter-defibrillator (ICD) D. Permanent pacemaker

E. Temporary intravenous pacemaker

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Vol 7(2) (2022) 58-59 | jchs-medicine.uitm.edu.my | eISSN 0127-984X

https://doi.org/10.24191/jchs.v7i2.12917

58 ANSWER TO JCHS-IQ-02-2022

Answer: C Discussion

The chest X-ray reveals implantable cardioverter-defibrillator (ICD)-pacemaker combination with two leads projected at the right atrium and ventricle. A pulse generator placed subcutaneously in the left infraclavicular area. There are shock coils seen as thickened radio-opaque structures on the lead. Automatic implantable cardioverter defibrillators function as patient monitoring and therapy in the event of life-threatening ventricular tachyarrhythmias.

Her Cardiac MRI reported increased left ventricular wall thickness (IVSDd 18mm, LPWDd 10mm, basal septal 22mm, mid septal 17mm, basal inferior 14mm, mid inferior 19mm, apical inferior 11mm, basal anterior 10mm and basal lateral 7mm). The maximum left ventricular thickness is required as one of the parameters in clinical risk prediction for sudden cardiac death. There was no systolic anterior motion of the mitral valve seen. She was diagnosed with Hypertrophic cardiomyopathy (HCM) with high sudden cardiac death risk score of 7.36% [1]. The indication of ICD in this case is for primary prevention from sudden cardiac death due to ventricular tachyarrhythmia [2, 3].

HCM is characterized by left ventricular hypertrophy of various morphologies. The presentation varies from asymptomatic, heart failure, chest pain or arrythmias to sudden death in the young depending on the site and extent of cardiac hypertrophy. The cardiac hypertrophy is caused by mutations in one of several sarcomere genes which encode components of the contractile apparatus.

Learning Points

• The indication and evidence base for cardiac implantable electronic devices continues to expand providing a better clinical outcome.

• ICDs comprises of a single lead with one or two shock coils which are radiopaque on chest x-ray.

Conflict of Interest Authors declare none.

JCHS-IQ-02-2022

High Risk of Sudden Cardiac Death Managed by Cardiac Implantable Electronic Device

Huzairi Sani

1,2,3

, Ahmad Bakhtiar

1,2

, Muhammad Hanis

1,2

, Khairul Shafiq

1,2

1 Cardiology Unit, Department of Internal Medicine, Faculty of Medicine, Universiti Teknologi MARA(UiTM), Sungai Buloh, Selangor, Malaysia 2 Cardiac Vascular and Lung Research Institute (CaVaLRI), Pusat Perubatan Universiti Teknologi MARA (UiTM), Sungai Buloh, Selangor, Malaysia

3 Integrative Pharmacogenomics Institute (iPROMISE), UiTM Puncak Alam Campus, Puncak Alam, Selangor, Malaysia

(4)

Vol 7(2) (2022) 58-59 | jchs-medicine.uitm.edu.my | eISSN 0127-984X

https://doi.org/10.24191/jchs.v7i2.12917 59

REFERENCES

1. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: The Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J, 2014. 35(39): 2733-79.

2. Maron, B.J., et al., Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. N Engl J Med, 2000. 342(6): 365-73.

3. Maron, B.J., et al., Prevention of sudden cardiac death with implantable cardioverter-defibrillators in children and adolescents with hypertrophic cardiomyopathy. J Am Coll Cardiol, 2013. 61(14): 1527-35.

Corresponding author:

Huzairi Sani,

Department of Internal Medicine, Faculty of Medicine,

Universiti Teknologi MARA (UiTM), Jalan Hospital, 47000 Sungai Buloh, Selangor, Malaysia.

Email: [email protected]

Received 25th March 2021

Received in revised form 7th June 2021

Accepted 7th June 2021

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