Online Clinical Case Study (June 2008)
The
content of the Office Cardiology Series is provided by:
Dr. LI Siu Lung, Steven
F.H.K.A.M. (Med), F.R.C.P. (Glasg), F.R.C.P. (Edin), F.R.C.P. (Lond), Specialist
in Cardiology
Dr. WONG Shou Pang, Alexander
F.R.C.P., F.H.K.A.M.(Med.), F.H.K.C.P., Specialist in Cardiology
臨床心臟科個案研究之內容誠蒙李少隆醫生及王壽鵬醫生提供。
A 42-year-old lady admitted for repeated syncope attacks
A 42-year-old lady was admitted because of repeated syncope attacks in the past few months. She had no coronary risk factors. The attacks were not associated with convulsions. There was no palpitation attack and she was in otherwise good health. Physical examination of her cardiovascular system and neurological system were unremarkable. Her blood pressure was normal but her echocardiogram showed marked concentric left ventricular hypertrophy with normal left ventricular systolic function. Her heart valves were normal on echocardiogram. This was her ECG:

Answers
| 1. |
What is the obvious conflict noted in the ECG in relation to the information given? |
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Despite the marked concentric left ventricular hypertrophy noted in the echocardiogram, the ECG showed no feature of left ventricular hypertrophy (LVH) and in fact, the ECG was somewhat in low voltage. |
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| 2. | What could be the diagnosis that can explain this phenomenon? |
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Echocardiographic demonstration of thick left ventricular walls with concomitant low voltage on ECG is characteristic of cardiac amyloidosis. On the other hand, hypertrophic obstructive cardiomyopathy (HOCM) and hypertensive heart disease usually show distinct changes of LVH on ECG. |
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| 3. | How would the diagnosis be confirmed? |
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Abdominal fat aspirate has been the single most useful diagnostic procedure with good sensitivity and safety. Biopsy of rectum, gingival, bone marrow, liver, kidney and various other tissues has also been used. If the abdominal fat aspirate is negative, endomyocardial biopsy may be helpful. |
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| 4. | What should be the treatment of this condition? |
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Treatment of cardiac amyloidosis is in general unsatisfactory and ineffective. Digoxin should be used with caution because these patients are particularly sensitive to digoxin and even normal doses may lead to serious arrhythmias due to selective binding of digoxin to amyloid fibrils in the myocardium. Similarly nifedipine may lead to exacerbation of congestive heart failure due to an enhanced negative inotropic effect. Careful use of low doses of diuretics and vasodilators may provide some symptomatic relief but hypotension could be a problem. For those with a conduction problem, permanent pacemaker implantation may prevent significant bradycardia. Cardiac transplantation has been performed but in general the results are inferior. |
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The figure shows equipment commonly used in dermatology practice.
| The
content of the Dermatology Series is provided by: Dr. CHAN Loi Yuen, Dr. TANG Yuk Ming, William & Dr. MAK Kam Har. Specialists in Dermatology & Venereology 皮膚科病例研究之內容誠蒙陳來源醫生、鄧旭明醫生及麥錦霞醫生提供。 |
Answers