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INTERESTING CASES

Case History :

A 36 year old female presented with a history of exertional angina since three years. She was known to have cyanotic congenital heart disease since childhood. This was found to correspond to sustained ventricular tachycardia on exercise testing. Because of first and second-degree heart block, antiarrhythmics medication was contraindicated until a pacemaker had been inserted. An epicardial AV sequential pacemaker was used. And the patient was placed on amiodarone. Exertional VT was controlled, but she suffered side effects from the amiodarone. She is apparently well and is not on any medications.

On examination she was mildly cyanotic and clubbed. Her oxygen saturation was 90%, Pulse 76/minute and regular, BP 100/70 mmHg. JVP normal. Cardiac apex displaced laterally. On auscultation there was a Loud single second heart sound. Grade 4 ejection systolic murmur at the base not varying with respiration. Grade 2 high-pitched decrescendo murmur down the left sternal border.

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Investigation Reports :

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Discussion :

An epicardial pacemaker was inserted to permit antiarrhythmics therapy. Treatment with amiodarone was poorly tolerated. She eventually discontinued it, and her ventricular tachycardia has not recurred. Her investigations show a functionally single ventricle physiology with reasonable ventricular function. She has only mild pulmonary stenosis, which has led to the development of pulmonary hypertension, which in turn has limited her exercise capacity because of falling saturations. No standard therapy is available. She isn't a candidate for either traditional or transplant surgery with the possible exception of heart/lung transplantation. Hence only palliative therapy could be advised.

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