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Case History :

19 year old female. As an infant, had a Blalock-Hanlon atrial septectomy. At age 1, had a Mustard operation and a patch repair of a VSD. At age 9, had a systemic tricuspid valve annuloplasty. After surgery, severe symptomatic systemic right ventricular dysfunction as well as rapid atrial arrhythmias.
Amiodarone started at age 9.
Now paroxysmal episodes of tachycardia occurring every two weeks for 10 minutes or so associated with lightheadedness and some dyspnea.
She can only manage two blocks on the flat and one floor vertically before becoming fatigued.
Current medications include amiodarone 400 mg daily, enalapril 10 mg BID, diagoxin 0.125 mg daily.
Appeared well.
Not cyanotic.
BP 120/50 mmHg. Rhythm regular at 70/minute.
JVP normal.
3+ left parasternal lift (RV).
Palpable second sound.
Grade 2 blowing pansystolic murmur at LLSB.
Grade 2 low pitched diastolic murmur LLSB.

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Investigation Reports :

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Discussion :

This 19 year old female has a "complex" Mustard state since she had both a Mustard repair and patch repair of VSD as an infant. Tricuspid regurgitation was an ongoing problem during childhood. By age 9, she had a tricuspid valve annuloplasty and revision of a VSD patch leak. Her postoperative course was complicated by symptomatic right ventricular failure and frequent atrial tachyarrhythmias. She has been on amiodarone for ten years. Her exercise capacity is poor and has been gradually worsening. Rhythm control has been reasonably good.

Her physical findings are typical of a Mustard repair except that she has a loud murmur of systemic tricuspid regurgitation and a softer murmur of systemic tricuspid stenosis. Her chest X-ray shows quite marked cardiomegaly with apparent right ventricular and right atrial dilation. Her echo shows fairly typical grade 3 systemic right ventricular dysfunction. Tricuspid regurgitation is severe and tricuspid stenosis is present. Heart cath shows pulmonary hypertension due to tricuspid stenosis and regurgitation. After wrestling with management options, we have elected to band her pulmonary artery to prepare her for a switch conversion of transposition. It is not clear how long her pulmonary left ventricle should be "trained" prior to arterial switch and what indices should be used to assess adequate training e.g. left ventricular mass, wall thickness. We will probably wait at least 6 months, maybe a year before undertaking the second stage.