Intravenous leiomyomatosis with intracardiac extension: first reported case in Australia.

Intravenous leiomyomatosis (IVL) with cardiac extension is a rare uterine tumour. We present an unusual case of uterine leiomyoma that progressed along the inferior vena cava into the right atrium. Complete one stage removal of the tumour was performed using cardiopulmonary bypass and circulatory arrest. The literature review reveals that this is the first reported case in Australia of IVL with intracardiac extension which was successfully removed with a single stage procedure.

Simplified cerebral protection using unilateral antegrade cerebral perfusion and moderate hypothermic circulatory arrest.

BACKGROUND: Antegrade cerebral perfusion is one of the most reliable methods of organ protection during hypothermic circulatory arrest for aortic arch surgery. We used a simplified antegrade cerebral perfusion technique with low mortality and morbidity. METHODS: Between January 2005 and August 2008, 21 patients underwent aortic arch surgery with unilateral antegrade selective cerebral perfusion through the brachiocephalic artery and moderate hypothermic circulatory arrest. The mean age for patients was 58.0+/-11.1 (27-82) years. Cardiopulmonary bypass was commenced and the ascending aorta was cross-clamped. Patients were cooled to 22-28 degrees C, whilst the proximal anastomosis was performed. The brachiochephalic artery was cannulated using a balloon tipped 15Fr catheter used for retrograde cardioplegia. Antegrade cerebral perfusion was established at the rate of 10 ml//kg/min. The perfusion pressure was controlled between 50 and 70 mm Hg whilst the distal anastomosis was completed. RESULTS: There were no operative deaths and no permanent neurological deficits. Four patients had temporary confusion. Mean antegrade cerebral perfusion time was 21.6+/-8.0 (12-48)min. Eight out of 20 patients had circulatory arrest at 28 degrees C and their mean circulatory arrest time was 22.8+/-4.7 (16-32)min. DISCUSSION: The mortality and neurological outcomes of aortic surgery using unilateral antegrade cerebral perfusion with moderate hypothermic circulatory arrest produced satisfactory results. Bilateral cannulation and deep hypothermia appear to be unnecessary in most cases. The coagulopathy from deep hypothermia is thereby avoided.

Mitral valve annular dilatation caused by left atrial myxoma.

We report a case of mitral valve annular dilatation caused by a large left atrial myxoma. A 69-year-old woman presented in pulmonary oedema. She was found to have a large left atrial myxoma prolapsing into the left ventricle in diastole causing severe functional mitral stenosis. At operation, the myxoma was completely excised from its attachment to the atrial septum. The mitral valve looked anatomically normal but the mitral annulus was dilated. The intraoperative Trans Oesophageal Echocardiogram (TOE) on weaning from cardiopulmonary bypass confirmed a dilated mitral annulus with moderate mitral regurgitation (MR). We elected not to place an annuloplasty ring in anticipation of improvement with postoperative remodelling. However, mitral regurgitation worsened after discharge becoming moderately severe and remains so after 1 year follow-up despite optimal medical treatment. This case suggests that annular dilatation can result from mechanical dilatation by a large left atrial myxoma. Intraoperative mitral valve annuloplasty should be considered in the presence of moderate MR as postoperative remodelling does not occur.

Ochronosis of the aortic valve.

Ochronosis is the bluish-black discolouration of connective tissue, including heart valves, joints, kidney and the skin. It is due to the deposition of homogentisic acid (HGA) commonly found in alkaptonuria. Ochronosis in the aortic valve is a rare occurrence and there is limited data available on the most appropriate choice of valve prosthesis in these patients. This case involves a 72-year-old male with symptomatic aortic stenosis and on echocardiogram a severe calcific trileaflet aortic stenosis with normal ejection fraction. Intraoperative aortic cannulation was routine and uncomplicated, and bluish-black discolouration of aortic valve was noted. Thorough decalcification was undertaken and a bioprosthetic valve was chosen in accordance with patient's age and preference. There were no complications post-operatively and the patient reported being well. Ochronosis affecting the aortic valve is a rare condition and there is limited data on the recurrence rate as well as the natural history of the disease. This case reports aims to provide data to facilitate further research to better understand the natural history of aortic valve ochronosis and rates of recurrence following bioprosthetic aortic valve replacement (AVR).

A simple technique for aortic valve replacement in patients with a patent left internal mammary artery bypass graft.

Aortic valve replacement in patients with a patent left internal mammary artery graft is often a challenge because of the difficulties with dissection of the left internal mammary artery and optimum myocardial protection. We describe a simple technique of aortic valve replacement with a beating heart and continuous coronary perfusion for this difficult group of patients.

Fatty infiltration of an aortic valve.

Adipose tissue is a normal constituent of the heart, but not a normal anatomic finding of cardiac valves. Fatty infiltration of the aortic valve is rare, with unknown significance on valve function. We report a case of fatty infiltration and replacement of the spongiosa layer in an incompetent aortic valve. The mechanism of fat infiltration is unknown, but may be explained by differentiation of preexisting valve interstitial cells secondary to valvular injury.

Management of life-threatening subcutaneous emphysema using subcutaneous penrose drains and colostomy bags.

Subcutaneous emphysema is a common complication post cardiothoracic surgery. Severe subcutaneous emphysema may cause respiratory obstruction and sometimes tracheostomy or intubation is required. We report a case of massive subcutaneous emphysema following aortic valve replacement. It was not relieved with initial bilateral chest tubes, but the subcutaneous Penrose drains produced a dramatic improvement and provided effective decompression of the subcutaneous emphysema. We also describe the colostomy bags, which covered the drains. These were useful for keeping the area sterile and measuring the amount of air through the individual drains.

Aortic valve lipoma.

Cardiac lipoma (especially on the aortic valve) is extremely rare. We report a patient suffering from shortness of breath, chest pain, and recent presyncopal episodes who was found to have a mass on the aortic valve with mild aortic regurgitation. The patient had an uneventful aortic valve replacement.

Mitral valve repair on the beating perfused heart.

It is difficult to assess the success of mitral valve repair in the arrested heart. Various techniques have been described. Transesophageal echocardiogram (TEE) provides excellent two-dimensional evaluation of the repair, but three-dimensional anatomic characteristics are limited. We describe a simple technique for performing mitral valve repair on the beating heart. This allows accurate evaluation of valvular competence and three-dimensional anatomic characteristics prior to closure of the atriotomy.