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1/6. Undiagnosed cardiomyopathy in a neonate: significance of low oxygen saturation during anaesthesia.

    A case study is described of a 7-day-old full term baby with bilateral congenital cataracts who underwent surgical removal of both cataracts 2 days apart. Problems with oxygen saturation during and after the first anaesthetic prompted further investigation that revealed a non-obstructive hypertrophic cardiomyopathy. The significance and possible causes of low oxygen saturation in a previously healthy neonate during anaesthesia are discussed. The likely diagnosis of Sengers syndrome, and the evaluation of asymptomatic babies with cardiac pathology are discussed.
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2/6. Hypertrophic cardiomyopathy and caesarean section: intraoperative use of transthoracic echocardiography.

    A pregnant woman with severe hypertrophic cardiomyopathy was monitored with transthoracic echocardiography to guide management of fluids and vasopressors during elective caesarean section. After insertion of intravenous, arterial and central venous cannulae, a transthoracic echocardiogram was performed noting left ventricular cavity size and contractility, systolic anterior motion of the mitral valve and left ventricular outflow tract obstruction. Following combined spinal-epidural anaesthesia, serial examinations were made intraoperatively. Short-lived haemodynamic instability ensued just before the incision and with administration of oxytocin. Her postoperative course was complicated by post-partum haemorrhage requiring radiological intervention but she ultimately recovered fully. Maintaining preload to avoid worsening of left ventricular outflow tract obstruction is essential in these patients. A transthoracic echocardiogram is easily performed, of low risk and provides more useful information than a pulmonary artery catheter.
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keywords = anaesthesia
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3/6. Pulmonary oedema in two parturients with hypertrophic obstructive cardiomyopathy (HOCM).

    Two patients with hypertrophic obstructive cardiomyopathy (HOCM) presented for delivery. The first had a repeat Caesarean section with general anaesthesia and the second gave birth vaginally with epidural analgesia. Both patients developed pulmonary oedema in the peripartum period. These cases highlight the delicate fluid requirements of the pregnant patient with HOCM. The fluid management of the parturient is discussed with particular emphasis on the pathophysiology of HOCM. The indications for invasive monitoring are presented.
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keywords = anaesthesia
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4/6. Anaesthesia for a patient with Friedreich's ataxia and cardiomyopathy.

    Friedreich's ataxia is an inherited neuromuscular disorder often associated with significant cardiac disease. We report a case of Friedreich's ataxia in a 13-year-old girl with ulcerative colitis and hypertrophic cardiomyopathy who was successfully managed for subtotal colectomy with general anaesthesia and epidural narcotic. Anaesthetic considerations included the maintenance of fluid volume and stable cardiovascular variables in the intra- and postoperative periods.
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keywords = anaesthesia
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5/6. The use of halothane in a patient with asymmetrical septal hypertrophy: a case report.

    An elderly patient with demonstrated asymmetrical intraventricular septal hypertrophy and ventriculo-aortic pressure gradient was anaesthetized with nitrous oxide with oxygen, narcotic, and muscle relaxant for abdominal surgery. In addition to the cardiovascular variables customarily monitored, a systolic time interval (STI) measured from the Q wave of the ECG to the foot of the radial pulse (the QF interval) was calculated in milliseconds beat-to-beat by a computer. With anaesthesia, and particularly following the beginning of operation, the QF interval lengthened as an indication of either decreased cardiac inotropy or increased pressure gradient across the aortic outflow tract. When halothane 0.25 per cent was added to the anaesthetic mixture, the QF interval shortened by about 20 milliseconds without an observed change in direct arterial pressure. Since halothane is a cardiac depressant and normally lengthens the STI, it apparently relaxed the muscular stenosis of the ventricular outflow tract and reduced the pressure gradient and, subsequently, the QF interval. By measuring cardiovascular function with this STI, the beneficial action of cardiac depression from low-dose halothane was observed, which would have escaped detection by common monitoring indices.
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keywords = anaesthesia
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6/6. Use of esmolol in the postbypass management of hypertrophic obstructive cardiomyopathy.

    In patients suffering from hypertrophic obstructive cardiomyopathy (HOCM), any catecholamine release during anaesthesia may aggravate the severity of the outflow tract obstruction and compromise cardiac output. In this event the situation may be improved by beta block. Esmolol, an ultra-short-acting beta-blocker (half-life 9 min) appears to be a suitable agent for this purpose. We describe its use in the perioperative management of a patient who underwent surgical correction of HOCM.
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keywords = anaesthesia
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