Cases reported "Heart Defects, Congenital"

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1/10. dissection in a right sided porcine-valved Dacron conduit.

    Seventeen years after corrective surgery for a double outlet right ventricle and pulmonary stenosis, a 30-year-old patient was admitted for reoperation for a critical stenosis of a porcine-valved Dacron conduit between the right ventricle and pulmonary artery. A residual ventricular septal defect was present. Induction of anaesthesia resulted in an inadequate pulmonary flow and uncontrollable ventricular arrhythmias. At autopsy the conduit showed a dissection between the conduit wall and the peel formation, leaving a residual lumen with a diameter of less than a third of the original. patients with a right sided bioprosthetic valved conduit must be evaluated regularly and operated before the stenosis becomes critical.
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ranking = 1
keywords = anaesthesia
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2/10. Anaesthesia in a child with sotos syndrome.

    sotos syndrome is a rare condition characterized by typical facies, early accelerated growth, large body size, developmental delay and congenital heart defects. Reports of anaesthetic management of these children are very rare. We report a case of general anaesthesia in a 2(1/2)-year-old boy with this condition, undergoing inguinal hernia repair. The child had a marked developmental delay, hypotonia and mitral regurgitation. The key points in the management of anaesthesia in sotos syndrome are discussed.
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ranking = 2
keywords = anaesthesia
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3/10. Epidural anaesthesia for labour and caesarean section in a parturient with a single ventricle and transposition of the great arteries.

    We describe a case of a 29-year-old parturient with a single ventricle and transposition of the great arteries who had lumbar epidural analgesia/anaesthesia with a local anaesthetic for labour, emergency Caesarean section and postoperative pain. Her outcome and that of her baby was successful. The anaesthetic techniques used in other parturients with similar congenital cardiac anomalies are reviewed.
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ranking = 5
keywords = anaesthesia
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4/10. 'Leopard' syndrome.

    'Leopard' syndrome is a rare inherited disorder associated with a high prevalence of cardiac abnormalities. General anaesthesia for dental treatment in a patient who had cardiomyopathy and bizarre electrocardiographic abnormalities associated with this syndrome is described. A thorough cardiac assessment is advised in a patient with multiple lentigines, although no clinical symptoms or signs may be found. Even if no cardiac abnormality is found before it is better to re-assess the patient, since abnormalities may develop later. The assessment should be repeated if any abnormality was detected before but without clinical significance, since the disease is progressive and may progress more rapidly in some patients than in others.
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ranking = 1
keywords = anaesthesia
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5/10. Hypoxaemia produced by an oesophageal stethoscope: a case report.

    A seven-month-old child with complex cyanotic heart disease desaturated dramatically following induction of anaesthesia. While a degree of hypoxaemia would have been acceptable in this infant, pulse oximetry detected an abrupt desaturation prompting the anaesthetist to consider other less common causes of cyanosis. This episode of desaturation subsided with the removal of a 12 french oesophageal stethoscope which had been inserted following induction. Further attempts to re-insert this oesophageal probe led to repeated episodes of desaturation. The most likely cause of this desaturation was a reduction in pulmonary blood flow due to compression by the oesophageal probe of an aorto-pulmonary collateral posterior to the oesophagus.
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ranking = 1
keywords = anaesthesia
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6/10. Sensitivity to diethyl-ester as a possible cause of repeated cardiac arrest.

    During induction of ether anaesthesia in a five year old girl with multiple cardiac defects (ASD, VSD and PS) cardiac arrest developed repeatedly. Radical surgery was subsequently performed uneventfully under morphine anaesthesia with cardiopulmonary bypass. ether appears to be the most likely factor accounting for the repeated arrests.
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ranking = 2
keywords = anaesthesia
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7/10. Low thoracic epidural anaesthesia for elective cholecystectomy in a patient with congenital heart disease and pulmonary hypertension.

    A 52-year-old male with pulmonary hypertension secondary to partial anomalous pulmonary venous return unassociated with atrial septal defect was given thoracic epidural anaesthesia for elective cholecystectomy. Partial anomalous pulmonary venous return is a rare congenital anomaly characterized by increased pulmonary blood flow which in severe cases results in pulmonary hypertension subjecting the right ventricle to strain. An epidural catheter was placed at the T11-T12 interspace and anaesthesia was established to the T4 dermatomal level with bupivacaine (180 mg) and lidocaine (100 mg). central venous pressure, pulmonary artery pressure, radial artery pressure, and cardiac output were monitored. There was minimal change in pulmonary arterial pressure, although there was a significant drop in systemic arterial pressure. Thoracic epidural block is recommended for upper abdominal surgery in clinical situations with pulmonary hypertension.
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ranking = 6
keywords = anaesthesia
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8/10. Auditory evoked responses and near infrared spectroscopy during cardiac arrest.

    We describe a patient who had a cardiac arrest during anaesthesia, in whom regional cerebral oxygen saturation was being measured by near infrared spectroscopy and the auditory evoked responses (AER) were being recorded. Both of these monitors provided useful information on cerebral oxygenation during cardiac arrest. Changes in the AER as the result of either reduced circulation or hypothermia are similar, and should these two situations occur simultaneously there could be difficulty in the interpretation of the AER.
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ranking = 1
keywords = anaesthesia
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9/10. Anaesthesia in a child with Patau's syndrome.

    A 5-year-old girl who had Patau's syndrome associated with double outlet right ventricle and pulmonary stenosis required general anaesthesia for the formation of a modified Blalock-Taussig shunt.
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ranking = 1
keywords = anaesthesia
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10/10. Anaesthetic managements for conjoined twins with complex cardiac anomalies.

    PURPOSE: To describe the perioperative assessment and anaesthetic management for surgical separation of three sets of conjoined twins with complex cardiac anomalies threatened with arterial desaturation and haemodynamic instability. CLINICAL FEATURES: Three sets of conjoined twins, one omphaloischiopagus, one omphalopagus, and one thoraco-omphalopagus, were considered for separation during the perinatal or infantile period. Preoperative functional evaluation including continuous pulse oximetry, capnography, and cardiac electrophysiological studies were considered to be as important as anatomical evaluation of the cardiac anomalies and cross-circulation by angiography in assessing the feasibility of surgical separation. Ipsilateral infusion of prostaglandin E1 and phenylephrine were applied to the cyanotic and healthy twins respectively, to restore arterial oxygenation intraoperatively and to avoid profound hypoxaemia. CONCLUSION: Surgical separation and anaesthesia should be well planned and rehearsed before clinical deterioration of the weaker twin. Aggressive pharmacological intervention and understanding of the cross-circulation pathophysiology is necessary to manage critical situations during surgical separation and in the postoperative period.
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ranking = 1
keywords = anaesthesia
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