1/2. Anaesthetic management of a patient with erythropoietic protoporphyria for ventricular septal defect closure.Erythropoietic protoporphyria (EPP) is due to a deficiency in ferrochelatase required for haem synthesis. We describe the anaesthetic management of a seven-year-old with EPP undergoing closure of a haemodynamically significant ventricular septal defect. Photosensitivity in EPP patients is triggered at wavelengths near 400 nm and light-excited porphyrins generate free radicals and singlet oxygen that lead to erythrocyte deformity and haemolysis. Stimuli that could trigger a porphyric crisis during anaesthesia and surgery were reduced by avoiding exposure to the sensitive 400 nm spectrum and using light sources covered with yellow acrylate filters in the operating room. Anaesthetic agents not previously associated with porphyric crisis were chosen. Whole blood priming of the extracorporeal circuit was performed to ensure adequate haemoglobin concentrations during the perioperative period.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
2/2. Pulmonary hypertension and pregnancy: a series of eight cases.This is the report of a series of eight patients with pulmonary hypertension (primary and secondary) who delivered at the McMaster University Medical Centre between 1978 and 1987. Seven of the eight patients delivered vaginally and had a successful outcome. The eighth patient was admitted as an emergency and died shortly after Caesarean section under general anaesthesia, performed to save the infant. The other seven patients were all managed by a team, including anaesthetists, cardiologists and obstetricians, from about 25 wk. The patients were hospitalized pre-partum and received oxygen therapy and anticoagulation with heparin. analgesia in labour was managed, once anticoagulation was reversed, by low concentrations of epidural bupivacaine (0.125%-0.375%) and fentanyl. The patients were monitored during labour and delivery with oximetry and arterial and central venous pressure lines. Pulmonary arterial lines were not used because of increased risk and questionable usefulness. Vaginal delivery was managed with vacuum extraction or forceps lift-out to minimize the stress of pushing. After delivery, all patients were monitored in an intensive care unit for several days, anticoagulation was restarted, and all patients were discharged home taking oral anticoagulant therapy. The successful management of pulmonary hypertension in pregnancy should include team management started early in pregnancy and controlled vaginal delivery utilizing epidural analgesia.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |