RESUMO
BACKGROUND: Ornithine transcarbamylase deficiency is an X-linked genetic disorder that induces accumulation of ammonia in the liver and is the most common urea cycle disorder. The clinical manifestation of ornithine transcarbamylase deficiency is hyperammonemia that causes irreversible neurological damage. Liver transplantation is a curative therapy for ornithine transcarbamylase deficiency. The aim of this study is to suggest, from our previous experience, an anesthesia management protocol of liver transplantation for ornithine transcarbamylase deficiency, particularly focused on liver transplantation for cases with uncontrolled hyperammonemia. METHOD: We retrospectively reviewed our anesthesia-related experience in all cases of liver transplantation for ornithine transcarbamylase deficiency in our center. RESULTS: Twenty-nine liver transplantation cases for ornithine transcarbamylase deficiency were found between November 2005 and March 2021 in our center. Of these, 25 cases were stable through the perioperative period. However, 2 cases with carrier donor graft had hyperammonemia after liver transplantation. Another two cases had uncontrolled hyperammonemia before liver transplantation, even with continuous hemodialysis. They underwent life-saving liver transplantation. Their metabolic status stabilized after the anhepatic phase. CONCLUSION: Liver transplantation for cases with uncontrolled hyperammonemia can be performed with proper management. Second, liver transplantation with carrier donors should be avoided because of the risk of postoperative recurrence.
Assuntos
Anestesia , Hiperamonemia , Transplante de Fígado , Doença da Deficiência de Ornitina Carbomoiltransferase , Humanos , Doença da Deficiência de Ornitina Carbomoiltransferase/cirurgia , Doença da Deficiência de Ornitina Carbomoiltransferase/tratamento farmacológico , Doença da Deficiência de Ornitina Carbomoiltransferase/genética , Hiperamonemia/cirurgia , Hiperamonemia/etiologia , Transplante de Fígado/efeitos adversos , Estudos Retrospectivos , Anestesia/efeitos adversosRESUMO
Central core disease is a rare muscular disorder in which anesthetic considerations for the prevention of malignant hyperthermia and for avoidance of residual neuromuscular block are required. A 63-year-old woman with central core disease underwent thoracoscopic sublobar lung resection under total IV anesthesia with a prepared anesthetic workstation. The rocuronium-induced neuromuscular block was monitored by using acceleromyography at the left adductor pollicis muscle and the right masseter muscle. The recovery of neuromuscular block at the masseter was slower than that at the adductor pollicis. The patient showed no symptoms of malignant hyperthermia and residual neuromuscular block and had an uneventful postoperative course. In the present case, malignant hyperthermia was successfully prevented with general anesthesia that is free of triggering agents using a prepared anesthetic machine. The authors speculate that the masseter may be an auxiliary site for neuromuscular monitoring to ensure recovery from neuromuscular block in patients with central core disease.
RESUMO
Systolic anterior motion of the mitral valve and left ventricular outflow tract obstruction are complications following transcatheter aortic valve implantation and can lead to hemodynamic collapse. Medical management for those complications is usually centered on a reduction in left ventricular contractility with negative inotropes. An 88-year-old woman underwent transcatheter aortic valve implantation for severe aortic stenosis. Hemodynamic collapse and exacerbation of mitral regurgitation occurred immediately after valve implantation. For suspected left ventricular outflow tract obstruction, medical management centered on negative inotropes was performed. Hemodynamics and left ventricular outflow tract obstruction improved over time; however, the oxygen supply-demand imbalance progressed. On postoperative day 5, the patient suddenly went into pulseless electrical activity. Cardiopulmonary resuscitation was performed for three minutes, resulting in the return of spontaneous circulation. Subsequent refractory hypotension and oxygen supply-demand imbalance improved with continuous infusion of adrenaline, dobutamine, and phenylephrine. Her hemodynamics remained stable after she was weaned off the pressor infusions, and negative inotropes were not required again. In summary, the cause of cardiac arrest was possibly due to excessive negative inotropic effects even though the effects contributed to improvement of left ventricular outflow tract obstruction. Anesthesiologists and intensivists should recognize the risk of cardiac arrest induced by negative inotropic effects and use negative inotropes with rigorous hemodynamic monitoring, even when left ventricular outflow tract obstruction is treated effectively.