RESUMO
With the ongoing development of safer anesthesia, pregnant women with cardiac disease are presenting more frequently for cesarean delivery. We report the successful anesthetic management of a 31-year-old parturient, on long-term anticoagulant therapy, who presented with acute cardiac tamponade due to perforation of the left atrium from a dislodged atrial septum defect occluder. The cesarean delivery was followed by temporary repair of the perforation without extracorporeal circulation (EC) due to concerns about excessive postpartum bleeding. The definitive repair of the atrial defect and the perforation were successfully accomplished under (EC) 5 days after delivery.
Assuntos
Anestesia/métodos , Tamponamento Cardíaco/cirurgia , Traumatismos Cardíacos/cirurgia , Comunicação Interatrial/cirurgia , Complicações Cardiovasculares na Gravidez/cirurgia , Adulto , Tamponamento Cardíaco/genética , Cesárea , Feminino , Traumatismos Cardíacos/complicações , Traumatismos Cardíacos/etiologia , Humanos , Gravidez , Falha de Prótese , Dispositivo para Oclusão Septal , Resultado do TratamentoRESUMO
Hypertrophic cardiomyopathy (HCM) is a rare presenting feature of congenital disorder of glycosylation type Ia (CDG-Ia). We report two female siblings with CDG-Ia and cardiomyopathy. Patient no. 1 died at 12 days of age from cardiac rupture and tamponade, which has not previously been reported in CDG-Ia. The second patient died at 2 months of age from HCM. The severe cardiac manifestations seen in our patients emphasize the importance of early cardiac assessment in all patients with CDG-Ia.
