Background: Patients with complex congenital heart disease increasingly undergo noncardiac surgeries because of advancements in surgical techniques and medical management. In Fontan circulation, maintaining an adequate transpulmonary gradient is essential for preserving both pulmonary blood flow and cardiac output. However, intraoperative circulatory monitoring of Fontan patients has not been established.
Case presentation: A 17-year-old girl required an emergency craniotomy for ruptured arteriovenous malformation. She had a surgical history of bidirectional Glenn operation and Fontan palliation for her congenital hypoplastic left heart syndrome and double outlet right ventricle. We performed general anesthesia with continuous monitoring of central venous pressure (CVP) and stroke volume variation (SVV). Transesophageal echocardiography was not conducted because of difficulty in manipulating the probe due to the patient's position and surgical setting. After incision of the dura, approximately 1700 ml of rapid blood loss from the arteriovenous malformation was observed in 30 min. In this period, CVP decreased from 15 to 5 mmHg or less. In contrast, there was only a slight rise in SVV from 5 to 8%. We rapidly administered fluid and then transfused blood, and CVP gradually recovered to 10-15 mmHg. During the surgery, circulatory collapse was not observed. The patient was transferred to the intensive care unit under sedation and controlled ventilation.
Conclusions: CVP decreased sharply, whereas SVV rose only slightly during acute bleeding in the present case. CVP monitoring may have been useful for the management of an acute bleeding case with a Fontan circulation but SVV may not have been reliable. As more patients with a Fontan circulation undergo noncardiac surgeries, appropriate circulatory monitoring in these patients should be further investigated.
Keywords: Central venous pressure; Fluid resuscitation; Fontan circulation; Hypoplastic left heart syndrome; Stroke volume variation.