AuthorsMichelle McGauvran, M.D., Gregory J. Latham, Peter Von Homeyer, M.D. Department of Anesthesiology, University of Washington, Seattle, WA Department of Anesthesiology, Seattle Children's Hospital, Seattle, WA ... [ view full abstract ]
Authors
Michelle McGauvran, M.D., Gregory J. Latham, Peter Von Homeyer, M.D.
Department of Anesthesiology, University of Washington, Seattle, WA
Department of Anesthesiology, Seattle Children's Hospital, Seattle, WA
Background
The congenital heart disease population presents a medically complex population for non-cardiac surgery and represents a rapidly growing segment of the adult population as more afflicted children live to adulthood. We present a case of a 53 y/o female with unrepaired tetralogy of Fallot with rapidly progressing cardio-renal syndrome undergoing general anesthesia for a laparoscopic peritoneal dialysis catheter placement. Our challenge was to induce and maintain general anesthesia in a patient with decompensated congenital heart disease for laparoscopy.
Case Description
Patient is a 53 y/o female with past medical history significant for thalidomide embryopathy, phocomelia, and tetralogy of fallot with pulmonary atresia. In addition, she also had discontinuous pulmonary arteries, history of MAPCA, a PDA to her left pulmonary artery, and an increasingly stenotic Blalock-Taussig shunt to her right pulmonary artery. The patient presented to the hospital with progressive cardio-renal syndrome, significant hepatic congestion and ascites refractory to diuresis and was scheduled for a peritoneal dialysis catheter placement.
She underwent the procedure under general anesthesia with standard ASA monitors, a dorsalis pedis arterial line, and access in her pre-existing central line. She required vasopressin boluses and a vasopressin drip to maintain her systemic vascular resistance and avoid “stealing” from pulmonary blood supply and avoid an increase in pulmonary vascular resistance. The patient was extubated successfully after the procedure, and is still using her peritoneal dialysis catheter today.
DISCUSSION
Only case reports exist of this population of patients surviving to this advanced age, with our patient’s particular pathology of complete pulmonary atresia being even more remarkable. To our knowledge, this is the first anesthetic to be reported in this specific patient population. Our case represents congenital heart disease pathology at its finest and provides a fascinating lesson in cardiac physiology.
