2018 FSA Posters

P060: BRAIN ABSCESSES, UNPALLIATED CYANOTIC HEART DISEASE, FACTOR VII DEFICIENCY, OH MY!
Clinton F McDaniel, MD, Gregory M Janelle, MD, FASE, FASA; University of Florida

Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (TOF/PA/MAPCAs) is one of the most variable and severe forms of TOF accounting for approximately one-fifth of all cases of TOF. Patients requiring non-cardiac surgical intervention with this rare defect must be carefully managed to achieve optimal surgical outcomes.

JC, a 35-year-old-male, presented with altered mental status and left-sided weakness after recently visiting El Salvador. He was hemodynamically stable on 2 liters nasal cannula. JC was diagnosed with multifocal cerebral abscesses leading to cerebral edema and midline shift requiring urgent drainage. He had a complicated congenital heart disease history including TOF/PA/MAPCAs s/p central shunt unifocalization & right modified Blalock-Taussig (BT) shunt in 1998. Both interventions thrombosed leading to unpalliated cyanotic heart disease. JC’s baseline SaO2 prior to presentation was 70-73%. His hematocrit was 65% due to secondary polycythemia from his chronic hypoxia. A recent transthoracic echocardiogram showed a large unrestricted perimembranous ventricular septal defect, prominent right ventricular hypertrophy and moderately dilated left ventricle with dyssynchronous septal motion and normal ejection fraction.  

JC was taken to the OR for a limited craniotomy to drain the largest abscess and intravenous antibiotics would treat the remaining abscesses per infectious disease recommendations. This would expedite OR time given his complicated cardiopulmonary status. Several hours before surgery he received 2 units of fresh frozen plasma (FFP) for an elevated PT/INR of 19.9/1.7. Repeat levels did not change. A STAT Factor VII level was 43% (normal >54%) indicating a component of Factor VII deficiency. Hematology consulted and recommended Recombinant Factor VIIa to increase his Factor VII level for his high-risk intracranial surgery. Intraoperatively, he had an awake arterial line placed with an uneventful standard intravenous induction and intubation. A joint decision was made with neurosurgery to not use pins or stealth to avoid further sympathetic stimulation. JC was maintained on a short-acting narcotic infusion and low dose (<0.5 MAC) inhalational anesthetic. He was extubated in the OR with no complications and transferred to the Neurosurgical ICU for close monitoring. He remained stable without further operative intervention.

MAPCAs are complex vessels that arise directly from the aorta or its branches and may leave some lung segments with excessive or insufficient blood flow. His chronic hypoxemia is due to the right-to-left intracardiac shunts of patients with unrepaired TOF/PA/MAPCAs. Supplemental oxygen was unlikely to significantly alter his pulmonary blood flow. Hyperventilation may have either not been possible or may have significantly adversely affected the Qp:Qs balance. In this case, it was important to maintain his hemoglobin and hematocrit levels high. Since the patient already received 2 units of FFP, further increases in his plasma volume through excessive fluid administration or FFP could create a relative dilutional anemia. This could make him decompensate from a respiratory standpoint as his degree of erythrocytosis is necessary for oxygenation and diluting his blood may increase oxygen demand requirements on his heart. This case illustrates the importance of communication between multiple subspecialties and the recognition and management of rare disease processes.