An 8 year old boy presents to the ED with abdominal pain for 2 days. He is diagnosed with acute appendicitis requiring immediate appendectomy. The child's Mom said that he has congenital heart disease and had corrective surgery soon after birth and again at two years of age. She recalls that the second procedure was called a "Fontan." After the second surgery the patient has been doing relatively well but he cannot exercise as much as the other children in his class. He has not eaten or drank anything for 12 hours; his blood pressure is 95/60 mmHg, heart rate is 120 bpm, respiratory rate is 28 bpm, he has a peripheral intravenous line is receiving maintenance fluid.

Study Questions:

• Classify CHD based on physiology of blood flow.
• What type of pathology may lead to the Fontan procedure as a solution?
• Explain general Fontan anatomy and physiology. What physiologic conditions are important to maintain?
• Is it necessary to give antibiotic prophylaxis?

• What is differential diagnosis?
• Which additional monitors can be used?

• What CVP constitutes adequate preload for Fontan patients?

• What is your differential diagnosis? Would a Valsalva maneuver help? What problems specific for Fontan physiology can cause this hypotension and hypoxia?
• What can be done to resolve the problem?

• What are the warning signs you see on ABG?
• What are the pros and cons of keeping the child on mechanical ventilation?

• Do you still plan to keep the child on mechanical ventilation in the PICU? If you decide to keep the child intubated, will you reparalyze him? What parameters would affect your decision?
• What postop pain management would you plan? Will it affect the PICU course? What the pros and cons of different methods?

• Congenital Heart Disease
• Congenital Heart Disease, G. Gordon, M.D.
• Single Ventricle and Fontan Circulation