Fontan / Single Ventricle Flashcards¶
Anki-compatible flashcards for Fontan circulation and single ventricle physiology.
Q: What is the fundamental principle of Fontan circulation? A: Passive pulmonary blood flow - systemic venous return flows directly to pulmonary arteries without a subpulmonary ventricle Tags: fontan, physiology, high-yield
Q: What are the three stages of single ventricle palliation? A: Stage 1: Norwood (newborn), Stage 2: Glenn/Hemi-Fontan (4-6 months), Stage 3: Fontan (2-4 years) Tags: fontan, staged-palliation, high-yield
Q: What does the Stage 1 Norwood procedure accomplish? A: Creates unobstructed systemic outflow, controls pulmonary blood flow (BT shunt or Sano), and ensures unobstructed atrial communication Tags: fontan, norwood, stage1
Q: What is a bidirectional Glenn (Stage 2)? A: SVC connected to right pulmonary artery (SVC flow goes to both lungs); IVC still returns to heart Tags: fontan, glenn, stage2
Q: What is a Fontan completion (Stage 3)? A: IVC flow directed to pulmonary arteries, completing the circuit; all systemic venous return now goes directly to lungs Tags: fontan, stage3
Q: What are the two main types of Fontan connections? A: Lateral tunnel (intracardiac baffle) and extracardiac conduit Tags: fontan, types
Q: What is the typical target oxygen saturation for a Fontan patient? A: 92-98% (near-normal saturations expected if no fenestration) Tags: fontan, oxygen, saturation
Q: What is a Fontan fenestration? A: Intentional small communication between Fontan pathway and atrium; allows R→L shunt, maintains cardiac output at cost of cyanosis Tags: fontan, fenestration
Q: What is Fontan-associated liver disease (FALD)? A: Chronic hepatic congestion leading to fibrosis, cirrhosis, and risk of hepatocellular carcinoma (HCC) Tags: fontan, complications, fald, high-yield
Q: What surveillance is recommended for Fontan-associated liver disease? A: Annual liver imaging (ultrasound or MRI) and serum AFP; liver biopsy/elastography for staging Tags: fontan, fald, surveillance, high-yield
Q: What is the risk of HCC in Fontan patients >20 years from surgery? A: ~1.3% (increasing with duration of Fontan) Tags: fontan, hcc, liver
Q: What is protein-losing enteropathy (PLE) in Fontan patients? A: Loss of protein through gut due to elevated systemic venous pressure and lymphatic dysfunction; presents with edema, ascites, low albumin Tags: fontan, ple, complications, high-yield
Q: What is the prognosis of PLE in Fontan patients? A: Poor prognosis; 5-year survival ~50%; consider transplant evaluation Tags: fontan, ple, prognosis
Q: What is plastic bronchitis in Fontan patients? A: Formation of bronchial casts from lymphatic dysfunction; presents with cough, dyspnea, expectoration of casts Tags: fontan, plastic-bronchitis, complications
Q: What anticoagulation is recommended for ALL Fontan patients? A: Thromboprophylaxis required: aspirin OR warfarin (no consensus on which is superior) Tags: fontan, anticoagulation, high-yield
Q: What are indications for warfarin over aspirin in Fontan patients? A: History of thrombus, atrial arrhythmias, fenestration, higher-risk anatomy Tags: fontan, anticoagulation, warfarin
Q: What arrhythmia is most common in Fontan patients? A: Intra-atrial reentrant tachycardia (IART) / atrial flutter Tags: fontan, arrhythmia
Q: Why are Fontan patients at high risk for arrhythmias? A: Atrial scarring from surgery, chronic atrial dilation, abnormal hemodynamics Tags: fontan, arrhythmia, pathophysiology
Q: What hemodynamic feature makes Fontan patients particularly intolerant of arrhythmias? A: Loss of AV synchrony severely impairs cardiac output in a circulation dependent on passive pulmonary blood flow Tags: fontan, arrhythmia, physiology
Q: What is "Fontan failure"? A: Progressive decline in Fontan circulation leading to exercise intolerance, cyanosis, arrhythmias, protein-losing enteropathy, liver disease Tags: fontan, failure
Q: What is the definitive treatment for Fontan failure? A: Heart transplantation (may need combined heart-liver transplant if severe FALD) Tags: fontan, failure, transplant
Q: What is the expected exercise capacity of Fontan patients? A: Reduced compared to normal (~65-70% of predicted VO2max); chronotropic incompetence common Tags: fontan, exercise
Q: What neurodevelopmental outcomes are seen in single ventricle patients? A: High risk of developmental delays, learning difficulties, executive function deficits; routine developmental assessment recommended Tags: fontan, neurodevelopment
Q: What surveillance studies should Fontan patients have annually? A: Echo, ECG/Holter, labs (LFTs, albumin, BNP), liver imaging, AFP; exercise test periodically Tags: fontan, surveillance, high-yield
Q: A Fontan patient presents with peripheral edema, ascites, and albumin of 2.0 g/dL. What complication is most likely? A: Protein-losing enteropathy (PLE) Tags: fontan, ple, clinical, high-yield