Definition: separates pulmonary and systemic blood flow in single ventricle physiology; accomplished by intra-atrial lateral tunnelling of the IVC flow into RPA, or extracardiac via conduit (extracardiac Fontan)
Physiology:
Better systemic oxygenation
Reduced volume load to the systemic ventricle
May be fenestrated to obtain adequate CO postoperative
High PVR (high CVP, low LAP). To lower PVR: milrinone, iNO
Exclude pulmonary venous obstruction (ECHO)
Ventricular dysfunction (high CVP, high LAP): commence low dose milrinone (0.25 mcg/kg/min) as more common diastolic dysfunction, exclude AV valve regurgitation (ECHO)
Dysrhythmias: avoid inotropic, chronotropic support if feasible
Outcome:
Low perioperative mortality <2%
Absence of death or transplantation after 1y: 80%, 5 yrs: 77%, 10 yrs: 75%, 25 yrs: 54%
Major morbidity: tacharrhythmias, thrombotic events, PLE
References:
[1] Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Tricuspid Atresia
[2] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S57-69: Giglia et al: Preoperative pulmonary hemodynamics and assessment of operability: is there a pulmonary vascular resistance that precludes cardiac operation?
[3] Interact Cardiovasc Thorac Surg. 2010 Mar;10(3):428-33: Gewillig et al: The Fontan circulation: who controls cardiac output?
[4] Interact Cardiovasc Thorac Surg. 2010 Feb;10(2):262-5. Gewillig et al: Volume load paradox while preparing for the Fontan: not too much for the ventricle, not too little for the lungs
[5] Pediatr Cardiol. 2007 Nov-Dec;28(6):448-56: Deal t al: Arrhythmia management in the Fontan patient.[6] Circulation. 2008 Jan 1;117(1):85-92: Khairy et al: Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery
[7] Pediatr Crit Care Med. 2011 Vol. 12, No.1: 39-45: Cholette et al: Children with single-ventricle physiology do not benefit from higher haemoglobin levels post cavopulmonary connection: Results of a prospective, randomized, controlled trial of a restrictive versus liberal red-cell transfusion strategy
All Marc’s PICU cardiology FOAM can be found on PICU Doctor and can be downloaded as a handy app for free on iPhone or Android. A list of contributors can be seen here.
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Fontan circulation
Tags: fontan
Marc Anders. Fontan circulation, Don't Forget the Bubbles, 2013. Available at:
https://doi.org/10.31440/DFTB.3498
Definition: separates pulmonary and systemic blood flow in single ventricle physiology; accomplished by intra-atrial lateral tunnelling of the IVC flow into RPA, or extracardiac via conduit (extracardiac Fontan)
Physiology:
Preoperative preparation:
ECG, CXR, CUS, FBE, clotting, UECs, PRBC (4), FFP (2), platelets (2), cryoprecipitate (2).
Risk assessment for Fontan candidates:
Postoperative management:
Discuss anticoagulation with surgeon…
Specific problems:
Outcome:
References:
[1] Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Tricuspid Atresia [2] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S57-69: Giglia et al: Preoperative pulmonary hemodynamics and assessment of operability: is there a pulmonary vascular resistance that precludes cardiac operation? [3] Interact Cardiovasc Thorac Surg. 2010 Mar;10(3):428-33: Gewillig et al: The Fontan circulation: who controls cardiac output? [4] Interact Cardiovasc Thorac Surg. 2010 Feb;10(2):262-5. Gewillig et al: Volume load paradox while preparing for the Fontan: not too much for the ventricle, not too little for the lungs [5] Pediatr Cardiol. 2007 Nov-Dec;28(6):448-56: Deal t al: Arrhythmia management in the Fontan patient.[6] Circulation. 2008 Jan 1;117(1):85-92: Khairy et al: Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery [7] Pediatr Crit Care Med. 2011 Vol. 12, No.1: 39-45: Cholette et al: Children with single-ventricle physiology do not benefit from higher haemoglobin levels post cavopulmonary connection: Results of a prospective, randomized, controlled trial of a restrictive versus liberal red-cell transfusion strategyAll Marc’s PICU cardiology FOAM can be found on PICU Doctor and can be downloaded as a handy app for free on iPhone or Android. A list of contributors can be seen here.
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