.
Patient (condition)
 
Aliases:
total cavopulmonary anastomosis patient with
Fontan circulation

Topic aliases are alternate phrasings for a particular topic.


Patients with a recently completed Fontan circulation complicated by a protein losing-enteropathy may have pleural effusions and intercostal catheters present

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Enquire specifically about the use of anticoagulants or antiplatelets such as warfarin or clopidogrel which are often administered to reduce the risk of thromboembolic complications.

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Balance the aspiration risk from maintaining adequate oral hydration with the risk of dehydration and hypoglycaemia.
Consider intravenous hydration in cyanotic patients.

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Caution with premedication in patients with cyanosis, pulmonary hypertension or ventricular dysfunction, as hypercapnia may elevate pulmonary vascular resistance

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Have a low threshhold for inserting an arterial line to monitor blood pressure for moderate and major procedures

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If central venous catheter/CVC to be used for inotropy, consider femoral to minimise the risk of central venous thrombosis which is potentially life threatening in these patients. #safety

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Consider that nitrous oxide may increase pulmonary vascular resistance and reduce cardiac output in this population reliant on passive venous return #ref

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If the patient requires IPPV, ensure a prolonged expiratory time to allow time for passive flow of blood from vena cavae through the pulmonary circulation

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Spontaneous ventilation is preferred as negative intrathoracic pressure aids passive blood flow into lungs.

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If choosing a spontaneous ventilation technique, monitor for and avoid excessive hypercapnia which can increase pulmonary vascular resistance.

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If not achieving adequate tidal volumes with low inspiratory pressures, consider increasing inspiratory pressures (20-30cm H2O) if required to facilitate a short inspiratory time. The potential costs of briefly high intrathoracic pressures are likely outweighed by prolonged expiration time and adequate ventilation.

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"Management of atrial tachycardia [in the patient with a Fontan circulation]: the faster the rhythm, the poorer the patient’s condition, the more scars on the chest, the less the clinician understands, the faster he should opt for urgent DC reconversion." heart.bmj.com/content/91/...

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In adults with a completed fontan circulation, the IVC conduit may have become progressively smaller relative to their size over time, and the fenestration may be calcified and closed.
#IS

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Maintain sinus rhythm where possible. Patient's with a fontan circulation tolerate atrial tachyarrhythmias poorly, and depend on adequate ventricular filling pressures to maintain an adequate cardiac output. refl.in/nz

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Maintaining preload is important to maintain an adequate transpulmonary gradient.
Be aware of the septic, bleeding, or dehydrated Fontan patient.

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Thrombosis is more likely to occur in patients with low cardiac output and more than usual atrial and systemic venous dilatation and blood stasis. Fontan patients have an increased incidence of coagulation factor abnormalities—as in any patient with hepatic congestion: protein C, protein S, and antithrombin III deficiency. Thrombosis may complicate an infection, especially when associated with dehydration. heart.bmj.com/content/91/... www.ncbi.nlm.nih.gov/pubm...

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Adenosine won't work in the completed Fontan patient in SVT as it is metabolised in the lungs before it makes into the heart.
DCR will be required.

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This study of 15 patients suggests a ketamine loading dose of 2mg/kg followed by an infusion does not increase pulmonary vascular resistance in children with pulmonary hypertension undergoing sevoflurane spont vent anesthesia refl.in/3z

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Good review paper in Heart on the fontan circulation. heart.bmj.com/content/91/...

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