Which one of the following statements regarding the Fontan operation is correct?
The Fontan operation is a palliative procedure in patients with complex cyanotic heart disease when a biventricular reapir is not possible. These patients have univentricular physiology with mixing of pulmonary and systemic blood in a dominant ventricle (a rudimentary ventricle is often present connected via a ‘VSD’). Life expectancy is not normal. The procedure consists of redirection of systemic venous blood to the pulmonary arteries (although there are a number of technical variations). The current modification is the total cavopulmonary connection (TCPC)
Glenn and Fontan operations. TCPC is the modern modification.
Surgery for TGA to redirect blood via baffles (atrial switch) is named either the Mustard procedure (prosthetic baffles) or the Senning procedure (intrinsic atrial tissue baffles). These approaches have been superseded by the more successful arterial switch procedure. Systemic arterial-to-pulmonary shunts (e.g. Balock–Taussig shunt) can be used in a staged approach to palliate a patient until a Fontan procedure is completed.
You are asked to review a 27-year-old female with complex congenital heart disease. She is normally managed at another centre, and limited information is available. She has had a number of operations in early life but has recently been well. Her parents tell you that she has ‘one main pumping chamber’. The history is of deterioration over the last week with fevers and headache. On examination the patient is cyanosed (baseline saturations 85% on room air) and agitated with GCS 13–15. Temperature is 38.4°C, BP is 120/80 mmHg, and heart rate is 100 bpm (regular). A bedside echocardiogram is attempted but the image quality is very poor. The ED team have initiated supportive treatment with high-flow O2 and IV fluids. Blood cultures have been taken. The chest X-ray is suspicious for right basal consolidation.
What would you advise?
This question emphasizes the risk of cerebral abscess in patients with cyanotic heart disease. Patients with cyanotic heart disease and evidence of sepsis with neurological deterioration require urgent investigation for cerebral abscess. This patient has no evidence of significant haemodynamic compromise. Saturations of 85% may be normal for a patient with univentricular physiology (mixing of systemic and pulmonary blood) and balanced pulmonary and systemic circulations. Clearly it would be important to treat potential chest sepsis and rule out endocarditis.
When describing cardiac anatomy, what does the term ‘situs solitus’ refer to?
Situs solitus refers to a normal orientation of the cardiac structure and abdominal viscera relative to the midline. For congenital heart disease the anatomy is defined from the atria (e.g. the morphological left atrium can be identified by its appendage but may be on the right side, this is situs inversus).
You are asked to review a 22-year-old male who has presented to the ED with sudden-onset chest pain and breathlessness. He has been diagnosed with a probable acute pulmonary embolism by the emergency team. They have asked for an echocardiogram to look for ‘right heart strain’ as he appears to be mildly compromised and they are considering thrombolysis if he decompensates. Auscultation of the heart sounds has revealed a loud continuous murmur and the CXR shows some pulmonary congestion.
What are you likely to see on echocardiography?
This case describes a young patient with sinus of Valsalva aneurysm rupture (acute symptoms, pulmonary congestion, and continuous murmur). Answer B describes a PDA which can be associated with a continuous murmur and endocarditis but not such an acute presentation. PE does not cause pulmonary congestion or a continuous murmur.
A haematology SHO contacts you regarding a patient with congenital cyanotic heart disease. The patient has trisomy 2 and an unrepaired complete AVSD with Eisenmenger physiology and chronic cyanosis. The patient was seen recently in clinic and was doing reasonably well. The full blood count has been highlighted to the haematology team as the patient has a haemoglobin (Hb) of 27. The haematology records document a previous venesection when the Hb was around the same figure and the patient had developed headaches thought to be due to hyperviscosity. The SHO would like some advice on whether they should arrange for daycase venesection to reduce the risk of hyperviscosity complications and symptoms.
What advice should you give?
Polycythaemia is a physiological adaptive process to chronic cyanosis. Venesection should be avoided unless there are clear hyperviscosity symptoms. Venesection is not associated with a reduced risk of stroke and may cause iron deficiency and circulatory collapse without careful volume replacement.
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