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Overview
Fontan or Fontan Kreutzer procedure is a paediatrics cardiac surgery which is done as a final surgical step for babies with hypoplastic left heart syndrome or HHLS.
In other words children with congenital cyanosis or blue blood syndrome have their atrial and ventricular blood mixes up leading to abnormalities in lungs circulation as their oxygenated and deoxygenated blood mixes up. Fontan is the final step in the surgical procedures to improve redirecting of the blood flow into ventricles.
What are the physical conditions benchmark for this surgery?
As the operation is tricky and is completely invasive, there are certain stringent physical benchmarks which are necessary for the operation. The operation works best in case of:
The pulmonary arteries are in good condition
There is minimal pulmonary vascular resistance.
Presence of a ventricle in good working condition for pumping blood for round circulation
Types of fontan surgery
There are several types of ways in which a font and operation can be done. The types mainly depend on the nature of the heart defect. The three main ways of ensuring blood flow back are:
Extra cardiac fontan
The steps followed in this procedure are
- The superior Vena cava is connected to the right pulmonary artery. However, this procedure can be done earlier by a bi-directional cavo pulmonary shunt operation
- While the inferior Vena cava is rerouted through conduit tube known as Gore Tex which takes it outside the heart and is connected to the pulmonary arteries.
- The arterial septum can be removed though it can be removed in previous surgeries as well
- Thus blood returns into the left atrium via the pulmonary vein and the flow might be faster in the right atrium and ventricle.
Lateral tunnel fontan
The steps followed in case of lateral tunnel fontan are
- In this case the superior and inferior Vena cava is joined by creating a large patch that makes tunnel in the right atrium so that the venous blood is redirected into the pulmonary artery and then into the lungs.
- The Vena cavas are patched directly to the pulmonary arteries above the heart
- Atrial septums are removed
- Thus blood returns from the pulmonary veins in to independent left atriums.
Atriopulmonary connection
This procedure is now rarely used, however the steps are given below
- Your right atrium is directly connected to the pulmonary artery in case the tricuspid valves are present their closed with a patch to prevent the backflow of blood into the ventricles
- The septal defects are closed.
- Thus blood nerves shows from both of anarchy was into the pulmonary artery to the right atrium
Causes
Why is it done?
Babies born with congenital heart defects have abnormality in the ventricular septation leading to admixture of blood. The key issues that may lead to the blood admixture cover the below:
- Congenital heart disease where the tricuspid heart valve or the pulmonary heart valve is missing or defectively formed
- Double inlet left or right ventricle defects
- Condition where left side of the heart is underdeveloped, also known as hypoplastic left heart syndrome (HLHS)
Note that fontan is recommended only in extreme cases where the regular less risky repair methods aren’t able to provide the required results. With this procedure the key objective is to enhance the flow of oxygenated blood and reduce the flow of venous blood to bring down the heart’s workload.
SYMPTOMS
Congenital heart defects are defects of heart which were present at the time of birth does there are chances that your doctors will diagnose the defect during the time of childbirth. However, in case the child delivery was normal here are the symptoms which indicates towards heart defects.
- Pale grey or blue skin colour or cyanosis a classic symptom of blood admixture
- Rapid breathing or shortness of breath
- Swelling around legs, abdomen or eyes
- Poor weight poor feeding etc.
DIAGNOSIS
As this is an invasive cardiac surgery one or two days before the schedule surgery did the patient needs to come and get admitted into the cardiac ward for the preliminary tests.
- Chest X Ray
- A cardio echo scan
- Various blood tests
- ECG
- Height the blue blood weight and other vitals measurement
RISK FACTORS
The risks associated with fontan surgery are
- Pleural effusion
- Pericardial effusion
- Hypoxia
- There can be certain lymphatic complications like clyclothorax, protein losing enteropathy, plastic bronchitis
- There can be certain liver related problems like hepatic fibrosis etc.
- Sometimes the patients might require a heart transplant after few years.
POST-OPERATIVE CARE
- After the operation the patient is shifted into the intensive care unit
- As there is not separate way for pumping blood in to the lungs, arterial blood flow needs to be increased in every possible ways.
- In ICU the patient should be nursed with pillows under head and the legs to assist flow of blood back to the lungs
- To assist in heart contraction infusion drugs like dopamine can be prescribed
- In order to open up the blood vessels and reduce the resistance in circulation drugs like nitroprusside or phenoxybenzamine might be prescribed.
- Initially the patient will be under artificial ventilation for breathing. However, the medical team will make sure that the patient is breathing on his own as soon as possible preferably within less than 12hours of surgery
- A close monitoring of fluids intake and loss should be done
- After shifting to cardiac ward the patient might be highly thirsty. However only 50% of fluid will intake will be allowed as there are high risks of pleural effusion or pericardial effusion
- Patient should be under proper diet full of proteins recommended by dietitian as post surgery there are chances of infection
- Breathing and coughing should be monitored
- Patient should be weighed daily for fluid assessments
- Patient might be administered with anticoagulant in blood thinners like warfarin, heparin and care should be taken so that patient doesn’t get any cuts or bruising
- Hospital stay should be around 14days for close monitoring of vitals
- After release from hospital, the patient needs to be checked by cardiologist and have blood test once or twice a week or as prescribed by the doctors
- Gradually over next couple of months, the medication and check-up schedules will reduce
- Also fluid intake capacity will increase and patient can return to normal life
Generally post surgery the hospital stay is around one to two weeks.
TREATMENT
This is an elaborative cardiac surgery which requires around 4-5 hours for completion and it is done while the patient is under anaesthesia.
- A patch that was placed in the upper right chamber of the heart is removed
- A wall known as baffle is built which directs the blood from lower parts of the body in to the pulmonary arteries i.e. the veins that goes in to the lungs
- Small hole is made into the baffle. This hole is known as fenestration. This will direct the blue blood into the right upper chamber.
- The whole works in a popup valve mechanism when the pressure in lungs is high.
- The size of the whole varies according to the nature and type of defect
- In most children a small hole is drilled and it closes itself over time
- In case the hole drilled is big enough it needs to be closed 6 to 12 months after the surgery through a catheterization process.
The time taken for the surgery to complete is around 5 hours
FACTORS AFFECTING COST
The estimated cost for fontan surgery in India is much lesser than other parts of the world. The comprehensive cost includes charges of
- Cardiologists, surgeons and medical teams
- Surgery charges
- Pre and post operative tests and medicine charges
- Cabin rent and other associated charges
FAQ
Although light to moderate exercise is permissible, heavy strenuous exercise should be avoided. It is recommended to check with your physician before enlisting for intense gym sessions.
Pregnancy involves stress and changes in the cardiovascular cycle which might risk the foetus health. Ask your doctors opinion and avoid complications
Once the surgery is done you will be required to visit cardiologist on a regular basis and undergo regular check-ups.
Although bit lower than the normal population post surgery if properly cared can live long and healthy life up to old age.
Generally the patients are around the age 1.5 years to 4 years. However it can be done for adults with heart defects as well.
