Congenital heart defect support for adults

This post is in response to queries received by Dr Helen in her call for cardiology questions.

Limaro asks

My son had a congenital heart defect repaired surgically at age 3. A recent news story indicated (as I understood it) that once a patient outgrows the Pediatric Cardiologists, there is no real data on problems as an adult. We are saving kids and infants today, that would not have made it just a few years ago. Are there procedures, studies or statistics on the long term success of these pediatric patients through adulthood?

It is true that there is scarce data on lifelong followup of babies who had congenital heart disease corrected when they were in childhood. There is a department in some paediatric centers called clinics of Adults Postsurgical Congenital heart disease. I gather that there are not many. This was the interest of Dr Jane Sommerville of Great Omond Street Hospital of London. I am uncertain of such hospitals in the USA, having myself been trained in the UK and Malaysia.

However, there are different types of congenital heart diseasesuch as the "blue babies" like Tetralogy of Fallot, Transposition of great Vessels (TGV), Total Anomalous Pulmonary Artery Drainage (TAPVD), and the complex congenital heart disease group like Tricuspid Atresia. The other group consists of non-blue babies including Atrial Septal Defect (ASD), Ventricular Septal Defect (VSD) and Patent Ductus Arteriosus (PDA).

It is clear that the non-blue babies do very well with cardiac corrective surgery and invariably, surgical correction is a cure in this age group. In fact the management of this group is now with interventional cardiology. Closure of ASD, VSD and PDA can now be done with closure devices introduced via the femoral artery and vein at the groin.

As for the blue babies group, the longterm outcome is more mixed. The Fallots can be totally corrected with good results. There are some reports of sudden death following good Fallot's repair, probably as a result of the RV incision with the older technique. Current technique avoids a RV ventriculotomy scar and so has better longterm results. The Rastelli switch operations has been good for TGV but they can have longterm problems and long term outcome depends on the case selection. The Mustard, and Senning's operation is almost never done. The TAPVD group and the complex congenital heart disease group will need more long term data. Sometimes, in this group, the initial operations are palliative, and the patient may require stage two, or three operations.

In conclusion, Limaro's comments that life long follow up data is lacking, is true. Of course there is also the group of neglected congenitals, like the ASD or VSD or PDA with severe pulmonary hypertension with Eisenmenger's syndrome of reversal of shunt. Those can no longer be operated, and their long term outcome is grime as this Eisenmenger's syndrome is uniformly fatal.

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