Management of this Condition and Interventions

Management of the condition will depend on the severity of the defect as well as the types of symptoms and signs that are being exhibited.  Any assessment will be concerned also with factors such as overall health, medical history and age.  How your doctor or consultant expects the condition to develop in the future (the prognosis) is also a factor to consider, although one characteristic of this disease is that there are many uncertainties as to how the condition will unfold in each individual.

Overall, the goal is to maintain the optimum ‘well being’ by reducing symptoms and avoiding future complications.  The treatment selected may include the following:

  • Regular Monitoring – If no symptoms are presented then generally the action would be to see your Doctor or consultant for a regular check up, to monitor any changes or deterioration in the condition.  Typically these check ups may include an echocardiogram, an ECG, a physical examination, and possibly an exercise stress test which monitors how well the heart responds to physical exertion.  One important part of the visit is to find out through conversation how the person has felt since their last visit.  For example, if children are not keeping up with their peers in physical activity this may be the first ‘symptom’ that perhaps the heart is not functioning as well as it was.
  • Medication – may be prescribed to enhance heart contraction and control abnormal heart rhythm.  Medication may be started if symptoms such as shortness of breath, decreased activity tolerance, oedema, and palpitations occur, or if the heart begins to enlarge.  Disturbances in the rhythm of the heart (arrhythmias) can be controlled using different medications such as beta blockers, digitalis, channel blockers and anti-arrhythmic drugs.  If there are signs or symptoms of heart failure these can also be treated with medications such as diuretics.
  • Surgery – surgical repair or replacement is recommended when signs and symptoms become more severe or troubling, for example if the heart is becoming significantly enlarged or inefficient.  There are several surgical techniques used to treat Ebstein’s and associated conditions that are sometimes present:
    • Repair of the Tricuspid Valve -The Tricuspid valve is made up of three separate ‘leaflets’ which in many cases can be surgically manipulated to form a valve that functions properly. The repair of the tricuspid valve requires enough natural tissue for this surgery to be an option. Recent research (Boston et al, 2006:694) states that “in general we believe that tricuspid valve repair is preferable to valve replacement whenever repair is feasible”.  These techniques have been refined over the last 25 years and are now regarded as far more effective click here to read further.
      Replacement of the Tricuspid Valve – In cases where it is not possible or desirable to attempt to repair the valve then it can be removed and replaced with a valve that is either mechanical, or which is bioprosthetic (consisting of specially treated tissue). Where mechanical valves are fitted then life-long blood thinning drugs will have to be taken, and there is always a chance that the valve will have to be replaced again in the future.
    • The Atrial Septal Defect (ASD) Closure procedure– Around 50% of those with Ebstein’s will also have this defect where there is a small hole between the left and right atria. When surgery takes place to repair or replace the tricuspid valve then this hole can be stitched closed during the operation.

Although this can make for quite daunting reading, a quote from some recent research by Boston et al gives an optimistic view of these surgical interventions:

” we conclude that young children can now be repaired with low mortality and good long term durability”

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