Pediatric Heart Expert Forum
PAB &DSO for future
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PAB &DSO for future

I am new in this forum and I expected that you will give your advice for my nephew. But its bad experience that I felt. Can someone tell me what should I do. Its very urgent......................................................Please advice asap.


I am from Chandigarh(INDIA). My nephew is under medical consultation with AIIMS, New Delhi. They are suggesting as follows.......
1. PAB
2. DSO and Valve Repair

Diagnose...........
1.CTGA
2.LT AV Valve Regurgitation
3. Dextrocardia
4.Left LV supplying blood to lungs & RV to the body.


Please suggest me the post surgical complication & remedy for that. From where to get the surgery done with minimal risk & what would b the expenditure.
For more information ,Please have the number of his father::::09034814572,09466510842.......
Please suggest its very urgent sir...................................eagerly waiting for the reply...................

Regards,

DIKSHA PANWAR.
Tags: cctga, L-TGA
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Dear Diskha,

First, this is not a forum for immediate consultation, so please do not have expectations that this is a clearinghouse for patient evaluation.  

Second, you give me no information regarding your nephew, including his age or how he is doing clinically.  That said, I am not able to advise you specifically without evaluating him.  For our other readers, congenitally corrected transposition of the great arteries, also referred to as “l-transposition” or “l-TGA”, occurs when the ventricles are switched.  This means that blood from the right atrium flows into a left ventricle, which then goes into the main pulmonary artery (MPA), through the lungs, to the left atrium, down into a right ventricle, and out to the body.  The blood flow through the heart goes to where it needs to go BUT the ventricles are “inverted”, which means that structurally you have the wrong “types” of ventricles doing the wrong type of work.  The pressure on the right side of the heart is low, and a normal right ventricle is designed for low pressure work.  Certainly, the inverted left ventricle, as designed for high pressure work, can handle it.  However, the right ventricle on the high pressure left side is not architecturally ready for its long term work to pump blood out to the body.  Over time, the left-sided morphologic right ventricle gets thicker, as all muscles do when dealing with higher resistance, but it also dilates.  With further dilation, it leads to a volume load that stretches the tricuspid valve (in this case, the valve between the LEFT atrium and the ventricle), causing it to leak.  As it leaks, it adds more of a volume load onto the ventricle.  Also, as the ventricle dilates, it compresses the right-sided left ventricle, which makes it hard for the heart to pump to the lungs as well.  All of this leads to congestive heart failure and poor cardiac output to the body.

There have been many approaches to this problem, including medications to try to decrease the left-sided volume loading, placing a band on the pulmonary artery to restrict flow the MPA (which increases right sided pressure, pushes back on the left-sided ventricle, and decreases the volume load), transplantation, and the “double-switch”.  A double-switch occurs when two surgical interventions are used.  The first is a Senning procedure, in which the atria are surgically baffled, or directed, across to the opposite ventricles.  In other words, the blood from the right atrium is directed across to the left-sided right ventricle and the blood from the left atrium is directed across to the right-sided left ventricle.  The second part of the switch then also moves the great arteries (the MPA and the aorta) plus the coronary arteries to their correct ventricles, so that the MPA arises now from the left-sided right ventricle and the aorta (and coronary arteries) arise from the right-sided left ventricle.  What this does is get the blood to the appropriate chambers and vessels, so you’d think everything was fixed.  But…it’s not all great.  First of all, it can be difficult to get the untrained right-sided left ventricle that has been working at low pressure for all of these years to suddenly start working at high pressure.  Typically, surgeons will place a band on the MPA to try to “train” the ventricle, although it is not known over the long term whether this gives a good quality result for the ventricle.  Second, it is extensive surgery within the atria to create the baffles, which leads to lots of scarring.  These scars set up the heart for dysfunction of the sinus node, the natural pacemaker of the heart, as well as other tachyarrhythmias (abnormal fast heart beats), such as atrial flutter and fibrillation.  The scarring can also lead to obstruction of inflow from the systemic or pulmonary veins.  Third, there is some evidence that those right-side left ventricles that require training do not hold up as well over the long haul, with decreased function seen.

For your nephew, without all of the data that I would need, I can’t say what his prognosis would be.  I can say that if he is asymptomatic and has only mild leakage of the tricuspid valve, you are not pushed to do anything urgently—this is assuming that his left-sided right ventricle is not significantly dilated.  As you may imagine, this is not a commonly performed procedure, only because l-TGA is not a common cardiac defect.  The Cleveland Clinic, one of the largest centers performing this procedure (prior to the retirement of their surgeon), reported on just 46 of these patients from a 9 year period.  Overall, this procedure has not been performed consistently well until the 1990’s, meaning that good long-term follow-up data is not abundant.

In regards to timing of the procedure, the Cleveland Clinic found that their best results occurred in patients under age 16 years, with significantly more perioperative complications occurring in older patients.  However, I would recommend that you discuss this thoroughly with your cardiologist as well as with the surgeon at the location that the procedure(s) will be performed to ascertain the best timing for this.  Make sure that you are familiar with their numbers and experience with this as well as their outcomes
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Thanx for your response and guidance for the same. The age of my nephew is 4 years. Please also guide what should be the procedure of treatment and the proper time.

Can you suggest from where my nephew's problem can be resolved with minimal post complications and the right place to go.  

Rough idea for the expenditure incurred and the time required.
Can you also suggest the name of pediatric thoracic surgeon.  Please also mention your mail id if you can spare your time to go through the reports.

Regards,


DIKSHA PANWAR.
Blank
Avatar_f_tn
Thanx for your response and guidance for the same. The age of my nephew is 4 years. Please also guide what should be the procedure of treatment and the proper time.

Can you suggest from where my nephew's problem can be resolved with minimal post complications and the right place to go.  

Rough idea for the expenditure incurred and the time required.
Can you also suggest the name of pediatric thoracic surgeon.  Please also mention your mail id if you can spare your time to go through the reports.

Regards,


DIKSHA PANWAR.
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