Info about sugery DSO

[Nagesh59]

Hi,

I am 25 year old having CCTGA with PS and VSD. When i went for diagnosis 6 months back my doctors suggested me to wait for six months and if the problem is increasing then go for Double Switch Operation(DSO). I will get tired if i walk against slope from 10-12 months and getting small pains nowadays.
I googled for info and found that most of the DSO operations done in childhood but i came to know about my disease recently.

Any suggestion regarding what i can do will be deeply appreciated.

Thanks,
Nagesh

[Nagesh59]

Hi,

I have been to hospital recently and my doctor told me no need of DSO as of now since i dont have any problem like breathing, fainting etc during one year. He suggested me to follow up check up yearly once.

But i get very mild chest pain sometimes so am just wondering will this lead to any big problems in future.

Please advice me.

Thanks,
Nagesh

[Jeffrey R Boris, M.D.]

Dear Ahicsa,

What your husband is experiencing is something that we have, unfortunately, been seeing in certain classes of adult patients with congenital heart defects.  Specifically, he was born with transposition of the great arteries, which means that the pulmonary artery, that takes blood to the lungs, comes off of the left ventricle with blood that has come back from the lungs.  The aorta, which takes blood to the body, arises from the right ventricle with blood that has come back from the body.  This creates two separate parallel circulatory systems, instead of one circulatory system in series.  The Senning procedure, in which there is a baffle surgically placed within the atria to send the blood from the body over to the left side of the heart and to send the blood from the lungs to the right side of the heart, is one of the older procedures used to attempt to palliate this defect.  This procedure has fallen out of favor for several reasons, many of which are now manifested in your husband.

Because of the extensive atrial surgery, the sinus node (the natural pacemaker of the heart) can be damaged.  This can lead to the need for a pacemaker.  As well, the right ventricle was never designed to be able to pump blood out under high pressure to the body.  Therefore, over time, it can fail, which leads to congestive heart failure, arrhythmias, and death.  Obviously, without evaluating your husband myself, I can’t say for sure what needs to be done.  However, I would consider several general concepts.  He should be followed by an adult congenital heart disease (ACHD) specialist who is not only familiar with this anatomy, physiology, and complications, but also has access to cardiac catheterization, electrophysiology, and surgical support appropriate for these patients.  Secondly, there has been no good data to demonstrate that ACE inhibitors (including lisinopril) decrease congestive heart failure symptoms or improve ventricular function in this subset of patients.  That said, working with an ACHD specialist who is aware of the literature may be able to help to improve things temporarily with medications as well as alternate pacemaker techniques.  But, his heart will likely not last for a normal life span.  Your husband may need to be eventually considered for heart transplantation.  Finally, and this is a very difficult topic to discuss, you and he need to plan for the event that he does not survive long term.  This means insurance, estate planning for you and your son, disability if he does survive but cannot work, etc.  The ACHD specialist hopefully works with a social worker who can help the two of you plan for and arrange these very important topics.

[Ahicsa]

My husband was born6, in 1986, he is 23 years old and the following is the diagnosis he got just a few days ago. We have a 6 month old child and I want to do everything possible for my husband to see his son grow up to be a man. We are desperate please tell me what this diagnosis means and what should we prepare for, what should we start doing, what should I do???
Diagnosis:
Congenital heart disease.
- Transposition of great vessels @-TGV).
- Post-operative Senning procedure (atrial switch).
- Right ventricular myopathy (RVEF = 20%).
- Mild tricuspid regurgitation.
Sick sinus syndrome.
- Post-operative permanent pacemaker (LA, LV) and AICD.
- Poor capture of ventricular electrode (loose contact). 100% atrial pacing
and 0% ventricular pacing, normal AV conduction.
This 23 year old patient was advised of his currently excellent compensation, following
complex corrective surgery. His future will depend essentially on right ventricular and
tricuspid valve function. At this time, the right ventricular (systemic) has severely
depressed function and severe hypertrophy, but adequate compensation. He was advised
to start medical (Lisinopril5 mg, one a day), in order to decrease RV loading conditions.
He requires close cardiovascular follow-up.

[Nagesh59]

Thank you so much.

[Jeffrey R Boris, M.D.]

Dear Nagesh,

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” operation (DSO).  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.

The largest series of DSOs was performed at the Cleveland Clinic.  They found that performing the operation after age 16 led to profound failure of the procedure.  Therefore, for you, this is not a reasonable option.  Without knowing the rest of how your heart is functioning and the specifics of the anatomy, I cannot say what the best approach is for you.  However, I would make sure that you are cared for at a center that has managed many of these patients.