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"Open CHAT with Alan D. Tong, M.D., FACC, and Benjamin Zeevi, M.D."

Written by:
Alan D. Tong, MD, FACC
Pediatric Cardiologist
Cedars-Sinai Medical Center
Los Angeles, CA
U.S.

Benjamin Zeevi, M.D.
Director, Pediatric Cardiac Catheterization Unit
Schneider Children's Medical Center of Israel
Petach-Tikva
Israel

Edited by: Mona Barmash

Posted: 12/22/97


MRI’s
Q. Will caths for diagnostic purposes eventually be eliminated by MRI's?
A.(AT) In some instances, yes. The problem is that there is some information (such as pressure measurements) that you have to go in and directly measure. Also, everyone knows what angiograms show- there aren't too many people who can look at an MRI and get the same information.

Q. What about PET scans when they become available?
A.(AT) My understanding of PET scans is that they are useful for determining the metabolic activity of a certain area of the body, such as the brain. I'm not sure what uses they will have for the heart. MRIs can see outside the heart pretty well - you can get very nice pictures of the aorta, as in a coarctation or narrowing. For the rest, cath. is pretty darn good.

Fontan

Q. Should kids with the fontan have EKG, stress test, echo, blood work done every year or is that at the discretion of the PC? Are there set guidelines?
A. (AT) Fontan patients should have routine check-ups. What is done at the visits is really individualized, although I think a chest x-ray, EKG, echo., and sat. check are pretty standard. All the other tests are really dependent on the person's condition. Holter if there is a suspicion of too fast (tachycardias) or too slow (sinus node dysfunction), or possible symptoms related to these. Not generally as a routine, screening test.

Q. What do you see as the long term prognosis for kids with fontans?
A.(BZ) We must remember that the fontan is a palliative operation but with The new modifications the prognosis is quite good. Many of the survivors are having an almost normal life. There are more and more women who gave birth following the fontan. We also must remember that we have only about 15-20 years follow-up on the first patients that underwent this operation. If the cardiac function is OK, they can do quite well during pregnancy.
A. (AT) Some types of heart disease with Fontans (tricuspid atresia,two-ventricle repairs) tend to do better. Since they've been done on a widespread basis for only 20 yrs., it's hard to tell what will happen when they are 50, 60, 70. The operation is also being done differently (and we hope, better) now. Some will eventually need a revision or transplant, but every person has a unique situation.

Q. Is it true that standard CPR doesn't work well for fontan patients?
A.(BZ) Their heart function depends a lot on blood volume and the pressure in the pulmonary arteries, so we have to take it into account while performing CPR.

Q. What constitutes ok cardiac function?
A.(BZ) Normal ventricular function is assessed by noninvasive techniques in asymptomatic patients.
A.(AT) Much of this is subjective - how is the person feeling, can they function normally. An echo or stress treadmill can also be helpful to look at ventricular function and exercise tolerance.

Truncus IV

Q. Is Truncus IV recognized at all or does everyone now call it Pulmonary Atresia w/VSD? Why is it not a Truncus defect and what about the long term prognosis?
A.(BZ) you are right about the new classification. The prognosis is the same for patients with pulmonary atresia and a VSD, and depends on the blood supply to the lungs. But like Truncus, it means that only one great artery is coming off the heart. The flow depends on the systemic pulmonary collaterals. Many times they have narrowing along their course that protects the lungs from too much flow.
A.(AT) Developmentally, PA/VSD is more accurate than TA IV - the way they get to that point is different that the other forms of Truncus. As Dr. Zeevi mentioned, prognosis is really dependent on the individual details of the anatomy. This is a good question for your cardiologist. :)

VSD Closure

Q. Can you tell me some information about a procedure for closing VSD using catheterization, and a device similar to a umbrella?
A.(BZ) To my best knowledge, the only place today that is able to perform VSD closure in the USA is Boston Children’s. They have a protocol for high risk patients, which means that the surgeons think that it will be easier to perform the closure in the cath lab. They are using the Cardioseal device, and they have performed more than 100 cases. Many of the patients are either pre or post-op. The same Cardioseal device is currently being used in a multi-national protocol for ASD closure.
A.(AT) I am aware of two or three devices which have been used infrequently (as in experimental or compassionate use only) for VSD closure. They are not very close to being widely available.

Q. What happens when the child grows?
A.(BZ) It is like the patch that the surgeons are using. The tissue around the defect grows.

Q. Do you know what research is been done in the UK?
A.(BZ) I know that some centers in the UK have used the old Rashkind device for VSD closure. The cardiologists at Guy’s Hospital are planning to use the Cardioseal device for VSD closure in the near future. The device is made like two umbrellas-each one has four arms and polyurethane cover.

Unifocalization

Q. Are many unifocalizations done in your country and is there an ideal age or size for the child?
A.(BZ) No. We are doing only few cases of unifocalization. To the best of my knowledge, there are few centers in the US that are performing the unifocalization procedures, for example, Dr. Hanley from San Francisco is doing the procedure very early, sometimes performing the complete repair at the newborn age. Each child is different and needs a special plan for the unifocalization. Unifocalization means building pulmonary arteries from systemic collaterals. Usually fewer collaterals are coming together with more true pulmonary arteries. If a patient has more true pulmonary arteries, the prognosis is better.

Comment:
In other words, if there are true pulmonaries, then there are likely to be fewer collaterals?
A.(BZ) Yes. The other very important issue is the size of the true pulmonary arteries. The bigger the better.
A.(BZ) The lack of a PV is not critical -usually a homograft or other valved tube needs to be put in at the complete repair.

General Anesthesia

Q. Why can't children have general anesthetics when undergoing catheterizations? I never did, and hated every minute of them.
A.(BZ) We perform all our cath’s under general anesthesia. As more caths today are interventional, caths in almost all centers are performed under general anesthesia
A.(BZ) At cath., our kids are either VERY asleep (no crying or other signs of pain) or under general anesthesia (for interventions).

Q. How frequently is Versed used?
A.(BZ) We use it for premedication in almost all patients.

Q. Is there a danger of too much versed affecting the respirations?
A.(BZ) If it is being administered as premedication, the danger is very small. But we have to be careful with every drug.

Transplants

Q. What is the life expectancy of a transplanted child?
A.(AT) The life expectancy of a child after a heart transplant depends on several factors. The main risks are that of organ rejection (which requires immune supressive drugs), and the possibility of infections which result from the immunosupression. Studies of pediatric heart transplant recipients show a 70-80% 5-10 year survival rate - this takes into account patients from all pediatric age groups, from newborns with such problems as hypoplastic left heart syndrome, to adolescents with cardiomyopathy. Since there are only about 300 pediatric heart transplants done in the U.S. each year, relatively few centers perform them in any number, and if possible it would be useful to ask about the institutional statistics pertaining to transplantation (numbers of patients, survival rates, etc.).

Incidentally, there was a recent report in the scientific literature in which two military physicians "trained" the immune system of a small mammal to not recognize a transplanted organ as foreign material. I recall that this involved a process which selectively blocked a specific part of the immune system, different from traditional drug therapy. Such a breakthrough at the human patient care level is probably not very likely for the foreseeable future, but if it does occur it would be a major breakthrough.

Q. Dr Tong, in your response about transplant, you used the phrase "the good centers". What would constitute a good center?
A. High volume, experienced surgeon, cardiologists, and staff, good outcomes data.


This article was reviewed prior to publication by:

Gil Wernovsky, M.D.
Director, CICU
The Children's Hospital of Philadelphia
Philadelphia, PA
U.S.


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