>> AFTER THE ARTERIAL SWITCH OPERATION,
THE AORTA, WHICH WAS SITTING OVER THE RIGHT VENTRICLE,
NOW SITS OVER THE LEFT VENTRICLE.
AND WE USE THE TERM NEO-AORTA FOR THAT--
FOR THAT VESSEL BECAUSE IT'S NOW A NEW AORTA.
IT'S IN A NEW POSITION.
THE PULMONARY ARTERY, WHICH WAS SITTING OVER THE LEFT
VENTRICLE, NOW SITS OVER THE RIGHT VENTRICLE.
THE VESSELS THEMSELVES ARE SWITCHED,
BUT THE BASE OF THE VESSELS WHICH CONTAIN THE VALVES
THAT LEAD TO THOSE VESSELS REMAIN ON EACH
OF THE VENTRICLES.
SO IN FACT, WHEN YOU DO AN ARTERIAL SWITCH OPERATION,
YOU BRING THE AORTA OVER TO THE LEFT VENTRICLE,
BUT YOU'RE PUTTING THAT OVER WHAT HAS DEVELOPED
AND ORIGINATED AS THE PULMONARY VALVE.
SO THE OLD PULMONARY VALVE BECOMES THE NEO,
OR NEW, AORTIC VALVE AS IT NOW CONNECTS TO THE AORTA.
AND AS WE FOLLOW THESE PATIENTS FORWARD IN TIME,
WHAT WE'VE LEARNED IS THAT SOMETIMES THAT PULMONARY
VALVE, THAT NEO-AORTIC VALVE,
NATURAL PULMONARY VALVE, CAN BEGIN TO LEAK A LITTLE BIT.
AND SO THAT'S SOMETHING THAT WE NEED TO FOLLOW.
>> TRANSPOSITION OF THE GREAT ARTERIES IS ONE
OF THOSE LESIONS IN WHICH THERE ARE RARELY OTHER
ASSOCIATED DEFECTS.
>> IN SOME, AFTER REPAIR THERE CAN BE DEVELOPMENT
OF NARROWING OF THE PULMONARY ARTERY.
>> NORMALLY, THE PULMONARY ARTERY IS BEHIND THE AORTA,
BUT IN THIS SITUATION, WE NOW HAVE TO PUT
THE PULMONARY ARTERY IN FRONT OF THE AORTA
BECAUSE IT'S SWITCHED.
THAT ENDS UP DRAPING THE TWO PULMONARY ARTERIES THAT GO
TO THE RIGHT LUNG AND THE LEFT LUNG ON TOP
OF THE AORTA.
AND THIS CAN SOMETIMES RESULT IN NARROWING
OF THOSE ARTERIES.
SO THAT'S ONE OF THE THINGS WE LOOK AT CAREFULLY
AND CAN BE FOLLOWED WITH ECHOCARDIOGRAPHY.
>> AND WE'VE NOW SEEN IN SOME OF OUR ADOLESCENTS
THAT THERE CAN BE NARROWINGS OF THE CORONARY ARTERIES
FOLLOWING THE SWITCH ITSELF.
>> HOW THIS PLAYS OUT LONG-TERM WITH THE CORONARY
ARTERY PROBLEMS THAT MANY PEOPLE GET--IF WE ALL LIVE
LONG ENOUGH, WE'LL HAVE CORONARY ARTERY PROBLEMS--
IS YET TO BE SEEN.
>> THERE ARE NO 40- OR 50- OR 70-YEAR-OLDS WHO HAVE HAD
AN ARTERIAL SWITCH PROCEDURE, SO WE DON'T KNOW,
REALLY WHAT'S GOING TO HAPPEN TO MANY OF THESE
PATIENTS WHEN THEY GET MUCH OLDER.
>> BUT IT IS SOMETHING THAT WE'LL NEED TO TRACK
IN THESE INFANTS.
WE'RE OPTIMISTIC THAT, ACTUALLY,
THEY'RE GOING TO DO FINE WITH THIS AND THEY WON'T
HAVE A SIGNIFICANT INCREASE IN CORONARY PROBLEMS,
BUT THAT'S STILL UNKNOWN.
>> THESE ARE PATIENTS THAT NEED TO CONTINUE TO BE
MONITORED AND CARED FOR BY A PEDIATRIC CARDIOLOGIST AND,
AS THEY GET OLDER NOW, BY OUR COLLEAGUES IN THE ADULT
CONGENITAL HEART REALM.
>> BUT FOR WHAT'S CALLED TRANSPOSITION WITH INTACT
VENTRICULAR SEPTUM, OR SIMPLE TRANSPOSITION,
THE SURVIVAL SHOULD BE EXCELLENT.
YOU SHOULD HAVE BETWEEN A 95 AND 100 PERCENT CHANCE OF SURVIVING
THE OPERATION, AND MOST KIDS HAVE GOOD EXERCISE CAPACITY,
ARE GOING TO SCHOOL AND LEADING RELATIVELY NORMAL LIVES.
>> GRACE IS--
>> YOU WOULD NEVER KNOW.
>> NO, YOU WOULD NEVER KNOW IN A MILLION YEARS--
>> THAT SHE EVER HAD ANY TYPE OF HEART DEFECT OR--
SHE'S GOING TO BE THREE IN MAY, AND SHE IS UNBELIEVABLE.
>> QUITE A PERSONALITY.
>> OH, YEAH, SHE'S A HAM.
>> SHE IS VERY OUTGOING.
SHE LOVES TO DANCE.
SHE LOVES TO SING.
SHE HAS SO MANY BRUISES AND BUMPS ON HER LEGS
FROM FALLING AND JUMPING AND CLIMBING AND CRAWLING AND,
YOU KNOW, WHATEVER, ANY KIND OF ACTIVITY.
WE REALLY--YOU KNOW, WE'VE BEEN TOLD THAT SHE REALLY
SHOULD HAVE NO LIMITATIONS.
>> ONE OF THE OTHER THINGS TO LOOK FOR AS AN OUTCOME,
SOMEWHAT OF A SHORT-TERM OUTCOME IN PATIENTS
WITH TRANSPOSITION, IS THAT IN SOME,
AFTER REPAIR THERE CAN BE DEVELOPMENT OF NARROWING
OF THE PULMONARY ARTERY.
IT'S A VERY SMALL PERCENTAGE,
BUT IT IS SOMETHING TO LOOK FOR.
AND WE'VE NOW SEEN IN SOME OF OUR ADOLESCENTS THAT
THERE CAN BE NARROWINGS OF THE CORONARY ARTERIES
FOLLOWING THE SWITCH ITSELF.
INTERESTINGLY, MANY OF THOSE PATIENTS ARE COMPLETELY
ASYMPTOMATIC AT THE TIME, BUT STILL NO QUESTION THAT
THIS IS A GROUP OF PATIENTS WE NEED TO CONTINUE
TO MONITOR AND FOLLOW.
>> WHAT I TELL FAMILIES IS THAT THERE ARE MORE ADULTS
NOW THAN THERE ARE CHILDREN LIVING WITH CONGENITAL HEART
DISEASE WHICH, FRANKLY, IS A TESTIMONY TO THE FACT WE'VE
BEEN PRETTY SUCCESSFUL AT DEALING WITH THESE
CONDITIONS.
>> OUR MISSION ULTIMATELY IS TO HELP CHILDREN WITH HEART
DISEASE AND TO HELP THEIR FAMILIES GET THROUGH THIS
DIFFICULT PROBLEM, TO DO WHATEVER WE CAN TO MAKE
THEIR LIVES BETTER.
>> I HOPE SHE JUST STAYS HAPPY AND DOES WHATEVER
SHE WANTS.
>> I HOPE SHE CAN LOOK BACK AT THESE PICTURES,
LOOK AT THEM AGAIN IN 20 YEARS AND REALIZE WHAT SHE
PUT US THROUGH.
>> YEAH, EXACTLY, RIGHT.
>> SHE'S JUST A GREAT KID AND LIKE ANY KID,
I MEAN, WHO'S HAD A HEART DEFECT,
WHO HASN'T HAD A HEART DEFECT.
YOU JUST--YOU WISH THE WORLD FOR THEM.
>> WHAT MOVES ME IS TO WORK WITH THE FAMILIES
AND TO WORK WITH THE CHILDREN AND TO GIVE THEM THE BEST
POSSIBLE CARE THAT WE CAN PROVIDE.
THAT'S FIRST AND FOREMOST.
>> AND TO SEE THEM GROW AND TO SEE THEM INTEGRATED
INTO THE FAMILY AND TO SEE THEM INTERFACE WITH THEIR PARENTS
AND THEIR SIBLINGS JUST AS YOU WOULD ANY OTHER ROUTINE
AND NORMAL CHILD.
>> TO BE ABLE TO TAKE THE HEART OF A CHILD AND FIX IT
SO THAT THE PHYSIOLOGY IS BETTER AND THE CHILD
HAS A CHANCE AT A LONG LIFE, THAT'S VERY GRATIFYING.
>> THERE'S NOTHING BETTER THAN SEEING THE KIDS COME
BACK WHEN THEY'RE FOUR OR FIVE YEARS OLD.
>> AND REALLY SEE THAT THEY'RE JUST LIKE ANY OTHER KID.
>> THROUGH THE FETAL HEART PROGRAM,
WE'RE NOW ABLE TO OFFER HOPE AND PROMISE FOR THE FUTURE
FOR THESE CHILDREN TO GO ON TO LEAD HAPPY AND HEALTHY
LIVES, AND THAT'S WHAT THIS IS ALL ABOUT.