Planning Lifelong Care for The Patient with a Single Ventricle Heart Defect

>> HELLO, MY NAME IS JACK RYCHIK. I'M A PEDIATRIC CARDIOLOGIST AT CHILDREN'S HOSPITAL OF PHILADELPHIA. I'M ALSO THE DIRECTOR OF OUR NEW SINGLE VENTRICLE SURVIVORSHIP PROGRAM AT THE CARDIAC CENTER OF THE CHILDREN'S HOSPITAL OF PHILADELPHIA. WE'RE UNDERTAKING A RELATIVELY NEW APPROACH TO MANAGING COMPLEX HEART DISEASE, IN PARTICULAR THE PATIENT WITH A SINGLE VENTRICLE. WE HAVE HAD AMAZING STRIDES, ACHIEVED AMAZING STRIDES IN SUCCESS FROM AN OPERATIVE STANDPOINT FOR OPERATING ON AND PERFORMING SURGERY IN OUR NEONATES WITH COMPLEX HEART DISEASE. THE GREATER CHALLENGE NOW, BEYOND JUST GETTING OPERATIVE SURVIVAL, IS TO CREATE AN APPROPRIATE QUALITY AND DURATION OF LIFE FOR THESE PATIENTS WHO ESSENTIALLY ONLY HAVE HALF A HEART, THOSE WITH A SINGLE VENTRICLE. WE'RE NOW LEARNING THAT UNFORTUNATELY, ALTHOUGH ALL THINGS MAY APPEAR NORMAL ON THE SURFACE, THAT MANY OF OUR TEENAGERS, YOUNG ADULTS WITH A SINGLE VENTRICLE WHO'VE UNDERGONE THE FONTAN OPERATION, CAN HAVE CHANGES IN THEIR END-ORGAN FUNCTION. WE ARE BEGINNING TO SEE CHANGES IN BONE HEALTH. WE'RE SEEING CHANGES IN CLOTTING FACTORS, IN THE LIKELIHOOD AND TENDENCY TOWARDS INCREASED ABILITY TOWARDS CLOTTING, WHERE WE DON'T WANT TO HAVE CLOTS FORM. WE'RE SEEING CHANGES IN NEUROCOGNITIVE OUTCOME, WHERE SOME OF OUR KIDS ARE NOT PERFORMING; BEHAVING AS WELL AS WE WOULD LIKE THEM TO FROM A DEVELOPMENTAL STANDPOINT. AND IN PARTICULAR, THERE'S A SIGNIFICANT FOCUS NOW IN LOOKING AT THE LIVER, REALIZING THAT THE LIVER IS THE FIRST ORGAN THAT SITS UNDERNEATH THE HEART, AND WHEN YOU HAVE A FONTAN CIRCULATION WITH ONLY HALF OF A HEART, THERE CAN BE CONVEYANCE OF CONGESTION TO THE LIVER AND SLOW CHANGES THAT TAKE PLACE IN THE LIVER THAT ULTIMATELY MANIFESTS AS CLINICAL PROBLEM IN THE SECOND AND THIRD DECADE OF LIFE. SO HERE AT OUR CARDIOLOGY 2012 SESSION, WE HAVE A SPECIAL SESSION ON SUNDAY LOOKING AT COMPLICATIONS AFTER THE FONTAN OPERATION. THERE ARE MANY, MANY ABSTRACTS THAT HAVE BEEN PRESENTED LOOKING AT HYPOPLASTIC LEFT HEART SYNDROME, ONE OF THE MOST COMMON FORMS OF SINGLE VENTRICLE AS WELL AS OTHER FORMS OF SINGLE VENTRICLE. THERE'S A TREMENDOUS AMOUNT OF KNOWLEDGE THAT'S BEING GENERATED ABOUT THE OVERALL OUTCOME FOR THESE PATIENTS. WE'RE BEGINNING TO CHARACTERIZE THE NEW STATE FOR THESE VERY SPECIAL HUMAN BEINGS WHO'VE REALLY NEVER WALKED THE FACE OF THE EARTH BEFORE. NOW THAT THEY'VE SURVIVED, THEY'RE IN THEIR 20S, GETTING INTO THEIR 30S, MANY, MANY CHALLENGES THAT THEY'RE BEGINNING TO FACE. AND IN THE FIELD OF CONGENITAL HEART DISEASE AND PEDIATRIC CARDIOLOGY, AND CONGENITAL HEART CARE IN GENERAL, WE DO HAVE A CHALLENGE IN FRONT OF US TO TRY OUR BEST TO IMPROVE OUR UNDERSTANDING OF THESE COMPLICATIONS, AND TO BEGIN TO EFFECT THERAPIES THAT WILL ULTIMATELY IMPROVE THE OUTCOME FOR THESE PATIENTS. THANKS.