Tele-Clinic Visits in Pediatric Marfan Patients Using Parental Echo: The Future?
Tele-Clinic Visits in Pediatric Marfan Patients Using Parental Echo: The Future?
Marfan syndrome (MFS), a connective tissue disorder seen in 1 in 3,000 individuals, causes
 progressive aortic root dilation that can result in aortic dissection and sudden death.
 Clinical care focuses on monitoring the aortic root by serial echocardiography (echo) to
 guide medical treatment and elective aortic root surgery in a specialized clinic every 6-12
 months. This monitoring protocol, coupled with surgical intervention, has doubled the median
 life expectancy which was previously only 32 years. However, this surveillance carries
 significant health care costs at >$50 million dollars/year on echos alone (at $3-4K each) in
 children and adolescents in the US, as well as substantial burden on families residing far
 from specialized centers. A clinic visit delivered to MFS patients via live-video
 conferencing at home (tele-visit) could shift this paradigm, if a home echo could be
 obtained.

 Here, the investigator will train parents of Pediatric Marfan patients to take echo images
 using a hand held device, height, weight, blood pressure, medical history, and listen to the
 heart of their child. Then, the investigators will ask them to take the equipment home and
 collect the same data at home during a tele-clinic visit, with further instruction by the
 study team through secure live-video conferencing.
In the proposed intervention, every patient (n=60) will have a tele-visit and an on-site
 clinic visit 1 day apart. Parents will have a 1-hour hands-on training session to acquire
 basic echo images on their children with the same hand-held device that will be used during
 the tele-visit. Tele-visit elements will include interim medical history by the parent and
 patient, and weight, height, vital signs, digital cardiac auscultation, and home echo
 (transferred via Internet for remote interpretation), all obtained by the parent. Two MFS
 physicians, following our routine MFS care protocol, will administer either the tele-visit or
 on-site clinic visit, masked to the findings of the other. Two independent echo readers will
 analyze home and clinic echos to measure reproducibility.
Inclusion Criteria:

 -  5-19 years of age (patient)

 -  seen in at least 2 prior clinic visits

 -  Marfan syndrome by revised Ghent criteria

 -  presence of parent at home

 Exclusion Criteria:

 -  prior aortic surgery

 -  known cardiomyopathy

 -  known arrhythmia

 -  aortic root > 4.5 cm in prior clinic visit

 -  pregnancy