Relationship Between Functional Health Status and Ventricular Performance After Fontan--Pediatric Heart Network

Conditions

Arrhythmia, Defect, Congenital Heart

Brief summary

The purpose of this cross-sectional study was to determine the interrelationships between health status and measures of cardiac performance in children 6 to 18 years of age with congenital heart disease who have undergone a Fontan procedure as surgical treatment for functional single ventricle. The goal was to develop a data set that will permit identification of a clinically relevant endpoint for subsequent trials of medical management of the Fontan patient.

Detailed description

BACKGROUND: Children born with one functional ventricle and who undergo a Fontan procedure, have a higher incidence of late complications than other congenital heart patients. While the Fontan procedure results in restoring near-normal systemic oxygen saturation, there are recognized problems and little information about the overall health status, functional status and ventricular performance in these children. DESIGN NARRATIVE: This cross-sectional observational study included children 6-18 years of age who had undergone a Fontan procedure for a congenital cardiac defect. Prospective data collection for each patient occured within a 3 month time period and included cardiac MRI, echocardiography, serology and exercise testing. Historical clinical and surgical data, as well as parent and child self reports of health status, was collected.

Atz AM, Zak V, Mahony L, Uzark K, D'agincourt N, Goldberg DJ, Williams RV, Breitbart RE, Colan SD, Burns KM, Margossian R, Henderson HT, Korsin R, Marino BS, Daniels K, McCrindle BW, Pediatric Heart Network Investigators.

2017-06-01196 citations

10.1016/j.jacc.2017.03.582

Assessment of Quality of Life in Young Patients with Single Ventricle after the Fontan Operation.

Uzark K, Zak V, Shrader P, McCrindle BW, Radojewski E, Varni JW, Daniels K, Handisides J, Hill KD, Lambert LM, Margossian R, Pemberton VL, Lai WW, Atz AM, Pediatric Heart Network Investigators.

2015-12-1066 citations

10.1016/j.jpeds.2015.11.016

Arrhythmias in a contemporary fontan cohort: prevalence and clinical associations in a multicenter cross-sectional study.

Stephenson EA, Lu M, Berul CI, Etheridge SP, Idriss SF, Margossian R, Reed JH, Prakash A, Sleeper LA, Vetter VL, Blaufox AD, Pediatric Heart Network Investigators.

2010-09-01131 citations

10.1016/j.jacc.2010.03.079

Interventions

None listed

Study design

Observational model

CASE_CROSSOVER

Time perspective

PROSPECTIVE

Eligibility

Sex/Gender

ALL

Age

6 Years to 18 Years

Healthy volunteers

No

Inclusion criteria

* Age 6 through 18 years at the time of enrollment. * Fontan surgery of any type at least 6 months prior to the time of testing. * Agreement and ability to have all of the following testing completed: 1. An echocardiogram at the study center, 2. Parent health status questionnaire, and 3. Blood testing. * Planned or ongoing cardiac care at the study center that will allow completion of study testing within 3 months of study enrollment. * Informed consent of a parent or guardian, and assent of the study participant if he/she is able to provide it, according to institutional guidelines.

Exclusion criteria

* Non-cardiac medical or psychiatric disorder that would prevent successful completion of planned study testing or would invalidate the results of study testing. * Ongoing or planned participation in another research protocol that would either prevent successful completion of planned study testing or invalidate the results of study testing. * Lack of reading fluency by the primary caregiver in both English and Spanish, languages for which the parental report health status questionnaire has been validated. * Pregnancy at the time of enrollment or pregnancy planned prior to completion of study testing.

Outcome results