Clinical Characteristics and Associations of the "Good Fontan" Patient

This study aims to determine what are some of the clinical characteristics and associations of Fontan patients who are doing well, as well as how accurate cardiology providers are at predicting the likelihood of future adverse event in their Fontan patients.

Study Overview

• Status: Completed
• Conditions: Congenital Heart Disease; Single-ventricle

Detailed Description

This is a multi-center prospective study that involves collecting data from patients as well as their cardiology providers in order to determine whether there is correlation between patient-reported quality of life assessments and clinician-identified assessments of their patients. The study also involves collecting data from the medical records of participating patients and entering it into our study database.

• Study Type: Observational
• Enrollment (Actual): 146

Contacts and Locations

Study Locations

• United States
  • Massachusetts
    • Boston, Massachusetts, United States, 02115
      • Boston Children's Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 10 years to 60 years (Child, Adult)
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

Patients with congenital heart disease who have undergone the Fontan procedure.

Description

Inclusion Criteria:

• Extracardiac or Lateral Funnel Fontan

Exclusion Criteria:

• Currently being evaluated for or listed for cardiac transplant
• Underwent Fontan revision or conversion
• Currently pregnant

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort
Pediatric Fontan Patients; Participants ages 10 to 18 who have had Lateral Tunnel or Extracardiac Fontan procedures.
Adult Fontan Patients; Participants ages 18 to 60 who have had Lateral Tunnel or Extracardiac Fontan procedures.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Surprise question	The ability of the cardiologist's response to the "surprise" question to predict adverse cardiac events within 1 year	1 year

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Clinical characteristics and associations of the "good Fontan"	Clinical characteristics and associations of this group (the "good Fontan") are different compared to those that are in the "at-risk" category (all others that do not fit into the "good Fontan" category). Data measurements will include relevant clinical data available for each patient, including but not limited to data from cardiac surgeries, catheterizations, MRIs, echocardiograms, exercise stress tests, current medications, labs, and patient responses to questionnaires.	1 year
Correlation between patient and provider assessments	There will be a positive correlation of the patient's own assessment of quality of life with the clinician-identified designation of a "good Fontan."	1 year
Clinician factors associated with adverse events conversation with patients	Clinician factors (years of experience, primary subspecialty, gender, parenthood) are associated with beliefs in regard to appropriate timing to discuss potential long-term adverse events to Fontan patients.	1 year

Collaborators and Investigators

• Sponsor: Boston Children's Hospital
• Collaborators: Massachusetts General Hospital; Yale University; Dartmouth College; University of Massachusetts, Worcester; Connecticut Children's Medical Center; University of Vermont Children's Hospital; New England Congenital Cardiology Association (NECCA); Child Heart Associates; Congenital Heart

Publications and helpful links

General Publications

• d'Udekem Y, Iyengar AJ, Cochrane AD, Grigg LE, Ramsay JM, Wheaton GR, Penny DJ, Brizard CP. The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation. 2007 Sep 11;116(11 Suppl):I157-64. doi: 10.1161/CIRCULATIONAHA.106.676445.
• Diller GP, Giardini A, Dimopoulos K, Gargiulo G, Muller J, Derrick G, Giannakoulas G, Khambadkone S, Lammers AE, Picchio FM, Gatzoulis MA, Hager A. Predictors of morbidity and mortality in contemporary Fontan patients: results from a multicenter study including cardiopulmonary exercise testing in 321 patients. Eur Heart J. 2010 Dec;31(24):3073-83. doi: 10.1093/eurheartj/ehq356. Epub 2010 Oct 7.
• Blume ED, Balkin EM, Aiyagari R, Ziniel S, Beke DM, Thiagarajan R, Taylor L, Kulik T, Pituch K, Wolfe J. Parental perspectives on suffering and quality of life at end-of-life in children with advanced heart disease: an exploratory study*. Pediatr Crit Care Med. 2014 May;15(4):336-42. doi: 10.1097/PCC.0000000000000072.
• Lakin JR, Robinson MG, Bernacki RE, Powers BW, Block SD, Cunningham R, Obermeyer Z. Estimating 1-Year Mortality for High-Risk Primary Care Patients Using the "Surprise" Question. JAMA Intern Med. 2016 Dec 1;176(12):1863-1865. doi: 10.1001/jamainternmed.2016.5928. No abstract available.
• Hamano J, Morita T, Inoue S, Ikenaga M, Matsumoto Y, Sekine R, Yamaguchi T, Hirohashi T, Tajima T, Tatara R, Watanabe H, Otani H, Takigawa C, Matsuda Y, Nagaoka H, Mori M, Yamamoto N, Shimizu M, Sasara T, Kinoshita H. Surprise Questions for Survival Prediction in Patients With Advanced Cancer: A Multicenter Prospective Cohort Study. Oncologist. 2015 Jul;20(7):839-44. doi: 10.1634/theoncologist.2015-0015. Epub 2015 Jun 8.

Study record dates

Study Major Dates

• Study Start (Actual): March 5, 2018
• Primary Completion (Actual): March 20, 2023
• Study Completion (Actual): April 18, 2023

Study Registration Dates

• First Submitted: March 6, 2018
• First Submitted That Met QC Criteria: March 16, 2018
• First Posted (Actual): March 20, 2018

Study Record Updates

• Last Update Posted (Actual): April 19, 2023
• Last Update Submitted That Met QC Criteria: April 18, 2023
• Last Verified: April 1, 2023

More Information

Terms related to this study

• Keywords: Fontan; Single Ventricle
• Additional Relevant MeSH Terms: Cardiovascular Diseases; Congenital Abnormalities; Cardiovascular Abnormalities; Heart Diseases; Heart Defects, Congenital; Univentricular Heart
• Other Study ID Numbers: IRB-P00024716

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No