Clinical Profile of the Adolescent/Adult Fontan Survivor

Nancy A Pike,Lynn V Doering,Alan B Lewis,John S Child,Deborah Koniak-Griffin,Lorraine S Evangelista

doi:10.1111/j.1747-0803.2010.00475.x

Abstract

The study aims to describe the clinical profile of the adult Fontan survivor and identify the worries, symptoms, and the impact of cardiac surveillance most commonly experienced. A descriptive, cross-sectional design was used. The study was performed in outpatient adult and pediatric cardiology clinics in university-affiliated and private practice offices. Fifty-four adolescent and adult patients with single ventricle congenital heart disease who have undergone the Fontan procedure participated in the study. The mean age was 26 ± 9 years with 52% female and 63% Caucasian. Demographic and clinical data were obtained by a standard intake form and retrospective chart reviews. The Congenital Heart Disease TNO/AZL Adult Quality Of Life questionnaire was completed to assess worries, symptoms, and the impact of cardiac surveillance. The majority were single (73%), employed or full-time students (93%), with health insurance (94%), had a single left ventricle (78%), the diagnosis of tricuspid atresia or double inlet left ventricle (59%), lateral tunnel Fontan type (44%), history of arrhythmias (76%), left ventricle ejection fraction percentage >50 (66%), oxygen saturations >90% (70%), frequent headaches (50%), scoliosis (22%), varicose veins, ascites, and liver cirrhosis (46%), normal body mass index (59%), and New York Heart Association class I (48%) and II-III (52%). Primary worries related to current health (83%), job/employment (69%), ability to work, (61%) and living independently (54%). The most bothersome symptoms were shortness of breath with ambulation (69%), dizziness (61%), and palpitations (61%). Fontan survivors experience residua and sequelae from multiple surgical procedures and the natural disease course. Our results support the need for ongoing assessment of both physical symptoms and psychosocial concerns, and suggest the need for multiple risk factor intervention strategies that improve physical and emotional health in Fontan survivors.

Highlights

Congenital heart disease (CHD) is a disease of pediatrics and represents a chronic adult disease population
The clinical profile of the general CHD population is changing related to advanced age, improved surgical techniques, and the survival of more complex congenital heart defects
The purpose of this study is to describe the clinical profile of adolescent/adult Fontan survivors; and identify the worries, symptoms, and the impact of cardiac surveillance most commonly experienced

Summary

Introduction

Congenital heart disease (CHD) is a disease of pediatrics and represents a chronic adult disease population. The clinical profile of the general CHD population is changing related to advanced age, improved surgical techniques, and the survival of more complex congenital heart defects. 1.3 million children in the United States with significant heart defects have survived into adulthood.[1] At the present time, there are as many adults with CHD as there are children with this disease.[2] this population is estimated to grow at a rate of 5% per year.[3] The current and future resources required to provide optimal care for this growing population reflect the increased size and number of adult CHD programs across the United States.[4]

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