Quality of Life 4: Long-term cardiological and psychosocial outcome in adults operated for congenital heart disease in early childhood: a longitudinal cohort-study of 40-53 years of follow-up.

Off al live births around the world, approximately 0.8% is born with a
congenital heart defect. In the Netherlands, about 1500
children are born with a congenital heart defect every year. Before surgical
therapy for these defects was possible, half
children died in their first year of life. Less than 15% reached adulthood.

Thanks to surgical correction, 20 years survival is about 85% nowadays.
Approximately 25.000 adults with operated congenital heart disease are alive in
the Netherlands right now. However, total correction is rare. Most patients
have residual lesions and sequelae. In Rotterdam, surgical correction of
congenital heart defects is performed since 1968.

The cohort of patients that was operated between 1968 and 1980 has been studied
before in 1990-1991, 2000-2001 and 2011-2012. In some subgroups of patients
survival and morbidity were nearly normal, whereas for other subgroups (for
example Mustard and Fallot patients) there were worrisome changes in cardiac
function and frequent need for re-intervention. Further deterioration of
cardiac function can be expected, especially for Mustard patients.

The previous studies also showed psychosocial problems in this cohort of
patients. Overall, favorable outcomes on psychosocial functioning and quality
of life were found, but also impairments as to educational and occupation
levels and physical functioning. Predictors for elevated
levels of long-term psychopathology were: being female, restrictions by the
scar, low exercise capacity and other physical
restrictions. Especially young females were at risk for psychosocial problems
(e.g. anxieties as to sexuality, pregnancy, delivery).
Based on these previous findings, an increase in psychosocial problems can be
expected when these patients get older. Accurate information about long term
follow up is therefore very important for treatment and prognosis of these
patients.

The current study proposal makes it possible to describe the natural and
unnatural history in this specific cohort of patients in the very long term.
Specific information will be acquired about the problems patients are
confronted with in this period of their life, when they are growing older.

This study will focus on the long-term outcome after surgical correction of
several congenital heart defects in a non-selected cohort. The repetitive
nature of this study will make it possible to analyze specific changes in
cardiac function over time.
Furthermore, the impact of a congenital heart defect on several psychological
variables and quality of life will be investigated.

The main goal of this project will be to obtain objective information about
mortality, morbidity and cardiac function and also about quality of life and
psychological functioning in a cohort of patients, who underwent surgical
correction of congenital heart disease at young age, specifically ASD, VSD,
tetralogy of Fallot, transposition of the great arteries and pulmonary
stenosis.

Single center, longitudinal cohort study of survival, morbidity and quality of
life in patients who underwent surgical correction of a congenital heart defect
in childhood before the age of 15 in Rotterdam, between 1968 and 1980. Included
are patients who underwent correction of an ASD, VSD, pulmonary stenosis,
tetralogy of Fallot and transposition of the great arteries. This cohort was
also studied between 1990-1991, 2000-2001 and 2011-2012.
All patients will be invited to our outpatient clinic of the department of
cardiology of the Erasmus Medical Center. This study will be conducted between
January 2020 and December 2021.

All patients will undergo extensive outpatient check-up, including medical
history taking, physical examination, ECG, blood testing, exercise testing,
echocardiography, holter examination, cardiac MRI or cardiact CT. Psychological
examination exists of computing
psychological questionnaires and a semi-structured interview. Testing wil be
done in one visit as far as possible, to minimise
patient burden. Results will be stored in a database and compared with the
results in the same patients of the prior studies.

Due to the non-invasive nature of the research, the health risks are very low.
In those cases where a cardiac MRI will be
performed, use of contrast is sometimes needed. In rare cases a patient is
allergic to the contrast agent used. Patients will be
asked specifically if they have had allergic reactions before. Special care
will be available at all times to prevent and treat an
allergic reaction (by antihistaminic and corticosteroids). The same approach
will be followed in case there is a contra-indication or MRI and a dynamic CT
will be performed.

For venapunction, standard precautions will be taken. For patients, taking part
in this study will mean that a they will spend about 7,5 hours in total in the
outpatient clinic, undergoing both medical and psychological tests. Most of the
studies performed will be the same as patients
would undergo in their routine visit.