T.P.E. Ruys, E. Utens, A.E. van den Bosch, J.A. A. E. Cuypers, M. Witsenburg, W.A. Helbing, A.J.J.C. Bogers, R. van Domburg, P. Opić, J.S. McGhie, J.J.M.
Takkenberg, J. W. Roos-Hesselink
Chapter 3 40
ABsTRAcT
Aim
To describe differences in cardiological and psychological outcome after surgical correction of Tetralogy of Fallot (ToF) over time.
methods
Survivors of ToF surgery performed in our centre: between 1980-1990 (ToF80) and between 1969-1980 (ToF70) were followed and evaluated 20-30 years after the initial correction in hos-pital. The study included ECG, echocardiography, exercise-testing, 24-hour Holter-monitoring and health-related quality of life questionnaire.
Results
Survival at 25 years after successful surgery was 93% for ToF70 and 98% for ToF80 (p=0.22).
Pulmonary valve replacement for pulmonary regurgitation was performed more often in the ToF80 patients (p=0.030). Right ventricular dysfunction was found in 7.2% ToF70 and in 15.6% ToF80 (p=0.11),and left ventricular end-diastolic diameters were 49 mm and 45 mm (p = 0.008) respectively. Mean aortic sinus diameter was 37mm in ToF70 and 33mm in ToF80 (p=0.001). Aortic regurgitation was present in 21.1% and 7.5%, respectively (p<0.001).
There was no difference in exercise capacity and quality of life between the ToF70 and ToF80 patients. ToF patients of both cohorts scored lower compared to healthy controls with re-spect to general health, but higher on social functioning and experienced less behavioural problems such as anxiety and aggression.
conclusion
Compared to patients operated on in the 70’s, patients from the 80’s showed less aortic root dilatation, aortic regurgitation and a smaller end diastolic left ventriclular diameter, indicat-ing an improvement of the left side of the heart. However, surprisindicat-ingly pulmonary valve replacement was needed more often in patients who were operated in the 80’s.
Keywords
Tetralogy of Fallot, survival, long-term outcome, quality of life, left and right ventricular dysfunction.
Evolution of Tetralogy of Fallot surgery 41 outcomes after ToF repair are reported good with regard to survival. However, a considerable proportion of patients develop late complications or have residual lesions.(2,3,4) The surgical technique for right ventricular outflow tract (RVOT) reconstruction frequently distorts the pulmonary valve causing pulmonary regurgitation (PR) or residual stenosis. Late pulmonary valve replacement is often needed.(2,4,5) In addition concern exists regarding the fate of the left ventricle dysfunction, aortic valve regurgitation, aortic dilatation and arrhythmias.(6,7)
A detailed description of the time-related instantaneous hazard or risk of death, re-oper-ation and pulmonary valve replacement (PVR), is required to assess the long-term outcome into adulthood for children operated for ToF. These late hazards are probably multifactorial.
There has been consistent progress in treatment strategy, both with regard to patient selec-tion and surgical techniques. In the 1970’s patients often first had a palliative shunt, followed by corrective surgery with a right ventricular (RV) incision at around 4 years of age. In the 1980’s surgeons attempted to avoid RV incision and shunts, the latter by operating earlier.
The use of the transannular patch became more common in the 80s. In the 90’s surgeons tried to avoid transannular patches, to decrease the reoperation rate for pulmonary valve dysfunction. (8) The impact of these developments on late outcome has not been studied.
Age at operation and new techniques may also influence psychological outcome. In a study by Knowles et al. patients with ToF reported lower functional health status then their healthy siblings, but quality of life and life satisfaction were comparable.( 9)
We analysed clinical and psychological outcome of patients who underwent surgical correction for ToF in our institute between 1980 and 1990, and compared the results with previously reported outcome of patients who underwent surgical correction for tetralogy
Chapter 3 42
of Fallot in our institute between 1969 and 1980. Assessment was done in a similar manner, enabling comparison of the two decades.
mEThods
Patients
The study population includes patients with ToF without major concomittant abnormalities such as pulmonary atresia with aorto-pulmonary collaterals or absent pulmonary valve.
The long term outcome of our first cohort of patients operated for ToF between 1969-1980 has been described extensively, previously.(2) Survival data were available in all of the 140 of the operated patients and in 2001 90 patients (64%) participated in the in-hospital protocol , as define below. Baseline characteristics of the patients who participated did not differ from the patients who did not participate. The data of these 90 patients studied and described in 2001, will be referred to as the ToF70 group. (2)
Between 1980 and 1990, 105 consecutive patients underwent surgical correction for ToF in our institute. Survival status was obtained in all patients. All survivors (n=95) were invited to participate in our study in 2010 using the same study protocol as used in ToF70. In total 82 patient (78%) participated. This group will be referred to as the ToF80 group. Institutional board approval was obtained (NL26121.078.08) and participating patients provided written informed consent.
Quality of life and behavioural problems assessment scores (the Short form 36 (SF 36) and the Adult Self-Report) of the ToF80 were not only compared with the ToF70 group, but also with the normal Dutch population adjusted for age and sex.(10)
study protocol
The study protocol in both ToF70 and ToF80 included physical examination, 12-lead electro-cardiography (ECG), 24-hour Holter monitoring, echoelectro-cardiography, bicycle exercise test, and quality of life assessment (SF 36 and Adult Self-report).
Events: Events were defined as any cardiac intervention (either surgical or catheter interven-tion), arrhythmia requiring treatment or heart failure.
Echocardiography: Transthoracic two-dimensional echocardiography studies were per-formed using the iE33 ultrasound system (Philips Medical Systems, Best, the Netherlands).
Chamber quantification, valvular function, systolic and diastolic function were assessed according to the current recommendations. (11,12i) Left and right ventricular function were assessed by eyeballing as this method was used in 2001 by the same echo-technician as at that time. Body surface area was defined using the DuBois formula, aortic diameter was corrected for body surface area. Aortic root dilatation was defined as the diameter at the
Evolution of Tetralogy of Fallot surgery 43
sinuses of Valsalva ratio above 1.3 of the predicted diameter by the formula described by Roman. (13ROMAN)
Exercise capacity: Maximal exercise capacity was obtained using bicycle ergometry. The exercise capacity measured in Watts and was indexed for age, sex and body height, standard-ized for the Dutch population.
Quality of life was assessed by the Dutch SF 36, for which good reliability and validity have been proven.(14) Higher scores indicate a better quality of life. Mean scale scores of patients were compared between the cohorts ToF70 and ToF80.
Behavioural/Emotional Problems were assessed using a screening questionnaire for psychopathology, using the Adult Self-Report (ASR) for the ToF80 patients and its previous, parallel version the Young Adult Self-Report (YASR) for the ToF70 patients (Achenbach, 1997, 2003). Age-equivalent Dutch normative data on the ASR (n=1211) was derived from Van-heusden et al.(15) For the historical comparison between the ToF80 and T0F70 patients, only the 88 identical and overlapping items of the ASR and YASR were used.
statistical methods
Continuous data were presented as mean with standard deviation and were compared with the Student-T or Mann-Whitney test. Discrete variables were presented in absolute numbers and percentages and were analysed using the chi-square or Fischer’s exact test. Cumulative survival rates were calculated with the Kaplan-Meier method and the Tarone-Ware test was used to compare the two cohorts of patients. In a Cox-regression model (multivariable stepwise upward method) we evaluated era of operation (ToF70 or ToF80), right ventricular incision, age at operation < 2 years, transannular patch and prior palliative shunt as potential predictors for pulmonary valve replacement for pulmonary regurgitation. The level of sig-nificance was p < 0.05 (2-sided). The data were analyzed with the SPSS version 16.0 software (SPSS Inc, Somers, NY).