BackgroundTetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease and the population of ToF repair survivors is growing rapidly. Adults with repaired ToF develop late complications. The aim of this study was to describe and analyze long-term follow-up of patients with repaired ToF.MethodsThis is a retrospective cohort study. Consecutive 83 patients with repaired ToF who did not undergo pulmonary valve replacement were included. Mean age of all patients was 30.5 ± 10.7. There were 49 (59%) male. Patients were divided into two groups according to the time since the repair (< 25 years and ≥ 25 years). The electrocardiographic (ECG), cardiopulmonary exercise testing (CPET), echocardiographic and cardiac magnetic resonance (CMR) data were reviewed retrospectively.ResultsIn CPET values were not significantly different in the two groups. In CMR volumes of left and right ventricles were not significantly different in the two groups. There were no differences between the groups in ventricular ejection fraction, mass of ventricles, or pulmonary regurgitation fraction. Among all the patients, ejection fraction and left and right ventricle mass, indexed pulmonary regurgitation volume measured by CMR did not correlate with the time since repair. In ECG among all the patients, ejection fraction of the RV, measured in CMR, negatively correlated with QRS duration (r = − 0.43; p < 0.001). There was a positive correlation between QRS duration and end diastolic volume of the RV (r = 0.30; p < 0.02), indexed end diastolic volume of the RV (r = 0.29; p = 0.04), RV mass (r = 0.36; p < 0.001) and left ventricle mass (r = 0.26; p = 0.04).ConclusionLong-term survival and clinical condition after surgical correction of ToF in infancy is generally good and the late functional status in ToF – operated patients could be excellent up to 25 years after the repair. QRS duration could be an utility and easy factor to assessment of right ventricular function.Trial registrationThe study protocol was approved by the local Ethics Committee. Each participant provided informed consent to participate in the study (license number 122.6120.88.2016 from 28.04.2016).
Liver status in adult patients after the Fontan procedure 181 and kidney or liver dysfunction. These complica tions influence the patient's life expectancy and quality of life. 6,7 The characteristic hemodynamic derangement in Fontan circulation causing liver abnormalities and pathology is commonly identified as Fontan associated liver disease (FALD). 2,8 This can lead to nodular hyperplasia, cirrhosis, and hepatocel lular carcinoma associated with ascites, variceal bleeding, and encephalopathy. Moreover, it is re lated with higher late morbidity and mortality in this particular population. 8-10 Recent studies have shown that elevated ve nous pressure (3 to 4 fold in comparison with INTRODUCTION The Fontan procedure was first described over 40 years ago and still remains the standard surgical treatment for patients with a functional single ventricular heart, affecting 35 per 100 000 newborns. 1-3 It is considered one of the most complex congenital heart diseases. Dur ing the procedure, a systemic venous return is di rectly connected with pulmonary circulation, with out a subpulmonary pump. 1,4,5 As a result, the cir culatory system is hemodynamically less efficient. Although the Fontan procedure is associated with low mortality, once patients reach adulthood, they can develop various cardiac and extracar diac complications, including plastic bronchitis
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