Quality of Life is Diminished in Patients with Tetralogy of Fallot with Mild Residual Disease: A Comparison of Tetralogy of Fallot and Isolated Valvar Pulmonary Stenosis.

The objective of this study is to compare quality of life (QOL) in patients with mild pulmonary insufficiency (PI) after Tetralogy of Fallot (TOF) repair or after balloon dilation for isolated valvar pulmonary stenosis (VPS). A cross-sectional study of patients with TOF (n = 12) and VPS (n = 19), ages 8-18 years, who underwent cardiac magnetic resonance (CMR) and cardiopulmonary exercise test (CPET) was conducted. Patients with genetic syndromes were excluded. The groups were matched by severity and duration of PI using propensity scores. PI was greater than mild if the regurgitant fraction by CMR was >20%. Health status and QOL assessment included Child Health Questionnaire Child Self-Report (CHQ-CF87), Child Health Questionnaire Parent Report (CHQ-PF50), and Pediatric Cardiac QOL Instrument (parent and patient). QOL scores were compared between groups. Due to propensity matching, the groups had, at worst, mild PI and normal right ventricular ejection fraction on CMR. Parental perception of QOL was significantly worse in TOF as compared to VPS in the domains of general perception of health (P = 0.03), physical functioning (PF; P = 0.004), and family cohesion (P = 0.048). There were no differences in self-reported QOL between groups. There was no association between QOL and right ventricular function on CMR or percent-predicted maximal oxygen consumption on CPET in both groups. Parent-perceived QOL, in the domains of general health perception, PF, and family cohesion, is significantly reduced in patients with TOF with mild residual disease suggesting that the psychosocial impact of congenital heart disease may be significant even with successful repair and satisfactory medical status.

Pulmonary valve replacement and quality-of-life assessment.

BACKGROUND: Chronic pulmonary regurgitation is common after repair of tetralogy of Fallot. Despite the deleterious effects of chronic pulmonary regurgitation on right ventricular function, many patients with repaired tetralogy of Fallot remain asymptomatic. Health is defined not only by the absence of disease but also by physical, mental, and social wellbeing. We sought to examine the impact of pulmonary valve replacement on quality of life in asymptomatic patients with repaired tetralogy of Fallot and chronic pulmonary regurgitation. METHODS: From January 2009 to December 2012, 25 (18 male) asymptomatic patients (mean age 23.4 ± 7.4 years) who underwent pulmonary valve replacement for significant pulmonary regurgitation were recruited. Cardiac magnetic resonance was performed pre- and postoperatively. Quality of life was assessed using the Chinese version of the SF-36v2 evaluation tool. Demographics, clinical data, magnetic resonance findings, and quality-of-life scores were collected and calculated for comparison. RESULTS: After surgery, the indexed right ventricular end-diastolic volume (193 ± 47.3 vs. 105.6 ± 29.6 mL m(-2), p < 0.001) and indexed right ventricular end-systolic volume (108.5 ± 32.9 vs. 61.1 ± 23 mL m(-2), p < 0.001) decreased significantly. The response rates for pre- and postoperative quality-of-life assessments were 100%. Patients demonstrated improvements in all 8 domains of the SF-36v2 assessment. The physical (46.5 ± 6.6 vs. 49.9 ± 6.4, p = 0.012) and mental (43.7 ± 7.8 vs. 51.9 ± 7.6, p < 0.001) component summary scores increased after surgery. CONCLUSION: Pulmonary valve replacement can improve the quality of life in patients with chronic asymptomatic pulmonary regurgitation.

Improved exercise performance and quality of life after percutaneous pulmonary valve implantation.

OBJECTIVE: Percutaneous pulmonary valve implantation (PPVI) has emerged as a new approach to treat patients with dysfunctional pulmonary valve conduits. Short- and midterm results have outlined hemodynamic improvements and increase in exercise performance. However, there is a lack of knowledge about quality of life at short term follow-up. PATIENTS AND METHODS: From July 2007 to March 2013, we investigated 59 patients (17 female; median age 22.8 years) undergoing PPVI in our institution. 46 had predominant pulmonary stenosis (PS) and 13 had predominant pulmonary regurgitation (PR). They answered the quality of life questionnaire (SF-36) and underwent a cardiopulmonary exercise test and Cardiovascular Magnetic Resonance before and 6 months after PPVI. RESULTS: Peak oxygen uptake improved significantly from 27.2 (18.9; 34.0) ml/min/kg to 29.2 (22.4; 35.3) ml/min/kg (p<.0001), and from 69.6 (55.9; 83.6) %predicted to 76.3 (67.9; 92.7) %predicted, respectively. Improvements were seen in both, the PS (71.9 to 78.3 %predicted; p<.0001) and PR (62.7 to 73.0 %predicted; p<.0001) group. Self-estimated quality of life was good already before PPVI but increased in almost all domains 6 months after PPVI in PS and PR group. Significant improvements developed in "physical function", "general health perception" and "health transition" in both groups, and "physical role functioning", "vitality" and "mental health" only in the PS group. CONCLUSIONS: In patients with dysfunctional pulmonary valve conduits exercise performance and quality of life improve substantially 6months after successful percutaneous pulmonary valve implantation.

The long-term influence of pulmonary valve regurgitation following repair of tetralogy of Fallot: does preservation of the pulmonary valve ring affect quality of life?

The transannular patch (TAP) repair used in the correction of tetralogy of Fallot (TOF) inevitably causes pulmonary regurgitation. We report herein the results of a long-term follow-up study conducted on 50 patients who had undergone a TAP repair 20-29 years earlier to evaluate the influence of pulmonary regurgitation on their late outcome and quality of life. As a control, 26 patients with an intact pulmonary valve ring and right ventricular outflow patch (RVP) confined to the subvalvular region were also studied. The 25-year survival rates of the TAP and RVP groups were 88.5% and 95.7%, respectively, and the event-free rates at 25 years were 73.3% and 90.9%, respectively. Although the absolute values of these rates were higher in the RVP group, there were no statistically significant differences between the two groups. To assess quality of life, the occupational status, childbearing ability, and late symptoms were evaluated, and found to be comparable between the two groups. Moreover, a treadmill submaximal stress test did not show any differences in exercise capacity between the two groups. In conclusion, the presence of a TAP does not significantly alter the late results or quality of life of patients who have undergone repair of TOF.