Search

Background: Comprehensive assessment of tetralogy of Fallot (TOF) outcomes extends beyond morbidity and mortality to incorporate patient-reported outcomes (PROs), including quality of life (QOL) and health status (HS)., Objectives: This study explored PROs in adolescents and adults with TOF and delineated variables associated with PROs., Methods: This was a cross-sectional observational study within a larger prospective registry of adolescents and adults with repaired TOF and moderate or greater pulmonary regurgitation from North America, Europe, and Asia. Participants completed PROs, including a QOL linear analogue scale (QOL-LAS) and an HS visual analogue scale (HS-VAS). Scores were classified according to age cohorts: <18, 18 to 25, 26 to 40, and >40 years., Results: The study included 607 patients (46.3% female; median age 28.5 years). Median QOL-LAS scores (0-100) were similar across age cohorts (85, 80, 80, 80; P = 0.056). Median HS-VAS scores (0-100) were lowest for the oldest cohort (77) compared with the 3 younger cohorts (85, 80, 80) (P = 0.004). With advancing age, there were increased reports of poor mobility (P < 0.001) and pain or discomfort (P = 0.004); problems in these dimensions were reported by 19.1% and 37.2% of patients aged >40 years, respectively. Of factors associated with superior PROs on multivariable regression modeling (ie, being White, being nonsyndromic, having employment, and having better left ventricular function; P < 0.05), asymptomatic status (functional class I) was the variable associated with the greatest number of QOL and HS measures (P < 0.001)., Conclusions: Strategies to improve TOF outcomes should consider PROs alongside conventional clinical variables. Factors associated with poorer PROs represent opportunities to intervene to improve the lives of patients with TOF., Competing Interests: Funding Support and Author Disclosures This study was funded by the Canadian Institutes of Health Research (MOP 119353). The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: The natural history of right ventricular (RV) and left ventricular (LV) size and function among adults with tetralogy of Fallot (TOF) repair and hemodynamically significant pulmonary regurgitation (PR) is not known. The main aim of this study was to determine changes in RV and LV size and function over time in an adult population with TOF repair and hemodynamically significant pulmonary regurgitation., Methods: Forty patients with repaired TOF and hemodynamically significant PR were included. These patients were identified on the basis of having more than one CMR between January 2008 and 2015. Patients with a prosthetic pulmonary valve or any cardiac intervention between CMR studies were excluded. Rate of progression (ROP) of RV dilation was determined for both indexed right ventricular end-systolic volume (RVESVi) and indexed right ventricular end-diastolic volume (RVEDVi), and calculated as the difference between the last and first volumes divided by the number of years between CMR#1 and CMR#2. Subjects were also divided into two groups based on the distribution of the ROP of RV dilation: Group I-rapid ROP (>50th percentile) and Group II-slower ROP (≤50th percentile)., Results: The interval between CMR#1 and CMR#2 was 3.9 ± 1.7 years (range 1-8 years). We did find a significant change in RVEDVi and RVESVi over this time period, although the magnitude of change was small. Nine patients (23%) had a reduction in right ventricular ejection fraction (RVEF) by greater than 5%, 13 patients (33%) had an increase in RVEDVi by greater than 10 mL/m 2 and seven patients (18%) had an increase in RVESVi by greater than 10 mL/m 2 . Median ROP for RVEDVi was 1.8 (range -10.4 to 21.8) mL/(m 2 year); RVESVi 1.1 (range -5.8 to 24.5) mL/(m 2 year) and RVEF -0.5 (range -8 to 4)%/year. Patients with a rapid ROP had significantly larger RV volumes at the time of CMR#1 and lower RVEF as compared to the slow ROP group. There was no overall significant change in LVEDVi, LVESVi, or LVEF over this time period., Conclusions: We have demonstrated, in a small population of patients with hemodynamically significant PR, that there is a small increase in RV volumes and decrease in RVEF over a mean 4-year period. We believe it to be reasonable practice to perform CMR at least every 4 years in asymptomatic patients with repaired TOF and hemodynamically significant PR. We found that LV volumes and function remained stable during the study period, suggesting that significant progressive LV changes are less likely to occur over a shorter time period. Our results inform a safe standardized approach to monitoring adults with hemodynamically significant PR post TOF repair and assist in planning allocation of this expensive and limited resource., (© 2016 Wiley Periodicals, Inc.)

Background: Chronic hemodynamically relevant pulmonary regurgitation (PR) resulting in important right ventricular dilation and ventricular dysfunction is commonly seen after tetralogy of Fallot (TOF) repair. Late adverse clinical outcomes, including exercise intolerance, arrhythmias, heart failure and/or death accelerate in the third decade of life and are cause for considerable concern. Timing of pulmonary valve replacement (PVR) to address chronic PR is controversial, particularly in asymptomatic individuals, and effect of PVR on clinical measures has not been determined., Methods: Canadian Outcomes Registry Late After Tetralogy of Fallot Repair (CORRELATE) is a prospective, multicentre, Canada-wide cohort study. Candidates will be included if they are ≥ 12 years of age, have had surgically repaired TOF resulting in moderate or severe PR, and are able to undergo cardiovascular magnetic resonance imaging. Enrollment of > 1000 individuals from 15 participating centres (Toronto, Montreal, Quebec City, Sherbrooke, Halifax, Calgary, Edmonton, and Vancouver) is anticipated. Clinical data, health-related quality of life metrics, and adverse outcomes will be entered into a web-based database. A central core lab will analyze all cardiovascular magnetic resonance studies (PR severity, right ventricular volumes, and ventricular function). Major adverse outcomes (sustained ventricular tachycardia and cardiovascular cause of death) will be centrally adjudicated., Results: To the best of our knowledge, CORRELATE will be the first prospective pan-Canadian cohort study of congenital heart disease in children and adults., Conclusions: CORRELATE will uniquely link clinical, imaging, and functional data in those with repaired TOF and important PR, thereby enabling critical evaluation of clinically relevant outcomes in those managed conservatively compared with those referred for PVR., (Copyright © 2014 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

The short lifespan of right ventricle-to-pulmonary artery (RV-PA) conduits used in repairs of complex congenital heart defects makes future surgical replacement inevitable. Percutaneous pulmonary valve implantation (PPVI) now offers an attractive alternative to surgery in some patients. The objectives of this study were to examine the pattern of conduit reoperations, the factors affecting conduit longevity, and to discuss the role of PPVI in these patients. Forty-nine patients (mean age 27 ± 8 years) with pulmonary atresia or pulmonary stenosis with tetralogy of Fallot who underwent surgery for RV-PA conduits from September 1974 to October 2011 were reviewed. A total of 106 RV-PA conduits were implanted, 57 of which were replacements. Second, third, and fourth conduits were required during the follow-up period in 39, 16, and 2 patients, respectively. Freedom from reoperation at 10 years for the first, second, and third conduits was 50%, 74%, and 86%, respectively. Significant independent predictors of shorter conduit longevity included smaller conduit and conduit type (homograft and other vs Dacron). Furthermore, a significant proportion (32 of 57 [56%]) of conduit replacements took place from ages 9 to 18 years. There were 37 adults whose current existing conduits had not yet failed, with 73% (27 of 37) potentially suitable in the future for PPVI on the basis of conduit size of 16 to 27 mm. In conclusion, multiple RV-PA conduit revisions were required in patients who survived to adulthood, with many replacements taking place during adolescence. Most conduits in this adult cohort met size criteria for PPVI, thereby offering these patients a potential alternative to surgical intervention for conduit failure., (Crown Copyright © 2013. Published by Elsevier Inc. All rights reserved.)

Background: tetralogy of Fallot (TOF) is a complex congenital heart disease with clinical and genetic heterogeneity. Of the few known causes, 22q11.2 deletion syndrome (22q11DS) is the most common. We sought to define other clinical subgroups by focusing on cardiac and extracardiac features., Methods: we prospectively screened a cohort of adults with TOF using an established protocol by which subjects were categorized as "syndromic" if they had at least 2 of 3 features: dysmorphic facies, learning difficulties, or voice abnormalities. We then compared the prevalence of cardiac and extracardiac features between subjects in the syndromic group (n = 56) and 112 age- and gender-matched subjects who did not meet our syndromic criteria., Results: the syndromic group was more likely than the nonsyndromic group to have pulmonary atresia and/or major aortopulmonary collateral arteries (25% vs 13%, P = .04). There was a trend toward a higher prevalence of one or more major congenital extracardiac anomalies, primarily involving the musculoskeletal and genitourinary systems (25% vs 13%, P = .06). Later-onset conditions, including neuropsychiatric disorders (32% vs 17%, P = .03), thyroid disorders (20% vs 4%, P = .001), and hearing deficits (20% vs 0, P < .001), were more common in the syndromic group. The syndromic group tested (n = 50) had neither 22q11.2 deletions nor karyotypic anomalies., Conclusion: similar to 22q11DS, adults with TOF meeting screening criteria for a possible genetic syndrome are enriched for more severe cardiac disease and late-onset extracardiac features. Increased awareness of this subgroup with a multisystem condition may be helpful for identifying individuals for referral to medical genetics and optimizing management.

Background: Adults with repaired Tetralogy of Fallot (rTOF) comprise one of the largest cohorts among adults with congenital heart disease (ACHD). These patients have a higher burden of atrial arrhythmias (AA), leading to increased adverse events, including stroke and transient ischemic attack (TIA). However, the data on factors associated with stroke/TIA in rTOF are limited, and classic risk factors may not apply. We studied event rates and associated factors for thromboembolism in a rTOF cohort. Methods: Retrospective cohort study of all adult patients age >18 years with rTOF followed at a single ACHD tertiary care center. AA of interest were atrial fibrillation (AF) and atrial flutter (AFL). Results: Data from 260 patients were identified, mean age 37.6 SD 13.3 years, followed over 5108 patient‐years (mean 16.6 SD 8.2 years). 43 patients had AF and/or AFL, and 30 patients had thromboembolic events, of which 19 patients had stroke/TIA. The event rate for any thromboembolism was 3.39 per 100 patient‐years follow‐up in patients with AA, compared to 1.80 in patients without (P =.07). In univariate analysis, older age and diabetes were associated with thromboembolic events. In multivariate analysis, only older age was associated with thromboembolic events. Conclusions: In our relatively young cohort of adults with rTOF, there was a high prevalence of AA, associated with nearly double the rate of thromboembolic events compared to patients without AA. Older age alone is independently associated with thromboembolic events. Further studies into assessment of silent AA are required, and routine assessments should be considered at an earlier age. [ABSTRACT FROM AUTHOR]

The short lifespan of right ventricleetoepulmonary artery (RV-PA) conduits used in repairs of complex congenital heart defects makes future surgical replacement inevitable. Percutaneous pulmonary valve implantation (PPVI) now offers an attractive alternative to surgery in some patients. The objectives of this study were to examine the pattern of conduit reoperations, the factors affecting conduit longevity, and to discuss the role of PPVI in these patients. Forty-nine patients (mean age 27±8 years) with pulmonary atresia or pulmonary stenosis with tetralogy of Fallot who underwent surgery for RV-PA conduits from September 1974 to October 2011 were reviewed. A total of 106 RV-PA conduits were implanted, 57 of which were replacements. Second, third, and fourth conduits were required during the follow-up period in 39, 16, and 2 patients, respectively. Freedom from reoperation at 10 years for the first, second, and third conduits was 50%, 74%, and 86%, respectively. Significant independent predictors of shorter conduit longevity included smaller conduit and conduit type (homograft and other vs Dacron). Furthermore, a significant proportion (32 of 57 [56%]) of conduit replacements took place from ages 9 to 18 years. There were 37 adults whose current existing conduits had not yet failed, with 73% (27 of 37) potentially suitable in the future for PPVI on the basis of conduit size of 16 to 27 mm. In conclusion, multiple RVPA conduit revisions were required in patients who survived to adulthood, with many replacements taking place during adolescence. Most conduits in this adult cohort met size criteria for PPVI, thereby offering these patients a potential alternative to surgical intervention for conduit failure. [ABSTRACT FROM AUTHOR]