Reconstruction of the RVOT with a conduit: Lifetime follow up

Background: The use of a conduit is an established surgical method for reconstruction of the right ventricular outflow tract in congenital heart disease. The most commonly used conduit is a homograft. Its limited durability makes reinterventions almost inevitable but the actual durability of a conduit in the adult population is poorly described. The introduction of transcatheter pulmonary valve replacement (TPVR) has expanded the possibilities for conduit reintervention but the impact of this new technique on clinical practice is unknown. Furthermore, little is known on the influence of reinterventions on quality of life. Conduit surgery and reintervention is among the most common surgical procedures in adult congenital heart disease but relative lack of knowledge complicates decision making. Method: Paper 1: The PubMed database was searched in May 2015 with the terms “homograft AND pulmonary valve,” generating 665 hits. Studies involving more than 50 patients with a mean or median age >18 years were included. Papers 2–4: The Swedish registry of congenital heart disease (SWEDCON) was used to collect data. Patients were identified by codes for classification of surgical procedures and a group variable specific for patients with a conduit. Results: Paper 1: Six studies with a cumulative total of 560 patients were found and included. Perioperative mortality was 0%–2.9%, and long-term mortality was 2%–8.8% at 8.1–10 years. Reinterventions of homografts were common during patients’ lifespans, with a 10-year event-free survival of 78%–80%. Early postoperative echocardiographic or magnetic resonance imaging defects appear to predict rapid homograft degeneration. Paper 2: Data on quality of life (EQ-5D) from the first and latest visit were collected from 103 patients with a mean age of 31 years. Mean time from first to latest visit was 3 years. Health perception (mean EQ-VAS) declined from 84.4 (SD=14.6) at the first visit to 78.6 (SD=18.3) at the latest visit (P=0.001). This decline was not observed in patients with reinterventions between visits (n=18). Low EQ-VAS was associated with symptoms and NYHA class II–IV. Problems in the EQ-5D dimension “usual activities” were more common in patients with reinterventions (25%) than in those without reinterventions (7%) (P=0.04). Paper 3: From 2000 to 2014, there was an increase in the number of adult patients with conduits from 122 to 536, including 60 surgical conduit replacements, 40 TPVRs, and 176 new conduit implantations. There was a significant increase in new implantations (P=0.007) and surgical conduit replacements (P=0.024) across all three time periods. Patients with new implantations were older (median age, 32 years) compared with those in the reoperation and TPVR groups (median age, 26 years), with the majority of patients having tetralogy of Fallot (57%). The majority of conduit reinterventions were surgical also after the introduction of TPVR in 2007, with no significant difference regarding diagnosis, gender, age, or previous number or longevity of conduits between surgical replacement and TPVR. Paper 4: A total of 574 patients with a conduit (mean age 36.1 years) were identified. Tetralogy of Fallot represented the largest group (45%). There were 769 operations and 50 TPVRs. Long-term survival after the first conduit operation (mean age 20.2 years) including perioperative mortality (<1%) was 93% at 20 years. The most common cause of death was cardiac-related. Higher age at first conduit operation was associated with an increased mortality risk. Event-free survival was 77% and 54% at 10 and 20 years, respectively. Ten-year event-free survival after the first conduit reintervention (n=176) was 70%, significantly lower than after the first conduit operation (P=0.04). Higher age at the first conduit operation had a protective association, whereas male gender and complex malformations were associated with an increased risk of further reintervention. Conclusion: Patients perceive a decline in their health over time after right ventricular outflow tract surgery. This decline was not observed in patients with further reinterventions. The number of patients with a conduit is increasing, and reinterventions for conduits are common. Since the introduction of TPVR, less than half of all patients with conduit failure are treated by this technique.Long-term mortality after conduit surgery and reinterventions is low, but the need for conduit reinterventions is substantial. Perioperative mortality is low in relation to cardiac-related death

Long-term outcome after right ventricle to pulmonary artery conduit surgery and reintervention

Background. Reconstruction of the right ventricular outflow tract with a conduit is an established surgical procedure in congenital heart disease and reinterventions are common. Objective. An increasing number of patients have a conduit, but there are few population-based studies of long-term outcomes after conduit surgery, reoperations, and transcatheter pulmonary valve replacement. Methods. In April 2015, all adult patients with a conduit were identified in the Swedish National Registry for Congenital Heart Disease (SWEDCON). Data on patients who died before age of 16 years are not included in the registry and thus not included in the study. Results. We found 574 patients with a mean age 36.1 years. The largest proportion had tetralogy of Fallot (45%). In total there were 762 operations and 50 transcatheter pulmonary valve replacements. Mean age at first conduit operation was 20.2 years. Long-term survival up to 48 years including perioperative mortality (<1%) was 93% at 20 years. The most common cause of death was cardiac-related. Higher age at first conduit operation was associated with increased mortality risk. Reintervention-free survival was 77% and 54% at 10 and 20 years, respectively. Conduit reinterventions were common. Ten-year reintervention-free survival after first conduit reintervention (n = 176) was significantly lower than after first conduit operation (70% vs 77% p = .04). Higher age at first conduit operation was associated with a reduced risk of reintervention, whereas male sex and complex malformations were associated with increased risk of reintervention. Conclusions. The mortality of repeated conduit reinterventions is low. The need for reintervention of conduits is considerable, and reintervention-free survival after the first conduit reintervention is poorer than after first conduit implantation. The findings in this study only applies for patients reaching 16 years of age

RV to PA conduits : impact of transcatheter pulmonary valve replacement in adults – a national register study

Objective: The use of a conduit is an established surgical method for reconstruction of the right ventricular outflow tract in congenital heart disease; however, its limited durability makes reintervention almost inevitable. We studied the trends in new implantation, reoperation, and transcatheter pulmonary valve replacement (TPVR) from a Swedish national perspective. Design and results: The Swedish registry of congenital heart disease (SWEDCON) was used to collect data. From 2000–2014, there was an increase in adult patients with conduits from 122 to 536. There were 60 surgical conduit replacements, 40 TPVRs and 176 new conduit implantations in the study period. Perioperative mortality was <1%. The yearly number of new implantations and reoperations both doubled over the study period. Patients with new implantations were older (mean age, 36 years) compared with the reoperation and TPVR groups (mean age, 26 years) with the majority of patients having tetralogy of Fallot. The majority of conduit reinterventions were surgical also after the introduction of TPVR in 2007, with no significant difference regarding diagnosis, gender, age, or previous number or longevity of conduits. Conclusion: The number of adults with conduits increased steadily with most conduit-related operations being new implantations in patients with tetralogy of Fallot. Surgical conduit replacements increased significantly and represented the majority of conduit reinterventions after the introduction of TPVR, indicating that TPVR is not used for the majority of patients with conduit failure. Diagnosis, gender, age, or previous number or longevity of conduits had no impact on choosing surgical replacement vs. TPVR

Decline in Self-reported Health (EQ-5D) over Time after Surgical Reconstruction of the Right Ventricular Outflow Tract: A Longitudinal Cohort Study of 103 Patients

ObjectivePatients with congenital heart disease may be operated with surgical reconstruction of the right ventricular outflow tract (RVOT). Reintervention is common in this group of patients. The aim of this study was to examine longitudinal self-reported health measured by the EQ-5D questionnaire. DesignData were collected from the Swedish Registry of Congenital Heart Disease. EQ-5D, which measures health outcome, was introduced into the registry in 2005. All adult patients with previous surgical reconstruction of the RVOT who had EQ-5D data from their first and latest visit were analyzed. ResultsAmong 103 patients (65 male and 38 female), mean age 31 (range 19-78 years), the diagnoses were: tetralogy of Fallot (n=66); truncus, transpositions, and double outlet right ventricle (n=23); and Ross-operated congenital aortic valve disease (n=14). Time from first to latest visit was 3 years (range 1-7 years). Eighteen patients underwent 26 reinterventions in the observational period from the first to latest visit, including operations, percutaneous interventions, pacemaker implantations, and ablations. Health perception, mean EQ-5D visual analog scale, VAS, declined from 84.4 (standard deviation (SD)=14.6) to 78.6 (SD=18.3) at the latest visit, P=.001. The decline is almost exclusively seen in patients without reinterventions. Low EQ-VAS was associated with symptoms and New York Heart Association class II-IV. Patient-reported problems in the EQ-5D dimension usual activities were more common in the patients having reinterventions (25%) than those without reintervention (7%), P=.04. ConclusionIn this longitudinal cohort study of patients with previous surgical reconstruction of the RVOT, health perception declined over time. The decline was not observed in patients undergoing any additional interventions

Association between exercise load, resting heart rate, and maximum heart rate and risk of future ST-segment elevation myocardial infarction (STEMI)

Objective This study aimed to examine the association between exercise workload, resting heart rate (RHR), maximum heart rate and the risk of developing ST-segment elevation myocardial infarction (STEMI).Methods The study included all participants from the UK Biobank who had undergone submaximal exercise stress testing. Patients with a history of STEMI were excluded. The allowed exercise load for each participant was calculated based on clinical characteristics and risk categories. We studied the participants who exercised to reach 50% or 35% of their expected maximum exercise tolerance. STEMI was adjudicated by the UK Biobank. We used Cox regression analysis to study how exercise tolerance and RHR were related to the risk of STEMI.Results A total of 66 949 participants were studied, of whom 274 developed STEMI during a median follow-up of 7.7 years. After adjusting for age, sex, blood pressure, smoking, forced vital capacity, forced expiratory volume in 1 s, peak expiratory flow and diabetes, we noted a significant association between RHR and the risk of STEMI (p=0.015). The HR for STEMI in the highest RHR quartile (&gt;90 beats/min) compared with that in the lowest quartile was 2.92 (95% CI 1.26 to 6.77). Neither the maximum achieved exercise load nor the ratio of the maximum heart rate to the maximum load was significantly associated with the risk of STEMI. However, a non-significant but stepwise inverse association was noted between the maximum load and the risk of STEMI.Conclusion RHR is an independent predictor of future STEMI. An RHR of &gt;90 beats/min is associated with an almost threefold increase in the risk of STEMI

Effect of medical treatment in patients with systemic right ventricle.

Objective: Congenitally corrected and surgical atrial redirected transposition of the great arteries (TGA) represents states where the morphological right ventricle serves as a systemic ventricle (S-RV). The S-RV is prone to failure, but data on medical treatment on this problem is limited. The purpose of this study was to evaluate the survival in adults with S-RV, with or without heart failure treatment. Design: The SWEDCON registry was used to collect data. All adults with S-RV and minimum follow-up of 1 year were included retrospectively. Medical treatment was defined as taking beta-blockers and/or ACE inhibitors and/or ARBs for more than 50% of the time. Results: We identified 343 patients with S-RV (median age: 21 years). Surgical atrial redirected TGA was present in 58% and congenitally corrected TGA in 42% of patients. The medically treated group (n = 126) had higher rates of impaired S-RV function, use of diuretics, pacemaker and higher NYHA functional class at baseline compared to controls. The proportion of patients with impaired functional class did not change over time in the medically treated group, but increased in controls (21% vs. 30%, p = .015). In Kaplan-Meier analysis, the mean follow-up was 10.3 years, no difference in survival was seen between the groups. Conclusions: Medical treatment may be beneficial in patients with S-RV and impaired functional class and appears to be safe in the long term. The treatment group had equal survival to controls, despite worse baseline characteristics, which might be a result of slower progression of disease in this group

The association between signs of medical distress preceding in-hospital cardiac arrest and 30-day survival - A register-based cohort study

Background: Identifying signs of medical distress prior to in-hospital cardiac arrest (IHCA) is important to prevent IHCA and improve survival. The primary objective of this study was to investigate the association between signs of medical distress present within 60 minutes prior to cardiac arrest and survival after cardiac arrest. Methods: The register-based cohort study included adult patients (&gt;= 18 years) with IHCA in the Swedish Registry of Cardiopulmonary Resuscitation (SRCR) from 2017-01-01 to 2020-07-15. Signs of distress prior to IHCA were defined as the medical signs arrhythmia, pulmonary oedema, hypotension, hypoxia or seizures present within 60 minutes prior to cardiac arrest (pre-arrest signs). Using multivariable logistic regression, the association between these pre-arrest signs and 30-day survival was analysed in both unadjusted and adjusted models. The covariates used were demographics, comorbidities, characteristics and treatment of cardiac arrest. Results: In total, 8525 patients were included. After adjusting for covariates, patients with arrhythmia had a 58% higher probability of 30-day survival. The adjusted probability of 30-day survival was 41% and 52% lower for patients with hypotension and hypoxia prior to IHCA, respectively. Pulmonary oedema and seizures were not associated with any change in 30-day survival. Conclusions: Among signs of medical distress prior to in-hospital cardiac arrest, arrhythmia was associated with a higher 30-day survival. Hypotension and hypoxia were associated with lower survival after IHCA. These findings indicate that future research on survival after cardiac arrest should take pre-arrest signs into account as it impacts the prerequisites for survival

Left-sided valvular heart disease and survival in out-of-hospital cardiac arrest: a nationwide registry-based study

Abstract Survival in left-sided valvular heart disease (VHD; aortic stenosis [AS], aortic regurgitation [AR], mitral stenosis [MS], mitral regurgitation [MR]) in out-of-hospital cardiac arrest (OHCA) is unknown. We studied all cases of OHCA in the Swedish Registry for Cardiopulmonary Resuscitation. All degrees of VHD, diagnosed prior to OHCA, were included. Association between VHD and survival was studied using logistic regression, gradient boosting and Cox regression. We studied time to cardiac arrest, comorbidities, survival, and cerebral performance category (CPC) score. We included 55,615 patients; 1948 with AS (3,5%), 384 AR (0,7%), 17 MS (0,03%), and 704 with MR (1,3%). Patients with MS were not described due to low case number. Time from VHD diagnosis to cardiac arrest was 3.7 years in AS, 4.5 years in AR and 4.1 years in MR. ROSC occurred in 28% with AS, 33% with AR, 36% with MR and 35% without VHD. Survival at 30 days was 5.2%, 10.4%, 9.2%, 11.4% in AS, AR, MR and without VHD, respectively. There were no survivors in people with AS presenting with asystole or PEA. CPC scores did not differ in those with VHD compared with no VHD. Odds ratio (OR) for MR and AR showed no difference in survival, while AS displayed OR 0.58 (95% CI 0.46–0.72), vs no VHD. AS is associated with halved survival in OHCA, while AR and MR do not affect survival. Survivors with AS have neurological outcomes comparable to patients without VHD

Non-vitamin K antagonist oral anticoagulants (NOACs) for thromboembolic prevention, are they safe in congenital heart disease? Results of a worldwide study

Background: Current guidelines consider vitamin K antagonists (VKA) the oral anticoagulant agents of choice in adults with atrial arrhythmias (AA) and moderate or complex forms of congenital heart disease, significant valvular lesions, or bioprosthetic valves, pending safety data on non-VKA oral anticoagulants (NOACs). Therefore, the international NOTE registry was initiated to assess safety, change in adherence and quality of life (QoL) associated with NOACs in adults with congenital heart disease (ACHD). Methods: An international multicenter prospective study of NOACs in ACHD was established. Follow-up occurred at 6 months and yearly thereafter. Primary endpoints were thromboembolism and major bleeding. Secondary endpoints included minor bleeding, change in therapy adherence (≥80% medication refill rate, ≥6 out of 8 on Morisky-8 questionnaire) and QoL (SF-36 questionnaire). Results: In total, 530 ACHD patients (mean age 47 SD 15 years; 55% male) with predominantly moderate or complex defects (85%), significant valvular lesions (46%) and/or bioprosthetic valves (11%) using NOACs (rivaroxaban 43%; apixaban 39%; dabigatran 12%; edoxaban 7%) were enrolled. The most common indication was AA (91%). Over a median follow-up of 1.0 [IQR 0.0–2.0] year, thromboembolic event rate was 1.0% [95%CI 0.4–2.0] (n = 6) per year, with 1.1% [95%CI 0.5–2.2] (n = 7) annualized rate of major bleeding and 6.3% [95%CI 4.5–8.5] (n = 37) annualized rate of minor bleeding. Adherence was sufficient during 2 years follow-up in 80–93% of patients. At 1-year follow-up, among the subset of previous VKA-users who completed the survey (n = 33), QoL improved in 6 out of 8 domains (p ≪ 0.05). Conclusions: Initial results from our worldwide prospective study suggest that NOACs are safe and may be effective for thromboembolic prevention in adults with heterogeneous forms of congenital heart disease.SCOPUS: ar.jinfo:eu-repo/semantics/publishe