Hospital resource utilization in a national cohort of functionally single ventricle patients undergoing surgical treatment.

Objective: The study objective was to provide a detailed overview of health resource use from birth to 18 years old for patients with functionally single ventricles and identify associated risk factors. Methods: All patients with functionally single ventricles treated between 2000 and 2017 in England and Wales were linked to hospital and outpatient records using data from the Linking AUdit and National datasets in Congenital HEart Services project. Hospital stay was described in yearly age intervals, and associated risk factors were explored using quantile regression. Results: A total of 3037 patients with functionally single ventricles were included, 1409 (46.3%) undergoing a Fontan procedure. During the first year of life, the median days spent in hospital was 60 (interquartile range, 37-102), mostly inpatient days, mirroring a mortality of 22.8%. This decreases to between 2 and 9 in-hospital days/year afterward. Between 2 and 18 years, most hospital days were outpatient, with a median of 1 to 5 days/year. Lower age at the first procedure, hypoplastic left heart syndrome/mitral atresia, unbalanced atrioventricular septal defect, preterm birth, congenital/acquired comorbidities, additional cardiac risk factors, and severity of illness markers were associated with fewer days at home and more intensive care unit days in the first year of life. Only markers of early severe illness were associated with fewer days at home in the first 6 months after the Fontan procedure. Conclusions: Hospital resource use in functionally single ventricle cases is not uniform, decreasing 10-fold during adolescence compared with the first year of life. There are subsets of patients with worse outcomes during their first year of life or with persistently high hospital use throughout their childhood, which could be the target of future research.

Protecting the stars of tomorrow: do international cardiovascular preparticipation screening policies account for the paediatric athlete? A systematic review and quality appraisal.

OBJECTIVE: (1) Identify and review current policies for the cardiovascular screening of athletes to assess their applicability to the paediatric population and (2) evaluate the quality of these policy documents using the Appraisal of Guidelines for Research & Evaluation II (AGREE II) tool. DESIGN: Systematic review and quality appraisal of policy documents. DATA SOURCES: A systematic search of PubMed, MEDLINE, Scopus, Web of Science, SportDiscus and CINAHL. ELIGIBILITY CRITERIA FOR SELECTING STUDIES: An article was included if it was a policy/position statement/guideline/consensus or recommendation paper relating to athletes and cardiovascular preparticipation screening. RESULTS AND SUMMARY: Of the 1630 articles screened, 13 met the inclusion criteria. Relevance to paediatric athletes was found to be high in 3 (23%), moderate in 6 (46%) and low in 4 (31%), and only 2 provide tailored guidance for the athlete aged 12-18 years. A median 5 related citations per policy investigated solely paediatric athletes, with study designs most commonly being retrospective (72%). AGREEII overall quality scores ranged from 25% to 92%, with a median of 75%. The lowest scoring domains were rigour of development; (median 32%) stakeholder involvement (median 47%) and Applicability (median 52%). CONCLUSION: Cardiac screening policies for athletes predominantly focus on adults, with few providing specific recommendations for paediatric athletes. The overall quality of the policies was moderate, with more recent documents scoring higher. Future research is needed in paediatric athletes to inform and develop cardiac screening guidelines, to improve the cardiac care of youth athletes.

Aortic root translocation and en bloc rotation of the outflow tracts surgery for complex forms of transposition of the great arteries and double outlet right ventricle: A multicenter study.

OBJECTIVE: There are several choices for the correction of complex transposition of the great arteries and double outlet right ventricle not amenable to the Rastelli-type surgery, but outcome data are limited to small series. This study aims to report results after the aortic root translocation and en bloc rotation of the outflow tract procedures. METHODS: This is a retrospective, multicentric, observational study. Clinical, anatomy, procedural, and detailed follow-up data (median, 4.43 years) were collected. RESULTS: A total of 70 patients (62.9% male; median age, 1 year; range 4 days to 12.4 years) were included: n = 43 in the aortic root translocation group and n = 27 in the en bloc rotation group. Those in the aortic root translocation group were older (P = .01) and more likely to have had previous procedures (P < .0001), but cardiac anatomy was similar in both groups. Aortic root translocation and en bloc rotation early mortality (30 days) was similar (4.7% vs 3.7%, P = .8). Late survival and freedom from any cardiac reintervention were 92.7% and 16.9% at 15 years overall, respectively. Freedom from right ventricular outflow tract/conduit reintervention was better in the en bloc rotation group than in the aortic root translocation group (100% vs 24.5%, P = .0003), but more patients in the en bloc rotation group had moderate (or worse) aortic valve regurgitation during follow-up (16% vs 2.6%, P = .07). CONCLUSIONS: Both aortic root translocation and en bloc rotation are valuable surgical options for the treatment of complex transposition of the great arteries and double outlet right ventricle. In the en bloc rotation group, there was better freedom from right ventricular outflow tract reinterventions, but a higher probability of aortic valve regurgitation. Identifying the main driving forces for these observed differences requires further study of these procedures.

The role of cardiopulmonary exercise testing in predicting mortality and morbidity in people with congenital heart disease: a systematic review and meta-analysis.

AIMS: The role of cardiopulmonary exercise testing (CPET) in predicting major adverse cardiovascular events (MACE) in people with congenital heart disease (ConHD) is unknown. A systematic review with meta-analysis was conducted to report the associations between CPET parameters and MACE in people with ConHD. METHODS AND RESULTS: Electronic databases were systematically searched on 30 April 2020 for eligible publications. Two authors independently screened publications for inclusion, extracted study data, and performed risk of bias assessment. Primary meta-analysis pooled univariate hazard ratios across studies. A total of 34 studies (18 335 participants; 26.2 ± 10.1 years; 54% ± 16% male) were pooled into a meta-analysis. More than 20 different CPET prognostic factors were reported across 6 ConHD types. Of the 34 studies included in the meta-analysis, 10 (29%), 23 (68%), and 1 (3%) were judged as a low, medium, and high risk of bias, respectively. Primary univariate meta-analysis showed consistent evidence that improved peak and submaximal CPET measures are associated with a reduce risk of MACE. This association was supported by a secondary meta-analysis of multivariate estimates and individual studies that could not be numerically pooled. CONCLUSION: Various maximal and submaximal CPET measures are prognostic of MACE across a variety of ConHD diagnoses. Further well-conducted prospective multicentre cohort studies are needed to confirm these findings.

Four-year outcomes after percutaneous coronary intervention of unprotected left main coronary artery disease in patients with stable angina and acute coronary syndrome.

Background. Percutaneous coronary intervention (PCI) of unprotected left main coronary artery disease (ULMCAD) have become a feasible and efficient alternative to coronary artery bypass surgery, especially in patients with acute coronary syndrome (ACS). There are limited data regarding early and late outcomes after ULMCAD PCI in patients with ACS and stable angina.The aim of this study was to compare early and four-year clinical outcomes in patients with ULMCAD PCI presenting as ACS or stable angina in a high-volume PCI center.Methods. We conducted a single center retrospective observational study, which included 146 patients with ULMCAD undergoing PCI between 2014 and 2018. Patients were divided in two groups: Group A included patients with stable angina (n = 70, 47.9%) and Group B patients with ACS (n = 76, 52.1%).Results. 30-day mortality was 8.22% overall, lower in Group A (1.43% vs 14.47%, p = 0.02). Mortality and major adverse cardiac events (MACE) rates at 4 years were significantly lower in Group A (9.64% vs 33.25%, p = 0.001, and 24.06% vs 40.11%, p = 0.012, respectively). Target lesion revascularization (TLR) at 4 year did not differ between groups (15% in Group A vs 12.76% in Group B, p = 0.5).Conclusions. In our study patients with ULMCAD and ACS undergoing PCI had higher early and long-term mortality and MACE rates compared to patients with stable angina, with similar TLR rate at 4-year follow-up.

Clinical and cost-effectiveness of the Ross procedure versus conventional aortic valve replacement in young adults.

Objectives: In young and middle-aged adults, there are three current options for aortic valve replacement (AVR), namely mechanical AVR (mechAVR), tissue AVR (biological AVR) and the Ross operation, with no clear guidance on the best option. We aim to compare the clinical effectiveness and cost-effectiveness of the Ross procedure with conventional AVR in young and middle-aged adults. Methods: This is a systematic literature review and meta-analysis of AVR options. Markov multistate model was adopted to compare cost-effectiveness. Lifetime costs, quality-adjusted life years (QALYs), net monetary benefit (NMB), population expected value of perfect information (EVPI) and expected value of partial perfect information were estimated. Results: We identified 48 cohorts with a total number of 12 975 patients (mean age 44.5 years, mean follow-up 7.1 years). Mortality, bleeding and thromboembolic events over the follow-up period were lowest after the Ross operation, compared with mechAVR and biological AVR (p<0.001). Aortic reoperation rates were lower after Ross compared with biological AVR, but slightly higher when compared with mechAVR (p<0.001). At a willingness-to-pay threshold of £20effective. At a willingness-to-pay threshold of £20, 000 per QALY000 per QALY, the Ross procedure is more cost-effective compared the Ross procedure is more cost-effective compared withwith conventional AVR, with a lifetime incremental NMB of £60 conventional AVR, with a lifetime incremental NMB of £60 952 (952 (££3030 236236 to to ££7979 464). Incremental costs were £12464). Incremental costs were £12 323 (323 (££61086108 to to ££1515 972) and incremental QALYs 3.66 (1.81972) and incremental QALYs 3.66 (1.81 to to 4.76). The population EVPI indicates that a trial costing up to £2.03 million could be cost 4.76). The population EVPI indicates that a trial costing up to £2.03 million could be cost--effective.At a willingness-to-pay threshold of £20 000 per QALY, the Ross procedure is more cost-effective compared with conventional AVR, with a lifetime incremental NMB of £60 952 (£30 236 to £79 464). Incremental costs were £12 323 (£6108 to £15 972) and incremental QALYs 3.66 (1.81 to 4.76). The population EVPI indicates that a trial costing up to £2.03 million could be cost-effective. Conclusions: In young and middle-aged adults with aortic valve disease, the Ross procedure may confer greater quality of life and be more cost-effective than conventional AVR. A high-quality randomised trial could be warranted and cost-effective.

Surgical versus balloon valvotomy in neonates and infants: results from the UK National Audit.

Objective: There are conflicting data on choosing balloon aortic valvoplasty (BAV) or surgical aortic valvotomy (SAV) in neonates and infants requiring intervention for aortic valve stenosis. We aim to report the outcome of both techniques based on results from the UK national registry. Methods: This is a retrospective study, including all patients under 1 year undergoing BAV/SAV between 2000 and 2012. A modulated renewal approach was used to examine the effect of reinterventions on outcomes. Results: A total of 647 patients (488 BAV, 159 SAV, 292 neonates) undergoing 888 aortic valve procedures were included, with a median age of 40 days. Unadjusted survival at 10 years was 90.6% after initial BAV and 84.9% after initial SAV. Unadjusted aortic valve replacement (AVR) rate at 10 years was 78% after initial BAV and 80.3% after initial SAV. Initial BAV and SAV had comparable outcomes at 10 years when adjusted by covariates (p>0.4). AVR rates were higher after BAV and SAV reinterventions compared with initial valvoplasty without reinterventions (reference BAV, HR=3 and 3.8, respectively, p<0.001). Neonates accounted for 29/35 of early deaths after the initial procedure, without significant differences between BAV and SAV, with all late outcomes being worse compared with infants (p<0.005). Conclusions: In a group of consecutive neonates and infants, BAV and SAV had comparable survival and freedom from reintervention as initial procedures and when performed as reinterventions. These findings support a treatment choice based on patient characteristics and centre expertise, and further research into the best patient profile for each choice.

The Ross procedure versus prosthetic and homograft aortic valve replacement: a systematic review and meta-analysis.

OBJECTIVES: Young adults undergoing aortic valve replacement (AVR) have decreased life expectancy compared to matched controls. The Ross procedure aims to improve valve lifespan while avoiding anticoagulation. We prepared a systematic review and meta-analysis to assess the Ross procedure compared to conventional AVR. METHODS: We searched MEDLINE, EMBASE and Cochrane CENTRAL for studies evaluating the Ross procedure versus any conventional AVR in adult patients. We performed screening, full-text assessment, risk of bias evaluation and data collection independently and in duplicate. We evaluated the risk of bias with the ROBINS-I and Cochrane tools and quality of evidence with the GRADE framework. We pooled data using the random- and fixed-effects models. RESULTS: Thirteen observational studies and 2 randomized controlled trials (RCTs) were identified (n = 5346). No observational study was rated as having low risk of bias. The Ross procedure was associated with decreased late mortality in observational and RCT data [mean length of follow-up 2.6 years, relative risk (RR) 0.56, 95% confidence interval (CI) 0.38-0.84, I2 = 58%, very low quality]. The RCT estimate of effect was similar (mean length of follow-up 8.8 years, RR 0.33, 95% CI 0.11-0.96, I2 = 66%, very low quality). No difference was observed in mortality <30 days after surgery. All-site reintervention was similar between groups in cohorts and significantly reduced by the Ross procedure in RCTs (RR 1.41, 95% CI 0.89-2.24, I2 = 55%, very low quality and RR 0.41, 95% CI 0.22-0.78, I2 = 68%, high quality, respectively). CONCLUSIONS: Observational data, with residual confounding, and RCT data suggest a late survival benefit with the Ross procedure with no increased risk of reintervention when compared to conventional AVR. Considering the quality of available evidence and limited follow-up, additional high-quality randomized studies are required to strengthen these findings. Systematic review PROSPERO registration: CRD42016052512.

Indications and results of systemic to pulmonary shunts: results from a national database.

OBJECTIVES: The systemic-to-pulmonary shunt (SPS) remains an important palliative therapy in many congenital heart defects. Unlike other surgical treatments, the mortality after shunt operations has risen. We used an audit dataset to investigate potential reasons for this change and to report national results. METHODS: A total of 1993 patients classified in 13 diagnoses underwent an SPS procedure between 2000 and 2013. Indication trends by era and also results before repair or next stage are reported. A dynamic hazard model with competing risks and modulated renewal was used to determine predictors of outcomes. RESULTS: The usage of SPS in Tetralogy of Fallot (ToF) has significantly decreased in the last decade, with cases of single ventricle (SV) and pulmonary atresia (PA) with septal communication increasing (P < 0.001 for trends). This is correlated with an increase of early mortality from 5.1% in the first half of the decade to 9.8% in the latter (P = 0.007 for trend). At 1.5 years, 13.9% of patients have died, 17.8% had a shunt reintervention and 68.3% of patients are alive and reintervention-free. Low weight, PA-intact septum, SV and central shunt type are among the factors associated with increased mortality, whereas PA-ventricular septal defect, corrected transposition, isomerism, central shunt and low weight are among those associated with increased reintervention, also having a dynamic effect on the relative risk when compared with ToF patients. Shunt reinterventions are not associated with worse outcomes when adjusted by other covariates, but they do have higher 30-day mortality if occurring earlier than 30 days from the index (P < 0.001). Patients operated in later years were found to have significantly lower survival at a distance from index. CONCLUSIONS: The observed historical rise in mortality for shunt operations relates to complex factors including changing practice for repair of ToF and for univentricular palliation. PA and SV patients are the groups of patients at the highest risk of death. Small size, shunt type and underlying anatomical defect are the main determinants of outcomes. Trends in indication and mortality seem to indicate that more severely ill patients benefit from shunting, but with an increase in mortality.

Outcomes following repair of anomalous coronary artery from the pulmonary artery in infants: results from a procedure-based national database.

BACKGROUND: Anomalous coronary artery from the pulmonary artery (ACAPA) is a very rare congenital anomaly that often occurs during infancy. Patients can present in a critical condition. METHODS: We analysed procedure-related data from a national audit database for the period 2000-2013. RESULTS: A total of 120 patients <1 year had repair of isolated ACAPA using a coronary transfer or the tunnel (Takeuchi) operation. Seven patients (6.8%) required a mitral valve procedure at index and eight patients (7.8%) had a mitral valve repair/replacement during follow-up, including mitral reoperations. Follow-up data (>30 days) were available in 102 patients and the mean follow-up time was 4.7 years. The 30-day overall mortality was 1.9%, higher for neonates (16.7% vs 1%, p=0.1) and after postoperative extracorporeal membrane oxygenation (ECMO) (20% vs 1%, p=0.09). At 10 years the survival estimate is 95.1%, freedom from coronary and mitral reintervention being 95.9% and 91.2%, respectively. Use of postoperative ECMO was a risk factor for long-term mortality (p<0.001). Risk factors for coronary reintervention were age under 30 days (p=0.06) and the need for postoperative ECMO (p=0.02). Age under 30 days (p=0.002) was a risk factor for mitral reintervention. CONCLUSIONS: To our knowledge this is the largest series to date. These preliminary national results show that early outcomes are good and medium-term attrition acceptable. Poor outcomes are correlated with early presentation, also with the need for postoperative circulatory support.