Cardiac Reintervention Research Articles

Successful Implementation of Enhanced Recovery After Surgery (ERAS) in Paediatric Cardiac Surgery in Australia

Fast-track or enhanced recovery after surgery (ERAS) is a care pathway for surgical patients based on a multidisciplinary team approach aimed at optimising recovery without increasing risk with protocols based on scientific evidence, which is monitored continuously to ensure compliance and improvement. These protocols have been shown to reduce the duration of postoperative mechanical ventilation and intensive care unit (ICU) length of stay (LOS) following paediatric cardiac surgery. We present the first structured implementation of ERAS in paediatric cardiac surgery in Australia. All patients enrolled in the ERAS pathway between October 2019 and July 2023 were identified. Demographic and perioperative data were collected retrospectively from hospital records for patients operated before June 2021 and prospectively from June 2021. A control group (non-ERAS) was identified using propensity matching from patients who underwent similar procedures and were not enrolled in the ERAS pathway (prior to October 2019). Patients were matched for age, weight, and comprehensive Aristotle score. Outcomes of interest were duration of postoperative mechanical ventilation, ICU LOS, readmission to the ICU, hospital LOS, cardiac reintervention rate, postoperative complication rate, and number of 30-day readmissions. Of 1,084 patients who underwent cardiac surgery during the study period (October 2019-July 2023), 121 patients (11.2%) followed the ERAS pathway. The median age at the time of surgery was 4.8 years (interquartile range [IQR] 2.8-8.8 years). The most common procedure was the closure of atrial septal defect (n=58, 47.9%). The median cardiopulmonary bypass and cross-clamp times were 40 min (IQR 28-53.5 minutes) and 24.5 min (IQR 13-34 minutes) respectively. The majority were extubated in the operating theatre (n=108, 89.3%). The median ICU and hospital LOS were 4.5 hrs (IQR 4.1-5.6 hours) and 4 days (IQR 4-5 days) respectively. None of the patients required readmission to the ICU within 24 hrs of discharge from the ICU. Three (3) patients (2.5%) required reintervention. When compared with the non-ERAS group, the duration of postoperative mechanical ventilation, ICU and hospital LOS were significantly lower in the ERAS group. There was no significant difference in the ICU readmission rate, reintervention rate, complication rate, and number of 30-day readmissions between both groups. ERAS after paediatric cardiac surgery is feasible and safe in select patients with low preoperative risk. This pathway reduces the duration of postoperative mechanical ventilation, ICU and hospital LOS without increasing risks, enabling the optimisation of resources.

Long term outcome after surgical ASD-closure at young age: Longitudinal follow-up up to 50 years after surgery

ObjectivesTo describe the clinical outcome and quality of life up to 50 years after surgical atrial septal defect (ASD) closure at young age. Primary outcome is defined as MACE (all-cause mortality, cardiac re-interventions, ischemic stroke, endocarditis, heart failure and symptomatic arrhythmia). MethodsSingle-center, longitudinal cohort-study evaluating 135 consecutive patients who underwent ASD-closure before the age of 15 years between 1968 and 1980. Participants were invited for extensive cardiac evaluation and assessment of quality-of-life every 10 years. ResultsEighty patients (86%) of 93 eligible survivors were included in this study (mean age 52 ± 5 years (range 41–63), 40% male). Median follow-up since surgery was 45 years (range 40–51). Cumulative survival after 50 years was 86% and comparable to the normal Dutch population. Cumulative event-free survival after 45 and 50-years was 59% and 46% respectively (re-intervention in 6, symptomatic arrhythmia in 25, and pacemaker implantation in 10 patients). Right ventricular ejection fraction on CMR was diminished in 6%. Exercise capacity was normal in 77%. There was no pulmonary hypertension. NT-proBNP was elevated in 61%. Quality of life was comparable with the general population. No predictors for late events were identified. ConclusionLong-term survival after surgical ASD-closure in childhood is good and not statistically different at 50 years compared to the normal Dutch population. Re-intervention rate is low, there is no pulmonary hypertension. Right ventricular function was diminished in 6%, exercise capacity was good and stable over time with quality of life comparable to the general population. However, supraventricular tachycardia is common.

Racial and Ethnic Disparities in Cardiac Reintervention After Pediatric Cardiac Surgical Procedures

BackgroundChildren undergoing cardiac surgical procedures may require postoperative surgical or catheter-based reintervention before discharge. We examined racial/ethnic variations in reintervention and associated in-hospital death. MethodsChildren undergoing cardiac surgical procedures from 2004 to 2015 were identified in the Pediatric Health Information Systems (PHIS) database. Regression analysis measured associations between race/ethnicity, in-hospital death, and postoperative cardiac surgical or catheter-based reintervention (surgical/catheter reintervention). ResultsOf 124,263 patients, 8265 (6.7%) had a surgical/catheter reintervention. Black patients had fewer reinterventions (5.9% vs 6.7%) and higher in-hospital mortality (3.9% vs 2.7%, P < .01) than White patients. After adjusting for sociodemographic and illness severity indicators, Black patients remained less likely to receive surgical/catheter reintervention (adjusted hazard ratio [aHR], 0.89; 95% CI, 0.82-0.98) despite having similar risk of death after reintervention (adjusted odds ratio, 1.17; 95% CI, 0.98-1.41) compared with White patients. The risk of death without surgical/catheter reintervention was also higher for Black (aHR, 1.26; 95% CI, 1.08-1.47) and other race/ethnicity (aHR, 1.33; 95% CI, 1.13-1.57) patients than for White patients. Similar trends were demonstrated when mechanical circulatory support and cardiac transplantation were included as reinterventions. ConclusionsPatients of Black and other race/ethnicity undergoing pediatric cardiac surgical procedures are more likely to die without postoperative cardiac reintervention than White patients. Black patients are also less likely to receive reintervention despite no significant difference in mortality with reintervention. Further studies should evaluate etiologies and methods of addressing these disparities.

Prevalence of Pericardial Late Gadolinium Enhancement in Patients After Cardiac Surgery: Clinical and Histological Correlations.

Pericardial late gadolinium enhancement (LGE) is usually associated with active pericarditis, but it is not infrequently found in patients after cardiac surgery even a long time after the intervention. The clinical relevance of this finding and its histological correlates are unknown. We sought to determine the prevalence of chronic pericardial LGE in patients after cardiac surgery. All consecutive patients with previous cardiac surgery, who were referred to cardiovascular magnetic resonance between January 2017 and December 2021 were enrolled in the study. Cardiovascular magnetic resonance examination protocol was adapted to clinical indication but always included standard LGE acquisitions. Two independent observers blinded to clinical data assessed the presence of pericardial enhancement on LGE sequences. Fifteen patients underwent cardiac reintervention and pericardial biopsies were obtained. The primary study end point was to assess the prevalence of pericardial enhancement after cardiac surgery and identify possible determinants. The secondary end point was to correlate pericardial enhancement with clinical symptoms and histopathology. Two hundred four patients were included in the study. The median time between surgery and cardiovascular magnetic resonance was 160 months (35-226 months). Pericardial LGE was observed in 90 patients (44%). All patients were asymptomatic, and no specific treatment for pericarditis was started. All patients remained asymptomatic at a 1-year clinical follow-up. Pericardial LGE was significantly correlated with the number of previous surgeries (P=0.03). Pericardial fibrosis was detected in all 15 pericardial biopsy specimens; pericardial LGE was present in 7 patients (47%) who underwent biopsy. Histological signs of low-grade inflammation were detected in 6 patients (40%) with severe, circumferential pericardial LGE but in no patient without pericardial enhancement. Pericardial LGE is a frequent finding even several years after cardiac surgery. Its histological correlate is a chronic subclinical post-pericardiotomy inflammation.

Arterial Switch for Transposition of the Great Arteries: Treatment Timing, Late Outcomes, and Risk Factors

BackgroundReports of long-term mortality and reintervention after transposition of the great arteries with intact ventricular septum treatment, although favorable, are mostly limited to single-center studies. Even less is known about hospital resource utilization (days at hospital) and the impact of treatment choices and timing on outcomes. ObjectivesThe purpose of this study was to describe survival, reintervention and hospital resource utilization after arterial switch operation (ASO) in a national dataset. MethodsFollow-up and life status data for all patients undergoing ASO between 2000 and 2017 in England and Wales were collected and explored using multivariable regressions and matching. ResultsA total of 1,772 patients were identified, with median ASO age of 9.5 days (IQR: 6.5-14.5 days). Mortality and cardiac reintervention at 10 years after ASO were 3.2% (95% CI: 2.5%-4.2%) and 10.7% (95% CI: 9.1%-12.2%), respectively. The median time spent in hospital during the ASO spell was 19 days (IQR: 14, 24). Over the first year after the ASO patients spent 7 days (IQR: 4-10 days) in hospital in total, decreasing to 1 outpatient day/year beyond the fifth year. In a subgroup with complete risk factor data (n = 652), ASO age, and balloon atrial septostomy (BAS) use were not associated with late mortality and reintervention, but cardiac or congenital comorbidities, low weight, and circulatory/renal support at ASO were. After matching for patient characteristics, BAS followed by ASO and ASO as first procedure, performed within the first 3 weeks of life, had comparable early and late outcomes, including hospital resource utilization. ConclusionsMortality and hospital resource utilization are low, while reintervention remains relatively frequent. Early ASO and individualized use of BAS allows for flexibility in treatment choices and a focus on at-risk patients.

The Evolution of Longitudinal Strain Across Various Univentricular Morphologies Prior to Superior Cavopulmonary Anastomosis

Infants with single-ventricle physiology have increased morbidity, mortality, and ventricular dysfunction in the pre-superior cavopulmonary anastomosis (pre-SCPA) period. Echocardiography-derived longitudinal strain (LS) is emerging as a reliable marker of single-ventricle performance. We aim to assess evolution of LS during the pre-SCPA period across univentricular morphologies and explore associations of LS with modifiable and nonmodifiable factors. Ninety-four term infants (36 females) with univentricular physiology who were discharged home prior to stage 2 palliation were serially analyzed for LS (single apical view) and other echo measures at initial hospital discharge and the last pre-SCPA encounter. Ventricular myocardium was tracked for strain along the septum and respective lateral wall for single right ventricular (RV) and left ventricular (LV) groups, and along both right and left lateral walls in functionally univentricular hearts with biventricular (BiV) morphology. Clinical data were obtained from the medical record. Longitudinal strain improved in the total cohort during the pre-SCPA period (16.48% ± 3.31% to 17.57% ± 3.81%, P= .003). Longitudinal strain improved between encounters in the single LV (P= .04) and BiV groups (P= .02). However, LS failed to improve in the RV group (P= .7) with lower LS at both visits compared with the other groups. The RV group, mostly composed of hypoplastic left heart syndrome patients-87% of cases-had a higher incidence of arrhythmias (57%) and unplanned reinterventions (60%), most of which were arch reinterventions. A subanalysis based on arch reintervention showed that LS improves between encounters in the single LV group needing arch reintervention (P= .05) compared with the single RV group needing arch reintervention (P= .89). Lower LS was independently associated with unplanned reinterventions at both encounters (P= .008 and .02). Single-ventricle LS evolves differently across ventricular morphologies during the pre-SCPA period and is related to the need for unplanned cardiac reinterventions. Lower LS is noted in the single RV group, who mostly have hypoplastic left heart syndrome.

Staged vs Complete Repair in Tetralogy of Fallot With Pulmonary Atresia

BackgroundWe sought to compare outcomes for infants with tetralogy of Fallot with pulmonary atresia (TOF/PA) and confluent pulmonary arteries who underwent staged or primary complete surgical repair. MethodsThis retrospective study included infants undergoing initial surgical intervention between 0 and 60 days of age with TOF/PA without aortopulmonary collaterals from 2009 to 2018 at 20 centers. The primary outcome was days alive and out of the hospital in the first year of life (DAOH365). Secondary outcomes were mortality at 1 year of age and a composite major complication outcome. Multivariable modeling with generalized estimating equations were used to compare outcomes between groups. ResultsOf 221 subjects, 142 underwent staged repair and 79 underwent primary complete repair. There was no significant difference in median DAOH365 between the staged and primary repair groups (317 days [interquartile range, 278-336] vs 338 days [interquartile range, 314-348], respectively; adjusted P = .13). Nine staged repair patients (7%) died in the first year of life vs 5 primary repair patients (6%; adjusted odds ratio, 1.00; 95% CI, 0.25-3.95). At least 1 major complication occurred in 37% of patients who underwent staged repair vs 41% of patients who underwent primary complete repair (P = .75), largely driven by the need for unplanned cardiac reinterventions. ConclusionsFor infants with TOF/PA with confluent pulmonary arteries, a surgical strategy of staged or primary complete repair resulted in statistically similar DAOH365, early mortality, and morbidity.

Is Anticoagulation Warranted after Left Atrial Appendage Ligation in Patients at Risk for Stroke after Cardiac Surgery?

Objectives: Left atrial appendage ligation (LAAL) may constitute alternative stroke prophylaxis in patients with atrial fibrillation (AF). Herein we describe the 30-day post discharge outcomes of cardiac surgery patients with elevated stroke risk with or without anticoagulation (AC) following epicardial LAAL. Methods: Data were reviewed for 479 consecutive adult patients who underwent epicardial LAAL from 2014-2019 (median CHA2DS2-VASc score = 4.0). There were 251 and 228 patients discharged with and without AC, respectively, who were followed for 30 days. Patients were matched via 1:1 Propensity Score Matching (PSM; n = 115 per group). Post-discharge outcomes included stroke, bleeding, readmission for cardiac re-intervention, mortality, and a composite endpoint comprised of the aforementioned outcomes. Results: There was no difference in post-discharge stroke incidence regardless of AC (adjusted cumulative incidence (ACI) 0.009 CI [0.001 - 0.043] with AC vs 0.009 CI [0.001 - 0.43] without AC; p = 0.826), post-discharge bleeding (ACI 0.018 CI [0.003 - 0.057] with AC vs 0.009 CI [0.001 - 0.046] without AC; p = 0.738), readmission for cardiac re-intervention (ACI 0.009 CI [0.009 - 0.009] with AC vs 0 CI [NA] without AC; p = 0.340, post-discharge mortality (ACI 0 CI NA with AC vs 0.009 CI [0.001 - 0.046] without AC; p = 0.123, or in the composite outcome (ACI 0.026 CI [0.007 - 0.069] with AC vs 0.027 CI [0.007 - 0.071] without AC; p = 0.824. Conclusion: Cessation of AC in patients with elevated stroke risk following epicardial LAAL during cardiac surgery does not affect stroke rate, mortality, or bleeding incidence up to 30 days post-discharge in this preliminary analysis.

Timing of surgical repair and resource utilisation in infants with complete atrioventricular septal defect.

Variation exists in the timing of surgery for balanced complete atrioventricular septal defect repair. We sought to explore associations between timing of repair and resource utilisation and clinical outcomes in the first year of life. In this retrospective single-centre cohort study, we included patients who underwent complete atrioventricular septal defect repair between 2005 and 2019. Patients with left or right ventricular outflow tract obstruction and major non-cardiac comorbidities (except trisomy 21) were excluded. The primary outcome was days alive and out of the hospital in the first year of life. Included were 79 infants, divided into tertiles based on age at surgery (1st = 46 to 137 days, 2nd = 140 - 176 days, 3rd = 178 - 316 days). There were no significant differences among age tertiles for days alive and out of the hospital in the first year of life by univariable analysis (tertile 1, median 351 days; tertile 2, 348 days; tertile 3, 354 days; p = 0.22). No patients died. Fewer post-operative ICU days were used in the oldest tertile relative to the youngest, but days of mechanical ventilation and hospitalisation were similar. Clinical outcomes after repair and resource utilisation in the first year of life were similar for unplanned cardiac reinterventions, outpatient cardiology clinic visits, and weight-for-age z-score at 1 year. Age at complete atrioventricular septal defect repair is not associated with important differences in clinical outcomes or resource utilisation in the first year of life.

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

ObjectiveThere 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. MethodsThis is a retrospective, multicentric, observational study. Clinical, anatomy, procedural, and detailed follow-up data (median, 4.43 years) were collected. ResultsA 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). ConclusionsBoth 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.

Factors associated with caregiver adherence to mobile health interstage home monitoring in infants with single ventricle or biventricular shunt-dependent heart disease.

Mobile health technology is an emerging tool in interstage home monitoring for infants with single ventricle heart disease or biventricular shunt-dependent defects. This study sought to describe adherence to mobile health monitoring and identify factors and outcomes associated with adherence to mobile health monitoring. This was a retrospective, single-institution study of infants who were followed in a mobile health-based interstage home monitoring programme between February 2016 and October 2020. The analysis included 105 infants and subjects were grouped by frequency of adherence to mobile health monitoring. Within the study cohort, 16 (15.2%) had 0% adherence, 25 (23.8%) had <50% adherence, and 64 (61.0%) had >50% adherence. The adherent groups had a higher percentage of infants who were male (p = 0.02), white race (p < 0.01), non-Hispanic or non-Latinx ethnicity (p < 0.01) and had mothers with primary English fluency (p < 0.01), married marital status (p < 0.01), and a prenatal diagnosis of faetal cardiac disease (p = 0.03). Adherent groups also had a higher percentage of infants with non-Medicaid primary insurance (p < 0.01) and residence in a neighbourhood with a higher median household income (p < 0.04). Frequency of adherence was not associated with interstage mortality, unplanned cardiac reinterventions, or hospital readmissions. Impact of mobile health interstage home monitoring on caregiver stress as well as use of multi-language, low literacy, affordable mobile health options for interstage home monitoring warrant further investigation.

Age at surgery and outcomes following neonatal cardiac surgery: An analysis from the Pediatric Cardiac Critical Care Consortium

ObjectiveThe optimal timing for neonatal cardiac surgery is a potentially modifiable factor that may affect outcomes. We studied the relationship between age at surgery (AAS) and outcomes across multiple hospitals, focusing on neonatal operations where timing appears is not emergency. MethodsWe studied neonates ≥37 weeks' gestation and ≥2.5 kg admitted to a treating hospital on or before day of life 2 undergoing selected index cardiac operations. The impact of AAS on outcomes was evaluated across the entire cohort and a standard risk subgroup (ie, free of preoperative mechanical ventilation, mechanical circulatory support, or other organ failure). Outcomes included mortality, major morbidity (ie, cardiac arrest, mechanical circulatory support, unplanned cardiac reintervention, or neurologic complication), and postoperative cardiac intensive care unit and hospital length of stay. Post hoc analyses focused on operations undertaken between day of life 2 and 7. ResultsWe studied 2536 neonates from 47 hospitals. AAS from day of life 2 through 7 was not associated with risk adjusted mortality or major morbidity among the entire cohort and the standard risk subgroup. Older AAS, although associated with modest increases in postoperative cardiac intensive care unit and hospital length of stay in the entire cohort, was not associated with hospital length of stay in the standard risk subgroup. ConclusionsAmong select nonemergency neonatal cardiac operations, AAS between day of life 2 and 7 was not found to be associated with risk adjusted mortality or major morbidity. Although delays in surgical timing may modestly increase preoperative resource use, studies of AAS and outcomes not evident at the time of discharge are needed.

Long-term results of coronary surgery with endoscopic vein harvesting.

Background In this study, we aimed to evaluate early and longterm outcomes of both isolated or concomitant coronary artery bypass grafting with the endoscopic vein harvesting technique. Methods Between November 2012 and May 2017, a total of 324 patients (259 males, 65 females; mean age: 63.2±9.8 years; range, 36 to 91 years) who underwent coronary artery bypass grafting, with or without concomitant procedures, using the endoscopic vein harvesting technique were retrospectively analyzed. Early postoperative outcomes and long-term follow-up data of the patients, such as cardiovascular or cerebral events, cardiac reinterventions, and the images of coronary angiography were recorded. Results Median logistic EuroSCORE and in hospital mortality was 3.99 (0.8-81) vs. 0 .9% f or i solated c oronary surgery and 13.34 (1.5-76.4) vs. 1.5% for concomitant procedures. The long-term data could be obtained in 288 patients with a median of 59.6 (7-90) months of follow-up. During this period, 22 (7.6%) patients underwent coronary angiography for control or treatment, 12 (4.2%) patients needed revascularization, and none of the patients underwent redo coronary surgery. Conclusion Our study results suggest that the endoscopic vein harvesting technique during coronary artery bypass grafting is safe in experienced hands.

Use of carotid artery cannulation during redo sternotomy in congenital cardiac surgery: a single-centre experience.

Management of resternotomy is often a difficult challenge in patients with congenital diseases who have undergone multiple sternotomies. Our goal was to report our single-centre experience with carotid cannulation using a graft interposed during redo sternotomy in complex cardiac reintervention procedures. We performed a retrospective review of all patients who had undergone complex redo sternotomies between January 2019 and May 2020 utilizing a cervical cannulation technique with a Gore-Tex graft interposed on the carotid artery. We classified our population study on the basis of the primary diagnosis and the type of surgery. The primary outcomes of our analysis were the evaluation of the safety of the procedure in terms of survival and freedom from neurological events. We analysed 22 patients who had undergone previous complex operations. The median age and weight at the time of reintervention were 130.35 (range 0.46-435) months and 31.5 (range 2.2-85) kg, respectively. Composite graft carotid cannulation provided adequate arterial flow in all patients with a median arterial flow of 3.5 l/min/m2 (range 0.6-6). One major cardiac injury occurred during sternotomy when emergency cardiopulmonary bypass (CPB) was initiated. Moreover, during their hospital stays, all patients had an uneventful recovery without neurological or vascular complications and no cervical wound infections. Carotid cannulation using interposition of a side graft on the common carotid artery for arterial inflow is a reliable and safe method for initiation of CPB in complex redo surgeries in patients with congenital disease. Complications directly associated with this type of cannulation are uncommon and allow surgical re-entry with overall low risks.

Adverse fibrosis remodeling and aortopulmonary collateral flow are associated with poor Fontan outcomes

BackgroundThe extent and significance in of cardiac remodeling in Fontan patients are unclear and were the subject of this study.MethodsThis retrospective cohort study compared cardiovascular magnetic resonance (CMR) imaging markers of cardiac function, myocardial fibrosis, and hemodynamics in young Fontan patients to controls.ResultsFifty-five Fontan patients and 44 healthy controls were included (median age 14 years (range 7–17 years) vs 13 years (range 4–14 years), p = 0.057). Fontan patients had a higher indexed end-diastolic ventricular volume (EDVI 129 ml/m2 vs 93 ml/m2, p < 0.001), and lower ejection fraction (EF 45% vs 58%, p < 0.001), circumferential (CS − 23.5% vs − 30.8%, p < 0.001), radial (6.4% vs 8.2%, p < 0.001), and longitudinal strain (− 13.3% vs − 24.8%, p < 0.001). Compared to healthy controls, Fontan patients had higher extracellular volume fraction (ECV) (26.3% vs 20.6%, p < 0.001) and native T1 (1041 ms vs 986 ms, p < 0.001). Patients with a dominant right ventricle demonstrated larger ventricles (EDVI 146 ml/m2 vs 120 ml/m2, p = 0.03), lower EF (41% vs 47%, p = 0.008), worse CS (− 20.1% vs − 25.6%, p = 0.003), and a trend towards higher ECV (28.3% versus 24.1%, p = 0.09). Worse EF and CS correlated with longer cumulative bypass (R = − 0.36, p = 0.003 and R = 0.46, p < 0.001), cross-clamp (R = − 0.41, p = 0.001 and R = 0.40, p = 0.003) and circulatory arrest times (R = − 0.42, p < 0.001 and R = 0.27, p = 0.03). T1 correlated with aortopulmonary collateral (APC) flow (R = 0.36, p = 0.009) which, in the linear regression model, was independent of ventricular morphology (p = 0.9) and EDVI (p = 0.2). The composite outcome (cardiac readmission, cardiac reintervention, Fontan failure or any clinically significant arrhythmia) was associated with increased native T1 (1063 ms vs 1026 ms, p = 0.029) and EDVI (146 ml/m2 vs 118 ml/m2, p = 0.013), as well as decreased EF (42% vs 46%, p = 0.045) and worse CS (− 22% vs − 25%, p = 0.029). APC flow (HR 5.5 CI 1.9–16.2, p = 0.002) was independently associated with the composite outcome, independent of ventricular morphology (HR 0.71 CI 0.30–1.69 p = 0.44) and T1 (HR1.006 CI 1.0–1.13, p = 0.07).ConclusionsPediatric Fontan patients have ventricular dysfunction, altered myocardial mechanics and increased fibrotic remodeling. Cumulative exposure to cardiopulmonary bypass and increased aortopulmonary collateral flow are associated with myocardial dysfunction and fibrosis. Cardiac dysfunction, fibrosis, and collateral flow are associated with adverse outcomes.

Associations Between Blood Biomarkers, Cardiac Function, and Adverse Outcome in a Young Fontan Cohort.

BackgroundPatients who have undergone the Fontan procedure are at high risk of circulatory failure. In an exploratory analysis we aimed to determine the prognostic value of blood biomarkers in a young cohort who have undergone the Fontan procedure.Methods and ResultsIn multicenter prospective studies patients who have undergone the Fontan procedure underwent blood sampling, cardiopulmonary exercise testing, and stress cardiac magnetic resonance imaging. Several biomarkers including NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide), GDF‐15 (growth differentiation factor 15), Gal‐3 (galectin‐3), ST2 (suppression of tumorigenicity 2), DLK‐1 (protein delta homolog 1), FABP‐4 (fatty acid‐binding protein 4), IGFBP‐1 (insulin‐like growth factor‐binding protein 1), IGFBP‐7, MMP‐2 (matrix metalloproteinase 2), and vWF (von Willebrand factor) were assessed in blood at 9.6 (7.1–12.1) years after Fontan completion. After this baseline study measurement, follow‐up information was collected on the incidence of adverse cardiac events, including cardiac death, out of hospital cardiac arrest, heart transplantation (listing), cardiac reintervention (severe events), hospitalization, and cardioversion/ablation for arrhythmias was collected and the relation with blood biomarkers was assessed by Cox proportional hazard analyses. The correlation between biomarkers and other clinical parameters was evaluated. We included 133 patients who have undergone the Fontan procedure, median age 13.2 (25th, 75th percentile 10.4–15.9) years, median age at Fontan 3.2 (2.5–3.9) years. After a median follow‐up of 6.2 (4.9–6.9) years, 36 (27.1%) patients experienced an event of whom 13 (9.8%) had a severe event. NT‐proBNP was associated with (all) events during follow‐up and remained predictive after correction for age, sex, and dominant ventricle (hazard ratio, 1.89; CI, 1.32–2.68). The severe event‐free survival was better in patients with low levels of GDF‐15 (P=0.005) and vWF (P=0.008) and high levels of DLK‐1 (P=0.041). There was a positive correlation (β=0.33, P=0.003) between DLK‐1 and stress cardiac magnetic resonance imaging functional reserve.ConclusionsNT‐proBNP, GDF‐15, vWF, DLK‐1, ST‐2 FABP‐4, and IGFBP‐7 levels relate to long‐term outcome in young patients who have undergone the Fontan procedure.

Surgical management of complete atrioventricular septal defect with tetralogy of Fallot: results from a national audit database

Abstract Background Complete atrioventricular septal defect (CAVSD) with tetralogy of Fallot (CAVSD-ToF) is an uncommon association, and the management is more difficult than for each lesion in isolation. Depending on the anatomy, clinical status and ventricular morphology, definitive repair is offered either as staged or single operation with few undergoing single or one and a half ventricle procedures. Currently available data are limited to single centre studies. Purpose We aim to use data from a national audit dataset to describe outcomes in the surgical management of CAVSD-ToF with data from the national audit. Methods All children with CAVSD and tetralogy of Fallot (including double outlet right ventricle and pulmonary atresia variants) undergoing the first cardiac procedure between 2000–2013 were included and all subsequent procedures extracted. Three treatment pathways were defined: definitive primary procedure (“primary complete repair”), staged repair or “unbalanced ventricles repair” (Glenn shunt or modified Fontan). Continuous data is expressed as median (range). Survival data obtained using the Kaplan Meier method and subgroup comparisons done using the log-rank test. Results A total of 125 patients were included, 50.4% male, first procedure at 150 (1–2770) days. Down syndrome was present in 79.4% (63/79 of those with reported non-cardiac comorbidities), while 8% (10/125) had heterotaxy syndrome. “Primary complete repair” was performed in 40% (n=50), at 319 (33–2770) days. Staged repair was undertaken in 28% (n=35), first palliation at 80 (1–733) days. An “unbalanced ventricles” approach was used in 16.8% (n=21), first procedure at 135 (1–1742) days). The other 15.2% (n=19), first procedure at 53 (range 1–436) days, had at least one palliation procedure, but no identifiable committed pathway. Survival at 5 years from the first procedure in “primary complete repair” compared to the remainder of the group is shown in the Figure. No significant differences in survival were found. Specifically in the “unbalanced ventricle repair” group, just 1 patient died during follow-up. A total of 85 patients (68%) achieved either primary or staged “complete repair”. Early mortality (30 day) after the “complete repair” was 6.6% (similar for both approaches, p=0.9). Survival at 5 years after “complete repair” was 82.7% (similar for both approaches, p=0.8). Freedom from any cardiac re-intervention at 5 years was 69.5% (77.6% after primary, 58.1% after staged repair, p=0.03). Conclusion Early-life mortality in this heterogeneous group of CAVSD-ToF remains high, even in patients receiving definitive “complete repair”. We found no survival differences in the medium term between primary repair and a palliation approach, but more cardiac re-interventions were undertaken after staged complete repair. Surprisingly good results were seen in the unbalanced ventricles group, but longer follow up is needed to examine their attrition and longer term outcome. Survival at 5 years by treatment option Funding Acknowledgement Type of funding source: None

Comprehensive Long-Term Follow up of Adults with Arterial Switch Operation – European Collaboration for Prospective Outcome Research in Congenital Heart disease (EPOCH-ASO)–Study Design and Protocols

Background: Long-term outcomes in adults with prior arterial switch operation (ASO) have not yet been well defined. The aim of this study is to elucidate incidence and predictors of adverse cardiac outcomes in a prospectively followed cohort of adults after their ASO. Methods: The comprehensive long-term follow up of adults with ASO is a project within the European collaboration for prospective outcome research in congenital heart disease (EPOCH). It is designed as a prospective, international multicenter cohort study. Consecutive patients (age ≥ 16 years) with prior ASO will be included at 11 European tertiary care centers. Participants will be followed according to a standardized protocol following international recommendations, including standardized protocols for imaging and for exercise testing. Results: Main outcome measures are all-cause and cardiac-related mortality, rate of cardiac re-intervention, neo-aortic dissection, myocardial infarction, stroke, infective endocarditis, sustained atrial and ventricular arrhythmias, new-onset or worsening pulmonary hypertension or heart failure. Secondary endpoints are frequency and progression of right ventricular outflow stenosis, neo-aortic root dilatation, neo-aortic valve regurgitation and ventricular dysfunction. The impact of demographic, anatomic (e.g., coronary artery anato-my) and functional variables on the above-mentioned outcomes, as well as quality of life and incidence of pregnancy related complications will also be assessed. Conclusion: The prospective, international, multicenter EPOCH-ASO study will provide a better understanding of adverse outcomes and their predictors in adults after ASO. The results of the EPOCH-ASO study may help to optimize future care of this novel patient cohort in adult cardiology.

Long-term follow-up after transatrial-transpulmonary repair of tetralogy of Fallot: influence of timing on outcome.

OBJECTIVESOur goal was to report the long-term serial follow-up after transatrial–transpulmonary repair of tetralogy of Fallot (TOF) and to describe the influence of the timing of the repair on outcome.METHODSWe included all patients with TOF who had undergone transatrial–transpulmonary repair between 1970 and 2012. Records were reviewed for patient demographics, operative details and events during the follow-up period (death, pulmonary valve replacement, cardiac reinterventions and hospitalization/intervention for arrhythmias). In patients with elective early primary repair of TOF after 1990, a subanalysis of the optimal timing of TOF repair was performed.RESULTSA total of 453 patients were included (63% male patients; 65% had transannular patch); 261 patients underwent primary elective repair after 1990. The median age at TOF repair was 0.7 years (25th–75th percentile 0.3–1.3) and decreased from 1.7 to 0.4 years from before 1990 to after 2000, respectively (P < 0.001). The median follow-up duration after TOF repair was 16.8 years (9.6–24.7). Events developed in 182 (40%) patients. In multivariable analysis, early repair of TOF (<6 months) [hazard ratio (HR) 3.06; P < 0.001] and complications after TOF repair (HR 2.18; P = 0.006) were found to be predictive for an event. In a subanalysis of the primary repair of TOF after 1990, the patients (n = 125) with elective early repair (<6 months) experienced significantly worse event-free survival compared to patients who had elective repair later (n = 136). In multivariable analysis, early repair (HR 3.00; P = 0.001) and postoperative complications (HR 2.12; P = 0.010) were associated with events in electively repaired patients with TOF.CONCLUSIONSTransatrial–transpulmonary repair of TOF before the age of 6 months may be associated with more events during the long-term follow-up period.

IS THE ROSS PROCEDURE A COST-EFFECTIVE ALTERNATIVE COMPARED TO MECHANICAL AORTIC VALVE REPLACEMENT IN NON-ELDERLY PATIENTS WITH AORTIC STENOSIS?

Controversy exists when managing young patients with aortic valve stenosis. Aortic valve replacement (AVR) with a mechanical prosthesis requires long-term anticoagulation and patients have an increased risk of thromboembolism and bleeding. The Ross procedure is proposed as an alternative, but it is associated with higher procedure costs and increased risk of cardiac re-interventions. We sought to determine the incremental cost per life year gained between the Ross procedure and mechanical AVR in patients under 55 years old with aortic stenosis.