Impact of educational videos on maternal anxiety caused by children's heart surgery.

BACKGROUND: Mothers of children undergoing surgery for congenital heart disease experience significant stress. Therefore, finding a strategy to decrease this is vital. AIMS: To examine whether adding educational digital video discs to routine education can reduce maternal anxiety and depression when their children undergo heart surgery and when surgical or post-surgical complications occur. STUDY DESIGN: In a teaching hospital, 120 mothers whose children underwent elective heart surgery were randomly and equally divided into two groups: mothers receiving routine education plus a digital video disc before surgery and mothers receiving only routine education. Mothers' anxiety and depression levels were compared before education, after education (before surgery), and on discharge day. The effect of watching the digital video disc on maternal anxiety and depression on discharge day was evaluated for the participants whose children had surgical or post-surgical complications. RESULTS: Compared with only routine education, mothers' anxiety after education and on the discharge day decreased more if digital video disc was added. Depression decreased more after education, but no difference was found on the discharge day. Anxiety levels of mothers whose children had surgical or post-surgical complications on the discharge day decreased more if they watched the digital video disc, compared with those receiving only routine education (Beck anxiety inventory score 3.4 ± 1.9 and 6.1 ± 2.4 respectively; p-value .001). CONCLUSIONS: Compared with only routine education, adding digital video disc could decrease mothers' anxiety, and until the day of discharge. Compared with only routine education, adding digital video disc could decrease mothers' anxiety on the discharge day if their child had surgical or post-surgical complications. RELEVANCE TO CLINICAL PRACTICE: Adding educational digital video disc to routine education could decrease mothers' anxiety until the day of discharge. It could also decrease mothers' anxiety if their child had surgical or post-surgical complications.

The Characterization of Postoperative Mechanical Respiratory Requirement in Neonates and Infants Undergoing Cardiac Surgery on Cardiopulmonary Bypass in a Single Tertiary Institution.

OBJECTIVES: Although neonates and infants undergoing cardiac surgery on cardiopulmonary bypass (CPB) are at high risk of developing perioperative morbidity and mortality, including lung injury, the intraoperative profile of lung injury in this cohort is not well-described. Given that the postoperative course of patients in the pediatric cardiac surgical arena has become increasingly expedited, the objective of this study was to characterize the profiles of postoperative mechanical ventilatory support in neonates and infants undergoing cardiac surgery on CPB and to examine the characteristics of lung mechanics and lung injury in this patient population who are potentially amendable to early postoperative recovery in a single tertiary pediatric institution. DESIGN: A retrospective data analysis of neonates and infants who underwent cardiac surgery on cardiopulmonary bypass. SETTING: A single-center, university teaching hospital. PARTICIPANTS: The study included 328 neonates and infants who underwent cardiac surgery on cardiopulmonary bypass. INTERVENTIONS: A subset of 128 patients were studied: 58 patients undergoing ventricular septal defect (VSD) repair, 36 patients undergoing complete atrioventricular canal (CAVC) repair, and 34 patients undergoing bidirectional Glenn (BDG) shunt surgery. MEASUREMENTS AND MAIN RESULTS: Of the entire cohort, 3.7% experienced in-hospital mortality. Among all surgical procedures, VSD repair (17.7%) was the most common, followed by CAVC repair (11.0%) and BDG shunt surgery (10.4%). Of patients who underwent VSD repair, CAVC repair, and BDG shunt surgery, 65.5%, 41.7%, and 67.6% were off mechanical ventilatory support within 24 hours postoperatively, respectively. In all three of the surgical repairs, lung compliance decreased after CPB compared to pre-CPB phase. Sixty point three percent of patients with VSD repair and 77.8% of patients with CAVC repair showed a PaO2/FIO2 (P/F) ratio of <300 after CPB. Post- CPB P/F ratios of 120 for VSD patients and 100 for CAVC patients were considered as optimal cutoff values to highly predict prolonged (>24 hours) postoperative mechanical ventilatory support. A higher volume of transfused platelets also was associated with postoperative ventilatory support ≥24 hours in patients undergoing VSD repair, CAVC repair, and BDG shunt surgery. CONCLUSIONS: There was a high incidence of lung injury after CPB in neonates and infants, even in surgeries amendable for early recovery. Given that CPB-related factors (CPB duration, crossclamp time) and volume of transfused platelet were significantly associated with prolonged postoperative ventilatory support, the underlying cause of cardiac surgery-related lung injury can be multi-factorial.

Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry.

The Patient Registry for Adolescents and Adults with Stable Fontan Circulation aims to describe a contemporary cohort of Fontan patients who could be eligible for a clinical trial investigating macitentan, an endothelin receptor antagonist. This international, non-interventional, multicentre, cross-sectional, observational registry enrolled patients with "stable" Fontan circulation ≥10 years following extra-cardiac conduit or lateral tunnel procedure. Main exclusion criteria were NYHA functional class IV, reoperation of Fontan circulation, or signs of disease worsening. Patient characteristics at enrolment are described; available data were collected during a single registration visit. Of the 266 screened patients, 254 were included in this analysis. At enrolment, median (interquartile range) age was 24 (20;30) years, 37%/63% of patients were from the USA/Europe, 54% were male, 54%/47% had undergone extra-cardiac conduit/lateral tunnel procedures, and 95% were in NYHA functional class I or II. History of arrhythmia was more common in older patients and patients with lateral tunnel; overall prevalence was 19%. Most laboratory values were within the normal range but mean creatinine clearance was abnormally low (87.7 ml/min). Angiotensin-converting enzyme inhibitors were used by 48% of patients and their use was associated with creatinine clearance <90 ml/min (p = 0.007), as was Fontan completion at an older age (p = 0.007). 53.4% of patients had clinical characteristics that could potentially meet an endothelin receptor antagonist trial's eligibility criteria. The PREpArE-Fontan registry describes a cohort of patients who could potentially participate in an endothelin receptor antagonist trial and identified early subtle signs of Fontan failure, even in "stable" patients.

Regional lung metabolic profile in a piglet model of cardiopulmonary bypass with circulatory arrest.

INTRODUCTION: Acute lung injury is common following cardiopulmonary bypass and deep hypothermic circulatory arrest for congenital heart surgery with the most severe injury in the dorsocaudal lung. Metabolomics offers promise in deducing mechanisms of disease states, providing risk stratification, and understanding therapeutic responses in regards to CPB/DHCA related organ injury. OBJECTIVES: Using an infant porcine model, we sought to determine the individual and additive effects of CPB/DHCA and lung region on the metabolic fingerprint, metabolic pathways, and individual metabolites in lung tissue. METHODS: Twenty-seven infant piglets were divided into two groups: mechanical ventilation + CPB/DHCA (n = 20) and mechanical ventilation only (n = 7). Lung tissue was obtained from dorsocaudal and ventral regions. Targeted analysis of 235 metabolites was performed using HPLC/MS-MS. Data was analyzed using Principal Component Analysis (PCA), Partial Least Square Discriminant Analysis (PLS-DA), ANOVA, and pathway analysis. RESULTS: Profound metabolic differences were found in dorsocaudal compared to ventral lung zones by PCA and PLS-DA (R2 = 0.7; Q2 = 0.59; p < 0.0005). While overshadowed by the regional differences, some differences by exposure to CPB/DHCA were seen as well. Seventy-four metabolites differed among groups and pathway analysis revealed 20 differential metabolic pathways. CONCLUSION: Our results demonstrate significant metabolic disturbances between dorsocaudal and ventral lung regions during supine mechanical ventilation with or without CPB/DHCA. CPB/DHCA also leads to metabolic differences and may have additive effects to the regional disturbances. Most pathways driving this pathology are involved in energy metabolism and the metabolism of amino acids, carbohydrates, and reduction-oxidation pathways.

Spectrum and outcome of the use of noninvasive ventilation in a pediatric cardiac intensive care unit: A single-center experience.

Background and Aims: The pediatric cardiac intensive care unit (PCICU) frequently uses noninvasive ventilation (NIV). There are several reasons for its use, including prophylactic use right after the patient has been extubated. It is also used when patients are experiencing acute respiratory failure due to either cardiac or noncardiac reasons but are still able to maintain their airways. The objective of this study was to understand the spectrum of use of NIV following congenital cardiac surgery and analyze the outcome. Methods and Results: A retrospective observational study was conducted in a 14-bed PCICU, reviewing data from August 2019 to August 2022. Among 1750 congenital cardiac surgeries, 523 patients (29.9%) received NIV. The median age of the population was 2.5 months. Factors such as higher Risk-Adjusted Classification for Congenital Heart Surgery-1 category, longer intraoperative cardiopulmonary bypass time, and aortic cross-clamp time were associated with increased NIV use. Preoperative ventilator needs, infections, genetic syndromes, diaphragmatic paralysis, high vasoactive inotrope score (VIS) in the first 24 h, neonatal age, and weight <5 kg were independently associated with increased NIV need. The NIV group had a longer intensive care unit (ICU) stay compared to non-NIV patients. The success rate of NIV was 84%, with 440 successful cases and 83 failures. The mortality rate in the success and failure groups was not significantly different (5.27% vs. 6.0%). Conclusions: NIV is widely used in PCICU, but it is associated with longer ICU stays. It proves beneficial after congenital cardiac surgery, especially for patients with specific risk factors. However, NIV may not directly impact mortality rates, suggesting that other factors contribute to patient survival.

Surgical Strategies for Preservation of Pulmonary Valve Function in a Radical Operation for Tetralogy of Fallot: A Systematic Review and Meta-Analysis.

Objective: To evaluate the efficacy and safety of different surgical strategies to preserve pulmonary valve function. Surgical procedures evaluated include intraoperative balloon pulmonary valvuloplasty (IBPV), pulmonary valve reconstruction, and commissurotomy and pulmonary cusp augmentation (PCA) in patients who underwent a radical operation for Tetralogy of Fallot (ToF). Materials and Methods: The five databases searched in the current study included the Cochrane Library, PubMed, China National Knowledge Infrastructure, VIP, and WanFang data. A systematic search for control trials was performed in each database from the start date of each database until December 2021. The Newcastle-Ottawa Scale (NOS) was used to evaluate the quality of included studies. Results: A total of 15 retrospective studies with a total number of 1,396 participants were included in this study. In subgroup 1 (IBPV vs. TAP), patients undergoing IBPV had a less degree of regurgitation at 1-2 years after the surgery. The reintervention rate increased in the IBPV group at 5 years. In subgroup 2 (pulmonary valve reconstruction vs. TAP), the degree of regurgitation decreased in the pulmonary valve reconstruction group at 1 month after the surgery. In subgroup 3 (valve-sparing operation vs. TAP), the comparison demonstrated decreased rates for surgical mortality and reintervention at 5-10 years after the surgery. Conclusion: We proposed that pulmonary valve function in a radical operation for ToF was preserved. IBPV, pulmonary valve reconstruction, and commissurotomy and PCA can be performed during the surgical procedure based on the developmental status and anatomical characteristics of the right ventricular outflow tract (RVOT), pulmonary valve, and pulmonary artery. Systematic Review Registration: [https://www.crd.york.ac.uk/prospero/], identifier [CRD42022300987].

Haemodynamic determinants of improved aerobic capacity after tricuspid valve surgery in Ebstein anomaly.

BACKGROUND: Although tricuspid valve surgery improves functional capacity in patients with Ebstein anomaly, it is not always associated with improvement in aerobic capacity. The purpose of this study was to identify the determinants of improved aerobic capacity after tricuspid valve surgery in adults with Ebstein anomaly with severe tricuspid regurgitation. METHODS: Retrospective study of patients with severe tricuspid regurgitation due to Ebstein anomaly that had tricuspid valve surgery at Mayo Clinic Rochester (2000-2019) and had preoperative and postoperative cardiopulmonary exercise tests and echocardiograms. The patients were divided into aerobic capacity(+) and aerobic capacity(-) groups depending on whether they had postoperative improvement in %-predicted peak oxygen consumption (VO2). RESULTS: Of 76 patients with severe tricuspid regurgitation due to Ebstein anomaly, 28 (37%) and 48 (63%) were in aerobic capacity(+) and aerobic capacity(-) groups, respectively. The average improvement in peak VO2 was 2.1±1.4 mL/kg/min and -0.9±0.4 mL/kg/min in the in aerobic capacity(+) and aerobic capacity(-) groups, respectively. Although both groups had similar severity of residual tricuspid regurgitation, the aerobic capacity(+) group had more postoperative improvement in right atrial (RA) function, left atrial (LA) function and left ventricular preload and stroke volume. Of the preoperative variables analysed, RA reservoir strain (relative risk 1.12; 95% CI 1.06 to 1.18); LA reservoir strain (relative risk 1.09; 95% CI 1.04 to 1.14) and LV stroke volume index (OR 1.04; 95% CI 1.01 to 1.07) were predictors of postoperative improvement in peak VO2. CONCLUSIONS: One-third of patients with severe tricuspid regurgitation due to Ebstein anomaly had postoperative improvement in aerobic capacity, and atrial function indices were the best predictors of postoperative improvement in aerobic capacity. These data provide new insight into the haemodynamic determinants of exercise capacity and lay the foundation for further studies to determine whether postoperative improvement in aerobic capacity translates to improved long-term survival, and whether timing of tricuspid valve surgery based on these echocardiographic indices will improve long-term outcomes.

Predictors of low exercise cardiac output in patients with severe pulmonic regurgitation.

BACKGROUND AND OBJECTIVES: Chronic pulmonic regurgitation (PR) following repair of congenital heart disease (CHD) impairs right ventricular function that impacts peak exercise cardiac index (pCI). We aimed to estimate in a non-invasive way pCI and peak oxygen consumption (pVO2) and to evaluate predictors of low pCI in patients with significant residual pulmonic regurgitation after CHD repair. METHOD: We included 82 patients (median age 19 years (range 10-54 years)) with residual pulmonic regurgitation fraction >40%. All underwent cardiac MRI and cardiopulmonary testing with measurement of pCI by thoracic impedancemetry. Low pCI was defined <7 L/min/m2. RESULTS: Low pCI was found in 18/82 patients. Peak indexed stroke volume (pSVi) tended to compensate chronotropic insufficiency only in patients with normal pCI (r=-0.31, p=0.01). Below 20 years of age, only 5/45 patients had low pCI but near-normal (≥6.5 L/min/m2). pVO2 (mL/kg/min) was correlated with pCI (r=0.58, p=0.0002) only in patients aged >20 years. Left ventricular stroke volume in MRI correlated with pSVi only in the group of patients with low pCI (r=0.54, p=0.02). No MRI measurements predicted low pCI. In multivariable analysis, only age predicted a low pCI (OR=1.082, 95% CI 1.035 to 1.131, p=0.001) with continuous increase of risk with age. CONCLUSIONS: In patients with severe PR, pVO2 is a partial reflection of pCI. Risk of low pCI increases with age. No resting MRI measurement predicts low haemodynamic response to exercise. Probably more suitable to detect ventricular dysfunction, pCI measurement could be an additional parameter to take into account when considering pulmonic valve replacement.

Augmentation of pulmonary blood flow and cardiac output by non-invasive external ventilation late after Fontan palliation.

OBJECTIVES: Although a life-preserving surgery for children with single ventricle physiology, the Fontan palliation is associated with striking morbidity and mortality with advancing age. Our primary objective was to evaluate the impact of non-invasive, external, thoraco-abdominal ventilation on pulmonary blood flow (PBF) and cardiac output (CO) as measured by cardiovascular magnetic resonance (CMR) imaging in adult Fontan subjects. METHODS: Adults with a dominant left ventricle post-Fontan palliation (lateral tunnel or extracardiac connections) and healthy controls matched by sex and age were studied. We evaluated vascular flows using phase-contrast CMR imaging during unassisted breathing, negative pressure ventilation (NPV) and biphasic ventilation (BPV). Measurements were made within target vessels (aorta, pulmonary arteries, vena cavae and Fontan circuit) at baseline and during each ventilation mode. RESULTS: Ten Fontan subjects (50% male, 24.5 years (IQR 20.8-34.0)) and 10 matched controls were studied. Changes in PBF and CO, respectively, were greater following BPV as compared with NPV. In subjects during NPV, PBF increased by 8% (Δ0.20 L/min/m2 (0.10-0.53), p=0.011) while CO did not change significantly (Δ0.17 L/min/m2 (-0.11-0.23), p=0.432); during BPV, PBF increased by 25% (Δ0.61 L/min/m2 (0.20-0.84), p=0.002) and CO increased by 16% (Δ0.47 L/min/m2 (0.21-0.71), p=0.010). Following BPV, change in PBF and CO were both significantly higher in subjects versus controls (0.61 L/min/m2 (0.2-0.84) vs -0.27 L/min/m2 (-0.55-0.13), p=0.001; and 0.47 L/min/m2 (0.21-0.71) vs 0.07 L/min/m2 (-0.47-0.33), p=0.034, respectively). CONCLUSION: External ventilation acutely augments PBF and CO in adult Fontan subjects. Confirmation of these findings in larger populations with longer duration of ventilation and extended follow-up will be required to determine sustainability of haemodynamic effects.

Surgical management of Ebstein anomaly: impact of the adult congenital heart disease anatomical and physiological classifications.

OBJECTIVES: Our goal was to evaluate the impact of the adult congenital heart disease anatomical and physiological (ACHD AP) classification system on the surgical management of Ebstein anomaly (EA) in adult patients. METHODS: From February 2000 through August 2017, data of patients aged at least 16 years, who underwent primary EA surgery, were retrospectively evaluated. The cohort was divided in 2 groups according to their ACHD AP classification: the moderate EA group (IIB, IIC) and the severe EA group (IID). Survival, freedom from reoperation and freedom from occurrence of major adverse advents were estimated. RESULTS: There were 33 patients (21 women, 12 men). Eighteen belonged to the moderate group, 15 to the severe group. There were 12 female patients (80%) in the severe group. Patients in the moderate group were younger than those in the severe group (P = 0.02): 32 ± 12 vs 44 ± 15 years old. Thirty tricuspid valve repairs and 3 replacements were performed. Repair was mainly performed in the moderate group (P = 0.02). Overall survival was 90.1 ± 5.4% at 9 months after the operation and did not change in the later follow-up period. It was 100% for patients in the moderate group and 80.0 ± 10.3% in the severe group (P = 0.07), and 75.0 ± 12.5% for female patients of in the severe group compared to 100% for the remaining patients (P = 0.025). Survival free from major adverse events, including reoperation, at 10 years was 60.0 ± 12.6% in the moderate and 38.1% ± 12.9% in the severe group (P = 0.03). No patient in the moderate group evolved to be in the severe group at late follow-up. CONCLUSION: Adult EA patients should undergo surgery earlier when they are still in the moderate ACHD AP classification.

Utility of 3D printed cardiac models in congenital heart disease: a scoping review.

OBJECTIVE: Three-dimensional printing (3DP) is a novel technology with applications in healthcare, particularly for congenital heart disease (CHD). We sought to explore the spectrum of use of 3D printed CHD models (3D-CM) and identify knowledge gaps within the published body of literature to guide future research. METHODS: We conducted a scoping review targeting published literature on the use of 3D-CMs. The databases of MEDLINE, EMBASE and Web of Science were searched from their inception until 19 July 2019. Inclusion criteria were primary research; studies reporting use of 3D-CMs; and human subjects. Exclusion criteria were studies where 3D-CMs were generated for proof of concept but not used; and studies focused on bioprinting or computational 3D-CMs. Studies were assessed for inclusion and data were extracted from eligible articles in duplicate. RESULTS: The search returned 648 results. Following assessment, 79 articles were included in the final qualitative synthesis. The majority (66%) of studies are case reports or series. 15% reported use of a control group. Three main areas of utilisation are for (1) surgical and interventional cardiology procedural planning (n=62), (2) simulation (n=25), and (3) education for medical personnel or patients and their families (n=17). Multiple studies used 3D-CMs for more than one of these areas. CONCLUSIONS: 3DP for CHD is a new technology with an evolving literature base. Most of the published literature are experiential reports as opposed to manuscripts on scientifically robust studies. Our study has identified gaps in the literature and addressed priority areas for future research.

Effects of cardiopulmonary bypass with low-priming volume on clinical outcomes in children undergoing congenital heart disease surgery.

BACKGROUND: Cardiopulmonary bypass (CPB) with high-priming volume can significantly activate the inflammatory response and increse the usage of packed red blood cells (PRBCs). As risks and complications related to transfusions are increasing, many cardiac centers are focusing on reducing the priming volume of CPB. In our center, efforts have also been made to reduce the priming volume, and the effects of CPB with low-priming volume on clinical outcomes in children undergoing congenital heart disease (CHD) surgery were investigated in this study to provide referential experiences for pediatric CPB. METHODS: The clinical case data of 158 children undergoing CHD surgery with CPB were collected. The children were divided into the low-priming-volume group (group A, n = 79) and the traditional group (group B, n = 79) according to the priming volume. The amount of PRBCs transfused, the postoperative hematological test results and the clinical outcomes of the two groups were compared by the independent sample t-test or the chi-square test. RESULTS: The amount of PRBCs transfused during CPB and during the whole operation were significantly lower in group A than in group B (p < 0.01), but the hemoglobin (Hb) concentration was higher in group A on the first day after surgery (p < 0.01) and before hospital discharge. However, the latter showed no statistical significant difference. The lowest postoperative platelet count was higher in group A than in group B (p < 0.05). There was no statistical difference in the postoperative inflammatory markers and the main clinical outcomes between the two groups. CONCLUSIONS: The usage of PRBCs in CPB with low-priming volume decreased significantly, but the postoperative Hb concentration and platelet count could still be maintained at a high level, improving the use efficiency of PRBCs. CPB with low-priming volume did not affect the postoperative recovery of patients, so it is worthy of continuous promotion and optimization.

Reintervention and survival in 1428 patients in the Australian and New Zealand Fontan Registry.

OBJECTIVE: Patients undergoing single-ventricle palliation have experienced significant improvement in survival in the recent era. However, a substantial proportion of these patients undergo reoperations. We performed a review of the Australia and New Zealand (ANZ) Fontan Registry to determine the overall reintervention and reoperative burden in these patients. METHODS: A retrospective longitudinal cohort study was performed using data from patients who underwent a Fontan operation between 1975 and 2016 from the ANZ Fontan Registry. The data obtained included Fontan operation, reinterventions and most recent follow-up status. We examined the type and timing of reinterventions and survival. RESULTS: Of the 1428 patients identified, 435 (30%) underwent at least one reintervention after the Fontan operation: 110 patients underwent early reintervention and 413 underwent late reinterventions. Excluding Fontan conversion and transplantation, 220 patients underwent at least one interventional procedure and 209 patients underwent at least one reoperation. Fenestration closure and pacemaker-related procedures were the most common catheter and surgical interventions, respectively. The cumulative incidence of reintervention following Fontan was 23%, 37% and 55% at 10, 20 and 30 years, respectively. Survival and freedom from failure were worse in patients requiring later reintervention after Fontan surgery (51% vs 83% and 42% vs 69%, respectively at 30 years, p<0.001). This difference persisted after excluding pacemaker-related procedures (p<0.001). Operative mortality for non-pacemaker late reoperations after Fontan was 6%. CONCLUSIONS: A substantial proportion of Fontan patients require further intervention to maintain effective single-ventricle circulation. Patients undergoing reoperation after Fontan have higher rates of mortality and failure, despite intervention.

Acute coronary artery obstruction following surgical repair of congenital heart disease.

OBJECTIVES: Acute coronary artery obstruction is a rare complication of congenital heart disease surgery but imposes a high burden of morbidity and mortality. Previous case series have described episodes in specific congenital heart lesions or surgical repairs but have not examined the complication in all-comers to congenital heart surgery. We hypothesize that shorter time from a clinically recognized postoperative sentinel event suggestive of coronary ischemia to diagnosis of coronary obstruction is associated with improved clinical outcomes. METHODS: This was a single-center, retrospective review of patients diagnosed with acute coronary artery obstruction by angiography following surgical repair of congenital heart disease between January 2000 and June 2016. RESULTS: In total, 34 patients were identified. The most common procedures associated with coronary artery obstruction were the Norwood procedure, arterial switch operation, and aortic valve repair/replacement. In total, 79% required mechanical circulatory support, 41% died, and 27% were listed for heart transplant. Patients who died or were listed for heart transplant had longer median sentinel-event-to-cardiac-catheterization time (28 [6-168] hours vs 10 [3-56] hours, P = .001), and longer median sentinel-event-to-intervention time (32 [11-350] hours vs 13 [5-59] hours, P = .003). Patients with hypoplastic left heart syndrome were at greater risk of death or transplant listing (odds ratio, 9.23, P = .03). CONCLUSIONS: Time from clinically relevant postoperative sentinel event to diagnosis of coronary artery obstruction by angiography was associated with transplant-listing-free survival. Clinicians should maintain a high index of suspicion for coronary obstruction and consider early catheterization and coronary angiography for patients in whom post-operative coronary compromise is suspected.

Adult congenital open-heart surgery: emergence of a new mortality score.

OBJECTIVES: In revised 2018 American Heart Association/American College of Cardiology guideline for the management of adults with congenital heart disease (ACHD), the committee introduced a classification that combines lesion anatomy and physiological status: ACHD anatomic physiological (AP) classification. Anatomy is described as of simple (I), moderate (II) or great (III) complexity, whereas physiology is listed in 4 categories of increasing severity (A, B, C and D). Can this classification predict early postoperative mortality? METHODS: ACHD AP classification was determined for 339 adults who underwent open-heart surgery between September 2012 and August 2018. In addition, the adult congenital heart surgery (ACHS) and Society of Thoracic Surgery-European Association for Cardio-Thoracic Surgery (STAT) mortality scores were calculated. A model based on binary logistic regression was applied. The event was early postoperative death. Mortality scores were estimated for each ACHD AP class. RESULTS: All patients could be categorized by the ACHD AP classification. The 354 procedures were performed with an early mortality of 3.4% (12/354). The mortality risk for the new mortality score, simply called ACAP score, ranged from 0.2% (95% confidence interval 0.08-0.41%) for ACHD AP class IA to 20% (16.04-24.64%) for IIID class. Observed over expected ratios of early mortality amounted to 0.87, 1.54 and 1.14, whereas areas under the curve of receiver operator characteristic were found to be 0.78, 0.64 and 0.88 for STAT, ACHS and ACAP scores, respectively. CONCLUSIONS: ACHD AP classification could embrace all procedures. In our setting, the ACAP score was more predictive of early mortality than the ACHS and STAT mortality scores. It should be validated by further studies and other centres.

Pulmonary artery size is associated with functional clinical status in the Fontan circulation.

OBJECTIVE: In the Fontan circulation, non-pulsatile pulmonary blood flow is suggested to negatively affect pulmonary artery growth. The pulmonary vasculature is regarded a key determinant of outcome after Fontan completion. We hypothesised that in Fontan patients pulmonary artery size correlates with follow-up and functional clinical status. METHODS: This is a single-centre, cross-sectional cohort study. Thirty-nine paediatric and adult Fontan patients with a concomitant cardiac magnetic resonance (CMR) scan and a cardiopulmonary exercise test between 2012 and 2013 were included. CMR-derived left and right pulmonary artery cross-sectional areas were expressed as Nakata index. Functional status was defined as peak oxygen consumption (pVO2) indexed for weight, as percentage of predicted (pred) and as New York Heart Association Functional Class (NYHA-FC). RESULTS: Age at CMR was 18±7.2 years. Time since Fontan completion was 11.9±7.4 years. Nakata index was lower versus the reference values (238.6±78.5 vs 330±30 mm2/m2, p<0.001). Nakata index correlated negatively with age at CMR (r=-0.393, p=0.013) and time since Fontan completion (r=-0.341, p=0.034). pVO2 was 27.9±8.9 mL/min/kg and pVO2pred was 58.1%±14.1%. Nakata index correlated positively with pVO2 (r=0.468, p=0.003) and pVO2pred (r=0.353, p=0.028). Nakata index correlated negatively with NYHA-FC (r=-0.450, p=0.004). Nakata index was an independent predictor (ß=0.359, p=0.007) for pVO2 (adjusted R2=0.442, with maximum heart rate and oxygen pulse at peak exercise). CONCLUSIONS: Pulmonary artery size expressed as Nakata index is a novel independent predictor for functional clinical status. Nakata index negatively correlated with follow-up duration, suggesting that chronic abnormal non-pulsatile pulmonary blood flow plays a role in lagging pulmonary arterial growth in the Fontan circulation.

Tricuspid regurgitation severity after atrial septal defect closure or pulmonic valve replacement.

OBJECTIVES: Cardiac surgery or catheter interventions are nowadays commonly performed to reduce volume loading of the right ventricle in adults with congenital heart disease. However, little is known, on the effect of such procedures on pre-existing tricuspid regurgitation (TR). We assessed the potential reduction in the severity of TR after atrial septal defect (ASD) closure and pulmonic valve replacement (PVR). METHODS: Demographics, clinical and echocardiographic characteristics of consecutive patients undergoing ASD closure or PVR between 2005 and 2014 at a single centre who had at least mild preoperative TR were collected and analysed. RESULTS: Overall, 162 patients (mean age at intervention 41.6±16.1 years, 38.3% male) were included: 101 after ASD closure (61 transcatheter vs 40 surgical) and 61 after PVR (3 transcatheter vs 58 surgical). Only 11.1% received concomitant tricuspid valve surgery (repair). There was significant reduction in the severity of TR in the overall population, from 38 (23.5%) patients having moderate or severe TR preoperatively to only 11 (6.8%) and 20 (12.3%) at 6 months and 12 months of follow-up, respectively (McNemar p<0.0001). There was a significant reduction in tricuspid valve annular diameter (p<0.0001), coaptation distance (p<0.0001) and systolic tenting area (p<0.0001). The reduction in TR was also observed in patients who did not have concomitant tricuspid valve (TV) repair (from 15.3% to 6.9% and 11.8% at 6 and 12 months, respectively, p<0.0001). On multivariable logistic regression including all univariable predictors of residual TR at 12 months, only RA area remained in the model (OR 1.2, 95% CI 1.04 to 1.37, p=0.01). CONCLUSIONS: ASD closure and PVR are associated with a significant reduction in tricuspid regurgitation, even among patients who do not undergo concomitant tricuspid valve surgery.

Determinants of long-term outcome of repaired pulmonary valve stenosis.

INTRODUCTION AND OBJECTIVES: Although pulmonary valve stenosis (PVS) is considered a low risk congenital heart disease, there have been reports of complications and the need for reintervention throughout follow-up. The aims of this study were to evaluate the long-term outcome of repaired PVS and to identify predictors of cardiovascular complications and reintervention. METHODS: We studied 158 adult patients with repaired PVS (repair procedures performed from 1957 to 2010) receiving active follow-up in a tertiary referral center. RESULTS: A total of 95 patients (60%) received surgical treatment, and 63 patients (40%) received percutaneous pulmonary balloon valvuloplasty. At the end of follow-up (27 years, IQR, 20-33 years), most patients (n=134, 84.8%) were in New York Heart Association functional class I, but 61 patients (38.6%) required a reintervention, mainly pulmonary valve replacement (17.7%, n=28), and 19 patients (12%) had at least one cardiovascular complication: 13 (8.2%) supraventricular arrhythmias, 6 (3.8%) heart failure, 5 (3.2%) stroke, 1 (0.6%) death, 1 (0.6%) thromboembolism, and 1 (0.6%) ventricular arrhythmia. Multivariate analysis showed that age at PVS repair (HR, 1.08; 95%CI, 1.04-1.12; P <.001) and the presence of cyanosis before PVS repair (HR, 5.23; 95%CI, 1.99-13.78; P=.001) were independent predictors for cardiovascular complications. CONCLUSIONS: Good long-term outcome can be expected after PVS repair, but complications and the need for reintervention may appear. Older age and the presence of cyanosis at PVS repair emerged as predictors of cardiovascular complications and identified a population that may merit stricter control.

68-year-old woman with paroxysmal atrial fibrillation.

We present the case of a 68-year-old woman, currently asymptomatic but with a history of paroxysmal atrial fibrillation and tachycardia-bradycardia syndrome, for which she underwent a DDD pacemaker implantation and was started on oral anticoagulants. A recent chest X-ray (figure 1), for respiratory infection, was performed and the patient was referred to our institution for evaluation. Following diagnostic procedures, the patient was successfully operated on. heartjnl;105/8/656/F1F1F1Figure 1Preoperative chest X-ray (A) and chest CT with contrast (B). QUESTION: What is the most likely diagnosis?Mitral stenosis.Pericardial cyst.Left atrial appendage aneurysm.Left atrial myxoma.Atrial septal defect.