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Aims The extracardiac conduit-Fontan (ECC) has become the preferred technique for univentricular heart palliation, but there are currently no data on the incidence of long-term arrhythmias. This study investigated the incidence of arrhythmias and relation to single ventricle morphology in the long-term follow-up (FU) in ECC. Methods and results All patients with ECC performed in our Centre between 1987 and 2017 were included (minimum FU 5 years). Of 353 consecutive patients, 303 [57.8% males, aging 8–50 (median 20) years at last FU] were considered and divided into two groups depending on left (194 in Group 1) or right (109 in Group 2) ventricular morphology. Eighty-five (28%) experienced ≥1 arrhythmic complications, with early and late arrhythmias in 17 (5.6%) and 73 (24.1%) patients, respectively. Notably, late bradyarrhythmias occurred after 6 years in 21 (11%) patients in Group 1, and in 15 (13.8%) in Group 2 [ P = 0.48]. Late tachyarrhythmias occurred in 55 (18.2%) patients after 12 years: 33 (17%) in Group 1 and 22 (20.2%) patients in Group 2 [ P   = 0.5]. Ventricular tachycardias (VT) were documented after 12.5 years in 14 (7.2%) patients of Group 1 and 15 (13.8%) of Group 2 [ P = 0.06] with a higher incidence in Group 2 during the FU [ P = 0.005]. Conclusion Extracardiac conduit is related to a significant arrhythmic risk in the long-term FU, higher than previously reported. Bradyarrhythmias occur earlier but are less frequent than tachyarrhythmias. Interestingly, patients with systemic right ventricle have a significantly higher incidence of VT, especially in a very long FU. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES We sought to evaluate the long-term survival and risk factors for morbidity and mortality in our cohort of patients after extracardiac (EC) Fontan. METHODS We collected and analysed follow-up data until September 2020 for all patients who underwent EC Fontan operation at our institution from November 1988 to November 2015. RESULTS Out of 406 patients treated with EC Fontan at a mean age of 5.4 ± 4.5 years, 372 (91.6%) were discharged home; 15% were lost to follow-up. The median follow-up was 14.6 years (interquartile range 8.7–20.3). Two hundred patients (54%) had a good long-term outcome, with an event-free long-term survival, but late adverse events of any kind needing interventional or surgical reoperations were reported in 128 patients (34%). Forty-four patients (12%) developed late Fontan failure. Thirty-seven survivors (10%) had late arrhythmias, with the need for pacemaker implantation. Obstruction of the cavopulmonary pathway occurred in 49 patients (13%), but just 8 patients needed conduit surgical replacement. At 32 years, the survival probability was 84%. The risk of orthotopic heart transplant, considering death as a competing event (showed as cumulative incidence), was 12.5%. A preoperative diagnosis of hypoplastic left heart syndrome was an independent risk factor for the need for heart transplant. CONCLUSIONS The EC Fontan shows satisfactory long-term survival and low incidence of adverse events and late failure. Conduit replacement is rare, and its longevity may not represent a substantial issue in these patients. However, as with other technical variants, the need for reoperations during long-term follow-up is a significant challenge. [ABSTRACT FROM AUTHOR]

Aims The aortic valve (AV) neocuspidization (Ozaki procedure) is a novel surgical technique for AV disease that preserves the natural motion and cardiodynamics of the aortic root. In this study, we sought to evaluate, by 4D-flow magnetic resonance imaging, the aortic blood flow characteristics after AV neocuspidization in paediatric patients. Methods and results Aortic root and ascending aorta haemodynamics were evaluated in a population of patients treated with the Ozaki procedure; results were compared with those of a group of patients operated with the Ross technique. Cardiovascular magnetic resonance studies were performed at 1.5 T using a 4D flow-sensitive sequence acquired with retrospective electrocardiogram-gating and respiratory navigator. Post-processing of 4D-flow analysis was performed to calculate flow eccentricity and wall shear stress. Twenty children were included in this study, 10 after Ozaki and 10 after Ross procedure. Median age at surgery was 10.7 years (range 3.9–16.5 years). No significant differences were observed in wall shear stress values measured at the level of the proximal ascending aorta between the two groups. The analysis of flow patterns showed no clear association between eccentric flow and the procedure performed. The Ozaki group showed just a slightly increased transvalvular maximum velocity. Conclusion Proximal aorta flow dynamics of children treated with the Ozaki and the Ross procedure are comparable. Similarly to the Ross, Ozaki technique restores a physiological laminar flow pattern in the short-term follow-up, with the advantage of not inducing a bivalvular disease, although further studies are warranted to evaluate its long-term results. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The aim of this study was to identify the predictors of death and of reintervention after mitral valve replacement (MVR) in children. METHODS A single-centre retrospective study was performed including 115 patients under the age of 18 undergoing MVR between 1982 and 2019. For all patients, the ratio of prosthetic valve size (diameter in mm) to weight (kg) at surgery was calculated and long-term result was assessed. The primary outcome was freedom from mitral valve (MV) re-replacement. The composite secondary outcome was freedom from death or transplant. RESULTS Fifty-four patients had a previous surgical attempt to MV repair. The median age at surgery was 5.5 years (interquartile range 1.21–9.87). Death/transplant-free survival was 77 ± 4% at 5 years and 72 ± 5% at 10 years. Univariate analysis showed a size/weight ratio higher than 2 and age <2 years as significant risk factors for death or transplant. Freedom from MV re-replacement at 5 and 10 years was 90 ± 3% and 72 ± 6%, respectively. Biological prosthesis implanted at first replacement (P  = 0.007) and size/weight ratio higher than 2 (P  = 0.048) were predictors of reoperation. Significant upsizing (P  < 0.0001) of mitral prosthesis was observed at re-replacement. CONCLUSIONS MVR is a viable strategy in children with unrepairable MV disease. Mortality can be predicted based on size/weight ratio and age <2 years. MV re-replacement can be performed with low morbidity and mortality and a larger-size prosthesis can often be placed at the time of redo. [ABSTRACT FROM AUTHOR]

OBJECTIVES: We sought to determine differences in baseline characteristics and clinical outcomes in a consecutive series of patients undergoing arterial switch operation (ASO), assessing the effect of coronary anatomy on postoperative mortality, both overall and adjusted for surgical era. METHODS: From January 2000 to May 2015, 283 consecutive patients underwent ASO for transposition of the great arteries. A total of 103 patients (36.4%) had an associated ventricular septal defect and 23 (8.1%) had an aortic arch obstruction. Coronary anomalies were present in 119 (42%) patients. RESULTS: Overall survival was 93.0 ± 1% at 15 years (19 events). Intraoperative revision of the coronary anastomosis was required in 8 patients (2.8%) due to inability to wean from cardiopulmonary bypass (CPB) or post-CPB myocardial ischaemia. Surgical revision was neither related to the underlying coronary anatomy (χ² =0.681, P = 0.4091) nor to the surgical era (χ² =0.682, P = 0.4090). Univariable analysis suggested decreased mortality rate for infants who underwent surgery during 2008 or later (P = 0.01). In multivariable analysis, intraoperative coronary button revision was the only predictor of mortality [P < 0.001, hazard ratio (HR) 12.01, 95% confidence interval (CI) 3.85--37.49], whereas surgical era (before 2008) failed to achieve statistical significance (P = 0.057, HR 2.19, 95% CI 0.96-12.04). CONCLUSIONS: In the recent era, unusual coronary patterns do not affect survival following ASO, whereas intraoperative coronary button revision is a predictor of mortality. Preventive strategies aimed towards intraoperative detection of impaired coronary perfusion are useful tools that might be considered for all patients in order to improve outcomes. [ABSTRACT FROM AUTHOR]

Objectives: To validate the preliminary surgical results of ‘integrated approach’ to pulmonary atresia, ventricular septal defect (VSD), and multiple aortopulmonary collateral arteries by retrospective analysis of our center experience. Methods: Between 01/94 and 03/02, 37 patients aged 22 days to 13 years underwent surgery for pulmonary atresia, VSD, and multiple aortopulmonary collaterals. Case selection was based on preoperative calculation of total neopulmonary arterial index (TNPAI), pulmonary arterial index (PAI), and pulmonary arteries-to-collateral arteries lung segment perfusion ratio (Spa:Sca). The decision for a possible VSD closure during one-stage procedures was based on an intraoperative pulmonary flow study. Twenty-five patients with a TNPAI equal to or greater than 150 mm2/m2 underwent primary unifocalization, irrespective of PAI and Spa:Sca. Conversely, 12 patients with a TNPAI less than 150 mm2/m2 and hypoplastic (PAI less than 100 mm2/m2) dominant (Spa:Sca greater than 1) pulmonary arteries received a first-stage right ventricular outflow tract reconstruction, followed by unifocalization and repair (i.e. VSD closure) in nine cases. Results: Among 34 patients who received total unifocalization, the overall repairability rate was 85% (first instance repairs: n=27; delayed VSD closure: n=2; 95% confidence interval, CI: 73–97%), with a survival rate at 7 years of 81%. Repaired survivors (n=26) are asymptomatic (n=22) or mildly symptomatic (n=4) at a follow-up interval of 43±28 months, with a 0.48±0.2 mean haemodynamic right ventricular/left ventricular pressure ratio, whereas palliated ones are waiting for either repair (n=3) or catheter study (n=2). Analysis of results has shown the following: (1) 100% (34/34 cases) feasibility of one-stage unifocalization in patients with a preoperative TNPAI equal to or greater than 150 mm2/m2, whereas combined repairability rate was 79% only (95% CI: 65–93%); (2) 100% (12/12 cases) fulfillment of criteria for second-stage repairability (acquired TNPAI greater than 150 mm2/m2) in all patients treated with right ventricular outflow tract reconstruction; and (3) 93% (95% CI: 83–100%) overall accuracy of intraoperative flow study in predicting either postrepair mean pulmonary arterial pressure (VSD closed: n=23) or balanced pulmonary to systemic blood flow ratio (VSD left open: n=4). Conclusions: Increasing experience with ‘integrated approach’ to pulmonary atresia, VSD, and multiple aortopulmonaty collaterals has confirmed the preliminary results of our surgical series. The pulmonary flow study remains the most accurate intraoperative test for successful management of VSD during unifocalization procedures [Copyright &y& Elsevier]

The article presents a case study of a 3-month-old man with broncodysplasia and mild oxygen-dependency, Topics discussed include result of Echocardiogram for right coronary artery (RCA), Cardiac computed tomography for RCA and left coronary artery (LCA), and findings of Aortic angiography.

The article evaluates morphological vessel analysis efficiency on pulmonary vascular physiology in patients with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries. It mentions shortcomings of the preoperative morphometric evaluation of the pulmonary blood sources. It also mentions preoperative morphometric evaluations of the pulmonary circulation.

The author focuses on postoperative neural development in the patients with hypoplastic left heart complex. He informs about a controversial dataregarding myocardial performance in the patients undergoing hybrid procedure as compared to patients undergoing Norwood operation. It informs that the outcome of result needs to be assessed.

OBJECTIVES Palliative Potts shunt has been proposed in children with suprasystemic pulmonary arterial hypertension (PAH). METHODS A retrospective multicentre study was performed to assess short- and long-term outcomes after Potts shunt. RESULTS From 2003 to 2014, 24 children underwent a Potts shunt [19 surgical, median age: 7.7 years (1.5–17 years), median weight: 19.5 kg (10.2–47 kg) and 5 transcatheter, median age: 8.1 years (2.3–9.7 years), median weight: 22 kg (12.5–31 kg)] for drug-refractory PAH. For the first time in humans, we performed an unidirectional valved Potts anastomosis in a child with infrasystemic PAH on intravenous epoprostenol who experienced repeated central line infections. Severe postoperative complications occurred in 6 patients (25.0%, all from the surgical group) including 3 early deaths (12.5%) related to low cardiac output. After a median follow-up (FU) of 2.1 years (range, 3 months to 14.3 years, ≥8 years in 7 patients), World Health Organization (WHO) functional class was dramatically improved in the 21 survivors, all being in WHO-functional class 1 or 2 (P < 0.05); none experienced syncope during the FU; none had overt right ventricular failure; mean 6-min walk distance improved from 42.3 ± 10.0% to 81.2 ± 9.7% of adjusted values for age and sex (P < 0.001), BNP/NT-proBNP levels normalized in all; and weaning of intravenous epoprostenol was obtained in all patients who received triple combination as pre-Potts anastomosis therapy. Finally, all survivors caught up to normal growth curves. Arterial oxygen saturation gradient between upper and lower limbs persisted at the last FU (94.7 ± 3.6% vs 81.6 ± 5.1%, P < 0.001). One patient required double lung transplantation 6 years after a surgical Potts shunt. CONCLUSIONS Palliative Potts shunt allows prolonged survival and dramatic, long-lasting improvement in functional capacities in children with severe, drug-refractory PAH. The Potts shunt might be considered as a first surgical or interventional step in the management of children with severe, drug-refractory PAH, leaving the door open for further lung transplantation, if needed. [ABSTRACT FROM PUBLISHER]

OBJECTIVES To identify factors associated with the surgical outcome in patients undergoing right ventricular outflow tract reconstruction (RVOTR) using decellularized tissue-engineered pulmonary valve conduits (TEPVc) and to study their safety and longevity. METHODS From April 2006 to April 2010, 93 patients underwent either palliative or corrective RVOTR using Matrix P (37) and Matrix P Plus (56) xenogenic decellularized TEPVc (size range 11–27 mm). Median age and weight at operation were 20 (0.16–290) months and 10.15 (2.65–86) kg respectively. Primary and redo surgery occurred in 40 and 60% of cases, respectively. Eighty-eight patients (94.6%) received conduit implantation in the framework of corrective surgery, whereas in 5 (5.4%) a palliative procedure was undertaken. Follow-up was complete in 91% of patients, with a median duration of 12 months (range: 2 days–51 months). Data analysis included diagnosis, type of surgery (palliative vs. corrective) and age at surgery. Predetermined primary outcomes were represented by conduit failure or dysfunction. RESULTS Two patients with Matrix P and two with Matrix P Plus died in the early post-operatively phase (4.3%). None of the deaths were conduit-related. One patient died at conduit replacement. Thirty-three patients (35.5%) experienced conduit failure whereas conduit dysfunction occurred in 27 patients (29%). Two-year freedom from conduit failure and dysfunction was 60.2% (95% CI: 50.1–69.6) and 77.4% (95% CI: 67.9–84.7), respectively. Reasons for failure were conduit stenosis in 20 cases (61%), pseudoaneurysm in 3 (9%), conduit dilatation (>50% of original diameter) in 2 (6%), stenosis of distal anastomosis involving pulmonary bifurcation in 6 (18%) and allograft dissection in 2 (6%). Histological examination showed inflammatory giant-type cells in the presence of a poor autologous cell seeding in all explanted specimens. Univariate and multivariate analyses showed an association between age at surgery ≤1 year and conduit dysfunction (adjusted HR: 2.29; 95% CI: 1.01–5.20, P = 0.04). CONCLUSIONS Compared with the other conduit for RVOTR Matrix conduits showed a high incidence of failure. Our results suggest that the use of Matrix conduits for RVOTR should be considered with caution. [ABSTRACT FROM AUTHOR]

Summary: The population of neonates and children with congenital heart defects presents about a 30% prevalence of associated genetic syndrome or additional extracardiac anomalies and may show an increased risk of death or major complication at cardiac surgery. Since a well-defined pattern of combined cardiac and extracardiac anomalies may be found in relation to specific genetic defects, correct understanding of the genetic issues may help improving diagnosis, surgical approach and final outcome of these patients. Hereby we review the medical and surgical issues correlated to the genetic asset in patients with congenital heart defects and genetic syndromes, including trisomy 21, deletion 22q11, Noonan/LEOPARD, Turner, Marfan and Williams syndromes. Recognition of specific surgical risk factors can lead to the preparation of specific diagnostic and perioperative protocols in order to reduce operative mortality and morbidity. [Copyright &y& Elsevier]

Objective: Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for failing single ventricle physiology. Methods: Between 1988 and 2002, 25 patients (mean age 9.3±7.1 years) underwent OHT for complex congenital heart disease (CHD) with a functional right (15 patients) or left (10 patients) single ventricle. Palliative staging towards Fontan completion had been previously accomplished in 22 patients (88%). Transition to OHT occurred from a shunt stage in 10, a bi-directional cavopulmonary anastomosis (BDG) stage in nine, and after Fontan failure in six patients. Results: Thirty-day survival was 68.0±9.3% with no additional mortality up to 14.1 years. OHT following BDG staging exhibited 100% long-term survival, as opposed to 66.7±15.7% for OHT after systemic-to-pulmonary shunt, and 33.3±19.2% for OHT following failing Fontan (p=0.032). Regression logistic modelling indicated failing Fontan circulation as predictor of higher mortality after OHT (p=0.041). Reintervention was necessary in four patients 40±11 months after OHT to address residual superior vena cava (two) and isthmic (two) stenosis. Overall freedom from reintervention was 88.3±8.1% at 5 years. Conclusions: OHT for structural CHD with single ventricle physiology entails substantial early mortality while BDG enables the best transition to heart transplant. OHT should be considered in the decision-making process as an alternative to Fontan completion in high-risk candidates, since rescue-OHT after failing Fontan seems unwarranted. [Copyright &y& Elsevier]