The Ross Operation in Young Patients: A 15-Year Experience Focused on Right Ventricle to Pulmonary Artery Conduit Outcomes.

Background: Data on long-term outcomes of the Ross operation in children and young adult patients are limited. The best pulmonary valve substitute for the right ventricular outflow tract reconstruction remains uncertain. This study aimed to assess the outcomes of right ventricular outflow tract reconstruction in the Ross operation in young patients using various pulmonary valve substitutes at a single institution. In addition, a comparison of reintervention rates between patients younger than 18 years and those older than 18 years was performed. Methods: The study assessed all patients (N = 110) who underwent the Ross operation at the University Hospital of Bordeaux, France, between 2004 and 2020. Results: The median follow-up time was 4.2 years, and the median age at operation was 15.9 years. There was no operative mortality and 1 late noncardiac death (0.8%). The overall survival rate at 10 years was 99.2%. The need for right ventricular outflow tract reoperation was lower with the pulmonary homograft compared with the Contegra conduit and Freestyle bioprosthesis: 94.3%, 93.8%, and 80% at 5 years, respectively, and 94.3%, 72.3%, and 34.3% at 10 years, respectively (P = 0.011). The probability of reintervention was not significantly different at 10 years among children vs adults (P = 0.22). Conclusions: The Ross procedure in children and young adults was associated with a lower requirement for right ventricular outflow tract reoperation when pulmonary homografts were used instead of xenografts.

Contexte: Il existe peu de données sur les résultats à long terme de l'intervention de Ross chez les enfants et chez les jeunes adultes. Par ailleurs, des doutes persistent quant au meilleur substitut pour remplacer la valve pulmonaire lors de la reconstruction de la voie d'éjection du ventricule droit. Notre étude visait à mesurer les résultats de la reconstruction de la voie d'éjection du ventricule droit après l'intervention de Ross chez de jeunes patients d'un même établissement chez qui différents substituts valvulaires ont été utilisés. De plus, le taux de réintervention chez les patients âgés de moins de 18 ans et celui chez les patients âgés de 18 ou plus ont été comparés. Méthodologie: Notre étude portait sur tous les patients (N = 110) ayant subi une intervention de Ross au Centre Universitaire de Bordeaux (France) entre 2004 et 2020. Résultats: La durée médiane du suivi a été de 4,2 années, et l'âge médian au moment de l'intervention chirurgicale était de 15,9 ans. Aucun décès précoce n'a été constaté au terme de l'intervention, mais un décès de cause non cardiaque est survenu ultérieurement (0,8 %). Le taux global de survie à 10 ans était de 99,2 %. La réintervention chirurgicale au niveau de la voie d'éjection du ventricule droit a été nécessaire moins fréquemment chez les patients ayant reçu une homogreffe que chez les patients ayant reçu un conduit Contegra ou une bioprothèse Freestyle : les taux sans réintervention s'élevaient respectivement à 94,3 %, 93,8 % et 80 % à 5 ans, et à 94,3 %, 72,3 % et 34,3 % à 10 ans (p = 0,011). En outre, les probabilités de réintervention chirurgicale chez les enfants et chez les adultes ne différaient pas de façon significative à 10 ans (p = 0,22). Conclusions: Le recours à des homogreffes pulmonaires plutôt qu'à des xénogreffes au cours des interventions de Ross pratiquées chez les enfants et les jeunes adultes est associé à un plus faible taux de réintervention.

Overview of the current use of levosimendan in France: a prospective observational cohort study.

BACKGROUND: Following the results of randomized controlled trials on levosimendan, French health authorities requested an update of the current use and side-effects of this medication on a national scale. METHOD: The France-LEVO registry was a prospective observational cohort study reflecting the indications, dosing regimens, and side-effects of levosimendan, as well as patient outcomes over a year. RESULTS: The patients included (n = 602) represented 29.6% of the national yearly use of levosimendan in France. They were treated for cardiogenic shock (n = 250, 41.5%), decompensated heart failure (n = 127, 21.1%), cardiac surgery-related low cardiac output prophylaxis and/or treatment (n = 86, 14.3%), and weaning from veno-arterial extracorporeal membrane oxygenation (n = 82, 13.6%). They received 0.18 ± 0.07 µg/kg/min levosimendan over 26 ± 8 h. An initial bolus was administered in 45 patients (7.5%), 103 (17.1%) received repeated infusions, and 461 (76.6%) received inotropes and or vasoactive agents concomitantly. Hypotension was reported in 218 patients (36.2%), atrial fibrillation in 85 (14.1%), and serious adverse events in 17 (2.8%). 136 patients (22.6%) died in hospital, and 26 (4.3%) during the 90-day follow-up. CONCLUSIONS: We observed that levosimendan was used in accordance with recent recommendations by French physicians. Hypotension and atrial fibrillation remained the most frequent side-effects, while serious adverse event potentially attributable to levosimendan were infrequent. The results suggest that this medication was safe and potentially associated with some benefit in the population studied.

Electrophysiological study prior to planned pulmonary valve replacement in patients with repaired tetralogy of Fallot.

AIM: Ventricular arrhythmias (VAs) are the most common cause of death in patients with repaired Tetralogy of Fallot (rTOF). However, risk stratifying remains challenging. We examined outcomes following programmed ventricular stimulation (PVS) with or without subsequent ablation in patients with rTOF planned for pulmonary valve replacement (PVR). METHODS: We included all consecutive patients with rTOF referred to our institution from 2010 to 2018 aged ≥18 years for PVR. Right ventricular (RV) voltage maps were acquired and PVS was performed from two different sites at baseline, and if non-inducible under isoproterenol. Catheter and/or surgical ablation was performed when patients were inducible or when slow conduction was present in anatomical isthmuses (AIs). Postablation PVS was undertaken to guide implantable cardioverter-defibrillator (ICD) implantation. RESULTS: Seventy-seven patients (36.2 ± 14.3 years old, 71% male) were included. Eighteen were inducible. In 28 patients (17 inducible, 11 non-inducible but with slow conduction) ablation was performed. Five had catheter ablation, surgical cryoablation in 9, both techniques in 14. ICDs were implanted in five patients. During a follow-up of 74 ± 40 months, no sudden cardiac death occurred. Three patients experienced sustained VAs, all were inducible during the initial EP study. Two of them had an ICD (low ejection fraction for one and important risk factor for arrhythmia for the second). No VAs were reported in the non-inducible group (p < .001). CONCLUSION: Preoperative EPS can help identifying patients with rTOF at risk for VAs, providing an opportunity for targeted ablation and may improve decision-making regarding ICD implantation.

Use of Extracorporeal Membrane Oxygenation in Pneumocystis Pneumonia of an Infant with AIDS.

Pneumocystis pneumonia is a common complication of cellular immunosuppression and may trigger severe pulmonary complications. Rapid onset of acquired immunodeficiency syndrome is possible in infants infected with human immunodeficiency virus (HIV). We report here the case of a 13-week-old girl who was previously healthy presenting with altered immunity and refractory acute respiratory distress syndrome (ARDS) initially attributed to bacterial pneumonia. Venovenous extracorporeal membrane oxygenation (VV-ECMO) was initiated because her condition was poor. An HIV infection was later fortuitously diagnosed after accidental exposure of a nurse to the child's urine. The mother had congenitally transmitted HIV to the child after late (undetected) infection during pregnancy. The lung lesions were finally attributed to Pneumocystis pneumonia. We prescribed combined antiretroviral, antibiotic, and steroid therapy aimed at preventing immune reconstitution inflammatory syndrome. VV-ECMO weaning progressed over 30 days to the time of decannulation, rapidly followed by extubation and hospital discharge. The case highlights the fact that rare curable causes of refractory pediatric ARDS should always be investigated early. VV-ECMO should not be excluded as an ARDS treatment for immunocompromised children.

Impact of the perioperative inotropic support in grown-up congenital heart patients undergoing cardiac surgery: a propensity score adjusted analysis.

BACKGROUND: Grown-up congenital heart (GUCH) patients represent a growing population with a high morbidity risk when undergoing reparative surgery. A main preoperative feature is right ventricular failure, which represents a risk factor for postoperative low cardiac output syndrome. Levosimendan has a potentially beneficial effect. This retrospective study included consecutive GUCH patients with surgeries in a tertiary cardiothoracic centre between 01-01-2013 and 01-10-2017, to test the hypothesis that the postoperative use of levosimendan might be associated with shorter time of mechanical ventilation, when compared with the use of milrinone. To adjust for bias related to the probability of treatment assignment, it uses the inverse propensity score weighting methodology. RESULTS: Overall 363 patients had GUCH surgeries during the study period, their mean age was 31.39 ± 15.31 years, 87 patients were eligible for analysis in the Levosimendan group and 117 in the Milrinone group. The propensity score used pre- and intraoperative variables and resulted in a good balance between covariates. The Levosimendan group included patients with higher preoperative risk scores, a higher prevalence of left and right ventricular failure, who required more often the addition of epinephrine, renal replacement therapy, prolonged mechanical ventilation and intensive care stay. However, after propensity score weighting, patients in the Levosimendan group had shorter durations of mechanical ventilation (average treatment effect - 37.59 h IQR [- 138.85 to - 19.13], p = 0.01) and intensive care stay (average treatment effect - 3.11 days IQR [- 10.03 to - 1.48], p = 0.009). The number of days of additional epinephrine support was shorter and the vasoactive inotropic scores lower. CONCLUSION: We report a beneficial effect in terms of duration of mechanical ventilation and intensive care stay, and on inotropic requirements of the use of levosimendan following GUCH surgeries. The use of levosimendan in this setting requires validation at a larger scale.

Neurally adjusted ventilatory assist for children on veno-venous ECMO.

NAVA may improve veno-venous ECMO weaning in children. This is a retrospective small series, describing for the first time proof-of-principle for the use of NAVA in children on VV ECMO. Six patients (age 1-48 months) needed veno-venous ECMO. Controlled conventional ventilation was replaced with assisted ventilation as soon as lung compliance improved, and could trigger initiation and termination of ventilation. NAVA was then initiated when diaphragmatic electrical activity (EAdi) allowed for triggering. NAVA was possible in all patients. Proportionate to EAdi (1.8-26 µV), initial peak inspiratory pressures ranged from 21 to 34 cm H2O, and the tidal volume (Vt) from 3 to 7 ml/kg. During weaning, peak pressures increased proportionally to EAdi increase (5.2-41 µV), with tidal volumes ranging from 6.6 to 8.6 ml/kg. ECMO was weaned after a median time of 1.75 days on NAVA. Following ECMO weaning, the median duration of mechanical ventilation, and intensive care unit stay were 4.5 days, and 13.5 days, respectively. Survival to hospital discharge was 100%. In conclusion, combining NAVA to ECMO in paediatric respiratory failure is safe and feasible, and may help in a smoother ECMO weaning, since NAVA allows the patient to drive the ventilator and regulate Vt according to needs.

Transposition of the great arteries: Rationale for tailored preoperative management.

As preoperative morbi-mortality remains significant, care of newborns with transposition of the great arteries is still challenging. In this review of the literature, we discuss the different treatments that could improve the patient's condition into the preoperative period. Instead of a standardized management, we advocate personalized care of these neonates. Considering the deleterious effects of hypoxia, special attention is given to the use of non-invasive technologies to assess oxygenation of the tissues. As a prolonged preoperative time with low cerebral oxygenation is associated with cerebral injuries, distinguishing neonates who should undergo early surgery from those who could wait longer is crucial and requires full expertise in the management of neonatal congenital heart disease. Finally, to treat these newborns as soon as possible, we support a planned delivery policy for foetuses with transposition of the great arteries.

Extending percutaneous left atrial appendage closure indications using the AMPLATZER™ Cardiac Plug device in patients with persistent left atrial appendage thrombus: The thrombus trapping technique.

BACKGROUND: Percutaneous left atrial appendage (LAA) closure has emerged as an alternative therapeutic option for the prevention of embolic stroke in high-risk patients with non-valvular atrial fibrillation. The presence of thrombus in the LAA is currently a contraindication to the procedure. AIM: To describe a modified LAA closure technique that allows a safe procedure in patients with LAA thrombus. METHODS: Between May 2013 and October 2014, LAA closure was performed in three patients with LAA thrombus (mean age 73.6±14 years; two men), using a modified technique that avoids manipulation of catheters or angiography in the LAA. RESULTS: Two patients had persistent thrombus despite appropriate antithrombotic therapy, while the other patient had a contraindication to systemic anticoagulation. The procedure was successful using the modified implantation technique in all patients. The implanted device was the AMPLATZER™ Cardiac Plug (St. Jude Medical, Minneapolis, MN, USA) in one patient and the Amulet™ (St. Jude Medical, Minneapolis, MN, USA) in two patients. No periprocedural complications occurred. After a mean follow-up of 8±2 months, no deaths or late complications were observed. CONCLUSIONS: Thrombus trapping is a feasible and effective technique for performing LAA occlusion in patients with thrombus within the LAA. This modification of the implantation technique may allow LAA closure indications to be extended to include patients with LAA thrombus, who were formerly considered unsuitable.

Pulmonary-Valve Replacement in Adults: Results With the Medtronic Freestyle Valve.

BACKGROUND: We used the Medtronic Freestyle valve (Medtronic, Minneapolis, MN) as an orthotopic conduit in pulmonary valve replacement in repaired tetralogy of Fallot and as part of the Ross procedure. Midterm outcomes and hemodynamic status of this conduit were analyzed and performances in both subgroups were compared. METHODS: From February 2002 to July 2012, 115 Freestyle valves were implanted in 52 patients with tetralogy of Fallot and 63 patients within the Ross procedure. Preoperative and perioperative data were reviewed retrospectively in this bicentric study. RESULTS: Mean age at valve surgery was 37 ± 13 years. Median implanted valve size was 27 mm (21 to 29). Early postoperative mortality was 3.48%. There was 100% follow-up for the survivors at a mean of 4.38 ± 2.52 years. There was 1 case of thromboembolism (0.89%), 6 endocarditis (5.4%), and 9 (7.8%) conduit re-interventions. Echocardiography at discharge and last follow-up showed average peak systolic transvalvular gradients of 12.4 ± 5.1 and 18.7 ± 8.8 mm Hg, respectively. Ten patients had significant proximal anastomotic gradients of greater than 50 mm Hg and 4 moderate conduit regurgitations. Survival was 96.52%. No valve degeneration was seen in 87.82% at 5 years. The only risk factor identified for valve re-intervention was conduit implantation without infundibular hood (p = 0.01 in multivariate analysis). CONCLUSIONS: Mid-term data show that Freestyle valves are well suited for pulmonary valve replacement in adults in both categories. The surgical technique used in valve implantation is important to ensure conduit durability. These results and accessibility to the Freestyle valve make this an acceptable alternative to homografts.

Single-centred experience with levosimendan in paediatric decompensated dilated cardiomyopathy.

BACKGROUND: Children with dilated cardiomyopathy in advanced heart failure may spend a long time awaiting heart transplantation. Consequently, mechanical circulatory support is sometimes required as a bridge to transplantation. Levosimendan, a positive inotropic agent, has been reported to be safe and efficient for the treatment of paediatric heart failure. AIMS: To report our experience with levosimendan in children with decompensated dilated cardiomyopathy. METHODS: Paediatric patients with dilated cardiomyopathy on the transplant waiting list and with criteria for mechanical support were included in this single-centred retrospective study. Each patient received at least one 24-hour infusion of levosimendan before mechanical circulatory support was considered. Biological and echocardiographic data were analysed. RESULTS: Six patients were included over a 24-month period. The median age was 25.5months (7.7-34.2months); 82 infusions were performed. Median B-type natriuretic peptide concentration decreased significantly between days 0 and 2 (2443ng/L [1458-3819ng/L] vs 1358ng/L [1025-2534ng/L]; P=0.003). While only a trend was noted in left ventricular ejection fraction improvement (P=0.054 by Simpson's method and P=0.068 by the Teicholz method), the subaortic velocity time integral rose significantly between days 0 and 8 (12.8cm/s [10-14.5cm/s] vs 15.3cm/s [14.3-16.9cm/s]; P=0.041). CONCLUSIONS: Levosimendan seems to improve haemodynamics in children with decompensated dilated cardiomyopathy; repeated infusions may delay the need for mechanical circulatory support while awaiting heart transplantation. This therapeutic agent should be systematically considered in this setting, in addition to conventional inotropic drugs.

Early to midterm results of total cavopulmonary connection in adult patients.

BACKGROUND: Total cavopulmonary connection (TCPC) has not been studied in adults. We investigated early and midterm morbidity and mortality in adults undergoing TCPC and assessed risk factors for mortality. METHODS: Between June 1994 and October 2010, 30 adults (21.3 ± 5.5 years) underwent TCPC (extracardiac conduit). Twenty-two patients who had palliated single ventricles underwent TCPC completions and 8 patients underwent TCPC conversions. Preoperative and perioperative data were reviewed retrospectively. RESULTS: Six of 9 patients with preoperative atrial flutter or fibrillation or intraatrial reentry tachycardia were treated in the catheterization room. An aortic cross-clamp was necessary in 12 patients, and 16 TCPCs were fenestrated. Mean follow-up was 51 months (range, 4-198 months). Early mortality was 10%: 2 of 8 conversions and 1 of 22 completions. There was 1 late conversion death (at 56 months postoperatively). Postoperatively, 4 patients required pacemakers and 1 patient required long-term antiarrhythmic medication, but no heart transplantations were necessary. Risk factors for early mortality were arrhythmia (p = 0.02), aortic cross-clamp (p = 0.054), and extracorporeal circulation in hypothermia (p = 0.03). Risk factors for overall mortality were conversion (p = 0.047), absence of fenestration (p = 0.036), surgery before January 2006 (p = 0.036), aortic cross-clamp (p = 0.018), extracorporeal circulation in hypothermia (p = 0.008), and arrhythmia (p = 0.005). New York Heart Association functional class had improved at the last follow-up: preoperatively, 17 patients were in class II and 12 patients were in class III versus 18 patients in class I and 9 patients in class II postoperatively (p < 0.001). At the last clinical visit, systemic ventricular function was maintained, and no late supraventricular arrhythmia was found. CONCLUSIONS: Early and midterm TCPC results for adults are encouraging for completion but are disappointing for conversion. Identified risk factors for mortality should improve patient selection for TCPC.

Evaluation of continuous veno-venous hemofiltration for the treatment of cardiogenic shock in conjunction with acute renal failure after cardiac surgery.

BACKGROUND: Cardiogenic dysfunction with acute renal failure (ARF) and diuretic drug resistance increases mortality after cardiac surgery with cardiopulmonary bypass (CPB) in adults. Until few years ago, intermittent renal replacement therapy (IRRT) was the only therapeutical strategy proposed to such patients. Few data are available in the literature regarding the use of continuous veno-venous haemofiltration (CVVH) in this clinical context. The aim of our observational study was to evaluate the impact of CVVH strategy on ARF in conjunction with cardiogenic shock after cardiac surgery and on its well-known associated poor outcome. METHODS: During the period 2005-2006, we prospectively collected data from our database as we controlled the renal replacement therapy using CVVH (n=73). We also retrospectively collected data from our computerised database on patients who were treated with IRRT (n=68, period 2002-2003). Among CVVH-treated patients, a multivariate analysis of the data aimed to identify risk factors associated with 30-day mortality. RESULTS: In patients who presented with ARF in conjunction with cardiogenic shock after cardiac surgery, 30-day mortality rate was 59% for the IRRT group and 42% for the CVVH group. Within the CVVH group, the logistic regression and multivariate analyses reported that some variables were associated with higher mortality risk: a score F concerning the urinary output criteria of the RIFLE (risk, injury, failure, loss, end-stage kidney disease) classification (for scores R or I: odds ratio (OR): 0.01, 95% confidence interval (95% CI): 0.02-0.59; p=0.01), plasma bilirubin (OR: 1.44, 95% CI: 1.12-1.84; p=0.04), total CVVH duration <50h over 72 h (>50h; OR: 0.009, 95% CI: 0.04-0.93; p=0.01), the need of catecholamine support (OR: 12.88, 95% CI: 1.95-84.96; p=0.01), tachycardia in the intensive care unit (ICU; OR: 1.64, 95% CI: 1.02-2.65; p=0.04), surgery duration (<300 min; OR: 0.11, 95% CI: 0.02-0.71; p=0.02) and combined cardiac surgery (OR: 7.00, 95% CI: 1.29-37.88; p=0.02). CONCLUSION: In patients with ARF in conjunction with cardiogenic shock after cardiac surgery, renal replacement therapeutic strategy based on long-lasting CVVH could improve patients' outcome. The identification of risk factors associated with a poor outcome would help to better manage such patients in the ICU. Low total duration of CVVH within the first 72 h was one criteria related to poor outcome. This suggests that CVVH must be initiated as soon as possible when ARF with diuretic resistance occurs in patients after cardiac surgery and continued as long as possible for the first 3 days.