Berlin Heart EXCOR Paediatric Ventricular Assist Device: Does Weight Matter?

BACKGROUND: Berlin Heart EXCOR (BH) ventricular assist devices provide mechanical long-term circulatory support in children with end-stage heart failure, as a bridge to transplantation or to recovery. Most studies are from large-volume paediatric cardiac centres. AIM: The aim of this study was to analyse the experiences of three French centres and to compare these with available published data. METHOD: We performed a retrospective observational study of three paediatric cardiac intensive care units. All children supported with BH devices were included. Morbidity and mortality data were collected and risk factors analysed. RESULTS: Fifty-four (54) patients (54% male) were included. Survival rate was 73% while on a BH device. Median age at BH device implantation was 17 months (range 2-180 months). The predominant indication was dilated cardiomyopathy (61%). Bi-ventricular assist device was used in 25 (46%) cases. The total length of long-term circulatory support was 3,373 days, with a mean length per patient of 62.5 days (range 5-267 days). Thirty-two (32) patients were transplanted (59%) and seven (13%) were successfully weaned. Type and length of support did not influence morbidity. Main complications were renal dysfunction (57%), bleeding (41%), and infection (39%). In multivariate analysis, a weight <5 kg was significantly associated with higher mortality. CONCLUSIONS: The weight seems to be the most important risk factor of mortality in this precarious condition.

Pulmonary atresia with ventricular septal defect and tetralogy of Fallot: transannular path augmentation versus systemic to pulmonary artery shunt for first-stage palliation.

BACKGROUND: Pulmonary atresia with ventricular septal defect and severe tetralogy of Fallot require a palliative procedure for pulmonary artery rehabilitation. For first-stage palliation, two main surgical options are still debated: right ventricle to pulmonary artery connection and modified Blalock-Taussig shunt. We compared the clinical outcomes of the two procedures. METHODS: From 1995 to 2018, 88 patients needed palliation (pulmonary atresia with ventricular septal defect n = 47; tetralogy of Fallot n = 41). Among these patients, 70 modified Blalock-Taussig shunt and 18 transannular path augmentation were performed before 6 months of age. Using a 1:1 propensity score match analysis, 20 patients were included in the analysis. The primary outcome was in-hospital mortality and pulmonary artery growth. RESULTS: After matching, the pre-operative Nakata was smaller in transannular path augmentation 54 ± 24 mm2/m2 than modified Blalock-Taussig shunt 109 ± 31 mm2/m2 (p < 0.001). The age and weight were similar (p = 0.31 and p = 0.9, respectively). There was no difference in in-hospital mortality (p = 0.3). The Nakata index before biventricular repair and delta Nakata were smaller in modified Blalock-Taussig shunt group (206 ± 80 mm2/m2, 75 ± 103 mm2/m2) than transannular path augmentation (365 ± 170 mm2/m2, 214 ± 165 mm2/m2; p = 0.03; p < 0.001). Median time to biventricular repair was similar (p = 0.46). The rate of interstage reintervention was similar (p = 0.63). CONCLUSIONS: The transannular path augmentation is better for the rehabilitation of the native pulmonary artery. Despite a smaller pulmonary artery, right ventricle to pulmonary artery connection is equivalent to modified Blalock-Taussig shunt for rate of biventricular repair and time to biventricular repair.

FOXC1 haploinsufficiency due to 6p25 deletion in a patient with rapidly progressing aortic valve disease.

6p25 deletion is a rare but well-known entity. The main clinical features include an abnormal facial appearance, developmental delay, and ocular anomalies. Cardiac anomalies are frequently seen but remain poorly delineated. We describe a 4-year-old girl with 6p25.3 deletion, which includes the FOXC1 gene, typical dysmorphic features associated with developmental delay and oculo-motor anomalies. Aortic valve dysplasia was diagnosed early in life. The cardiac lesion progressed very rapidly between the age of 3 and 4 years requiring aortic valve replacement. Genomic analysis of blood and excised valve tissue showed down-regulation of FOXC1 but also FOXC2 expression in the diseased aortic valve. This allows us to speculate on the potential role of FOXC1 in aortic valve anomalies.

Predictive value of E/A and E/E' Doppler indexes for cardiac events in heart transplant recipients.

BACKGROUND: Doppler-derived indexes associated with high left ventricular filling pressures are risk factors for cardiac events in various populations. The aim of this study was to evaluate the predictive value of two of these Doppler indexes during the long-term follow-up of heart transplantation. METHODS AND RESULTS: In this cohort study, we measured E/A ratio combining early filling velocity (E) with late filling velocity (A) and E/E' ratio combining E wave with relaxation velocity on tissue doppler (E') in 122 transplant heart recipients, 6.9±5.9 years after transplantation. The patients were followed for 97±32 months after Doppler measurements. We found an E/A>2 in 68 patients and an E/E'>15 in 23 patients. Forty patients had a cardiac event (cardiac death, retransplantation, acute coronary events, hospitalization for heart failure, treated acute rejection episodes, and coronary revascularization procedures) during the follow-up. In multivariate analysis, E/A and E/E' were significantly associated with cardiac events (hazard ratio 2.2, 95% CI: 1.1-4.4; P=.02 and hazard ratio 2.3, 95% CI: 1.1-4.8; P=.02, respectively). E/E', E/A, and significant coronary stenoses were the strongest predictors of cardiac events. CONCLUSIONS: E/A and E/E' Doppler indexes may be used to predict cardiac events during the long-term follow-up of heart transplant recipients.

Analysis of Fluid-Structure Interaction Mechanisms for a Native Aortic Valve, Patient-Specific Ozaki Procedure, and a Bioprosthetic Valve.

The Ozaki procedure is a surgical technique which avoids to implant foreign aortic valve prostheses in human heart, using the patient's own pericardium. Although this approach has well-identified benefits, it is still a topic of debate in the cardiac surgical community, which prevents its larger use to treat valve pathologies. This is linked to the actual lack of knowledge regarding the dynamics of tissue deformations and surrounding blood flow for this autograft pericardial valve. So far, there is no numerical study examining the coupling between the blood flow characteristics and the Ozaki leaflets dynamics. To fill this gap, we propose here a comprehensive comparison of various performance criteria between a healthy native valve, its pericardium-based counterpart, and a bioprosthetic solution, this is done using a three-dimensional fluid-structure interaction solver. Our findings reveal similar physiological dynamics between the valves but with the emergence of fluttering for the Ozaki leaflets and higher velocity and wall shear stress for the bioprosthetic heart valve.

Three-dimensional modelling of aortic leaflet coaptation and load-bearing surfaces: in silico design of aortic valve neocuspidizations.

OBJECTIVES: Three-dimensional (3D) modelling of aortic leaflets remains difficult due to insufficient resolution of medical imaging. We aimed to model the coaptation and load-bearing surfaces of the aortic leaflets and adapt this workflow to aid in the design of aortic valve neocuspidizations. METHODS: Geometric morphometrics, using landmarks and semilandmarks, was applied to the geometric determinants of the aortic leaflets from computed tomography, followed by an isogeometric analysis using Non-Uniform Rational Basis Splines (NURBS). Ten aortic valve models were generated, measuring determinants of leaflet geometry defined as 3D NURBS curves, and leaflet coaptation and load-bearing surfaces were defined as 3D NURBS surfaces. Neocuspidizations were obtained by either shifting the upper central coaptation landmark towards the sinotubular junction or using parametric neo-landmarks placed on a centreline drawn between the centroid of the aortic root base and centroid of a circle circumscribing the 3 upper commissural landmarks. RESULTS: The ratio of the leaflet free margin length to the geometric height was 1.83, whereas the ratio of the commissural coaptation height to the central coaptation height was 1.93. The median coaptation surface was 137 mm2 (IQR 58) and the median load-bearing surface was 203 mm2 (60) per leaflet. Neocuspidization multiplied the central coaptation height by 3.7 and the coaptation surfaces by 1.97 and 1.92 using the native coaptation axis and centroid coaptation axis, respectively. CONCLUSIONS: Geometric morphometrics reliably defined the coaptation and load-bearing surfaces of aortic leaflets, enabling an experimental 3D design for the in silico neocuspidization of aortic valves.

Partial Atrioventricular Septal Defect with Left Atrioventricular Valve Aneurysm Mimicking Valve Perforation.

Left atrioventricular valve aneurysm is a rare condition. Here we present a rare case of partial atrioventricular septal defect with an extremely thin left atrioventricular valve aneurysm mimicking valve perforation. Preoperative echocardiography demonstrated severe left sided atrioventricular valve regurgitation on the "cleft" and leaflet perforation. But we discovered a left sided atrioventricular valve aneurysm instead of a valve perforation. The "cleft" edge and the aneurysm were closed.

Right Ventricular Outflow Tract Reconstruction in Truncus Arteriosus: A 30-Year Two-Center Comparison between Homografts and Bovine Jugular Vein.

INTRODUCTION: Homografts and bovine jugular vein are the most commonly used conduits for right ventricular outflow tract reconstruction at the time of primary repair of truncus arteriosus. METHODS: We reviewed all truncus patients from 1990 to 2020 in two mid-volume centers. Inclusion criteria were primary repair, age under one year, and implantation of either homograft or bovine jugular vein. Kaplan-Meier analysis was used to estimate survival, freedom from reoperation on right ventricular outflow tract, and freedom from right ventricular outflow tract reoperation or catheter intervention. RESULTS: Seventy-three patients met the inclusion criteria, homografts were implanted in 31, and bovine jugular vein in 42. There was no difference in preoperative characteristics between the two groups. There were 25/73 (34%) early postoperative deaths and no late deaths. Follow-up for survivals was 17.5 (interquartile range 13.5) years for homograft group, and 11.5 (interquartile range 8.5) years for bovine jugular vein group (P=0.002). Freedom from reoperation on right ventricular outflow tract at one, five, and 10 years in the homograft group were 100%, 83%, and 53%; and in bovine jugular vein group, it was 100%, 85%, and 50% (P=0.79). There was no difference in freedom from reoperation or catheter intervention (P=0.32). CONCLUSION: Bovine jugular vein was equivalent to homografts up to 10 years in terms of survival and freedom from right ventricular outflow tract reoperation or catheter intervention. The choice of either valved conduit did not influence the durability of the right ventricle-pulmonary artery conduit in truncus arteriosus.

Ventricular septal defect with anomalous origin of the left descending coronary artery from the right sinus of Valsava and an intraseptal course.

Patients with anomalous aortic origin of the left anterior descending coronary artery (AAOCA) from the right sinus of Valsava, and associated with a trans-septal course, are recommended for surgery only when symptoms of ischemia are present. The transconal unroofing method is straightforward and provides good anatomic result. In absence of significant coronary compression, surgical management of the trans-septal coronary course is proposed if the patient is a candidate to cardiac surgery for another reason, such as congenital heart disease. We describe a transconal approach in a patient with a trans-septal coronary artery and a ventricular septal defect.

Cryopreserved homograft in the Ross procedure: outcomes and prognostic factors.

BACKGROUND AND AIM OF THE STUDY: The study aim was to determine the dynamics and risk factors related to cryopreserved homografts implanted to reconstruct the right ventricular outflow tract (RVOT) during the Ross procedure. METHODS: A retrospective study, conducted between 1993 and 2009, included 107 consecutive patients who underwent a Ross procedure with implantation of a homograft (102 pulmonary, five aortic). The median patient age at implant was 18.8 years (range: 2 months - 67 years). The main cardiac defects were congenital aortic stenosis (n = 44), congenital aortic regurgitation (n = 20), and combined (n = 23) and acquired (n = 20) aortic regurgitation. The study endpoints were homograft stenosis (peak gradient > or = 20 mmHg), regurgitation (grade > or = moderate), homograft dysfunction (stenosis > or = 50 mmHg or regurgitation > or = moderate), homograft explant, and failure (explant or balloon dilation). The mean follow up was 5.7 +/- 0.4 years. The statistical analysis was conducted using univariate and multivariate Cox regression tests. RESULTS: The overall patient survival was 97% at 18 years. Homograft stenosis occurred in 54% of patients, regurgitation in 18%, dysfunction in 27%, explant in 5%, and failure in 6%. Freedom from homograft dysfunction was 75% and 55% at five and 10 years, respectively, while freedom from homograft failure was 99% and 84% at five and 10 years, respectively. The main multivariate risk factors for dysfunction were: homograft diameter < 22 mm (OR: 11, p = 0.019), aortic homograft (OR: 18, p = 0.019) and preoperative right ventricle/left ventricle pressure ratio > 0.4 (OR: 5, p = 0.018). Univariate risk factors for failure were: homograft diameter < 22 mm (p = 0.006), donor age < 30 years (p = 0.03), cold ischemia time < 2 days (p = 0.04), and decontamination time < 12 h (p = 0.01). CONCLUSION: In the Ross procedure, the cryopreserved homograft represents an excellent means to reconstruct the RVOT, with a good long-term longevity. Almost 85% of patients did not require any surgery or percutaneous intervention at 10 years after implantation. The homograft long-term durability depends on both homograft-related and patient-related factors. Pulmonary homografts, with a diameter > 22 mm, a donor age > 30 years, and a cold ischemia time > 2 days are to be preferred.

Right ventricular remodelling after endo-exclusion during pulmonary valve replacement: evaluation by cardiac magnetic resonance.

OBJECTIVES: Pulmonary valve replacement (PVR) performed for pulmonary valve regurgitation is the most common indication for reoperation during mid-to-long-term follow-up after tetralogy of Fallot repair. An aneurysmal dilation of the infundibulum is often associated secondary to the infundibulotomy performed in the first operation. The right ventricular outflow tract reconstruction with endo-exclusion aims to exclude the non-contractile segments of the dilated right ventricular. This study intends to assess the safety and efficiency of the endo-exclusion technique. METHODS: Between January 2010 and December 2018, 86 patients underwent a PVR with (n = 46) or without (n = 40) endo-exclusion. The current study compares the outcomes in terms of survival, reintervention, structural valve deterioration, right ventricular function (volume and right ventricular ejection fraction) and pulmonary valve gradient. The median follow-up time was 4.45 years (1.9 months to 9.87 years). RESULTS: There was no 30-day mortality. There was no difference in the freedom from reintervention at 7 years (without endo-exclusion, 97%, versus with endo-exclusion, 94%, log-rank = 0.68) or in the freedom from structural pulmonary valve deterioration at 7 years (without endo-exclusion, 94%, versus with endo-exclusion, 89%, log-rank = 0.94). No significant difference was observed in the indexed right ventricular end-diastolic volume (102.2 ± 34 ml/m2 in the PVR without endo-exclusion group and 93.3 ± 22 ml/m2 in the PVR with endo-exclusion group, P = 0.61). No significant difference was observed in the right ventricular function (right ventricular ejection fraction: 46 ± 11% in the PVR without endo-exclusion group and 46 ± 9% in the PVR with endo-exclusion group, P = 0.88). CONCLUSIONS: PVR with or without endo-exclusion is a safe and effective procedure. PVR with endo-exclusion allows implantation without structural deformation of the valve and therefore excellent short- and medium-term results.

Anterograde blood flow associated with modified Blalock-Taussig shunt does not modify pulmonary artery growth compared with modified Blalock-Taussig shunt alone.

BACKGROUND: The main difference between extreme tetralogy of Fallot (TOF) and pulmonary atresia with ventricle septal defect (PA/VSD) is anterograde pulmonary blood flow (APBF). It is speculated that the association of modified Blalock-Taussig shunt (mBTS) with APBF favours shunt thrombosis, but promotes better pulmonary artery growth. AIM: To compare pulmonary artery growth after mBTS between TOF and PA/VSD. METHODS: From 1995 to 2018, 77 mBTS procedures were performed in infants (aged<1 year): 45 for TOF; 32 for PA/VSD. Using a 1/1 propensity score-matched analysis, 38 patients were included (19 per group). Delta Nakata was defined as the difference in the Nakata index before biventricular repair and before mBTS. RESULTS: After matching, the preoperative Nakata index was similar in the two groups (TOF 101±34 vs. PA/VSD 106±35 mm2/m2; P=0.75). Age and weight were similar (TOF 24±20 days, 3.3±0.6kg vs. PA/VSD 24±33 days, 3.3±0.9kg; P=0.84 and P=0.77, respectively). There was no difference in rates of in-hospital mortality (TOF 0% vs. PA/VSD 10%; P=0.13) or mBTS thrombosis (TOF 15% vs. PA/VSD 10%; P=0.63). The left and right pulmonary artery diameters at time of biventricular repair were similar (TOF 7.5±2.2 and 6.7±2.1 vs. PA/VSD 8±2.7 and 7.1±2.5mm; P=0.43 and P=0.78, respectively), as were delta Nakata (TOF 112±102 vs. PA/VSD 107±66 mm2/m2; P=0.89), median age for biventricular repair (P=0.83) and reintervention rates (TOF 10% vs. PA/VSD 15%; P=0.67). CONCLUSIONS: We found no difference in pulmonary artery growth between APBF with mBTS versus mBTS alone. Thus, we could not show an increase in mBTS thrombosis with APBF.

Predictive value of achieved blood pressure for cardiac events in the long-term follow-up of heart transplant recipients.

BACKGROUND: A low diastolic blood pressure (DBP) is associated with increased cardiovascular events in patients with coronary artery disease or chronic kidney disease. AIM: The aim of this study was to assess the association of blood pressure with cardiac events during the long-term follow-up of heart transplant recipients. PATIENTS AND METHODS: In this prospective cohort study, we performed ambulatory blood pressure monitoring and home blood pressure monitoring in 76 transplant heart recipients 13.5±6.6 years after transplantation. The patients were followed for 54±17 months after blood pressure monitoring. RESULTS: Twenty-one patients had a cardiac event (cardiac death, acute coronary event, coronary revascularization procedure, and hospitalization for heart failure) during the follow-up. In the Kaplan-Meier survival analysis, we found that a DBP below the median value (<81 mmHg) was associated significantly with cardiac events (log-rank: P=0.01). In a multivariate model, plasma creatinine and left ventricular ejection fraction (LVEF), but not DBP, were associated significantly with cardiac events. Low DBP was associated significantly with LVEF less than 55% (P=0.004). CONCLUSION: A DBP below the median value predicts cardiac events during the long-term follow-up of heart transplant recipients, but is not an independent predictor. The association between low DBP and low LVEF explains at least in part the predictive value of low DBP.

Impact of a centre and home-based cardiac rehabilitation program on the quality of life of teenagers and young adults with congenital heart disease: The QUALI-REHAB study rationale, design and methods.

BACKGROUND: Advances in congenital heart disease (CHD) have transferred the mortality from childhood to adulthood. Exercise capacity in young patients with CHD remains lower than in the general population, resulting in deconditioning and impaired quality of life. Evidence based-medicine in cardiac rehabilitation in this age group with CHD remains limited. We present the QUALI-REHAB study rationale, design and methods. METHODS: The QUALI-REHAB trial is a nationwide, multicentre, randomised, controlled study, aiming to assess the impact of a combined centre and home-based cardiac rehabilitation program on the quality of life of adolescents and young adults (13 to 25 years old) with CHD. Patients with a maximum oxygen uptake (VO2max) < 80% and/or a ventilatory anaerobic threshold (VAT) < 55% of predicted VO2max, will be eligible. Patients will be randomised into 2 groups (12-week cardiac rehabilitation program vs. controls). The primary outcome is the change in the PedsQL quality of life score between baseline and 12-month follow-up. A total of 130 patients are required to observe a significant increase of 7 ±â€¯13.5 points in the PedsQL, with a power of 80% and an alpha risk of 5%. The secondary outcomes are: VO2max, VAT, stroke volume, clinical outcomes, physical and psychological status, safety and acceptability. CONCLUSION: After focusing on the survival in CHD, current research is opening on secondary prevention and patient-related outcomes. The QUALI-REHAB trial intends to assess if a combined centre and home-based rehabilitation program, could improve the quality of life and the exercise capacity in youth with CHD. TRIAL REGISTRATION: Clinicaltrials.gov (NCT03690518).

Surgical management of children presenting with surgical-needed tracheal stenosis.

OBJECTIVES: The purpose of this work was to assess epidemiological aspects, surgical approach, morbidity and mortality rates of patients presenting with tracheal stenosis requiring surgery, and the evolution of surgical techniques over the last years. METHODS: We performed a retrospective observational study from 1990 to 2017 in a pediatric tertiary-care center with needing surgery for tracheal stenosis. We analyzed clinical patients' characteristics, type of stenosis, type of surgery and follow-up. RESULTS: Twenty-eight children presented with tracheal stenosis, half of them with congenital stenosis (complete tracheal rings) and the other half with acquired stenosis (neoplasic or post intubation injury). 39.3% of these stenoses were associated with a vascular ring (61.5% in case of congenital stenosis). Depending on the extent of the stenosis and its origin, the surgery could be performed endoscopically or by an external approach. Enlargement tracheoplasty with an autograft (14.3%) was replaced by slide tracheoplasty with Cardio Pulmonary By-Pass (CPBP, 28.6%) with improved results for the treatment of long segment tracheal stenosis, involving more than 30% of the tracheal length (all were congenital in our study). Slide tracheoplasty has been performed since the late 90's in our institution. 25% of children have had a resection and anastomosis of the trachea because they had a stenosis involving less than 30% of tracheal length. Endoscopic surgery was performed for membranous stenoses, which were often seen after intubation or tracheotomy (32.1% of patients). CONCLUSION: Effective treatment of surgical tracheal stenosis was performed in 28 children between 1990 and 2015. Surgical techniques have evolved over time, leading to a better management of this rare and serious disease.

Partial Atrioventricular Septal Defect with Left Atrioventricular Valve Aneurysm Mimicking Valve Perforation

ABSTRACT Left atrioventricular valve aneurysm is a rare condition. Here we present a rare case of partial atrioventricular septal defect with an extremely thin left atrioventricular valve aneurysm mimicking valve perforation. Preoperative echocardiography demonstrated severe left sided atrioventricular valve regurgitation on the "cleft" and leaflet perforation. But we discovered a left sided atrioventricular valve aneurysm instead of a valve perforation. The "cleft" edge and the aneurysm were closed.

Right Ventricular Outflow Tract Reconstruction in Truncus Arteriosus: A 30-Year Two-Center Comparison between Homografts and Bovine Jugular Vein

ABSTRACT Introduction: Homografts and bovine jugular vein are the most commonly used conduits for right ventricular outflow tract reconstruction at the time of primary repair of truncus arteriosus. Methods: We reviewed all truncus patients from 1990 to 2020 in two mid-volume centers. Inclusion criteria were primary repair, age under one year, and implantation of either homograft or bovine jugular vein. Kaplan-Meier analysis was used to estimate survival, freedom from reoperation on right ventricular outflow tract, and freedom from right ventricular outflow tract reoperation or catheter intervention. Results: Seventy-three patients met the inclusion criteria, homografts were implanted in 31, and bovine jugular vein in 42. There was no difference in preoperative characteristics between the two groups. There were 25/73 (34%) early postoperative deaths and no late deaths. Follow-up for survivals was 17.5 (interquartile range 13.5) years for homograft group, and 11.5 (interquartile range 8.5) years for bovine jugular vein group (P=0.002). Freedom from reoperation on right ventricular outflow tract at one, five, and 10 years in the homograft group were 100%, 83%, and 53%; and in bovine jugular vein group, it was 100%, 85%, and 50% (P=0.79). There was no difference in freedom from reoperation or catheter intervention (P=0.32). Conclusion: Bovine jugular vein was equivalent to homografts up to 10 years in terms of survival and freedom from right ventricular outflow tract reoperation or catheter intervention. The choice of either valved conduit did not influence the durability of the right ventricle-pulmonary artery conduit in truncus arteriosus.