Archives of Cardiovascular Diseases Supplements最新文献

Introduction

Balloon atrial septostomy (BAS) improves oxygenation in neonates with Transposition of the Great Vessels (TGV). New non-EC marked BAS catheters have obtained temporary approval in France.

Objective

We aim to investigate outcomes of neonatal BAS in the current area.

Methods

An on-going multi-center French prospective observational registry was set up in September 2022 including all neonates undergoing BAS for TGV.

Results/Expected results

To date, 60 neonates (93.5% of term-babies, weight: 3.1 [2.8–3.4] Kg, prenatal diagnosis: 88.5%, isolated form of TGV: 68.9%) underwent 62 BAS (50% on general anaesthesia) in 14 participating centres. The procedure was performed over night or during weekend in 39.3% of cases. BAS was performed most often the day of birth (65.6%), in the catheter lab (59%) using an umbilical venous route (67.2%). The latter required conversion to a femoral route in 22.0% of patients. BAS was guided by ultrasounds and fluoroscopy in 90.2% and 55.7% of cases, respectively. Two to 4 BAS manoeuvre were required in 82.0% of cases. In 14.8% of patients, the use of a second type of BAS catheter was required. Z5 and Z6 catheters were used in 45.6% and 54.4% of cases, respectively. Overall, BAS was successfully achieved in 93.5% of cases. BAS failures included 3 crossing failures (n = 3) and a failure of enlargement of the atrial septum with a low-volume Z5 catheter (n = 1). One transient complete atrio-ventricular block was also reported.

Conclusion/Perspectives

To date, BAS success rate seems lower than expected. The continuation of this study and its expected European extension will likely contribute to clarify these points in a context of world shortage in dedicated low profile/high volume catheters.

Introduction

Appropriate communication between physicians and parents is key when announcing a diagnosis of critical congenital heart disease (CHD) in the perinatal period. However, young physicians often struggle with anxiety and self-confidence, hindering their ability to communicate adequately. Simulation-based learning has demonstrated efficacy in improving medical training, but its application in communication training is limited.

Objective

The aim of this study is to evaluate whether simulation-based training can improve the communication skills of paediatric cardiology fellows in announcing a diagnosis of critical CHD. The study will be implemented in collaboration with the associations of children's parents with CHD.

Methods

A randomized controlled trial will be conducted to compare the effect of simulation-based training versus theoretical training alone. The simulation group will receive both theoretical and practical training, while the control group will only receive theoretical training. The primary endpoint is to evaluate the effect of simulation-based training on the announcement of CHD diagnosis paediatric cardiologists in training. The primary endpoint will be measured by the difference in proficiency score (BBN Skills) for announcing the diagnosis, video judged by a panel of experts, and blinded to group assignment. The study will also assess self-assessment scores of anxiety and self-confidence before making the announcement.

Results/Expected results

It is expected to show an improvement in communication skills among the simulation group, regardless of the level of young doctors, as well as likely showing a decrease in anxiety and an increase in confidence scores in the simulation group.

Conclusion/Perspectives

This nationwide study could provide a uniform curriculum for training, written in collaboration with patients’ associations involving expert parents that may be further integrated into the national graduate program. It could then easily be applied to other clinical situations, for example, when announcing poor surgical outcomes, a treatment limitation, etc.

Introduction

Cardiac surgeries with cardiopulmonary bypass (CPB) cause a complex systemic immuno-inflammatory response, which can lead to postoperative morbidity, particularly in young children. A better understanding of mechanisms involved is needed to improve the outcome. In a whole blood transcriptome study, we have recently identified 2,175 differentially expressed genes after CPB, mainly associated with the immune response. A group of 24 co-expressed immune-related genes correlated with postoperative complications was also identified.

Objective

To validate the transcriptomic analysis data by flow cytometry.

Methods

Peripheral blood mononuclear cells (PBMC) have been isolated from whole blood (4 ml EDTA tube) before and immediately after CPB in children less than 3 months of age after obtaining parental consent. The extensive phenotype of myeloid and lymphoid cells and their ability to secrete cytokines after in vitro stimulation were assessed using flow cytometry. Surface expression of proteins encoded by 5 of 24 co-expressed immune-related genes (VSIG4, LILRB4, LTF, MSR1 and PGLYRP1) were also investigated.

Results/Expected results

Among 6 patients included (Table 1), we noted important phenotypic changes of immune cells after CPB. The expression of HLA-DR, CD11b, CD86 in CD14 + cells (monocytes) and CD3 + lymphocytes function (PD1, ICOS) are decreased on their surfaces after CPB (Fig. 1). After lipopolysaccharide (LPS) in vitro stimulation, monocytes display an altered ability to produce IL1β after CPB (Median Fluorescence Intensity MFI : 2620 1610 - 3975 vs 3786 3200 - 7538, p = 0.03). Finally, as suggested by transcriptomic analysis, monocytic expression of some immune-related proteins (MSR1 and VSIG4) is modified by CPB.

Conclusion/Perspectives

We reported an immediate immune dysfunction after CPB, concerning myeloid and lymphoid cells. The involvement of MSR1 and VSIG4 genes, previously unknown in the CPB-related inflammation, need to be further explored to improve post-operative outcome.

Introduction

Cardiac catheterization (CC) has largely improved the diagnosis and treatment of congenital heart disease in children over the last decades, but it leads to exposure to low doses of X-rays. Children have a greater radiation sensitivity, however, little is known about the long-term radiation associated cancer risks.

Objective

This study aims to assess the risk of lympho-hematopoietic malignancies among children diagnosed and/or treated with CC.

Methods

The COCCINELLE cohort includes children from 15 French paediatric cardiology departments who underwent a first CC between 2000–2013, before 16 years old. The cohort was linked with national registries to identify cancer cases and collect information on predisposing factors to cancer (PF). Individual cumulative doses to active bone marrow (ABM) were calculated with the PCXMC software, based on detailed dosimetric information retrieved from reports of 1,139 CC procedures. Standardised incidence ratios (SIRs) and relative risks (RR) of haematological malignancies associated with the 2-year lagged ABM dose were calculated.

Results/Expected results

The cohort included 17,104 children, followed 6.5 years on average. 22,227 CC procedures were collected, with a mean individual ABM cumulative dose of 3.0 mGy. 38 lympho-hematopoietic malignancies were counted (23 lymphoma and 15 leukemia). A significant excess of cancer incidence was observed (SIR = 3.8 [95% confidence interval 2.9; 4.9]), which wasn’t anymore observed after excluding patients with PF (SIR = 1.3 [0.6; 2.7]). The ABM dose wasn’t significantly associated with the risk of lympho-hematopoietic malignancies (RR/mGy = 1.00 [0.88; 1.10]) or lymphoma (RR/mGy = 1.03 [0.90; 1.14]) after adjustment for attained age, gender and PF.

Conclusion/Perspectives

No significant increase of cancer incidence was observed after excluding patients with PF and no dose-response relationship was observed between the risk of lympho-hematopoietic malignancies or lymphoma and cumulative ABM dose arising from CC procedure. The extension of the COCCINELLE study and the European Harmonic project will help to better assess health effects of CC exposure during childhood.

Introduction

The risk of cancer in adults with congenital heart disease (ACHD) is 23% higher than in the general population.

Objective

The objective of our study was to assess cancer screening coverage in ACHD.

Methods

This was a prospective, observational, bicentric study of ACHD patients. Data were collected through a self-questionnaire proposed to all consecutive patients over 25 years from September 2022 to March 2023. Information on cervical, breast and colorectal cancer screening was collected and compared with screening data provided by Santé Publique France.

Results/Expected results

A total of 263 patients were included. The mean age was 44 years (range, 25–78), 122 (46%) patients were male, and 99 (38%) patients had a complex CHD. Eleven (4%) patients had a history of cancer. The screening participation rate for breast cancer was higher in ACHD patients (72%) compared to the general population from Ile de France region (39%, P < 0.001) and from the whole French population (51%, P = 0.002). For colorectal and cervical cancers, the screening participation rate were 36% and 65%, compared to 31% and 51% in Île de France region and 34% and 59% in the whole French population, respectively with no statistically significant difference (Figure 1).

Conclusion/Perspectives

Cancer screening coverage in ACHD patients was greater than non-congenital patients for breast cancer, but comparable for colorectal and cervical cancers. The screening participation rate remained however lower than European objectives for all cancers. Our data call for specific actions to better screen this aging population at high risk for cancer.

Introduction

Pulmonary valve replacement (PVR) is indicated for right ventricle outflow tract (RVOT) dysfunction. The choice between the transcatheter PVR (TPVR) and the surgical PVR (SPVR) is debated.

Objective

We aimed to compare the TPVR to the SPVR, considered previously as the gold standard, to determine the best choice for each patient.

Methods

A retrospective observational study was conducted in Lille University hospital. All patients who had PVR between January 2012 and May 2021 were included. The primary outcome was the incidence of valve failure defined as the occurrence of death, endocarditis or the need for secondary intervention.

Results

In total, 273 patients were included (100 patients with TPVR (36.6%) and 173 with SPVR (63.4%)). Age at the intervention was 25.1 ± 14.8 years for TPVR and 34.9 ± 15.7 years for SPVR. The outlet malformation was the main pathology (65% of TPVR and 61.3% of SPVR) followed by the Ross intervention (18% of TPVR and 11.6% of SPVR). The mean duration of the follow-up was 3.6 ± 2,8 years for TPVR and 3.9 ± 0,9 years for SPVR. Valve diameters were higher in the SPVR group (9% < 20 mm – 44% 20–22 mm and 47% > 22 mm for TPVR; and 3.6% < 22 mm and 96,4% > 22 mm for SPVR) and post-intervention transvalvular gradient was higher in the TPVR group.

The primary outcome was not significantly different in TPVR than in SPVR (16.33% vs. 8.7% respectively, P = 0.846), and the freedom from primary outcome at 9 years of follow up was 57% in TPVR and 84% in SPVR (Figure 1), after adjusting for predefined confounders (age at the intervention, size of the valve, and disease).

The risks factors for primary outcome were number of previous surgeries (P = 0.0190) and small size of the valve (threshold < 22 mm, hazard ratio 0.27, P = 0.0363).

Conclusion/Perspectives

The choice between TPVR and SPVR should be individualized based on each patient, their risk factors and the size of their right ventricle outflow tract. A larger size of the valve (> 22 mm if possible) may be preferable mainly in patients with several previous surgeries.

Introduction

Absence of connection of both coronary arteries to the aorta is an extremely rare congenital malformation. Most cases reported are anatomic variants of anomalous left coronary artery to pulmonary artery.

Objective

We describe here four cases of patients born without any coronary artery connecting connected to the aorta (Figure 1).

Results/Expected results

Case 1: a male infant was diagnosed with aortic coarctation and perimembranous ventricular septal defect (VSD). Left ventricular dysfunction appeared four months after coarctation repair and pulmonary artery banding. Heart catheterization showed no coronary artery in the aorta and a single coronary artery connected to the right pulmonary artery.

Case 2: a female neonate born with multiples VSD and aortic coarctation died during coarctation repair. At autopsy, there were no epicardial coronary arteries except a short segment in the posterior atrioventricular sulcus. Two small dimples were seen in aortic root. No proximal coronary artery connected to the aorta or the pulmonary arteries. Several intramyocardial arterial vessels of different sizes were detected, without any communication with the ventricles.

Case 3: a male neonate was born with pulmonary atresia with intact ventricular septum. Cardiac angiography revealed multiple coronary-ventricular fistulae and no coronary arteries from the aorta. Autopsy confirmed 1) absence of epicardial coronary arteries except a very short segment of left anterior descending and small fistulous apical segments 2) two large coronary-ventricular fistulas. There were no coronary ostia within the aorta or the pulmonary tree.

Case 4: a coronarography revealed a single coronary artery originating from the left ventricle just below the aortic valve in a 64-year old with syncope at exercise. Surgical reimplantation was performed with success.

Conclusion/Perspectives

Those exceptional coronary malformations have a poor prognosis and are often diagnosed at autopsy. Total absence of epicardial coronary arteries, described only once in the literature, leads us to reconsider current knowledge of human coronary artery development.

Introduction

The unique features of neonatal Ebstein's anomaly (EA) are the presence of pulmonary atresia, arterial duct (AD) patency, and the variability of pulmonary vascular resistance. Promoting early ductal closure after ruling out or relieving anatomic pulmonary obstruction might improve neonatal survival. Transcatheter ductal closure is an interesting option but can be challenging in tiny neonates with large tortuous AD due to the limited available sizes of low-profile approved devices.

Objective

We report on 2 consecutive term newborns with EA and large AD in whom mechanical AD stimulus during failed attempts of transcatheter closure led after 2 days to definitive closure.

Methods

Two consecutive term neonates with EA were approached from the femoral vein for an attempted ductal closure. An 8 mm-AVPII was implanted in the AD (pulmonary end: 6.3 mm, length 8.9 mm) of the first 9-day-old patient (3 kg) and was removed before release for important leak and device instability. Delivery of 10 mm AVPII was laborious and procedure was aborted after important bleeding. A MVP-9Q was implanted in the AD (pulmonary end: 7.6 mm, length 16.1 mm) of the second 14-day-old patient (2.8 kg) and was removed before release after immediate migration to the pulmonary artery trunk. The procedure was aborted in the absence of a larger low-profile occlusion device in the armamentarium.

Results/Expected results

Patients were sent back to the ICU for surveillance and a re-discussion of the management strategy. Follow-up ultrasound showed a spontaneous and progressive diminution in the AD size until complete closure two days postoperative with good follow-up outcomes.

Conclusion/Perspectives

Transcatheter closure should be attempted in similar cases requiring early ductal closure. Surgical ligation can be postponed a couple of days after failed interventions when clinically possible. Further studies seem interesting to evaluate whether mechanical stimulation of the ductal wall can be a useful alternative to initiate spontaneous ductal closure in pre-term and term neonates.

Introduction

The decision regarding optimal treatment for atrial septal defect (ASD) in adults should be given by a multidisciplinary ACHD team; the best treatment in the presence of right ventricular overload is timely closure, irrespective of age. So far, despite new percutaneous approach for sinus venosus ASD closure, surgery remains gold standard, performed via a median sternotomy on cardiopulmonary bypass.

Methods

Minimally invasive cardiac surgery offers the possibility to repair ASD with a video-assisted approach through a right mini thoracotomy. We report the use of a partial thoracoscopic, combining a small anterior thoracotomy to thoracoscopy in 3 adults of 20, 42 and 46 years old, 1 woman and 2 men.

Results/Expected results

Our female patient was offered a periaareolar incision. All 3 patients had a sinus venosus ASD with right ventricular enlargement. They had no pulmonary hypertension and all had one isolated right superior pulmonary vein connected to the superior vena cava. None had a persistent left superior vena cava draining to the coronary sinus and none had significant tricuspid regurgitation. All ASD were successfully repaired with a two-patch technique, by means of 2 ports positioned on the right chest wall to allow the insertion of surgical instruments and a small thoracotomy. Mean time of cardiopulmonary bypass was 188 minutes, mean time of aortic cross-clamp was 122 minutes and mean time of operation was 242 minutes. Post-operative mechanical ventilation duration averaged 309 minutes and the mean duration of ICU stay was less than 24 hours for all. No death, residual shunt, moderate tricuspid regurgitation or lung atelectasis was found at 2 months.

Conclusion/Perspectives

This partial thoracoscopic approach appears as a feasible and safe approach for surgical closure of sinus venosus ASD in adults. Furthermore, it seems to allow less pain and better cosmetic outcome, particularly in women, using a periareolar incision.